• Allergy & Immunology
    • 1

      Introduction to Clinical Immunology: Overview of the Immune Response, Autoimmune Conditions, and Immunosuppressive Therapeutics for Rheumatic Diseases

      By Steven K. Lundy, PhD; Alison Gizinski, MD; David A. Fox, MD
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      Introduction to Clinical Immunology: Overview of the Immune Response, Autoimmune Conditions, and Immunosuppressive Therapeutics for Rheumatic Diseases

      • STEVEN K. LUNDY, PHDResearch Assistant Professor, Division of Rheumatology, Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, MI
      • ALISON GIZINSKI, MDAssistant Professor, Division of Rheumatology, Department of Internal Medicine, University of Arkansas for Medical Sciences, Little Rock, AR
      • DAVID A. FOX, MDProfessor, Division of Rheumatology, Department of Internal Medicine, University of Michigan Medical School and Health System, Ann Arbor, MI

      The immune system is a complex network of cells and mediators that must balance the task of protecting the host from invasive threats. From a clinical perspective, many diseases and conditions have an obvious link to improper functioning of the immune system, and insufficient immune responses can lead to uncontrolled acute and chronic infections. The immune system may also be important in tumor surveillance and control, cardiovascular disease, health complications related to obesity, neuromuscular diseases, depression, and dementia. Thus, a working knowledge of the role of immunity in disease processes is becoming increasingly important in almost all aspects of clinical practice. This review provides an overview of the immune response and discusses immune cell populations and major branches of immunity, compartmentalization and specialized immune niches, antigen recognition in innate and adaptive immunity, immune tolerance toward self antigens, inflammation and innate immune responses, adaptive immune responses and helper T (Th) cell subsets, components of the immune response that are important targets of treatment in autoimmune diseases, mechanisms of action of biologics used to treat autoimmune diseases and their approved uses, and mechanisms of other drugs commonly used in the treatment of autoimmune diseases. Figures show the development of erythrocytes, platelets, lymphocytes, and other immune system cells originating from hematopoietic stem cells that first reside in the fetal liver and later migrate to the bone marrow, antigen–major histocompatibility complex recognition by T cell receptor control of T cell survival and activation, and Th cells as central determinants of the adaptive immune response toward different stimuli. Tables list cell populations involved in innate and adaptive immunity, pattern recognition receptors with known ligands, autoantibody-mediated human diseases: examples of pathogenic mechanisms, selected Food and Drug Administration–approved autoimmune disease indications for biologics, and mechanism of action of biologics used to treat autoimmune diseases.

       

      This review contains 3 highly rendered figures, 5 tables, and 64 references.

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    • 2

      Deficiencies of Innate and Adaptive Immunity

      By Soma Jyonouchi, MD; Kathleen E. Sullivan, MD, PhD
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      Deficiencies of Innate and Adaptive Immunity

      • SOMA JYONOUCHI, MDInstructor, Department of Pediatrics, Division of Allergy/Immunology, The Children’s Hospital of Philadelphia, Philadelphia, PA
      • KATHLEEN E. SULLIVAN, MD, PHDProfessor of Pediatrics, University of Pennsylvania School of Medicine, Chief, Division of Allergy/Immunology, The Children’s Hospital of Philadelphia, Philadelphia, PA

      Defects in B cell function, T cell function, and innate immunity comprise the majority of primary immune deficiencies. Each compartment has a characteristic set of archetypical features. Defects in B cell function are characterized by poor immunoglobulin production, which, in turn, leads to recurrent sinopulmonary infections. Defects in T cell function are characterized by delayed clearance of viruses, susceptibility to opportunistic infections, and a high rate of autoimmune disease. Defects of innate immunity are typically associated with early-onset, severe infections. This chapter describes defects in immunoglobulin production or function, T cell disorders, defects in Toll-like receptor (TLR) signaling, defects in the interleukin-12 (IL-12)/interferon-gamma signaling pathway, and defects of T helper type 17 (Th17) immunity. Tables outline specific pathogens that should alert the clinician to potential immune deficiency, provide a comparison of immunoglobulin production defects, and describe severe combined immune deficiency types. Figures include schematic representations of the TLR signaling pathway, the IL-12/interferon-gamma signaling pathway, and the Th17 immune response.

      This chapter contains 3 highly rendered figures, 3 tables, 72 references, and 5 MCQs.

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    • 3

      Allergic Response

      By Joud Hajjar, MD; Lawrence B. Schwartz, MD, PhD
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      Allergic Response

      • JOUD HAJJAR, MDAllergy and Immunology Fellow, Department of Internal Medicine, Virginia Commonwealth University, Richmond, VA
      • LAWRENCE B. SCHWARTZ, MD, PHDCharles & Evelyn Thomas Professor of Medicine, Department of Internal Medicine, Virginia Commonwealth University, Richmond, VA

      This chapter begins with a definition of allergic response and a discussion of the epidemiology of atopic disorders, as well as the development of IgE-mediated hypersensitivity. Subjects covered in a section on humoral and cellular mechanisms of allergic sensitization include antigen-presenting cells and sensitization, T cells, and IgE and IgE receptors. A discussion of mast cells and basophils includes information on mediators, biomarkers, eosinophils, and eosinophil mediators. Therapy of atopic disorders is also discussed. Figures depict inflammatory mechanisms in allergic inflammation, microscopy of a mast cell before and after the introduction of antigen, and mediators released by activated human mast cells. Tables outline selected cytokines and chemokines involved in IgE-mediated allergic inflammation, serum tryptase levels, examples of patterns of serum total tryptase elevations and interpretations, and a summary of therapeutic interventions for allergic diseases.
      This chapter contains 3 highly rendered figures, 4 tables, 84 references, 1 teaching slide set, and 5 MCQs.

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    • 4

      Allergic Rhinitis, Conjunctivitis, and Sinusitis

      By Robert Naclerio, MD
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      Allergic Rhinitis, Conjunctivitis, and Sinusitis

      • ROBERT NACLERIO, MDProfessor and Chief, Department of Surgery, Section of Otolaryngology, Head and Neck Surgery, University of Chicago, Chicago, IL

      Allergic rhinitis is an immunoglobulin E–mediated inflammatory response in the nose to foreign substances known as allergens. Allergic conjunctivitis is the ocular counterpart of allergic rhinitis, and the two often occur together. Sinusitis refers to inflammation of the sinuses, although the term rhinosinusitis is more accurate than the term sinusitis for the following reasons: sinusitis without rhinitis is rare; the mucosa of the nose and sinuses is contiguous, and most sinusitis starts in the lateral wall of the nose; and the symptoms of both entities overlap. This review details the epidemiology, etiology and pathophysiology, diagnosis, differential diagnosis, and management of allergic rhinitis, allergic conjunctivitis, and sinusitis. Figures show the paranasal sinuses, and computed tomography scans show an acute exacerbation of chronic sinusitis, an example of a unilateral right maxillary sinusitis that was caused by a sinus lift prior to a dental implant, a case of allergic fungal sinusitis, and a unilateral nasal polyp and opacification of the left maxillary sinus. Tables list a guideline for primary care physicians seeing a new rhinitis patient, combinations for added-on therapy of allergic rhinitis, considerations to review before applying guidelines for the treatment of allergic rhinitis, factors predisposing to sinusitis, and warning signs on physical examination.

      This review contains 5 highly rendered figures, 5 tables, and 51 references.

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    • 5

      Diagnostic and Therapeutic Principles in Allergy

      By Jonathan Tam, MD; Dorothy S. Cheung, MD; Mitchell H. Grayson, MD
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      Diagnostic and Therapeutic Principles in Allergy

      • JONATHAN TAM, MDInstructor, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI
      • DOROTHY S. CHEUNG, MDAssistant Professor of Pediatrics and Medicine, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI
      • MITCHELL H. GRAYSON, MDAssociate Professor of Pediatrics, Medicine, Department of Pediatrics, Microbiology and Molecular Genetics, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI

      By definition, allergy is an untoward physiologic event mediated by immune mechanisms, usually involving the interaction of an allergen with the allergic antibody, immunoglobulin E (IgE). This chapter begins with a brief description of the so-called “hygiene hypothesis,” which posits that greater exposure to infectious agents (bacterial endotoxins in particular) early in life reduces the likelihood of subsequent allergy. A section on the importance of a careful and thorough medical history follows, with a table presenting inhaled aeroallergens that cause rhinitis, conjunctivitis, and asthma. The physical examination of a patient with a suspected allergic illness is reviewed. A section on assays of IgE includes skin testing (i.e., epicutaneous and intradermal testing, with photographs, videos, and a table outlining the time before skin testing to stop antihistamines), serum-specific IgE testing (with a figure illustrating the test), basophil histamine release assay, and the interpretation of IgE test results. A section on treatment discusses environmental control; a table presents concepts on environmental control for allergy management. A discussion of pharmacologic agents begins with a figure illustrating mechanisms of action of medications used in allergic diseases and covers antihistamines and decongestants, anti-IgE therapy with bronchodilators, corticosteroids, inhaled corticosteroid/long-acting bronchodilator combinations, leukotriene antagonists, and theophylline. Information on immunotherapy is also presented.
      This chapter contains 4 highly rendered figures, 4 videos, 3 tables, 28 references, and 5 MCQs.

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    • 6

      Urticaria and Angioedema

      By Justin R. Chen, MD; David A. Khan, MD
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      Urticaria and Angioedema

      • JUSTIN R. CHEN, MDFellow Physician, Division of Allergy & Immunology, Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX
      • DAVID A. KHAN, MDProfessor of Internal Medicine and Pediatrics, Division of Allergy & Immunology, Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX

      Urticaria and angioedema are common diseases with diverse origins that constitute a substantial component of medical practice. Urticaria, or hives, refers to one or more areas of intensely pruritic papules or plaques with swelling of the superficial dermis (wheal) surrounded by local erythema (flare). Angioedema refers to deep dermal subcutaneous swelling that may manifest as swelling of the mucosa of the face, tongue, pharynx, larynx, or intestines that can be alarming and, in some cases, life threatening. These conditions are heterogeneous in their presentation and chronicity. Although allergies are responsible for some cases, autoimmunity and dysregulation of the bradykinin system often play a significant role, leading to challenging diagnostic and therapeutic dilemmas. This review discusses the epidemiology, natural history, pathophysiology, diagnosis, and treatment of acute and chronic urticaria and angioedema. Emphasis is placed on physical triggers, the role of proper laboratory testing, and alternative agents for refractory cases. Emerging therapies for hereditary and acquired angioedema syndromes are also covered. Tables list the causes of acute and chronic urticaria, an escalating treatment approach for difficult cases, and a comparison of available parenteral therapies specific to bradykinin-mediated angioedema. Figures illustrate the mechanisms of urticaria, photographs of typical presentations, and an evidence-based diagnostic algorithm for clinicians. 

       

      This review contains 9 highly rendered figures, 5 tables, and 100 references.

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    • 7

      Anaphylaxis

      By Cem Akin, MD, PhD
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      Anaphylaxis

      • CEM AKIN, MD, PHDAssociate Physician, Harvard Medical School, Brigham and Women’s Hospital, Department of Rheumatology, Immunology, and Allergy, Boston, MA

      Anaphylaxis, a serious allergic reaction, is rapid in onset and marked by flushing, urticaria, angioedema, pruritus, bronchospasm, and abdominal cramping with nausea, vomiting, and diarrhea. It is not uncommon; approximate lifetime prevalence of anaphylaxis was estimated to be 0.5 to 2% or possibly higher due to the common academic belief that the incidence of anaphylactic reactions is underreported. Rarely, anaphylaxis may cause death, most commonly from drugs, foods, and insect stings. This review covers the epidemiology, etiology, pathogenesis, diagnosis, clinical manifestations, treatment, and prognosis. Figures show inflammatory pathways in allergic inflammation and mast cell degranulation and pathways of activation. Tables list the diagnostic criteria, selected mast cell activators of possible clinical relevance, signs and symptoms that may be encountered according to tissue site, and common considerations in the differential diagnosis.

      This review contains ­2 highly rendered figures, 4 tables, and 73 references.

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    • 8

      Food Allergies

      By Matthew Greenhawt, MD, MBA, MSc
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      Food Allergies

      • MATTHEW GREENHAWT, MD, MBA, MSCAssistant Professor, Division of Allergy and Clinical Immunology, University of Michigan Medical School, University of Michigan Health System, Ann Arbor, MI

      Food allergy represents a rapidly growing public health problem in the United States and other westernized nations. Adverse reactions to foods are categorized as either immunologic or nonimmunologic reactions. This distinction is highly important but often confusing to patients and physicians unfamiliar with allergy, who may simply describe any adverse reaction to a food as an “allergy.” A food allergy is an immune-mediated, adverse reaction to one or more protein allergens in a particular food item involving recognition of that protein by specifically targeted IgE or allergen-specific T cells. This chapter discusses the definition, pathophysiology, epidemiology, testing, management, prognosis, and natural history of food allergy. Clinical manifestations are systematically covered, including cutaneous, respiratory, cardiovascular, and gastrointestinal reactions, as well as eosinophilic esophagitis, food protein–induced enterocolitis syndrome, and oral allergy syndrome. Emerging treatments such as food oral immunotherapy are also reviewed. Tables outline signs and symptoms of immediate hypersensitivity reactions to food, the prevalence of major food allergens in the United States, common patterns of cross-reactivity among foods, clinical criteria for the diagnosis of anaphylaxis, and clinical studies involving treatment for food allergies. Figures illustrate the classification of adverse reactions to food, esophageal histology, visual and radiographic features of eosinophilic esophagitis, and a food allergy action plan.
      This chapter contains 4 highly rendered figures, 5 tables, 82 references, and 5 MCQs.

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    • 9

      Drug Allergies

      By James L. Baldwin, MD; Aimee L. Speck, MD
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      Drug Allergies

      • JAMES L. BALDWIN, MDDivision Chief, Division of Allergy and Clinical Immunology, Department of Medicine, University of Michigan School of Medicine, Ann Arbor, MI
      • AIMEE L. SPECK, MDFellow, Division of Allergy and Clinical Immunology, University of Michigan School of Medicine, Ann Arbor, MI

      Adverse drug reactions (ADRs) are an important public health problem. An ADR is defined by the World Health Organization as an unintended, noxious response to a drug that occurs at a dose usually tolerated by normal subjects. The classification of ADRs by Rawlins and Thompson divides ADRs into two major subtypes: (1) type A reactions, which are dose dependent and predictable, and (2) type B reactions, which are uncommon and unpredictable. The majority of ADRs are type A reactions, which include four subtypes: overdosage or toxicity, side effects, secondary effects, and interactions. Type B reactions constitute approximately 10 to 15% of all ADRs and include four subtypes: drug intolerance, idiosyncratic reactions, pseudoallergic reactions, and drug hypersensitivity reactions. This chapter reviews the epidemiology of ADRs, risk factors for drug hypersensitivity reactions, the classification of drug reactions, diagnostic tests, reactions to specific drugs, and management of the patient with drug allergy. Figures illustrate drugs as haptens and prohaptens, the Gell and Coombs system, the four basic immunologic mechanisms for drug reactions, the chemical structure of different β-lactam antibiotics, penicillin skin testing, sulfonamide metabolism and haptenation, nonsteroidal antiinflammatory drug effects, and patient management. Tables outline the classification of ADRs, drugs frequently implicated in allergic drug reactions, and reagents and concentrations recommended for prick and intradermal skin testing.

      This review contains 8 highly rendered figures, 3 tables, and 83 references.

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    • 10

      Allergic Reactions to Hymenoptera

      By David B. K. Golden, MD, FACP
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      Allergic Reactions to Hymenoptera

      • DAVID B. K. GOLDEN, MD, FACPAssociate Professor of Medicine, Division of Allergy-Clinical Immunology, Johns Hopkins University School of Medicine, Baltimore, MD

      Allergic reactions to insect venom can occur in individuals of all ages and may be preceded by a number of uneventful stings. These allergic reactions can be fatal. In the United States, at least 40 deaths due to insect stings occur each year; this number may be higher as some unexplained deaths may be caused by insect stings. This review details the epidemiology, etiology, pathogenesis, diagnosis, differential diagnosis, and acute and preventive treatment for allergic reactions to Hymenoptera. Figures show the honeybee, the European hornet, the Eastern yellow jacket, the red imported fire ant, the paper wasp, and the appearance of a pustule resulting from the sting of a fire ant. Tables list the risk of systemic reactions and clinical recommendations based on reaction to previous stings and venom skin test or serum immunoglobulin E test results, stinging insects of the order Hymenoptera, risk factors for severe reactions to stings, and risk factors for relapse after discontinuing venom immunotherapy.

      This review contains 6 highly rendered figures, 4 tables, and 62 references.

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    • 11

      Igg4-related Disease and Retroperitoneal Fibrosis

      By John H. Stone, MD, MPH
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      Igg4-related Disease and Retroperitoneal Fibrosis

      • JOHN H. STONE, MD, MPHDirector, Clinical Rheumatology, Massachusetts General Hospital, Professor of Medicine, Harvard Medical School, Boston, MA

      IgG4-related disease (IgG4-RD) has been observed to affect almost every organ system, with consistent histopathologic findings across systems. IgG4-RD can mimic malignant, infectious, and inflammatory disorders; accordingly, consideration of the histopathologic features of tissue biopsies and rigorous clinicopathologic correlations are essential to avoid misdiagnosis. Since the early 2000s, IgG4-RD has increasingly been recognized as a cause of what was previously referred to as “idiopathic” retroperitoneal fibrosis (RPF), and this IgG4-related RPF is now considered to comprise an important subset of IgG4-RD. This review includes an overview of IgG4-RD and discusses the pathology, pathophysiology, and clinical manifestations of IgG4. IgG4-related RPF is also discussed in this review, with topics including IgG4-related RPF versus RPF of other causes, the differences between RPF and other subsets of IgG4-RD, and treatment of both IgG4-RD and IgG4-related RPF. Figures show the histopathology features of IgG4-RD, immunostaining of tissue for IgG4, IgG4-related RPF and chronic periaortitis, “Mikulicz disease”, IgG4-RD of the lung, IgG4-related renal disease, and type 1 (IgG4-related) pancreatitis. The table lists conditions known previously by other names that often fall within the spectrum of IgG4-RD.

      This review contains 7 highly rendered figures, 1 table, and 66 references.

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    • 12

      Disorders of Macrophages and Dendritic Cells

      By Alexei A. Grom, MD; Michael B. Jordan, MD, PhD; Jun Qin Mo, MD
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      Disorders of Macrophages and Dendritic Cells

      • ALEXEI A. GROM, MDAssociate Professor of Pediatrics, Division of Rheumatology, Cincinnati Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH
      • MICHAEL B. JORDAN, MD, PHDAssistant Professor of Pediatrics, Divisions of Immunobiology and Bone Marrow Transplantation and Immunodeficiency, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
      • JUN QIN MO, MDAssociate Professor of Pediatrics, Division of Pathology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH

      The mononuclear phagocytic system consists of dendritic cells (DCs) and monocytes/macrophages, historically referred to as histiocytes. Abnormal accumulation and behavior of these cells may lead to the development of a spectrum of diseases collectively known as histiocytoses. Clinically, histiocytic disorders comprise a wide variety of conditions that affect both children and adults, and range from benign skin lesions to rapidly progressive life-threatening systemic disorders. This chapter describes DCs and macrophages in detail, and examines dendritic cell–related disorders, both Langerhans cell-related (Langerhans cell histiocytosis) and non–Langerhans cell–related (juvenile xanthogranuloma, multicentric reticulohistiocytosis, and Erdheim-Chester disease), plus hemophagocytic lymphohistiocytosis (HLH), macrophage activation syndrome, and Rosai-Dorfman disease. Figures illustrate Langerhans cell histiocytosis, juvenile xanthogranuloma, hemorrhagic crusted patches, abdominal plaques and macerated eczematous patches, as well as disseminated red papules and plaques. Tables list the disorders of histiocytes and revised diagnostic guidelines for HLH. This chapter contains 76 references.

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    • 13

      Transplant Immunology: Basic Immunology and Clinical Practice

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      Transplant Immunology: Basic Immunology and Clinical Practice

      Engraftment of a transplanted organ into an allogeneic host triggers a cascade of immunologic responses in the host that are designed to facilitate graft rejection. Modern donor-to-host matching techniques and immunosuppression protocols have successfully tempered this natural immune response so that graft survival has dramatically improved. However, optimizing graft survival by precisely downregulating the host response to graft rejection while preserving host immune defenses against pathologic and infectious agents remains poorly understood and elusive in current clinical practice. This review discusses transplant immunology with respect to host versus graft and the basis of allorecognition, as well as clinical management of the transplanted allograft. Figures show human leukocyte antigen (HLA), direct allorecognition, T cell receptor and CD3, T cell–associated second messenger signaling pathway, CD8 molecules directly ligating class I HLAs and CD4 molecules directly binding HLA class II, detection of alloantibodies by enzyme-linked immunosorbent assay or flow cytometry, recipient-donor crossmatch, histopathology of kidney allograft with antibody-mediated rejection, and an algorithm for assessment and management of renal allograft rejection. Tables list costimulatory molecules and ABO blood group compatibility for solid-organ transplantation.

      This review contains 9 highly rendered figures, 2 tables, and 65 references.

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  • Cardiovascular Medicine
    • 1

      Approach to the Cardiovascular Patient

      By Catherine M. Otto, MD; David M. Shavelle, MD
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      Approach to the Cardiovascular Patient

      • CATHERINE M. OTTO, MDJ. Ward Kennedy-Hamilton Endowed Chair in Cardiology, Professor of Medicine, Departmentof Medicine, Division of Cardiology, University of Washington School of Medicine, Seattle, WA
      • DAVID M. SHAVELLE, MDAssociate Clinical Professor of Medicine, Keck School of Medicine at USC, Director, Interventional Cardiology Fellowship, Director, Cardiac Catheterization Laboratories, USC Medical Center, Los Angeles County, Los Angeles, CA

      The complete evaluation of the cardiovascular patient begins with a thorough history and a detailed physical examination. These two initial steps will often lead to the correct diagnosis and assist in excluding life-threatening conditions. The history and physical examination findings should be assessed in the overall clinical status of the patient, including the patient's specific complaints, lifestyle, comorbidities, and treatment expectations. This chapter discusses the cardiovascular conditions that frequently require evaluation: chest pain, dyspnea, palpitations, syncope, claudication, and cardiac murmurs; and reviews the background, history and physical examination, and diagnostic tests available for each. Diagnostic algorithms are provided, and the appropriate use of invasive and noninvasive cardiac testing for each condition is discussed.

      This review contains 8 highly rendered figures, 12 tables, and 52 references.

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    • 2

      Cardiovascular Biomarkers

      By Parul U. Gandhi, MD; James L. Januzzi Jr, MD
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      Cardiovascular Biomarkers

      • PARUL U. GANDHI, MDClinical and Research Fellow, Department of Medicine, Cardiology Division, Massachusetts General Hospital, Boston, MA
      • JAMES L. JANUZZI JR, MDRoman W. DeSanctis Endowed Clinical Scholar, Department of Medicine, Cardiology Division, Massachusetts General Hospital, Hutter Family Professor of Medicine, Harvard Medical School, Boston, MA

      The value of circulating biomarkers to care for patients with cardiovascular disease has grown significantly over the last few decades. The majority of clinical data focus on the use of natriuretic peptides (NPs) for the diagnosis, prognosis, and management of patients with heart failure (HF) and troponin measurements in patients with suspected or proven acute coronary syndrome (ACS). Part of the reason for the slow adoption of biomarkers beyond these two classes has been limitation in the optimal modes of application of new assays. Future studies are needed to clarify the use of biomarkers, with the ultimate goal of simplifying the diagnosis, prognosis, and patient care of complex cardiovascular conditions. This chapter reviews the use of established biomarkers for HF, ACS, and atrial fibrillation (AF). Tables include a summary of emerging and established cardiovascular biomarkers, characteristics of B-type natriuretic peptide and amino-terminal pro-B-type natriuretic peptide, cutoff points for NP measurement, differential diagnosis of elevated NP concentrations, biomarkers in HF with preserved ejection fraction, summary of NP management trials, third universal definition of myocardial infarction, and guidelines for recommendations of biomarkers in HF. Figures depict the various causes of NP release, the complex mechanism of troponin release in patients with HF, the ischemic and nonischemic etiologies of troponin release, timing of biomarker release during myocardial infarction, and the biomarkers involved in the pathogenesis of AF. Algorithms demonstrate evaluating outpatients with dyspnea in the clinic using NPs in their workup and the use of troponin to assist with determining an appropriate management strategy for a patient with ACS.

      This review contains 7 highly rendered figures, 8 tables, and 202 references.

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    • 3

      Cardiac Catheterization and Intervention

      By Dharam J. Kumbhani, MD, SM, MRCP, FACC; Deepak L. Bhatt, MD, MPH, FACP
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      Cardiac Catheterization and Intervention

      • DHARAM J. KUMBHANI, MD, SM, MRCP, FACCAssistant Professor of Medicine, Division of Cardiology, University of Texas Southwestern Medical School, Dallas, TX
      • DEEPAK L. BHATT, MD, MPH, FACPExecutive Director of Interventional Cardiovascular Programs, Brigham and Womens Hospital Heart & Vascular Center, Professor of Medicine, Harvard Medical School, Boston, MA

      Cardiac catheterization involves the insertion of a catheter (hollow polymer-coated tubing) into a blood vessel of the heart or into one of its chambers. Cardiac catheterization procedures are one of the most commonly performed cardiac procedures today. This review outlines the basics of angiography and coronary anatomy, the technical details of cardiac catheterizations, preferred access sites, and hemodynamic measurements. The basic steps in coronary intervention are listed. Common indications and contraindications for cardiac catheterization and intervention are described, as are appropriate use criteria for diagnostic catheterization and coronary intervention, fractional flow reserve (FFR) and intravascular ultrasonography, and complications of cardiac catheterization and percutaneous coronary intervention. Future directions in the field are discussed. Tables describe normal hemodynamic measurements, derived measurements during right heart catheterization, coronary artery disease prognostic index for medically managed patients, American College of Cardiology (ACC)/American Heart Association (AHA) guidelines regarding indications for coronary angiography, ACC/AHA appropriate use criteria for diagnostic catheterization, common indications for FFR, and risk of cardiac catheterization and coronary angiography. Figures include an overview of coronary anatomy, angiograms of the coronary arteries, images of a normal cardiac cycle and hemodynamic waveforms, the design of a stent, FFR evaluation, basic intravascular ultrasonography measurements, and coronary imaging with an optical coherence tomography system.

      This review contains 7 highly rendered figures, 8 tables, and 62 references.

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    • 4

      Chronic Stable Angina

      By Benjamin J. Scirica, MD, MPH; J. Antonio T. Gutierrez, MD
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      Chronic Stable Angina

      • BENJAMIN J. SCIRICA, MD, MPHSenior Investigator, TIMI Study Group, Cardiovascular Division, Brigham and Women’s Hospital, Associate Professor of Medicine, Harvard Medical School, Boston, MA
      • J. ANTONIO T. GUTIERREZ, MDCardiovascular Medicine Fellow, Brigham and Women's Hospital, Boston, MA

      By definition, chronic stable angina is angina that has been stable with regard to frequency and severity for at least 2 months. Chronic stable angina is the initial manifestation of coronary heart disease in approximately 50% of patients. Typically, this type of angina occurs in the setting of atherosclerotic coronary arterial narrowing, although other causes are possible. This review covers the epidemiology, pathophysiology, initial evaluation, differential diagnosis, management, and treatment of patients with chronic stable angina. Figures show noninvasive testing and the probability of coronary artery disease; diagnosis of patients with suspected ischemic heart disease; probability of severe coronary artery disease; coronary outcomes for high- versus low-intensity statin therapy; optimal medical therapy (OMT) versus OMT and percutaneous coronary intervention for chronic angina; OMT versus percutaneous coronary intervention for stable coronary heart disease; and coronary artery bypass grafting versus percutaneous coronary intervention for diabetes and coronary artery disease. Tables list the grading of angina pectoris by the Canadian Cardiovascular Society classification system, the differential diagnosis of chest pain, conditions promoting myocardial oxygen supply and demand mismatch, the features of typical angina, the classification of chest pain, a comparison of the pretest likelihood of coronary heart disease (CHD) in low-risk and high-risk symptomatic patients, the posttest probability of significant CHD based on pretest probabilities of CHD and normal or abnormal results of noninvasive studies, survival according to risk groups based on Duke treadmill scores, high- and moderate-intensity statin therapy, revascularization to improve survival compared with medical therapy, revascularization to improve symptoms with significant anatomic (≥ 50% left main or ≥ 70% nonleft main coronary artery disease) or physiologic (fractional flow reserve ≤ 0.80) coronary artery stenoses, and questions recommended by an expert panel for patients with chronic stable angina at follow-up visits.

      This review contains 7 highly rendered figures, 12 tables, and 109 references.

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    • 5

      Unstable Angina and Other Acute Coronary Syndromes

      By R. Scott Wright, MD, FACC, FESC, FAHA; Joseph G. Murphy, MD, FACC, FESC
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      Unstable Angina and Other Acute Coronary Syndromes

      • R. SCOTT WRIGHT, MD, FACC, FESC, FAHAProfessor of Medicine, Consultant in Cardiology and the Coronary Care Unit, Mayo Clinic, Rochester, MN
      • JOSEPH G. MURPHY, MD, FACC, FESCProfessor of Medicine, Consultant in Cardiology and the Coronary Care Unit, Chair, Section of Scientific Publications, Mayo Clinic, Rochester, MN

      Patients with coronary artery disease (CAD) present clinically when their disease enters an unstable phase known as an acute coronary syndrome (ACS), in which the cap of a previously stable atheromatous coronary plaque ruptures or erodes, which in turn activates a thrombotic cascade that may lead to coronary artery occlusion, myocardial infarction (MI), cardiogenic shock, and patient death. There are nearly 2 million episodes of ACS in the United States annually; it is the most common reason for hospitalization with CAD and is the leading cause of death in the developed world. ACS patients include those with unstable angina (UA), non–ST segment elevation myocardial infarction (non-STEMI), and ST segment elevation myocardial infarction (STEMI) and patients who die suddenly of an arrhythmia precipitated by coronary occlusion. The distinction among various ACS subgroups reflects varying characteristics of clinical presentation (presence or absence of elevated cardiac biomarkers) and the type of electrocardiographic (ECG) changes manifested on the initial ECG at the time of hospitalization. This chapter focuses on UA and non-STEMI. A graph outlines mortality risks faced by patients with varying degrees of renal insufficiency. An algorithm describes the suggested management of patients admitted with UA or non-STEMI. Tables describe the risk stratification of the patient with chest pain, categories of Killip class, examination findings of a patient with high-risk ACS, diagnosis of MI, causes of troponin elevation other than ischemic heart disease, initial risk stratification of ACS patients, and long-term medical therapies and goals in ACS patients.

      This review contains 2 highly rendered figures, 11 tables, and 76 references.

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    • 6

      St-segment Elevation Myocardial Infarction

      By Grant William Reed, MD; Christopher Paul Cannon, MD
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      St-segment Elevation Myocardial Infarction

      • GRANT WILLIAM REED, MDFellow, Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, OH
      • CHRISTOPHER PAUL CANNON, MD Cardiovascular Division, Brigham and Women’s Hospital, Professor of Medicine, Harvard Medical School, Executive Director of Cardiometabolic Trials, Harvard Clinical Research Institute, Boston, MA

      Patients with acute coronary syndrome fall into two groups: those with unstable angina or non—ST segment elevation (formerly non—Q wave) myocardial infarction (NSTEMI) and those with acute ST segment elevation (formerly Q wave) myocardial infarction (STEMI). STEMI is the focus of this chapter. The epidemiology, pathophysiology, diagnosis, differential diagnosis, and complications of STEMI are elaborated. Reperfusion therapy (including time to reperfusion; diagnostic coronary angiography; primary, facilitated, rescue, and late percutaneous coronary intervention [PCI]; thrombolytic therapy and choice of thrombolytic agent; early invasive strategy; coronary artery bypass grafting; and therapeutic hypothermia), medical therapy (including aspirin, P2Y12 inhibitors, glycoprotein IIb/IIIa inhibitors, anticoagulants, nitrates, beta blockers, inhibition of the renin-angiotensin-aldosterone system, oxygen, analgesia, lipid-lowering therapy, prophylactic antiarrhythmics, and magnesium), risk stratification, secondary prevention, and post-STEMI care are also covered. Tables delineate classifications of MI as defined by proximal cause of myocardial ischemia, Killip classification of acute MI and mortality rates, differential diagnosis of ST segment elevation, contraindications for administering thrombolytic agents, and major recommendations for antithrombotic therapy in patients with STEMI treated with primary PCI and thrombolysis. Algorithms indicate a diagnostic approach to acute coronary syndromes and corresponding pathology and reperfusion strategies. Electrocardiogram changes in STEMI and corresponding territory of myocardium are depicted. A variety of graphs are included.

      This review contains 11 highly rendered figures, 6 tables, and 151 references.

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    • 7

      Heart Failure

      By Sachin P. Shah, MD; Mandeep R. Mehra, MD
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      Heart Failure

      • SACHIN P. SHAH, MDCenter for Advanced Heart Disease, Brigham and Women’s Hospital Heart and Vascular Center, Harvard Medical School, Boston, MA, Director, Cardiovascular Intensive Care Unit, Lahey Hospital and Medical Center, Burlington, MA
      • MANDEEP R. MEHRA, MDMedical Director, Brigham and Women’s Hospital Heart and Vascular Center, Harvard Medical School, Boston, MA

      Heart failure is a syndrome related to abnormal cardiac performance with a consequence of impaired cardiac output at rest or with exertion and/or congestion, which usually leads to symptoms of fatigue, dyspnea, and edema. The syndrome is characterized by various phenotypes related to a vast array of etiologies with diverse management targets. The current broad categorization of heart failure separates patients based on ejection fraction. Further description of the phenotype beyond ejection fraction is imperative to correctly identify the etiology of heart failure and, ultimately, to choose medical, device, and surgical therapies appropriately. This review covers the epidemiology of heart failure, defining the phenotype and etiology of heart failure, recognition and management of acute decompensated heart failure, management of chronic heart failure with a reduced ejection fraction, implantable cardioverter-defibrillators in heart failure with a reduced ejection fraction, management of heart failure with a preserved ejection fraction, and advanced heart failure. Figures show the evolution of therapy in chronic heart failure from the symptom-directed model, the complex pathophysiology and principal aberrations underlying heart failure with preserved ejection fraction, and concepts underlying surgical therapy in advanced heart failure using Laplace’s law. Tables list various etiologies of heart failure; sensitivity and specificity of clinical, biomarker, and radiographic data in the diagnosis of acute decompensated heart failure; drugs and devices with a demonstrated survival benefit in heart failure with a reduced ejection fraction; neurohormonal antagonist dosing in heart failure with a reduced ejection fraction; randomized, placebo-controlled trials in heart failure with a preserved ejection fraction; categorization of heart failure according to American Heart Association/American College of Cardiology heart failure stage, New York Heart Association functional class, and Interagency Registry for Mechanically Assisted Circulatory Support level; and poor prognostic indicators in heart failure.

      This review contains 3 highly rendered figures, 7 tables, and 113 references.

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    • 8

      Heart Transplantation

      By Michael M. Givertz, MD
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      Heart Transplantation

      • MICHAEL M. GIVERTZ, MDMedical Director, Heart Transplant and Circulatory Assist, Brigham and Women's Hospital, Associate Professor, Harvard Medical School, Boston, MA

      Heart failure is a major public health problem with significant associated morbidity and mortality. Heart transplantation remains the standard of care for highly selected patients with end-stage heart failure and absence of contraindications to transplantation. This chapter discusses indications and contraindications for transplantation; recipient evaluation, selection, and management; donor selection; timing of the procedure and surgical technique; medical management, including immunosuppression, prevention and treatment of infections, and other standard or preventive therapy; late complications; and functional status and long-term survival. Tables describe patient referral to a specialized center for heart transplantations; guidelines of indications for cardiac transplantation; organ dysfunction; pretransplantation evaluation; waiting lists; therapeutic options for patients with advanced or refractory heart failure; treating highly sensitized patients; suggested vaccinations; guidelines for donor hearts with severe infection; high-risk donor behavior; hemodynamic effect of commonly used parenteral agents; frequency of follow-up evaluations; revised International Society for Heart and Lung Transplantation (ISHLT) formulation for diagnosis of cardiac allograft rejection and suggested treatment; function of immunosuppressive agents; administration, dosing, monitoring, and adverse effects of commonly used immunosuppressants; common agents that interfere with tacrolimus and cyclosporine; cytomegalovirus prophylaxis and valganciclovir based on estimated renal function; cumulative morbidity rates in adult heart transplant survivors; and therapies to prevent and treat osteoporosis posttransplantation. Figures depict the progression of heart failure; change in functional status over time in patients with chronic heart failure; US heart transplantations in 2012; percentage of US adult wait-listed patients who received a donor heart transplant within a year and donation rates by state; bicaval surgical technique; endomyocardial biopsies; timeline of infection following solid-organ transplantation; cardiac allograft vasculopathy; and squamous cell carcinomas in a heart transplant patient. Graphs show adult worldwide heart transplantation volume from 1982 to 2010; changing characteristics of US adult heart transplant recipients; relative risk of death and development of cardiac allograft vasculopathy; posttransplantation immunosuppression at 1 and 5 years in the ISHLT Registry; older donor age and risk of developing cardiac allograft vasculopathy; freedom from malignancy in the ISHLT Registry; employment status of adult heart transplant recipients; adult heart transplant survival; and patient survival among US heart transplant recipients by gender and race.

      This review contains 18 highly rendered figures, 20 tables, and 109 references.

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    • 9

      Supraventricular Tachycardia

      By Saurabh Kumar, BSc(Med)/MBBS, PhD; Laurence M. Epstein, MD
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      Supraventricular Tachycardia

      • SAURABH KUMAR, BSC(MED)/MBBS, PHDAdvanced Clinical Electrophysiology Fellow, Cardiac Arrhythmia Service, Brigham and Woman’s Hospital, Boston, MA
      • LAURENCE M. EPSTEIN, MDChief, Cardiac Arrhythmia Service, Associate Professor of Medicine, Harvard Medical School, Cardiac Arrhythmia Service, Brigham and Women’s Hospital, Boston, MA

      Supraventricular tachycardias (SVTs) comprise a group of usually benign arrhythmias that originate from cardiac tissue at or above the His bundle. SVTs include inappropriate sinus tachycardia, atrial tachycardias (ATs), atrial flutter (AFL), junctional tachycardia, atrioventricular nodal reentrant tachycardia (AVNRT), and forms of accessory pathway–mediated reentrant tachycardias (atrioventricular reentrant tachycardia [AVRT]). Although mostly benign, symptoms can be debilitating, in the form of palpitations, shortness of breath, chest discomfort, dizziness, and/or syncope; rarely, SVTs can result in cardiomyopathy due to incessant arrhythmia. This review covers the epidemiology, diagnosis, management, and classification of SVTs. Figures show differential diagnosis for wide (> 120 ms) QRS complex tachycardia, differential diagnosis for narrow QRS complex tachycardia, method for assessing RP interval during SVT, example of a short RP tachycardia with a positive deflection at the end of the QRS complex in lead V1 and a pseudo S wave in lead III, example of a short RP tachycardia with an RP greater than 70 ms, example of a long RP tachycardia, example of preexcited atrial fibrillation in a 17-year-old man, the utility of the response to intravenous adenosine in determining the cause of tachycardia, mechanism of AVNRT, accessory pathway conduction, mechanism of orthodromic and antidromic AVRT, distribution of focal ATs, a schematic showing the different types of AFL circuits, and an electrocardiogram (ECG) example of typical AFL. Tables list a summary of mechanism and definition of SVTs, ECG criteria to differentiate ventricular tachycardia from SVT in wide-complex tachycardia, and drugs used to maintain sinus rhythm in patients with SVT.

       

      This review contains 14 highly rendered figures, 3 tables, and 74 references.

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    • 10

      Atrial Fibrillation

      By Gregory F. Michaud, MD; Roy M. John, MD, PhD
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      Atrial Fibrillation

      • GREGORY F. MICHAUD, MDDirector, Center for the Advanced Management of Atrial Fibrillation, Brigham and Women’s, Hospital, Assistant Professor of Medicine, Harvard Medical School, Boston, MD
      • ROY M. JOHN, MD, PHDAssociate Director EP Laboratory, Director, Experimental Research, Cardiac Arrhythmia Service, Brigham and Women’s Hospital, Assistant Professor of Medicine, Harvard Medical School, Boston, MA

      Atrial fibrillation (AF) is an abnormal rhythm characterized by chaotic atrial electrical activity resulting in loss of atrial contraction, an irregular and unpredictable heart rate, and a tendency for thrombus formation. The prevalence of AF is estimated at 1 to 2%, but it’s likely higher than that because one-third of patients may have no symptoms and might never seek medical attention. Data suggest that 1 in 4 people over the age of 40 will develop AF in their lifetime. About 10% of patients over age 80 have experienced the arrhythmia, and some estimates predict the prevalence will double in the next 50 years. This chapter discusses the pathophysiology, genetics, diagnosis, classification, and treatment of AF. Figures show atrial fibrillation and coarse atrial fibrillation plus common right atrial flutter. One algorithm is for oral anticoagulation therapy, and a second shows a recommended hierarchical choice of antiarrhythmic therapies versus catheter ablation for recurrent symptomatic atrial fibrillation. Tables list classification, diagnostic evaluation of, clinical consequences of, and conditions often associated with atrial fibrillation. Three scoring systems are included: 1) for congestive heart failure, hypertension, diabetes, stroke, and transient ischemic attack; 2) to assess the risk of bleeding with oral anticoagulation, and 3) data and proportion of patients from the Euro Heart Survey. Other tables include long-term anticoagulation guidelines for atrial fibrillation, intravenous drugs used for acute rate control, oral drugs used for chronic rate control, and antiarrhythmic drugs for conversion of atrial fibrillation and/or maintenance of sinus rhythm. In addition, there’s a summary of randomized trials weighing rate control and rhythm control strategies, plus schemes for categorizing thromboembolism risk.

      This review contains 4 highly rendered figures, 13 tables, and 129 references.

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    • 11

      Ventricular Arrhythmias

      By Roy M. John, MD, PhD; William G. Stevenson, MD
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      Ventricular Arrhythmias

      • ROY M. JOHN, MD, PHDAssociate Director EP Laboratory, Director, Experimental Research, Cardiac Arrhythmia Service, Brigham and Women’s Hospital, Assistant Professor of Medicine, Harvard Medical School, Boston, MA
      • WILLIAM G. STEVENSON, MDDirector, Clinical Cardiac Electrophysiology, Brigham and Women’s Hospital, Boston, MA

      Ventricular arrhythmias are common in all forms of heart disease and are an important cause of cardiac arrest and sudden death. Many ventricular arrhythmias are benign but may serve as a marker for underlying disease or its severity. Others are life threatening. The significance of an arrhythmia is determined by the specific characteristics of the arrhythmia and the associated heart disease, and these features guide evaluation and therapy. This review discusses various mechanisms and types of ventricular arrhythmias and management based on clinical presentation (including patients with symptomatic arrhythmia and increased risk of sudden death without arrhythmia symptoms). Genetic arrhythmia syndromes, such as abnormalities of repolarization and the QT interval, catecholaminergic polymorphic ventricular tachycardia (VT), and inherited cardiomyopathies, are discussed in depth. Under the rubric of management of ventricular arrhythmias, drug therapy for ventricular arrhythmias, implantable cardioverter-defibrillators (ICDs), and catheter ablation for VT are also covered. Tables chart out guideline recommendations for ICD therapy, drugs for the management of ventricular arrhythmias, and indications and contraindications for catheter ablation of ventricular arrhythmias. Electrocardiograms are provided, as well as management algorithms for ventricular arrhythmias based on patient presentation, and an algorithm for identifying patients with systolic heart failure and left ventricular ejection less than or equal to 35% who are candidates for consideration of an ICD for primary prevention of sudden cardiac death.

      This review contains 5 highly rendered figures, 3 tables, and 60 references.

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    • 12

      Pacemaker Therapy

      By Shamai A. Grossman, MD, MS
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      Pacemaker Therapy

      • SHAMAI A. GROSSMAN, MD, MSAssociate Professor of Emergency Medicine, Harvard Medical School, Vice Chair for Health Care Quality, Harvard Medical Faculty Physicians, Beth Israel Deaconess Medical Center, Boston, MA

      The number of permanent pacemakers implanted per year increased by 55.6% between 1993 and 2009, and is continuing to rise. Accordingly, the number of patients treated in the emergency department who have permanent pacemakers is increasing, and it is important for physicians in the emergency department to be familiar with the operation and potential complications of these devices. This review covers the pathophysiology, stabilization and assessment, diagnosis and treatment, and disposition and outcomes for patients with pacemakers presenting to the emergency department. Figures show examples of dual chamber pulse generators from four major pacemaker manufacturers, VVI pacing with a lower rate limit of 60 beats per minute,  DDD pacing with a lower rate limit of 60 beats per minute and an upper rate limit of 120 beats per minute, a 12-lead electrocardiogram with bifascicular block, a proprietary algorithm (Managed Ventricular Pacing, Medtronic Inc.) aimed at reducing ventricular pacing, and an example of a pacemaker pocket infection. Tables list North American Society of Pacing and Electrophysiology/British Pacing and Electrophysiology Group generic five-position code for antibradycardia pacing, and Levels of Evidence and Society Guideline Recommendations for Selected Pacing Indications.

      This review contains 6 highly rendered figures, 2 tables, and 27 references.

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    • 13

      Valvular Heart Disease

      By Miriam S. Jacob, MD; Brian P. Griffin, MD
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      Valvular Heart Disease

      • MIRIAM S. JACOB, MDAdvanced Fellow in Heart Failure, Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland OH
      • BRIAN P. GRIFFIN, MDJohn and Rosemary Brown Chair in Cardiovascular Medicine, Cleveland Clinic, Cleveland OH

      Valvular heart disease is an important cause of cardiac morbidity in developed countries despite a decline in the prevalence of rheumatic disease in those countries. This chapter discusses the many etiologies of valvular heart disease and presents methods for assessment and management. Specific valvular lesions discussed include mitral stenosis, mitral regurgitation, mitral valve prolapse, aortic stenosis, aortic regurgitation, and tricuspid and pulmonary disease. The section on tricuspid disease includes a discussion of mechanical prostheses (ball-in-cage and tilting-disk) and biologic prostheses (xenografts, allografts, and autografts) and their complications. The chapter concludes with a discussion of anorexiant-induced valvular disorder. Figures include color photographs of pathologic specimens, numerous echocardiographic images of valvular lesions, plus fluoroscopies of a percutaneous mitral valve repair and a transcatheter aortic valve replacement. Tables include causes of specific valvular lesions, assessment of patients with valvular heart disease, auscultatory findings associated with common valvular problems, risk factors for severe adverse outcomes with infective endocarditis, and American Heart Association recommendations for endocarditis prophylaxis.

      This review contains 11 highly rendered figures, 5 tables, and 153 references.

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    • 14

      Congenital Heart Disease in Adults

      By Heather L. Bartlett, MD; Luke J. Lamers, MD; Susan E. Haynes, MD; David J. Skorton, MD
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      Congenital Heart Disease in Adults

      • HEATHER L. BARTLETT, MDAssistant Professor, Department of Pediatrics, Director, Adult Congenital Heart Disease Program, University of Iowa Children’s Hospital, Iowa City, IA
      • LUKE J. LAMERS, MDAssistant Professor, Department of Pediatrics, Director, Pediatric Cardiac Catheterization Laboratory, University of Wisconsin, Madison, WI
      • SUSAN E. HAYNES, MDAssociate Professor, Department of Pediatrics, University of Iowa Children’s Hospital, Iowa City, IA
      • DAVID J. SKORTON, MDProfessor, Departments of Medicine and Pediatrics, Weill Cornell Medical College, New York, NY and Department of Biomedical Engineering, Cornell University, Ithaca, NY

      With advances in medical and surgical care, an increasing number of children with congenital diseases of the heart and vasculature now survive to adulthood. The proportion of adults who are affected by congenital heart disease is expected to continue to increase. Thus, it is important for clinicians to be knowledgeable about the care of these patients. This review examines acyanotic disorders (shunts and valvular lesions), vascular anomalies, cyanotic disorders, and women’s health issues. Figures show the anatomy of atrial septal defects, transcatheter closure of atrial septal defects, an anatomic cross section showing the atrioventricular septum, the anatomic positions of ventricular septal defects, a computed tomography scan of aortic coarctation, angiogram of a persistent left superior vena cava draining into the right atrium, systemic artery-to-pulmonary artery shunts, magnetic resonance image of a patient with repaired tetralogy of Fallot and long-standing pulmonary valve insufficiency, treatment of pulmonary valve regurgitation with a transcatheter pulmonary valve, computed tomographic images of a patient with atrial switch palliation of transposition of the great arteries and multiple baffle obstructions, stages in the repair of functional single ventricles, and echocardiograms of a patient with Ebstein anomaly. Tables list recommendations for pulmonary valve replacement in repaired tetralogy of Fallot, conditions in which pregnancy is high risk, and cardiac indications for fetal echocardiography.

      This review contains 12 highly rendered figures, 3 tables, and 63 references.

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    • 15

      Hypertension

      By Gary L. Schwartz, MC, FACP, FAHA, FASH
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      Hypertension

      • GARY L. SCHWARTZ, MC, FACP, FAHA, FASHConsultant, Division of Nephrology and Hypertension, Professor of Medicine, College of Medicine, Mayo Clinic, Rochester Minnesota

      Hypertension is the most common chronic disorder in the United States. The prevalence increases with age: for a normotensive middle-aged person in the United States, the lifetime risk of developing hypertension approaches 90%. With the aging of populations in most developed and developing societies, hypertension will become an increasingly prevalent disorder in coming years. Hypertension is a major risk factor for stroke, myocardial infarction, heart failure, chronic kidney disease, progressive atherosclerosis, and dementia. This chapter presents a definition of hypertension and reviews its epidemiology, etiology, pathogenesis, and diagnosis. Secondary hypertension is discussed. The two most common treatments for essential hypertension are discussed. Reasons for resistance to treatment are described, as are treatments for specific groups. A figure illustrates the drug treatment for essential hypertension. Tables present information on the diagnosis and treatment of essential hypertension, secondary hypertension, and hypertensive crisis.

      This review contains 1 figure, 14 tables, and 101 references.

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    • 16

      Diseases of the Aorta

      By Anna M. Booher, MD; Kim A. Eagle, MD
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      Diseases of the Aorta

      • ANNA M. BOOHER, MDClinical Assistant Professor, Division of Cardiovascular Medicine, Department of Internal Medicine, University of Michigan Health System, Ann Arbor, MI
      • KIM A. EAGLE, MDAlbion Walter Hewlett Professor of Internal Medicine, Chief of Clinical Cardiology, Clinical Director, Cardiovascular Center, Division of Cardiovascular Medicine, Department of Internal Medicine, University of Michigan Health System, Ann Arbor, MI

      This chapter covers the major presentations affecting the aorta: aortic aneurysms (abdominal aortic aneurysms and thoracic aortic aneurysms), acute aortic syndromes (including aortic dissection, intramural hematoma, and penetrating atherosclerotic ulcer), other nonacute aortic processes, and traumatic disease of the aorta. The section on abdominal aortic aneurysms covers screening, clinical presentation, diagnostic evaluation, management to reduce the risk of aneurysm rupture, open surgical treatment and endovascular aortic repair, and the role of medical therapy. The section on thoracic aortic aneurysms also covers pathophysiology, etiology, and inherited and inflammatory conditions. Aortic dissections affect either the ascending aorta (type A) or the descending aorta (type B) and may be classified as acute or chronic. The discussion of aortic dissection describes the clinical presentation, diagnostic steps and decisions, and treatment for both type A and type B dissections. The figures include two algorithms: a potential management strategy for patients with thoracic aortic aneurysm and a logical procedure for the evaluation and treatment of a suspected aortic dissection. Figures also include illustrations, computed tomographic images, and echocardiograms of various aortic presentations. Tables list normal aortic dimensions by computed tomographic angiography and echocardiography, etiology and associated factors in diseases of the aorta, revised Ghent criteria for the diagnosis of Marfan syndrome, size criteria for elective surgical intervention in thoracic aortic aneurysm, and independent predictors of in-hospital death. Also included is a follow-up imaging timeline for acute aortic syndromes.

      This chapter contains 9 figures, 6 tables, 132 references, and 5 Board-styled MCQs.

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    • 17

      Peripheral Artery Diseases

      By Mark A. Creager, MD
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      Peripheral Artery Diseases

      • MARK A. CREAGER, MD Professor of Medicine, Harvard Medical School, Director, Vascular Center, Head, Vascular Medicine Section, Cardiovascular Division, Brigham and Women’s Hospital, Boston, MA

      Peripheral arterial diseases (PADs) compromise blood flow to the limbs. Common causes of arterial obstruction include atherosclerosis, thrombus, embolism, vasculitis, arterial entrapment, adventitial cysts, fibromuscular dysplasia, arterial dissection, trauma, and vasospasm. The most frequently encountered cause of PAD is peripheral atherosclerosis. This chapter considers its epidemiology and risk factors, as well as its diagnosis, including clinical presentation and noninvasive diagnostic tests. This chapter also discusses acute arterial occlusion, atheroembolism, popliteal artery entrapment, thromboangiitis obliterans, and acrocyanosis, as well as the etiology, diagnosis, and treatment of Raynaud phenomenon. The chapter contains 4 tables and 7 figures. Tables describe the Fontaine classification and clinical categories of chronic limb ischemia, provide examples of leg segmental pressure measurements in a patient with calf claudication and foot pain, and summarize secondary causes of Raynaud phenomenon. Figures include a photograph of an ischemic foot demonstrating dependent rubor, measurement of the ankle:brachial index, ultrasonography of a stenosis of the right common femoral artery, magnetic resonance angiograms of patients with calf claudication, arteriograms of critical ischemia of the foot and of disabling claudication of the leg, and ischemia of the toes caused by atheroemboli. This chapter contains 80 references.

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    • 18

      Venous Thromboembolism

      By Samuel Z. Goldhaber, MD
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      Venous Thromboembolism

      • SAMUEL Z. GOLDHABER, MDProfessor of Medicine, Harvard Medical School Director, Venous Thromboembolism Research Group

      Venous thromboembolism, which involves venous thrombosis and pulmonary embolism, is a leading cause of morbidity and mortality in hospitalized patients and is being seen with increasing frequency in outpatients. This chapter discusses the risk factors, etiology, classification, pathophysiology, natural history, prognosis, diagnosis (including venous thrombosis, recurrent venous thrombosis, and pulmonary embolism), prophylaxis, and treatment of venous thromboembolism (including the pharmacology of antithrombotic agents), as well as venous thromboembolism in pregnancy and miscellaneous thromboembolic disorders (including thrombosis of unusual sites). Tables in the chapter present a model for determining clinical suspicion of deep vein thrombosis, test results that effectively confirm or exclude deep vein thrombosis, a model for determining a clinical suspicion of pulmonary embolism, test results that effectively confirm or exclude pulmonary embolism, drug and food interactions with warfarin by level of supporting evidence and direction of interaction, risk categories for venous thromboembolism, and recommendations for prophylaxis. Figures include a venogram illustrating thrombi in the left iliac vein, perfusion scans showing multiple perfusion defects, a computed tomographic pulmonary angiogram demonstrating intraluminal filling defects, a diagnostic approach for suspected pulmonary embolism, and an algorithm for selecting duration of anticoagulation after venous thromboembolism. There are 105 references in this chapter.

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    • 19

      Diseases of the Pericardium, Cardiac Tumors, and Cardiac Trauma

      By Terrence D. Welch, MD; Kyle W Klarich, MD; Jae K. Oh, MD
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      Diseases of the Pericardium, Cardiac Tumors, and Cardiac Trauma

      • TERRENCE D. WELCH, MDAssistant Professor of Medicine, Section of Cardiology, Dartmouth-Hitchcock Medical Center, Lebanon, NH
      • KYLE W KLARICH, MDProfessor of Medicine, Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN
      • JAE K. OH, MDSamsung Professor of Cardiovascular Diseases, Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN

      The pericardium consists of a fibrous sac and a serous membrane. Because of its simple structure, the clinical syndromes involving the pericardium are relatively few but vary substantially in severity. Cardiac tumors may be either primary or secondary and either benign or malignant, with attachment sites throughout the endocardium. Cardiovascular trauma should be suspected in all patients with chest injuries or severe generalized trauma. Cardiovascular injury may be either blunt or penetrating. This review covers pericardial disease, cardiac tumors, and cardiovascular trauma. Figures show an electrocardiogram in acute pericarditis; acute pericarditis with delayed gadolinium enhancement of the pericardium shown with cardiac magnetic resonance imaging; underlying cause of pericardial effusion requiring pericardiocentesis; pericardial pressure-volume curves; large pericardial effusion with swinging motion of the heart resulting in electrical alternans; typical pulsed-wave Doppler pattern of tamponade; underlying causes of constrictive pericarditis in patients undergoing pericardiectomy; pericardial calcification seen on a chest radiograph; thickened pericardium; typical pulsed-wave Doppler pattern of constrictive pericarditis; typical mitral annular tissue velocities in constrictive pericarditis; a diagnostic algorithm for the echocardiographic diagnosis of constrictive pericarditis; simultaneous right ventricular and left ventricular pressure tracings in restrictive cardiomyopathy; computed tomographic scan showing inflammatory constrictive pericarditis; systolic and diastolic transesophageal echocardiographic images of a large left atrial myxoma attached to the atrial septum; a decision tree of management options for patients with suspected papillary; transesophageal echocardiographic examples of aortic valve, mitral valve, left ventricular outflow tract, and tricuspid valve papillary fibroelastomas; and transesophageal short-axis view of the descending thoracic aorta in a hypotensive patient after a motor vehicle accident. The table lists tamponade versus constriction versus restrictive cardiomyopathy.

      This review contains 18 highly rendered figures, 1 table, and 77 references.

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  • Competency-based Patient Care
    • 1

      On Being a Physician

      By Elizabeth G. Nabel, MD, FACP
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      On Being a Physician

      • ELIZABETH G. NABEL, MD, FACPPresident, Brigham and Women’s Hospital, Professor of Medicine, Harvard Medical School, Boston, MA

      The role of a physician as healer has grown more complex, and emphasis will increasingly be on patient and family-centric care. Physicians must provide compassionate, appropriate, and effective patient care by demonstrating competence in the attributes that are essential to successful medical practice. Beyond simply gaining medical knowledge, modern physicians embrace lifelong learning and need effective interpersonal and communication skills. Medical professionalism encompasses multiple attributes, and physicians are increasingly becoming part of a larger health care team. To ensure that physicians are trained in an environment that fosters innovation and alleviates administrative burdens, the Accreditation Council for Graduate Medical Education has recently revamped the standards of accreditation for today’s more than 130 specialties and subspecialties.

      This review contains six references.

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    • 2

      Ethical and Social Issues in Medicine

      By Roberta Springer Loewy, PhD (PHIL, ETHICS); Erich H. Loewy, MD, FACP (deceased); Faith T. Fitzgerald, MD, MACP
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      Ethical and Social Issues in Medicine

      • ROBERTA SPRINGER LOEWY, PHD (PHIL, ETHICS)Professor and Bioethics Education Consultant, VCF, University of California, Davis, Sacramento, CA
      • ERICH H. LOEWY, MD, FACP (DECEASED)Professor and Founding Chair of the Bioethics Program (Emeritus), University of California, Davis, Sacramento, CA
      • FAITH T. FITZGERALD, MD, MACPProfessor of Internal Medicine, University of California, Davis, Sacramento, CA

      So rapidly has the field of health care ethics continued to grow that, when recently “googled,” the term produced 28.2 million hits. The challenge is to address the ethical and social issues in medicine in this very limited article space. It remains an impossible task to present more than a superficial discussion of these complex issues and the complicated cases in which they are to be found. Like good medicine, good ethics cannot be practiced by algorithm. The authors have opted to provide an operational guide to help clinicians sort through the ethical and social quandaries they must face on a daily basis. To that end, the authors have chosen to divide this chapter into the following sections:
      1. A brief description of the biopsychosocial nature of ethics and how it differs from personal morality
      2. A method for identifying and dealing with ethical issues
      3. A discussion of the role of bioethicists and ethics committees
      4. The professional fiduciary role of clinicians
      5. Listings of some of the common key bioethical and legal terms (online access only)
      6. A very brief discussion of the terms cited in the above listings (online access only)

      This reviews contains 2 tables (common bioethical concepts and legal concepts), 6 references, 1 appendix, and 24 additional readings.

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    • 3

      Principles of Neurologic Ethics

      By Thomas I. Cochrane, MD, MBA
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      Principles of Neurologic Ethics

      • THOMAS I. COCHRANE, MD, MBAAssociate Neurologist, Division of Neuromuscular Disease, Department of Neurology, Brigham and Women’s Hospital, Assistant Professor of Neurology, Harvard Medical School, Boston, MA

      Many neurologic diseases affect the brain and thus cognition and personality. Many people consider some neurologic conditions “worse than death” and would prefer not to be kept alive using medical technology if there is no reasonable chance of recovery. The clinician caring for patients with neurologic disease will frequently be confronted with decisions about whether to initiate or continue life-sustaining therapies. And because neurologic disease often impairs the ability to understand and to decide, clinicians are more often called on to make decisions for patients who cannot decide for themselves. This chapter covers general principles, including respect for autonomy, beneficence, nonmaleficence, justice, informed consent, and implied consent. Discussion about making decisions for others includes competence and decision-making capacity, advance directives (powers of attorney and living wills), substituted judgment, and best interests. Decisions about life-sustaining treatment include withholding versus withdrawing, acts versus omissions, and limiting life-sustaining treatment for the patient with no surrogate. Also covered are decisions in the face of prognostic uncertainty, and futility, as well as commonly encountered problems such as states of severely disordered consciousness, coma, vegetative state, minimally conscious state, and brain death (including ethical controversies). Organ donation after both brain death and cardiac death is discussed. The section on dementia covers feeding tubes for patients with advanced dementia, the locked-in state, and neuroenhancement. Tables include elements of informed consent, elements of competence, assumed ethical priority of potential surrogates, steps that aid decision making in the face of prognostic uncertainty, and the American Academy of Neurology’s criteria for determination of brain death. This chapter includes 23 references.

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    • 4

      Quality of Care: Performance Measurement and Quality Improvement in Clinical Practice

      By Sonali P. Desai, MD, MPH; Allen Kachalia, MD, JD
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      Quality of Care: Performance Measurement and Quality Improvement in Clinical Practice

      • SONALI P. DESAI, MD, MPHAmbulatory Director, Patient Safety, Center for Clinical Excellence, Associate Director of Quality, Department of Medicine, Division of Rheumatology, Brigham and Women's Hospital, Boston, MA
      • ALLEN KACHALIA, MD, JDAssociate Chief Quality Officer, Co-Director, Center for Clinical Excellence, Brigham and Women's Hospital, Boston, MA

      Attention to the quality of care within the United States health care system has grown tremendously over the past decade. We have witnessed a significant change in how quality improvement and clinical performance measurement are approached. The current focus on quality and safety stems in part from the increasingly clear realization that more services and technological advancement are not automatically equivalent to high-quality care. Much of the discussion about cost and quality in health care is shifting towards the concept of value. Value is defined as health outcomes achieved per dollar spent (in other words, an assessment of the quality of care per cost). This chapter reviews the current state of quality improvement in health care and, because improvement cannot be determined without measurement, reviews several aspects of effective clinical performance measurement. Since many measures are already in place, the chapter describes some of the organizations involved in quality measurement and improvement, as well the approaches they utilize. It looks at the multiple strategies in place to improve quality, from process management to collaboration, from financial incentives to transparency, and reviews newer models of care delivery that may materialize in the near future. Tables list types of quality measures, characteristics to consider when developing a quality measure, and organizations involved in quality improvement and performance measurement. A figure shows strategies used by the federal government to spur performance measurement and quality improvement.

      This review contains 1 highly rendered figure, 3 tables, and 56 references.

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    • 5

      Practicing Evidence-based Medicine

      By Michael Barnett, MD; Niteesh Choudhry, MD, PhD
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      Practicing Evidence-based Medicine

      • MICHAEL BARNETT, MDFellow in General Internal Medicine, Division of General Internal Medicine and Primary Care, Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA
      • NITEESH CHOUDHRY, MD, PHDAssociate Professor, Division of Pharmacoepidemiology and Pharmacoeconomics, Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA

      Today, a plethora of resources for evidence-based medicine (EBM) are available via alert services, compendia, and more. In theory, a clinician researching a topic or looking for information regarding a clinical decision should easily find the literature or synopses needed. However, the real challenge lies in recognizing which resources (out of hundreds or possibly thousands) present the best and most reliable evidence. As well, evidence from research is only part of the decision calculus, and the clinician, not the evidence, makes the final decisions. Medical decision analysis attempts to formalize the process and reduce it to algebra, but it is difficult or impossible to represent all the components of a decision mathematically and validly let alone do so in “real time” for individual patients. This review discusses these challenges and more, including how to ask answerable questions, understand the hierarchy for evidence-based information resources, critically appraise evidence, and apply research results to patient care. Figures show the total number of new articles in Medline from 1965 to 2012, a “4S” hierarchy of preappraised medicine, percentage of physician and medical student respondents with a correct or incorrect answer to a question about calculating the positive predictive value of a hypothetical screening test, a nomogram for Bayes’s rule, an example of nomogram use for pulmonary embolism, and a model for evidence-informed clinical decisions. Tables list selected barriers to the implementation of EBM; Patient, Intervention, Comparison, and Outcome (PICO) framework for formulating clinical questions; guides for assessing medical texts for evidence-based features; clinically useful measures of disease frequency and statistical significance and precision; definitions of clinically useful measures of diagnostic test performance and interpretation; definitions of clinically useful measures of treatment effects from clinical trials; summary of results and derived calculations from the North American Symptomatic Carotid Endarterectomy Trial (NASCET); and selected number needed to treat values for common therapies.

      This review contains 6 highly rendered figures, 9 tables, and 28 references.

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    • 6

      Medical Evaluation of the Surgical Patient

      By Marie Gerhard-Herman, MD; Jonathan Gates, MD
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      Medical Evaluation of the Surgical Patient

      • MARIE GERHARD-HERMAN, MDDepartment of Medicine, Cardiovascular Division, Brigham and Womens Hospital, Boston, MA
      • JONATHAN GATES, MDDirector of the Burn and Trauma Unit, Department of Surgery, Brigham and Womens Hospital, Boston, MA

      Medical evaluation prior to surgery includes risk assessment and the institution of therapies to decrease perioperative morbidity and mortality to improve patient outcomes. The most effective medical consultation for surgical patients begins with an assessment of the individual patient and knowledge of the planned surgery and anesthesia followed by clear communication of a concise and specific recommended plan of perioperative care to the surgical team. This chapter describes anesthetic, cardiac, pulmonary, hepatic, nutritional, and endocrine risk assessment. Perioperative thrombotic management and postoperative care and complications, including fluid management; pulmonary, cardiac, renal complications; and delirium are discussed. Tables outline the American Society of Anesthesiologists class and perioperative mortality risk, a comparison of the Revised Cardiac Risk Index and National Surgery Quality Improvement Program, Duke Activity Status Index, high-risk stress test findings, markers for increased perioperative risk in pulmonary hypertension, aortic stenosis and nonemergent noncardiac surgery, risk factors for pulmonary complications in noncardiac surgery, the Model for End-Stage Liver Disease score to predict postoperative mortality, venous thromboembolism risk factors and options for pharmacologic prophylactic regimens, perioperative management of warfarin, and Brigham and Women’s Hospital guidelines for postoperative blood product replacement. Figures include a care algorithm for noncardiac surgery, an illustration of types of myocardial infarction, and an algorithm for the treatment of postoperative delirium.

      This chapter contains 3 highly rendered figures, 12 tables, 68 references, and 5 MCQs.

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  • Dermatology
    • 1

      Approach to the Diagnosis of Skin Disease

      By Robert T. Brodell, MD; Miles Dunbar, MS; Stephen E. Helms, MD
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      Approach to the Diagnosis of Skin Disease

      • ROBERT T. BRODELL, MDProfessor and Chair, Department of Dermatology and Professor of Pathology, University of Mississippi Medical School, Jackson, MI, Instructor in Dermatology, University of Rochester School of Medicine and Dentistry, Rochester, NY
      • MILES DUNBAR, MSUniversity of Mississippi School of Medicine, Jackson, MI
      • STEPHEN E. HELMS, MDAssociate Professor of Internal Medicine, Dermatology Section, Northeastern Ohio Universities College of Medicine, Rootstown, Ohio, Assistant Clinical Professor of Dermatology, Case Western Reserve University School of Medicine, Cleveland, Ohio, Professor of Dermatology, University of Mississippi Medical School, Jackson, MI

      Patients frequently see their primary care physician for skin disease; however, primary care physicians treat few cases of individual skin conditions, and the types of disease they treat tend to be few in number. The relative inexperience with cutaneous presentations gives rise to possible error in the dermatologic care offered by nondermatologists. An accurate diagnosis of a cutaneous disease is most reliably achieved by a stepwise approach to patient evaluation, beginning with an examination of the morphologic features of the skin lesions and frequently culminating in diagnostic testing. This chapter reviews the primary skin lesions that allow categorization of dermatologic disease (e.g., papulosquamous diseases, blistering diseases, nonscaling erythematous and infiltrative diseases, and tumors) and presents a method by which the physician can narrow the possible causes of a specific presentation and arrive at a diagnosis in a cost-effective manner. Tables list consensus-based terminology used by dermatologists to describe morphologic features of skin lesions, all with photographs, as well as categories of skin disease with accompanying photographs. Figures include schematic drawings and clinical photographs of primary lesions that are characteristic of various skin diseases.
      This chapter contains 12 highly rendered figures, 4 tables, 1 sidebar, 17 references, 5 MCQs, and 1 teaching slide set.

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    • 2

      Papulosquamous Disorders

      By Elizabeth A. Abel, MD
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      Papulosquamous Disorders

      • ELIZABETH A. ABEL, MDAdjunct Clinical Professor of Dermatology, Stanford University School of Medicine, Stanford, CA, Private Practice, California Skin Institute, Mountain View, CA

      Papulosquamous disorders comprise a group of dermatoses that have distinct morphologic features. The characteristic primary lesion of these disorders is a papule, usually erythematous, that has a variable amount of scaling on the surface. Plaques or patches form through coalescence of the primary lesions. Some common papulosquamous dermatoses are pityriasis rosea, lichen planus, seborrheic dermatitis, tinea corporis, pityriasis rubra pilaris, psoriasis, and parapsoriasis. The etiology, diagnosis, and treatment of pityriasis rosea, lichen planus, and seborrheic dermatitis (including seborrheic dermatitis associated with AIDS) are discussed in this chapter. Also discussed are the diagnosis and treatment of pityriasis rubra, parapsoriasis (pityriasis lichenoides and small- and large-plaque parapsoriasis), and erythroderma. This chapter includes color photographs of the aforementioned dermatoses plus the Koebner phenomenon, lichen planus of the mucous membranes, and erythroderma in Sézary syndrome.

      This review contains 11 highly rendered figures and 79 references.

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    • 3

      Psoriasis

      By Elizabeth A. Abel, MD; Mark Lebwohl, MD
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      Psoriasis

      • ELIZABETH A. ABEL, MDAdjunct Clinical Professor of Dermatology, Stanford University School of Medicine, Stanford, CA, Private Practice, California Skin Institute, Mountain View, CA
      • MARK LEBWOHL, MDSol and Clara Kest Professor and Chairman, Department of Dermatology, Mount Sinai School of Medicine, New York City, NY

      Psoriasis is an immune-mediated inflammatory skin disorder characterized by chronic, scaling, erythematous patches and plaques of skin. This chapter discusses the epidemiology and pathogenesis of psoriasis, as well as the role of genetic factors, psychological stress, climate, concurrent infection, and drugs in the etiology of the disorder. A diagnostic summary highlights histopathologic features and reviews clinical variants, including plaque-type, guttate, erythrodermic, and pustular psoriasis, and special presentations, such as nail psoriasis and psoriatic arthritis. The chapter details various treatments and their indications and side effects; a discussion of topical therapies addresses topical corticosteroids, vitamin D analogues, tazarotene, tars, and anthralin. Phototherapy with ultraviolet B light (UVB); photochemotherapy with psoralen plus ultraviolet A light (PUVA); and systemic therapies such as methotrexate, acitretin, cyclosporine, tacrolimus, hydroxyurea, sulfasalazine, combination therapy, and other systemic therapies are reviewed. The chapter also highlights the current status of biologic therapies, including alefacept, etanercept, infliximab, adalimumab, and ustekinumab, plus some newer agents. The chapter includes 1 table and 21 figures, with the table ranking topical steroids by potency. Figures include various images of plaque psoriasis, psoriasis of the palms, scaling plaques on the feet, inverse psoriasis of the vulva and inguinal folds, guttate psoriasis, erythrodermic psoriasis, pustular psoriasis, psoriasis involving the fingernails and toenails, plus psoriasis in a child before and after phototherapy.

      This review contains 12 highly rendered figures, 1 table, and 142 references.

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    • 4

      Eczematous Disorders, Atopic Dermatitis, and Ichthyses

      By Savina Aneja, BA; Lauren Y. Cao, MD, MS; James S. Taylor, MD
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      Eczematous Disorders, Atopic Dermatitis, and Ichthyses

      • SAVINA ANEJA, BACase Western Reserve University School of Medicine, Cleveland, OH
      • LAUREN Y. CAO, MD, MSCase Western Reserve University School of Medicine, Cleveland, OH
      • JAMES S. TAYLOR, MDDepartment of Dermatology, Dermatology-Plastic Surgery Institute, Cleveland Clinic, Cleveland, OH

      There are several types of eczematous disorders: contact dermatitis and seborrheic dermatitis are common; less common eczematous disorders are nummular and dyshidrotic eczemas. Atopic dermatitis (AD) is a common chronic inflammatory dermatosis. Etiology and pathogenesis, diagnosis, and differential diagnosis are discussed, along with a thorough description of treatment for AD. Figures show examples of severe eczematous patches in an infant and lichenified patches. The tables list diagnostic criteria for AD and outline treatment options. The ichthyoses (characterized by excessive scaling) section includes descriptions of ichthyosis vulgaris, X-linked ichthyosis, lamellar ichthyosis, congenital ichthyosiform erythroderma, epidermolytic hyperkeratosis, and acquired ichthyoses. Treatment for ichthyoses is also discussed. Figures illustrate clinical presentations of severe eczematous patches, lichenified patches, erythroderma in an infant, and acquired ichthyosis. Tables present the diagnostic criteria and differential diagnosis of AD.
      This chapter contains 9 figures, 2 tables, 84 references, 5 Board-styled MCQs, and 1 teaching slide set.

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    • 5

      Contact Dermatitis and Related Disorders

      By Seth R. Stevens, MD
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      Contact Dermatitis and Related Disorders

      • SETH R. STEVENS, MD Partner Physician, Southern California Permanente Medical Group, Woodland Hills, CA, and Assistant Clinical Professor, Case Medical School, Cleveland, OH

      Contact dermatitis is an acute or chronic skin inflammation resulting from interaction with a chemical, biologic, or physical agent. This chapter discusses the major types of contact dermatitis, including irritant contact dermatitis and allergic contact dermatitis (ACD), and their predisposing factors, pathogenesis, diagnosis, risk reduction, and treatment. Also reviewed are specific etiologic forms of contact dermatitis (including topical medication allergy dermatitis, systemic contact dermatitis, clothing, textile and shoe dermatitis, and occupational contact dermatitis) as well as subtypes of contact dermatitis (including photosensitivity and latex allergy dermatitis). Figures show different types of dermatitis (such as chronic eczematous dermatitis, acute and ACD, and photocontact dermatitis), along with specific reactions from causes such as wearing a bib, a leather hatband, or sandals and from poison oak, glyceryl thioglycolate, tosylamide formaldehyde resin, rosin applied to a violin bow, bacitracin, and powdered natural rubber latex gloves. Tables list body sites affected by contact allergens, misconceptions about ACD, criteria for determining who should be given a patch test, key points in diagnosis of ACD, North America patch-test results from 2003 through 2004, topical sensitizers and potential systemic cross-reactants, substances that may cause systemic contact dermatitis, clinical features of systemic contact dermatitis, criteria for establishing occupational causation of contact dermatitis, topical and systemic photosensitizers, and distinguishing features of phototoxic versus photoallergic contact dermatitis. A sidebar lists Internet resources on contact dermatitis.

      This review contains 12 highly rendered figures, 11 tables, and 160 references.

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    • 6

      Cutaneous Adverse Drug Reactions

      By Neil H. Shear, MD, FRCPC; Sandra Knowles, BScPhm; Lori Shapiro, MD, FRCPC
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      Cutaneous Adverse Drug Reactions

      • NEIL H. SHEAR, MD, FRCPCProfessor and Chief of Dermatology, Department of Medicine, Divisions of Dermatology and Clinical Pharmacology, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, CA
      • SANDRA KNOWLES, BSCPHMAssistant Professor (Status Only), Department of Pharmacy, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, CA
      • LORI SHAPIRO, MD, FRCPCAssistant Professor of Medicine, Department of Medicine, Division of Dermatology, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, CA

      An adverse drug reaction is defined as any noxious, unintended, and undesired effect of a drug that occurs at doses used in humans for prophylaxis, diagnosis, or therapy. A cutaneous eruption is one of the most common manifestations of an adverse drug reaction. This chapter reviews the epidemiology, etiology, diagnosis, clinical manifestations, and differential diagnosis of adverse drug reactions, as well as laboratory tests for them. Also discussed are the types of cutaneous eruption: exanthematous eruption, urticarial eruption, blistering eruption, pustular eruption, and others. The simple and complex forms of each type of eruption are reviewed. The chapter includes 4 tables and 12 figures. Tables present the warning signs of a serious drug eruption, clinical features of hypersensitivity syndrome reaction and serum sickness-like reaction, characteristics of Stevens-Johnson Syndrome and toxic epidermal necrolysis, and clinical pearls to identify anticoagulant-induced skin necrosis. Figures illustrate hypersensitivity syndrome reaction, a fixed drug eruption from tetracycline, pseudoporphyria from naproxen, linear immunoglobulin A disease induced by vancomycin, pemphigus foliaceus from taking enalapril, pemphigus vulgaris from taking penicillamine, toxic epidermal necrolysis after starting phenytoin therapy, acneiform drug eruption due to gefitinib, acute generalized exanthematous pustulosis from cloxacillin, coumarin-induced skin necrosis, a lichenoid drug eruption associated with ramipril, and leukocytoclastic vasculitis from hydrochlorothiazide. This chapter contains 106 references.

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    • 7

      Fungal, Bacterial, and Viral Infections of the Skin

      By Jan V. Hirschmann, MD
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      Fungal, Bacterial, and Viral Infections of the Skin

      • JAN V. HIRSCHMANN, MDProfessor of Medicine, University of Washington School of Medicine, Staff Physician, Puget Sound VA Medical Center, Seattle, WA

      The skin can become infected by viruses, fungi, and bacteria, including some that ordinarily are harmless colonizing organisms. The most common fungal infections are caused by dermatophytes, which can involve the hair, nails, and skin. Potassium hydroxide (KOH) preparations of specimens from affected areas typically demonstrate hyphae, and either topical or systemic antifungal therapy usually cures or controls the process. The most common bacterial pathogens are Staphylococcus aureus and group A streptococci, which, alone or together, can cause a wide variety of disorders, including impetigo, ecthyma, and cellulitis. Topical antibiotics may suffice for impetigo, but ecthyma and cellulitis require systemic treatment. S. aureus, including methicillin-resistant strains, can also cause furuncles, carbuncles, and cutaneous abscesses. For these infections, incision and drainage without antibiotics are usually curative. Warts are the most common cutaneous viral infection, and eradication can be difficult, especially where the skin is thick, such as the palms and soles, or the patient is immunocompromised. Most therapies consist of trying to destroy the viruses by mechanical, chemical, or immune mechanisms. This review covers dermatophyte infections, yeast infections, bacterial infections, and viral infections of the skin. Figures show the classic annular lesion of tinea corporis, a typical kerion presenting as a zoophilic Microsporum canis infection of the scalp (tinea capitis), tinea corporis, tinea barbae, tinea pedis between and under the toes and on the plantar surface, inflammatory tinea pedis, tinea unguium, tinea manuum, angular cheilitis, prominent satellite lesions of discrete vesicles associated with candidiasis, facial candidiasis, Candida paronychia, tinea versicolor, nonbullous impetigo, bullous impetigo, ecthyma, leg cellulitis, erythema and edema on the cheeks, eyelids, and nose, furuncle, carbuncle, nasal folliculitis, pitted keratolysis, trichomycosis axillaris, necrotizing fasciitis, Fournier gangrene, folliculitis, plantar wart, condyloma acuminatum, and benign lesions of bowenoid papulosis. Tables list dermatophyte species, terminology of dermatophyte infections, topical agents for dermatophyte infections, treatment options for impetigo (adult doses), and treatment options for erythrasma.

       

      This review contains 28 highly rendered figures, 5 tables, and 33 references

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    • 8

      Infestations

      By Dirk Elston, MD
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      Infestations

      • DIRK ELSTON, MDDirector, Ackerman Academy of Dermatopathology, New York, NY

      This review looks at parasitic diseases of the skin. Scabies, caused by the human itch mite (Sarcoptes scabiei), and pediculosis, caused by the bloodsucking louse, are the most prevalent parasitic diseases in temperate regions. For treatment of scabies, ivermectin is suitable for mass drug administration during severe outbreaks, although patients with heavy scabies infestation may exhibit Mazzotti reactions during treatment with oral ivermectin. Another promising scabicide is Tinospora cordifolia lotion. The increase in global travel has also meant a worldwide increase in parasitic disorders endemic to tropical regions; these disorders include cutaneous larva migrans, pyodermas, arthropod-reactive dermatitis, myiasis, tungiasis, urticaria, and cutaneous and mucocutaneous leishmaniasis. Finally, patients with delusional parasitosis will express the belief that parasitical organisms are infesting their skin. Pimozide, an antipsychotic, has been successfully used to treat delusional parasitosis.

      This module contains 16 highly rendered figures, 2 tables, 15 references, and 5 MCQs.

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    • 9

      Vesiculobullous Diseases

      By Jacob Levitt, MD, FAAD; Annette Czernik, MD, FAAD; Bonnie Koo, MD
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      Vesiculobullous Diseases

      • JACOB LEVITT, MD, FAADVice Chairman, Residency Director, and Associate Professor, The Mount Sinai School of Medicine, New York, NY
      • ANNETTE CZERNIK, MD, FAADAssistant Professor, The Mount Sinai School of Medicine, New York, NY
      • BONNIE KOO, MDUniversity of California, Irvine, Irvine, CA

      Vesiculobullous diseases can be characterized most easily by the anatomic location of the blister cleft, which can result from autoimmune-mediated adhesion protein dysfunction, genetically faulty proteins, drug-induced keratinocyte necrosis, and infectious or traumatic causes. This chapter discusses several paradigmatic vesiculobullous diseases: pemphigus (including pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus, and Hailey-Hailey disease), bullous pemphigoid, cicatricial pemphigoid, epidermolysis bullosa acquisita, dermatitis herpetiformis, linear IgA bullous dermatosis, epidermolysis bullosa, and various infectious vesiculobullous diseases. Clinical presentation, diagnosis and monitoring, and treatment of these diseases are provided. Tables list systemic drugs used for treating pemphigus; the diseases and disease subtypes, protein defects, inheritance patterns, and clinical features of epidermolysis bullosa; and blister planes for various diseases. Figures (30 in all) illustrate presentations of vesiculobullous diseases.

      This review contains 30 figures, 3 tables, and 137 references.

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    • 10

      Malignant Cutaneous Tumors

      By Allan C. Halpern, MD; Patricia L. Myskowski, MD
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      Malignant Cutaneous Tumors

      • ALLAN C. HALPERN, MDChief, Dermatology Service, Memorial Sloan-Kettering Cancer Center, New York, NY
      • PATRICIA L. MYSKOWSKI, MDAttending Physician, Dermatology Service, Memorial Sloan-Kettering Cancer Center, New York, NY

      This chapter reviews the most common malignant cutaneous tumors. The section on malignant tumors of the epidermis discusses nonmelanoma skin cancer (i.e., basal cell carcinoma and squamous cell carcinoma) and malignant melanoma. The section on malignant tumors of the dermis covers metastatic tumors, primary tumors (Merkel cell carcinoma, Paget disease, extramammary Paget disease, angiosarcoma, and dermatofibrosarcoma protuberans), and Kaposi sarcoma (i.e., classic Kaposi sarcoma, African Kaposi sarcoma, organ-transplant Kaposi sarcoma, and HIV-associated Kaposi sarcoma). The final section covers cutaneous lymphomas. The coverage of each disease includes a discussion of epidemiology, etiology, diagnosis, differential diagnosis, treatment, and prognosis. Tables provide the adjusted estimated relative risks of melanoma by nevus type and number, the American Joint Committee on Cancer (AJCC) TNM classification and staging system, the estimated probability of 10-year survival in patients with primary cutaneous melanoma, and an overview of overview of therapy for cutaneous T cell lymphoma. Figures illustrate the presentation of many malignant cutaneous tumors.

      This review contains 10 highly rendered figures, 5 tables, and 105 references.

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    • 11

      Benign Cutaneous Tumors

      By Elizabeth A. Abel, MD
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      Benign Cutaneous Tumors

      • ELIZABETH A. ABEL, MDAdjunct Clinical Professor of Dermatology, Stanford University School of Medicine, Stanford, CA, Private Practice, California Skin Institute, Mountain View, CA

      Tumors of the cutaneous surface may arise from the epidermis, dermis, or subcutaneous tissue or from any of the specialized cell types in the skin or its appendages. Broad categories include tumors derived from epithelial, melanocytic, or connective tissue structures. Within each location or cell type, lesions are classified as benign, malignant, or, in certain cases, premalignant. Benign epithelial tumors include tumors of the surface epidermis that form keratin, tumors of the epidermal appendages, and cysts of the skin. Melanocytic (pigment-forming) lesions are very common. One of the most frequently encountered forms is the nevus cell nevus. Tumors that are derived from connective tissue include fibromas, histiocytomas, lipomas, leiomyomas, and hemangiomas. This chapter provides an overview of each type of tumor, including sections on epithelial tumors, tumors of the epidermal appendages, familial tumor syndromes, melanocytic tumors, neural tumors, connective tissue tumors, vascular birthmarks, acquired vascular disorders, Kimura disease, lipoma, leiomyoma, and lymphangioma circumscriptum. The sections discuss various forms and their diagnosis, differential diagnosis, and treatment. Figures accompany the descriptions.

      This chapter contains 83 references, 26 highly rendered figures, and 5 MCQs.

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    • 12

      Acne Vulgaris and Rosacea

      By James Q. Del Rosso, DO
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      Acne Vulgaris and Rosacea

      • JAMES Q. DEL ROSSO, DOAdjunct Clinical Professor (Dermatology), Touro University College of Osteopathic Medicine, Henderson, NV

      Acne vulgaris is the most common disorder seen in general dermatology practice, accounting for approximately 10% of visits each year. Both sexes and all ethnicities are affected, usually in the late preteenage or early teenage years. Both inflammatory and comedonal lesions of acne vulgaris characteristically involve the face, but truncal involvement is also relatively common. Multiple clinical presentations may be observed, with severity often progressing over time during adolescence. Severe forms of acne vulgaris can be especially disfiguring and debilitating, and are more likely to lead to permanent scarring. Therapeutic options are chosen primarily on the basis of clinical severity, with adjustments in treatment made on the basis of response or disease progression. Rosacea begins in adulthood, usually in the third decade of life or later. The disorder predominantly affects the central face in fair-skinned people, mostly those of northern European ancestry, although individuals of any race may be affected. Rosacea may present as one or more of a variety of clinical phenotypes (subtypes); it is a chronic disorder characterized by periods of exacerbation and remission. Fortunately, rosacea is not associated with scarring, although a subset of patients may develop localized proliferations of sebaceous and fibrous tissue called a phyma. Like acne vulgaris, rosacea may also adversely impact quality of life. Figures in this chapter illustrate acne vulgaris and inflammatory papules. Tables detail laboratory evaluation for women with acne vulgaris and hyperandrogenism, surgical/physical modality options for specific acne lesions and acne scars, major topical therapies for acne vulgaris, and commonly prescribed systemic therapies for acne. This chapter contains 50 references.

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    • 13

      Disorders of Hair

      By James Q. Del Rosso, DO
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      Disorders of Hair

      • JAMES Q. DEL ROSSO, DOAdjunct Clinical Professor (Dermatology), Touro University College of Osteopathic Medicine, Henderson, NV

      A basic knowledge of the hair growth cycle is needed to evaluate disorders of hair growth. This chapter presents a broad overview of the physiology and evaluation of hair growth, as well as discussions of specific types of alopecia. The epidemiology, pathogenesis, diagnosis, and treatment of androgenetic alopecia, the most common type of nonscarring hair loss, are covered. Diffuse hair shedding is generalized hair loss over the entire scalp. Diagnosis and treatment of telogen effluvium, anagen arrest (anagen effluvium), and other causes of diffuse hair shedding are covered in detail. Alopecia areata, typically characterized by patchy hair loss; cicatricial alopecia, which results from permanent scarring of the hair follicles; and miscellaneous causes of hair loss are also discussed. Tables list the causes of diffuse and cicatricial alopecia, telogen effluvium, and miscellaneous chemicals and categories of drugs that can cause alopecia, as well as miscellaneous causes of hair loss. Included is an algorithm outlining the approach to diagnosing nonscarring alopecia, as well as a variety of clinical photographs.

      This review contains 9 highly rendered figures, 6 tables, and 42 references.

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    • 14

      Diseases of the Nail Unit

      By Jennifer Nguyen, MD; George Cotserelis, MD
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      Diseases of the Nail Unit

      • JENNIFER NGUYEN, MDAssistant Professor of Dermatology, Department of Dermatology, Perelman School of Medicine of the University of Pennsylvania, Philadelphia, PA
      • GEORGE COTSERELIS, MDMilton Bixler Hartzell Professor of Dermatology, Department of Dermatology, Perelman School of Medicine of the University of Pennsylvania, Philadelphia, PA

      The human nail is a complex unit that includes five major modified cutaneous structures: the nail matrix, nail bed, nail plate, nail folds, and cuticle (eponychium). This chapter discusses the function and structure of the five nail components and the pathophysiology affecting each. Also reviewed are nail findings associated with underlying systemic and dermatologic conditions: splinter hemorrhages, koilonychia, transverse nail-plate depressions (Beau’s lines), onycholysis, leukonychia, clubbing, nail-plate pitting, and longitudinal pigmented bands. Infections of the nail are discussed, which include bacterial paronychia, chronic paronychia, and onychomycosis. Figures illustrate the longitudinal section of the fingernail, multiple pigmented longitudinal bands, psoriasis involving the fingernail, late-stage lichen planus of the fingernail, transverse linear grooves, Pseudomonas aeruginosa causing a green nail, psoriasis of the nail, melanonychia striata, and a nail specimen for potassium hydroxide preparation. Tables describe antifungal treatment for toenail onychomycosis as well as selected dermatologic disorders that affect the nail unit. This chapter contains 50 references.

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    • 15

      Disorders of Pigmentation

      By Pearl E. Grimes, MD
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      Disorders of Pigmentation

      • PEARL E. GRIMES, MDClinical Professor of Dermatology, Division of Dermatology, University of California Los Angeles David Geffen School of Medicine, Los Angeles, CA; Director of the Vitiligo and Pigmentation Institute of Southern California, Los Angeles, CA

      The disorders of skin pigmentation discussed in this chapter fall into two categories: disorders of hyperpigmentation (melasma, postinflammatory hyperpigmentation, drug-induced hyperpigmentation, erythema dyschromicum perstans, lentigines, confluent and reticulated papillomatosis of Gougerot and Carteaud, and Dowling-Degos disease) and disorders of hypopigmentation (vitiligo, albinism, piebaldism, and idiopathic guttate hypomelanosis). The definition, epidemiology, etiology and pathogenesis, diagnosis, differential diagnosis, and treatment are discussed for each condition. Figures show examples of melasma, hyperpigmentation secondary to acne, vitiligo and its response to treatment with tacrolimus and with narrow-band ultraviolet B (UVB) light, Vogt-Koyanagi-Harada syndrome, and piebaldism. A table lists therapeutic approaches to vitiligo. This chapter contains 182 references.

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    • 16

      Pruritus

      By Hong-Liang Tey, MRCP(UK); Gil Yosipovitch, MD; Jeffrey D. Bernhard, MD, FRCP(Edin)
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      Pruritus

      • HONG-LIANG TEY, MRCP(UK)Consultant, National Skin Centre, Singapore
      • GIL YOSIPOVITCH, MDProfessor and Chair, Department of Dermatology and Itch Center, Temple University School of Medicine, Philadelphia, PA
      • JEFFREY D. BERNHARD, MD, FRCP(EDIN)Editor Emeritus, Journal of the American Academy of Dermatology, Professor Emeritus, University of Massachusetts Medical School, MA

      Pruritus, or itch, can be defined as a sensation that elicits the desire to scratch. Normal physiologic "acute" itch occurs daily and can usually be abolished by scratching the affected area. On the other hand, chronic itch (defined as itch that persists for 6 weeks or more) is often made worse by scratching and is associated with significant morbidity. The focus of this chapter is on chronic pruritus. Discussion includes causes, clinical evaluation, investigation of, and treatment for chronic pruritus. Tables cover the etiologic classification of chronic pruritus, a morphologic approach to typically pruritic dermatoses and their classic distribution (with illustrative images), systemic diseases and associated clinical signs, localized pruritus and underlying neuropathy, screening tests for pruritus, further investigations following results of clinical findings and screening tests, general measures for patients managing pruritus, topical treatment, topical calcineurin inhibitors, systemic therapies, recommended stepwise treatment options, and phototherapy. Also included are a patient history checklist, an algorithm outlining the approach to chronic pruritus, and images depicting various forms of pruritus.

      This review contains 16 highly rendered figures (including table images), 13 tables, and 41 references.

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    • 17

      Cutaneous Manifestations of Systemic Diseases

      By Mark Lebwohl, MD
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      Cutaneous Manifestations of Systemic Diseases

      • MARK LEBWOHL, MDSol and Clara Kest Professor and Chairman, Department of Dermatology, Mount Sinai School of Medicine, New York City, NY

      This chapter reviews key cutaneous manifestations of systemic diseases that should be recognized by most physicians and highlights recent developments in the diagnosis and management of those disorders. In many of the disorders presented, workup and therapy of the underlying systemic condition are essential to a favorable outcome. Cardiopulmonary, vascular, endocrinologic, gastrointestinal, hematologic, immunodeficiency, infectious, neurologic, renal, and rheumatologic diseases are discussed. Many of the syndromes characterized by physical symptoms are accompanied by photographs demonstrating the manifestations for the reader's reference.

      This review contains 28 figures and 104 references.

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  • Endocrinology & Metabolism
    • 1

      Approach to the Patient With Endocrine Disorders

      By Graham T. McMahon, MD, MMSc; Robert G. Dluhy, MD
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      Approach to the Patient With Endocrine Disorders

      • GRAHAM T. MCMAHON, MD, MMSCAssociate Professor of Medicine, Harvard Medical School, Division of Endocrinology, Diabetes and Hypertension, Brigham and Women’s Hospital, Boston, MA
      • ROBERT G. DLUHY, MDProfessor of Medicine, Harvard Medical School, Division of Endocrinology, Diabetes and Hypertension, Brigham and Women’s Hospital, Boston, MA

      This chapter reviews endocrine systems, the endocrine patient, presentations of endocrine diseases, the endocrine history, the physical examination, endocrine testing, imaging in endocrinology, the endocrinologist, the chronic care relationship, self-evaluation and the endocrinologist, and engagement with technology. Tables outline hormones and their associated syndromes, clusters of contrasting symptoms and signs of over- and underactivity of the thyroid and adrenal glands, evaluation of an endocrine problem, and variability in selected hormone concentrations over time. Figures illustrate the use of feedback loops regulating calcium homeostasis in the differential diagnosis of hypercalcemia, how physiologic changes in growth hormone levels can be used to detect syndromes of growth hormone excess and insufficiency, the evaluation of incidentaloma, and the chronic care model.

      This chapter contains 4 highly rendered figures, 4 tables, 8 references, and 5 MCQs.

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    • 2

      Adrenal Insufficiency

      By D. Lynn Loriaux, MD, PhD, MACP
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      Adrenal Insufficiency

      • D. LYNN LORIAUX, MD, PHD, MACPProfessor of Medicine and Chief, Division of Endocrinology and Metabolism, Oregon Health and Science University, Portland, OR

      Adrenal insufficiency (Addison disease) can be categorized as primary or secondary; the former results from adrenal cortex destruction, whereas the latter is caused by disruption of pituitary secretion of adrenocorticotropic hormone. The clinical pictures are the same, and their signs can be differentiated only by the presence of hyperpigmentation and vitiligo in autoimmune disease. Diagnosing both chronic and acute syndromes requires laboratory confirmation; however, the only available diagnostic test for adrenal insufficiency is cosyntropin stimulation. Relative adrenal insufficiency is a hypothetical situation stemming from misinterpretation of this test, and there is no pathophysiologic evidence of its existence. The most common form of congenital adrenal hyperplasia is the 21-hydroxylase deficiency syndrome.

      This module contains 1 highly rendered figure, 2 tables, 4 references, and 5 MCQs.

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    • 3

      Cushing Syndrome

      By Lynnette Nieman, MD
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      Cushing Syndrome

      • LYNNETTE NIEMAN, MDProgram on Adult and Reproductive Endocrinology, National Institutes of Health, Bethesda, MD

      Cushing syndrome is a condition with protean manifestations that are caused by chronic exposure to excess glucocorticoids. Treatment with supraphysiologic doses of glucocorticoids is the most common cause. Pathologic hypercortisolism may result from autonomous adrenal production or as a result of the action of excessive adrenocorticotropic hormone (ACTH) production by a tumor, which stimulates adrenal cortisol production. Primary adrenal forms include unilateral adenoma or carcinoma or, rarely, bilateral hyperplasia and/or nodules. This chapter covers the epidemiology, etiology, pathophysiology, and diagnosis of Cushing syndrome. Clinical manifestations, physical examination findings, and laboratory tests, including tests of the blood and other body fluids, imaging studies, and biopsy, are discussed. The differential diagnosis, treatment options, complications, and prognosis are described. Tables outline clinical features and causes of Cushing syndrome, abnormalities associated with primary adrenal causes of Cushing syndrome, diagnostic accuracy of screening tests, endogenous hypercortisolism without Cushing syndrome, and medical therapy for Cushing syndrome. Figures illustrate the causes of Cushing syndrome and a comparison of the hypothalamic-pituitary-adrenal axis in patients with ACTH-dependent Cushing syndrome and those with pseudo–Cushing syndrome. Algorithms show the evaluation of possible Cushing syndrome and evaluation of the causes of Cushing syndrome. Second-line treatments for Cushing syndrome when surgery fails or is not possible are also detailed.

      This chapter contains 5 highly rendered figures, 6 tables, 49 references, and 5 MCQs.

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    • 4

      Adrenal Hypertension

      By Naomi D.L. Fisher, MD; Gail K. Adler, MD, PhD
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      Adrenal Hypertension

      • NAOMI D.L. FISHER, MDDivision of Endocrinology, Diabetes, and Hypertension, Brigham and Women’s Hospital, Boston, MA
      • GAIL K. ADLER, MD, PHDDivision of Endocrinology, Diabetes, and Hypertension, Brigham and Women’s Hospital, Boston, MA

      The secondary causes of hypertension are associated with the excess of the principal hormones produced by the adrenal glands: cortisol, epinephrine, and aldosterone. Excess aldosterone production is recognized as primary hyperaldosteronism, or primary aldosteronism (PA). Individuals with PA are at increased risk for a variety of disorders, including atrial fibrillation, coronary artery disease, myocardial infarction, and stroke. Pheochromocytoma is a very rare tumor (accounting for fewer than one in 10,000 hypertension cases) and is marked by high secretions of catecholamines, mostly epinephrine as well as norepinephrine. Cushing disease and Cushing syndrome are addressed in a separate review.

      This module contains 5 highly rendered figures, 4 tables, 39 references, and 5 MCQs.

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    • 5

      Adrenal Neoplasia

      By Anand Vaidya, MD, MMSc
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      Adrenal Neoplasia

      • ANAND VAIDYA, MD, MMSCDirector, Center for Adrenal Disorders, Division of Endocrinology, Diabetes, and Hypertension, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA

      The adrenal glands are composed of two distinct tissue types: the cortex, which serves as a factory for adrenal steroidogenesis, and the medulla, which produces catecholamines as a neuroendocrine organ. Neoplasia of the adrenal is approached by considering both whether the tumor is benign or malignant and whether it may represent a hormonally active tumor that can contribute to cardiometabolic disease or a hormonally silent tumor. Adrenal neoplasia is rarely malignant. This module discusses the approach to an incidentally discovered adrenal mass, with emphasis on the clinical determination as to whether it is benign or malignant and hormonally functional or nonfunctional. The pathogenesis and genetics of hyperaldosteronism and pheochromocytoma-paraganglioma are reviewed, as are the pathogenesis and management of adrenocortical carcinoma. Tables describe the differential diagnosis and diagnostic approach for an incidentally discovered adrenal mass; suggested biochemical screening tests for incidentally discovered adrenal masses; radiographic features of adrenal masses to determine benign or malignant potential; and the genetics of primary hyperaldosteronism, pheochromocytoma, and paraganglioma syndromes. A drawing shows the genetic mechanisms of hyperaldosteronism in familial hyperaldosteronism type III. Algorithms outline the suggested management approach for the incidentally discovered adrenal mass, genetic counseling and testing for patients with pheochromocytoma or paraganglioma, testing for family members of patients with pheochromocytoma and positive genetic testing, patients with stage I–III adrenocortical carcinoma, and patients with advanced adrenocortical carcinoma.

      This module contains 6 highly rendered figures, 6 tables, 55 references, and 5 MCQs.

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    • 6

      The Pituitary

      By Shlomo Melmed, MD, FACP
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      The Pituitary

      • SHLOMO MELMED, MD, FACPSenior Vice President, Academic Affairs, Dean of Medical Faculty, Cedars Sinai Medical Center, 8700 Beverly Blvd, Los Angeles, CA 90048

      The pituitary gland plays a prominent role in regulating the critical hormonal functions of growth, development, reproduction, stress homeostasis, and metabolic control; for this reason, the pituitary has been termed the master gland. The anterior pituitary synthesizes and secretes adrenocorticotropic hormone (ACTH), growth hormone (GH), prolactin (PRL), thyroid-stimulating hormone (TSH), follicle-stimulating hormone (FSH), and luteinizing hormone (LH). The posterior pituitary secretes vasopressin (also known as antidiuretic hormone [ADH]) and oxytocin, which are synthesized in the hypothalamus. This chapter describes genetic syndromes associated with pituitary tumors, including multiple endocrine neoplasia type 1. Information is provided on the diagnosis and treatment of disorders associated with the underproduction and hypersecretion of pituitary hormones, including Cushing syndrome, pituitary mass effects, and pituitary failure. Figures illustrate pituitary anatomy and physiology and management of prolactinoma. Tables describe hypothalamic and related pituitary hormones; pituitary hormones, their mediators, and their effects; effects of pituitary adenomas; causes of hyperprolactinemia; dopamine agonists in the treatment of prolactinomas; findings in adult GH deficiency; causes and features of acromegaly; clinical features of Cushing syndrome; causes of pituitary failure; and replacement therapy for hypopituitarism in adults.

      This review contains 4 references, 11 tables, and 43 references.

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    • 7

      The Thyroid

      By Paul W. Ladenson, MD
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      The Thyroid

      • PAUL W. LADENSON, MDJohn Eager Howard Professor of Endocrinology & Metabolism, Professor of Medicine, Pathology, Oncology, Radiology & Radiological Sciences, and International Health, University Distinguished Service Professor, Director, Division of Endocrinology & Metabolism, Johns Hopkins Medical Institutions, Baltimore, Maryland

      Thyroid dysfunction includes hypothyroidism and thyrotoxicosis (sometimes referred to as hyperthyroidism). Hypothyroidism occurs more commonly in women than in men. Certain individuals are at higher risk for developing the disorder, with iodine deficiency an important cause of congenital hypothyroidism in some parts of the world. The leading cause of hypothyroidism is autoimmune thyroiditis (Hashimoto disease); other causes include thyroid surgery, thyroid irradiation, certain drugs and toxins, and diseases that interfere with thyrotropin-releasing hormone production. Some disorders and life stress can produce symptoms similar to hypothyroidism, making clinical diagnosis more difficult. Abnormalities in routine laboratory tests are the first clue, and measurement of serum thyroid-stimulating hormone (TSH) should usually be the first subsequent test. The key to diagnosis is simply for the physician to keep the condition in mind. Management of the disorder includes thyroid hormone therapy, but there are special therapeutic issues to be considered. Thyrotoxicosis falls into three etiologic categories: abnormal stimulation of the thyroid gland, gland autonomy, and gland inflammation with unregulated thyroid hormone release. Physical signs are often key to diagnosis and differential diagnosis. It is vital for the physician to establish the underlying cause of thyrotoxicosis because the etiology determines the therapy. Surgery is the best choice for treating hyperthyroidism in several settings. Complications include atrial fibrillation, heart failure, thyroid crisis (thyroid storm), and ocular and orbital involvement for patients with diffuse toxic goiter (Graves disease). There are several types of thyroiditis: Hashimoto disease, lymphocytic thyroiditis (called painless thyroiditis, silent thyroiditis, or postpartum thyroiditis), subacute thyroiditis, acute or suppurative thyroiditis, and Reidel thyroiditis. Evidence suggests there is a genetic predisposition to Graves disease and thyroid nodules, but environmental factors include smoking, large amounts of dietary iodine, stressful life events, and preceding infection with certain bacterial agents. Thyroid nodules are relatively common in adults, and thyroid cancer is the seventh most common malignancy in the United States. The cause of most thyroid cancers is unknown. Most present as a thyroid nodule in an otherwise asymptomatic and euthyroid patient. Treatment includes surgical thyroidectomy, radioiodine, and TSH-suppressive thyroid hormone therapy. This chapter contains 128 references.

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    • 8

      Diseases of Calcium Metabolism and Metabolic Bone Disease

      By Carolyn Becker, MD
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      Diseases of Calcium Metabolism and Metabolic Bone Disease

      • CAROLYN BECKER, MDAssociate Professor, Division of Endocrinology, Diabetes and Hypertension, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA

      Calcium is the chief mineral component of the skeleton and plays major roles in neurologic transmission, muscle contraction, and blood coagulation, in addition to being a ubiquitous intracellular signal. This chapter reviews calcium metabolism and the perturbations in this system, which predominately manifest as abnormalities in serum calcium concentration or as alterations in bone mineral density. The disorders of calcium hemostasis discussed include hypercalcemia, hyperparathyroidism, non-parathyroid hormone-mediated hypercalcemia, and hypocalcemia. The epidemiology, pathogenesis, diagnosis, treatment, and complications of osteoporosis are reviewed. Other conditions discussed include osteomalacia and Paget’s disease of bone. Figures include regulation of serum calcium levels, the Venus Fly Trap model of the calcium-sensing receptor, secretion of parathyroid hormone in relation to serum ionized calcium level, the RANK-RANKL-osteoprotegerin regulation of bone remodeling, and radiologic differences between “typical” osteoporotic and “atypical” femur fracture. Tables describe the differential diagnosis of hypercalcemia, the 2008 National Institutes of Health Working Group recommendations regarding follow-up testing for patients with primary hyperparathyroidism who do not undergo surgery, a potential workup for non–PTH-mediated hypercalcemia, therapy for acute hypercalcemia, the differential diagnosis of hypocalcemia (as well as its management), risk factors for osteoporosis, a workup for secondary causes of osteoporosis, and a table of osteoporosis therapies. Also included are Internet resources for osteoporosis and bone metabolism. This chapter contains 112 references.

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    • 9

      Testes and Testicular Disorders

      By Elizabeth G. Nabel, MD, FACP
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      Testes and Testicular Disorders

      • ELIZABETH G. NABEL, MD, FACPPresident, Brigham and Women’s Hospital, Professor of Medicine, Harvard Medical School, Boston, MA

      The testes have two functions, the secretion of testosterone by the Leydig cells and the production of sperm by the seminiferous tubules. Although the cumulative effect of testosterone is to produce and maintain a phenotypic male, testosterone levels also affect such features as bone density, muscle mass, hemoglobin concentration, energy, and libido. This chapter discusses the physiology of gonadotropins and testosterone, spermatogenesis, and male reproductive function throughout life. Etiology, diagnosis, and treatment of primary and secondary hypogonadism are presented, including congenital anomalies and acquired conditions. Androgen insensitivity and gynecomastia are reviewed. The epidemiology, pathophysiology, diagnosis, and treatment of erectile dysfunction are summarized. Figures illustrate longitudinal changes in serum testosterone concentrations and free testosterone index in healthy men; and serum testosterone concentrations during long-term administration of injected, transdermal, and gel testosterone to hypogonadal men. Tables listing causes of primary and secondary hypogonadism are included. This chapter has 56 references.

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    • 10

      Type 1 Diabetes Mellitus

      By Joseph I. Wolfsdorf, MB, BCh; Katharine Garvey, MD, MPH
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      Type 1 Diabetes Mellitus

      • JOSEPH I. WOLFSDORF, MB, BCHProfessor of Pediatrics, Harvard Medical School, Boston, MA
      • KATHARINE GARVEY, MD, MPHInstructor of Pediatrics, Harvard Medical School, Boston, MA

      Type 1 diabetes mellitus is a heterogeneous metabolic disease characterized by destruction of the pancreatic beta cells resulting in an absolute deficiency of insulin secretion with subsequent hyperglycemia. This review details the definition and classification, epidemiology, pathophysiology, pathogenesis, prevention, diagnosis, and management of type 1 diabetes mellitus. Figures show the opposing actions of insulin and glucagon, particularly within the liver, on substrate flow and plasma levels; plasma glucose, insulin and C-peptide levels; the structure of human proinsulin; the cellular actions of insulin; measurement of insulin levels after the administration of glucose; the pathways that lead from insulin deficiency to the major clinical manifestations of type 1 diabetes mellitus; the pathogenesis of type 1 autoimmune diabetes mellitus; the relationship between hemoglobin A1C and calculated average glucose level; basal-bolus and insulin pump regimens; and management of diabetic ketoacidosis. Tables list the etiologic classification of diabetes mellitus, criteria for the diagnosis of diabetes, American Diabetes Association standards for glycemic control in diabetes mellitus, insulin preparations, potential advantages of continuous subcutaneous insulin infusion compared with multiple daily injections, cardiovascular risk factor screening and treatment, and typical admission laboratory findings and monitoring in diabetic ketoacidosis.

      This review contains 10 highly rendered figures, 7 tables, and 66 references.

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    • 11

      Hypoglycemia

      By F. John Service, MD, PhD; Adrian Vella, MD
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      Hypoglycemia

      • F. JOHN SERVICE, MD, PHDEmeritus Professor of Medicine, Mayo Clinic College of Medicine, Rochester, MN
      • ADRIAN VELLA, MDProfessor of Medicine, Mayo Clinic College of Medicine, Rochester, MN

      Hypoglycemia is a clinical syndrome of diverse causes characterized by episodes of low blood glucose. These episodes are typically marked by central nervous system manifestations (i.e., neuroglycopenia). This chapter reviews the classification, etiology, and diagnosis of hypoglycemia and discusses five etiologic conditions: insulinoma, factitious hypoglycemia, noninsulinoma pancreatogenous hypoglycemia syndrome, insulin autoimmune hypoglycemia, and hypoglycemia after gastric bypass. The epidemiology, diagnosis, and management of each condition are reviewed. One figure illustrates changes in serum glucose, insulin, C-peptide, proinsulin, and beta-hydroxybutyrate that occur during a 72-hour diagnostic fast. A second figure shows pancreatic ducts with insulin-positive cells associated with the ducts (nesidioblastosis). Tables provide the clinical classification of hypoglycemic disorders and the protocol for a prolonged supervised fast. This chapter contains 38 references.

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    • 12

      Type 2 Diabetes Mellitus

      By Matthew C. Riddle, MD; Saul Genuth, MD, FACP
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      Type 2 Diabetes Mellitus

      • MATTHEW C. RIDDLE, MDProfessor, Department of Medicine, and Head, Section of Diabetes, Oregon Health & Sciences University, Portland, OR
      • SAUL GENUTH, MD, FACPProfessor, Division of Clinical and Molecular Endocrinology, Case Western Reserve University School of Medicine, Cleveland, OH

      Type 2 diabetes mellitus (DM) is defined by high levels of plasma glucose and is associated with many long-term complications caused or enhanced by hyperglycemia and related metabolic abnormalities. This chapter discusses prediabetes; the metabolic syndrome; and the epidemiology, risk factors, pathophysiology, pathogenesis, prevention, screening, diagnosis, and management of type 2 DM. Figures illustrate the age-related prevalence of diabetes in the United States; insulin response to a graded infusion of glucose; insulin resistance in lean and obese diabetic and nondiabetic persons; plasma glucose, insulin, and glucagon responses to glucose ingestion in patients with type 2 DM; typical 24-hour glucose and insulin patterns in patients with type 2 DM; and the prevalence of retinopathy before and after the diagnosis of DM. Tables list American Diabetes Association diagnostic criteria for impaired fasting glucose (IFG), impaired glucose tolerance (IGT), and DM; and formulations and properties of oral and parenteral drugs commonly used for type 2 DM. This chapter contains 113 references.

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    • 13

      Gestational Diabetes Mellitus

      By Ellen W. Seely, MD; Chloe A. Zera, MD, MPH
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      Gestational Diabetes Mellitus

      • ELLEN W. SEELY, MDDirector of Clinical Research, Division of Endocrinology, Diabetes, and Hypertension, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA
      • CHLOE A. ZERA, MD, MPHDivision of Maternal Fetal Medicine, Department of Obstetrics, Gynecology and Reproductive Biology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA

      Gestational diabetes mellitus (GDM) has historically been defined as glucose intolerance first identified during pregnancy. The definition fails to distinguish overt (pre-gestational) diabetes diagnosed during pregnancy from glucose intolerance induced by pregnancy. Recently, the recognition that overt diabetes may first be identified in pregnancy has led to the recommendation that diabetes diagnosed in the first trimester should be termed type 2 diabetes (T2DM) rather than GDM , a clinically relevant difference in terminology as the outcomes and management of T2DM in pregnancy are distinct from outcomes and management of GDM. This chapter discusses the epidemiology, pathophysiology, screening, diagnosis, treatment and impact of GDM, as well as the obstetric management of GDM and management of GDM after pregnancy. Tables describe the risk factors for, and complications of, GDM; the diagnosis of pregestational (overt) diabetes; a comparison of third-trimester GDM diagnostic strategies; and Institute of Medicine gestational weight gain guidelines. A figure shows the cumulative incidence of type 2 diabetes following GDM pregnancy. This chapter contains 57 references.

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    • 14

      Complications of Diabetes Mellitus

      By Samuel Dagogo-Jack, MD, MBBS, FRCP, FACP
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      Complications of Diabetes Mellitus

      • SAMUEL DAGOGO-JACK, MD, MBBS, FRCP, FACPA. C. Mullins Professor & Chief, Division of Endocrinology, Diabetes and Metabolism, University of Tennessee Health Science Center, Memphis, TN

      The long-term complications of diabetes mellitus include microvascular, neuropathic, and macrovascular complications. Due to the long period of asymptomatic or undiagnosed diabetes, up to 25% of type 2 diabetes patients have already developed one or more microvascular complication by the time of diagnosis. This review covers microvascular complications, nephropathy, neuropathy, and macrovascular complications of diabetes mellitus. Figures show the pathways linking high blood glucose levels to the microvascular and neuropathic complications of diabetes, fundus photographs of both nonproliferative (or background) and proliferative retinopathy, the natural history of nephropathy in type 1 diabetes, data on the cumulative incidence of the first cardiovascular event and first nonfatal myocardial infarction, stroke, or death from the Diabetes Control and Complications Trial. Tables list screening for diabetes complications in adults, foot care recommendations for patients with diabetes, comparison of major trials of intensive glucose control and CVD outcomes, cardiovascular outcome studies with newer classes of antidiabetes agents, American Diabetes Association treatment recommendations for dyslipidemia in patients with diabetes, and targets of intervention for CVD risk reduction in diabetes,

       This review contains 8 highly rendered figures, 6 tables, and 120 references.

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    • 15

      Secondary Forms of Diabetes Mellitus

      By Ildiko Lingvay, MD, MPH, MSCS; Philip Raskin, MD, FACP
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      Secondary Forms of Diabetes Mellitus

      • ILDIKO LINGVAY, MD, MPH, MSCSAssistant Professor, University of Texas Southwestern Medical Center at Dallas, Dallas, TX
      • PHILIP RASKIN, MD, FACPProfessor of Medicine, Clifton and Betsy Robinson Chair in Biomedical Research, University of Texas Southwestern Medical Center at Dallas, Director, University Diabetic Treatment Center, Parkland Memorial Hospital, Dallas, TX

      Up to 5% of cases of diabetes mellitus have a specific identifiable cause and do not meet the diagnostic criteria for type 1, type 2, or gestational diabetes. These are called secondary forms of diabetes. The most common causes are pancreatic disorders; endocrinopathies; drugs, chemical agents or toxins; and genetic mutations or syndromes, including maturity-onset diabetes of the young (MODY). This chapter discusses the pathogenesis, diagnosis, management, complications, and prognosis of these forms of diabetes. Tables list the endocrinopathies; the drugs, chemicals, and toxins; and the genetic disorders that can cause secondary diabetes. This chapter contains 15 references.

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    • 16

      Obesity

      By Jonathan Q. Purnell, MD
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      Obesity

      • JONATHAN Q. PURNELL, MDAssociate Professor, Department of Medicine, and Medical Director, Multidisciplinary Obesity Clinic, Oregon Health & Science University, Portland, OR

      Obesity and its comorbid disorders are leading causes of morbidity and premature mortality around the world. Obese persons are also vulnerable to low self-esteem and depression because of the psychological and social stigmata that can be associated with obesity. Despite societal prejudicial perceptions that obesity develops because of deficient self-control, research has provided insight into the physiology behind unwanted weight gain. This chapter describes the epidemiology and etiology of obesity, associated risk factors, classification of obesity by body mass index and weight distribution, and neuroendocrine control of appetite and energy expenditure. Diagnostic goals are to identify secondary causes of obesity and identify comorbid conditions. Treatment typically begins with caloric restriction and alterations of the fat, carbohydrate, and protein content in the diet, along with exercise. Pharmacologic treatment with phentermine, sibutramine, orlistat, bupropion, and topiramate are discussed, as is bariatric surgery, complications of obesity, and its prognosis. Figures illustrate the age-adjusted prevalence of overweight and obese adults in the United States, according to age, sex, and race/ethnicity; offer a feedback model for body-weight regulation; and include the evaluation, laboratory testing, and treatment of overweight and obese patients. Tables describe classification of weight and risk of comorbid conditions; list criteria for metabolic syndrome; list medications commonly associated with weight gain and obesity, with possible alternative agents; describe pharmacologic agents approved by the Food and Drug Administration for treatment of obesity; and present the most commonly used bariatric procedures. This chapter contains 177 references.

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    • 17

      Diagnosis and Treatment of Dyslipidemia

      By John D. Brunzell, MD, FACP; Alan Failor, MD
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      Diagnosis and Treatment of Dyslipidemia

      • JOHN D. BRUNZELL, MD, FACPProfessor Emeritus, Active, Department of Medicine, Division of Metabolism, Endocrinology and Nutrition, University of Washington, Seattle, WA 98195
      • ALAN FAILOR, MDClinical Professor, Department of Medicine, Division of Metabolism, Endocrinology and Nutrition, University of Washington, Seattle, WA 98195

      Disorders of lipoprotein metabolism in conjunction with the prevalence of high-fat diets, obesity, and physical inactivity have resulted in an epidemic of atherosclerotic disease in the United States and other developed countries. The interaction of genetic disorders with these adverse environmental factors leads to the premature development of atherosclerosis. This chapter discusses lipoprotein composition and metabolism and the genetic and environmental defects that can lead to dyslipidemias. Primary disorders of dyslipidemia discussed include the metabolic syndrome, familial combined hyperlipidemia, familial hypertriglyceridemia, familial hypercholesterolemia (both heterozygous and homozygous), familial defective apolipoprotein B-100, a disorder causing increased levels of lipoprotein(a), and remnant removal disease. Secondary disorders discussed include those caused by endocrine disorders, renal disorders, and gastrointestinal disorders. The clinical manifestations of hyperlipidemia and how they correspond to specific lipid disorders are presented, including abnormal levels of lipoproteins, cutaneous manifestations (e.g., xanthomas), signs and symptoms that constitute the chylomicronemia syndrome, and nonalcoholic fatty liver disease. Management principles, including diet and exercise modification and drug therapy, are discussed for patients with elevated cholesterol levels, elevated triglyceride levels, low levels of high-density lipoprotein cholesterol, and atherosclerosis accompanied by normal lipid levels. Primary and secondary prevention of coronary artery disease using statins, bile-acid sequestrants, fibrates, and niacin are covered. Management recommendations for patients of varied age groups are reviewed, including screening for hypercholesterolemia in children, the management of dyslipidemia in women, and the management of dyslipidemia in older patients. The eight tables present the major apolipoproteins and their functions, the Adult Treatment Panel III (ATP-III) classifications of the various lipid levels, the effects of selected drugs on lipoprotein levels, the clinical features of the metabolic syndrome, the ATP-III low-density lipoprotein (LDL) cholesterol goals and cutpoints for therapeutic lifestyle changes and drug therapy in different risk categories, ATP-III major risk factors that modify LDL cholesterol goals, drug treatment for lipid disorders, and doses of statins required to achieve a 30% to 40% reduction of LDL cholesterol levels. The six figures illustrate aspects of lipid metabolism. This chapter contains 108 references.

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    • 18

      Metabolic Disorders: Inborn Errors of Carbohydrate Metabolism

      By Sameer S. Chopra, MD, PhD; Gerald T. Berry, MD
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      Metabolic Disorders: Inborn Errors of Carbohydrate Metabolism

      • SAMEER S. CHOPRA, MD, PHDClinical Fellow in Medicine, Dana-Farber Cancer Institute, Boston, MA
      • GERALD T. BERRY, MDDirector, Metabolism Program, Division of Genetics and Genomics, Boston's Children's Hospital, Boston, MA

      The small-molecule diseases include inborn errors of carbohydrate metabolism, ammonia, amino acid, organic acid, and fatty acid metabolism. Many of these entities are now routinely detected through newborn screening in most states in the United States. Although several of these diseases have effective therapies that largely eliminate the signs and symptoms, in many, the disease process is without an effective treatment or may be brought under control but not corrected. Newborn screening and/or the implementation of effective therapy in infancy and childhood are now allowing patients to survive into adulthood. Many variant or hypomorphic gene mutations are now being detected, and the affected patients are not manifesting clinical disease until late adolescence or adulthood. This chapter reviews the essential features of the diagnosis, treatment, and, where appropriate, natural history of inborn errors of carbohydrate metabolism, including the glycogen storage diseases with primary hepatic involvement and fasting hypoglycemia. The inborn errors of amino acids, ammonia, organic acid, and fatty acid metabolism are reviewed. A figure shows the process of galactose metabolism.

      This chapter contains 1 highly rendered figure, 7 references, 9 suggested readings, and 5 MCQs.

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    • 19

      Metabolic Disorders: Inborn Errors of Amino Acid, Ammonia, Organic Acid, and Fatty Acid Metabolism

      By Sameer S. Chopra, MD, PhD; Gerard T. Berry, MD
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      Metabolic Disorders: Inborn Errors of Amino Acid, Ammonia, Organic Acid, and Fatty Acid Metabolism

      • SAMEER S. CHOPRA, MD, PHDClinical Fellow in Medicine, Dana-Farber Cancer Institute, Boston, MA
      • GERARD T. BERRY, MDDirector, Metabolism Program, Division of Genetics and Genomics, Boston's Children's Hospital, Boston, MA

      The small molecule diseases include inborn errors of carbohydrate, ammonia, amino acid, organic acid, and fatty acid metabolism. They are central among the biochemical genetic disorders that may present with life-threatening illnesses during infancy and childhood. Many of these disorders are now detected through routine newborn screening. Internists should be familiar with small molecule metabolic disorders as early diagnosis and therapy may enable many patients to survive into adulthood. Additionally, because some patients may not manifest symptoms until late adolescence or adulthood, recognition of the possibility of an inborn error of metabolism in a patient with unusual signs or symptoms may lead to referral to a metabolic specialist and timely diagnosis and treatment. This module reviews the diagnosis, treatment, and natural history of disorders of amino acid, ammonia, organic acid, and fatty acid metabolism. Figures show the methionine-homocysteine-cysteine pathway and branched-chain amino acid metabolism.

      This module contains 2 highly rendered figures, 18 references, 53 recommended readings, and 5 MCQs.

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    • 20

      The Porphyrias

      By Karl E. Anderson, MD, FACP; Attallah Kappas, MD
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      The Porphyrias

      • KARL E. ANDERSON, MD, FACPProfessor, Departments of Preventive Medicine and Community Health, Internal Medicine, and Pharmacology and Toxicology, University of Texas Medical Branch, Galveston, TX
      • ATTALLAH KAPPAS, MDSherman Fairchild Professor, Physician-in-Chief, Emeritus, The Rockefeller University, Attending Physician, Rockefeller University Hospital, New York, NY

      The porphyrias are uncommon disorders caused by deficiencies in the activities of the enzymes of the heme biosynthetic pathway. The enzymatic defects that cause porphyrias may be either inherited or acquired, and there is significant interplay between the genetic defect and acquired or environmental factors. Acute forms of the porphyrias may be life threatening and may be misdiagnosed because of the nonspecific nature of the clinical presentation (e.g., acute abdominal pain, psychiatric disturbances, and polyneuropathies). The course of the acute forms of disease is characterized by long latent periods interrupted by acute attacks, which are associated with substantial morbidity and mortality. Porphyrias may be classified as neurovisceral or photosensitive, depending on their prominent clinical characteristic, but in some cases of porphyria, both symptoms are present. Alternatively, the porphyrias can be classified as hepatic or erythropoietic, depending on the principal site of expression of the specific enzymatic defect involved, but the expressions overlap in some porphyrias. The neurovisceral porphyrias correspond with the hepatic porphyrias (ie, acute intermittent porphyria [AIP], variegate porphyria [VP], hereditary coproporphyria [HCP], and ALA deficiency porphyria [ADP]); and the photosensitive porphyrias correspond with the erythropoietic porphyrias (i.e., porphyria cutanea tarda [PCT], hepatoerythropoietic porphyria [HEP], erythropoietic protoporphyria [EPP], and congenital erythropoietic porphyria [CEP]). This chapter covers the classification and pathophysiology of porphyrias and the epidemiology, molecular defects and pathophysiology, diagnosis, and treatment of each of the clinical presentations of porphyria. A table lists the drugs that are safe and unsafe for patients with AIP, VP, HCP, and ADP. Figures illustrate the classification and major symptoms of the porphyrias and the corresponding defect in the biosynthesis of heme that causes the disease; the steps in the biosynthesis of heme; and the mechanisms that precipitate symptoms in AIP. This chapter contains 99 references.

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  • Ethics & Professionalism
    • 1

      Management of Psychosocial Issues in Terminal Illness

      By Jane DeLima Thomas, MD; Eva Reitschuler-Cross, MD; Susan D. Block, MD
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      Management of Psychosocial Issues in Terminal Illness

      • JANE DELIMA THOMAS, MDAttending Physician, Department of Psychosocial Oncology and Palliative Care, Dana-Farber Cancer Institute and Brigham and Women’s Hospital, Instructor in Medicine, Harvard Medical School, Boston, MA
      • EVA REITSCHULER-CROSS, MDClinical Assistant Professor of Medicine, University of Pittsburgh, Section of Palliative Care and Medical Ethics, University of Pittsburgh Medical Center, Pittsburgh, PA
      • SUSAN D. BLOCK, MDChair, Department of Psychosocial Oncology and Palliative Care, Dana-Farber Cancer Institute and Brigham and Women’s Hospital Co-Director, HMS Center for Palliative Care, Professor of Psychiatry and Medicine, Harvard Medical School, Boston, MA

      Patients facing serious or life-threatening illness experience challenges to their psychological, social, and spiritual lives as well as to their physical function and comfort. Physicians may be accustomed to focusing on the biomedical aspects of illness, but they have a critical role in assessing the patient's psychosocial issues to identify sources of distress and help implement a plan for mitigating them. An appropriate psychosocial assessment requires a methodical and rigorous approach and includes assessment of any psychosocial issue affected by or affecting a patient's experience of illness. This chapter outlines a structured approach to addressing psychosocial issues by discussing (1) the doctor-patient relationship; (2) coping with illness; (3) family dynamics and caregiving; (4) ethnic and cultural issues; (5) religious, spiritual, and existential issues; (6) mental health issues, including adjustment disorder, depression, anxiety, personality disorders, aberrant drug behaviors, and major mental health issues; and (7) grief and bereavement. Tables outline psychosocial assessment questions, factors predisposing patients with serious illness to depression, risk factors for suicide in patients with terminal illness, and classes of antidepressants, anxiolytics, and sedatives. The Alcohol Use Disorders Identification Test-Consumption (AUDIT-C) questionnaire is provided, as well as a list of Web sites with further resources about psychosocial issues in serious illness.

      This review contains 1 highly rendered figure, 6 tables, and 216 references.

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    • 2

      Brain Death and Organ Donation

      By Thomas I. Cochrane, MD, MBA
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      Brain Death and Organ Donation

      • THOMAS I. COCHRANE, MD, MBAAssociate Neurologist, Division of Neuromuscular Disease, Department of Neurology, Brigham and Women’s Hospital, Assistant Professor of Neurology, Harvard Medical School, Boston, MA

      Brain death is the state of irreversible loss of the clinical functions of the brain. A patient must meet strict criteria to be declared brain dead. They must have suffered a known and demonstrably irreversible brain injury and must not have a condition that could render neurologic testing unreliable. If the patient meets these criteria, a formal brain death examination can be performed. The three findings in brain death are coma or unresponsiveness, absence of brainstem reflexes, and apnea. Brain death is closely tied to organ donation, because brain-dead patients represent approximately 90% of deceased donors and thus a large majority of donated organs. This review details a definition and overview of brain death, determination of brain death, and controversy over brain death, as well as the types of organ donation (living donation versus deceased donation), donation after brain death, and donation after cardiac death. A figure presents a comparison of organ donation after brain death and after cardiac death, and a table lists the American Academy of Neurology Criteria for Determination of Brain Death.

      This review contains 1 highly rendered figure, 1 table, and 18 references.

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    • 3

      Advance Care Planning

      By Lauren Jodi Van Scoy, MD; Michael Green, MD, MS; Benjamin Levi, MD, PhD
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      Advance Care Planning

      • LAUREN JODI VAN SCOY, MDAssistant Professor, Departments of Medicine and Humanities, Penn State College of Medicine, Hershey, PA
      • MICHAEL GREEN, MD, MSProfessor, Departments of Humanities and Medicine, Penn State College of Medicine, Hershey, PA
      • BENJAMIN LEVI, MD, PHDProfessor, Departments of Humanities and Pediatrics, Penn State College of Medicine, Hershey, PA

      Advance care planning (ACP) is defined by the Institute of Medicine as an iterative process that involves discussing end-of-life issues, clarifying relevant values and goals of care, and embodying preferences through written documents and medical orders. ACP is predicated on the principle of respect for autonomy, which recognizes an individual’s right to accept or decline medical therapies. With the development of medical technologies that can sustain life (including mere physiologic existence), effective ACP has become a critical yet underused process for patients, their families, and clinicians. This review discusses the emergence of ACP, promises and pitfalls of advance directives, and promising approaches, including ACP interventions and research, as well as a focus on public engagement and future directions. Figures show a timeline of important advances in ACP since 1990, key features of the comprehensive ACP process, the three core aspects or pillars for implementation of ACP, stages of change for ACP behaviors, and two commercially available end-of-life games. Tables list theoretical pros and cons of advance directives, ACP resources, examples of recent research studies on ACP interventions, types and examples of ACP resources, and public engagement campaigns.

       

      This review contains 5 highly rendered figures, 5 tables, and 100 references.

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    • 4

      Clinical Trial Design and Statistics

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      Clinical Trial Design and Statistics

      A clinical trial is a planned experiment designed to prospectively measure the efficacy or effectiveness of an intervention by comparing outcomes in a group of subjects treated with the test intervention with those observed in one or more comparable group(s) of subjects receiving another intervention.  Historically, the gold standard for a clinical trial has been a prospective, randomized, double-blind study, but it is sometimes impractical or unethical to conduct such in clinical medicine and surgery. Conventional outcomes have traditionally been clinical end points; with the rise of new technologies, however, they are increasingly being supplemented and/or replaced by surrogate end points, such as serum biomarkers. Because patients are involved, safety considerations and ethical principles must be incorporated into all phases of clinical trial design, conduct, data analysis, and presentation. This review covers the history of clinical trials, clinical trial phases, ethical issues, implementing the study, basic biostatistics for data analysis, and other resources. Figures show drug development and clinical trial process, and type I and II error. Tables list Food and Drug Administration new drug application types, and types of missing data in clinical trials.

      This review contains 2 highly rendered figures, 2 tables, and 38 references

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  • Gastroenterology
    • 1

      Esophageal Disorders

      By Michael F. Vaezi, MD, PhD, MSc (EPI)
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      Esophageal Disorders

      • MICHAEL F. VAEZI, MD, PHD, MSC (EPI)Professor of Medicine, Clinical Director of Division of Gastroenterology, Hepatology and Nutrition, Vanderbilt University Medical Center, Nashville, TN

      Typically, symptoms that may indicate the presence of an esophageal disorder include heartburn, dysphagia, odynophagia, and regurgitation. Endoscopy is the technique of choice to evaluate the mucosa of the esophagus and to detect structural abnormalities, whereas esophageal manometry is the standard test to diagnose motor disorders of the esophageal body and the lower esophageal sphincter. This review examines normal esophageal anatomy and physiology, the diagnosis of esophageal disorders, disease states causing dysphagia, and gastroesophageal reflux disease. Figures show the cross-sectional anatomy of the esophagus; an algorithm for the evaluation of dysphagia; the anatomy of the gastroesophageal junction; esophagograms of patients with achalasia, late-stage achalasia, and diffuse esophageal spasm; endoscopic views of esophageal strictures; a proximal esophageal web on barium swallow in a patient with Plummer-Vinson syndrome; an endoscopic view of the esophagus of a 25-year-old man with a 3-year history of severe dysphagia; photographs of midesophageal traction diverticulum, multiple epiphrenic diverticula, long-segment Barrett esophagus, and severe Candida esophagitis; and a treatment algorithm for extraesophageal manifestations of gastroesophageal reflux disease. Tables list the high-resolution manometry classification of esophageal motility disorders, causes of esophageal strictures, classic endoscopic findings in patients with eosinophilic esophagitis, the Los Angeles classification of erosive esophagitis, categories of dysplasia, surveillance of Barrett metaplasia, medications implicated in pill-induced esophagitis, and classification of caustic esophageal injury.

      This review contains 13 highly rendered figures, 8 tables, and 111 references.

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    • 2

      Gastrointestinal Bleeding

      By Deepak Agrawal, MD; Don C. Rockey, MD
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      Gastrointestinal Bleeding

      • DEEPAK AGRAWAL, MDDirector, Endoscopy, Parkland Memorial Hospital, Assistant Professor of Medicine, University of Texas Southwestern, Dallas, TX
      • DON C. ROCKEY, MDChief, Division of Digestive and Liver Diseases, Professor of Medicine, University of Texas Southwestern, Dallas, TX

      Gastrointestinal bleeding (GIB) is commonly encountered in clinical practice, in both outpatient and inpatient settings. Minor bleeding, such as from hemorrhoids, is exceedingly common. Major bleeding requiring a high level of care results in approximately 1 million hospital admissions in the United States every year. Approximately 50% of these admissions are for upper gastrointestinal bleeding (UGIB), 40% for lower gastrointestinal bleeding (LGIB), and 10% for obscure GIB. This chapter discusses acute gastrointestinal bleeding covering history, physical examination, and laboratory studies, as well as endoscopic management and radiologic imaging. A section on UGIB covers endoscopic management, medical management, and radiologic and surgical management, variceal bleeding, Mallory-Weiss tear, Dieulafoy’s lesions, Cameron’s lesions, gastric antral vascular ectasia (GAVE), and portal hypertensive gastropathy. A section on LGIB covers diverticulosis, hemorrhoids, angiodysplasia, rectal ulcers, radiation proctitis, ischemic colitis, rectal varices, postpolypectomy bleeding, anal fissures, cancer, and polyps. Obscure GIB includes Meckel’s diverticulum, small intestinal diverticula, small intestine neoplasms, small intestine Dieulafoy’s lesion, non-steroidal antiinflammatory drug–induced small intestine erosions, and blue rubber bleb nevus syndrome. Figures include various bleeding ulcers, the wireless capsule, the bleeding vessel, a duodenal bulbar ulcer, treatment of a high-risk gastric ulcer, formation of varices, varices with a spurting lesion, various treatments for esophageal varices, a Mallory-Weiss tear, a Dieulafoy lesion in the rectum, severe portal hypertensive gastropathy, vascular ectasia, and ischemic colitis, plus an algorithm for obscure GIB. Tables cover major causes, terms and their definitions, clinical high-risk criteria for rebleeding and mortality, endoscopic high-risk stigmata for rebleeding and indications for endoscopic therapy, differentiation of portal hypertensive gastropathy and GAVE, and uncommon causes of obscure GIB. This chapter contains 21 highly rendered figures, 6 tables, 110 references, 5 MCQs.

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    • 3

      Gastrointestinal Motility and Functional Disorders

      By Adil E. Bharucha, MBBS, MD
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      Gastrointestinal Motility and Functional Disorders

      • ADIL E. BHARUCHA, MBBS, MDProfessor of Medicine, Director, Motility Interest Group, Mayo Clinic, Rochester, MN

      Gastrointestinal (GI) motility disorders represent diseases characterized by abnormal, predominantly impaired, sometimes exaggerated, movement of contents through the GI tract due to neuromuscular dysfunctions in the absence of mucosal disease and mechanical causes of impaired passage. By contrast, functional GI disorders represent illnesses, defined only by GI symptoms, which occur in the absence of mucosal or structural abnormality or of known biochemical or metabolic disorders. The first section of this chapter discusses the enteric and extrinsic neural regulation of GI sensorimotor functions and normal GI motility in humans. Disorders such as gastroparesis (including diabetic gastroparesis, idiopathic gastroparesis, and postsurgical gastroparesis), dumping syndrome, intestinal pseudo-obstruction, small intestinal bacterial overgrowth, megacolon (including Hirschsprung disease, toxic megacolon, and colonic pseudo-obstruction), chronic constipation (including defecatory disorders, normal transit constipation, and slow transit constipation), functional dyspepsia, functional diarrhea and irritable bowel syndrome, and fecal incontinence are then discussed in depth. Tables present a comparison of GI motility and functional disorders, the causes of gastroparesis, the etiology of intestinal pseudo-obstruction and fecal incontinence, common medical conditions and medications associated with constipation, and the symptom severity scale in fecal incontinence. Illustrations, graphs, magnetic resonance images, and algorithms are provided.
      This chapter contains 10 highly rendered figures, 6 tables, 92 references, and 5 MCQs.

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    • 4

      Diseases Producing Malabsorption and Maldigestion

      By Rupa Mukherjee, MD; Ciarán P. Kelly, MD
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      Diseases Producing Malabsorption and Maldigestion

      • RUPA MUKHERJEE, MDClinical Instructor in Medicine, Department of Gastroenterology, The Celiac Center, Harvard Medical School, Boston, MA
      • CIARÁN P. KELLY, MDProfessor of Medicine, Medical Director of The Celiac Center, Director of Gastroenterology Fellowship Training Program, Harvard Medical School, Boston, MA

      Malabsorption refers to the impaired intestinal absorption of nutrients. It can result from congenital defects in absorption and the transport of ions and nutrients, defects in hydrolysis within the intestinal lumen, acquired defects in the intestinal absorptive cells that line the surface of the intestine, impaired bile production, or interruption of enterohepatic circulation or secondary to pancreatic insufficiency. Maldigestion, another factor in nutrient absorption, refers to the impaired digestion of nutrients within the intestinal lumen or at the terminal digestive site of the brush border membrane of mucosal epithelial cells. Although malabsorption and maldigestion are pathophysiologically distinct, they are interdependent, and in clinical practice, malabsorption has come to signify derangements in either or both processes. This chapter discusses the clinical manifestations of malabsorption and tests for suspected malabsorption. The diseases that can cause malabsorption, their diagnosis, and treatment recommendations are included. Figures illustrate the diagnosis and management of celiac disease; an approach to gluten challenge for the diagnosis or exclusion of celiac disease in patients maintained on a gluten-free diet without previous definitive diagnostic testing; and the histologic features of celiac disease, Crohn disease, collagenous sprue, autoimmune enteropathy, eosinophilic gastritis, and intestinal lymphangiectasia. Tables list causes of malabsorptive syndromes and tests of digestive-absorptive function.
      This chapter contains 9 highly rendered figures, 2 tables, 139 references, 1 teaching slide set, and 5 MCQs.

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    • 5

      Peptic Ulcer Diseases

      By Edward A. Lew, MD, MPH
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      Peptic Ulcer Diseases

      • EDWARD A. LEW, MD, MPHStaff Gastroenterologist, VA Boston Healthcare System, Brigham and Women’s Hospital, Assistant Professor of Medicine, Harvard Medical School, Boston, MA

      Peptic ulcers are defects or breaks in the inner lining of the gastrointestinal (GI) tract. Although the pathogenesis is multifactorial they tend to arise when there is an imbalance between protective and aggressive factors, such as when GI mucosal defense mechanisms are impaired in the presence of gastric acid and pepsin. Peptic ulcers extend through the mucosa and the muscularis mucosae, a thin layer of smooth muscle separating the mucosa from the deeper submucosa, muscularis propria, and serosa. Peptic ulcer disease affects up to 10% of men and 4% of women in Western countries at some time in their lives. This chapter discusses the pathogenesis of peptic ulcer disease and the etiologic contribution of Helicobacter pylori infection, nonsteroidal anti-inflammatory drugs, and gastrinoma or other hypersecretory states. Also addressed are rare and unusual causes for ulcers and GI bleeding. A section on the diagnosis of peptic ulcers discusses clinical manifestations, physical examination findings, laboratory and imaging studies, and surgical diagnosis. Differential diagnosis is also reviewed. Tests to establish the etiology of peptic ulcer disease include endoscopy, quantitative serologic tests, the urea breath test, and the fecal antigen test. Discussed separately are treatments for uncomplicated duodenal ulcers, uncomplicated gastric ulcers, intractable duodenal or gastric ulcers, complicated peptic ulcers (bleeding ulcers, acute stress ulcers, perforated ulcers, obstructing ulcers, fistulizing ulcers, and Cameron ulcers), H. pylori ulcers, and gastric cancer. Figures illustrate the etiopathogenesis of peptic ulcers, prevalence of H. pylori infection in duodenal and gastric ulcer patients compared with normal controls, the approach to a patient with new and undiagnosed ulcerlike symptoms refractory to antisecretory therapy, an upper GI series showing an uncomplicated duodenal ulcer, a chest x-ray showing pneumoperitoneum from a perforated duodenal ulcer, gastric biopsy samples showing H. pylori organisms, and the approach to treatment and follow-up in patients with either complicated or uncomplicated duodenal or gastric ulcer. Tables list differential diagnoses of peptic ulcer disease, commonly used regimens to eradicate H. pylori, additional antimicrobial agents with activity against H. pylori, and FDA-approved antisecretory drugs for active peptic ulcer disease. This chapter contains 76 references.

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    • 6

      Gastrointestinal Tract Infections

      By Luisa M. Stamm, MD, PhD; Marcia B. Goldberg, MD
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      Gastrointestinal Tract Infections

      • LUISA M. STAMM, MD, PHDResearch Fellow, Division of Infectious Diseases, Harvard Medical School, and Clinical Fellow, Massachusetts General Hospital, Boston, MA
      • MARCIA B. GOLDBERG, MDAssociate Professor of Medicine, Division of Infectious Diseases, Harvard Medical School, and Physician, Massachusetts General Hospital, Boston, MA

      The most notable symptoms associated with gastrointestinal infections are vomiting and diarrhea. In the United States, acute diarrhea (more than three loose stools in a day for less than 2 weeks) is a common medical complaint. Worldwide, it is estimated that 2 billion cases of diarrheal disease occur each year. It is the second leading cause of death for children. This chapter details the general approach to diarrheal illnesses and looks specifically at Salmonella, bacteremia and vascular infections, gastroenteritis, enteric fever, the carrier state (Salmonella organisms in the stool for more than 1 year after initial infection), Campylobacter and Shigella infections, Escherichia coli gastrointestinal infections, Yersinia and Vibrio cholerae infections, infections caused by Vibrio other than toxigenic V. cholerae, Clostridium difficile, enterotoxin-mediated causes of diarrhea, norovirus infections, gastrointestinal infections due to viruses other than norovirus, and parasitic gastrointestinal infections. Figures show the clinical presentation of gastrointestinal tract disease, the evaluation of acute diarrhea, the growth of enteric pathogens on selective agar, the pathology of Y. pseudotuberculosis infection, and imaging in C. difficile infection. Tables cover gastrointestinal infections, pathogenesis correlates with signs of disease, infectious innocula of selected gastrointestinal pathogens, selected therapies for enteric pathogens, the percentage of Salmonella and Shigella isolates resistant to selected antibiotics in the United States, clinical and laboratory findings in untreated enteric fever, vaccinations for enteric fever, clinical categories and recommendations for the treatment of C. difficile infection, the Kaplan criteria for establishing norovirus as the cause of an outbreak of gastroenteritis, and recommendations for the prevention and control of norovirus outbreaks. This chapters contains 88 references.

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    • 7

      Diverticulosis, Diverticulitis, and Appendicitis

      By William V. Harford, MD, FACP
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      Diverticulosis, Diverticulitis, and Appendicitis

      • WILLIAM V. HARFORD, MD, FACPProfessor, Internal Medicine, University of Texas Southwestern Medical Center, Director, GI Endoscopy, VA Medical Center, Dallas, TX

      Colonic diverticula are herniations of colonic mucosa and submucosa through the muscularis propria. They occur where perforating arteries traverse the circular muscle layer, in parallel rows between the mesenteric and antimesenteric taenia. Colonic diverticular disease may present as diverticulosis, diverticulitis, or diverticular bleeding. Of patients with known diverticulosis, only 10% to 20% will develop diverticulitis. Diverticulitis varies in presentation and severity. This chapter discusses the diagnosis, differential diagnosis, and management of diverticulitis and its complications. Appendicitis is generally caused by obstruction of the lumen of the appendix, followed by infection. In the United States, the lifetime risk of appendicitis is about 9% for males and 7% for females. This chapter also discusses the diagnosis of appendicitis (including typical and atypical presentations and appendicitis as it presents in special groups of patients) and its management. Figures illustrate imaging study findings in diverticulitis and appendicitis. Tables describe the modified Hinchey classification of acute diverticulitis, the differential diagnosis of acute diverticulosis, and the differential diagnosis of appendicitis. This chapter contains 92 references.

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    • 8

      Appendicitis

      By James Creswell Simpson, MD; Sarah Sebbag, MD, CM, CCFP(EM)
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      Appendicitis

      • JAMES CRESWELL SIMPSON, MDResident Physician, Department of Anesthesiology, Critical Care and Pain Medicine, Massachusetts General Hospital, Boston, MA
      • SARAH SEBBAG, MD, CM, CCFP(EM)Director, Emergency Ultrasound, Ochsner Health System, Department of Emergency Medicine, New Orleans, LA

      Appendicitis is defined as inflammation of the vermiform appendix. It is the most common abdominal surgical emergency and occurs at an annual rate of approximately one in 10,000 in the United States. The lifetime risk of appendicitis is about 9% for males and 7% for females; approximately 80% of cases occur before 45 years of age. Appendicitis rarely occurs in infants; it increases in frequency between 2 and 4 years of age and reaches a peak between the ages of 10 and 19 years. However, clinicians must maintain a high index of suspicion in patients of all age groups. This review covers the pathophysiology, stabilization and assessment, and diagnosis and treatment of complicated and uncomplicated appendicitis. The disposition and outcomes are also reviewed. Figures show an image of appendicitis on a bedside sonogram, and a computed tomographic image of appendicitis. Tables list likelihood ratios of signs and symptoms of appendicitis, the sonographic appearance of appendicitis, the Alvarado scoring system, and the differential diagnosis of appendicitis.

      Key words: appendicitis, obstructed appendiceal lumen, rebound abdomen, right lower quadrant pain, ruptured appendix

      This review contains 2 highly rendered figures, 4 tables, and 33 references.

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    • 9

      Peritonitis and Intra-abdominal Abscesses

      By W. Conrad Liles, MD, PhD; E. Patchen Dellinger, MD
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      Peritonitis and Intra-abdominal Abscesses

      • W. CONRAD LILES, MD, PHDProfessor and Vice-Chair of Medicine, Director, Division of Infectious Diseases, University of Toronto, Toronto, ON
      • E. PATCHEN DELLINGER, MDProfessor and Vice-Chair, Department of Surgery, University of Washington, Chief, Division of General Surgery, University of Washington Medical Center, Seattle, WA

      Peritonitis is a diffuse or localized inflammatory process affecting the peritoneal lining. Peritonitis has acute and chronic forms and may have a variety of causes. The etiology, epidemiology, diagnosis, and treatment of acute peritonitis caused by bacteria or fungi, including primary and secondary peritonitis, are discussed in this chapter. Primary or spontaneous peritonitis has no underlying intra-abdominal disorder as a direct cause of the infection but usually involves an underlying disorder that inhibits normal host defenses in the peritoneal cavity. Secondary peritonitis has an intra-abdominal focus that initiates the infection. Tertiary peritonitis is a relatively new term that refers to the persistence of intra-abdominal infection after the initial treatment of secondary peritonitis. Peritonitis in dialysis patients is also discussed. Intra-abdominal abscesses may present as complications of abdominal surgery, intra-abdominal conditions (e.g., diverticulitis, appendicitis, biliary tract disease, pancreatitis, perforated viscus), or penetrating abdominal trauma; as fever of obscure origin; or as dysfunction of neighboring organs (e.g., so-called lower lobe pneumonia related to a subphrenic abscess). Intra-abdominal abscesses are classified according to the anatomic location in which they occur: intraperitoneal, retroperitoneal, or visceral. Discussion of the different intra-abdominal abscesses in this chapter includes their diagnosis, bacteriology, and treatment. A figure shows CT scans before and after treatment of a multilobular liver abscess. This chapter contains 101 references.

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    • 10

      Enteral and Parenteral Nutrition

      By Kris M. Mogensen, MS, RD, LDN, CNSC; Malcolm K. Robinson, MD
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      Enteral and Parenteral Nutrition

      • KRIS M. MOGENSEN, MS, RD, LDN, CNSCTeam Leader Dietitian, Department of Nutrition, Brigham and Women’s Hospital, Boston, MA
      • MALCOLM K. ROBINSON, MDDirector, Nutrition Support Service, Brigham and Women’s Hospital, Assistant Professor of Surgery, Department of Surgery, Harvard Medical School, Boston, MA

      Alternative routes of nutrient administration are available for patients who are unable to eat or digest sufficient food to prevent malnutrition. These routes include enteral (administered through the gastrointestinal tract) and parenteral (administered intravenously). This review details the clinical consequences of malnutrition, nutritional assessment, the benefits of nutrition support therapy,  determining the nutrient prescription, special considerations in nutrition support therapy, aspects of obtaining enteral or parenteral access, monitoring of patients receiving nutrition support therapy, and complications and ethical issues associated with enteral and parenteral nutrition. Figures include algorithms showing the identification of malnutrition, the nutrition support decision process, and the approach to gastric residual monitoring; nasogastric tube displacement leading to pneumothorax; proper placement of a long or “midline” catheter versus a peripherally inserted central catheter; and photographs of a 43-year-old man with Crohn disease complicated by enterocutaneous fistula formation, distal small bowel obstruction, and evisceration of the small bowel after developing a pelvic abscess. Tables list acute illness- or injury-related malnutrition; chronic disease−related malnutrition; social or environmental circumstances−related malnutrition; indications and contraindications to enteral and parenteral nutrition; selected examples of predictive equations; electrolyte provision in parenteral nutrition; parenteral vitamin and trace element requirements; complications associated with enteral and parenteral nutrition; and indications, contraindications, and complications of gastrostomy tube placement.

      This review contains 6 highly rendered figures, 10 tables, and 167 references.

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    • 11

      Diseases of the Pancreas

      By Sunil Sheth, MD; Gyanprakash Ketwaroo, MD, MSc; Steven Freedman, MD, PhD
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      Diseases of the Pancreas

      • SUNIL SHETH, MDCo-Director, The Pancreas Center, Division of Gastroenterology, Beth Israel Deaconess Medical Center, Boston, MA
      • GYANPRAKASH KETWAROO, MD, MSCClinical Fellow, The Pancreas Center, Division of Gastroenterology, Beth Israel Deaconess Medical Center, Boston, MA
      • STEVEN FREEDMAN, MD, PHDDirector, The Pancreas Center, Division of Gastroenterology, Beth Israel Deaconess Medical Center, Boston, MA

      Acute pancreatitis is characterized by an acute inflamatory process of the pancreas associated with abdominal pain and elevations in serum levels of pancreatic enzymes. The acute inflammation usually completely resolves with restoration of normal pancreatic architecture and function. By contrast, chronic pancreatitis is characterized by the presence of ongoing inflammation and irreversible damage to the gland. Recurrent attacks of acute pancreatitis may lead to chronic pancreatitis over time. This chapter discusses the epidemiology, etiology, pathogenesis, diagnosis, and treatment of acute and chronic pancreatitis. Figures illustrate findings on imaging studies in patients with pancreatic disorders. Tables list causes and complications of acute pancreatitis, nonpancreatic causes of elevated amylase and lipase levels, and diagnostic tests for chronic pancreatitis.

      This chapter contains 6 figures, 4 tables, 115 references, and 5 Board-styled MCQs.

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    • 12

      Gallstones and Biliary Tract Disease

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      Gallstones and Biliary Tract Disease

      Gallstones (cholelithiasis) are hardened deposits of digestive fluid that can form in the gallbladder and bile ducts. Gallstones range in size from as small as a grain of sand to as large as a billiard ball. Some people develop a single gallstone, whereas others develop many. Gallstone disease or disorders may encompass biliary pain, acute and chronic cholecystitis, choledocholithiasis, and gallstone pancreatitis. Gallstones (cholelithiasis) and biliary tract diseases constitute a common and costly health problem in the United States. This chapter reviews the formation of the two principal types of stone, the cholesterol stone and the pigment stone, that form in the gallbladder and biliary tract. The prevention of gallstones is discussed. The diagnosis, differential diagnosis, treatment, and complications of acute cholecystitis are presented. Diagnosis and treatment of chronic cholecystitis, asymptomatic cholelithiasis, choledocholithiasis, Mirizzi syndrome, and gallbladder polyps are also discussed. Common bile duct stricture, primary sclerosing cholangitis, recurrent pyogenic cholangitis, choledochal cyst, and sphincter of Oddi dysfunction are summarized. Figures illustrate the mechanism of gallstone formation and imaging study results in a variety of biliary tract disorders. Tables list risk factors for cholesterol and pigment gallstone formation, a modified classification system for choledochal cysts and surgical procedure of choice, and a clinical classification system for biliary-specific abdominal pain associated with sphincter of Oddi dysfunction.
      This chapter contains 6 highly rendered figures, 3 tables, 122 references, 1 teaching slide set, and 5 MCQs.

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    • 13

      Pancreatic, Gastric, and Other Gastrointestinal Cancers

      By Vinay Chandrasekhara, MD; Gregory G. Ginsberg, MD
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      Pancreatic, Gastric, and Other Gastrointestinal Cancers

      • VINAY CHANDRASEKHARA, MDInstructor of Medicine, Gastroenterology Division, Penn Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA
      • GREGORY G. GINSBERG, MDProfessor of Medicine, Gastroenterology Division, Director of Endoscopic Services, Penn Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA

      According to 2009 estimates from the American Cancer Society, cancers originating in the gastrointestinal tract rank second in both incidence and cancer-related deaths. One in four deaths in the United States is caused by cancer, with 25% of cancer-related deaths caused by gastrointestinal (GI) malignancies; more than 50% of these deaths are caused by cancer of the pancreas, stomach, esophagus, liver, or biliary tract. Recent advances in molecular biology, medical genetics, and imaging and endoscopic techniques, as well as the development of antitumor agents, have significantly altered the approaches to the prevention, diagnosis, and treatment of GI cancers. The chapter covers esophageal, gastric, pancreatic, hepatocellular, biliary tract, and anal cancers, as well as GI stromal tumors and gastric lymphoma. Coverage of all cancers includes diagnosis and treatment; various sections include information on epidemiology, etiology, risk factors, screening and prevention, molecular mutations, pathogenesis, and/or metastatic disease. Figures depict a barium esophagogram showing squamous cell carcinoma; imaging of esophageal cancer, gastric cancer, and pancreatic cancer; a pedigree of a family with inactivation of germline mutation of E-cadherin; hereditary gastric cancer; gastric cancer survival rates after gastrectomy; axial T1-weighted magnetic resonance imaging (MRI) showing cancer of the pancreatic head; and T1- and T2-weighted MRIs of intrahepatic bile duct carcinoma. Tables provide information on new cases and mortality from GI cancer in 2009; guidelines for diagnosis and surveillance of Barrett esophagus; the declining incidence of gastric cancer in Japan, Slovenia, and the United States; TNM staging of gastric cancer, pancreatic cancer, and hepatocellular carcinoma; the incidence of familial pancreatic carcinoma; molecular mutations involved in pancreatic cancer; staging of pancreatic intraepithelial neoplasia; and the Chinese University Prognostic Index. This chapter contains 235 references.

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    • 14

      Pathophysiology and Diagnosis of Ulcerative Colitis and Crohn Disease

      By Davendra Sohal, MD, MPH; Weijing Sun, MD; Daniel Haller, MD, FACP
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      Pathophysiology and Diagnosis of Ulcerative Colitis and Crohn Disease

      • DAVENDRA SOHAL, MD, MPHFellow, Division of Hematology-Oncology, Department of Medicine, University of Pennsylvania, Abramson Cancer Center, Philadelphia, PA
      • WEIJING SUN, MDAssociate Professor of Medicine, Division of Hematology-Oncology, Department of Medicine, Abramson Cancer Center, University of Pennsylvania, Philadelphia, PA
      • DANIEL HALLER, MD, FACPProfessor of Medicine, Division of Hematology-Oncology, Department of Medicine, Abramson Cancer Center, University of Pennsylvania, Philadelphia, PA

      Inflammatory bowel disease (IBD) encompasses both ulcerative colitis and Crohn disease, and is characterized by recurrent bouts of inflammation of the gastrointestinal tract. IBD affects approximately 4 million people worldwide, and rates are gradually increasing. This review covers the etiology, epidemiology, definition and pathophysiology, extraintestinal manifestations, and other disease-related complications of IBD. Figures show the distribution of ulcerative colitis and Crohn disease by location, several colonoscopic photographs of patients with ulcerative colitis as well as those with Crohn disease, computed tomography images of patients with Crohn disease, small bowel follow-through and fluoroscopic spot images of a patient with chronic structuring Crohn disease, and a computed tomographic scan showing extraenteric manifestations of Crohn disease. Tables list the differential diagnosis of ulcerative colitis, types of infectious colitis, complications of IBD, diagnostic criteria of toxic colitis, physical signs of Crohn disease, differences between Crohn disease and ulcerative colitis, and common extraintestinal manifestations of IBD.

      This review contains 11 highly rendered figures, 7 tables, and 63 references.

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    • 15

      Management of Ulcerative Colitis and Crohn Disease

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      Management of Ulcerative Colitis and Crohn Disease

      There have been considerable advances in the treatment of inflammatory bowel disease (IBD) since the 20th century, and there are multiple options available to the clinician. The management of IBD depends on the location of disease, as well as its clinical, endoscopic and histologic severity. The field is currently undergoing a paradigm shift from a step-up approach (starting treatment with a “milder” medication and, if this treatment fails, moving on to a more powerful medication) to a top-down approach (in which stronger medications are given earlier in the disease course). This review details the medications used to treat IBD, nutrition in IBD patients, and surgical treatment of IBD. Figures show step-up therapy, top-down therapy, strictureplasty technique, gross pathology image of a patient with Crohn disease and previous ileocecal resection, and ileal pouch anal anastomosis surgery. Tables list 5-aminosalicylic acid medications, antibiotics, corticosteroids, immunomodulators, and biologic medications used to treat IBD, as well as causes of nutritional deficiencies in IBD patients.

      This review contains 5 highly rendered figures, 6 tables, and 96 references.

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    • 16

      Constipation in the Emergency Department

      By Julia B. Greer, MD, MPH; Miguel D. Regueiro, MD
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      Constipation in the Emergency Department

      • JULIA B. GREER, MD, MPHAssistant Professor of Medicine, Department of Medicine, Division of Gastroenterology, Hepatology and Nutrition, University of Pittsburgh School of Medicine, Pittsburgh, PA
      • MIGUEL D. REGUEIRO, MDProfessor of Medicine, Department of Medicine, Division of Gastroenterology, Hepatology and Nutrition, University of Pittsburgh School of Medicine, Co-Director, Inflammatory Bowel Disease Center, University of Pittsburgh Medical Center, Pittsburgh, PA

      Constipation can be classified as either primary constipation or secondary constipation. Constipation can be distressing to patients and can lead to serious complications, including bowel obstruction, perforation, volvulus, and proctitis. Emergency physicians should be aware of the evaluation, diagnosis, and management of patients presenting with the chief complaint of constipation. This review covers the risk factors, pathophysiology, assessment, diagnosis and treatment, and disposition and outcomes for patients presenting to the emergency department with constipation. Figures show radiographic and schematic images of several diagnoses which may present with the chief complaint of constipation. Tables list the primary and secondary causes of constipation, the criteria for the diagnosis of irritable bowel syndrome, and the mechanism of action of common medications used to treat constipation.

      This review contains 6 highly rendered figures, 5 tables, and 66 references.

      Key words:  bowel obstruction, constipation, 

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    • 17

      Hernias in the Emergency Department

      By Julia B. Greer, MD, MPH; Miguel D. Regueiro, MD
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      Hernias in the Emergency Department

      • JULIA B. GREER, MD, MPHAssistant Professor of Medicine, Department of Medicine, Division of Gastroenterology, Hepatology and Nutrition, University of Pittsburgh School of Medicine, Pittsburgh, PA
      • MIGUEL D. REGUEIRO, MDProfessor of Medicine, Department of Medicine, Division of Gastroenterology, Hepatology and Nutrition, University of Pittsburgh School of Medicine, Co-Director, Inflammatory Bowel Disease Center, University of Pittsburgh Medical Center, Pittsburgh, PA

      Hernia is defined as an abnormal protrusion of an organ or tissue through a pathologic defect in its surrounding wall. Overall, hernia is common and is generally believed to be a benign condition associated with some morbidity, although it is not thought to be associated with significant mortality. Between 2001 and 2010, 2.3 million inpatient abdominal hernia repairs were performed in the United States, of which 567,000 were performed emergently. In some cases, a hernia can be a deadly condition. In 2002, hernia was listed as the cause of death for 1,595 US citizens. This review covers the pathophysiology, stabilization and assessment, diagnosis and treatment, and disposition and outcomes of hernia. Figures show anatomic locations of the various abdominal wall, groin, lumbar, and pelvic floor hernias; a direct inguinal hernia; an indirect inguinal hernia; point-of-care sonograms showing a ventral wall hernia and an abdominal wall hernia; and the differential diagnosis of an abdominal mass based on anatomic location. Tables list risk factors for the development of inguinal hernia, sex-based differences in inguinal hernia development, risk factors for the development of incisional hernia, factors to consider when assessing the patient for a hernia, and factors associated with the highest rates of incarceration in patients with groin hernia.
       

      Key words: emergent hernia, hernia incarceration, incisional hernia, inguinal hernia, strangulated hernia

      This review contains 6 highly rendered figures, 5 tables, and 66 references.

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  • Geriatric Medicine
    • 1

      Approach to the Geriatric Patient

      By Tia Kostas, MD; Mark Simone, MD; James L. Rudolph, MD, SM
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      Approach to the Geriatric Patient

      • TIA KOSTAS, MDAssistant Professor of Medicine, Section of Geriatrics & Palliative Medicine, Department of Medicine, University of Chicago, Chicago, IL
      • MARK SIMONE, MDInstructor of Medicine, Harvard Medical School, Associate Program Director-Primary Care, Mount auburn Hospital Internal Medicine Residency, Director, Quality Improvement, Division of Geriatric Medicine, Department of Medicine, Mount Auburn Hospital, Cambridge, MA
      • JAMES L. RUDOLPH, MD, SMAssociate Professor of Medicine, Harvard Medical School, Chief (Interim) Geriatrics and Palliative Care, Director, Boston, GRECC, VA Boston Healthcare System, Jamaica Plain, MA, Acting Clinical Chief, Associate Epiclemiologist, Division of Aging, Department of Medicine, Brigham and Women’s Hospital, Boston, MA

      As of 2012, over one in eight Americans is over the age of 65, and this number is rising, particularly in the 85+ age group. This segment of the population has a rate of hospitalization three times higher than that for persons of all ages. General internists and family medicine physicians provide a large portion of care for this age group and should therefore be comfortable using a comprehensive approach to geriatric assessment. This review describes general considerations regarding geriatric care, including the process of taking a functional history and clinical implications of geriatric care. The geriatric assessment process is discussed in terms of physical, cognitive, social, and medical domains. The benefits of geriatric assessment in primary care, specialty care, and hospitalized patients are described. Tables outline activities of daily living, sensory changes with aging, major causes of visual impairment in the geriatric population, major neurocognitive disorder diagnostic criteria, medications to avoid or use with caution based on Beers criteria and Screening Tool of Older individuals’ Potentially inappropriate Prescriptions criteria, U.S. Preventive Services Task Force–recommended services relevant to older adults, and vaccinations in older adults. Figures illustrate the key vulnerabilities of older adults; outcomes linked to functional dependence; common disorders associated with cognitive concerns; domains of cognition and examples of impairment in theDiagnostic and Statistical Manual of Mental Disorders, fifth edition; the social and medical domains of geriatric assessment; barriers to medication adherence in older patients; and resources for medication appropriateness in older adults.

      This review contains 8 highly rendered figures, 8 tables, 110 references, and 5 MCQs.

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    • 2

      Assessment and Management of the Geriatric Patient

      By Tia Kostas, MD; Mark Simone, MD; James L. Rudolph, MD, SM
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      Assessment and Management of the Geriatric Patient

      • TIA KOSTAS, MDAssistant Professor of Medicine, Section of Geriatrics & Palliative Medicine, Department of Medicine, University of Chicago, Chicago, IL
      • MARK SIMONE, MDInstructor of Medicine, Harvard Medical School, Associate Program Director-Primary Care, Mount auburn Hospital Internal Medicine Residency, Director, Quality Improvement, Division of Geriatric Medicine, Department of Medicine, Mount Auburn Hospital, Cambridge, MA
      • JAMES L. RUDOLPH, MD, SMAssociate Professor of Medicine, Harvard Medical School, Chief (Interim) Geriatrics and Palliative Care, Director, Boston, GRECC, VA Boston Healthcare System, Jamaica Plain, MA, Acting Clinical Chief, Associate Epiclemiologist, Division of Aging, Department of Medicine, Brigham and Women’s Hospital, Boston, MA

      Adults age 65 years and older make up an increasing percentage of the patient population. Physicians should recognize predisposing factors in their assessment of the geriatric patient, looking out for conditions such as homeostatic failure, multimorbidity, and functional disability, as well as clinical disorders that do not fit into discrete disease or organ system categories and are often described as geriatric syndrome. Through this and other strategies, such as evaluating patients at risk and implementing evidence-based therapy, physicians can substantially improve the well-being, functional independence, and quality of life of older adults. This review addresses the assessment and management of several geriatric syndromes, detailing cognitive impairment, falls and gait disturbances, malnutrition and weight loss, and pressure ulcers. Figures show types of dementia, nonpharmacologic interventions for cognitive impairment, a checklist for assessment of a delirious patient, nonpharmacologic management of delirium, the Timed Up and Go test, modifiable fall risk factors, and etiologies and management of weight loss. Tables list cognitive screens, cognition enhancers, delirium prevalence, confusion assessment method for diagnosis of delirium, differential features of delirium and dementia, common causes of unintentional weight loss in older adults, appetite enhancers and side effects, extrinsic forces involved in creating pressure ulcers, National Pressure Ulcer Advisory Panel staging system for pressure ulcers, specialty mattresses for pressure relief, and pressure ulcer dressings.

      This review contains 8 highly rendered figures, 11 tables, and 84 references.

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    • 3

      Rehabilitation of Geriatric Patients

      By Stephanie A. Studenski, MD, MPH; Cynthia J. Brown, MD, MSPH; Susan E. Hardy, MD, PhD
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      Rehabilitation of Geriatric Patients

      • STEPHANIE A. STUDENSKI, MD, MPH
      • CYNTHIA J. BROWN, MD, MSPH
      • SUSAN E. HARDY, MD, PHD

      Primary care physicians play an important role in the rehabilitation of geriatric patients: they must be able to assess rehabilitation potential, determine specific patient needs, and then determine the appropriate setting to optimize patient care. Rehabilitation service planning is usually based on a different model of diagnosis and treatment than traditional medical care. This chapter describes the key aspects of rehabilitation planning for stroke, amputation and peripheral vascular disease, hip fracture, rheumatoid arthritis, and arthroplasty. The revised World Health Organization International Classification of Functioning, Disability and Health (ICIDH-2), included in a figure, can be used to assess the causes of disability, plan treatment approaches, and determine the outcomes of care. Tables describe selected results of the neurologic examination of a patient with stroke, assessment and management of complications of stroke and other disabling conditions, and pharmacologic therapy for poststroke depression. An algorithm shows the selection of the appropriate setting for rehabilitation after hospitalization for acute stroke. The Orpington Prognostic Scale for estimating stroke recovery is provided, as is a list of current Internet resources pertaining to geriatric care. This chapter contains 96 references.

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    • 4

      Mistreatment of Elders

      By Emily I. Gorman, MD; Judith Linden, MD
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      Mistreatment of Elders

      • EMILY I. GORMAN, MDDepartment of Emergency Medicine, Boston Medical Center and Boston University School of Medicine, Department of Emergency Medicine, Boston, MA
      • JUDITH LINDEN, MDAssociate Professor and Vice Chair for Education, Department of Emergency Medicine, Boston University School of Medicine, Boston Medical Center, Boston, MA

      Elder mistreatment affects a considerable proportion of individuals older than 60 to 65 years of age and may include intentional abuse (physical, sexual, emotional, or financial) and neglect. As the proportion of the population that is older than 65 years of age increases, elder mistreatment will become an increasingly common issue. Only a minority of cases of elder abuse are reported; thus, an interview with the patient should be conducted in private if elder mistreatment is suspected. Patient risk factors for elder mistreatment include cognitive or behavioral impairment, poor physical health, and poor social supports. This review examines the approach to the patient, as well as definitive treatment, disposition, and outcomes for victims of elder abuse. The figure shows an algorithm for elder abuse assessment and intervention. Tables list types of elder abuse, factors predisposing to elder mistreatment, indicators of abuse, and the Elder Abuse Suspicion Index.

      This review contains 1 highly rendered figure, 4 tables, and 42 references.

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  • Hematology
    • Hematologic Malignancies
      • 1

        Acute Leukemia

        By Richard A. Larson, MD; Roland B. Walter, MD, PhD, MS
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        Acute Leukemia

        • RICHARD A. LARSON, MDProfessor of Medicine, Pritzker School of Medicine, University of Chicago. Chicago, IL
        • ROLAND B. WALTER, MD, PHD, MSAssistant Member, Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA, Associate Professor of Medicine, Division of Hematology/Department of Medicine, University of Washington, Seattle, WA

        The acute leukemias are malignant clonal disorders characterized by aberrant differentiation and proliferation of transformed hematopoietic progenitor cells. These cells accumulate within the bone marrow and lead to suppression of the production of normal blood cells, with resulting symptoms from varying degrees of anemia, neutropenia, and thrombocytopenia or from infiltration into tissues. They are currently classified by their presumed cell of origin, although the field is moving rapidly to genetic subclassification. This review covers epidemiology; etiology; classification of leukemia by morphology, immunophenotyping, and cytogenetic/molecular abnormalities; cytogenetics of acute leukemia; general principles of therapy; acute myeloid leukemia; acute lymphoblastic leukemia; and future possibilities. The figure shows the incidence of acute leukemias in the United States. Tables list World Health Organization (WHO) classification of acute myeloid leukemia and related neoplasms, expression of cell surface and cytoplasmic markers for the diagnosis of acute myeloid leukemia and mixed-phenotype acute leukemia, WHO classification of acute lymphoblastic leukemia, WHO classification of acute leukemias of ambiguous lineage, WHO classification of myelodysplastic syndromes, European LeukemiaNet cytogenetic and molecular genetic subsets in acute myeloid leukemia with prognostic importance, cytogenetic and molecular subtypes of acute lymphoblastic leukemia, terminology used in leukemia treatment, and treatment outcome for adults with acute leukemia.

        This review contains 1 highly rendered figure, 9 tables, and 117 references.

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      • 2

        Essential Thrombocythemia and Myelofibrosis

        By Srdan Verstovsek, MD, PhD; Hagop M. Kantarjian, MD
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        Essential Thrombocythemia and Myelofibrosis

        • SRDAN VERSTOVSEK, MD, PHDProfessor, Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX
        • HAGOP M. KANTARJIAN, MDProfessor and Chair, Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX

        This review details two major clonal stem cell disorders: essential thrombocythemia (ET) and myelofibrosis (MF). ET is distinguished by a sustained proliferation of megakaryocytes that results in peripheral blood thrombocytosis. Primary myelofibrosis (PMF) is associated with extramedullary hematopoiesis, splenomegaly, a leukoerythroblastic blood picture, and varying degrees of marrow fibrosis with marked megakaryocyte hyperplasia and atypia. The epidemiology, etiology/genetics, pathogenesis, diagnosis (including clinical manifestations and laboratory tests), differentials, management, and prognosis of each disorder are examined. Also included is the evaluation of treatment options for MF, including interferon alfa, JAK inhibitors, and allogeneic stem cell transplantation, the latter of which is still the only curative treatment for MF. Figures show treatment algorithms for ET and MF. Tables list the current criteria for the diagnosis of ET and PMF via the World Health Organization (WHO), the guidelines for diagnosis of post-ET MF via the International Working Group for Myelofibrosis Research and Treatment (IWG-MRT), prognostic factors in the International Prognostic Score for ET (IPSET) and IPSET-thrombosis, prognostic scoring systems for MF, and the clinical activity of JAK2 inhibitors.

        This review contains ­2 highly rendered figures, 6 tables, and 60 references. 

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      • 3

        Chronic Myeloid Leukemia

        By Elias Jabbour, MD; Susan O'Brien, MD
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        Chronic Myeloid Leukemia

        • ELIAS JABBOUR, MDAssociate Professor of Medicine, Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX
        • SUSAN O'BRIEN, MDProfessor of Medicine, Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX

        Chronic myeloid leukemia (CML), also known as chronic myelogenous leukemia, chronic myelocytic leukemia, and chronic granulocytic leukemia, is characterized by the expansion of myeloid progenitor cells at various stages of maturation, their premature release into the circulation, and a tendency to home to extramedullary sites. Symptoms at presentation reflect the increase in mass and turnover of the leukemic cells, although as many as 50% of patients are asymptomatic at diagnosis and come to attention through unexpected findings on routine blood tests. In treatment, the ongoing development of established and novel tyrosine kinase inhibitors (TKIs) has made for closer to normal life spans in patients diagnosed with CML. This review serves as an overview of CML, detailing its epidemiology and etiology, pathophysiology, diagnosis, treatment (including an assessment of the latest clinical trials involving TKIs), and management of patients with advanced phases. Figures show BCR-ABL signaling pathways and mechanisms of resistance to imatinib. Tables list stages of CML, differential diagnosis of CML and Philadelphia chromosome–negative myeloproliferative disorders, a summary of pivotal phase III trials of approved TKIs for the treatment of front-line or relapsed CML, response evaluation to TKIs used as first-line therapy, and a summary of important phase II trials of second- and third-generation TKIs after previous TKI failure.

        This review contains 2 highly rendered figures, 5 tables, and 87 references.

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      • 4

        Chronic Lymphocytic Leukemia and Other Chronic Lymphoid Leukemias

        By Tait D. Shanafelt, MD
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        Chronic Lymphocytic Leukemia and Other Chronic Lymphoid Leukemias

        • TAIT D. SHANAFELT, MDProfessor of Medicine, Division of Hematology, Mayo Clinic, Rochester, MN

        The chronic lymphoid leukemias are a group of generally indolent B cell malignancies that include chronic lymphocytic leukemia (CLL), prolymphocytic leukemia, hairy cell leukemia, and large granular lymphocyte leukemia. The unique aspects of diagnosis and management for each condition are discussed separately, with the primary focus being on CLL, the most common form of leukemia in most Western countries. Charts show the percentages of CLL patients’ survival divided into Rai risk category, treatment-free survival, and overall survival, and an algorithm displays approaches to selecting therapy for CLL patients. Tables list how CLL and other B cell lymphoproliferative disorders can be distinguished using immunophenotyping, chromosome categories by fluorescence in situ hybridization for predicting CLL patient survival, criteria indications for the initiation of therapy from the International Workshop on CLL and the National Cancer Institute CLL Working Group, and criteria for high-risk CLL disease.

        This review contains ­4 highly rendered figures, 4 tables, and 172 references. 

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      • 5

        Plasma Cell Disorders

        By Morie A. Gertz, MD, MACP
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        Plasma Cell Disorders

        • MORIE A. GERTZ, MD, MACPChair, Department of Medicine, Roland Seidler Junior Professor of the Art of Medicine, Mayo Clinic, Rochester, MN

        Multiple myeloma represents 1.4% of all new patients with cancer and will result in an estimated 11,090 deaths in 2014. It is twice as common in black men as in white men and 2.5 times more common in black women than in white women. Myeloma is the 14th most common cause of cancer in the United States, with a median age at diagnosis of 69 years. Multiple myeloma is defined by the presence of a clonal growth of plasma cells, usually in the bone marrow, but patients may also present with extramedullary disease. Anemia and bone disease are common in patients with multiple myeloma. Multiple myeloma cells display multiple genetic abnormalities, with no one specific genetic lesion common to a majority of patients. This module describes the immunologic profile of multiple myeloma and its diagnosis, monoclonal gammopathy of undetermined significance, smoldering multiple myeloma, plasmacytoma, plasma cell leukemia, the clinical presentation of multiple myeloma bone disease, anemia, renal impairment, hypercalcemia, and neurologic symptoms associated with multiple myeloma. Therapy for transplantation-eligible and non–transplantation-eligible patients, maintenance treatment for multiple myeloma, Waldenström macroglobulinemia, and amyloidosis are also discussed. Tables outline the risk of monoclonal gammopathy of undetermined significance evolution, the myeloma staging system, recommended diagnostic testing and uniform response criteria for myeloma, and commonly used regimens in the treatment of myeloma. Figures include a magnetic resonance image showing multiple plasmacytomas, tibial lytic lesion from myeloma, calvarial lytic lesions, a positron emission tomographic scan in a myeloma patient, and hyperviscosity causing retinal hemorrhages.

        This review contains 5 highly rendered figures, 5 tables, and 149 references.

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      • 6

        Lymphomas

        By Kieron Dunleavy, MD; Wyndham H. Wilson, MD, PhD
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        Lymphomas

        • KIERON DUNLEAVY, MDAttending Physician/Investigator, Lymphoma Therapeutics Section, Metabolism Branch, National Cancer Institute, Bethesoa, Maryland
        • WYNDHAM H. WILSON, MD, PHDSenior Investigator, Chief, Lymphoma Therapeutics Section, Metabolism Branch, National Cancer Institute, Bethesoa, Maryland

        Lymphoma is the fifth most common type of cancer in the United States, with 74,490 new cases estimated in 2009. Approximately 15% of patients with lymphoma have Hodgkin lymphoma; the remainder have one of the non-Hodgkin lymphomas. The incidence of non-Hodgkin lymphoma has increased steadily over recent decades. This chapter reviews the epidemiology, classification, clinical features, pathology, diagnostic evaluation, staging and prognosis, and treatment of Hodgkin and non-Hodgkin lymphoma. Other topics discussed include the acute and chronic effects of therapy for Hodgkin disease, as well as the subtypes of non-Hodgkin lymphomas, including indolent B cell lymphoma, follicular lymphoma, small lymphocytic lymphoma, mantle cell lymphoma, marginal-zone lymphoma, diffuse large B cell lymphoma (DLBCL), primary central nervous system lymphoma (PCNSL), Burkitt lymphoma, and HIV-related non-Hodgkin lymphoma. Figures illustrate the cellular appearance of Hodgkin lymphoma subtypes and DLBCL, diagnosis of DLBCL subtypes by gene expression, computed tomography and plain chest film in primary mediastinal cell lymphoma, MRI of the brain in PCNSL, and gene expression and gene expression predictors of survival among patients with DLBCL treated with rituximab, cyclophosphamide, hydroxydaunorubicin, vincristine [Oncovin], and prednisone (R-CHOP). Tables describe the Ann Arbor classification and the Cotswold modification for staging of lymphoma; the International Prognostic Score for advanced Hodgkin lymphoma; the World Health Organization classification of hematopoietic neoplasms; chromosomal translocations in non-Hodgkin lymphoma; the Eastern Cooperative Oncology Group performance scale; the International Prognostic Index for aggressive non-Hodgkin lymphoma; and the Follicular Lymphoma International Prognostic Index. This chapter has 185 references.

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    • Principles of Nonmalignant Hematology
      • 1

        Approach to the Patient With Benign Hematologic Disorders

        By J. Mark Sloan, MD; David C. Seldin, MD, PhD
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        Approach to the Patient With Benign Hematologic Disorders

        • J. MARK SLOAN, MDAssistant Professor of Medicine, Boston Univeristy School of Medicine, Boston, MA
        • DAVID C. SELDIN, MD, PHDChief, Section of Hematology-Oncology, Professor of Medicine and Microbiology, Boston University School of Medicine, Boston, MA

        Hematology principally concerns the function and disorders of the formed elements of the blood—red blood cells (RBCs), white blood cells (WBCs), and platelets—as well as those factors governing hemostasis. Hematologists have been a powerful force in basic biomedical and translational research. Their work, propelled partly by the ease of collection of blood and bone marrow for study, has enabled an understanding of many blood disorders at a fundamental molecular level. Techniques developed for the study of hematology are often adopted by other disciplines. This chapter discusses the anatomy of the hematopoietic system, hematopoiesis and the bone marrow, physical examination of the hematology patient, evaluation of the complete blood count (CBC) and peripheral blood smear, and coagulation. Tables delineate CBC parameters with normal ranges; peripheral smear findings, descriptions, and RBC indices and significance; laboratory findings in erythrocytosis; diseases commonly associated with eosinophilia and useful workup; common medications strongly associated with thrombocytopenia; and the 4Ts score for determining pretest probability of heparin-induced thrombocytopenia. Figures depict the three fractions of centrifuged blood, the lymph node, hematopoietic stem cells, bone marrow aspirate and biopsy procedure, architecture of the bone marrow microenvironment, petechiae, WBC types found in the smear of peripheral blood, the direct antiglobulin test, myeloid cells, and the coagulation system.

        This review contains 10 highly rendered figures, 6 tables, and 40 references.

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      • 2

        Red Blood Cell Function and Disorders of Iron Metabolism

        By Robert T. Means Jr, MD, FACP
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        Red Blood Cell Function and Disorders of Iron Metabolism

        • ROBERT T. MEANS JR, MD, FACPDean of Medicine, Professor of Internal Medicine, Office of the Dean, James H. Quillen College of Medicine, East Tennessee State University, Johnson City, TN

        The red blood cell, or erythrocyte, is an anucleate biconcave disk, approximately 7 µm in diameter. The principal function of the red blood cell is to exchange carbon dioxide for oxygen while in the pulmonary circulation, exchange that oxygen for carbon dioxide in the peripheral tissue, and carry that carbon dioxide back to the lungs. The physicochemical composition of the red cell is aligned to optimize that function. The state of tissue oxygenation, in turn, regulates the production of red cells. This review covers red blood cell function, iron metabolism, iron deficiency, iron overload, and primary iron overload. Figures show a model of the hemoglobin molecule showing the relative alignment of the α chains and β chains; the normal oxygen-hemoglobin dissociation curve shifted by changes in temperature, pH, and the intracellular concentration of 2,3-diphosphoglycerate; body iron supply and storage; regulation of hepcidin expression and its role in disease; blood smear from a patient with iron deficiency; and mechanisms contributing to iron overload in iron-loading anemias. Tables list laboratory results associated with decreased iron balance, causes of iron deficiency, oral iron replacement therapy, parenteral iron replacement therapy, primary iron overload syndromes, secondary iron overload syndromes, laboratory results associated with increased iron stores, and other rare disorders of iron overload.

        This review contains 6 highly rendered figures, 8 tables, and 108 references.

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      • 3

        Transfusion Medicine

        By Harvey G. Klein, MD
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        Transfusion Medicine

        • HARVEY G. KLEIN, MDThe Department of Transfusion Medicine is at the National Institutes of Health (NIH). I am Chief, Department of Transfusion Medicine. I also hold Professorships in both the Departments of Medicine and Pathology at the Johns Hopkins School of Medicine

        Transfusion medicine has advanced to a laboratory-based clinical discipline because of key discoveries and technical advances. These include the discovery of blood group antigens and the understanding of the host immune response to these antigens, development of methods of anticoagulation and storage of blood, and creation of plastic bags that allow sterile fractionation of whole blood into components. The potential of blood to act as an agent of disease transmission has heavily shaped both the donation process and transfusion practice. This chapter offers information to help the physician decide whether to transfuse. It includes sections on blood donation (autologous and directed), on postdonation screening procedures for the presence of viral agents (e.g., hepatitis, retrovirus, and emerging infectious pathogens), on pretransfusion testing (i.e., antigen phenotyping and testing for the presence of antibodies), and on blood components. Sections give specific information on transfusion of red cells, platelets, fresh frozen plasma, and recombinant clotting factors. Indications and complications of apheresis are described. Complications of transfusions are discussed, as are future prospects for transfusion therapy. Tables detail the advantages and disadvantages of autologous donation, estimated risks of blood transfusion, characteristics of blood products and indications for their use, plasma and recombinant clotting factors, indications for recombinant factor VIIa therapy, indications for the use of irradiated blood products, indications for the use of cytomegalovirus-negative blood products, and recommendations for therapeutic apheresis. This chapter contains 154 references.

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      • 4

        Hematopoietic Cell Transplantation

        By Frederick R. Appelbaum, MD
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        Hematopoietic Cell Transplantation

        • FREDERICK R. APPELBAUM, MDDirector, Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA

        Because hematopoietic stem cells can be collected from the peripheral blood, bone marrow, and umbilical cord blood, the term bone marrow transplantation is being replaced by the more inclusive term hematopoietic cell transplantation. With transplantation, an abnormal but nonmalignant lymphohematopoietic system can be replaced with a healthy one, making transplantation an effective therapy for a variety of nonmalignant diseases. Five types of hematopoietic cell transplantation are discussed in this chapter: syngeneic transplantation, allogeneic transplantation, autologous transplantation, peripheral blood cell transplantation, and umbilical cord blood transplantation. Specifics of the transplant procedure, including the preparative regimen, stem cell collection and infusion, and engraftment, are covered. Some complications of hematopoietic cell transplantation include early and late direct toxicities of the ablative preparative regimen, direct toxicities of reduced intensity conditioning, graft failure, graft versus host disease (GVHD), and a variety of infectious diseases. Hematopoietic cell transplantation can be used to combat specific diseases, such as immunodeficiency diseases, aplastic anemia, thalassemia, sickle cell anemia, and a number of malignant diseases. Treatment of posttransplant relapse is also discussed. Figures include charts showing the number of stem cell transplants performed between 1980 and 2009 and the patterns of myeloid recovery, plus photomicrographs depicting a liver biopsy in veno-occlusive liver disease, and a skin biopsy in acute GVHD. Tables show the clinical staging of acute GVHD and disease-free survival after hematopoietic cell transplantation. This chapter contains 126 references.

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    • Nonmalignant Hematologic Disorders
      • 1

        Anemia: Production Defects

        By Stavrula Otis, MD; Elizabeth A. Price, MD, MPH
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        Anemia: Production Defects

        • STAVRULA OTIS, MDClinical Instructor in Medicine, Stanford University, Stanford, CA
        • ELIZABETH A. PRICE, MD, MPHAssistant Professor in Medicine (Hematology), Stanford University, Stanford, CA

        Red blood cell production defects cause anemia that is marked by a low absolute reticulocyte count. Examination of the peripheral blood count and the bone marrow aids in classifying these disorders as defects associated with apparently normal bone marrow, defects associated with marrow aplasia or replacement, or defects associated with marrow erythroid hyperplasia and ineffective erythropoiesis. This review groups disorders within these classifications and provides aids to diagnosis and treatment options for each. Tables cover differential diagnosis of hypochromic anemias as well as the causes of aplastic anemia, cobalamin deficiency, and folic acid deficiency. Figures show peripheral blood smear findings in severe liver disease or starvation; results of bone marrow biopsy in a patient with aplastic anemia; a marrow smear indicating parvovirus infection; ringed sideroblasts from idiopathic sideroblastic anemia; and the molecular structure of folic acid. Diagrams show cobalamin assimilation and the role that cobalamin and folic acid deficiencies play in the pathogenesis of megaloblastic anemias.

        This review contains 7 highly rendered figures, 4 tables, and 216 references.

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      • 2

        Hemoglobinopathies and Hemolytic Anemias

        By Stavrula Otis, MD; Elizabeth A. Price, MD, MPH
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        Hemoglobinopathies and Hemolytic Anemias

        • STAVRULA OTIS, MDClinical Instructor in Medicine, Stanford University, Stanford, CA
        • ELIZABETH A. PRICE, MD, MPHAssistant Professor in Medicine (Hematology), Stanford University, Stanford, CA

        Alteration of the erythrocyte membrane usually signals the reticuloendothelial macrophages to remove the damaged red blood cell (RBC) from the circulation. In extraordinary circumstances, however, the damage to the membrane is so great that the erythrocyte undergoes hemolysis, and its intracellular contents, including hemoglobin, are liberated into the plasma. This chapter describes the structural and functional features of normal erythrocytes and diseases involving membrane architecture, RBC proteins, and extracorpuscular factors that can lead to shortened RBC survival. The chapter contains major discussions of sickle cell disease and the thalassemias. Included are tables providing information on erythrocyte metabolism and etiologies of hemolysis in glucose-6-phosphate dehydrogenase (G6PD) deficiency; figures illustrating histologic features of abnormal erythrocytes and sickle cells are also provided.

        This review contains 7 figures, 2 tables, and 243 references.

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      • 3

        Polycythemias

        By Virginia C. Broudy, MD
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        Polycythemias

        • VIRGINIA C. BROUDY, MD

        Polycythemia, also called erythrocytosis, is an increase in the number of circulating red blood cells per volume of blood, as reflected by an elevated hematocrit or hemoglobin level. The three major categories of polycythemia are relative polycythemia, secondary polycythemia, and polycythemia vera. Included in this chapter are discussions on initial evaluation; familial polycythemia; and polycythemia caused by renal and hepatic disorders, drug use, and appropriate increases in erythropoietin production. A flowchart depicts an approach to the evaluation of a patient with polycythemia. Other figures depict the oxygen-hemoglobin dissociation curve characteristic of various polycythemias and a gene mutation associated with familial polycythemia.

        This review contains 3 highly rendered figures and 27 references.

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      • 4

        Nonmalignant Disorders of Leukocytes

        By Andrew A. Lane, MD, PhD; Nancy Berliner, MD
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        Nonmalignant Disorders of Leukocytes

        • ANDREW A. LANE, MD, PHDFellow, Hematology-Oncology, Dana-Farber/Partners, Boston, MA
        • NANCY BERLINER, MDChief, Division of Hematology, Brigham and Women’s Hospital; Professor of Medicine, Harvard Medical School, Boston, MA

        Leukocytes are white blood cells; they protect the body against infections and participate in many types of immunologic and inflammatory responses. The two types of leukocytes are lymphocytes and phagocytes (which include neutrophils, monocytes, macrophages, and eosinophils). This chapter discusses each type of leukocyte separately, focusing on physiology and then specific disorders. Tables present normal leukocyte values in peripheral blood, drugs associated with neutropenia, intrinsic disorders of neutrophils that cause neutropenia, guidelines for management and prevention of febrile neutropenia, selected disorders of neutrophil function, diagnostic criteria for hemophagocytic lymphohistiocytosis, causes of eosinophilia, and causes of lymphocytosis. Figures include electron micrographs of a neutrophil, monocyte, and eosinophil; a graph showing the maturation process of neutrophils; where body neutrophils are found in the normal state; a diagram of the neutrophil response to bacterial invasion; where neutrophils in the peripheral blood exist; and an algorithm presenting the evaluation steps in a patient with recurrent infections from a phagocytic disorder.

        This review contains 6 highly rendered figures, 9 tables, and 79 references.

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      • 5

        Hemostasis and Its Regulation

        By Lawrence L. K. Leung, MD
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        Hemostasis and Its Regulation

        • LAWRENCE L. K. LEUNG, MDChief of Staff, Maureen Lyles D’Ambrogio Professor of Medicine, Stanford University School of Medicine, Chief of Staff, Veterans Affairs Palo Alto Health Care System, Stanford, CA

        Hemostasis, the process of blood clot formation, is a coordinated series of responses to vessel injury. It requires complex interactions between platelets, the clotting cascade, blood flow and shear, endothelial cells, and fibrinolysis. This review covers platelet plug formation, clotting cascade, initiation and propagation of blood clot formation, control mechanisms, overview of blood coagulation, blood coagulation as part of the host defense system, heterogeneity of endothelial cells and vascular bed–specific hemostasis, platelet production and thrombopoietin, and coagulation tests and their use. Figures show activated platelets, platelet aggregation, the classic and revised view of the clotting cascade, the inhibition of thrombin by antithrombin, the protein C/protein S pathway, the synergism between nitric oxide (NO) and prostacyclin (PGI2), tissue-type plasminogen activator, the transformation of fibrinogen to fibrin, activated protein C (APC) and carboxypeptidase B-2 (CPB-2) at the site of vascular injury, and an algorithm detailing the exposure of tissue factor at a vascular wound that initiates the clotting cascade. The table lists natural antithrombotic mechanisms of endothelial cells.

        This review contains 10 highly rendered figures, 1 table, and 45 references.

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      • 6

        Platelet Disorders

        By James L. Zehnder, MD; Lawrence L. K. Leung, MD
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        Platelet Disorders

        • JAMES L. ZEHNDER, MDProfessor of Pathology and Medicine (Hematology), Departments of Pathology and Medicine, Stanford University School of Medicine, Stanford, CA
        • LAWRENCE L. K. LEUNG, MDChief of Staff, Maureen Lyles D’Ambrogio Professor of Medicine, Stanford University School of Medicine, Chief of Staff, Veterans Affairs Palo Alto Health Care System, Stanford, CA

        A bleeding disorder may be suspected when a patient reports spontaneous or excessive bleeding or bruising, often secondary to trauma. Possible causes can vary between abnormal platelet number or function, abnormal vascular integrity, coagulation defects, fibrinolysis, or a combination thereof. This review addresses hemorrhagic disorders associated with quantitative or qualitative platelet abnormalities, such as thrombocytopenia, platelet function disorders, thrombocytosis and thrombocythemia, and vascular purpuras. Hemorrhagic dis­orders associated with abnormalities in coagulation (e.g., von Willebrand disease and hemophilia) are not covered. An algorithm shows evidence-based practice guidelines for the management of immune thrombocytopenic purpura. Tables list questions regarding bleeding and bruising to ask patients, clinical manifestations of hemorrhagic disorders, typical results of tests for hemostatic function in bleeding disorders, causes of thrombocytopenia, other forms of drug-induced thrombocytopenia, classification of platelet function disorders, and selected platelet-modifying agents.

        This review contains ­1 highly rendered figure, 7 tables, and 82 references. 

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      • 7

        Microangiopathic and Vascular Disorders

        By Nathan T. Connell, MD, MPH
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        Microangiopathic and Vascular Disorders

        • NATHAN T. CONNELL, MD, MPHInstructor in Medicine, Department of Medicine, Harvard Medical School, Associate Physician, Hematology Division, Brigham and Women’s Hospital, Boston, MA

        The thrombotic microangiopathies are characterized by microangiopathic hemolytic anemia and thrombocytopenia and can be classified as autoimmune, drug induced, complement mediated, and infectious/other. Reaching a definitive diagnosis for these disorders can be challenging due to the similarity of presenting symptoms and laboratory findings. Specific disorders described in this review include thrombotic thrombocytopenic purpura, the hemolytic-uremic syndrome, thrombotic microangiopathies of pregnancy (including preeclampsia and HELLP syndrome), disseminated intravascular coagulation, and antiphospholipid syndrome. Vascular disorders that lead to hematologic abnormalities are also discussed. Figures show the major classifications of the thrombotic microangiopathies; ADAMTS13 activity in normal and thrombotic thrombocytopenic purpura plasma; a fragmented red blood cell (arrow), also known as a schistocyte or helmet cell; major considerations in the initial treatment of thrombotic thrombocytopenic purpura and options for refractory patients as well as treatment considerations after discontinuation of plasma exchange; and a diagram of the complement pathway showing regulatory proteins as well as the site of action for the monoclonal antibody eculizumab. Tables list medications associated with thrombotic thrombocytopenia purpura, diagnostic criteria for HELLP, major classifications and examples of the causes of disseminated intravascular coagulation, diagnostic criteria for the antiphospholipid syndrome, vascular purpuras, and criteria for diagnosing hereditary hemorrhagic telangiectasia.

        This review contains 5 highly rendered figures, 6 tables, and 69 references.

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      • 8

        Thrombotic Disorders

        By Lawrence L. K. Leung, MD
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        Thrombotic Disorders

        • LAWRENCE L. K. LEUNG, MDChief of Staff, Maureen Lyles D’Ambrogio Professor of Medicine, Stanford University School of Medicine, Chief of Staff, Veterans Affairs Palo Alto Health Care System, Stanford, CA

        The three main elements in the pathophysiology of thrombosis are endothelial injury, a decrease in blood flow, and an imbalance between procoagulant and anticoagulant factors. The latter element can be either hereditary (e.g., antithrombin deficiency) or acquired (e.g., antiphospholipid syndrome). This review details the assessment of patients with thrombotic disorders, hereditary and acquired hypercoagulable states, and the management of venous thromboembolism. Figures show how the degradation of thrombin-activated factor V Leiden by activated protein C (APC) is significantly slower than that of normal activated factor V (factor Va), leading to enhanced thrombin generation; how normal factor V serves as a cofactor of APC in the inhibition of factor VIIIa, whereas factor V Leiden has a poor cofactor function; and how IgG antibodies recognize platelet factor 4–heparin complexes in heparin-induced thrombocytopenia. Tables list inherited and acquired hypercoagulable states, questions for assessing thrombosis, screening tests for patients with suspected hypercoagulable states, clinical features that suggest thrombophilia, frequency and relative risk of venous thrombosis in selected hypercoagulable states, proposed clinical and laboratory criteria for antiphospholipid syndrome, the classification of antiphospholipid antibodies, the 4Ts scoring system for heparin-induced thrombocytopenia, and general guidelines for the management of patients with venous thromboembolism.

        This review contains 2 highly rendered figures, 9 tables, and 168 references.

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      • 9

        Hemophilia

        By Aric Parnes, MD; Lisa Rotenstein, MD, MBA
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        Hemophilia

        • ARIC PARNES, MDHematology Division, Instructor in Medicine, Harvard Medical School, Brigham and Women’s Hospital, Boston, MA
        • LISA ROTENSTEIN, MD, MBAHarvard Medical School, Brigham and Women’s Hospital, Boston, MA

        Hemophilia is a family of rare bleeding disorders characterized by deficiency of clotting factors. Hemophilia A is an inherited deficiency of factor VIII, whereas hemophilia B (Christmas disease) represents a deficiency of factor IX. Both hemophilia A and B are X-linked diseases, with hemophilia A accounting for 80 to 85% of cases and hemophilia B 15 to 20%. Although hemophilia has historically referred to deficiencies of factors VIII and IX, it is important to recognize that similar bleeding disorders can occur with other missing clotting factors, although this is far more rare. This review covers the definition, history, epidemiology, biology/genetics, clinical manifestations, diagnosis, differential diagnosis, treatment, complications, measures of quality of care, and prognosis of hemophilia, as well as future directions. Figures show the clotting cascade, the genetic makeup of severe hemophilia A, an algorithm for diagnosing hemophilia, and hemophilic arthropathy in a patient’s knees. Tables list severity in hemophilia A and B, treatment of acute bleeding in hemophilia A and B, frequency of dosing in acute bleeding, and treatment of acute bleeding with inhibitors.

        This review contains 4 highly rendered figures, 4 tables, and 59 references.

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      • 10

        Von Willebrand Disease

        By Meghan Campo, MD; Elisabeth M. Battinelli, MD, PhD
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        Von Willebrand Disease

        • MEGHAN CAMPO, MDInstructor in Medicine, Department of Hematology Oncology, Harvard Medical School, Dana-Farber Cancer Institute, Boston, MA
        • ELISABETH M. BATTINELLI, MD, PHDAssistant Professor of Medicine, Associate Physician, Department of Hematology, Harvard Medical School, Brigham and Wom-en’s Hospital, Boston, MA

        Von Willebrand disease is the most common hereditary bleeding disorder. The disease is caused by inherited defects in the concentration, structure, or function of von Willebrand protein, a multimeric protein that mediates the initial adhesion of platelets at sites of vascular injury and binds and stabilizes blood clotting factor VIII in plasma. Defects in von Willebrand factor concentration, structure, or function that were not inherited can also occur; this is termed acquired von Willebrand syndrome. These defects occur as a consequence of other medical disorders (valvular heart disease, thrombocythemia, malignant neoplasms, and myeloproliferative and autoimmune diseases). This review examines the laboratory evaluation, clinical variants, and treatment of von Willebrand disease. Figures show the initial assessment of von Willebrand disease, and a treatment algorithm for von Willebrand disease. Tables list types of von Willebrand disease and medications used to treat von Willebrand disease.

        This review contains 2 highly rendered figures, 2 tables, and 10 references.

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  • Hepatology
    • 1

      Evaluating the Patient With Liver Disease

      By Andrew J. Muir, MD, MHS
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      Evaluating the Patient With Liver Disease

      • ANDREW J. MUIR, MD, MHSClinical Director of Hepatology, Division of Gastroenterology, Department of Medicine, Duke University School of Medicine, Durham, NC

      Until the advanced stages of cirrhosis, the identification of liver disease can be challenging for clinicians. In the earlier stages of the condition, most forms of chronic liver disease are asymptomatic or associated with vague and rather nonspecific complaints, such as fatigue. Even in the setting of cirrhosis, liver enzymes may be normal or mildly elevated. Patients with liver disease are currently recognized through a variety of routes, including screening programs, routine laboratory testing, and imaging performed for other complaints. This chapter discusses the approach to the evaluation of patients identified with liver disease, including the role of the history, physical examination, and diagnostic studies. In addition to understanding the etiology of the liver disease, understanding the severity and the risk of liver failure is critical to the evaluation process. Specific sections discuss mild elevation in aspartate aminotransferase (AST) and alanine aminotransferase (ALT), elevated alkaline phosphatase, elevated bilirubin, severe elevation in AST/ALT, and liver lesions. Figures include algorithms for the initial evaluation of patients with abnormal liver tests and the natural history of chronic liver disease; a breakdown of the etiology of liver disease among patients waiting for liver transplantation in the United States in 2012; and illustrations of the West Haven criteria for hepatic encephalopathy and the initial approach to patients with liver masses. Tables show the groups at risk for hepatitis C, hepatitis B, and nonalcoholic fatty liver disease; common patterns of drug-induced liver injury; common laboratory tests for the etiology of patients with liver disease; the Child-Pugh score for patients with cirrhosis; and the etiologies of, and diagnostic tests for, acute liver failure. This chapter contains 5 highly rendered figures, 5 tables, 75 references, 5 MCQs.

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    • 2

      Viral Hepatitis

      By Esperance A.K. Schaefer, MD, MPH; Jules L. Dienstag, MD
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      Viral Hepatitis

      • ESPERANCE A.K. SCHAEFER, MD, MPHResearch fellow, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA
      • JULES L. DIENSTAG, MDCarl W. Walter Professor of Medicine and Dean for Medical, Education, Harvard Medical School and Professor of Medicine, Massachusetts General Hospital, Boston, MA

      Despite a common tropism for the liver, the hepatitis viruses encompass a heterogeneous group of diseases with varying modes of acquisition, symptoms, chronicity, and complications. The five major hepatitis viruses, hepatitis A, B, C, D, and E, can be categorized by their modes of acquisition (parenteral versus enteral) and by their risk of chronicity. Beginning with the approach to the patient with viral hepatitis, this chapter reviews both the acute and chronic clinical features, diagnoses, and treatment options for hepatitis A, B, C, D, and E. Nonhepatotropic viruses that may cause liver inflammation are covered as well: herpes simplex virus, varicella-zoster virus, Epstein-Barr virus, cytomegalovirus, human parvovirus B19, adenovirus, yellow fever, rubella (German measles) and rubeola (measles), and coxsackievirus B. Treatment options are covered and listed in depth and details. Tables include characteristics of types of viral hepatitis, differential diagnosis of acute hepatitis, and treatment guidelines for hepatitis B.

      This review contains 4 highly rendered figures, 12 tables, and 145 references.

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    • 3

      Chronic Liver Diseases

      By Sandra Ciesek, MD; Michael P. Manns, MD
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      Chronic Liver Diseases

      • SANDRA CIESEK, MDDepartment of Gastroenterology, Hepatology, and Enterology, The Hannover Medical School, Hannover, Germany
      • MICHAEL P. MANNS, MDProfessor and Chairman, Department of Gastroenterology, Hepatology, and Enterology, The Hannover Medical School, Hannover, Germany

      The term chronic hepatitis encompasses many distinct clinical and pathologic diseases affecting the liver, the most important of which are autoimmune hepatitis (AIH), chronic hepatitis B with or without hepatitis D, and chronic hepatitis C caused by hepatitis C virus (HCV). The standard treatment of AIH is immunosuppressive therapy either with prednisolone alone or in combination with azathioprine. Although mycophenolate mofetil or cyclosporine can be used for retherapy in the case of treatment failure, controlled clinical trials are missing for these immunosuppressive drugs; thus, they are not part of the American Association for the Study of Liver Diseases (AASLD) practice guidelines for AIH.

      Chronic hepatitis B is a major global health care problem as 5% of the world’s population, or approximately 350 million persons, is chronically infected. Anti–hepatitis B virus (HBV) drugs can be divided into three classes: alfa interferons, nucleoside analogue (lamivudine, entecavir, telbivudine), and nucleotide analogue (adefovir dipivoxil, tenofovir dipivoxil). Interferon alfa has a number of potential side effects, and careful consideration must be given to its use. Tenofovir, which falls under the nucleotide analogue class, was approved by the Food and Drug Administration in 2008 and has been shown in clinical trials to have more potent activity in serum-suppressing HBV DNA levels than the comparable adefovir.

      More prevalent than chronic hepatitis B is chronic hepatitis C due to the high chronicity rate of HCV infection (approximately 160 million people are chronically infected with HCV worldwide). Until 2011, the only treatment for chronic HCV was a combination therapy of pegylated interferon alfa and ribavirin (RBV); however, new treatments for chronic hepatitis C include the directly acting antivirals (DAAs) sofosbuvir and simeprevir, although host-targeting agents are also being developed. The side effects of pegylated interferon/RBV therapy and DAAs should be considered, of which only sofosbuvir has shown no specific side effects so far in clinical studies.

      This module contains 5 highly rendered figures, 6 tables, 61 references, and 5 MCQs.

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    • 4

      Liver and Pancreas Transplantation

      By Julie A. Thompson, MD; Aleksandra Kukla, MD
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      Liver and Pancreas Transplantation

      • JULIE A. THOMPSON, MDAssistant Professor, Division of Gastroenterology and Hepatology, University of Minnesota, Minneapolis, MN
      • ALEKSANDRA KUKLA, MDAssistant Professor of Medicine, Medical Director of Pancreas Transplant Program, Division of Renal Diseases and Hypertension, University of Minnesota, Minneapolis, MN

      More than 6,000 liver transplantations are performed annually in the United States. Enhancements in patient selection and surgical technique and the availability of more powerful immunosuppressive agents have resulted in steady improvement in patient survival. As a result, liver transplantation has been accepted as the standard of care for patients with severe acute or chronic liver disease in whom conventional modalities of therapy have failed. The major obstacle to patients receiving the procedure is the critical shortage of donor organs. Many more recipients of liver transplantation are now receiving the bulk of their care from general internists, gastroenterologists, and primary care physicians. As a result, recognition of potential long-term complications and the need for appropriate immunizations and regular screening visits have become increasingly important. This chapter discusses who qualifies as a candidate for liver transplantation, contraindications to transplantation, timing of transplantation, operative procedures, complications of transplantation (e.g., perioperative and surgical complications, immunologic complications, infectious complications, complications of medical and immunosuppressive therapy, and disease-specific complications), and transplantation outcome. Pancreas transplantation, which aims at providing physiologic insulin replacement, is a therapy that reliably achieves euglycemia in patients with type 1 diabetes mellitus. The discussion of pancreas transplantation focuses on topics such as evaluation of candidates for transplantation (including islet transplantation); contraindications to transplantation; operative procedures; outcome survival; and the effect of transplantation on disorders associated with type 1 diabetes mellitus. The figures show estimated 3-month survival as a function of the Model for End-Stage Liver Disease (MELD) score, the sections of the liver that can be used for transplantation, an algorithm for evaluation of patients with type 1 diabetes mellitus being considered for pancreas transplantation, and an illustration of enteric drainage technique used in whole pancreas transplantation. The tables provide the common indications for liver transplantation, the scoring system for the Child-Turcotte-Pugh classification of liver disease severity, drug interactions with immunosuppressants, and immunization recommendations for liver transplant patients. 
      This chapter contains 4 highly rendered figures, 4 tables, 101 references, 5 MCQs, and 1 teaching slide set.

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    • 5

      Cirrhosis and Complications of Portal Hypertension

      By Andres Cardenas, MD; Isabel Graupera, MD; Elsa Sola, MD; Pere Ginès, MD, PhD
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      Cirrhosis and Complications of Portal Hypertension

      • ANDRES CARDENAS, MDGI Unit, Hospital Clínic and University of Barcelona, Institut d’Investigacions Biomèdiques August Pi-Sunyer, Ciber de Enfermedades Hepaticas y Digestivas, Instituto Reina Sofía de Investigación Nefrológica, Barcelona, Spain
      • ISABEL GRAUPERA, MDLiver Unit, Hospital Clínic and University of Barcelona, Institut d’Investigacions Biomèdiques August Pi-Sunyer, Ciber de Enfermedades Hepaticas y Digestivas, Instituto Reina Sofía de Investigación Nefrológica, Barcelona, Spain
      • ELSA SOLA, MDLiver Unit, Hospital Clínic and University of Barcelona, Institut d’Investigacions Biomèdiques August Pi-Sunyer, Ciber de Enfermedades Hepaticas y Digestivas, Instituto Reina Sofía de Investigación Nefrológica, Barcelona, Spain
      • PERE GINÈS, MD, PHDChairman, Liver Unit, Hospital Clínic, Professor of Medicine, University of Barcelona, Institut d’Investigacions Biomèdiques August Pi-Sunyer, Ciber de Enfermedades Hepaticas y Digestivas, Instituto Reina Sofía de Investigación Nefrológica, Barcelona, Spain

      Cirrhosis is the most advanced stage of all the different types of chronic liver diseases. It is defined as a diffuse disorganization of normal hepatic structure by extensive fibrosis associated with regenerative nodules. Hepatic fibrosis is potentially reversible if the causative agent is removed. However, advanced cirrhosis leads to major alterations in the hepatic vascular bed and is usually irreversible. Cirrhosis is a progressive and severe clinical condition associated with considerable morbidity and high mortality. It leads to a wide spectrum of characteristic clinical manifestations, mainly attributable to hepatic insufficiency and portal hypertension. Major complications of portal hypertension include ascites, gastrointestinal (GI) variceal bleeding, hepatic encephalopathy (HE), renal failure, and bacterial infections. In recent years, major advances in the understanding of the natural history and pathophysiology of cirrhosis and the treatment of its complications have led to improved management, quality of life, and life expectancy of patients with this disease. Cirrhosis is also a risk factor for developing hepatocellular carcinoma (HCC). Decompensated cirrhosis carries a poor short-term prognosis; thus, orthotopic liver transplantation (OLT) should always be considered in suitable candidates. This chapter describes the epidemiology, etiology and genetic factors, pathogenesis, diagnosis, general management, and treatment of cirrhosis. Complications of cirrhosis are discussed, including ascites, spontaneous bacterial peritonitis, dilutional hyponatremia, hepatorenal syndrome, variceal bleeding, hepatopulmonary syndrome and postpulmonary hypertension, HE, and HCC. Indications and contraindications for liver transplantation are described. Figures show liver biopsy results and ultrasound images in cirrhosis from hepatitis C, a patient with tense ascites, transjugular intrahepatic portosystemic shunting (TIPS), large esophageal varices with red spots, and HCC. Tables outline the main causes of cirrhosis and the diagnostic methods for identifying them, the Child-Pugh score, diagnostic criteria for hepatorenal syndrome, grades of HE, and indications for liver transplantation.
      This chapter contains 6 highly rendered figures, 6 tables, 73 references, and 5 MCQs.

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  • Human Genetics
    • 1

      Genomics Overview

      By W. Gregory Feero, MD, PhD
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      Genomics Overview

      • W. GREGORY FEERO, MD, PHDSpecial Advisor to the Director, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, Faculty, Maine-Dartmouth Family Medicine Residency, Augusta, ME

      New genomic applications are affecting internal medicine subspecialties and will soon affect the practices of all physicians. This chapter discusses the fields of genetics versus genomics and details the fundamentals of a genomic approach to health care. It includes special considerations such as the intersection between genomics and evidence-based medicine, genetic discrimination, the regulation of genetic testing, and the marketing of genetic testing directly to consumers. The chapter looks at genome-wide association studies and clinical care, as well as sequencing technologies. Tables offer examples of patterns of inheritance, clinical recommendations and red flags raised by family history, and intended uses for genetic tests. One figure shows an example pedigree obtained by using the US surgeon general's My Family Health Portrait family history tool, while the other shows the chromosomal locations of genetic markers associated with disease risk discovered in genome-wide association studies between 2005 and 2009. This chapter contains 41 references.

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    • 2

      Practice of Genetics in Clinical Medicine

      By Bruce R. Korf, MD, PhD, FACMG; Carlos Gallego, MD
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      Practice of Genetics in Clinical Medicine

      • BRUCE R. KORF, MD, PHD, FACMGProfessor and Chair, Department of Genetics, University of Alabama at Birmingham, Birmingham, AL
      • CARLOS GALLEGO, MDInternist Geneticist, Huntsville Hospital

      This review provides a general overview of the genetic approach in medical practice, discusses the principles of genetic testing, including interpretation of genetic tests and direct-to-consumer genomic testing, and looks at genetic counseling and approaches to treatment. The internist should become familiar with genetic disorders such as those associated with mutations in single genes or changes in chromosome number or structure. This is the traditional area of focus for medical geneticists and is likely to remain so. The internist should be familiar with basic principles of care for individuals with the more common of these conditions and needs to recognize clues that suggest the presence of these disorders, especially in family history. The section on genetics of common disorders focuses on pharmacogenetics, risk assessment, and prevention. Figures illustrate commonly used standard pedigree symbols and examples of autosomal recessive, autosomal dominant, X-linked recessive, and maternal inheritance. Tables offer different forms of genetic testing and types of gene mutations at genome and DNA levels.

      This review contains 2 figures, 2 tables, and 83 references.

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  • Infectious Diseases
    • Overview of Infectious Diseases
      • 1

        Introduction to Clinical Immunology: Overview of the Immune Response, Autoimmune Conditions, and Immunosuppressive Therapeutics for Rheumatic Diseases

        By Steven K. Lundy, PhD; Alison Gizinski, MD; David A. Fox, MD
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        Introduction to Clinical Immunology: Overview of the Immune Response, Autoimmune Conditions, and Immunosuppressive Therapeutics for Rheumatic Diseases

        • STEVEN K. LUNDY, PHDResearch Assistant Professor, Division of Rheumatology, Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, MI
        • ALISON GIZINSKI, MDAssistant Professor, Division of Rheumatology, Department of Internal Medicine, University of Arkansas for Medical Sciences, Little Rock, AR
        • DAVID A. FOX, MDProfessor, Division of Rheumatology, Department of Internal Medicine, University of Michigan Medical School and Health System, Ann Arbor, MI

        The immune system is a complex network of cells and mediators that must balance the task of protecting the host from invasive threats. From a clinical perspective, many diseases and conditions have an obvious link to improper functioning of the immune system, and insufficient immune responses can lead to uncontrolled acute and chronic infections. The immune system may also be important in tumor surveillance and control, cardiovascular disease, health complications related to obesity, neuromuscular diseases, depression, and dementia. Thus, a working knowledge of the role of immunity in disease processes is becoming increasingly important in almost all aspects of clinical practice. This review provides an overview of the immune response and discusses immune cell populations and major branches of immunity, compartmentalization and specialized immune niches, antigen recognition in innate and adaptive immunity, immune tolerance toward self antigens, inflammation and innate immune responses, adaptive immune responses and helper T (Th) cell subsets, components of the immune response that are important targets of treatment in autoimmune diseases, mechanisms of action of biologics used to treat autoimmune diseases and their approved uses, and mechanisms of other drugs commonly used in the treatment of autoimmune diseases. Figures show the development of erythrocytes, platelets, lymphocytes, and other immune system cells originating from hematopoietic stem cells that first reside in the fetal liver and later migrate to the bone marrow, antigen–major histocompatibility complex recognition by T cell receptor control of T cell survival and activation, and Th cells as central determinants of the adaptive immune response toward different stimuli. Tables list cell populations involved in innate and adaptive immunity, pattern recognition receptors with known ligands, autoantibody-mediated human diseases: examples of pathogenic mechanisms, selected Food and Drug Administration–approved autoimmune disease indications for biologics, and mechanism of action of biologics used to treat autoimmune diseases.

         

        This review contains 3 highly rendered figures, 5 tables, and 64 references.

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      • 2

        Vaccines and Vaccination

        By Gary J. Nabel, MD, PhD; Joseph P. Casazza, MD, PhD
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        Vaccines and Vaccination

        • GARY J. NABEL, MD, PHD
        • JOSEPH P. CASAZZA, MD, PHD

        In the 200 years since Jenner published Variolae Vaccinae, 10 major diseases have been brought under control by vaccination: smallpox, diphtheria, tetanus, yellow fever, pertussis, Haemophilus influenza type b (Hib), poliomyelitis, measles, mumps, and rubella. There are now at least 28 licensed vaccines for 24 infectious diseases. With this success come new challenges. New vaccines have crowded an already full childhood vaccination schedule. Increases in the number of immunocompromised individuals in society have simultaneously underscored the need for vaccination and raised the risk of adverse reactions. These circumstances, coupled with the need to monitor and maximize vaccine safety, require that the practitioner remain vigilant about vaccines and vaccination. This chapter includes a concise compilation of the latest Advisory Committee on Immunization Practices (ACIP) recommendations for vaccinating infants, children, and adults (including pregnant and breast-feeding women and immunologically impaired individuals), along with an explanation of the reasons for these recommendations; a detailed description of the different components of modern vaccines and their roles in vaccine stability and immunity; and current recommendations for post-exposure prophylaxis.

        This review contains 17 tables and 208 references.

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      • 3

        General Principles of Antibiotic Therapy

        By Alyssa R. Letourneau, MD, MPH; Michael S. Calderwood, MD, MPH
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        General Principles of Antibiotic Therapy

        • ALYSSA R. LETOURNEAU, MD, MPHDepartment of Medicine , Harvard Medical School, Assistant Director, Antimicrobial Stewardship Program, Massachusetts General Hospital, Boston, MA
        • MICHAEL S. CALDERWOOD, MD, MPHAssistant Professor, Department of Medicine, Harvard Medical School, Assistant Hospital Epidemiologist/Associate Director, Antimicrobial Stewardship, Brigham and Women’s Hospital, Boston, MA

        The essential feature of effective antibiotic agents is the ability to inhibit the growth of microorganisms at concentrations tolerated by the host. Antibiotic agents generally target anatomic structures or biosynthetic pathways unique to bacteria. The appropriate choice of an antibiotic for an infection depends on the following: clinician’s level of suspicion; the infecting organism and its antibiotic susceptibilities; the type of infection; factors associated with specific antibiotic agents; host factors; and public health considerations. This review provides an overview of antibiotic therapy and covers identifying the cause of an infection, determination of bacterial susceptibility to specific drugs, site of infection and ancillary therapy, antibiotic drug targets, pharmacodynamic parameters, factors affecting dosage and route of administration, host factors, complications of antibiotic therapy, Clostridium difficile infection, and antimicrobial resistance. Figures show sites of action for major antibiotic classes and pharmacodynamic parameters determining antibiotic efficacy. Tables list pharmacodynamic parameters predictive of outcome, antibiotics with equivalent oral and intravenous bioavailability, and direct drug toxicity of the major antibiotic classes.

         

        This review contains 2 highly rendered figures, 3 tables, and 43 references.

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      • 4

        Specific Antibiotic Agents

        By Alyssa R. Letourneau, MD, MPH; Michael S. Calderwood, MD, MPH
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        Specific Antibiotic Agents

        • ALYSSA R. LETOURNEAU, MD, MPHDepartment of Medicine , Harvard Medical School, Assistant Director, Antimicrobial Stewardship Program, Massachusetts General Hospital, Boston, MA
        • MICHAEL S. CALDERWOOD, MD, MPHAssistant Professor, Department of Medicine, Harvard Medical School, Assistant Hospital Epidemiologist/Associate Director, Antimicrobial Stewardship, Brigham and Women’s Hospital, Boston, MA

        The simultaneous use of multiple antibiotics in a shotgun fashion should be avoided because of the problems of drug toxicity and hypersensitivity reactions, microbial superinfections, and antagonisms between certain agents. Most bacterial infections can be treated satisfactorily with a single antibiotic agent. There are a limited number of situations, however, in which the simultaneous administration of different antibiotics is warranted. This review covers specific antimicrobial agents, including β-lactam antibiotics, aminoglycosides, polymyxins, tetracyclines, macrolides, clindamycin, nitroimidazoles, chloramphenicol, vancomycin, lipoglycopeptides, oxazolidinones, daptomycin, streptogramins, sulfonamides and trimethoprim, fluoroquinolones, nitrofurantoin, fosfomycin, rifamycins, and fidaxomicin, and provides empirical therapy recommendations. Figures show an overview of penicillin antibiotics, an overview of β-lactam/β-lactamase inhibitor combinations, and a positive D-zone test for inducible clindamycin resistance. Tables list antibacterial guidelines for initial inpatient empirical therapy and empirical sepsis guidelines.

         

        This review contains 3 highly rendered figures, 2 tables, and 78 references.

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    • Infectious Syndromes
      • 1

        Hyperthermia, Fever, and Fever of Undetermined Origin

        By Harvey B. Simon, MD, FACP
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        Hyperthermia, Fever, and Fever of Undetermined Origin

        • HARVEY B. SIMON, MD, FACPAssociate Professor of Medicine, Harvard Medical School, Health, Sciences, Technology Faculty, Massachusetts Institute of Technology, Physician, Massachusetts General Hospital, Boston, MA

        Abnormal elevation of body temperature, or pyrexia, can occur in one of two ways: hyperthermia or fever. In hyperthermia, thermal control mechanisms fail, so that heat production exceeds heat dissipation. In contrast, in fever, the hypothalamic thermal set point rises, and intact thermal control mechanisms are brought into play to bring body temperature up to the new set point. The distinction between fever and hyperthermia is more than academic: hyperthermia is best treated with drugs that lower the thermal set point, such as aspirin, other cyclooxygenase inhibitors, or acetaminophen. Fever of undetermined origin (FUO) presents one of the most challenging and perplexing problems in clinical medicine. Such fevers may persist for weeks or months in the absence of characteristic clinical findings or clues. Ultimately, most such obscure fevers prove to be caused by common diseases presenting in an atypical fashion rather than by rare and exotic illnesses. This chapter discusses fever and hyperthermia and further clarifies the distinction between fever and hyperthermia. The section on hyperthermia discusses the etiology of hyperthermia; the epidemiology, pathophysiology, diagnosis, differential diagnosis, treatment, and prevention of heatstroke; and the diagnosis and treatment of neuroleptic malignant syndrome, serotonin syndrome, and malignant hyperthermia of anesthesia. The section on fever discusses the pathogenesis, diagnosis, and treatment of fever, as well as the possible benefits and complications of elevated body temperature. The section on FUO presents the defining criteria of FUO; the etiologic classification of FUO, including the infections and neoplasms possibly responsible; and the diagnosis of FUO, including laboratory and radiologic studies, biopsies, and the role of exploratory laparotomy. Tables describe the causes of hyperthermia; the causes of FUO as reported in various studies over five decades; and a comprehensive list of causes of FUO. An algorithm depicts the progressive steps in the pathogenesis of fever and hyperthermia.

        This review contains 1 highly rendered figure, 3 tables, and 107 references.

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      • 2

        Sepsis

        By Steven P. LaRosa, MD; Steven M. Opal, MD
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        Sepsis

        • STEVEN P. LAROSA, MDStaff Physician, Infectious Disease Division, Beverly Hospital/Lahey Health System, Beverly, MA
        • STEVEN M. OPAL, MDChief, Infectious Disease Division, Memorial Hospital of Rhode Island, Pawtucket, RI

        Sepsis, along with the multiorgan failure that often accompanies the systemic inflammatory response syndrome (SIRS), is a leading cause of mortality in the intensive care unit. Although modest improvements in the prognosis have been made over the past two decades and promising new therapies continue to be investigated, innovations in the management of septic shock are still required. This chapter discusses the definitions, epidemiology, and pathogenesis (including microbial factors, host-derived mediators, and organ dysfunction) relating to sepsis. Management of severe sepsis and septic shock is also described. Tables outline the terminology of sepsis, the PIRO classification system, host-derived inflammatory mediators in septic shock, standard laboratory values in sepsis, multiple organ dysfunction syndrome in severe sepsis, hemodynamic findings, suggested empirical antibiotic choices, and current experimental therapies in the treatment of septic shock. Graphs show trends in sepsis cases and mortality rates in septic shock patients and patients with acute respiratory distress syndrome. Illustrations demonstrate the interaction between bacterial endotoxin and bacterial superantigens and neutrophil–endothelial cell interactions in sepsis.

        This review contains 5 highly rendered figures, 8 tables, and 143 references.

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      • 3

        Pneumonia and Other Pulmonary Infections

        By Joel T. Katz, MD, FACP
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        Pneumonia and Other Pulmonary Infections

        • JOEL T. KATZ, MD, FACPMedicine Education Office, Brigham and Woman’s Hospital, Boston, MA

        Pulmonary infections span a wide spectrum, ranging from self-limited to life-threatening and from acute to chronic. Although overall hospitalization rates are declining, hospitalizations for acute lower respiratory tract infections have increased steadily since 1980, particularly in the elderly. Taken together, pneumonia and influenza rank as the sixth leading cause of death in the United States and the leading infectious cause of death in the United States and the world. This chapter details the pathophysiology, epidemiology, general features, and treatment of pulmonary infections, particularly bacterial pneumonia. The chapter includes a Pneumonia Severity Index calculator. Tables list the major causes of pulmonary infection, host defense mechanisms against pulmonary infection, initial empirical antibiotic therapy in patients with suspected community-acquired pneumonia, initial antibiotic therapy for community-acquired pneumonia in outpatients and in patients who require hospitalization, and antibiotic choices for aspiration pneumonia. This chapter contains 105 references.

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      • 4

        Bacterial Infections of the Upper Respiratory Tract

        By Harvey B. Simon, MD, FACP
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        Bacterial Infections of the Upper Respiratory Tract

        • HARVEY B. SIMON, MD, FACPAssociate Professor of Medicine, Harvard Medical School, Health, Sciences, Technology Faculty, Massachusetts Institute of Technology, Physician, Massachusetts General Hospital, Boston, MA

        Upper respiratory tract infections range from common, benign processes to uncommon, potentially lethal processes. The overview of bacterial infections of the upper respiratory tract includes discussions of pathophysiology, etiology, and complications. Specific types are discussed separately with their own etiology, diagnosis, and treatment discussions. These include sinusitis, otitis (acute otitis media, chronic otitis media, and otitis externa), pharyngitis, deep tissue infections (e.g., peritonsillar abscess, retropharyngeal and parapharyngeal infections, and Ludwig angina), and acute epiglottitis (supraglottitis). Miscellaneous infections of the upper respiratory tract are also discussed. Figures include a diagram showing a sagittal section through the nose, mouth, and larynx; a diagram showing the sinuses; and an x-ray, CT scan, and sinus film showing signs of infection. Tables list the major flora of the upper respiratory tract and sites where they may cause infection; and antibiotic therapy for sinusitis, acute otitis media in children, and streptococcal pharyngitis. This chapter contains 67 references.

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      • 5

        Respiratory Viral Infections

        By Michael G. Ison, MD, MSc
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        Respiratory Viral Infections

        • MICHAEL G. ISON, MD, MSCAssociate Professor, Divisions of Infectious Diseases & Organ Transplantation, Northwestern University Feinberg School of Medicine, Chicago, IL

        The respiratory tract can be infected by a diverse group of viruses that produce syndromes ranging in severity from mild colds to fulminant pneumonias. Respiratory viral infections are a leading cause of morbidity, hospitalization, and mortality throughout the world; influenza and pneumonia were the most prevalent infectious causes of death during the 20th century in the United States. Respiratory viral infections are also a common cause of acute illness and physician visits in the United States. This chapter discusses the etiology, pathophysiology, and approach to the diagnosis of respiratory viral infections, as well as common syndromes, including common colds, pharyngitis and laryngitis, acute bronchitis, influenza syndrome, croup, bronchiolitis, reactive airway disease exacerbation, and pneumonia. The chapter also describes infections caused by specific agents, such as adenoviruses; human and zoonotic coronaviruses, including the MERS-CoV, which has emerged as a newly recognized pathogen causing severe respiratory viral infections; human metapneumovirus (hMPV); influenza virus; parainfluenza viruses; respiratory syncytial virus (RSV); and rhinovirus. The discussion of these infections includes classification and pathogenesis, epidemiology and transmission, diagnosis, complications, treatment, and prevention. Most notably, the emergence of the 2009 pandemic influenza A/H1N1 virus has enhanced our understanding of the pathogenesis and management of influenza. The chapter notes several novel antivirals, including DAS181, CMX001 (brincidofovir), and ALN-RSV01, that hold promise as future therapies against common respiratory viruses.

        Tables describe epidemiologic features of principal human respiratory viruses, laboratory methods for diagnosis of respiratory viral infections, and agents used to prevent and treat influenza. A sidebar lists Internet resources for respiratory viral infections. The chapter is also enhanced by a graph, numerous depictions of the viruses discussed, and a chest radiograph of a patient infected with influenza.

        This review contains 10 highly rendered figures, 3 tables, and 109 references.

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      • 6

        Acute Bacterial Meningitis

        By Karen L. Roos, MD
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        Acute Bacterial Meningitis

        • KAREN L. ROOS, MDJohn and Nancy Nelson Professor of Neurology and Professor of Neurological Surgery, Indiana University School of Medicine, Indianapolis, IN

        Acute bacterial meningitis is a life-threatening infection. By definition, meningitis is an infection of the meninges and the subarachnoid space. Bacterial meningitis is associated with an inflammatory response that involves the meninges, the subarachnoid space, the brain parenchyma, and the cerebral arteries and veins. As such, bacterial meningoencephalitis is the more accurate descriptive term. This chapter discusses the epidemiology, etiology, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, complications, and prognosis of the disease. The discussion of diagnosis covers clinical manifestations, physical examination findings, laboratory tests, and imaging studies. The discussion of treatment covers empirical therapy, specific antimicrobial therapy, and dexamethasone therapy. Graphs compare causative organisms and clinical manifestations of community-acquired meningitis. Illustrations depict proper patient positioning for detecting nuchal rigidity, Kernig sign, Brudzinski sign, and lumbar puncture, as well as a sagittal view of a lumbar puncture needle as it is advanced into the subarachnoid space. An algorithm delineates the approach to the patient with symptoms and signs of bacterial meningitis. Tables outline bacterial pathogens based on predisposing and associated conditions, cerebrospinal fluid diagnostic studies for meningitis, the appearance of the organism on a Gram stain, empirical antimicrobial therapy based on predisposing and associated conditions, recommendations for specific antibiotic therapy in bacterial meningitis, and recommended doses for antibiotics commonly used in the treatment of bacterial meningitis.

         

        This review contains 8 highly rendered figures, 6 tables, and 75 references.

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      • 7

        Acute Viral Meningitis

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        Acute Viral Meningitis

        Acute viral meningitis refers to inflammation of the meninges of the brain in response to a viral pathogen. Viruses cause meningitis, encephalitis, myelitis, or a combination of these, meningoencephalitis or encephalomyelitis. Viral meningitis is typically a self-limited disorder with no permanent neurologic sequelae. This chapter reviews the epidemiology, etiology, diagnosis, differential diagnosis, treatment, complications, and prognosis. Tables describe Wallgren’s criteria for aseptic meningitis, important arboviral infections found in North America, herpes family viruses and meningitis, classic cerebrospinal fluid (CSF) abnormalities with viral meningitis, Centers for Disease Control and Prevention criteria for confirming arboviral meningitis, basic CSF studies for viral meningitis, and etiology of CSF pleocytosis. Figures depict common causes of viral meningitis, nuchal rigidity, examination for Kernig sign, and Brudzinski sign for meningeal irritation.

        This review contains 4 highly rendered figures, 7 tables, and 16 references.

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      • 8

        Brain and Spinal Abscesses

        By Allan R. Tunkel, MD, PhD; W. Michael Scheld, MD
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        Brain and Spinal Abscesses

        • ALLAN R. TUNKEL, MD, PHDProfessor of Medicine, Drexel University College of Medicine, Philadelphia, PA, Chair, Department of Medicine, Monmouth Medical Center, Long Branch, NJ
        • W. MICHAEL SCHELD, MDProfessor of Medicine and Neurosurgery, University of Virginia School of Medicine, Charlottesville, VA

        Brain and spinal abscesses are often devastating infections that can lead to substantial morbidity and mortality if not recognized and treated in a timely manner. The clinical presentation depends upon the route of spread of infection to the central nervous system, location of the lesion, and severity of increased intracranial pressure. Brain abscess is defined as a focal intracranial infection that is initiated as an area of cerebritis and evolves into a collection of pus that is surrounded by a vascularized capsule; patients most often develop brain abscess by contiguous spread, hematogenous dissemination, or trauma. This chapter discusses the epidemiology, etiology, pathogenesis, diagnosis, management, and prognosis for brain abscess. Also examined are epidemiology and pathogenesis, etiology, diagnosis, management, and prognosis for both cranial subdural empyema and epidural abscess and spinal epidural abscess and subdural empyema. Tables list predisposing conditions and likely etiologic agents in brain abscess; histopathologic findings in the stages of brain abscess formation; presenting symptoms and signs in patients with brain abscess; antimicrobial therapy of brain abscess based on isolated pathogen; predisposing conditions and empirical antimicrobial therapy in patients with presumed bacterial brain abscess; recommended dosages of antimicrobial agents in adults with brain abscess; and normal renal and hepatic function. Figures in this chapter are images of intra-axial fluid collection; a subdural fluid collection; a ring-enhancing subdural empyema; an epidural abscess; a large epidural fluid collection with midline shift; a dorsal epidural collection; and loculated ring enhancement suggesting an epidural abscess.

        This review contains 6 highly rendered figures, 6 tables, and 167 references.

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      • 9

        Septic Arthritis, Septic Bursitis, and Osteomyelitis

        By Cameron Ashbaugh, MD
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        Septic Arthritis, Septic Bursitis, and Osteomyelitis

        • CAMERON ASHBAUGH, MDAssistant Professor of Medicine, Harvard Medical School, Associate Physician, Division of Infectious Diseases, Brigham and Women's Hospital, Boston, MA

        Infections of joints and bones are important causes of morbidity due to the potential for permanent injury to structures necessary for mechanical support and useful motion. The spectrum of disease is broad, with host factors, pathogen, site of infection, and comorbidities all influencing outcome. In some cases of bone infection, cure may not be possible, and the therapeutic goal becomes control. This review details the epidemiology, pathogenesis, diagnosis, differential diagnosis, treatment, and prognosis of septic arthritis, septic bursitis, vertebral body osteomyelitis, pedal osteomyelitis in association with diabetes, and chronic posttraumatic osteomyelitis with union or malunion.

        Figures include algorithms for the initial evaluation and management of a suspected septic joint, suspected vertebral body osteomyelitis, osteomyelitis in diabetic patients with neuropathic ulcers, and chronic posttraumatic osteomyelitis; pathophysiology of bone infection; magnetic resonance imaging (MRI) of vertebral body osteomyelitis; probe-to-bone test for diagnosing osteomyelitis in the diabetic foot; MRI of diabetic foot infection with osteomyelitis; nonsurgical treatment of osteomyelitis of the foot in a patient with diabetes; cutaneous sinus; a healed tibial fracture with malunion and chronic osteomyelitis; and a sequestrum associated with chronic tibial osteomyelitis. Tables describe representative studies with likelihood ratios for diagnostic tests used in the evaluation of native joint septic arthritis and diabetic pedal osteomyelitis; risk factors for the development of septic arthritis in patients with underlying joint disease; microbiology in septic arthritis, vertebral body osteomyelitis, and diabetic pedal osteomyelitis; empirical antibiotic therapy for septic arthritis; suggested tests and test frequencies to monitor for antibiotic toxicity; the two most commonly referenced classification schemes for osteomyelitis; supportive findings for diagnosis of chronic osteomyelitis; antibiotic therapy for vertebral osteomyelitis awaiting culture results and with unknown or established microbiology; International Working Group on the Diabetic Foot approach to diagnosis and management of diabetic pedal osteomyelitis; antibiotic therapy for diabetic pedal osteomyelitis with unknown or established microbiology; and antibiotic duration for diabetic pedal osteomyelitis. 

        This review contains 13 highly rendered figures, 15 tables, and 187 references.

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      • 10

        Infective Endocarditis

        By Patrick T. O'Gara, MD, FACC, FAHA, FACP
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        Infective Endocarditis

        • PATRICK T. O'GARA, MD, FACC, FAHA, FACPDirector, Clinical Cardiology, Brigham and Women’s Hospital, Professor of Medicine, Harvard Medical School, Boston, MA

        Infective endocarditis is a microbial infection of a cardiac valve or the mural endocardium caused by bacteria or fungi. Forms of this infection include subacute bacterial endocarditis (SBE) and acute bacterial endocarditis (ABE). Etiology and epidemiology are discussed. There is a section on pathogenesis followed by specific clinical presentations, including endocarditis associated with parenteral drug abuse as well as prosthetic valve endocarditis (PVE). Diagnosis and cardiac complications of endocarditis, treatment, recommendations for prophylaxis, and prognosis are addressed. There are several figures showing manifestations and anatomic relations from the infection. Tables describe microorganisms that cause native valve endocarditis, the etiology of PVE, the Duke criteria for diagnosis of infective endocarditis, antimicrobial therapy for endocarditis in adults, guidelines to prevent endocarditis, and recommendations and regimens for endocarditis prophylaxis. This chapter contains 99 references.

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      • 11

        Vaginitis and Sexually Transmitted Diseases

        By Joel T. Katz, MD, FACP
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        Vaginitis and Sexually Transmitted Diseases

        • JOEL T. KATZ, MD, FACPMedicine Education Office, Brigham and Woman’s Hospital, Boston, MA

        Sexually transmitted diseases (STDs) are among the most common causes of infectious illness worldwide, and therefore, familiarity with the recognition and management of these infectious pathogens is critical for physicians. This chapter reviews the epidemiology and transmission of STDs and describes the principles of taking a sexual history, reporting STDs, and screening for them. Urethritis, vulvovaginitis, mucopurulent cervicitis, pelvic inflammatory disease (PID), and genital ulcer disease are discussed. STDs in men who have sex with men is discussed, as is anorectal STDs in women. One figure shows a Gram stain in gonorrhea; the other shows ectopic pregnancy and tubal infertility in PID. Tables cover recommended STD screening; clinical features and management of vulvovaginitis; Amsel criteria for the diagnosis of bacterial vaginosis; treatment regimens for PID; clinical features, laboratory diagnosis, and treatment of genital ulcers; and treatment of genital herpes in immunocompetent patients. This chapter contains 106 references.

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      • 12

        Diseases Due to Chlamydiaceae

        By Walter E. Stamm, MD, FACP (deceased)
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        Diseases Due to Chlamydiaceae

        • WALTER E. STAMM, MD, FACP (DECEASED)

        The Chlamydiaceae are obligate intracellular bacteria that produce a wide variety of infections in many mammalian and avian species. Three species belonging to two genera of Chlamydiaceae infect humans: Chlamydia trachomatis, Chlamydophila psittaci, and Chlamydophila pneumoniae. C. trachomatis is exclusively a human pathogen and is transmitted from person to person via sexual contact, perinatal transmission, or close contact in households. C. psittaci, in contrast, is more widely distributed in nature, producing genital, conjunctival, intestinal, or respiratory infections in many avian and mammalian species. C. pneumoniae is a fastidious organism that produces upper respiratory tract infection and pneumonitis in both children and adults. This chapter details the epidemiology, pathogenesis, diagnosis, and treatment of chlamydial diseases. Sexually transmitted diseases, perinatal infections, adult inclusion conjunctivitis, trachoma, and psittacosis are covered. The chapter also includes tables outlining comparative features of the three species and treatment of sexually transmitted diseases caused by C. trachomatis, as well as a figure illustrating the life cycle of Chlamydiaceae. 

        This review contains 1 highly rendered figure, 2 tables, and 87 references.

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      • 13

        Syphilis and the Nonvenereal Treponematoses

        By Michael Augenbraun, MD, FACP
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        Syphilis and the Nonvenereal Treponematoses

        • MICHAEL AUGENBRAUN, MD, FACP

        Syphilis is an infectious disease with complex acute and chronic manifestations that is transmitted primarily through sexual contact. The disease has been recognized for many centuries, although its origin remains unknown. This chapter’s discussion of the epidemiology of syphilis includes figures illustrating the rate of syphilis in the United States by state and county and the rate of syphilis in the United States from 1941 to 2009. The etiology, pathogenesis and disease course, diagnosis, differential diagnosis, treatment, and complications of syphilis are discussed. Special cases of syphilis—in pregnant women, in children, in HIV-infected patients, and congenital syphilis—are also considered. Illustrations include a dark-field microphotograph of treponemes and photographs of a syphilitic chancre, the classic aculopapular rash from spirochetemia, condylomata lata, and a gumma. Tables outline the clinical manifestations of syphilis in adults and the treatment of syphilis. The nonvenereal treponematoses—yaws, endemic syphilis, and pinta—are a group of infections distributed throughout tropical and semitropical areas of the world. They are primarily noted to cause a variety of skin and skeletal lesions. There is little biologic difference between the treponemes that cause these conditions. The epidemiology, pathogenesis, diagnosis, differential diagnosis, and treatment of the nonvenereal treponematoses are discussed.

        This review contains 7 highly rendered figures, 2 tables, and 60 references.

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      • 14

        Urinary Tract Infections

        By Sigal Yawetz, MD
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        Urinary Tract Infections

        • SIGAL YAWETZ, MDAssociate Physician, Brigham and Women’s Hospital, Boston, MA; Assistant Professor of Medicine, Harvard Medical School, Boston, MA

        Urinary tract infection (UTI) is the most common bacterial infection, affecting women far more than men. Aerobic gram-negative bacteria are the most common uropathogens causing UTI, with Escherichia coli remaining the most predominant organism in complicated infections. UTI can result in a variety of infections and inflammations, from asymptomatic bacteriuria to typical symptomatic cystitis to acute pyelonephritis, as well as bacterial prostatitis in men.

        In general, antimicrobial therapy is warranted for any symptomatic infection of the urinary tract. However, new consensus treatment guidelines for uncomplicated UTI in women, set by the Infectious Diseases Society of America and the European Society for Microbiology of Infection Diseases in 2010, account for the increasing antimicrobial resistance of pathogens and focus on first-line empirical treatment regimens. To reduce the use of antibiotics, treatment and prevention of recurrent UTI may involve several strategies on varying levels of effectiveness; some of the more well-tested options include probiotics, antiseptics, and topical estrogen. Antimicrobial approaches should be reserved for women in whom these options prove to be ineffective.

        This review contains 7 highly rendered figures, 7 tables, and 120 references.

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      • 15

        Gastrointestinal Tract Infections

        By Luisa M. Stamm, MD, PhD; Marcia B. Goldberg, MD
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        Gastrointestinal Tract Infections

        • LUISA M. STAMM, MD, PHDResearch Fellow, Division of Infectious Diseases, Harvard Medical School, and Clinical Fellow, Massachusetts General Hospital, Boston, MA
        • MARCIA B. GOLDBERG, MDAssociate Professor of Medicine, Division of Infectious Diseases, Harvard Medical School, and Physician, Massachusetts General Hospital, Boston, MA

        The most notable symptoms associated with gastrointestinal infections are vomiting and diarrhea. In the United States, acute diarrhea (more than three loose stools in a day for less than 2 weeks) is a common medical complaint. Worldwide, it is estimated that 2 billion cases of diarrheal disease occur each year. It is the second leading cause of death for children. This chapter details the general approach to diarrheal illnesses and looks specifically at Salmonella, bacteremia and vascular infections, gastroenteritis, enteric fever, the carrier state (Salmonella organisms in the stool for more than 1 year after initial infection), Campylobacter and Shigella infections, Escherichia coli gastrointestinal infections, Yersinia and Vibrio cholerae infections, infections caused by Vibrio other than toxigenic V. choleraeClostridium difficile, enterotoxin-mediated causes of diarrhea, norovirus infections, gastrointestinal infections due to viruses other than norovirus, and parasitic gastrointestinal infections. Figures show the clinical presentation of gastrointestinal tract disease, the evaluation of acute diarrhea, the growth of enteric pathogens on selective agar, the pathology of Y. pseudotuberculosis infection, and imaging in C. difficile infection. Tables cover gastrointestinal infections, pathogenesis correlates with signs of disease, infectious innocula of selected gastrointestinal pathogens, selected therapies for enteric pathogens, the percentage of Salmonella and Shigella isolates resistant to selected antibiotics in the United States, clinical and laboratory findings in untreated enteric fever, vaccinations for enteric fever, clinical categories and recommendations for the treatment of C. difficile infection, the Kaplan criteria for establishing norovirus as the cause of an outbreak of gastroenteritis, and recommendations for the prevention and control of norovirus outbreaks.

        This review contains 88 references.

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      • 16

        Peritonitis and Intra-abdominal Abscesses

        By W. Conrad Liles, MD, PhD; E. Patchen Dellinger, MD
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        Peritonitis and Intra-abdominal Abscesses

        • W. CONRAD LILES, MD, PHDProfessor and Vice-Chair of Medicine, Director, Division of Infectious Diseases, University of Toronto, Toronto, ON
        • E. PATCHEN DELLINGER, MDProfessor and Vice-Chair, Department of Surgery, University of Washington, Chief, Division of General Surgery, University of Washington Medical Center, Seattle, WA

        Peritonitis is a diffuse or localized inflammatory process affecting the peritoneal lining. Peritonitis has acute and chronic forms and may have a variety of causes. The etiology, epidemiology, diagnosis, and treatment of acute peritonitis caused by bacteria or fungi, including primary and secondary peritonitis, are discussed in this chapter. Primary or spontaneous peritonitis has no underlying intra-abdominal disorder as a direct cause of the infection but usually involves an underlying disorder that inhibits normal host defenses in the peritoneal cavity. Secondary peritonitis has an intra-abdominal focus that initiates the infection. Tertiary peritonitis is a relatively new term that refers to the persistence of intra-abdominal infection after the initial treatment of secondary peritonitis. Peritonitis in dialysis patients is also discussed. Intra-abdominal abscesses may present as complications of abdominal surgery, intra-abdominal conditions (e.g., diverticulitis, appendicitis, biliary tract disease, pancreatitis, perforated viscus), or penetrating abdominal trauma; as fever of obscure origin; or as dysfunction of neighboring organs (e.g., so-called lower lobe pneumonia related to a subphrenic abscess). Intra-abdominal abscesses are classified according to the anatomic location in which they occur: intraperitoneal, retroperitoneal, or visceral. Discussion of the different intra-abdominal abscesses in this chapter includes their diagnosis, bacteriology, and treatment. A figure shows CT scans before and after treatment of a multilobular liver abscess. This chapter contains 101 references.

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    • Infectious Pathogens
      • 1

        Herpesvirus Infections

        By Martin S. Hirsch, MD
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        Herpesvirus Infections

        • MARTIN S. HIRSCH, MD

        The herpes group of viruses is composed of at least eight human viruses and numerous animal viruses. The human herpesviruses include herpes simplex virus types 1 and 2 (HSV-1 and HSV-2), varicella-zoster virus (VZV), cytomegalovirus (CMV), Epstein-Barr virus (EBV), and human herpesvirus types 6 (HHV-6), 7 (HHV-7), and 8 (HHV-8, also known as Kaposi sarcoma–associated herpesvirus). Human herpesviruses share the properties of latency and reactivation. Members of the group can cause productive lytic infections, in which infectious virus is produced and cells are killed, or nonproductive lytic infections, in which viral DNA persists but complete replication does not occur and cells survive. After acute lytic infections, herpesviruses often persist in a latent form for years; periodic reactivations are followed by recurrent lytic infections. Sites of latency vary: HSV and VZV persist in neural ganglion cells, EBV persists in B cells, and CMV probably remains latent in many cell types. The sites of latency for HHV-6 and HHV-7 have not been identified, although both herpesviruses have been detected in salivary glands. All human herpesviruses have a worldwide distribution. Considerable efforts are being directed toward the development of vaccines and antiviral agents that will be active against herpesviruses. This chapter discusses the epidemiology, pathogenesis, diagnosis, prevention, and treatment of herpes simplex virus and varicella-zoster virus and their clinical syndromes. The descriptions of the clinical syndromes include complications and clinical features, as well as descriptions of symptoms. Tables provide information on chemotherapy for primary genital and mucocutaneous herpes infection, suppression of severe and recurring genital herpes infection, and varicella-zoster infection. Figures provide photographic illustrations of the various clinical syndromes. A sidebar about herpesvirus information on the Internet provides further detail.

        This review contains 123 references, 4 tables, and 6 highly rendered figures.

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      • 2

        Viral Hepatitis

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        Viral Hepatitis

        Despite a common tropism for the liver, the hepatitis viruses encompass a heterogeneous group of diseases with varying modes of acquisition, symptoms, chronicity, and complications. The five major hepatitis viruses, hepatitis A, B, C, D, and E, can be categorized by their modes of acquisition (parenteral versus enteral) and by their risk of chronicity. Beginning with the approach to the patient with viral hepatitis, this chapter reviews both the acute and chronic clinical features, diagnoses, and treatment options for hepatitis A, B, C, D, and E. Nonhepatotropic viruses that may cause liver inflammation are covered as well: herpes simplex virus, varicella-zoster virus, Epstein-Barr virus, cytomegalovirus, human parvovirus B19, adenovirus, yellow fever, rubella (German measles) and rubeola (measles), and coxsackievirus B. Treatment options are covered and listed in depth and details. Tables include characteristics of types of viral hepatitis, differential diagnosis of acute hepatitis, and treatment guidelines for hepatitis B.

        This review contains 4 highly rendered figures, 12 tables, and 145 references.

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      • 3

        Viral Zoonoses

        By Lyle R. Petersen, MD, MPH; Duane J. Gubler, SCD
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        Viral Zoonoses

        • LYLE R. PETERSEN, MD, MPH
        • DUANE J. GUBLER, SCD

        Human infection by zoonotic viruses—pathogens that normally infect animals—may result in no obvious illness, a nonspecific viral syndrome, or more severe illness typically characterized by hemorrhagic fever, encephalitis, or rash arthralgia. Transmission usually occurs by direct contact with or a bite from an infected animal or arthropod. Viral families discussed include Flaviviridae, Bunyaviridae, Arenaviridae, Filoviridae, Togaviridae, Rhabdoviridae, Paramyxoviridae, and Reoviridae, with consideration given to the epidemiology, diagnosis, treatment, and prevention of specific viruses. Hemorrhagic fevers addressed include dengue fever, dengue hemorrhagic fever, yellow fever, Crimean-Congo hemorrhagic fever, and Rift Valley fever; hantavirus infections; and the Marburg and Ebola viruses. Encephalitic fever–causing viruses discussed include La Crosse; Japanese; Murray Valley; St. Louis; tick-borne; West Nile; Powassan; eastern, western, and Venezuelan equine; rabies; Nipah; Barmah Forest; and Colorado tick fever. Rash arthralgia may be caused by the Barmah Forest, Chikungunya, Mayaro, O’nyong-nyong, Ross River, and dengue viruses. Other viral zoonoses considered include monkey B virus, ruminant and primate poxvirus, Newcastle, and foot-and-mouth diseases, as well as vesicular stomatitis virus infection. A diagram depicts the generalized arbovirus maintenance cycle. Tables list the important viral zoonoses that cause human disease, the principal hantaviruses that cause human disease, the arenaviruses that cause significant human illness, and the viral zoonoses endemic in the United States. 

        This review contains 1 highly rendered figure, 4 tables, and 81 references.

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      • 4

        HIV and AIDS

        By Daniel R. Kuritzkes, MD, FACP
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        HIV and AIDS

        • DANIEL R. KURITZKES, MD, FACP

        In the quarter-century since the first report of AIDS in the United States, HIV infection has spread throughout the population, disproportionately affecting black women, Hispanic women, and men who have sex with men. The prognosis for persons infected with HIV has improved dramatically with the introduction and evolution of highly active antiretroviral therapy (HAART). The underlying principle of HAART is that a combination of potent antiretrovirals, each of which requires different mutations in the HIV genome for resistance to develop, can suppress replication sufficiently to prevent mutation and the emergence of resistance. The prospect that currently available antiretroviral therapy (ART) regimens may suppress HIV replication indefinitely provides the hope that infected patients will have life expectancies similar to those of age-matched uninfected individuals. For these patients, HIV care has shifted from an emphasis on treatment and prevention of the complications of HIV disease itself to a focus on suppression of HIV replication and management of short- and long-term complications of HIV, ART toxicities, and aging. This chapter describes the epidemiology, pathophysiology and pathogenesis, prevention, diagnosis, and management of acute and chronic HIV infection and AIDS, with figures and tables illustrating each chapter section.


        This review contains 9 highly rendered figures, 22 tables, and 249 references.

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      • 5

        Infections Due to Gram-positive Cocci

        By Dennis L. Stevens, PhD, MD, FACP, FIDSA
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        Infections Due to Gram-positive Cocci

        • DENNIS L. STEVENS, PHD, MD, FACP, FIDSAChief Infectious Diseases, Veterans Affairs Medical Center, Boise, ID, Professor of Medicine, University of Washington School of Medicine, Seattle, WA

        The gram-positive cocci that produce infection include pneumococci, group A streptococci, non?group A streptococci (including groups B, C, D, G, and nongroupable streptococci), anaerobic streptococci, enterococci, and staphylococci. This chapter discusses the pathogenesis, diagnosis, and treatment of infections associated with each of these gram-positive cocci, including methicillin-resistant Staphylococcus aureus (MRSA). The clinical infections caused by each of these organisms are reviewed. Tables describe the incidence of pneumococcal disease according to age and underlying disease, factors associated with adverse outcomes in pneumococcal pneumonia; medically important streptococci and enterococci; antibiotic treatment for penicillin-resistant Streptococcus pneumoniae, enterococcal infections, and staphylococcal infections; laboratory tests for streptococcal pharyngitis; clinical manifestations and antibiotic treatment for staphylococcal toxic-shock syndrome (TSS); revised Jones criteria for the diagnosis of acute rheumatic fever, and drug treatment of acute rheumatic fever.

        This review contains 105 references.

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      • 6

        Infections Due to Escherichia Coli and Other Enteric Gram-negative Bacilli

        By Michael S. Donnenberg, MD
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        Infections Due to Escherichia Coli and Other Enteric Gram-negative Bacilli

        • MICHAEL S. DONNENBERG, MDProfessor of Medicine, Associate Chairman for Research, Department of Medicine, University of Maryland School of Medicine, 20 Penn Street, HSF2 S403D, Baltimore, MD 21201, 410-706-7562

        This chapter describes infections caused by Escherichia coli and related members of the family Enterobacteriaceae, excluding genera that principally cause enteric infections. The family Enterobacteriaceae comprises facultative anaerobic gram-negative bacilli that ferment sugars and reside principally in the gastrointestinal tract of vertebrates, although some are found primarily in the environment.  E. coli, the most common facultative anaerobe in the human intestine, is distinguished from other members of the family Enterobacteriaceae primarily on the basis of  E. colis ability to ferment lactose and to produce indole and its inability to hydrolyze urea. Diarrhea and the extraintestinal infections caused by  E. coli are discussed in this chapter. A table describes infections by the six pathotypes of  E. coli that cause diarrhea, and figures show the pathogenesis of enterotoxigenic, enteropathogenic, and enterohemorrhagic  E. coli. Organisms of the genus  E. coli are noted for their ability to take two forms: (1) typical bacillary swimmer cells, which express a variety of surface fimbriae as well as flagella, and (2) highly elongated swarm cells, which express hundreds of flagella and few other surface structures. The pathogenesis, epidemiology, diagnosis, and treatment of Proteus infections are reviewed. The genus Klebsiella includes five species, but  K. pneumoniae and, to a lesser extent,K. oxytoca are responsible for the vast majority of human Klebsiella infections. These organisms are found in the environment and in the GI tract. Enterobacter and Serratia are closely related to Klebsiella; like Klebsiella, they are principally opportunistic pathogens that cause a variety of nosocomial infections. The epidemiology, etiology, diagnosis, and treatment of these infections are reviewed.

        This review has 72 references.

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      • 7

        Infections Due to Brucella, Francisella, Yersinia Pestis, and Bartonella

        By W. Conrad Liles, MD, PhD
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        Infections Due to Brucella, Francisella, Yersinia Pestis, and Bartonella

        • W. CONRAD LILES, MD, PHDProfessor and Vice-Chair of Medicine, Director, Division of Infectious Diseases, University of Toronto, Toronto, ON

        Brucellosis, tularemia, (bubonic) plague, and bartonellosis are zoonoses (i.e., infectious diseases that can be transmitted from animals to humans) caused by gram-negative bacilli. The causative agents of these diseases are Brucella species, Francisella tularensis, Yersinia pestis, and Bartonella species, respectively. Infected arthropods, such as ticks or fleas, can serve as vectors for the transmission of tularemia, (bubonic) plague, and bartonellosis. In general, the diagnosis of these zoonoses requires the physician to consider the clinical presentation (which is not always distinctive) in light of the epidemiology of these diseases. This chapter contains discussions of epidemiology, etiology, pathogenesis, diagnosis and differential diagnosis, treatment, complications, and prognosis of brucellosis, tularemia, plague, and Bartonella infections (including trench fever, cat-scratch disease, and bacillary angiomatosis). Also included are tables outlining diagnosis and treatment and a figure showing the distribution of tularemia in the United States from 1990 to 2000. 

        This review contains 1 highly rendered figure, 2 tables, and 81 references.

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      • 8

        Anaerobic Infections

        By Anthony W. Chow, MD, FACP, FRCPC
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        Anaerobic Infections

        • ANTHONY W. CHOW, MD, FACP, FRCPCProfessor Emeritus, Division of Infectious Diseases, Department of Medicine, University of British Columbia and Vancouver, Hospital, Vancouver, British Columbia, Canada.

        Anaerobic infections characteristically entail multiple organisms (polymicrobial), both anaerobic and facultative (mixed). The organisms tend to be acquired endogenously. The particular mix of pathogens reflects the combined influence of the complex commensal flora at a specific body site and the unique microbiota of the underlying conditions. Because these organisms are generally of low pathogenicity, anaerobic or mixed infections generally develop as a consequence of either structural alterations in the normal mucosal barrier or tissue ischemia with lowered oxidation-reduction potential. This chapter reviews the pathogenesis, clinical features, and management of anaerobic infections. Three etiologic categories of anaerobic infections are discussed: those caused by BacteroidesPrevotellaPorphyromonas, and other mixed anaerobes; those caused by Actinomyces species; and those caused by Clostridium species. Clostridia are gram-positive, spore-forming bacilli; all species are obligate anaerobes, but C. perfringens is relatively aerotolerant. There are three major groups of clostridia, each of which is discussed in this chapter: histotoxic species (C. perfringensC. novyi, C. septicumC. bifermentans, and C. sordellii), enterotoxigenic species (C. perfringens and C. difficile), and neurotoxic species (C. tetani and C. botulinum). Figures include Gram stains ofBacteroides fragilisFusobacterium nucleatumC. perfringens, and Actinomyces israelii; a diagram of the biphasic disease model of mixed infection in intra-abdominal sepsis; photographs of sulfur granules in actinomycosis; and a drawing of opisthotonos in tetanus. Tables list the predominant normal flora at various body sites; anaerobic bacteria and their distribution in normal flora and in infection; the effect of host conditions on the indigenous microflora at various body sites; microbial virulence factors important in mixed anaerobic infections; anaerobic infections associated with specific infections; predicted in vitro susceptibility of clinically important anaerobes to major classes of antimicrobial agents; empirical antimicrobial regimens for suspected anaerobic or mixed infections; and recommendations for tetanus prophylaxis in routine wound management. This chapter contains 92 references.

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      • 9

        Lyme Disease and Other Spirochetal Zoonose

        By David C. Tompkins, MD; Benjamin J. Luft, MD, FACP
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        Lyme Disease and Other Spirochetal Zoonose

        • DAVID C. TOMPKINS, MDVice Chairman, Department of Medicine, Head, Division of Infectious Diseases, Lutheran Medical Center, Brooklyn, NY
        • BENJAMIN J. LUFT, MD, FACPEdmund D. Pellegrino Professor, Division of Infectious Diseases, Department of Medicine, SUNY at Stony Brook, Stony Brook, NY

        This review discusses the epidemiology, pathology, clinical features, laboratory findings, and treatment for Lyme disease, leptospirosis, relapsing fever, and rat-bite fever. Lyme disease is a tick-borne illness caused by the spirochete Borrelia burgdorferi. Lyme disease is a progressive infectious disease with a wide array of clinical manifestations. In most persons, the initial sign of infection is the development of erythema migrans. In general, three stages of the illness can be distinguished: early localized disease, early disseminated disease, and persisting late disease. A photograph shows an erythema migrans lesion, and an algorithm for diagnosis of Lyme disease is provided. A table lists the antibiotic regimens used for the different stages and manifestations of Lyme disease. Leptospirosis is a worldwide zoonosis caused by spirochetes from the genus Leptospira; these spirochetes are shown in a photomicrograph. The disease is acquired by contact with infected animals or exposure to contaminated soil or freshwater and can cause illness ranging from asymptomatic infection to severe multisystem disease with a significant mortality. Relapsing fever is an acute louse-borne or tick-borne infection caused by spirochetes of the genus Borrelia and is characterized by recurrent febrile episodes separated by asymptomatic intervals. Rat-bite fever is infection caused by Streptobacillus moniliformis in the United States and Europe or by Spirillum minus in Asia. A table details the differing characteristics of rat-bite fever from these two organisms.

        This review has 124 references.

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      • 10

        Infections Due to Rickettsia and Related Organisms

        By Aurélie Renvoisé, MD; Didier Raoult, MD, PhD
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        Infections Due to Rickettsia and Related Organisms

        • AURÉLIE RENVOISÉ, MDUnité des Rickettsies, CNRS-IRD UMR6236-198, Université de la Méditerranée, Faculté de Médecine, 27 bd Jean Moulin, 13385 Marseille cedex 5, France
        • DIDIER RAOULT, MD, PHDUnité des Rickettsies, CNRS-IRD UMR6236-198, Université de la Méditerranée, Faculté de Médecine, 27 bd Jean Moulin, 13385 Marseille cedex 5, France

        This review defines rickettsiae and discusses etiology, pathogenesis, diagnosis, and complications of infections due to rickettsiae. Diseases, largely transmitted by mites, fleas, lice, or ticks, include various forms of spotted fever and typhus. Also discussed are the etiology, pathogenesis, diagnosis, and complications from human ehrlichioses and anaplasmoses, and Q fever (both chronic and acute). Figures show the world distribution of pathogenic Rickettsia, purpuric macules, eschar, peripheral blood smear, and natural history of Q fever. Tables list the taxonomic positions of bacteria involved in human rickettsioses, ehrlichioses, anaplasmoses, and Coxiella burnetii, infections due to Rickettsiawith corresponding etiological agents, and synonyms for species that underwent taxonomy reorganization.

        This review contains 92 references.

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      • 11

        Mycoplasma Infections

        By R. Doug Hardy, MD
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        Mycoplasma Infections

        • R. DOUG HARDY, MD

        Mycoplasmas are the smallest known free-living organisms. Their size-150 to 350 nm-is closer to that of viruses than of bacteria. Mycoplasmas, however, are able to grow in cell-free media and possess both RNA and DNA. Notably, they lack a cell wall and are bound by a cell membrane. The absence of a rigid cell wall explains many of the biologic properties of mycoplasmas, including resistance to beta-lactam antibiotics and marked pleomorphism among individual cells. At least 13 Mycoplasma species, two Acholeplasma species, and one Ureaplasma species have been isolated from humans with varying frequency; most of these species are thought to be normal inhabitants of oral and urogenital mucous membranes. Only four species, M. pneumoniae, M. hominis, U. urealyticum, and U. urealyticum, have been shown conclusively to be pathogenic in humans. This chapter covers the epidemiology, pathogenesis, pathology, immunology, diagnosis, and treatment of infections caused by mycoplasmas. Pneumonia and genitourinary tract infections (including nongonococcal urethritis and pelvic inflammatory disease) are discussed in major sections. Tables list the accuracy of diagnostic tests for M. pneumoniae and the antimicrobial drugs and doses used in the treatment of mycoplasma infections. A figure illustrates the spread of M. pneumoniaewithin a family. This chapter contains 122 references.

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      • 12

        Tuberculosis

        By Jonathan B. Parr, MD, MPH; Michael K. Leonard Jr, MD; Henry M. Blumberg, MD, FACP
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        Tuberculosis

        • JONATHAN B. PARR, MD, MPHClinical Instructor, Division of Global Health Equity, Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA
        • MICHAEL K. LEONARD JR, MDID Consultants, Charlotte, NC
        • HENRY M. BLUMBERG, MD, FACPProfessor of Medicine, Epidemiology, and Global Health, Division of Infectious Diseases, Emory University School of Medicine, Atlanta, GA

        Tuberculosis is a bacterial disease caused by Mycobacterium tuberculosis, a relatively slow-growing, aerobic, acid-fast bacillus. Classically, tuberculosis is a pulmonary disease, but disseminated and extrapulmonary disease may also occur, especially in immunocompromised persons. Tuberculosis is transmitted person to person and is usually contracted by inhalation of M. tuberculosis carried in droplets coughed out by an infectious person. After M. tuberculosis enters the body, the host’s cell-mediated immunity may contain the organism but not eradicate all the bacilli, resulting in latent tuberculosis infection (LTBI). M. tuberculosis can remain dormant and persist (e.g., within macrophages), and in such cases, persons are at risk for reactivation and development of active tuberculosis. This review discusses the epidemiology, genetics, pathogenesis, diagnosis, and treatment of tuberculosis in its various forms, as well as screening for latent tuberculosis. Figures illustrate the global incidence of tuberculosis, the numbers of cases in the United States from 1992 through 2011, the natural history of tuberculosis, atypical findings in HIV-infected patients, findings of vertebral tuberculosis, treatment completion rates for pulmonary tuberculosis, and treatment of drug-susceptible pulmonary tuberculosis. Tables show the criteria for a positive tuberculin skin test by risk group, recommended doses and adverse effects of antituberculosis medications for adults, treatment guidelines for drug-susceptible pulmonary tuberculosis in adults, evidence-based guidelines for duration of therapy for drug-susceptible extrapulmonary tuberculosis and adjunctive use of corticosteroids, potential regimens for the treatment of drug-resistant tuberculosis, and guidelines for the treatment of latent tuberculosis in adults.

        This review contains 7 highly rendered figures, 6 tables, and 144 references.

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      • 13

        Infections Due to Mycobacterium Leprae and Nontuberculous Mycobacteria

        By Carlos Franco-Paredes, MD, MPH; Michael K. Leonard Jr, MD; Henry M. Blumberg, MD, FACP
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        Infections Due to Mycobacterium Leprae and Nontuberculous Mycobacteria

        • CARLOS FRANCO-PAREDES, MD, MPHDirector, Clinical and Translational Research Training Programs
        • MICHAEL K. LEONARD JR, MDID Consultants, Charlotte, NC
        • HENRY M. BLUMBERG, MD, FACPProfessor of Medicine, Epidemiology, and Global Health, Division of Infectious Diseases, Emory University School of Medicine, Atlanta, GA

        Mycobacterium leprae infection (i.e., leprosy) is a disease that has been recognized—and often misunderstood—since ancient times. The emergence of HIV/AIDS and the development of newer culture methodologies and molecular diagnostic tools have brought about increased interest in the epidemiology, diagnosis, and treatment of human infections from nontuberculous mycobacteria (NTM). More than 140 species of NTM have been identified; approximately 50 of these may be pathogenic for humans, causing a broad spectrum of disease. This chapter covers both M. leprae and selected NTM organisms, including M. avium complex; M. kansasiiM. marinum; and rapidly growing mycobacteria such as M. chelonaeM. fortuitum, and M. abscessus. The section on leprosy encompasses subsections on diagnosis, clinical manifestations and classification, laboratory studies, treatment, and leprosy reactions. Treatments for nontuberculous mycobacteria infections are also covered. Figures include a natural history of leprosy, tuberculoid leprosy, lepromatous leprosy, and various forms of borderline leprosy, as well as type 1 and type 2 leprosy reaction. Tables include the Ridley-Jopling classification of leprosy, recommendations for treatment of leprosy, clinical characteristics and treatment of leprosy, major clinical syndromes associated with nontuberculous mycobacterial infections, diagnosing nontuberculous mycobacterial lung disease, a listing of slow and rapidly growing mycobacteria that are human pathogens, plus treatment regimens for selected nontuberculous mycobacterial infections in adults.

        This review contains 59 references.

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      • 14

        Mycotic Infections

        By Carol A. Kauffman, MD, FACP
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        Mycotic Infections

        • CAROL A. KAUFFMAN, MD, FACPChief, Infectious Diseases Section, Veterans Affairs Ann Arbor Healthcare System, Professor, Internal Medicine, University of Michigan Medical School, Ann Arbor, Michigan

        Endemic mycoses are caused by fungi that share several important characteristics but differ greatly in other respects. They include histoplasmosis, blastomycosis, coccidioidomycosis, and sporotrichosis. Discussions on their epidemiology, pathogenesis, clinical presentation, diagnosis, differential diagnosis, treatment, and prognosis are included. A section on antifungal therapy is included, discussing the use of azoles and amphotericin B. Figures show photomicrographs of biopsy samples from patients with histoplasmosis, blastomycosis, and coccidioidomycosis. Tables list selected drugs that may interact with azoles to change serum levels and information on the administration of amphotericin B for treatment of endemic mycoses.

        This review contains 3 highly rendered figures, 3 tables, and 71 references.

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      • 15

        Mycotic Infections in the Compromised Host

        By Jo-Anne H. Young, MD, FACP
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        Mycotic Infections in the Compromised Host

        • JO-ANNE H. YOUNG, MD, FACP

        Opportunistic fungal infections have become increasingly important over the past several decades, paradoxically because advances in medical practice have improved the survival of debilitated and immunosuppressed patients. The mycotic infections that appear in the compromised host are candidiasis, cryptococcosis, pneumocystosis, aspergillosis, zygomycosis, and fusariosis. The aspergillosis section addresses allergic bronchopulmonary aspergillosis and invasive aspergillosis separately. Timely diagnosis of opportunistic fungal infection depends on understanding host characteristics, environmental risk factors, clinical presentation, and diagnostic testing, which each section covers. There is also a section on infection by dematiaceous fungi. Figures illustrate the cysts of Pneumocystis and the bimodal distribution of Aspergillus infection after bone marrow transplantation. Tables describe the desensitization of adult patients with sulfa allergy; the stages of allergic bronchopulmonary aspergillosis; the treatment of infections caused by Candida, Cryptococcus, Aspergillus, and Fusarium species; and the treatment and prophylaxis of Pneumocystis jiroveci pneumonia. 

        This review contains 2 highly rendered figures, 7 tables, and 108 references.

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      • 16

        Protozoan Infections

        By Wesley C. Van Voorhis, MD, PhD, FACP
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        Protozoan Infections

        • WESLEY C. VAN VOORHIS, MD, PHD, FACPProfessor of Medicine and Adjunct Professor of Pathobiology, Head Allergy and Infections Diseases Division, Department of Medicine, University of Washington School of Medicine, Attending Physician, Infectious Diseases & Tropical Medicine Clinic, University of Washington Medical Center, Seattle, WA

        Protozoans cause a number of important infectious diseases. This chapter discusses malaria, babesiosis, toxoplasmosis, trichomoniasis, leishmaniasis, and trypanosomiasis; in addition, the chapter describes the intestinal protozoan infections caused by Giardia lamblia; Dientamoeba fragilis; Entamoeba histolytica; Blastocystis hominis; the coccidial organisms Cystoisospora belli, Cryptosporidium, and Cyclospora cayetanensis; Balantidium coli; and microsporidia. Figures illustrate the taxonomy of pathogenic protozoans; the life cycle of malaria; identification of species of malaria based on forms seen on blood smears; Babesia parasites in erythrocytes; the life cycle, tachyzoites, and tissue cysts ofToxoplasma gondii; G. lamblia trophozoites and cysts; a D. fragilis trophozoite; E. histolytica cyst and trophozoite forms; Entamoeba coli; clinical involvement in amebiasis; B. hominis; cysts of Cryptosporidium, Cyclospora, and Cystoisospora; Acanthamoeba polyphaga cyst and histopathologic features; histopathology of Naeglaria meningoencephalitis;Leishmania (Viannia) braziliensis cutaneous infection; amastigotes of Old World leishmaniasis; the insect vector for Chagas disease; Romaña sign in acute Chagas disease;Trypanosoma cruzi, and T. brucei. Tables list the differentiating features of Plasmodium species that cause malaria; dosages, principal side effects, and main limitations of antimalarial drugs; and forms of New World leishmaniasis. A sidebar provides Internet links on protozoan infection. 

        This review contains 22 highly rendered figures, 3 tables, and 121 references.

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      • 17

        Helminthic Infections

        By Wesley C. Van Voorhis, MD, PhD, FACP
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        Helminthic Infections

        • WESLEY C. VAN VOORHIS, MD, PHD, FACPProfessor of Medicine and Adjunct Professor of Pathobiology, Head Allergy and Infections Diseases Division, Department of Medicine, University of Washington School of Medicine, Attending Physician, Infectious Diseases & Tropical Medicine Clinic, University of Washington Medical Center, Seattle, WA

        Helminthic parasites are multicellular worms that possess differentiated organ systems. They (with a few exceptions) do not replicate in the human host, and they tend to elicit eosinophilia within the tissues and blood of infected humans. Helminthic parasites include nematodes (roundworms), cestodes (tapeworms), and trematodes (flukes). The major intestinal nematodes are roundworm, pinworm, hookworm, whipworm, and Strongyloides stercoralis. Trichinellosis is caused by five species of the nematode Trichinella and develops after ingestion of infected meat, usually pork or the meat of certain carnivores. Nematode infections of the major tissue are anisakiasis, visceral larva migrans,Angiostrongylus cantonensis infection, mammomonogamosis (syngamosis), gnathostomiasis, dracunculiasis, and filariasis. Trematode and cestode infections are also described in this chapter, including infections by fish, beef, and pork tapeworms, as well as cysticercosis. Disease from ParagonimusClonorchisFasciolaFasciolopsis, and Schistosoma is covered. Echinococcus infection and hydatid cyst disease are discussed. Tables describe intestinal nematode infection and treatment and filarial parasites of humans. Figures illustrate a variety of helminthic parasites and their life cycles.

        This review contains 136 references.

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    • Special Hosts and Exposures
      • 1

        Fever in Returning Travelers

        By Mary E. Wilson, MD; Lin H. Chen, MD
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        Fever in Returning Travelers

        • MARY E. WILSON, MDVisiting Professor of Epidemiology and Biostatistics, School of Medicine, University of California, San Francisco, CA; Adjunct Professor of Global Health and Population, Harvard T.H. Chan School of Public Health, Boston, MA
        • LIN H. CHEN, MDDirector, Travel Medicine Center, Mount Auburn Hospital, Cambridge, MA; Associate Professor of Medicine, Harvard Medical School, Boston, MA

        Fevers in individuals with recent tropical travel require rapid assessment because many of the potential causes are treatable and/or have important public health implications. History and clinical findings are essential to identify the likely causes of the fever and, thus, recognize those that require urgent intervention.This review outlines obtaining the travel history, incubation periods, undifferentiated fever, and evaluation based on syndromes. Figures show the most common diagnoses and range of incubation periods; the top specific diagnoses in febrile returned travelers by geographic region; countries/areas at risk for dengue transmission; images of Aedes aegypti and Aedes albopictus, vectors of dengue virus and chikungunya virus; countries and territories where chikungunya cases have been reported (as of March 10, 2015), excluding where imported cases only are documented; cases of Ebola virus disease in Africa, 1976–2015, by species and size; and an algorithm for approach to workup. Tables list Web sites and resources for travel medicine; the epidemiology of fever in travelers; key areas of exposure queries in returned travelers; obtaining exposure history in febrile returned travelers; estimated protective effect of previous vaccination; common posttravel infections, by incubation period; guiding principles for care of febrile patients returning from tropical travel; common clinical findings and associated infections; key cannot-miss diagnoses with fever; malaria antigens; fever syndromes, diagnoses, and diagnostic tests to be considered; and major hemorrhagic fevers and their distribution based on world regions.

        This review contains 7 highly rendered figures, 12 tables, and 151 references.

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      • 2

        Infections in Cancer Patients

        By Gowri Satyanarayana, MD; Sarah P. Hammond, MD
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        Infections in Cancer Patients

        • GOWRI SATYANARAYANA, MDAssistant Professor of Internal Medicine, Division of Infectious Diseases, Department of Medicine, Vanderbilt University, Nashville, TN
        • SARAH P. HAMMOND, MDAssistant Professor of Medicine, Harvard Medical School, Division of Infectious Diseases, Department of Medicine, Brigham and Women’s Hospital, Boston, MA

        Infection is a common complication in cancer patients who receive cytotoxic chemotherapy and other targeted therapies. A clear understanding of the underlying host risk factors, the immunologic effects of specific cancer therapies, and the anatomic location of the tumor and its impact on surrounding structures helps predict the types of infection to which the host is most vulnerable. This review covers fever in the setting of neutropenia, infection risk with biological and targeted cancer therapy, and infections in patients with solid tumors. Figures show risk for fever and neutropenia, and empirical antibiotic algorithm for initial management of febrile neutropenia in patients at high risk for complications, radiographic findings associated with invasive fungal disease, a schematic of hepatitis B virus infection and reactivation after anti-CD20 therapy, and management of nonpurulent cellulitis in cancer patients. Tables list the Multinational Association of Supportive Care in Cancer (MASCC) risk index score, the Clinical Index of Stable Febrile Neutropenia (CISNE) scoring system, common bacterial pathogens that cause infection in neutropenic patients, assessment of specific signs and symptoms during febrile neutropenia, empirical antibiotic management of febrile neutropenia, criteria for addition of vancomycin to empirical antibiotic regimen for febrile neutropenia, and biological and targeted agents commonly used to treat hematologic malignancy and associated infections.

         

        This review contains 5 highly rendered figures, 7 tables, and 107 references

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  • Interdisciplinary Medicine
    • 1

      Adult Preventive Health Care

      By Jennifer S. Haas, MD, MSc
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      Adult Preventive Health Care

      • JENNIFER S. HAAS, MD, MSCProfessor of Medicine, Department of Medicine, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA

      Over the past quarter century, prevention has become a major activity in primary care. During a typical day, primary care clinicians spend much of their time managing asymptomatic conditions in which the main goal is to prevent death or complications (e.g., hypertension, hyperlipidemia, osteoporosis). This chapter focuses primarily on preventive screening recommendations from the United States Preventive Services Task Force (USPSTF). The rationale and evolution of preventive care guidelines are discussed. Advantages and disadvantages of cervical, colorectal, breast, prostate, ovarian and lung cancer screening are explained, along with recommendations regarding behavioral counseling, especially for smoking cessation and alcohol use. Graphs are included. Tables delineate major causes of death in the United States, criteria for evaluating a screening program, sample board examination questions about screening, government-sponsored preventive guidelines programs, the USPSTF grading system, strongly recommended noncancer preventive services in adults, the recommended adult immunization schedule, recommended and strongly recommended measures for cancer prevention, recommended preventive noncancer screening measures, and selected recommendations for counseling and patient education.

      This review contains 5 figures, 10 tables, and 77 references.

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    • 2

      Diet

      By Elizabeth G. Nabel, MD, FACP
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      Diet

      • ELIZABETH G. NABEL, MD, FACPPresident, Brigham and Women’s Hospital, Professor of Medicine, Harvard Medical School, Boston, MA

      An unhealthy diet is a major risk factor for chronic diseases such as cardiovascular diseases, cancer, diabetes, and conditions related to obesity. In the 20th century, the average American diet shifted from one based on fresh, minimally processed vegetable foods to one based on animal products and highly refined, processed foods, leading to an increased consumption of calories, fat, cholesterol, refined sugar, animal protein, sodium, and alcohol and far less fiber and starch than was healthful. As a result, more than one third of US adults are obese, with an estimated medical cost of $147 billion. Physicians have an important role in educating patients about healthful nutrition and in providing dietary guidelines. This module discusses the role of energy in weight loss; the structure of fat and cholesterol, their effects on blood lipid levels and cardiovascular risk, and related dietary recommendations; carbohydrates; dietary fiber; proteins; vitamin and mineral consumption; water and food consumption; and the relationship between diet and health. Tables review the principles of a healthy diet; recommended daily intake of fat and other nutrients; types of dietary fiber and representative food sources; types of vitamins; essential minerals and trace elements; and dietary guidelines for healthy people. Figures include a graph showing the percentage of adults who are healthy weight, overweight, and obese and the structure of fat and cholesterol.

      This review contains 2 highly rendered figures, 6 tables, and 37 references.

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    • 3

      Exercise

      By Elizabeth G. Nabel, MD, FACP
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      Exercise

      • ELIZABETH G. NABEL, MD, FACPPresident, Brigham and Women’s Hospital, Professor of Medicine, Harvard Medical School, Boston, MA

      Numerous observational studies have demonstrated an inverse relationship between physical activity and risk of many chronic illnesses. The protective effect of exercise is strongest against coronary artery disease, hypertension, stroke, type 2 diabetes mellitus, obesity, anxiety, depression, osteoporosis, and cancers of the colon and breast. Despite these proven benefits, only 25% of adults in the United States exercise at recommended levels. Globally, physical inactivity is the fourth leading risk factor for death, followed by overweight and obesity. This module describes exercise physiology, including cardiovascular response to dynamic exercise, pulmonary response, musculoskeletal response, metabolic effects, effects on blood lipid levels, hematologic effects, effects on vascular inflammation, effects on body fluids, and psychological effects. Exercise and the elderly and the relationship between exercise and longevity are reviewed. Prescribing exercise and complications of exercise are also discussed. Tables describe the categories of patients screened for possible coronary artery disease, exercise time required to consume 2,000 kcal, and exercise advice for patients. Figures include a graph showing the number of adults who met the federal physical activity guidelines criteria, the top 10 global risk factors for death in 2004, the process of providing energy for the muscle, and trends in physician prescriptions for exercise.

      This module contains 4 highly rendered figures, 3 tables, 35 references, and 5 MCQs.

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    • 4

      Reducing the Risk of Injury and Disease

      By Harold C. Sox, MD, MACP
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      Reducing the Risk of Injury and Disease

      • HAROLD C. SOX, MD, MACP

      Public interest in disease and injury prevention is very high, driven by a steady accumulation of high-quality evidence that preventive interventions do reduce cause-specific death rates. The purpose of these interventions is to eliminate the root causes of diseases that precede death (e.g., heart disease, cancer, and stroke). This chapter presents a review of health risks posed by substance abuse (tobacco, alcohol, and drugs), accidents (e.g., from motor vehicles, accidental poisoning, falling, fire, drowning, and firearms), and domestic violence. The physician’s role in prevention is to identify risk factors for disease and injury and counsel patients about modifying potentially harmful behaviors. Figures illustrate the life expectancy of men and women, and years of potential life lost from various causes, in the United States. Tables list recommendations of the United States Preventive Services Task Force (USPSTF), years of smoking abstinence needed to reduce the risk of disease, elements of a successful smoking cessation strategy, stages of readiness for smoking cessation, the test performance of screening questionnaires for alcohol abuse, the CAGE questionnaire, unintentional and undetermined poisoning deaths in 11 states, and risk factors for falls among the elderly. A sidebar provides links to domestic violence information on the Internet.

      This review contains 3 highly rendered figures, 9 tables, and 87 references.

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    • 5

      Complementary, Alternative, and Integrative Medicine

      By Helene M. Langevin, MD
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      Complementary, Alternative, and Integrative Medicine

      • HELENE M. LANGEVIN, MDDirector, Osher Center for Integrative Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA

      Complementary and alternative medicine (CAM) refers to a group of diverse medical and health care systems, practices, and products that are not considered to be part of conventional or allopathic medicine. Common CAM practices (e.g., acupuncture, meditation, and therapeutic massage) are gradually becoming incorporated into conventional care in response to patients looking to alternative sources for information and advice about health matters and increased understanding of various CAM methods through evidence-based testing. However, although the claims of some methods are supported with academic research, well-founded concerns remain in many popularized CAM practices regarding the lack of evidence and placebo effects. It is thus imperative for physicians to be comfortable in discussing CAM-related topics with patients and be able to appropriately and informatively guide them in a way that harnesses potential benefits and avoids potential harm. In this review, the major CAM therapies in the United States are examined, including the settings in which they are being used, evidence base status, and efficacy of some of the most commonly used modalities. Figures show percentages of use for various CAM approaches. Tables show major CAM health practices, CAM therapies with significant increases between 2002 and 2007, Internet sources for evidence-based information and tools for patient education, sources for health care provider information, and resources to assess dietary supplement–drug interactions.

      This review contains 5 highly rendered figures, 5 tables, and 155 references.

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    • 6

      Occupational Medicine

      By Tee L. Guidotti, MD, MPH, FACP, DABT, QEP (Air Quality), FRCPC, FCBOM, FFOM
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      Occupational Medicine

      • TEE L. GUIDOTTI, MD, MPH, FACP, DABT, QEP (AIR QUALITY), FRCPC, FCBOM, FFOMVice President for Health/Safety/Environment & Sustainability, Medical Advisory Services, Rockville, Maryland

      Occupational medicine concerns keeping workers healthy, screening for early signs of health problems, and promoting good health through workplace initiatives. According to recent estimates, 55,000 deaths and 3.8 million disabling injuries per year result from occupational illness at a cost of $125 to $155 billion. This chapter covers the basic principles and clinical evaluation of occupational disease, guidelines for taking a patient’s occupational history, and exposure assessment. Major occupational disorders in developed countries are discussed, including respiratory tract disorders, skin disorders, disorders of the kidneys, liver disease, central and peripheral nervous systems, organs of sensation, occupational cancer, musculoskeletal disorders, hematologic disorders, endocrine and reproductive effects, stress and psychogenic effects, and nonspecific illness. Tables outline critical dimensions of occupational medicine, the conceptual approach to a patient with a suspected workplace injury or illness, common hazards with widely available tests for exposure, historically common occupational disorders, and established occupational carcinogens. Figures includes a chest film of asbestosis, a sample occupational and environmental history questionnaire, lungs in various states of pneumoconiosis, a photograph of contact dermatitis, and a graph showing hearing decline at 4,000 Hz frequency. Illustrations demonstrate the impact of occupational exposure on the kidneys and liver and the anatomy of the hearing mechanism.

      This review contains 9 highly rendered figures, 5 tables, and 112 references.

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    • 7

      Medical Evaluation of the Surgical Patient

      By Marie Gerhard-Herman, MD; Jonathan Gates, MD
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      Medical Evaluation of the Surgical Patient

      • MARIE GERHARD-HERMAN, MDDepartment of Medicine, Cardiovascular Division, Brigham and Womens Hospital, Boston, MA
      • JONATHAN GATES, MDDirector of the Burn and Trauma Unit, Department of Surgery, Brigham and Womens Hospital, Boston, MA

      Medical evaluation prior to surgery includes risk assessment and the institution of therapies to decrease perioperative morbidity and mortality to improve patient outcomes. The most effective medical consultation for surgical patients begins with an assessment of the individual patient and knowledge of the planned surgery and anesthesia followed by clear communication of a concise and specific recommended plan of perioperative care to the surgical team. This chapter describes anesthetic, cardiac, pulmonary, hepatic, nutritional, and endocrine risk assessment. Perioperative thrombotic management and postoperative care and complications, including fluid management; pulmonary, cardiac, renal complications; and delirium are discussed. Tables outline the American Society of Anesthesiologists class and perioperative mortality risk, a comparison of the Revised Cardiac Risk Index and National Surgery Quality Improvement Program, Duke Activity Status Index, high-risk stress test findings, markers for increased perioperative risk in pulmonary hypertension, aortic stenosis and nonemergent noncardiac surgery, risk factors for pulmonary complications in noncardiac surgery, the Model for End-Stage Liver Disease score to predict postoperative mortality, venous thromboembolism risk factors and options for pharmacologic prophylactic regimens, perioperative management of warfarin, and Brigham and Women’s Hospital guidelines for postoperative blood product replacement. Figures include a care algorithm for noncardiac surgery, an illustration of types of myocardial infarction, and an algorithm for the treatment of postoperative delirium.

      This review contains 3 highly rendered figures, 12 tables, and 68 references.

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    • 8

      Bioterrorism

      By Jeffrey Duchin, MD; John D. Malone, MD, MPH
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      Bioterrorism

      • JEFFREY DUCHIN, MD
      • JOHN D. MALONE, MD, MPHAdjunct Professor of Medicine, Center for Disaster and Humanitarian Assistance Medicine, Uniformed Services University of the Health Sciences, Bethesda, MD; National Naval Medical Center, Bethesda, MD

      Well before the 2001 anthrax outbreak, public health and government leaders in the United States recognized the need for increased preparedness to detect and respond to acts of biologic terrorism. In April 2000, the Centers for Disease Control and Prevention (CDC) published a strategic plan for preparedness and response to biologic and chemical terrorism. This chapter describes the clinician's role in recognizing potential bioterrorism agents and responding accordingly, as presented in the CDC plan. It also reviews data analyses arising from the United States military and civilian smallpox vaccination programs of 2002 to 2004. The critical biologic agent categories for public health preparedness are presented in a table. Current information is provided on the diagnosis and management of the following agents of bioterrorism: smallpox, anthrax, plague, botulism, tularemia, and hemorrhagic fever viruses. A sidebar provides Internet resources for authoritative information and guidelines related to bioterrorism. This chapter contains 169 references.

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    • 9

      The Future of Transplant Biology and Surgery

      By Marc Colaco, MD, MBA; Anthony Atala, MD
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      The Future of Transplant Biology and Surgery

      • MARC COLACO, MD, MBAResident, Department of Urology, Wake Forest School of Medicine, Winston-Salem, NC
      • ANTHONY ATALA, MDDirector, Wake Forest Institute for Regenerative Medicine, and W.H. Boyce Professor and Chair, Department of Urology, Wake Forest University, Winston-Salem, NC

      Although organ transplantation remains the mainstay of treatment for patients with severely compromised organ function, with the growing number of patients in need of treatment and the lack of organ supply, medical scientists have begun seeking out alternatives. In the last two decades, researchers have attempted to grow native and stem cells, engineer tissues, and design treatment modalities using regenerative medicine techniques for almost every tissue of the human body. This chapter discusses the basics of tissue engineering, including cell isolation and biomaterial selection. It then outlines specific advances and potential surgical uses.

      This review contains 9 figures, 2 tables, and 135 references.

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    • 10

      Management of Poisoning and Drug Overdose

      By Timothy J. Wiegand, MD; Manish M. Patel, MD; Kent R. Olson, MD, FACEP, FAACT, FACMT
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      Management of Poisoning and Drug Overdose

      • TIMOTHY J. WIEGAND, MD
      • MANISH M. PATEL, MD
      • KENT R. OLSON, MD, FACEP, FAACT, FACMT

      Drug overdose and poisoning are leading causes of emergency department visits and hospital admissions in the United States, accounting for more than 500,000 emergency department visits and 11,000 deaths each year. This chapter discusses the approach to the patient with poisoning or drug overdose, beginning with the initial stabilization period in which the physician proceeds through the ABCDs (airway, breathing, circulation, dextrose, decontamination) of stabilization. The management of some of the more common complications of poisoning and drug overdose are summarized and include coma, hypotension and cardiac dysrhythmias, hypertension, seizures, hyperthermia, hypothermia, and rhabdomyolysis. The physician should also perform a careful diagnostic evaluation that includes a directed history, physical examination, and the appropriate laboratory tests. The next step is to prevent further absorption of the drug or poison by decontaminating the skin or gastrointestinal tract and, possibly, by administering antidotes and performing other measures that enhance elimination of the drug from the body. The diagnosis and treatment of overdoses of a number of specific drugs and poisons that a physician may encounter, as well as food poisoning and smoke inhalation, are discussed. Tables present the ABCDs of initial stabilization of the poisoned patient; mechanisms of drug-induced hypotension; causes of cardiac disturbances; drug-induced seizures; drug-induced hyperthermia; autonomic syndromes induced by drugs or poison; the use of the clinical laboratory in the initial diagnosis of poisoning; methods of gastrointestinal decontamination; methods of and indications for enhanced drug removal; toxicity of common beta blockers; common stimulant drugs; corrosive agents; dosing of digoxin-specific antibodies; poisoning with ethylene glycol or methanol; manifestations of excessive acetylcholine activity; common tricyclic and other antidepressants; seafood poisonings; drugs or classes that require activated charcoal treatment; and special circumstances for use of activated charcoal.

      This review contains 3 highly rendered figures, 17 tables, and 193 references.

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    • 11

      Bites and Stings

      By Lawrence M. Lewis, MD; William H. Dribben, MD; Mark D. Levine, MD
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      Bites and Stings

      • LAWRENCE M. LEWIS, MDAssociate Professor of Emergency Medicine, Washington University in St. Louis and Barnes-Jewish Hospital, St. Louis, MO
      • WILLIAM H. DRIBBEN, MDAssociate Professor of Emergency Medicine, Washington University in St. Louis and Barnes-Jewish Hospital, St. Louis, MO
      • MARK D. LEVINE, MDAssociate Professor of Emergency Medicine, Washington University in St. Louis and Barnes-Jewish Hospital, St. Louis, MO

      This chapter reviews the diagnosis and treatment of the following kinds of bites and stings: mammalian bites (human and domestic and nondomestic animal), snake bites (e.g., coral snake, pit viper), spider bites (e.g., black widow, brown recluse, tarantula), scorpion stings, insect bites and stings (e.g., fire ant, kissing bug, bedbug, caterpillar, moth, blister beetle, tick), and marine envenomations (toxic vertebrates and invertebrates). Photographs show a closed fist bite wound, a coral snake, a diamondback rattlesnake, a copperhead, a black widow spider, and a brown recluse spider. Tables cover the following topics: common and important pathogens and antibiotic selection for various mammalian-bite wounds; a US rabies postexposure prophylaxis guide; postexposure prophylaxis for hepatitis B after human bites; HIV exposures and postexposure prophylaxis recommendations; bite wounds that require prophylactic antibiotics; tetanus prophylaxis in routine wound management; a rabies postexposure prophylaxis schedule; recommendations for tetanus prophylaxis after animal bites; a grading system for severity of Centruroides sculpturatusenvenomation; risk factors correlated with poor outcome; and pathogens associated with aquatic infections.

      This review contains 7 highly rendered figures, 10 tables, and 264 references.

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    • 12

      Toxic Gases

      By Stephanie T. Weiss, MD, PhD; Kathryn W. Weibrecht, MD
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      Toxic Gases

      • STEPHANIE T. WEISS, MD, PHDAssociate Director, Emergency Medicine, Morton Hospital and Medical Center, Taunton, MA
      • KATHRYN W. WEIBRECHT, MDAssociate Director, Emergency Medicine, Morton Hospital, Taunton, MA

      This review looks at the potential causes, diagnoses, and possible treatments for three asphyxiant gases: carbon monoxide, hydrogen cyanide, and hydrogen sulfide. Exposure to these gases can lead to central nervous system depression, unconsciousness, and death due to tissue hypoxia. These gases are among the most common causes of fatalities related to toxic gas poisoning, with carbon monoxide responsible for 36% and hydrogen sulfide 7.7%. It is necessary to remove victims affected by poisoning immediately from the source of the toxic gas, administer oxygen, and assess their stability. As symptoms of these gases can differ widely, ranging from broad and unspecific to highly morbid, and may require different levels of care, the correct diagnosis should also rely on inferences from the patient history and the context of the admission, including evidence of fire and chemical reactions. Normobaric oxygen and hyperbaric oxygen are the two main treatments for carbon monoxide, although studies have been inconclusive in regards to the effectiveness of hyperbaric oxygen. The Cyanokit (containing hydroxocobalamin) is considered to be more effective for hydrogen cyanide when compared with the Cyanide Antidote Kit due to the former’s low toxicity and high effectiveness. Hydrogen sulfide is often used as a suicide agent, the mortality of which is close to 100%. Figures show the mechanisms by which the asphyxiant gases carry out their negative effects on the human body. Tables show the half-life of carboxyhemoglobin with oxygen therapy and a comparison between the Cyanide Antidote Kit and the Cyanokit. 

      This review contains 3 highly rendered figures, 2 tables, and 43 references. 


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    • 13

      Toxic Plants and Mushrooms

      By Marie King, MD, PhD; Richard Church, MD
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      Toxic Plants and Mushrooms

      • MARIE KING, MD, PHDEmergency Physician, Harrington Hospital, Southbridge, MA
      • RICHARD CHURCH, MDAssistant Professor, Emergency Medicine and Toxicology, University of Massachusetts, Worcester, MA

      Many species of plants and mushrooms exist that, when consumed, can induce poisoning in individuals, causing a range of side effects. As the toxins do not always correspond to an antidote, it is important to have the ability to identify each harmful species to determine the appropriate treatment. This review gives an overview of some of the more prevalent toxic plants and mushrooms, detailing their principles of toxicity, recommendations for immediate stabilization, keys to proper diagnosis and definitive therapy, and patient disposition and outcomes. Figures show photographs of the various toxic plants and mushrooms featured. Tables show a list of toxic species for both plants and mushrooms, including their common names, the toxins contained within, their effects, and the corresponding antidote (if any).

      This review contains 16 highly rendered figures, 2 tables, and 44 references.

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    • 14

      Anticholinergic Toxicity

      By Jeffrey T. Lai, MD; Kavita M. Babu, MD
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      Anticholinergic Toxicity

      • JEFFREY T. LAI, MDResident Physician, Department of Emergency Medicine, University of Massachusetts Medical School, Worcester, MA
      • KAVITA M. BABU, MDAssociate Professor, Division of Medical Toxicology, Department of Emergency Medicine, University of Massachusetts Medical School, Worcester, MA

      Anticholinergic compounds oppose the action of the endogenous neurotransmitter acetylcholine at its target receptors and are found in over-the-counter and prescription medication, natural products, and plants. Anticholinergic medications, such as atropine and scopolamine, are used for the treatment of a wide range of conditions, including bradycardia, motion sickness, and insomnia. Antihistaminergic medications, such as diphenhydramine, also possess anticholinergic activity and are used in the treatment of seasonal allergies, common cold symptoms, and allergic reactions. Other medications, such as antidepressants (especially the older tricyclic class), antipsychotics, muscle relaxants, and anticonvulsants, can act as anticholinergic agents or produce anticholinergic side effects. Toxicity can result from therapeutic misadventure, intentional overdose, recreational use, and pediatric exposures. This review covers the principles of toxicity, immediate stabilization, diagnosis and definitive therapy, and disposition and outcomes. Figures show the anticholinergic toxidrome, look-alike structures, and electrocardiographic changes in tricyclic antidepressant overdose. Tables list medications with anticholinergic activity and selected botanicals that cause anticholinergic toxicity.

      Key words: anticholinergic overdose, anticholinergic toxicity, anticholinergic toxidrome, physostigmine, tricyclic antidepressant toxicity

      This review contains 3 highly rendered figures, 2 tables, and 49 references.

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    • 15

      Cholinergic Toxicity

      By Steven B. Bird, MD, FACEP, FACMT
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      Cholinergic Toxicity

      • STEVEN B. BIRD, MD, FACEP, FACMTAssociate Professor of Emergency Medicine, Division of Medical Toxicology, University of Massachusetts Medical School, Worcester, MA

      Cholinergic drugs exert their functions by inhibiting acetylcholinesterase, the enzyme responsible for hydrolyzing acetylcholine and ending neuronal or neuromuscular neurotransmission. These compounds are used in clinical medicine to treat various disorders, as pesticides, and as weapons of mass destruction. This review describes the drugs that affect the cholinergic system and discusses stabilization, diagnosis and definitive therapy, principles and controversies of definitive care, and disposition and outcomes for these agents. Figures show acetylcholinesterase hydrolysis of acetylcholine, neurotransmission in the nervous system, the mechanism of inhibition of acetylcholinesterase by an organophosphorus (OP) compound, and the general chemical structure of thion OP and oxon OP agents and carbamates. Tables list OP pesticides associated with OP-induced delayed neuropathy, the effects of cholinesterase inhibition, the symptoms of cholinergic poisoning according to severity, and a treatment algorithm for acetylcholinesterase poisoning.

      This review contains 4 highly rendered figures, 4 tables, and 85 references.

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    • 16

      Caustics

      By Lynn A. Farrugia, MD; Kavita Babu, MD, FACEP, FACMT
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      Caustics

      • LYNN A. FARRUGIA, MDEmergency Medicine Residency Program, University of Massachusetts Medical School, Worcester, MA
      • KAVITA BABU, MD, FACEP, FACMTFellowship Director, Division of Medical Toxicology, Associate Professor, Department of Emergency Medi-cine, University of Massachusetts Medical School, Worcester, MA

      A caustic is any substance capable of causing full-thickness damage on contact with healthy, intact tissue. Caustic agents are generally classified by pH as acids or bases. Irritants are those substances that do not produce true breakdown of tissue but cause discomfort and inflammation, such as vomiting, burning eyes, or coughing. This review covers caustic and irritant agents, dermal caustic exposure, caustic inhalation and pulmonary irritants, caustic ingestion, and ocular caustic exposure, along with special consideration of hydrofluoric acid, including hydrofluoric acid and dermal exposure, hydrofluoric acid ingestion, hydrofluoric acid inhalation, ocular hydrofluoric acid exposure, and systematic hydrofluoric acid toxicity. Figures show classification of burns; chemical burns; an autopsy specimen of the tongue, epiglottis, and esophagus after caustic ingestion; and an autopsy specimen of the stomach after caustic ingestion. Tables list common caustic and irritant agents, household products containing caustic and irritant agents, agents for which water or saline irrigation is not recommended, indications for endoscopy after caustic ingestion, ocular chemical burn management, and common chemicals and products containing fluoride.

      Key words: caustic eye injury, caustic ingestion, caustic injury, chemical burn, hydrofluoric acid, pulmonary irritants 

      This review contains 5 highly rendered figures, 6 tables, and 53 references.

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    • 17

      Drugs of Abuse

      By Matthew D. Zuckerman , MD; Kavita Babu, MD, FACEP, FACMT
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      Drugs of Abuse

      • MATTHEW D. ZUCKERMAN , MDAssistant Professor, Department of Emergency Medicine, Medical Toxicology, University of Colorado Anschutz Medical Campus, Aurora, CO
      • KAVITA BABU, MD, FACEP, FACMTFellowship Director, Division of Medical Toxicology, Associate Professor, Department of Emergency Medi-cine, University of Massachusetts Medical School, Worcester, MA

      The term “drugs of abuse” lacks a formal medical definition. Historically, discussions of drugs of abuse focused on “street drugs”; however, the adverse effects of the nonmedical use of prescription medications, such as opiates, benzodiazepines, and therapeutic amphetamines, are increasingly seen. The purpose of this review is to aid the clinician in identifying and treating a broad representation of drugs of abuse, which may include those illicitly produced in laboratories (e.g., methamphetamine), diverted pharmaceuticals (oxycodone), and herbal products (marijuana). This review covers stimulants, hallucinogens, cannabinoids, and sedative-hypnotics. Figures show substances ranked according to weighted harm score on a normalized scale from 0 being no harm to 100 being extreme harm to self and others, a treatment algorithm for sympathomimetic toxicity, a treatment algorithm for sedative-hypnotic overdose, and a treatment algorithm for opioid overdose. Tables list commonly abused sympathomimetic agents, modern novel drugs of abuse, commonly abused sedative-hypnotic agents, commonly abused opiates, and pitfalls of the drug screen.

       

      This review contains 4 highly rendered figures, 5 tables, and 89 references

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    • 18

      Psychoactive Medications

      By Mark J. Neavyn, MD; Kavita Babu, MD, FACEP, FACMT
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      Psychoactive Medications

      • MARK J. NEAVYN, MDDirector of Medical Toxicology, Department of Emergency Medicine, Hartford Hospital, Hartford, CT
      • KAVITA BABU, MD, FACEP, FACMTFellowship Director, Division of Medical Toxicology, Associate Professor, Department of Emergency Medi-cine, University of Massachusetts Medical School, Worcester, MA

      Psychoactive medications are defined as medications that affect the central nervous system neurotransmitter pathways with the intention to modulate mood or consciousness. This broad category of medications includes sedative-hypnotic agents such as benzodiazepines and barbiturates, antidepressants, neuroleptics, and mood stabilizers. The principal source of exposure for these medications is through prescription drug use and misuse. This review discusses the principles of toxicity, immediate stabilization, diagnosis and definitive therapy, and disposition and outcomes related to sedative-hypnotics, antidepressants, neuroleptics, and lithium. Tables include common benzodiazepine and barbiturate compounds, dosing instructions for multidose activated charcoal, flumazenil dosing recommendations, commonly available tricyclic and atypical (noncyclic) antidepressants, dosing recommendations for sodium bicarbonate in serum alkalinization, benzodiazepine dosing recommendations in serotonin syndrome, dosing recommendations for cyproheptadine, signs and symptoms that differentiate  neuroleptic malignant syndrome from serotonin syndrome, and indications for renal replacement therapy based on lithium concentration and clinical setting. Figures show action potentials in the His-Purkinje syndrome, an electrocardiogram tracing demonstrating a terminal R wave, and a QT interval nomogram.

      This review contains 2 highly rendered figures, 9 tables, and 101 references.

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  • Nephrology
    • 1

      Approach to the Patient With Kidney Disease

      By Ajay K. Singh, MBBS, FRCP (UK), MBA; Jameela A. Kari, MD, FRCP (UK), FRCPCH
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      Approach to the Patient With Kidney Disease

      • AJAY K. SINGH, MBBS, FRCP (UK), MBAAssociate Professor of Medicine, Associate Dean for Global Education and Continuing Education, Harvard Medical School, Director, Continuing Medical Education, Department of Medicine and Renal Division, Brigham and Women’s Hospital, Boston, MA
      • JAMEELA A. KARI, MD, FRCP (UK), FRCPCHProfessor of Pediatrics and Consultant Pediatric Nephrologist, King Abdulaziz University, Jeddah, Saudi Arabia

      Diseases of the kidney can present with a variety of different clinical presentations. These presentations are best considered syndromically: prerenal, renal, and postrenal. Besides this commonly used framework, kidney disease can have the kidney as the prime mover, such as with a primary glomerular disease, versus the kidney as a target of a systemic process, for example, as in the hepatorenal syndrome, where the primary abnormality physiologically is with the liver. Therefore, the most important aspect when approaching patients with kidney disease is to use a systematic framework. This framework applies regardless of whether kidney disease occurs in adults or children. This review covers determination of disease duration, assessment of kidney function, measurement of kidney function, staging of kidney disease, imaging techniques for the genitourinary system, major syndromes of kidney disease, and asymptomatic urinary sediment abnormalities. Figures show an approach to the patient with renal disease, components of urinary sediment, and an approach to the patient with hematuria. Tables list considerations for referral to nephrology, the National Kidney Foundation classification of chronic kidney disease, categories of proteinuria, correlation between dipstick positivity for protein and protein concentration in urine, causes of hematuria, and causes of hematuria by age and gender.

       

      This review contains 3 highly rendered figures, 6 tables, and 26 references.

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    • 2

      Disorders of Water and Sodium Balance

      By Richard H. Sterns, MD, FACP
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      Disorders of Water and Sodium Balance

      • RICHARD H. STERNS, MD, FACPProfessor of Medicine, University of Rochester School of Medicine and Dentistry, Chief of Medicine, Rochester General Hospital and the Genesee Hospital, Rochester, NY

      Water accounts for approximately half of an adult human’s body weight. Two thirds of body water is intracellular, and the remaining one third is contained in the extracellular fluid compartment, which includes intravascular (plasma) and interstitial fluid. Small amounts of water are also contained in bone, dense connective tissue, digestive secretions, and cerebrospinal fluid. To maintain the stability of the internal milieu, body fluids are processed by the kidney, guided by intricate physiologic control systems that regulate fluid volume and composition. This chapter reviews the regulation of body fluid volumes, cell volume regulation in hypotonicity and hypertonicity, disorders of water excess (hyponatremia), water deficiency (hypernatremia), water conservation (diabetes insipidus), saltwater excess (edematous states), and saltwater deficiency (volume depletion). Tables describe control of body fluid volumes, causes of nonhypotonic hyponatremia, causes and treatment of acute hyponatremia, causes of the syndrome of inappropriate release of antidiuretic hormone (SIADH), and causes of hypernatremia. Figures illustrate sodium reabsorption by the renal tubules, the relationship between plasma vasopressin levels, renal sodium handling, and dose-response curves for a loop diuretic in patients with normal and reduced renal function.

      This chapter contains 4 highly rendered figures, 5 tables, 88 references, and 5 MCQs.

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    • 3

      Disorders of Acid-base and Potassium Balance

      By Stuart L. Linas, MD, FACP
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      Disorders of Acid-base and Potassium Balance

      • STUART L. LINAS, MD, FACP

      Water accounts for approximately half of an adult human's body weight. Two thirds of body water is intracellular, and the remaining one third is contained in the extracellular fluid compartment, which includes intravascular (plasma) and interstitial fluid. Small amounts of water are also contained in bone, dense connective tissue, digestive secretions, and cerebrospinal fluid. To maintain stability of the internal milieu, body fluids are processed by the kidney, guided by intricate physiologic control systems that regulate fluid volume and composition. This chapter reviews the principles of body fluid hemostasis and discusses disorders of water excess (hyponatremia), water deficiency (hypernatremia), water conservation (diabetes insipidus), saltwater excess (edematous states), and saltwater deficiency (volume depletion). Figures illustrate the relation between the plasma hydrogen ion concentration and the blood pH, proximal tubular bicarbonate reabsorption, renal secretion of hydrogen ions, renal glutamine metabolism and ammonia diffusion, metabolic alkalosis, and the electrocardiographic changes in and approach to causes of hypokalemia and hyperkalemia. Tables describe causes of metabolic acidosis with a high and a normal ion gap; causes of type 1 renal tubular acidosis; causes of type 4 renal tubular acidosis and aldosterone resistance; causes of metabolic alkalosis, respiratory acidosis, and respiratory alkalosis; evaluation of the renal defect in hyperkalemia; and treatment of hyperkalemia. This chapter contains 50 references.

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    • 4

      Glomerular Diseases

      By Ramon G.B. Bonegio, MD, PhD; David J. Salant, MD
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      Glomerular Diseases

      • RAMON G.B. BONEGIO, MD, PHDAssistant Professor of Medicine, Renal Section, Boston University School of Medicine, Boston, MA
      • DAVID J. SALANT, MDProfessor of Medicine, Boston University School of Medicine, Chief of Nephrology Section, Boston Medical Center, Boston, MA

      Some disease processes that affect the glomerulus are acute and self-limited, whereas others lead to progressive loss of renal function. Glomerular diseases cause more than half the cases of chronic kidney disease. This chapter first briefly reviews normal glomerular structure and function, clinical classifications, terminology, diagnosis, and epidemiology of glomerular disease. Pathogenesis of glomerular injury is discussed, and then more detailed approaches to diagnosis. General principles in the management of glomerular diseases are presented. Specific glomerular diseases and their management, discussed at length, are divided into nephritic, nephrotic, and nephritic-nephrotic syndromes. Figures illustrate the pathogenesis and findings on light microscopy, electron microscopy, and immunostaining of a variety of glomerular diseases, including poststreptococcal glomerulonephritis, IgA nephropathy, anti–glomerular basement membrane disease, focal and segmental glomerulosclerosis (FSGS), membranous nephropathy, and membranoproliferative glomerulonephritis (MPGN). Also illustrated are treatment algorithms for FSGS and minimal change disease. Tables list the clinical presentation and common causes of glomerular disease, diseases associated with mesangial IgA deposition, causes of FSGS, conditions associated with membranous nephropathy, diseases associated with minimal change lesions, causes of dense deposit disease, and causes of MPGN types I to III. This chapter contains 140 references.

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    • 5

      Tubulointerstitial Diseases

      By Gerald B. Appel, MD, FACP, FASN; Premila Bhat, MD; Pietro Canetta, MD
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      Tubulointerstitial Diseases

      • GERALD B. APPEL, MD, FACP, FASNProfessor of Clinical Medicine, Division of Nephrology, Columbia University Medical Center, New York, NY
      • PREMILA BHAT, MDAtlantic Dialysis Management Services, L.L.C
      • PIETRO CANETTA, MDClinical Fellow, Division of Nephrology, Columbia University Medical Center, New York, NY

      Tubulointerstitial diseases predominantly involve the tubules and the interstitium (the space between the tubules). They may be acute or chronic. Drug-induced acute interstitial nephritis (AIN) can be caused by antibiotics or nonsteroidal anti-inflammatory drugs. Drug-induced chronic interstitial nephritis can be caused by analgesic agents, lithium, antineoplastic agents, and cyclosporine. Physical factors causing interstitial damage are obstructive nephropathy, reflux nephropathy, and radiation nephritis. Discussions of infectious tubulointerstitial nephritis, metabolic and toxic tubulointerstitial nephritis, interstitial disease associated with vascular damage, tubulointerstitial disease caused by dysproteinemias and other tumors, and cystic disease of the kidney are also included. There are 13 figures, including micrographs, CT scans, pyelograms, and photographs of gross specimens, that illustrate the clinical findings of these diseases. Tables list the etiologies of tubulointerstitial diseases and drugs associated with AIN. This chapter has 161 references.

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    • 6

      Acute Kidney Injury

      By Anupam Agarwal, MD, FASN; Paul W. Sanders, MD, FACP
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      Acute Kidney Injury

      • ANUPAM AGARWAL, MD, FASNProfessor and Director, Division of Nephrology, University of Alabama at Birmingham, Birmingham, AL
      • PAUL W. SANDERS, MD, FACPProfessor and Director, Nephrology Research and Training Center, University of Alabama at Birmingham, Chief, Renal Section, Birmingham Veterans Affairs Medical Center, Birmingham, AL

      Acute renal failure (ARF) has been defined as a syndrome in which an abrupt decrease in renal function produces retention of nitrogenous waste products. Translating this abstract description into a clinically useful, accurate, and widely accepted definition has been challenging, in large part because of the focus on serum creatinine concentration, which is easily obtained but has the inherent limitation of poor detection of rapid or subtle, but clinically important, changes in the glomerular filtration rate (GFR). In recent years, therefore, the term acute kidney injury (AKI) has replaced ARF because AKI denotes the entire clinical spectrum from mild increases in serum creatinine to overt renal failure. AKI is defined by the Risk-Injury-Failure-Loss-ESRD (RIFLE) criteria, based on serum creatinine concentration and urine flow rate. The Acute Kidney Injury Network (AKIN) subsequently modified the definition further and divided AKI into three stages. This chapter includes discussions of the etiology and diagnosis of AKI in hospitalized patients and community-acquired AKI. The specific causes, management, and complications of AKI are also discussed. Figures illustrate the pathophysiologic classification of AKI and the effect of hyperkalemia on cardiac conduction—electrocardiogram (ECG) changes. A worksheet for following patients with AKI is provided. Tables describe the RIFLE and AKIN criteria in AKI; prevention, diagnostic evaluation, daily evaluation and management of hospitalized patients, and urinary diagnostic indices in AKI, as well as features that help differentiate between acute and chronic kidney failure; details of furosemide dosing; and treatment modalities for hyperkalemia. This chapter contains 96 references.

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    • 7

      Management of Chronic Kidney Disease and Its Complications

      By Joshua S. Hundert, MD; Ajay K. Singh, MBBS, FRCP (UK), MBA
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      Management of Chronic Kidney Disease and Its Complications

      • JOSHUA S. HUNDERT, MDClinical Fellow, Department of Medicine, Division of Nephrology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA
      • AJAY K. SINGH, MBBS, FRCP (UK), MBAAssociate Professor of Medicine, Associate Dean for Global Education and Continuing Education, Harvard Medical School, Director, Continuing Medical Education, Department of Medicine and Renal Division, Brigham and Women’s Hospital, Boston, MA

      Management of early renal failure helps in the reduction or prevention of end-stage renal disease. The monitoring of renal function is discussed, and the chapter includes a table that shows commonly used methods for monitoring. Risk factors for chronic renal failure include stroke and cardiac disease. Risk factors for renal disease progression are diabetes mellitus, hypertension, proteinuria, smoking, protein intake, and hyperlipidemia. Complications of chronic renal failure that are addressed include sodium and water imbalance, potassium imbalance, acidosis, calcium and phosphorus imbalance, and anemia. There is also a section that discusses the case for early referral to a nephrologist. Tables present the equations used to estimate the glomerular filtration rate (GFR); stages of chronic kidney disease and the appropriate steps in their management; risk factors for chronic kidney disease in which the testing of proteinuria and estimation of GFR are indicated; appropriate diet for patients who have chronic kidney disease; and guidelines for diagnosing and treating anemia resulting from chronic kidney disease. An algorithm outlines the steps in management of calcium and phosphate in patients with kidney disease.

      This review contains 3 highly rendered figures, 6 tables, and 93 references.

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    • 8

      Chronic Kidney Failure and Dialysis

      By Raghu V. Durvasula, MD; Jonathan Himmelfarb, MD
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      Chronic Kidney Failure and Dialysis

      • RAGHU V. DURVASULA, MDAssistant Professor of Medicine, Department of Medicine, Division of Nephrology, University of Washington School of Medicine, Seattle, WA
      • JONATHAN HIMMELFARB, MDDepartment of Medicine, Division of Nephrology, University of Washington School of Medicine, Seattle, WA

      Chronic kidney disease (CKD) is a clinical syndrome arising from progressive kidney injury, formerly known as chronic renal failure, chronic renal disease, and chronic renal insufficiency. It is classified into five stages based primarily on glomerular filtration rate (GFR). This article discusses the epidemiology of CKD and end-stage renal disease (ESRD), as well as etiology and genetics, pathophysiology, and pathogenesis. The section on diagnosis looks at clinical manifestations and physical findings, laboratory (and other) tests, imaging studies, and biopsy. A short section on differential diagnosis is followed by a discussion of treatment, including hemodialysis and peritoneal dialysis. Long-term complications of patients on dialysis include cardiovascular disease, renal osteodystrophy, dialysis-related amyloidosis, and acquired cystic disease (renal cell carcinoma). The final section addresses prognosis and socioeconomic burden. Figures include the classification system for CKD, prevalence of CKD in the United States, rising prevalence, risk of, and leading causes of ESRD in the United States, plus the changing prevalence of ESRD over time, clinical manifestations of uremia, and an overview of hemodialysis circuit. Tables look at the burden of CKD relative to other chronic disorders, the specific hereditary causes of kidney disease, and situations when serum creatinine does not accurately predict GFR. Other tables list equations for estimating GFR, the causes of CKD without shrunken kidneys, and clinical features distinguishing chronic kidney disease from acute kidney injury. ESRD and indications for initiation of dialysis are presented, as well as typical composition of dialysate and reasons for failure of peritoneal dialysis. This chapter contains 71 references.

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    • 9

      Pharmacologic Approach to Renal Insufficiency

      By Ali J. Olyaei, PharmD; William M. Bennett, MD
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      Pharmacologic Approach to Renal Insufficiency

      • ALI J. OLYAEI, PHARMD
      • WILLIAM M. BENNETT, MDMedical Director, Legacy Transplant Services, Professor of Pharmacy Practice, Legacy Good Samaritan Medical Center, Oregon State University, Portland, OR

      Prescribing drugs in patients with kidney disease is complex: drug dosing needs to be adjusted by the stage of kidney disease (whether chronic kidney disease [CKD] stages 1 through 5 or acute kidney injury [AKI] stages 1 through 3); because potential interactions with other agents that are being used need to be considered; and because of the possibility of extracorporeal treatment that might need to be used (e.g., continuous renal replacement therapy [CRRT], peritoneal dialysis [PD], or hemodialysis [HD]). Besides this complexity, there has been an explosion in the classes of new agents and the routes of delivery of these agents. The purpose of this chapter is to review the basic pharmacokinetic and pharmacologic principles that should guide therapy and to summarize basic recommendations for patients with CKD and AKI. The general principles for drug dosing in CKD and AKI include pharmacokinetics in renal failure; bioavailability; volume of distribution; protein binding; and biotransformation. A stepwise approach to dosage adjustment is described and created as an algorithmic approach. Drug dosing considerations in dialysis patients and in AKI patients are covered as well.
      This chapter contains 2 algorithms, 7 tables, 25 references, 5 Board-styled MCQs, and 1 Teaching Slide Set.

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    • 10

      Nephrolithiasis

      By Orson W. Moe, MD; Khashayar Sakhaee, MD; Naim M. Maalouf, MD
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      Nephrolithiasis

      • ORSON W. MOE, MDProfessor, Department of Internal Medicine, Charles and Jane Pak Center for Mineral Metabolism and Clinical Research, University of Texas Southwestern Medical Center, Dallas, TX
      • KHASHAYAR SAKHAEE, MDProfessor, Department of Internal Medicine, Charles and Jane Pak Center for Mineral Metabolism and Clinical Research, University of Texas Southwestern Medical Center, Dallas, TX
      • NAIM M. MAALOUF, MDAssistant Professor, Department of Internal Medicine, Charles and Jane Pak Center for Mineral Metabolism and Clinical Research, University of Texas Southwestern Medical Center, Dallas, TX

      Nephrolithiasis is a global health issue permeating all geographic regions and socioeconomic classes. The current estimate yields an annual incidence of 1%, a prevalence of 3 to 5%, and a lifetime risk of 25%. The incidence of urolithiasis is rising with time. Nephrolithiasis is recurrent in the majority of cases, rendering it a lifetime affliction in most individuals. It is more common in males than in females during most of adult life except in the sixth decade, when the incidence equalizes. Geographic variations exist in stone prevalence, and regional "stone belts" have been identified and attributed to genetic and environmental factors such as a hot climate (fluid loss) and sun exposure (vitamin D). This chapter discusses the epidemiology, etiology and genetics, pathophysiology, diagnosis, and treatment of nephrolithiasis, including management of chronic and recurrent cases. A figure illustrates the pathophysiology of stone formation. Tables describe the causes and stone types in nephrolithiasis, monogenic causes, diagnostic tests, and interpretation of urinary biochemical profiles. This chapter has 93 references.

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    • 11

      Vascular Diseases of the Kidney

      By Ronald J. Falk, MD, FACP; Julieanne G. McGregor, MD; Vimal K. Derebail, MD, MPH; Abhijit V. Kshirsagar, MD, MPH
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      Vascular Diseases of the Kidney

      • RONALD J. FALK, MD, FACPAllan Brewster Distinguished Professor of Medicine; Chief, Division of Nephrology and Hypertension; Director, UNC Kidney Center, Department of Medicine, Division of Nephrology and Hypertension, University of North Carolina at Chapel Hill School of Medicine, Chapel Hill, NC
      • JULIEANNE G. MCGREGOR, MDAssistant Professor, Department of Medicine, Division of Nephrologyand Hypertension, University of North Carolina at Chapel Hill School of Medicine, Chapel Hill, NC
      • VIMAL K. DEREBAIL, MD, MPHAssistant Professor, Department of Medicine, Division of Nephrology and Hypertension, University of North Carolina at Chapel Hill School of Medicine, Chapel Hill, NC
      • ABHIJIT V. KSHIRSAGAR, MD, MPHAssociate Professor, Department of Medicine, Division of Nephrology and Hypertension, University of North Carolina at Chapel Hill School of Medicine, Chapel Hill, NC

      A number of local and systemic disease processes affect the renal vascular tree. Although the underlying mechanisms may differ, vascular diseases of the kidney all characteristically cause varying degrees of vessel obstruction, eventually leading to an impairment of renal blood flow. Acute impairment in renal function occurs in most vascular diseases of the kidney. This chapter discusses the major vascular diseases of the kidney, categorized according to the size of the affected vessel. Large-vessel diseases described are renal artery stenosis from atherosclerosis and fibromuscular dysplasia, Takayasu arteritis, and renal vein thrombosis. Medium-size-vessel diseases described are polyarteritis nodosa and Kawasaki disease. Small-vessel diseases described are those traditionally associated with glomerulopathy, including rapidly progressive glomerulonephritis and Henoch-Schönlein purpura; thrombotic microangiopathy, including thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome, preeclampsia, antiphospholipid syndrome, systemic sclerosis, and malignant hypertension; sickle cell disease; and atheroembolic disease. Tables describe the American College of Rheumatology classification criteria for Takayasu arteritis and induction therapy for rapidly progressive glomerulonephritis. Figures show an overview of vascular disease of the kidney; an arteriogram of renal artery stenosis demonstrating an ostial lesion; light microscopy showing typical crescentic glomerulonephritis; purpuric eruptions in small vessel vasculitides, Henoch-Schönlein purpura, hypersensitivity vasculitis, and antineutrophil cytoplasmic autoantibody–associated disease; light microscopy showing the characteristic onion-skin lesion in scleroderma renal crisis; electron microscopy showing a glomerular capillary with thrombotic thrombocytopenic purpura; injection microradioangiograms comparing the vasa recta of a normal kidney with that in a patient with sickle cell disease; and a light microscopy that reveals an atheroembolus lodged in a small renal artery and occlusion of the vascular lumen. This chapter contains 150 references.

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    • 12

      Benign Prostatic Hyperplasia

      By Michael J. Barry, MD, FACP
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      Benign Prostatic Hyperplasia

      • MICHAEL J. BARRY, MD, FACPPhysician Medical Services, Massachusetts General Hospital, Clinical Professor of Medicine, Harvard Medical School, Boston, MA

      Benign prostatic hyperplasia (BPH) is a common cause of morbidity in older men in developed countries. BPH causes lower urinary tract symptoms (LUTS) and occasionally results in such complications as acute urinary retention (AUR), urinary tract infection, and even obstructive uropathy. Although the development of medical treatments has reduced the role of surgery, prostatectomy remains a widely performed procedure. Epidemiology, risk factors, and pathophysiology are discussed. Diagnosis includes clinical features, history, physical examination, and laboratory testing. Management can include watchful waiting, medical treatment, surgery, and minimally invasive treatment. Management of AUR is also addressed. A table lists the alpha blockers commonly used in the treatment of men with LUTS attributed to BPH. Figures illustrate the prevalence of BPH histology with age, the anatomy of the prostate gland, the International Prostate Symptom Score (IPSS) for assessing BPH, and 4-year outcomes in men with moderate and severe LUTS attributable to BPH.

      This chapter contains 4 highly rendered figures, 1 table, 70 references, and 5 MCQs.

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    • 13

      Cardiovascular Disease in Patients With Kidney Disease

      By Ernest I. Mandel, MD; David M. Charytan, MD, MSc
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      Cardiovascular Disease in Patients With Kidney Disease

      • ERNEST I. MANDEL, MDClinical Fellow in Medicine, Harvard Medical School and Renal Division, Brigham and Women’s Hospital, Boston, MA
      • DAVID M. CHARYTAN, MD, MSCAssistant Professor of Medicine, Harvard Medical School, Associate Staff Physician, Renal Division, Brigham & Women’s Hospital, Boston, MA

      Cardiovascular disease (CVD) is prevalent in patients with chronic kidney disease (CKD) and is the leading cause of morbidity and mortality in dialysis patients. Clinical practice guidelines established by the National Kidney Foundation identify five stages of CKD defined by estimated glomerular filtration rate (eGFR) as determined by the abbreviated Modification of Diet in Renal Disease (MDRD) study equation. The National Health and Nutrition Epidemiology Survey (NHANES III) estimated that 13% of adults in the United States have CKD. An estimated 5 to 10% of the world's population—a staggering 300 to 600 million people—have CKD. This chapter covers the epidemiology, clinical spectrum, pathogenesis, and management of CVD in patients who have CKD. The clinical spectrum of CVD in patients with CKD is divided into four major categories: arterial disease, myocardial disease, disorders of cardiac rhythm, and valvular disease. Sections discuss some or all of the following: epidemiology/pathophysiology, diagnosis, management, risk factors, intervention, prevention. The chapter includes 4 tables and 6 figures. Figures present age-standardized rates of death, Kaplan–Meier estimates of rates of death, mortality by stage of CKD, hazard ratios and 95% confidence intervals for all-cause and cardiovascular mortality, cardio-renal syndrome pathophysiology, and ratios for actual to expected number of occurrences of sudden death. Tables list the five stages of CKD, types of CVD associated with CKD, risk factors for CVD in patients with CKD/end-stage renal disease (ESRD), and factors contributing to platelet dysfunction and enhanced bleeding risk in CKD/ESRD. This chapter contains 191 references.

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    • 14

      Kidney Transplantation 1: an Overview--recipient Evaluation and Immunosuppression

      By Jamil Azzi, MD; Belinda T. Lee, MD; Anil Chandraker, MD
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      Kidney Transplantation 1: an Overview--recipient Evaluation and Immunosuppression

      • JAMIL AZZI, MDInstructor of Medicine, Harvard Medical School, Brigham and Women’s Hospital, Boston, MA
      • BELINDA T. LEE, MDCharles Bernard Carpenter Transplant Fellow, Harvard Medical School, Brigham and Women’s Hospital, Boston, MA
      • ANIL CHANDRAKER, MDMedical Director of Kidney and Pancreas Transplantation, Associate Professor of Medicine, Harvard Medical School, Brigham and Women’s Hospital, Boston, MA

      Half a century after the first successful kidney transplantation, we still stand at the crossroads of immunology and transplantation, where science meets art in the management of complex end-stage renal disease (ESRD) patients. Successful transplantation requires not only a lifetime’s commitment from patients but also a multidisciplinary approach, bringing together surgeons, transplant nephrologists, primary care physicians, scientists, and nurses to provide coordinated care. Although transplantation is the treatment of choice for the vast majority of ESRD patients, many patients remain on dialysis due to a relative imbalance between demand for and supply of suitable organs. This chapter provides a comprehensive overview of recipient evaluation and immunosuppression. Risk factors that prohibit transplantation are discussed, as are human leukocyte antigen/ABO compatibility, transplant immunobiology, induction therapy, maintenance therapy, transplantation for special populations, and future directions in the field. Tables outline Amsterdam Living Donation Forum guidelines, ABO blood group compatibilities, and pretransplant immunologic testing. Visual aids include graphs, charts, cell illustrations, and an evaluative algorithm.

      This chapter contains 10 figures, 3 tables, 101 references, and 5 Board-styled MCQs.

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    • 15

      Kidney Transplantation 2: Care of the Kidney Transplant Recipient

      By Belinda T. Lee, MD; Jamil Azzi, MD; Anil Chandraker, MD
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      Kidney Transplantation 2: Care of the Kidney Transplant Recipient

      • BELINDA T. LEE, MDCharles Bernard Carpenter Transplant Fellow, Harvard Medical School, Brigham and Women’s Hospital, Boston, MA
      • JAMIL AZZI, MDInstructor of Medicine, Harvard Medical School, Brigham and Women’s Hospital, Boston, MA
      • ANIL CHANDRAKER, MDMedical Director of Kidney and Pancreas Transplantation, Associate Professor of Medicine, Harvard Medical School, Brigham and Women’s Hospital, Boston, MA

      This chapter discusses the goals and challenges of caring for the kidney transplant recipient. This area has become increasingly specialized in recent years, with physicians needing a good understanding of immunology, nephrology, pharmacology, and infectious diseases as well as a good grasp of internal medicine. Topics covered include cardiovascular disease, new on-set diabetes, hypertension, dyslipidemia, infectious complications, malignancy, bone disease, recurrence of disease, drug reactions, rejection, and other challenges that face the kidney transplant population. Tables outline relevant medications, recommended vaccines, virus-associated malignancies, cancer rates, common drug interactions, and diagnostic categories. Figures include graphs, charts, and microscopic photos.
      This chapter contains 7 figures, 7 tables, 111 references, and 5 Board-styled MCQs.

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    • 16

      Kidney Disease in the Cancer Patient

      By Colm Magee, MD, MPH, FRCPI; Lynn Redahan, MD, MRCPI
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      Kidney Disease in the Cancer Patient

      • COLM MAGEE, MD, MPH, FRCPIDepartment of Nephrology, Consultant Nephrologist and Lynn Redahan, MRCPI, Senior Registrar in Nephrology, Beaumont Hospital, Beaumont, Dublin, Ireland
      • LYNN REDAHAN, MD, MRCPIDepartment of Nephrology, Consultant Nephrologist and Lynn Redahan, MRCPI, Senior Registrar in Nephrology, Beaumont Hospital, Beaumont, Dublin, Ireland

      The spectrum of kidney disease in the cancer patient is wide. Kidney dysfunction can result from the cancer itself or its treatment. The presentation in this population is varied and may manifest as acute kidney injury (AKI) or chronic kidney disease. In addition, other manifestations of kidney disease can include proteinuria, hypertension, and electrolyte disturbances. As new cancer treatments emerge, the range of therapy-associated renal syndromes increases. This chapter deals predominantly with causes and management of renal dysfunction that are specific to the cancer patient, including those caused by hypercalcemia; hepatorenal syndrome; the use of interleukin-2 (IL-2) and bisphosphonate; glomerular, tubular, interstitial, and vascular diseases; multiple myeloma (MM); and tumor infiltration. The chapter also examines postrenal causes of AKI, electrolyte disorders, and hematopoietic stem cell transplantation (HSCT). Tables provide the features of kidney disease in the cancer patient, the pathogenesis of hypercalcemia, strategies for preventing and managing AKI with IL-2 therapy, laboratory findings with hemolytic-uremic syndrome/thrombocytopenic purpura, the causes of acute tubular necrosis in MM, a summary of electrolyte disturbances in the cancer patient, indications for HSCT, and a summary of the management of patients with post-HSCT AKI. The chapter is also enhanced by ultrasound and computed tomographic scans, histology images, and an illustration of tumor lysis syndrome.

      This chapter contains 105 references, 8 tables, 4 highly rendered figures, and 5 MCQs.

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  • Neurology
    • Neurologic Symptoms
      • 1

        Dizziness

        By Kevin A. Kerber, MD, MS; Robert W. Baloh, MD
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        Dizziness

        • KEVIN A. KERBER, MD, MSAssistant Professor, Department of Neurology, University of Michigan, Ann Arbor, MI
        • ROBERT W. BALOH, MDProfessor, Departments of Neurology and Surgery (Head and Neck), UCLA Medical Center, Reed Neurological Research Center, Los Angeles, CA

        Dizziness is the quintessential symptom presentation in all of clinical medicine. It is a common reason that patients present to a physician. This chapter provides background information about the vestibular system, then reviews key aspects of history-taking and examination of the patient, then discusses specific disorders and common presentation types. Throughout the chapter the focus is on neurologic and vestibular disorders. Normal vestibular anatomy and physiology are discussed, followed by recommendations for history-taking and the physical examination. Specific disorders that cause dizziness are explored, along with common causes of non-specific dizziness. Common presentations are discussed, including acute severe dizziness, recurrent attacks, and recurrent positional vertigo. Finally, the chapter looks at laboratory investigations in diagnosis and management. Figures include population prevalence of dizziness symptoms, the anatomy of inner structures, primary afferent vestibular nerve activity, the head thrust test, the Dix-Hallpike maneuver, the supine positional test, the canalith repositioning procedure, and the barbecue roll maneuver. Tables list physiologic properties and clinical features of the components of the peripheral vestibular system, information to be acquired from history of the present illness, common symptoms patients report as dizziness, examination components, distinguishing among common peripheral and central vertigo syndromes, common causes of nonspecific dizziness, types of dizziness presentations, relevant imaging abnormalities on neuroimaging studies, vestibular testing components, and medical therapy for symptomatic dizziness.

        This review contains 8 highly rendered figures, 10 tables, and 68 references.

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      • 2

        Headache and Facial Pain

        By Elizabeth W. Loder, MD, MPH
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        Headache and Facial Pain

        • ELIZABETH W. LODER, MD, MPHAssociate Professor of Neurology, Harvard Medical School, Chief, Division of Headache and Pain, Department of Neurology, Brigham and Women’s Hospital, Boston, MA

        Headaches are a near-universal experience, with a 1-year prevalence of 90% and a lifetime prevalence of 99%. Headaches and pain to the head account for roughly 3% of visits to US emergency departments annually, making them the fourth most common reason for seeking emergency care. There are numerous types of headaches, and although the majority are benign, types exist that may result from serious and potentially life-threatening causes. As such, it is important for the physician to consider a broad differential diagnosis for every headache patient. This review discusses the classification of headaches, identifies pain-sensitive structures in the head, discusses the history and examination in patients with headache, and describes many of the primary and secondary headaches. Figures show the areas of the brain sensitive to pain; 1-year prevalence of migraine in men, women, and children; frequency of attacks in migraineurs; prevalence of headaches by age group and in patients with cerebrovascular disorders; and symptoms of idiopathic intracranial hypertension. Tables list the major categories of headache disorders, key elements of the headache history, helpful questions to ask, features of selected primary and secondary headaches, reasons to consider neuroimaging, efficacy of selected over-the-counter medications, triptans available in the United States, medication options for urgent or emergency treatment of migraine, selected preventive medications for migraine, generally accepted indications for preventive treatment, general principles for the use of preventive medications, titration schedules for preventive medication, interval or short-term preventive treatment of menstrual migraine, strategies for managing increase in migraines in patients starting estrogen replacement therapy, transition medications for rapid, temporary suppression of headaches, medications possibly effective for cluster and hypnic headaches, differential diagnosis of the acute, severe, new-onset headache, and etiologies of papilledema and headache.

        This review contains 6 highly rendered figures, 20 tables, and 112 references.

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      • 3

        Pain Syndromes Other Than Headache

        By Edgar L. Ross, MD
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        Pain Syndromes Other Than Headache

        • EDGAR L. ROSS, MD

        Chronic pain is very common in the United States. Although effective treatments are available, pain remains poorly managed in many patients. This review covers how to assess a patient with pain; the management of acute and chronic pain; and peripheral neuropathy, complex regional pain syndrome (CRPS), fibromyalgia, myofascial pain, back and spine pain, and cancer pain. Figures show how pain transducers (nociceptors) monitor and influence tissue conditions, sensory processing in the spinal cord dorsal horn, and classification of chronic pain syndromes. Tables list the International Association for the Study of Pain’s definitions for the management of pain; differences between acute and chronic pain; physiologic mechanisms sustaining pain; pain descriptors and types of pain; distinguishing hyperalgesia and allodynia; patient classifications and group characteristics; treatments and medication classes used for acute pain as well as chronic pain; two categories of chronic pain, differences between nociceptive and neuropathic pain; behavioral and rehabilitative therapies used for chronic pain; the Budapest diagnostic criteria for CRPS; diagnostic approaches to CRPS; signs and symptoms of fibromyalgia; conditions that can mimic myofascial pain; clinical presentation of myofascial pain; differences between myofascial pain and fibromyalgia; trigger-point examination; myofascial pain treatment; diagnostic categories of spinal pain; spinal pain red flags; the Waddell criteria; and cancer pain syndromes, characteristics, treatment options, and side-effect management options.

        This review contains 3 highly rendered figures, 31 tables, and 82 references.

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      • 4

        Sleep Disorders

        By Sudhansu Chokroverty, MD, FRCP, FACP
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        Sleep Disorders

        • SUDHANSU CHOKROVERTY, MD, FRCP, FACPProfessor and Director of Sleep Research, Medical Director of Devry Technology Training Program, Co-Chair Emeritus of Neurology, Department of Neurology, JFK Neuroscience Institute, Edison, NJ, Professor of Neuroscience, Seton Hall University, South Orange, NJ, Clinical Professor of Neurology, Robert Wood Johnson Medical School, New Brunswick, NJ

        Recent research has generated an enormous fund of knowledge about the neurobiology of sleep and wakefulness. Sleeping and waking brain circuits can now be studied by sophisticated neuroimaging techniques that map different areas of the brain during different sleep states and stages. Although the exact biologic functions of sleep are not known, sleep is essential, and sleep deprivation leads to impaired attention and decreased performance. Sleep is also believed to have restorative, conservative, adaptive, thermoregulatory, and consolidative functions. This review discusses the physiology of sleep, including its two independent states, rapid eye movement (REM) and non–rapid eye movement (NREM) sleep, as well as functional neuroanatomy, physiologic changes during sleep, and circadian rhythms. The classification and diagnosis of sleep disorders are discussed generally. The diagnosis and treatment of the following disorders are described: obstructive sleep apnea syndrome, narcolepsy-cataplexy sydrome, idiopathic hypersomnia, restless legs syndrome (RLS) and periodic limb movements in sleep, circadian rhythm sleep disorders, insomnias, nocturnal frontal lobe epilepsy, and parasomnias. Sleep-related movement disorders and the relationship between sleep and psychiatric disorders are also discussed. Tables describe behavioral and physiologic characteristics of states of awareness, the international classification of sleep disorders, common sleep complaints, comorbid insomnia disorders, causes of excessive daytime somnolence, laboratory tests to assess sleep disorders, essential diagnostic criteria for RLS and Willis-Ekbom disease, and drug therapy for insomnia. Figures include polysomnographic recording showing wakefulness in an adult; stage 1, 2, and 3 NREM sleep in an adult; REM sleep in an adult; a patient with sleep apnea syndrome; a patient with Cheyne-Stokes breathing; a patient with RLS; and a patient with dream-enacting behavior; schematic sagittal section of the brainstem of the cat; schematic diagram of the McCarley-Hobson model of REM sleep mechanism; the Lu-Saper “flip-flop” model; the Luppi model to explain REM sleep mechanism; and a wrist actigraph from a man with bipolar disorder.

        This review contains 14 highly rendered figures, 8 tables, 115 references, and 5 MCQs.

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    • Categories of Neurologic Diseases
      • Cognitive and Behavioral Neurology
        • 1

          Alzheimer Disease and Related Disorders

          By David S. Knopman, MD
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          Alzheimer Disease and Related Disorders

          • DAVID S. KNOPMAN, MDProfessor of Neurology, Department of Neurology, Mayo Clinic, Rochester, MN

          Alzheimer disease (AD) is histopathologically defined by neurofibrillary tangles and neuritic plaques in the cerebral cortex. The term AD is also commonly used as a clinical diagnosis for a dementia syndrome in which anterograde amnesia is a dominant symptom. In light of the applicability of AD biomarkers across the spectrum of the disease, the term AD should be limited to describing the pathology. When the clinical syndrome is described, it should be denoted by “dementia due to AD” or “AD dementia” to indicate the syndrome and the etiology. The most common cognitive deficit in both mild cognitive impairment (MCI) and dementia due to AD is anterograde amnesia, the basis of which is an inability to learn new information. The definition of AD underwent a substantial updating in 2011 with the publication of three documents from work groups convened under the auspices of the National Institute on Aging and the Alzheimer’s Association (NIAAA) that describe the definitions of AD dementia, MCI due to AD, and a preclinical AD state. This chapter explains the epidemiology, pathophysiology, complications, prognosis, molecular biology, and genetics of AD, including the role of the apolipoprotein E (APOE) gene. Protective and risk factors are discussed. Clinical manifestations, clinical assessment, laboratory testing, imaging studies, differential diagnosis, primary and secondary therapies, and caregiver support are described. This chapter also discusses vascular dementia, Lewy body disease, and frontotemporal lobar degenerations. Tables provide the definition of dementia and AD dementia, an index of cerebrovascular disease in cognitive impairment, essentials of criteria for the clinical diagnosis of dementia with Lewy bodies, and diagnostic criteria for behavior variant frontotemporal dementia. A histogram of the prevalence of AD by gender and age is provided, as are schematic overviews of the amyloid-β peptide production pathway and the sequence of biomarker abnormalities in AD. Photomicrographs, magnetic resonance imaging (MRI) scans, and positron emission tomographic scans of the brain in patients with AD are shown. MRI scans of the brains of patients with vascular dementia and frontotemporal lobar degenerations are provided, as are photomicrographs of Pick bodies and a TDP43-positive neuron.

           

          This chapter contains 12 highly rendered figures, 5 tables, 146 references, 1 teaching slide set, and 5 MCQs.

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        • 2

          Cognitive Disorders Other Than Alzheimer Disease

          By Seth A. Gale, MD; Kirk R. Daffner, MD, FAAN
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          Cognitive Disorders Other Than Alzheimer Disease

          • SETH A. GALE, MDAssociate Neurologist, Division of Cognitive and Behavioral Neurology, Brigham and Women’s Hospital, and Instructor of Neurology, Harvard Medical School, Boston, MA
          • KIRK R. DAFFNER, MD, FAANChief, Division of Cognitive and Behavioral Neurology, Brigham and Women’s Hospital, J. David and Virginia Wimberly Professor of Neurology, Harvard Medical School, Boston, MA

          Higher cognitive functions, such as abstract reasoning, complex decision making, and language, are the mental faculties that separate our species from other animals. When these faculties become impaired as a result of neurologic disease, striking and devastating behavioral changes result. Many neurologic diseases are associated with impaired cognition and behavior, and their etiologies are as varied as their clinical presentations. In this review, the focus is on dementia with Lewy bodies (DLB), the frontotemporal dementias (FTDs), and vascular cognitive impairment (VCI). The review covers the epidemiology and diagnosis of  DBB, FTDs, and VCIs, as well as the etiology and genetics. Figures show neuroimaging in DLB, management of DLB, FTLD clinical syndromes, FTLD clinicopathologic correlations: approximate distribution of pathotypes for behavioral-variant FTD and primary progressive aphasia (PPA) variants, PPA-semantic subtype, PPA-logopenic subtype, post-stroke VCI, and subcortical ischemic vascular disease subtype of VCI. Tables list diagnostic criteria for DLB; FTD genetics: gene/protein relationship, clinical syndrome, salient gestures; VCI: clinical and pathologic features of the main subtypes; summary guidance based on VCI prevention studies; and summary guidance based on VCI treatment studies.

          This review contains 8 highly rendered figures, 5 tables, and 254 references.

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        • 3

          Stroke and Other Cerebrovascular Diseases

          By Scott E. Kasner, MD; Christina A. Wilson, MD, PhD
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          Stroke and Other Cerebrovascular Diseases

          • SCOTT E. KASNER, MDProfessor, Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Director, Comprehensive Stroke Center, University of Pennsylvania Health System, Philadelphia, PA
          • CHRISTINA A. WILSON, MD, PHDAssistant Professor, Department of Neurology, University of Florida, Gainesville, FL

          Stroke is a sudden neurologic deficit caused by either ischemia or hemorrhage. Ischemic stroke is classically labeled as either thrombotic or embolic, but these two mechanisms may be impossible to distinguish clinically. Ischemic stroke mechanisms are more reliably categorized as cardioembolism, large-vessel atherothromboembolism, small-vessel occlusive disease, other identified mechanism, or cryptogenic (idiopathic). Hemorrhagic stroke includes intracerebral hemorrhage and subarachnoid hemorrhage. This chapter discusses the epidemiology, etiology, neuroimaging and diagnosis, treatment, and prevention of both ischemic and hemorrhagic stroke. Prompt management of acute stroke is important because there is a crucial link between intervention time and patient outcome. Key measures for stroke management from the Joint Commission Primary Stroke Center are indicated. An algorithm for diagnostic evaluation of suspected stroke and a clinical pathway for managing acute stroke patients are provided. Other figures illustrate early computed tomographic findings and magnetic resonance imaging findings in acute ischemic stroke, potential sources of cardioembolism, cerebrovascular anatomy and common sites of atherosclerosis, and angiography of intra-arterial thrombolysis for acute ischemic stroke. Tables delineate the differential diagnosis of acute ischemic stroke, clinical features of the major cerebrovascular occlusive syndromes, the ABCDscoring system, indications and contraindications for intravenous recombinant tissue plasminogen (rt-PA) treatment in acute ischemic stroke, modifiable risk factors for stroke, antiplatelet therapies for prevention of ischemic stroke, and management strategies for elevated intracranial pressure.

           

          This chapter contains 7 highly rendered figures, 7 tables, 99 references, 5 MCQs, and 1 teaching slide set.

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        • 4

          Multiple Sclerosis and Related Disorders

          By J. William Lindsey, MD
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          Multiple Sclerosis and Related Disorders

          • J. WILLIAM LINDSEY, MDProfessor, Department of Neurology, University of Texas Health Science Center at Houston, Houston, TX

          Multiple sclerosis (MS) is a relatively common cause of neurologic symptoms and disability in young adults. The distinguishing pathologic features of MS are loss of myelin and inflammation in the central nervous system (CNS). The myelin sheath is essential for rapid conduction of nerve signals along large-diameter axons. Oligodendrocytes produce and maintain myelin in the CNS, and Schwann cells produce and maintain myelin in the peripheral nerves. In addition to MS, there are a number of related disorders causing demyelination, inflammation, or both in the CNS. This chapter discusses MS and related disorders, including neuromyelitis optica, optic neuritis, acute disseminated encephalomyelitis, transverse myelitis, Behçet syndrome, neurosarcoidosis, inherited demyelinating diseases (leukodystrophies, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy [CADASIL]), and virus-induced demyelination (progressive multifocal leukoencephalopathy, subacute sclerosing panencephalitis). The section on MS covers epidemiology, etiology/genetics, pathogenesis, diagnosis, differential diagnosis, management, and prognosis. Figures include organization of the microenvironment of larger-diameter axons, typical magnetic resonance imaging findings in MS and neuromyelitis optica, postgadolinium images of the cervical spine in MS, and an approach to treatment of relapsing-remitting MS. Tables list MS and related disorders, distribution of neurologic deficits at the onset of MS, differential diagnosis of MS, disease-modifying therapies for relapsing-remitting MS, and selected leukodystrophies, as well as diagnostic criteria and selected symptomatic therapies for MS.

          This review contains 3 highly rendered figures, 7 tables, and 82 references.

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        • 5

          Traumatic Brain and Spinal Cord Injuries

          By Mohit Datta, MD; Geoffrey S.F. Ling, MD, PhD, FAAN
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          Traumatic Brain and Spinal Cord Injuries

          • MOHIT DATTA, MD
          • GEOFFREY S.F. LING, MD, PHD, FAANUniformed Services University of the Health Sciences, Bethesda, MD

          Traumatic brain and spinal cord injuries are significant causes of permanent disability and death. In 2010, 823,000 traumatic brain injuries were reported in the United States alone; in fact, the actual number is likely considerably higher because mild traumatic brain injuries and concussions are underreported. The number of new traumatic spinal cord injuries has been estimated at 12,000 annually. Survival from these injuries has increased due to improvements in medical care. This review covers mild traumatic brain injury and concussion, moderate to severe traumatic brain injury, and traumatic spinal cord injury. Figures include computed tomography scans showing a frontal contusion, diffuse cerebral edema and intracranial air from a gunshot wound, a subdural hematoma, an epidural hematoma, a skull fracture with epidural hematoma, and a spinal fracture from a gunshot wound. Tables list requirements for players with concussion, key guidelines for prehospital management of moderate to severe traumatic brain injury, key guidelines for management of moderate to severe traumatic brain injury, brain herniation brain code, key clinical practice guidelines for managing cervical spine and spinal cord injury, and the American Spinal Injury Association’s neurologic classification of spinal cord injury.

          This review contains 6 highly rendered figures, 6 tables, and 61 references.

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        • 6

          Epilepsy and Related Disorders

          By Barbara Dworetzky, MD; Jong Woo Lee, MD, PhD
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          Epilepsy and Related Disorders

          • BARBARA DWORETZKY, MDAssociate Professor of Neurology, Harvard Medical School, Chief, Division of Epilepsy, EEG, and Sleep Neurology, Director, The Edward B. Bromfield Epilepsy Program, Brigham and Women’s Hospital, Boston, MA
          • JONG WOO LEE, MD, PHDAssistant Professor of Neurology, Harvard Medical School, Director, ICU EEG Monitoring, The Edward B. Bromfield Epilepsy Program, Brigham and Women’s Hospital, Boston, MA

          Epilepsy is a chronic disorder of the brain characterized by recurrent unprovoked seizures. A seizure is a sudden change in behavior that is accompanied by electrical discharges in the brain. Many patients presenting with a first-ever seizure are surprised to find that it is a very common event. A reversible or avoidable seizure precipitant, such as alcohol, argues against underlying epilepsy and therefore against treatment with medication. This chapter discusses the epidemiology, etiology, and classification of epilepsy and provides detailed descriptions of neonatal syndromes, syndromes of infancy and early childhood, and syndromes of late childhood and adolescence. The pathophysiology, diagnosis, and differential diagnosis are described, as are syncope, migraine, and psychogenic nonepileptic seizures. Two case histories are provided, as are sections on treatment (polytherapy, brand-name versus generic drugs, surgery, stimulation therapy, dietary treatments), complications of epilepsy and related disorders, prognosis, and quality measures. Special topics discussed are women?s issues and the elderly. Figures illustrate a left midtemporal epileptic discharge, wave activity during drowsiness, cortical dysplasias, convulsive syncope, rhythmic theta activity, right hippocamal sclerosis, and right temporal hypometabolism. Tables describe international classifications of epileptic seizures and of epilepsies, epilepsy syndromes and related seizure disorders, differential diagnosis of seizure, differentiating epileptic versus nonepileptic seizures, antiepileptic drugs, status epilepticus protocol for treatment, when to consider referral to a specialist, and quality measures in epilepsy. This chapter contains 111 references.

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      • Neuromuscular Diseases
        • 1

          Motor Neuron Diseases

          By Merit E. Cudkowicz, MD, MSc; James D. Berry, MD, MPH; Elena Ratti, MD
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          Motor Neuron Diseases

          • MERIT E. CUDKOWICZ, MD, MSCChief of Neurology, Massachusetts General Hospital, Julieanne Dorn Professor of Neurology Harvard Medical School, Neurological Clinical Research Institute, Massachusetts General Hospital, Boston, MA
          • JAMES D. BERRY, MD, MPHAssistant Professor of Neurology, Neurological Clinical Research Institute, Massachusetts General Hospital, Boston, MA
          • ELENA RATTI, MDClinical Research Fellow, Neurological Clinical Research Institute, Massachusetts General Hospital, Boston, MA

          The motor neuron diseases (MNDs) are a family of diseases commonly categorized by their propensity to affect upper or lower motor neurons and by their mode of inheritance. The chapter provides some content on infectious MNDs caused by viral infections affecting the motor neurons in the anterior horn of the spinal cord. However, the chapter devotes most of its attention to the inherited and sporadically occurring MNDs. The majority of research into adult MND focuses on amyotrophic lateral sclerosis (ALS) due to its high prevalence, rapid progression, and phenotypical similarities between its inherited form and its sporadic form. As our knowledge of genetic mechanisms underlying ALS pathology has grown, common themes have emerged. These include abnormalities in RNA biology, axonal transport, protein folding, and inflammatory responses. These themes currently drive much of the direction in ALS experimental therapy development. It is clear that MND is complex and involves several different molecular pathways. Given this complexity, ALS might not be a single disease entity, and if this is the case, treatment approaches may need to be targeted to specific pathologies rather than all ALS patients on a broad scale. Chapter content is enhanced by tables outlining the types of MNDs, criteria for supporting a diagnosis, first-line workup, the genes associated with ALS, ALS efficacy outcome measures, symptom management of ALS, and spinal muscular atrophy classification. Mechanisms of ALS are illustrated, and clinical photographs demonstrate symptoms. This chapter contains 252 references. 

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        • 2

          Radiculopathies and Neuropathies

          By William S. David, MD, PhD; Kathy Chuang, MD
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          Radiculopathies and Neuropathies

          • WILLIAM S. DAVID, MD, PHDAssociate Professor of Neurology, Harvard Medical School, Director of the EMG Laboratory and Neuromuscular Diagnostic Center, Massachusetts General Hospital, 165 Cambridge Street, Suite 820, Boston, MA 02114
          • KATHY CHUANG, MDClinical Fellow in Neuromuscular Medicine, Massachusetts General Hospital and Brigham and Women’s Hospital, 165 Cambridge Street, Suite 820, Boston, MA 02114

          “Radiculopathies” are disorders of nerve roots, whereas “neuropathies” are disorders of the peripheral nerve. These disorders may involve single roots or nerves, multiple roots or nerves, and even other aspects of the nervous system. This chapter reviews the anatomy and pathophysiology of the peripheral nervous system; the general approach to radiculopathies and neuropathies, including clinical manifestations and localization, diagnostic studies, and treatment; radiculopathies, including anatomy, cervical radiculopathy, lumbosacral radiculopathy, thoracic radiculopathy, and cauda equina syndrome; and neuropathies, including  mononeuropathies and polyneuropathies. Tables describe the innervation of select nerve roots and peripheral nerves, differences between root and nerve lesions, commonly used neuropathic pain medications, distinctive patterns of neuropathy with limited differential diagnoses, differential diagnosis of demyelinating polyneuropathy, drugs that may cause polyneuropathy, and neuropathies associated with diabetes mellitus. Figures show the anatomy of a spinal segment, nerve fascicles, ultrasound images of the median nerve, magnetic resonance imaging of the lumbosacral spine, the Spurling maneuver, and physical examination maneuvers for lumbosacral radiculopathies.

          This review contains 6 highly rendered figures, 8 tables, and 77 references.

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        • 3

          Neuromuscular Junction Disorders

          By Anthony A. Amato, MD; Mohammad Kian Salajegheh, MD
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          Neuromuscular Junction Disorders

          • ANTHONY A. AMATO, MDChief, Division of Neuromuscular Disease, Department of Neurology, Brigham and Women’s Hospital, Professor of Neurology, Harvard Medical School, Boston, MA
          • MOHAMMAD KIAN SALAJEGHEH, MDDirector, Peripheral Nerve Clinic, Associate Neurologist, Department of Neurology, Brigham and Women’s Hospital, Assistant Professor of Neurology, Harvard Medical School, Boston, MA

          The three main components of the neuromuscular junction (NMJ) include the presynaptic region, the synaptic cleft, and the postsynaptic region. The NMJ acts as an interface between the motor nerve and muscle by converting the motor nerve electric currents into chemical signals and then back into electric currents in the muscle. This chapter reviews electrodiagnostic testing in NMJ disorders, including repetitive nerve stimulation and single-fiber electromyography. Myasthenia gravis, congenital myasthenic syndromes, Lambert-Eaton myasthenic syndrome, botulism, and organophosphate poisoning and other toxins are discussed, including epidemiology, etiology/genetics, pathogenesis, diagnosis, differential diagnosis, management, complications, and prognosis. Tables include an overview of neuromuscular disorders, drugs with adverse effects on the NMJ, common immunomodulatory agents used for treatment of myasthenia gravis, congenital myasthenic syndromes, and toxins and venoms. Figures illustrate the NMJ structure and function, structure of the presynaptic and postsynaptic regions, electrodiagnostic studies in NMJ disorders, and dysfunction of the NMJ in acetylcholine receptor myasthenia gravis.

          This review contains 5 highly rendered figures, 5 tables, and 65 references.

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        • 4

          Myopathies

          By Anthony A. Amato, MD; Thomas I. Cochrane, MD, MBA
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          Myopathies

          • ANTHONY A. AMATO, MDChief, Division of Neuromuscular Disease, Department of Neurology, Brigham and Women’s Hospital, Professor of Neurology, Harvard Medical School, Boston, MA
          • THOMAS I. COCHRANE, MD, MBAAssociate Neurologist, Division of Neuromuscular Disease, Department of Neurology, Brigham and Women’s Hospital, Assistant Professor of Neurology, Harvard Medical School, Boston, MA

          Muscle disease (myopathy) can be acquired or hereditary. Symptoms include skeletal muscle weakness, atrophy, muscle cramps or myalgias, and impaired function of respiratory, pharyngeal, facial, or ocular muscles. Clinicians must identify treatable myopathies and initiate therapy before permanent weakness occurs. For patients with untreatable disorders, proper supportive care, rehabilitation, genetic counseling, and psychological support are critical. This chapter covers common myopathies, including muscular dystrophies; malignant hyperthermia; metabolic myopathies; mitochondrial myopathies and encephalopathies; ion channelopathies, periodic paralyses, and nondystrophic myotonias; and drug-induced myopathies. Clinical presentation, diagnosis, pathogenesis, and therapy are emphasized. Tables describe genetic classification of the limb-girdle and distal muscular dystrophies; proteins involved in myofibrillar myopathy; other distal myopathies; and antirheumatic, antiinflammatory, and immunosuppressive drug-induced myopathy. Figures show the sarcolemmal membrane and enzymatic proteins associated with muscular dystrophies, sarcomeric and nuclear proteins associated with muscular dystrophies, and major metabolic pathways used by muscle.

          This review contains 3 highly rendered figures, 4 tables, and 107 references.

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      • Movement Disorders
        • 1

          Parkinsonism and Related Disorders

          By Anthony E. Lang, MD; Elizabeth J. Slow, MD, PhD
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          Parkinsonism and Related Disorders

          • ANTHONY E. LANG, MDProfessor, Morton and Gloria Shulman Movement Disorders Clinic and The Edmond J. Safra Program in Parkinson’s Disease, Toronto Western Hospital, University of Toronto, Toronto, ON
          • ELIZABETH J. SLOW, MD, PHDAssistant Professor, Morton and Gloria Shulman Movement Disorders Clinic and The Edmond J. Safra Program in Parkinson’s Disease, Toronto Western Hospital, University of Toronto, Toronto, ON

          Parkinsonism describes the core clinical criteria of tremor, bradykinesia, rigidity, and postural instability. There is a large differential diagnosis, but the most common cause of parkinsonism is due to Parkinson disease. This review details the epidemiology, etiology/genetics, pathogenesis, diagnosis and differential diagnosis, management, and prognosis of Parkinson disease, dementia with Lewy bodies, progressive supranuclear palsy, corticobasal degeneration, vascular parkinsonism, normal pressure hydrocephalus, and drug-induced parkinsonism. Figures show Parkinson disease features; timeline of the clinical course of Parkinson disease; summary of the diagnosis, management, and prognosis of Parkinson disease; an algorithm for the treatment of tremor-predominant Parkinson disease; an algorithm for the treatment of Parkinson disease with predominance of bradykinesia and rigidity; magnetic resonance imaging changes in multiple system atrophy; magnetic resonance imaging changes in progressive supranuclear palsy; and enlarged ventricles associated with normal pressure hydrocephalus. Tables list differential diagnosis of parkinsonism; clues to alternative, non-Parkinson disease causes of parkinsonism (i.e., red flags of parkinsonism); distinguishing other diseases causing parkinsonism from Parkinson disease; treatment of motor symptoms of Parkinson disease; peripheral and central side effects of dopaminergic (dopamine agonist and levodopa) therapy; and treatment of nonmotor symptoms of Parkinson disease.

          This review contains 8 highly rendered figures, 6 tables, and 88 references.

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        • 2

          Hyperkinetic Movement Disorders

          By Devin Mackay, MD; Edison Miyawaki, MD, PhD
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          Hyperkinetic Movement Disorders

          • DEVIN MACKAY, MDClinical Fellow in Neurology, Massachusetts General Hospital and Brigham and Women’s Hospital, Harvard Medical School, Boston, MA
          • EDISON MIYAWAKI, MD, PHDAssociate Neurologist, Brigham and Women’s Hospital and Assistant Professor of Neurology, Harvard Medical School, Brigham and Women’s Hospital, Boston, MA

          The hyperkinetic movement disorders include heterogeneous diseases and syndromes, all characterized by one or a variety of excessive, involuntary movements. The hyperkinetic movement disorders are heterogeneous in clinical presentation, but a rational and practical approach to diagnosis exists based on new genetic correlations and targeted laboratory investigations. Treatments informed by a still-developing picture of motor pathophysiology offer significant benefit for these disorders. This chapter discusses choreiform disorders, including patterns in choreiform diagnosis; tremor disorders; paroxysmal disorders, including tics and myoclonus; dystonias, including monogenic primary dystonias; and pathophysiology and treatment in the hyperkinetic movement disorders. Figures include clinical photos, computed tomography scans, and an algorithm representing cortical-subcortical circuitry. Tables delineate definitions, distinguishing clinical features, medications, genetics, protein products, and treatments associated with various disorders. 

          This review contains 6 figures, 12 tables, and 145 references.

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        • 3

          Myoclonus

          By Hiroshi Shibasaki, MD, PhD; Marina A.J. Koning-Tijssen, MD, PhD
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          Myoclonus

          • HIROSHI SHIBASAKI, MD, PHDEmeritus Professor, Department of Neurology and Human Brain Research Center, Kyoto University School of Medicine, Kyoto, Japan
          • MARINA A.J. KONING-TIJSSEN, MD, PHDProfessor, Department of Neurology, University Medical Center Groningen, Groningen, The Netherlands

          Myoclonus is defined as sudden, brief, jerky, shocklike, involuntary movements involving the extremities, face, and trunk, without loss of consciousness. Myoclonus is one of the most commonly encountered involuntary movements, and it can be seen in association with a variety of conditions. This review covers  the epidemiology, classification, and diagnosis of myoclonus, as well as a thorough discussion of cortical myoclonus, myoclonus of brainstem origin, myoclonus of spinal cord origin, and myoclonus of undetermined origin. Figures show the principle of jerk-locked back-averaging, example data of jerk-lock averaging, schematic diagrams of cortical reflex myoclonus and spontaneous cortical myoclonus, a polygraphic electromyogram (EMG) recorded from a patient with postanoxic, reticular reflex myoclonus, surface negative slow electroencephalographic (EEG) potentials recorded before psychogenic jerks and voluntary movements mimicking the jerks in a patient with a diagnosis of psychogenic truncal movements, and an EEG-EMG polygraph in a patient with Creutzfeldt-Jakob disease. Tables list the classification of myoclonus based on the estimated site of origin, causes of cortical myoclonus, and causes of progressive myoclonus epilepsy and gene abnormalities.

          Key words: cortical myoclonus, EEG-EMG polygraph, epilepsy syndromes, involuntary movements, myoclonus

          This review contains 6 highly rendered figures, 3 tables, and 63 references.

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        • 4

          Ataxias

          By Hélio A.G. Teive, MD, PhD; Orlando G.P. Barsottini, MD, PhD
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          Ataxias

          • HÉLIO A.G. TEIVE, MD, PHDAssociate Professor of Neurology, Internal Medicine Department, Neurology Service, Ataxia Unit, Universidade Federal do Paraná
          • ORLANDO G.P. BARSOTTINI, MD, PHDAdjunct Professor of Neurology, Department of Neurology and Neurosurgery, Division of General Neurology and Ataxia Unit, Universidade Federal de São Paulo

          Ataxia is a disorder of balance and coordination. The most common forms are cerebellar ataxia and afferent/sensory ataxia. Ataxias can be classified as primary or secondary, as well as hereditary, nonhereditary degenerative, and sporadic. This review covers the primary (congenital, hereditary, and nonhereditary degenerative ataxias) and secondary cerebellar ataxias and afferent/sensory ataxias. Hereditary ataxias are classified as autosomal recessive cerebellar ataxias (such as Friedreich ataxia and ataxia-telangiectasia), autosomal dominant cerebellar ataxias (currently known as spinocerebellar ataxias, such as spinocerebellar ataxia type 3 or Machado-Joseph disease), episodic ataxias, X-linked cerebellar ataxias, and mitochondrial ataxias. Idiopathic degenerative cerebellar ataxias include the cerebellar form of multiple system atrophy (MSA-C) and idiopathic late-onset cerebellar ataxias. Secondary cerebellar ataxias or acquired ataxias include ataxias due to exogenous or endogenous nongenetic causes, such as toxic, paraneoplastic, immune mediated, nutritional, infectious, and secondary to focal injury to the cerebellum. Afferent/sensory ataxia represents a special group of ataxias with many possible causes and is associated with peripheral neuropathies, dorsal root ganglionopathies, sensory nerve root involvement, and posterior spinal column involvement. Figures show several clinical and radiologic (magnetic resonance imaging) signs of autosomal recessive, autosomal dominant ataxias, and multiple system atrophy (type C). Tables list the most common neurologic signs of cerebellar and afferent/sensory ataxias, the main forms of autosomal recessive and autosomal dominant cerebellar ataxias, and the most common causes of secondary cerebellar and afferent/sensory ataxias.

          Key words: afferent/sensory ataxias, ataxias, autosomal recessive cerebellar ataxias, congenital ataxias, nonhereditary degenerative ataxias, secondary cerebellar ataxias, spinocerebellar ataxias

          This review contains 5 highly rendered figures, 4 tables, and 99 references.

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        • 5

          Tremors

          By Alfonso Fasano, MD, PhD; Günther Deuschl, MD, PhD
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          Tremors

          • ALFONSO FASANO, MD, PHDAssociate Professor of Medicine, Division of Neurology, University of Toronto, Morton and Gloria Shulman Movement Disorders Clinic and the Edmond J. Safra Program in Parkinson’s Disease, Toronto Western Hospital, Toronto, ON, Canada
          • GÜNTHER DEUSCHL, MD, PHDProfessor of Neurology, Department of Neurology, Christian-Albrechts-University, University-Hospital Schleswig-Holstein, Kiel, Germany

          Tremor is the most common movement disorder and denotes a rhythmic and involuntary movement of one or several regions of the body. This review covers disease definition, essential tremor, enhanced physiologic tremor, parkinsonian tremor, dystonic tremor, orthostatic tremor, cerebellar tremor, Holmes tremor, neuropathic tremor, palatal tremor, drug-induced and toxic tremors, functional tremor, rare tremor syndromes, tremorlike conditions, and treatment of tremor. Figures show action tremor assessment, the central nervous system circuits of tremor, magnetic resonance imaging findings in specific tremor conditions, general management of tremor patients, an algorithm for the treatment of parkinsonian tremor, and an algorithm for the treatment of dystonic tremor and primary writing tremor. Tables list types of tremor according to the condition of activation, tremor conditions in newborns and during childhood, clinical features of the most common tremor syndromes, motor signs other than tremor and nonmotor features of essential tremor patients, Movement Disorder Society consensus criteria for the diagnosis of essential tremor, genetic and environmental causes of essential tremor, causes of enhanced physiologic tremor, drugs and toxins known to cause tremor, paroxysmal tremors, pseudorhythmic myoclonus in the differential diagnosis of tremor, and pharmacologic management of essential tremor.

          Key words: essential tremor, movement disorder, pathologic tremor, physiologic tremor, tremor

          This review contains 6 highly rendered figures, 7 videos, 11 tables, and 163 references.

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        • 6

          Chorea: Classification, Differential Diagnosis, and Treatment

          By James P. Battista, MD; Ruth H. Walker, MB, ChB, PhD
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          Chorea: Classification, Differential Diagnosis, and Treatment

          • JAMES P. BATTISTA, MDMovement Disorders Division, Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY
          • RUTH H. WALKER, MB, CHB, PHDMovement Disorders Clinic, Department of Neurology, James J. Peters VAMC, Bronx, NY; Movement Disorders Division, Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY

          Chorea is a common hyperkinetic movement disorder that can have a large differential diagnosis. Causes can include genetic and sporadic etiologies, including metabolic abnormalities, autoimmune, structural, paraneoplastic, psychogenic, and iatrogenic causes. The workup can be challenging due to this large number of possible causes, and can include basic metabolic and specialized blood analysis such as genetic testing, in addition to imaging studies and cerebrospinal fluid analysis. Treatment of symptoms outside the realm of reversible causes can be challenging, and is a major focus of current research. This review covers sporadic causes, genetic causes, and management. Figures show a flowchart for evaluation of patients with chorea, a computed tomography scan of a Huntington disease patient showing caudate head atrophy, brain computed tomography scan showing calcification, and acanthocytosis. Videos show mild and moderate Huntington disease, Huntington disease-like 2,  and chorea-acanthocytosis. Tables list metabolic abnormalities that can manifest as chorea, paraneoplastic and nonparaneoplastic syndromes associated with chorea, and reported successful therapies for chorea.

          This review contains 4 highly rendered figures, 4 videos, 3 tables, and 108 references.

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        • 7

          Tics

          By Justyna R. Sarna, MD, PhD ; Tamara Pringsheim, MD, MSc
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          Tics

          • JUSTYNA R. SARNA, MD, PHD Clinical Assistant Professor, Division of Neurology, Department of Clinical Neurosciences, Hotchkiss Brain Institute, University of Calgary, Calgary, AB
          • TAMARA PRINGSHEIM, MD, MSC Assistant Professor, Division of Neurology, Department of Clinical Neurosciences, Hotchkiss Brain Institute, University of Calgary, Calgary, AB

          Tourette syndrome (TS) was originally described and conceptualized by Gilles de la Tourette in 1885. Since then, our understanding of tic disorders has grown immensely and continues to evolve at a rapid pace. Tics are abrupt, usually brief and repetitive, nonrhythmic movements (motor tics) and sounds (vocal tics). Motor tics can be further subdivided into simple and complex motor tics. Simple tics are sudden, meaningless movements most commonly involving eye blinking, facial grimacing, mouth gestures, and shoulder shrugs. Complex motor tics typically involve a series of stereotyped movements that may appear to be purposeful. Vocal (also called phonic) tics are similarly subdivided into simple and complex types. This review covers disease definition/subclassification, epidemiology, etiology/genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, and treatment of tic disorders. Figures show a risperidone safety monitoring template for children, an aripiprazole safety monitoring template for children, and adult antipsychotic safety monitoring recommendations. The video shows how to perform the extrapyramidal symptom rating scale. Tables list classification of tic disorders, medication dosing suggestions, and TS deep brain stimulation guidelines.

          This review contains 3 highly rendered figures, 1 video, 3 tables, and 115 references.

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    • Neurology in General Medicine and Surgery
      • 1

        Neurologic Complications of Cancer

        By Soma Sengupta, MD, PhD; Eudocia Q. Lee, MD, MPH; Patrick Y. Wen, MD, PhD
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        Neurologic Complications of Cancer

        • SOMA SENGUPTA, MD, PHDNeuro-Oncology Fellow, Dana-Farber Cancer Institute, Boston, MA
        • EUDOCIA Q. LEE, MD, MPHInstructor in Neurology, Harvard Medical School, Center for Neuro-Oncology, Dana-Farber Cancer Institute, and Division of Neurology, Department of Neurology, Brigham and Women’s Hospital, Boston, MA
        • PATRICK Y. WEN, MD, PHDProfessor of Neurology, Harvard Medical School, Director, Center for Neuro-Oncology, Dana-Farber Cancer Institute, and Division of Neuro-Oncology, Department of Neurology, Brigham and Women’s Hospital, Boston, MA

        Neurologic complications of cancer can be subdivided into direct, metastatic complications (brain metastases, epidural cord compression, leptomeningeal disease, metastases to peripheral nerve or muscle) or indirect, nonmetastatic complications (infections; iatrogenic, metabolic, vascular, or nutritional effects; paraneoplastic syndromes). This chapter focuses on the medical management of brain tumors, including seizures, peritumoral edema, venous thromboembolism, fatigue and cognitive symptoms, as well as brain metastases, leptomeningeal disease, metastatic epidural spinal cord compression, paraneoplastic syndromes, and examples of more common paraneoplastic syndromes. The section on neurologic complications of cancer therapies covers chemotherapy, radiotherapy, immunotherapies, steroids, and stem cell transplantation. Discussions of other complications of cancer look at strokes and vascular disease, delirium and cognitive sequelae, as well as myelopathy and neuropathy. Figures include brain metastases, leptomeningeal metastases, and radiation necrosis. Tables list types of paraneoplastic syndromes affecting various systems, complications of chemotherapeutic and biologic agents, central nervous system and peripheral nervous system toxicity of chemotherapy, and negative central nervous system effects of radiotherapy.

        This review contains 3 highly rendered figures, 4 tables, and 141 references.

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      • 2

        Metabolic Encephalopathy

        By Matthew McCoyd, MD; Sean Ruland, DO; José Biller, MD, FACP, FAAN, FANA, FAHA
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        Metabolic Encephalopathy

        • MATTHEW MCCOYD, MDAssistant Professor, Department of Neurology, Loyola University Chicago, Stritch School of Medicine, Maywood, IL 60153
        • SEAN RULAND, DOAssociate Professor, Department of Neurology, Loyola University Chicago, Stritch School of Medicine, Maywood, IL 61053
        • JOSÉ BILLER, MD, FACP, FAAN, FANA, FAHAProfessor and Chair, Department of Neurology, Loyola University Chicago, Stritch School of Medicine, Maywood, IL 60153

        Encephalopathy is a general term used to describe diffuse cerebral dysfunction and may be due to myriad primary central nervous system and systemic etiologies. Acute encephalopathy has a rapid onset between hours and days and is commonly due to toxic and metabolic factors that may lead to reversible or permanent cerebral injury. The hallmark of toxic and metabolic encephalopathies is impaired arousal (i.e., hypokinetic delirium), although occasionally an agitated delirium may manifest. Toxic and metabolic encephalopathies may range in severity from the acute confusional state to frank coma. As permanent injury may occur, an organized approach is needed to make an accurate and rapid diagnosis of potentially treatable etiologies. This chapter covers the diagnostic approach to metabolic encephalopathies, specific etiologies (including septic, toxic, and hypoxic-ischemic encephalopathy; electrolyte and acid-base disturbances; and end-organ failure), nutritional deficiencies, and encephalopathy related to endocrine disorders. Figures depict characteristic changes in the posterior brain white matter on imaging, cortical laminar necrosis after severe hypoxic-ischemic insult, osmotic demyelination syndrome of the basis pontis, triphasic frontal slowing, and necrosis of mammillary bodies in a patient with Wernicke encephalopathy. Tables outline the initial management and assessment of patients with acute encephalopathy of uncertain cause, Glasgow Coma Scale, Full Outline of Unresponsiveness (FOUR) score, and West Haven classification for severity of hepatic encephalopathy. 


        This review contains 5 highly rendered figures, 4 tables, and 50 references.

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      • 3

        Uroneurology

        By Jai H. Seth, MBBS, MRCS, BSc, MSc; Jalesh N. Panicker, MBBS, MD, DM, MRCP
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        Uroneurology

        • JAI H. SETH, MBBS, MRCS, BSC, MSCSpecialist Registrar and Research Fellow in Urology, Department of Uro-Neurology, National Hospital for Neurology and Neurosurgery, Institute of Neurology, London, United Kingdom
        • JALESH N. PANICKER, MBBS, MD, DM, MRCPConsultant in Uro-Neurology, Department of Uro-Neurology, National Hospital for Neurology and Neurosurgery, Institute of Neurology, London, United Kingdom

        The function of the pelvic organs, including the lower urinary tract (LUT), is controlled by a complex network of nerves. This leaves patients with neurologic disease vulnerable to LUT and pelvic organ dysfunction. Physicians often come across urogenital complaints in their patients with neurologic disease, the symptoms of which can result in significant distress and loss of dignity and quality of life. Due to the health and economic burden that accompanies neurogenic pelvic organ dysfunction, it is important for clinicians to understand the common forms of dysfunction, essential investigations, and modes of management. This chapter covers bladder dysfunction from a physician’s perspective. Topics include neurologic control of the LUT, large bowel, and sexual functions; male and female sexual response; neurogenic bladder dysfunction and its management; diagnostic evaluation; management of neurogenic sexual dysfunction; management of erectile dysfunction; ejaculatory dysfunction; sexual dysfunction in women; and fecal incontinence. Figures illustrate efferent innervation of the LUT, neurologic detrusor overactivity, a urethral pressure profile in a patient with Fowler syndrome, an example bladder diary, an example bladder scan, and normal and obstructed flow patterns. Tables list common causes of injury at the suprapontine, suprasacral, and infrasacral levels and storage and voiding systems.

        This review contains 6 highly rendered figures, 2 tables, and 53 references.

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      • Neurologic Infectious Diseases
        • 1

          Encephalitis

          By Shamik Bhattacharyya, MD; Jennifer L. Lyons, MD
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          Encephalitis

          • SHAMIK BHATTACHARYYA, MDDivision of Neurological Infections, Department of Neurology Brigham and Women’s Hospital and Harvard Medical School Boston, MA
          • JENNIFER L. LYONS, MDDivision of Neurological Infections, Department of Neurology Brigham and Women’s Hospital and Harvard Medical School Boston, MA

          In 2013, the International Encephalitis Consortium proposed clinical criteria for acute encephalitis consisting of 24 hours of altered level of consciousness, lethargy, or personality change and at least three additional supportive features. Although viruses are the most common cause of acute encephalitis, not all encephalitides are acute, viral, or even infectious. Chronic encephalitis can be pathologically similar to acute encephalitis, but the causative agents and clinical manifestations vary. Management of encephalitis is largely supportive; however, for many common encephalitides primary preventive approaches exist. This module reviews the epidemiology, manifestations, diagnosis, management, and prevention of various encephalitides, including herpes family encephalitis (herpes simplex virus, varicella-zoster virus, cytomegalovirus, human herpesvirus 6), arbovirus encephalitis (West Nile virus, eastern equine encephalitis virus, tick-borne encephalitis virus, Japanese encephalitis virus), other encephalitides associated with viruses (influenza virus, human immunodeficiency virus, John Cunningham virus, rabies virus), encephalitides associated with bacteria (Mycoplasma pneumonia, Listeria monocytogenes), and autoimmune encephalitis (acute disseminated encephalomyelitis, paraneoplastic and other autoimmune encephalitides, immune reconstitution inflammatory syndrome). Tables include the International Encephalitis Consortium’s supportive feature of encephalitis, differential diagnosis for magnetic resonance imaging (MRI) findings in the patient with suspected encephalitis, diagnostic considerations for triaging workup of infection-associated encephalitis, differential diagnosis of arboviral infections by location of travel or residence, and preventive strategies for select infectious encephalitis. Figures include MRIs showing patients with herpes simplex encephalitis, varicella-zoster virus, eastern equine encephalitis, HIV, Listeria monocytogenes, acute disseminated encephalomyelitis, acute hemorrhagic leukoencephalitis, and immune reconstitution inflammatory syndrome.

          This module contains 8 highly rendered figures, 5 tables, 78 references, and 5 MCQs.

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        • 2

          Acute Viral Meningitis

          By Karen L. Roos, MD; Jared R. Brosch, MD
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          Acute Viral Meningitis

          • KAREN L. ROOS, MDJohn and Nancy Nelson Professor of Neurology and Professor of Neurological Surgery, Indiana University School of Medicine, Indianapolis, IN
          • JARED R. BROSCH, MDDeptment of Neurology, Assistant Professor of Neurology, Indiana University School of Medicine, Indianapolis, IN

          Acute viral meningitis refers to inflammation of the meninges of the brain in response to a viral pathogen. Viruses cause meningitis, encephalitis, myelitis, or a combination of these, meningoencephalitis or encephalomyelitis. Viral meningitis is typically a self-limited disorder with no permanent neurologic sequelae. This chapter reviews the epidemiology, etiology, diagnosis, differential diagnosis, treatment, complications, and prognosis. Tables describe Wallgren’s criteria for aseptic meningitis, important arboviral infections found in North America, herpes family viruses and meningitis, classic cerebrospinal fluid (CSF) abnormalities with viral meningitis, Centers for Disease Control and Prevention criteria for confirming arboviral meningitis, basic CSF studies for viral meningitis, and etiology of CSF pleocytosis. Figures depict common causes of viral meningitis, nuchal rigidity, examination for Kernig sign, and Brudzinski sign for meningeal irritation.

          This review contains 4 highly rendered figures, 7 tables, and 16 references.

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        • 3

          Acute Bacterial Meningitis

          By Karen L. Roos, MD
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          Acute Bacterial Meningitis

          • KAREN L. ROOS, MDJohn and Nancy Nelson Professor of Neurology and Professor of Neurological Surgery, Indiana University School of Medicine, Indianapolis, IN

          Acute bacterial meningitis is a life-threatening infection. By definition, meningitis is an infection of the meninges and the subarachnoid space. Bacterial meningitis is associated with an inflammatory response that involves the meninges, the subarachnoid space, the brain parenchyma, and the cerebral arteries and veins. As such, bacterial meningoencephalitis is the more accurate descriptive term. This chapter discusses the epidemiology, etiology, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, complications, and prognosis of the disease. The discussion of diagnosis covers clinical manifestations, physical examination findings, laboratory tests, and imaging studies. The discussion of treatment covers empirical therapy, specific antimicrobial therapy, and dexamethasone therapy. Graphs compare causative organisms and clinical manifestations of community-acquired meningitis. Illustrations depict proper patient positioning for detecting nuchal rigidity, Kernig sign, Brudzinski sign, and lumbar puncture, as well as a sagittal view of a lumbar puncture needle as it is advanced into the subarachnoid space. An algorithm delineates the approach to the patient with symptoms and signs of bacterial meningitis. Tables outline bacterial pathogens based on predisposing and associated conditions, cerebrospinal fluid diagnostic studies for meningitis, the appearance of the organism on a Gram stain, empirical antimicrobial therapy based on predisposing and associated conditions, recommendations for specific antibiotic therapy in bacterial meningitis, and recommended doses for antibiotics commonly used in the treatment of bacterial meningitis.

           

          This review contains 8 highly rendered figures, 6 tables, and 75 references.

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        • 4

          Brain and Spinal Abscesses

          By Allan R. Tunkel, MD, PhD; W. Michael Scheld, MD
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          Brain and Spinal Abscesses

          • ALLAN R. TUNKEL, MD, PHDProfessor of Medicine, Drexel University College of Medicine, Philadelphia, PA, Chair, Department of Medicine, Monmouth Medical Center, Long Branch, NJ
          • W. MICHAEL SCHELD, MDProfessor of Medicine and Neurosurgery, University of Virginia School of Medicine, Charlottesville, VA

          Brain and spinal abscesses are often devastating infections that can lead to substantial morbidity and mortality if not recognized and treated in a timely manner. The clinical presentation depends upon the route of spread of infection to the central nervous system, location of the lesion, and severity of increased intracranial pressure. Brain abscess is defined as a focal intracranial infection that is initiated as an area of cerebritis and evolves into a collection of pus that is surrounded by a vascularized capsule; patients most often develop brain abscess by contiguous spread, hematogenous dissemination, or trauma. This chapter discusses the epidemiology, etiology, pathogenesis, diagnosis, management, and prognosis for brain abscess. Also examined are epidemiology and pathogenesis, etiology, diagnosis, management, and prognosis for both cranial subdural empyema and epidural abscess and spinal epidural abscess and subdural empyema. Tables list predisposing conditions and likely etiologic agents in brain abscess; histopathologic findings in the stages of brain abscess formation; presenting symptoms and signs in patients with brain abscess; antimicrobial therapy of brain abscess based on isolated pathogen; predisposing conditions and empirical antimicrobial therapy in patients with presumed bacterial brain abscess; recommended dosages of antimicrobial agents in adults with brain abscess; and normal renal and hepatic function. Figures in this chapter are images of intra-axial fluid collection; a subdural fluid collection; a ring-enhancing subdural empyema; an epidural abscess; a large epidural fluid collection with midline shift; a dorsal epidural collection; and loculated ring enhancement suggesting an epidural abscess. This chapter contains 6 highly rendered figures, 6 tables, 167 references, 5 MCQs.

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        • 5

          Central Nervous System Diseases Due to Slow Viruses and Prions

          By Francisco González-Scarano, MD
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          Central Nervous System Diseases Due to Slow Viruses and Prions

          • FRANCISCO GONZÁLEZ-SCARANO, MD

          Several central nervous system diseases whose common elements include a long incubation period and a progressive clinical course were once called slow virus infections, because most of them are in fact caused by viruses. However, one group of these CNS diseases is now believed to be caused by abnormally configured proteins known as prions; rather than an etiologic designation, therefore, on the whole these diseases are better characterized by their chronicity, their transmissibility, and at this point, their inexorably deteriorating natural history. This chapter reviews the more common of these: HIV-associated dementia (HAD or HIVD), human T cell lymphotropic virus type 1 (HTLV-1)-associated myelopathy, Creutzfeldt-Jakob disease (CJD), progressive multifocal leukoencephalopathy (PML), and subacute sclerosing encephalitis (SSPE), which is associated with a variant of measles virus. Figures illustrate the pathogenesis and the pathology of HIV dementia, propagation of scrapie prion protein (PrP) in brain neurons, and spongiform brain changes of CJD. Tables list the stages of HAD and the clinical and pathologic characteristics distinguishing classic CJD and varient CJD.

          This review contains ­5 highly rendered figures, 2 tables, and 57 references. 

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        • 6

          Primary and Metastatic Central Nervous System Malignancies

          By Fabio M. Iwamoto, MD; Howard A. Fine, MD
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          Primary and Metastatic Central Nervous System Malignancies

          • FABIO M. IWAMOTO, MDAssistant Clinical Investigator, Neuro-Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD
          • HOWARD A. FINE, MDSenior Investigator and Chief, Neuro-Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD

          About 13,000 deaths each year in the United States are attributed to primary central nervous system (CNS) malignancies. An estimated 20% of patients with cancer eventually develop clinically apparent CNS metastases, and an estimated 170,000 cases of brain metastases are diagnosed in the United States yearly. Autopsy studies suggest that as many as 50% of patients dying from advanced cancer may have metastasis to the CNS. This chapter provides an overview of primary and metastatic CNS malignancies with in-depth discussion of gliomas, primary CNS lymphoma, meningioma, brain metastases, leptomeningeal metastases, and metastatic epidural spinal cord compression. Discussions cover epidemiology, etiology, diagnosis, and treatment of gliomas, including surgery, radiotherapy, and chemotherapy for both newly diagnosed gliomas and recurrent gliomas. The epidemiology, diagnosis, treatment and prognosis for primary CNS lymphomas are reviewed, as well as the epidemiology, etiology, diagnosis, treatment, and prognosis for meningiomas. Epidemiology, diagnosis, and prognosis for brain metastases are briefly discussed, and the section on treatment includes surgery, stereotactic radiosurgery, and whole-brain radiotherapy for patients with three or fewer brain metastases. The sections on leptomeningeal metastases and metastatic epidural spinal cord compression cover diagnosis, treatment, and prognosis. This chapter contains 126 references.

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    • Special Topics in Neurology
      • 1

        Women's Neurology

        By M. Angela O’Neal, MD
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        Women's Neurology

        • M. ANGELA O’NEAL, MDDirector of Women’s Neurology, Department of Neurology, Brigham and Women’s Hospital, Boston, MA

        This review details some of the features important to consider in women with neurologic disease, including medication and disease effects on both reproductive health and pregnancy/fetal development, as well as hormonal effects on neurologic disease. A case-based approach is used to discuss diseases that affect women throughout their life cycle (multiple sclerosis [MS] and epilepsy), disorders that affect only women (eclampsia), and those that affect women preferentially (migraine, cerebral venous thrombosis, reversible cerebral vasospasm, and Alzheimer disease). The epidemiology, differential diagnosis, pathophysiology, management, and prognosis are reviewed for each disorder. Tables include US Food and Drug Administration pharmaceutical pregnancy categories, learning objectives, migraine with aura, alternative diagnostic criteria for migraine without aura, migraine aura versus transient ischemic attacks, red flags to secondary headache, abortive headache therapy in pregnancy, migraine preventive medications and pregnancy, MS therapies in pregnancy, pregnancy consulting points for MS patients on a disease-modifying therapy, and general recommendations for women with epilepsy and pregnancy. Figures show fluid-attenuated inversion recovery and gradient echo magnetic resonance images of the right anterior parietal region; a magnetic resonance venogram demonstrating occlusion in the superior sagittal sinus; posterior reversible encephalopathy syndrome; and computed tomographic (CT) images of a hemorrhage in the left parietal region, left frontal subarachnoid bleeding, and left middle cerebral beading.

        This review contains 5 highly rendered figures, 11 tables, and 29 references.

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      • 2

        Principles of Neurologic Ethics

        By Thomas I. Cochrane, MD, MBA
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        Principles of Neurologic Ethics

        • THOMAS I. COCHRANE, MD, MBAAssociate Neurologist, Division of Neuromuscular Disease, Department of Neurology, Brigham and Women’s Hospital, Assistant Professor of Neurology, Harvard Medical School, Boston, MA

        Many neurologic diseases affect the brain and thus cognition and personality. Many people consider some neurologic conditions “worse than death” and would prefer not to be kept alive using medical technology if there is no reasonable chance of recovery. The clinician caring for patients with neurologic disease will frequently be confronted with decisions about whether to initiate or continue life-sustaining therapies. And because neurologic disease often impairs the ability to understand and to decide, clinicians are more often called on to make decisions for patients who cannot decide for themselves. This chapter covers general principles, including respect for autonomy, beneficence, nonmaleficence, justice, informed consent, and implied consent. Discussion about making decisions for others includes competence and decision-making capacity, advance directives (powers of attorney and living wills), substituted judgment, and best interests. Decisions about life-sustaining treatment include withholding versus withdrawing, acts versus omissions, and limiting life-sustaining treatment for the patient with no surrogate. Also covered are decisions in the face of prognostic uncertainty, and futility, as well as commonly encountered problems such as states of severely disordered consciousness, coma, vegetative state, minimally conscious state, and brain death (including ethical controversies). Organ donation after both brain death and cardiac death is discussed. The section on dementia covers feeding tubes for patients with advanced dementia, the locked-in state, and neuroenhancement. Tables include elements of informed consent, elements of competence, assumed ethical priority of potential surrogates, steps that aid decision making in the face of prognostic uncertainty, and the American Academy of Neurology’s criteria for determination of brain death. This chapter includes 23 references.

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      • 3

        The Neurologic Examination

        By Nicholas J. Beimer, MD; Douglas J. Gelb, MD, PhD
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        The Neurologic Examination

        • NICHOLAS J. BEIMER, MDClinical Instructor, Department of Neurology, University of Michigan, Ann Arbor, MI
        • DOUGLAS J. GELB, MD, PHDProfessor, Department of Neurology, University of Michigan, Ann Arbor, MI

        All physicians, regardless of their medical specialty or the setting in which they treat patients, must be able to perform a neurologic examination. In the outpatient office, up to 9 to 10% of all symptoms suggest the possibility of neurologic disease and up to 5% of emergency department visits are due to primary neurologic disease. The neurologic examination is critical in triaging these patients, selecting diagnostic tests, and indicating management. This review covers how to think about the neurologic examination, the screening examination, and diagnosis-focused neurologic examinations with an emphasis on stroke, epilepsy, encephalopathy and coma, neurodegenerative diseases, neuromuscular disease, and functional disorders. The figure shows a conceptual approach to the neurologic examination. The tables list components of the screening neurologic examination, neurologic examination focus points for suspected stroke and suspected epilepsy, lateralization and localization of common seizure semiologies, and neurologic examination focus points for encephalopathy/coma, suspected neurodegenerative disease, suspected neuromuscular disease, and suspected functional neurologic disorders.

        This review contains 1 highly rendered figure, 8 tables, and 14 references.

        Key words: Neurologic examination, neurodegenerative disease, neuromuscular disease, neurologic screening

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  • Oncology
    • Principles of Oncology
      • 1

        Cancer Epidemiology and Prevention

        By Alfred I. Neugut, MD, PhD, FACP; David P. H. Wu, MD
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        Cancer Epidemiology and Prevention

        • ALFRED I. NEUGUT, MD, PHD, FACPMyron Studner Professor of Cancer Research, Professor of Medicine and Public Health, Columbia University Medical Center, New York, NY
        • DAVID P. H. WU, MDPostdoctoral Clinical Fellow in Hematology Oncology, Columbia University Medical Center, New York, NY

        Recently surpassing heart disease, cancer is now the leading cause of death (one in four) in the United States. Worldwide, cancer control is becoming increasingly important as life expectancy improves because of lower infant mortality and fewer deaths from infectious diseases. Morbidity and mortality from many forms of cancer can be controlled through primary or secondary prevention. Primary prevention can be defined as risk modification to lower cancer occurrence. Secondary prevention refers to the use of screening tests to detect cancers at early stages. Environmental carcinogens, inherited factors that predispose to cancer, and screening and early detection are covered in major sections. Also included are discussions of infectious agents, occupational carcinogens, iatrogenic causes, carcinogens affecting the reproductive system, and miscellaneous environmental causes. Tables outline established causes of human cancer, common hereditary cancers and syndromes attributable to germline mutations in predisposing genes, and the American Cancer Society’s recommendations for early detection of cancer. This chapter contains 138 references.

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      • 2

        Molecular Genetics of Cancer

        By Leif W. Ellisen, MD, PhD
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        Molecular Genetics of Cancer

        • LEIF W. ELLISEN, MD, PHDAssociate Professor of Medicine, Harvard Medical School, Boston, MA

        The uncontrolled clonal expansion of a cell, which often leads to invasion of surrounding tissues and metastatic spread, produces cancer. A clear histologic and molecular genetic evolution from precancerous lesions to frankly malignant and invasive cancer has been defined for some tumors (e.g., colon and bladder cancers). In rare cases, mutations may occur and be passed on in the germline, resulting in genetic predisposition to cancer (i.e., familial cancer syndromes). Environmental factors are also thought to contribute to the development of cancer. Interactions between environmental factors and subtle germline genetic variations that distinguish individuals may in some cases constitute an important determinant of cancer risk within the general population. Finally, viral infection has been linked to the development of specific cancers. Oncogenes and proto-oncogenes, and germline genetic analysis and cancer risk assessment are covered. Also discussed are genetic alterations and abnormalities, tumor suppressor genes, tumor progression, genetic mechanisms of treatment sensitivity and resistance, and emerging trends in cancer genomics and risk assessment. Figures illustrate activation of proto-oncogenes, the Knudsen two-hit model of tumor initiation, allelic losses in tumors, the retinoblastoma gene (RB1) cell cycle pathway, the p53 cellular stress and DNA damage response pathway, microsatellite instability and DNA mismatch repair, multiple oncogenes and tumor suppressors, tumor progression, cellular senescence and telomerase activation, tumor angiogenesis, chemotherapy drug resistance, targeting of oncogenic proteins by imatinib mesylate, analysis of expression profiles using high-density microarrays, and the spectrum of risk alleles for breast cancer predisposition. Tables outline oncogene and tumor suppressor gene mutations. This chapter contains 119 references.

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      • 3

        Principles of Cancer Treatment

        By Eric H. Rubin, MD; William N. Hait, MD, PhD
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        Principles of Cancer Treatment

        • ERIC H. RUBIN, MDTherapeutic Area Head, Merck Research Laboratories, North Wales
        • WILLIAM N. HAIT, MD, PHDOrtho Biotech Oncology Senior Vice President and Worldwide Head, Oncology Research and Development, Johnson & Johnson, Raritan, NJ

        This chapter is divided into three primary sections dealing with management of cancer patients, the specific basis of cancer treatment, and specific chemotherapeutic agents. The first section outlines the diagnosis, staging, performance status, and treatment of cancer. The discussion of the specific basis of cancer treatment describes cancer biology (including its transformation and proliferation, cell viability and cell death, and invasion and metastases) and the principles of cancer pharmacology. The discussion on pharmacology details dose response and schedule, drug resistance, combination chemotherapy, common toxicities, and pharmacokinetics and pharmacogenetics. Among the specific chemotherapeutic agents discussed are drugs that alter nucleic acid synthesis or function (including DNA alkylating and cross-linking agents, inhibitors of nucleic acid synthesis, and DNA topoisomerase inhibitors); antimicrotubule drugs (eg, vinca alkaloids, taxanes, and estramustine); drugs that affect growth factors, receptors, and signal transduction pathways; drugs that inhibit metastases or angiogenesis; gene-based therapies; and immunotherapies. Tables describe the World Health Organization Performance Scale and chemotherapeutic agents, and figures illustrate targets for new cancer therapeutics and mechanisms of chemotherapeutic drug resistance. This chapter contains 90 references.

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      • 4

        Emergencies in Hematology and Oncology

        By Thorvardur R. Halfdanarson, MD; William J. Hogan, MBBCH; Timothy J. Moynihan, MD
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        Emergencies in Hematology and Oncology

        • THORVARDUR R. HALFDANARSON, MDAssistant Professor, University of Iowa Hospitals and Clinics, Department of Internal Medicine, Division of Hemtology, Oncology and Blood & Marrow Transplantation, Iowa City, IA
        • WILLIAM J. HOGAN, MBBCHAssistant Professor, Division of Hematology, Mayo Clinic, Rochester, MN
        • TIMOTHY J. MOYNIHAN, MDDepartment of Oncology, Mayo Clinic, Rochester, MN

        With the rising incidence of malignancies and expanding treatment options, clinicians must learn to recognize and treat emergencies associated with them. Oncologic emergencies can be broadly classified according to organ systems, which can facilitate recognition and management. Pathophysiology, presentation, diagnosis, and treatment are discussed for complications categorized by metabolic emergencies (hypercalcemia of malignancy, tumor lysis syndrome, lactic acidosis), neurologic emergencies (malignant spinal cord compression, brain metastases, and increased intracranial pressure), cardiovascular emergencies (malignant pericardial effusion and tamponade, superior vena cava syndrome), hematologic emergencies (hyperviscosity due to monoclonal proteins, hyperleukocytosis and leukostasis), infectious emergencies (neutropenic fever, neutropenic enterocolitis, fever associated with splenectomy or functional asplenia), and pulmonary emergencies (acute airway obstruction, acute airway hemorrhage). Figures illustrate spinal cord compression, brain metastases, electrical alternans, malignant pericardial effusion, superior vena cava syndrome, hyperleukocytosis, and an algorithm for initial management of fever and neutropenia. Tables cover management of hypercalcemia of malignancy; the Cairo-Bishop definition of laboratory and clinical tumor lysis; grading, risk stratification, and management of tumor lysis syndrome; management of intracranial hypertension and seizures; infection in patients with neutropenic fever; the Multinational Association for Supportive Care in Cancer Risk Index; and indications for the addition of a gram-positive antibiotic to the initial empirical regimen. This chapter contains 181 references.

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      • 5

        Tumor Immunology

        By Bruce G. Redman, DO; Alfred E. Chang, MD, FACS
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        Tumor Immunology

        • BRUCE G. REDMAN, DOProfessor of Medicine, University of Michigan Comprehensive Cancer Center, Ann Arbor, MI
        • ALFRED E. CHANG, MD, FACSHugh Cabot Professor of Surgery, University of Michigan Medical School, Ann Arbor, MI

        The development of cancer may represent a failure of immunosurveillance—a theory founded on the premise that the immune system has the capacity to recognize tumor-associated antigens and develop specific T cell responses to those antigens. The ability to intervene and enhance the immune system to achieve a beneficial antitumor response remains an area of intense clinical research. Considerable progress has been made in expanding our knowledge of the targets for an immune response and about the full repertoire of cellular and humoral constituents involved in the generation of an effective antitumor response. Tumor cells display a variety of mechanisms by which they evade immune detection and destruction and render the immune response ineffective. With a more complete understanding of these escape mechanisms, clinical investigators are devising strategies to enhance the development of a robust immune response in the tumor-bearing host (active tumor immunity) or by the adoptive transfer of activated effector cells or tumor-specific antibodies into the tumor-bearing host (passive tumor immunity). Illustrations depict immune-mediated rejection of transplanted tumors, T cell interaction with a dendritic antigen-presenting cell (APC), interaction between APCs and T cells, and novel immunotherapies. Tables list host immune response evidence and five major cellular constituents of cellular effector response.

        This review contains 8 highly rendered figures, 2 tables, 50 references, 1 teaching slide set, and 5 MCQs.

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    • Respiratory Tract Malignancies
      • 1

        Head and Neck Cancer

        By Lucy F. Chen, MD; Everett E. Vokes, MD
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        Head and Neck Cancer

        • LUCY F. CHEN, MDFellow, Department of Medicine, Section of Hematology / Oncology, University of Chicago, Chicago, Illinois
        • EVERETT E. VOKES, MDChairman, Department of Medicine, Deputy Director, Cancer Research Center, John E. Ultmann Professor of Medicine Radiation and Cellular Oncology, University of Chicago, Chicago, Illinois

        The vast majority of head and neck tumors arise from the mucosa of the upper aerodigestive tract and are squamous cell carcinomas. Anatomically, head and neck cancers are heterogeneous—arising from the pharynx (including the nasopharynx, oropharynx, and hypopharynx), the oral cavity, the larynx, and the cervical esophagus. The etiology, pathogenesis, genetic alterations, diagnosis, staging, and treatment of squamous cell carcinomas of the head and neck are reviewed in this chapter. Salivary gland tumors arise either from one of the three major salivary glands or from the minor salivary glands lining the mucosa of the upper aerodigestive tract. Their etiology, histology, and treatment differ from those of head and neck tumors and are briefly reviewed. Figures depict the sagittal section of the upper aerodigestive tract and the trial design for the intergroup organ preservation study in laryngeal cancer. This chapter has 74 references.

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      • 2

        Lung Cancer

        By Jeffrey Crawford, MD; John Strickler, MD
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        Lung Cancer

        • JEFFREY CRAWFORD, MDGeorge Barth Geller Professor for Research in Cancer, Chief, Division of Medical Oncology, Department of Medicine, Duke University Medical Center, NC 27710
        • JOHN STRICKLER, MDFellow, Divisions of Hematology, Medical Oncology, and Cellular Therapy, Department of Medicine, Duke University Medical Center, Durham, NC 27710

        In the United States, lung cancer is the second most common cancer, surpassed only by prostate cancer in men and breast cancer in women. But lung cancer is the leading cause of cancer deaths, accounting for 29% and 26% of all cancer-related deaths in men and women, respectively. The four major pathologic cell types of lung cancer are small cell carcinoma, adenocarcinoma, squamous cell carcinoma, and large cell carcinoma. Because they have overlapping clinical behaviors and responses to treatment, adenocarcinoma, squamous cell carcinoma, and large cell carcinoma are generally grouped together in the category of non–small cell lung cancer (NSCLC). This chapter discusses both NSCLC and small cell lung cancer (SCLC), including lung cancer in those who have never smoked, prevention of lung cancer, with sections on diagnosis, biomarkers, treatment, and supportive care. Tables provide further detail regarding signs and symptoms of lung cancer; prognostic biomarkers, predictive biomarkers, and therapy for NSCLC; survival benefit of adjuvant chemotherapy by stage; chemotherapy regimens for adjuvant therapy established from randomized trials; and therapy and prognosis for SCLC. Figures illustrate various cancer death rates, percentages of high school students who smoke, common causes of cancer death, differential frequencies of EGFR and K-ras mutations, a tumor-nodes-metastasis four-stage system used in clinical and surgical evaluation of lung cancer, and lymph node sites (nodal stations) in the chest. This chapter contains 181 references.

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    • Gastrointestinal Malignancies
      • 1

        Colorectal Cancer

        By Cathy Eng, MD, FACP
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        Colorectal Cancer

        • CATHY ENG, MD, FACPAssociate Professor, Associate Director of the Colorectal Center, The University of Texas M.D. Anderson Cancer Center, Houston, TX

        Colorectal cancer is the third most common cancer and the second leading cause of cancer death in the United States. Although environmental factors, including diet and lifestyle, clearly play a role in the etiology of colorectal cancer, as many as 25% of patients with colorectal cancer have a family history of the disease, which suggests the involvement of a genetic factor. Inherited colon cancers can be divided into two main types: the well-studied but rare familial adenomatous polyposis (FAP) syndrome, and the increasingly well-characterized, more common hereditary nonpolyposis colorectal cancer (HNPCC, a.k.a. Lynch Syndrome). The prevention, screening, diagnosis, and treatment of cancers of the colon and rectum are covered in this chapter. Figures illustrate various forms of adenomatous polyps, the tumor, node, metastasis (TNM) staging system for colorectal cancer, and the five-year survival rate in patients with colorectal carcinoma. Tables describe risk factors; possible chemopreventive agents; evidence supporting the effectiveness of screening tests; features and usage issues with different fecal occult blood tests; recommendations for early detection, screening, and surveillance for patients at different levels of risk; colorectal cancer staging systems; indicators of poor prognosis; and chemotherapeutic and biologic agents in the treatment of colorectal cancer. This chapter contains 193 references.

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      • 2

        Pancreatic, Gastric, and Other Gastrointestinal Cancers

        By Weijing Sun, MD; Daniel Haller, MD, FACP; Davendra Sohal, MD, MPH
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        Pancreatic, Gastric, and Other Gastrointestinal Cancers

        • WEIJING SUN, MDAssociate Professor of Medicine, Division of Hematology-Oncology, Department of Medicine, Abramson Cancer Center, University of Pennsylvania, Philadelphia, PA
        • DANIEL HALLER, MD, FACPProfessor of Medicine, Division of Hematology-Oncology, Department of Medicine, Abramson Cancer Center, University of Pennsylvania, Philadelphia, PA
        • DAVENDRA SOHAL, MD, MPHFellow, Division of Hematology-Oncology, Department of Medicine, University of Pennsylvania, Abramson Cancer Center, Philadelphia, PA

        According to 2009 estimates from the American Cancer Society, cancers originating in the gastrointestinal tract rank second in both incidence and cancer-related deaths. One in four deaths in the United States is caused by cancer, with 25% of cancer-related deaths caused by gastrointestinal (GI) malignancies; more than 50% of these deaths are caused by cancer of the pancreas, stomach, esophagus, liver, or biliary tract. Recent advances in molecular biology, medical genetics, and imaging and endoscopic techniques, as well as the development of antitumor agents, have significantly altered the approaches to the prevention, diagnosis, and treatment of GI cancers. The chapter covers esophageal, gastric, pancreatic, hepatocellular, biliary tract, and anal cancers, as well as GI stromal tumors and gastric lymphoma. Coverage of all cancers includes diagnosis and treatment; various sections include information on epidemiology, etiology, risk factors, screening and prevention, molecular mutations, pathogenesis, and/or metastatic disease. Figures depict a barium esophagogram showing squamous cell carcinoma; imaging of esophageal cancer, gastric cancer, and pancreatic cancer; a pedigree of a family with inactivation of germline mutation of E-cadherin; hereditary gastric cancer; gastric cancer survival rates after gastrectomy; axial T1-weighted magnetic resonance imaging (MRI) showing cancer of the pancreatic head; and T1- and T2-weighted MRIs of intrahepatic bile duct carcinoma. Tables provide information on new cases and mortality from GI cancer in 2009; guidelines for diagnosis and surveillance of Barrett esophagus; the declining incidence of gastric cancer in Japan, Slovenia, and the United States; TNM staging of gastric cancer, pancreatic cancer, and hepatocellular carcinoma; the incidence of familial pancreatic carcinoma; molecular mutations involved in pancreatic cancer; staging of pancreatic intraepithelial neoplasia; and the Chinese University Prognostic Index. This chapter contains 235 references.

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    • Breast, Gynecologic, and Genitourinary Malignancies
      • 1

        Breast Cancer

        By Nancy E. Davidson, MD
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        Breast Cancer

        • NANCY E. DAVIDSON, MDDirector, University of Pittsburgh Cancer Institute and UPMC Cancer Center, Hillman Professor of Oncology, University of Pittsburgh, Pittsburgh, PA

        Invasive breast cancer, the most common nonskin cancer in women in the United States, will be diagnosed in 235,000 women in this country in 2013 and is expected to result in approximately 40,000 deaths. Incidence and mortality reached a plateau and appear to be dropping in both the United States and parts of western Europe. This decline has been attributed to several factors, such as early detection through the use of screening mammography and appropriate use of systemic adjuvant therapy, as well as decreased use of hormone replacement therapy. However, the global burden of breast cancer remains great, and global breast cancer incidence increased from 641,000 in 1980 to 1,643,000 in 2010, an annual rate of increase of 3.1%. This chapter examines the etiology, epidemiology, prevention, screening, staging, and prognosis of breast cancer. The diagnoses and treatments of the four stages of breast cancer are also included. Figures include algorithms used for the systemic treatment of stage IV breast cancer and hormone therapy for women with stage IV breast cancer. Tables describe selected outcomes from the National Surgical Adjuvant Breast and Bowel Project (NSABP) P-1 and P-2 chemoprevention trials, tamoxifen chemoprevention trials for breast cancer, the TNM staging system and stage groupings for breast cancer, some commonly used adjuvant chemotherapy regimens, an algorithm for suggested treatment for patients with operable breast cancer from the 2011 St. Gallen consensus conference, guidelines for surveillance of asymptomatic early breast cancer survivors from the American Society of Clinical Oncology, and newer agents for metastatic breast cancer commercially available in the United States.

        This review contains 2 highly rendered figures, 8 tables, and 108 references.

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      • 2

        Gynecologic Cancer

        By Stephen A. Cannistra, MD; Christina I. Herold, MD
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        Gynecologic Cancer

        • STEPHEN A. CANNISTRA, MDProfessor of Medicine, Harvard Medical School, Director, Gynecologic Medical Oncology, Beth Israel Deaconess Medical Center, Boston, MA
        • CHRISTINA I. HEROLD, MDInstructor of Medicine, Harvard Medical School, Gynecologic Medical Oncology, Beth Israel Deaconess Medical Center, Boston, MA

        This chapter focuses on the three types of gynecologic cancer—epithelial cancer of the ovary, cancer of the uterine cervix, and cancer of the endometrium (uterine cancer)—and reviews their epidemiology, diagnosis, differential diagnosis, surgical features, and staging, as well as their risk factors and clinical features. Also discussed are methods of treatment and the management of relapse. Epithelial ovarian cancer occurs at a mean age of 60 years in the United States and is the most lethal of gynecologic tract tumors. However, a recent trial has demonstrated a survival advantage through the use of intraperitoneal chemotherapy for appropriate patients with optimally debulked ovarian cancer.

        Invasive cervical cancer is uncommon in developed countries, partly because of the effectiveness of Pap smear screening. Nevertheless, cancer of the uterine cervix is the third most common gynecologic cancer diagnosis and cause of death among gynecologic cancers in the United States. However, for women with early-stage cervical cancer, data from several randomized trials indicate an improvement in response rate and survival through the use of combination platinum-based regimens for platinum-sensitive relapse. Also noted is an improvement in survival using combined-modality chemoradiation in appropriate patients with locally advanced cervical cancer.

        Endometrial cancer is the most frequent tumor of the gynecologic tract; it is estimated that it occurred in over 46,000 women and caused more than 8,000 deaths in the United States in 2011. Recent data indicate improvement in survival using adjuvant platinum-based chemotherapy in appropriate patients with high-risk endometrial cancer.

        Tables in this chapter review the common histologic types of epithelial ovarian cancer, selected signs and symptoms of ovarian cancer, the International Federation of Gynecology and Obstetrics (FIGO) staging system for epithelial ovarian cancer, differential diagnosis of a complex cyst detected by transvaginal sonography, selected adverse prognostic factors in epithelial ovarian cancer, common chemotherapy agents used in the treatment of epithelial ovarian cancer, the FIGO surgical staging of endometrial cancer, and postoperative management considerations for patients with uterine cancer. Figures illustrate the four histologic subtypes of epithelial ovarian cancer, the intraoperative appearance of stage III epithelial ovarian cancer, and FIGO staging of cervical cancer.

        This review contains 6 highly rendered figures, 8 tables, and 150 references.

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      • 3

        Prostate Cancer

        By Jonathan E. Rosenberg, MD; Philip W. Kantoff, MD
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        Prostate Cancer

        • JONATHAN E. ROSENBERG, MDAssistant Professor of Medicine, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA
        • PHILIP W. KANTOFF, MDProfessor of Medicine, Dana-Faber Cancer Institute, Harvard Medical School, Boston, MA

        Prostate cancer is the most commonly diagnosed noncutaneous malignancy in men in the United States. This chapter discusses the epidemiology, pathogenesis, and diagnosis of prostate cancer, as well as risk factors, the use of digital rectal examination and prostate-specific antigen measurement for screening, and staging for the disease. Also reviewed are the natural history of untreated prostate cancer; the treatment of localized and advanced prostate cancer, including prostatectomy, radiation therapy, and androgen deprivation therapy; and the prevention of prostate cancer. Figures illustrate the incidence rates of prostate cancer by race, age-adjusted and/or age-specific cancer of the prostate, the risk of a diagnosis in 20 years (based on being cancer free at certain ages), the 5-year survival rate, and the overall survival in patients with early prostate cancer treated with observation or radical prostatectomy. Tables in this chapter review the clinical staging definitions and the combined-modality staging approach to prostate cancer. This chapter contains 116 references.

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      • 4

        Management of Bladder Cancer

        By Joaquim Bellmunt, MD, PhD; Rosa Nadal, MD, PhD
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        Management of Bladder Cancer

        • JOAQUIM BELLMUNT, MD, PHDAssociate Professor of Medicine, Harvard Medical School, Lank Center for Genitourinary Oncology, Dana-Farber Cancer Institute/Brigham and Women’s Hospital, Harvard Medical School, Boston, MA
        • ROSA NADAL, MD, PHDClinical Fellow Genitourinary Oncology, The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, MD

        Bladder cancer is the fourth most common cancer in men and the tenth most common in women. Patients commonly present with painless hematuria. Urinary frequency, urinary tract infection, upper tract obstruction, and pain are also possible; however, the physical examination of the patients is often unremarkable. Clinical diagnosis depends on urine cytology, bladder ultrasonography, and office cystoscopy. This review describes the epidemiology, pathology and natural history, clinical presentation and workup, staging and grading, non–muscle-invasive bladder cancer, bladder preservation strategies, nontraditional approaches, chemotherapy for metastatic disease, immunotherapy, and predictive factors of response to chemotherapy for bladder cancer. Figures show representative images of urothelial cell carcinoma, diagrams showing a cystoscopy for a man and bladder cancer staging, urinary diversion, and computed tomographic scans of a patient with hepatic metastases from bladder cancer before and after treatment with gemcitabine-cisplatin. Tables list the tumor-node-metastasis staging system for bladder cancer, the histologic classification of tumors of the urinary tract (WHO 1973 versus WHO 2004), risk group stratification based on the European Organization for Research and Treatment of Cancer Scoring System, types of bacillus Calmette-Guérin failure/recurrence and treatment recommendations, unfavorable factors for bladder preservation chemoradiation, consensus criteria for patients unfit for cisplatin-based regimens, and first-line combination chemotherapy regimens.

        This review contains 5 highly rendered figures, 7 tables, and 80 references.

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      • 5

        Clinical Management of Renal Cell Carcinoma

        By Rana R. McKay, MD; Marina D. Kaymakcalan, PharmD; Guillermo De Velasco, MD; Suzanne S. Mickey, BS; Andre P. Fay, MD; Toni K. Choueiri, MD
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        Clinical Management of Renal Cell Carcinoma

        • RANA R. MCKAY, MDDepartment of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, Boston, MA
        • MARINA D. KAYMAKCALAN, PHARMDDepartment of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, Boston, MA
        • GUILLERMO DE VELASCO, MDDepartment of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, Boston, MA
        • SUZANNE S. MICKEY, BSDepartment of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, Boston, MA
        • ANDRE P. FAY, MDDepartment of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, Boston, MA
        • TONI K. CHOUEIRI, MDDepartment of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, Boston, MA

        Renal cell carcinoma (RCC) is the most common type of kidney cancer, and its incidence has continued to increase in the United States. Well-defined risk factors include smoking, obesity, and hypertension. There have been significant developments in the management of RCC over the past decade, pertaining to both patients with localized disease and those with advanced disease. In particular, agents targeting the vascular endothelial growth factor and mammalian target of rapamycin pathways have considerably improved survival for patients with metastatic disease. This review covers the epidemiology and pathogenesis of RCC, management of both localized and advanced disease, selection and sequencing of systemic therapy, non–clear cell RCC, and new targets and future directions for RCC. Figures show the morphologic features of RCC, the pathogenesis of RCC, and a huge primary RCC occupying a substantial part of the abdominal cavity. Tables include a summary of clinical and molecular characteristics of hereditary RCC syndromes and randomized phase III trials of approved first-line treatments in metastatic RCC.

        This review contains 3 highly rendered figures, 2 tables, and 59 references.

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    • Other Solid Tumors
      • 1

        Sarcomas of Soft Tissue and Bone

        By Adam Lerner, MD; Huihong Xu, MD; Karen H. Antman, MD
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        Sarcomas of Soft Tissue and Bone

        • ADAM LERNER, MDProfessor of Medicine and Pathology, Boston University School of Medicine, Boston, MA
        • HUIHONG XU, MDAssistant Professor of Pathology, Boston University School of Medicine, Boston, MA
        • KAREN H. ANTMAN, MDDean, Boston University School of Medicine, Provost, Boston University Medical School Campus, Boston, MA

        Sarcomas originate from bone or soft tissue. The most common bone sarcomas are osteosarcomas, Ewing sarcomas, and chondrosarcomas. Soft tissue sarcomas develop in fibrous tissue, fat, muscle, blood vessels, and nerves. Historically, soft tissue sarcomas of the trunk and extremities were reported separately from those of visceral organs (e.g., gastrointestinal and gynecologic sarcomas). This chapter discusses the classification, epidemiology, diagnosis, staging, and treatment of sarcomas of bone and cartilage, and classic soft tissue sarcomas. Management of Kaposi sarcoma, gastrointestinal stromal tumors (GISTs), mesothelioma, and rhabdomyosarcoma is also described. Figures include images of patients with osteosarcoma, liposarcoma, uterine leiomyosarcoma, GIST, and osteosarcoma in a patient with Paget disease of bone. Tables list epidemiologic features of sarcomas, a summary of sarcomas by histology, familial syndromes associated with increased risk of sarcoma, survival rates in sarcoma patients, staging of soft tissue sarcomas, and results of a meta-analysis of doxorubicin-based adjuvant chemotherapy for localized resectable soft tissue sarcoma. This chapter contains 126 references.

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      • 2

        Management of Gastrointestinal Stromal Tumors

        By César Serrano, MD, PhD; Suzanne George, MD
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        Management of Gastrointestinal Stromal Tumors

        • CÉSAR SERRANO, MD, PHDHead, Sarcoma Translational Research Laboratory and Sarcoma Unit, Oncology Department, Vall d’Hebron Institute of Oncology, Vall d’Hebron University Hospital, Barcelona, Spain
        • SUZANNE GEORGE, MDClinical Director, Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute/Harvard Medical School, Boston, MA

        Gastrointestinal stromal tumors (GISTs) are the most common tumors of mesenchymal origin in the gastrointestinal tract. These tumors are believed to arise from the interstitial cells of Cajal. The oncogenic activation of KIT or platelet-derived growth factor receptor–α is common to these tumors, and GISTS are considered to be a successful model for rational development of personalized treatments against oncogenic driver mutations in cancer. This review covers the epidemiology, etiology and genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, complications, and prognosis of GISTs. Figures show KIT primary and secondary mutations, the clinical and molecular progression of GISTs, a contrast-enhanced computed tomographic scan showing a gastric GIST presenting as a huge abdominal mass, a magnetic resonance image showing a rectal GIST at baseline and responding to neoadjuvant imatinib after 8 months of treatment, and hematoxylin-eosin stain of a fusocellular and an epithelioid GIST. Tables list demographics and clinical characteristics, associated genetic syndromes, relevant differential diagnosis, risk stratification systems used in GIST, and the comparative activity of approved regimens for GIST.

        This review contains 5 highly rendered figures, 5 tables, and 74 references.

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      • 3

        Primary and Metastatic Central Nervous System Malignancies

        By Fabio M. Iwamoto, MD; Howard A. Fine, MD
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        Primary and Metastatic Central Nervous System Malignancies

        • FABIO M. IWAMOTO, MDAssistant Clinical Investigator, Neuro-Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD
        • HOWARD A. FINE, MDSenior Investigator and Chief, Neuro-Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD

        About 13,000 deaths each year in the United States are attributed to primary central nervous system (CNS) malignancies. An estimated 20% of patients with cancer eventually develop clinically apparent CNS metastases, and an estimated 170,000 cases of brain metastases are diagnosed in the United States yearly. Autopsy studies suggest that as many as 50% of patients dying from advanced cancer may have metastasis to the CNS. This chapter provides an overview of primary and metastatic CNS malignancies with in-depth discussion of gliomas, primary CNS lymphoma, meningioma, brain metastases, leptomeningeal metastases, and metastatic epidural spinal cord compression. Discussions cover epidemiology, etiology, diagnosis, and treatment of gliomas, including surgery, radiotherapy, and chemotherapy for both newly diagnosed gliomas and recurrent gliomas. The epidemiology, diagnosis, treatment and prognosis for primary CNS lymphomas are reviewed, as well as the epidemiology, etiology, diagnosis, treatment, and prognosis for meningiomas. Epidemiology, diagnosis, and prognosis for brain metastases are briefly discussed, and the section on treatment includes surgery, stereotactic radiosurgery, and whole-brain radiotherapy for patients with three or fewer brain metastases. The sections on leptomeningeal metastases and metastatic epidural spinal cord compression cover diagnosis, treatment, and prognosis. This chapter contains 126 references.

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  • Palliative Medicine
    • 1

      Principles and Practices of Palliative Care

      By Kristen G. Schaefer, MD; Rachelle E. Bernacki, MD, MS
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      Principles and Practices of Palliative Care

      • KRISTEN G. SCHAEFER, MDDirector of Medical Student and Resident Education, Division of Adult Palliative Care, Department of Psychosocial Oncology and Palliative Care, Dana-Farber Cancer Institute, Center for Palliative Care, Harvard Medical School, Division of General Internal Medicine, Department of Medicine, Brigham and Women’s Hospital, Boston, MA
      • RACHELLE E. BERNACKI, MD, MSDirector of Quality Initiatives, Division of Adult Palliative Care, Department of Psychosocial Oncology and Palliative Care, Dana-Farber Cancer Institute, Center for Palliative Care, Harvard Medical School, Division of Aging, Department of Medicine, Brigham and Women’s Hospital, Boston, MA

      The trajectories of serious illness and dying have changed in the last century; in the past, patients lived shorter lives and often died quickly of infectious disease, whereas patients in the 21st century live longer and often with prolonged debility in the advanced stages of illness. As a result, patients with serious illness can suffer undertreated symptoms and often feel poorly prepared for the final stages of disease. With more options for advanced life support and other aggressive interventions at the end of life, patients and families face increasingly complex medical decisions in the terminal phase of illness, and the treatments they receive do not always align with their goals and values. Emerging evidence suggests that integrating palliative care into the treatment of advanced illness can improve outcomes, decrease costs, and improve both patient and family satisfaction. Consequently, patient access to high-quality specialty-level palliative care is becoming standard of care at most academic cancer centers and more available in the community. This chapter describes the practice and principles of specialty-level palliative care and outlines specific "generalist" palliative care competencies essential for all physicians caring for patients with serious illness, including prognostication, patient-centered communication, and the navigation of ethical dilemmas in the field of palliative care. Tables outline the philosophy of palliative care, domains of suffering, location of death of hospice patients, the palliative performance scale, median survival times for cancer syndromes, indicators associated with a poorer prognosis in congestive heart failure, the NURSE mnemonic for accepting and responding to emotion, palliative care communication competencies in the intensive care unit, and the SPIKES mnemonic for breaking bad news. Figures depict causes of death in 1900 versus 2010, palliative care through the trajectory of serious illness, theoretical trajectories of disease, life expectancies for women and men, mortality at 1 year post discharge, and a model for patient-centered communication.

      This review contains 6 highly rendered figures, 9 tables, and 169 references.

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    • 2

      Symptom Management in Palliative Medicine

      By Kathy J. Selvaggi, MD; Janet L. Abrahm, MD
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      Symptom Management in Palliative Medicine

      • KATHY J. SELVAGGI, MDAssistant Professor of Medicine, Harvard Medical School, Director, Intensive Palliative Care Unit, Brigham and Women’s Hospital, Boston, MA
      • JANET L. ABRAHM, MDProfessor of Medicine, Harvard Medical School, Division Chief, Adult Palliative Care, Dana-Farber Cancer Institute and Brigham and Women’s Hospital, Boston, MA

      Palliative care is an interdisciplinary specialty focused on providing comfort, communication, and support for patients, families, and professional caregivers throughout the course of a life-limiting illness. This chapter discusses assessment and treatment of symptoms and disorders that commonly contribute to patient distress during these illnesses: pain, disorders of the respiratory and gastrointestinal systems, skin disorders, hot flashes, fatigue, pruritis, insomnia, and delirium. This chapter reviews care of the imminently dying patient, discusses methods for assessing patients' symptoms, and provides two examples of valid and reliable symptom measurement systems: the Edmonton Symptom Assessment Scale and the Memorial Symptom Assessment Scale. Achieving symptom control requires the physician to assess patient suffering in all dimensions: physical, psychological, social, and spiritual. The extent of the assessment may be modified, however, based on patients’ prognosis as well as their goals and the burden and benefit of the diagnostic intervention. A 10-step protocol for terminal wean is presented. Signs that patients are entering their final days and symptom management in the last hours of a patient's life are discussed. Tables list the modified Edmonton Symptom Assessment Scale; the Memorial Symptom Assessment Scale; the DOLOPLUS-2 scale (behavioral pain assessment in the elderly); relative potencies of commonly used opioids; conversions between the transdermal fentanyl patch and morphine; symptomatic treatment for dyspnea, cough, and hiccups; pharmacologic treatment of nausea and vomiting; a progressive bowel regimen for patients receiving opioid therapy; treatments for constipation; etiology-based treatment for oral problems; risk factors for pressure ulcers; and applicable medications for physical and psychological sources of distress near the end of life. This chapter contains 12 tables, 120 references, 5 MCQs.

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    • 3

      Management of Psychosocial Issues in Terminal Illness

      By Eva Reitschuler-Cross, MD; Susan D. Block, MD; Jane DeLima Thomas, MD
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      Management of Psychosocial Issues in Terminal Illness

      • EVA REITSCHULER-CROSS, MDClinical Assistant Professor of Medicine, University of Pittsburgh, Section of Palliative Care and Medical Ethics, University of Pittsburgh Medical Center, Pittsburgh, PA
      • SUSAN D. BLOCK, MDChair, Department of Psychosocial Oncology and Palliative Care, Dana-Farber Cancer Institute and Brigham and Women’s Hospital Co-Director, HMS Center for Palliative Care, Professor of Psychiatry and Medicine, Harvard Medical School, Boston, MA
      • JANE DELIMA THOMAS, MDAttending Physician, Department of Psychosocial Oncology and Palliative Care, Dana-Farber Cancer Institute and Brigham and Women’s Hospital, Instructor in Medicine, Harvard Medical School, Boston, MA

      Patients facing serious or life-threatening illness experience challenges to their psychological, social, and spiritual lives as well as to their physical function and comfort. Physicians may be accustomed to focusing on the biomedical aspects of illness, but they have a critical role in assessing the patient's psychosocial issues to identify sources of distress and help implement a plan for mitigating them. An appropriate psychosocial assessment requires a methodical and rigorous approach and includes assessment of any psychosocial issue affected by or affecting a patient's experience of illness. This chapter outlines a structured approach to addressing psychosocial issues by discussing (1) the doctor-patient relationship; (2) coping with illness; (3) family dynamics and caregiving; (4) ethnic and cultural issues; (5) religious, spiritual, and existential issues; (6) mental health issues, including adjustment disorder, depression, anxiety, personality disorders, aberrant drug behaviors, and major mental health issues; and (7) grief and bereavement. Tables outline psychosocial assessment questions, factors predisposing patients with serious illness to depression, risk factors for suicide in patients with terminal illness, and classes of antidepressants, anxiolytics, and sedatives. The Alcohol Use Disorders Identification Test-Consumption (AUDIT-C) questionnaire is provided, as well as a list of Web sites with further resources about psychosocial issues in serious illness.


      This chapter contains 1 highly rendered figure, 6 tables, 216 references, and 5 MCQs.

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  • Psychiatry
    • 1

      Schizophrenia

      By Gunvant K. Thaker, MD; William T. Carpenter Jr, MD
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      Schizophrenia

      • GUNVANT K. THAKER, MDProfessor of Psychiatry, Maryland Psychiatric Research Center, Department of Psychiatry, University of Maryland School of Medicine, Baltimore, MD
      • WILLIAM T. CARPENTER JR, MDProfessor of Psychiatry and Pharmacology, Maryland Psychiatric Research Center, Department of Psychiatry, University of Maryland School of Medicine, Baltimore, MD

      Schizophrenia is a clinical syndrome probably comprising several as-yet undefined disease entities. There is substantial heterogeneity between cases, which presumably reflects multiple overlapping etiologic factors, including contributions from several genes. The presence of schizophrenia is indicated by chronic psychotic symptoms, especially hallucinations and delusions. Disorganization of thought and behavior are common and distinguish schizophrenia from the many other causes of reality-distortion symptoms (eg, psychotomimetic drugs). Diminished emotional experience and expression, low drive, and reduced speech are observed in a subgroup of patients. Most patients have subtle impairments in cognition. Cognitive impairments and the emotional and social aspects of the disease often appear early in life; the psychotic symptoms typically begin in late adolescence or early adulthood in men and a little later in women. This chapter discusses the epidemiology, etiology and genetics, and pathophysiology of schizophrenia; its clinical course; the diagnosis and differential diagnosis; and its treatment, including pharmacologic management and psychosocial interventions. A figure illustrates comparative findings on functional magnetic resonance in schizophrenic and nonschizophrenic patients. Tables list diagnostic criteria for schizophrenia; cognitive disturbances seen in schizophrenic speech; medical conditions, drugs, and medications associated with psychotic symptoms; and antipsychotic drugs used in the treatment of schizophrenia. This chapter contains 63 references.

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    • 2

      Anxiety Disorders

      By Brian M. Iacoviello, PhD; Sanjay J. Mathew, MD
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      Anxiety Disorders

      • BRIAN M. IACOVIELLO, PHDPsychology Fellow, Department of Psychiatry, VA VISN3 Mental Illness Research, Education and Clinical Center (MIRECC) and Mount Sinai School of Medicine, New York, NY
      • SANJAY J. MATHEW, MDDirector, Mood Disorders Center, Associate Professor of Psychaitry, Baylor College of Medicine, Medical Director, Comprehensive Mental Health Program, Michael E. Debakey VA Medical Center, Houston, TX, Menninger Department of Psychiatry and Behavioral Sciences

      Anxiety disorders include generalized anxiety disorder, panic disorder, social anxiety disorder, acute stress disorder, posttraumatic stress disorder, and obsessive-compulsive disorder. They all share symptoms to a great extent, but they also have distinct clinical features and typical presentations. Diagnosis for each requires the presence of specific criteria. Anxiety disorders are best managed through a systematic approach to diagnosis, treatment, and, when necessary, referral to a specialist. Treatment includes pharmacotherapy and psychotherapy, with cognitive-behavioral therapy repeatedly shown to be among the most effective. If a physician decides to refer the patient to a mental health professional, it's best to tread lightly because some patients will resist.

      This review contains 1 figure, 4 tables, and 33 references.

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    • 3

      Mood Disorders

      By Hasan A. Baloch, MD; Jair C. Soares, MD
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      Mood Disorders

      • HASAN A. BALOCH, MDAssistant Professor, Department of Psychiatry, Medical Director, Pediatric Bipolar Program, CERT-BD. 10616 Neuroscience Hospital CB#7160, UNC School of Medicine, Chapel Hill, NC 27599-7160, Phone: (919) 966-8485, FAX: (919) 843-3950
      • JAIR C. SOARES, MDProfessor and Chairman, Department of Psychiatry and Behavioral Sciences, UT Houston Medical School

      Affective disorders are among the most common disorders in psychiatry. They are generally classified according to the persistence and extent of symptoms and by the polarity of these symptoms. The two poles of the affective spectrum are mania and depression. Bipolar disorder is characterized by the presence of the mania or hypomania and often depression. Unipolar depression is defined by depression in the absence of a lifetime history of mania or hypomania. These differences are not merely categorical but have important implications for the prognosis and treatment of these conditions. Bipolar disorder, for example, is better treated using mood-stabilizing medication, whereas unipolar depression responds optimally to antidepressant medications. In addition, prognostically, unipolar depression may sometimes be limited to one episode in a lifetime, whereas bipolar disorder is typically a lifelong condition. The course of both conditions, however, is often chronic, and frequently patients can present with unipolar depression only to later develop manic symptoms. A thorough understanding of both conditions is therefore required to treat patients presenting with affective symptomatology. This chapter discusses the epidemiology, etiology and genetics, pathogenesis, diagnosis, and treatment of unipolar depression and bipolar disorder. Figures illustrate gray matter differences with lithium use and the bipolar spectrum. Tables list the pharmacokinetics of commonly used antidepressants and medications commonly used in the treatment of bipolar disorder.

      This review contains 2 figures, 2 tables, and 135 references.

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    • 4

      The Eating Disorders

      By W. Stewart Agras, MD
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      The Eating Disorders

      • W. STEWART AGRAS, MDProfessor of Psychiatry, Department of Psychiatry, Stanford University School of Medicine, Stanford CA

      The eating disorders–anorexia nervosa, bulimia nervosa, binge eating disorder, and night eating syndrome–tend to be chronic conditions and are frequently accompanied by depression, anxiety disorders, and personality disorders. In addition, serious medical problems are associated with anorexia nervosa (because of chronic starvation) and with binge-eating disorder (because of obesity). Eating disorders may also be associated with marked life impairment, faulty interpersonal interactions, and social withdrawal. In women, the lifetime prevalence of eating disorders that meet full diagnostic criteria is about 4%. If subclinical cases are included, the overall prevalence of eating disorders in the female population is probably between 6 and 8%. Ten percent of cases of eating disorders occur in males. This chapter discusses the epidemiology of eating disorders and reviews the genetic and cultural factors that contribute to their development. The diagnosis and treatment of each of the eating disorders are considered separately. A table lists the points to be considered for management of bulimia nervosa once the diagnosis is made, and an algorithm outlines decision points in the management of anorexia nervosa. This chapter contains 34 references.

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    • 5

      Adjustment Disorders

      By James J. Strain, MD; Matthew Friedman, MD, PhD
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      Adjustment Disorders

      • JAMES J. STRAIN, MDProfessor of Psychiatry, Ichan School of Medicine at Mount Sinai, New York, NY
      • MATTHEW FRIEDMAN, MD, PHDNational Center for PTSD, US. Department of Veterans Affairs, Dartmouth Medical School, VA Medical Center, White River Junction

      The adjustment disorder (AD) diagnosis has clinical appeal to both doctors and patients. The idea of temporary emotional symptoms resulting directly from a stressful life event is viewed as a more normal human reaction than an idiopathic pathologic psychiatric state and is therefore less stigmatizing. Additionally, the disorder's more benign course (especially in adults) encourages a clinician to be more prognostically optimistic. This chapter discusses the prevalence, epidemiology, course and prognosis, and etiology of ADs. Also reviewed are AD subtypes proposed but not accepted for the Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM-5), including the acute stress disorder/posttraumatic stress disorder subtype and the bereavement-related subtype. Treatment options are covered, including psychotherapy, pharmacotherapy, and primary care. Tables outline diagnostic criteria for ADs in DSM-IV, stress-related disorders in DSM-5, DSM classifications, ADs in mental illness and medical settings, and subtypes of DSM-IV, text revision adjustment disorders. Graphs categorize patients diagnosed with ADs according to type of illness and the prognosis for recovery from ADs in adolescents and adults.
      This chapter contains 2 highly rendered figures, 6 tables, 109 references, and 5 MCQs.

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    • 6

      Management of Somatic Symptoms

      By Andreas Schröder, MD, PhD; Joel E. Dimsdale, MD
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      Management of Somatic Symptoms

      • ANDREAS SCHRÖDER, MD, PHDConsultant in Psychiatry, The Research Clinic for Functional Disorders, Aarhus University Hospital, Aarhus, Denmark
      • JOEL E. DIMSDALE, MDDistinguished Professor of Psychiatry Emeritus, Department of Psychiatry, University of California, San Diego, La Jolla, CA

      Somatic symptoms that cannot be attributed to organic disease account for 15 to 20% of primary care consultations and up to 50% in specialized settings. About 6% of the general population has chronic somatic symptoms that affect functioning and quality of life. This chapter focuses on the recognition and effective management of patients with excessive and disabling somatic symptoms. The clinical presentation of somatic symptoms is categorized into three groups of patients: those with multiple somatic symptoms, those with health anxiety, and those with conversion disorder. The chapter provides information to assist with making a diagnosis and differential diagnosis. Management includes ways to improve the physician–patient interaction that will benefit the patient, a step-care model based on illness severity and complexity, and psychological and pharmacologic treatment. The chapter is enhanced by figures and tables that summarize health anxiety, symptoms, differential diagnoses, and management strategies, as well as by case studies and examples.

      This review contains  5 highly rendered figures, 10 tables, and 235 references.

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    • 7

      Male Sexual Dysfunction

      By Michael Eisenberg, MD; Kathleen Hwang, MD
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      Male Sexual Dysfunction

      • MICHAEL EISENBERG, MDDirector, Male Reproductive Medicine and Surgery, Assistant Professor, Departments of Urology and Obstetrics & Gynecology, Stanford University, Stanford, California
      • KATHLEEN HWANG, MDDepartment of Urology, Brown University, Providence, Rhode Island

      Normal male sexual function requires complex interactions among psychological, neurologic, hormonal, and vascular systems. Under the influence of proper stimulation, the acquisition and maintenance of a penile erection occur. Male sexual dysfunction includes erectile dysfunction (ED), impaired libido, and abnormal ejaculation, which occur due to aberrations in normal male sexual response. Estimates suggest a prevalence of approximately 10 to 20% in the adult male population. Thus, sexual dysfunction is a common problem in this country. This chapter focuses on several key, common components of male sexual dysfunction: namely, ED, premature ejaculation (PE), and Peyronie disease (PD). Figures include management algorithms, a graph depicting distribution of intravaginal ejaculation latency time values in a random cohort of men, and a Sexual Health Inventory for Men (SHIM). Tables list medications associated with ED, oral therapies for the treatment of PD, intralesional therapies for PD, indications for surgical correction of PD, recommended questions to establish the diagnosis of PE, treatment options for PE, and causes of delayed ejaculation, anejaculation, and anorgasmia.
      This chapter contains 5 highly rendered figures, 8 tables, 116 references, 1 teaching slide set, and 5 MCQs.

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    • 8

      Unhealthy Alcohol Use

      By Stephen R. Holt, MD, MSc; David A. Fiellin, MD
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      Unhealthy Alcohol Use

      • STEPHEN R. HOLT, MD, MSCAssistant Professor of Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT
      • DAVID A. FIELLIN, MDProfessor of Medicine, Investigative Medicine and Public Health, Department of Medicine, Yale University School of Medicine, New Haven, CT

      Alcohol use is associated with a wide range of behavioral and medical disorders. An estimated 9 to 44% of patients seen in primary care meet the criteria for unhealthy alcohol use—a spectrum of alcohol use that ranges from at-risk drinking to alcohol use disorders. To manage patients effectively, physicians should recognize all forms of unhealthy alcohol use, from the earliest to the most advanced stages. They should be able to manage patients along the entire spectrum of the disease process.

      This chapter provides an overview of the major clinical features and recent developments in the identification and management of alcohol-related problems in clinical practice. The chapter distinguishes three types of alcohol use, discusses the epidemiology and genetic components of alcohol-use disorders, and describes the medical, psychological, and behavior-related problems associated with alcohol abuse. Despite the prevalence of alcohol-related problems and their impact on health, most studies demonstrate that alcohol-related problems are not routinely detected in primary care settings. Early detection of medically hazardous drinking levels before severe complications arise may prevent late sequelae, and the chapter provides screening strategies, including the commonly studied CAGE questionnaire.

      After diagnosis, brief intervention therapy for at-risk and problem drinkers and self-help groups are two common approaches to management, but in addition to providing office-based evaluation, management, and referral to self-help groups, primary care physicians will often need to provide more intensive services to patients who meet the criteria for alcohol abuse and dependence. Alcohol withdrawal syndrome can occur in alcohol-dependent persons who stop drinking alcohol or who reduce their alcohol intake, and patients who do not respond to brief intervention therapies may require referral to specialists and formal alcohol treatment programs. Relapse prevention is sometimes accomplished with pharmacotherapy as an adjunct to psychotherapy. Tables present the World Health Organization’s criteria for harmful drinking and the DSM-5 definition for alcohol use disorder; methods for the screening and diagnosis of alcohol problems; management of patients with unhealthy alcohol use; stages of readiness to change addictive behaviors and stage-specific approaches to therapy; components of a brief intervention; common objectives of alcohol treatment programs; and pharmacotherapeutic agents used in the treatment of alcohol dependency. An algorithm demonstrates common problems related to unhealthy alcohol use, and a graph demonstrates the association of heavy episodic drinking, alcohol abuse, and alcohol dependence with major depression.

      This chapter contains 168 references, 8 tables, 2 highly rendered figures, and 5 MCQs.

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    • 9

      Drug Abuse and Dependence

      By F. Gerard Moeller, MD
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      Drug Abuse and Dependence

      • F. GERARD MOELLER, MDDirector, Center for Neurobehavioral Research on Addictions, Professor, Department of Psychiatry, University of Texas Health Science Center at Houston, Houston, TX

      There is a consistent body of evidence showing that substance abuse and dependence can worsen preexisting medical conditions, can temporarily mimic medical and psychiatric disorders, and can themselves cause medical problems, including life-threatening overdose. Substance use disorders are common in young and middle-aged persons: the lifetime prevalence of these syndromes, including alcoholism, is over 20% for men and about 15% for women. This chapter discusses dependence, abuse, substance use disorder, and substance-induced disorders involving depressants, stimulants, opioids, cannabinoids, hallucinogens, N-methyl-D-aspartate (NMDA) receptor channel blockers, and inhalants. Epidemiology, etiology, pathophysiology, diagnosis (including clinical assessment and laboratory tests), and treatment are reviewed. Treatment of intoxication, overdose, withdrawal, and rehabilitation is discussed. A figure illustrates the neurocircuitry of addiction. Tables describe the Diagnostic and Statistical Manual of Mental Disorders, 4th Edition (DSM-IV) diagnostic criteria for abuse and dependence; frequently misused drugs; neural effects of commonly abused drugs; the natural history of drug dependence; conditions affecting the outcome of urinary drug tests; and pharmacologic options for treatment of drug overdose. This chapter contains 111 references.

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    • 10

      Personality Disorders

      By Yosefa A. Ehrlich, BS; Amir Garakani, MD; Stephanie R. Pavlos, MA; Larry Siever, MD
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      Personality Disorders

      • YOSEFA A. EHRLICH, BSDoctoral Student in Clinical Psychology, City University of New York Graduate Center, New York, NY
      • AMIR GARAKANI, MDAssistant Clinical Professor of Psychiatry, Icahn School of Medicine at Mount Sinai, New York, NY
      • STEPHANIE R. PAVLOS, MADoctoral Student in Clinical Psychology, St. Johns University, Queens, NY
      • LARRY SIEVER, MDProfessor of Psychiatry, Icahn School of Medicine at Mount Sinai, New York, NY

      Personality can be defined as an organizational system of self that shapes the manner in which a person interacts with his or her environment. Personality traits develop in adolescence or early adulthood and are thought to be shaped by early childhood experiences and enduring throughout a lifetime. Personality traits that prevent an individual from being able to function in society or that cause significant distress are diagnosed as personality disorders. A thorough history is needed to rule out other psychiatric and medical disorders. This chapter reviews the diagnostic criteria, differential diagnosis, comorbidity, prevalence, etiology (including genetics and neurobiology), prognosis, and treatment of paranoid, schizoid, schizotypal, borderline, antisocial, narcissistic, histrionic, avoidant, obsessive-compulsive, and dependent personality disorders. A discussion of the relevance of personality disorders to primary care practices and approaches to managing such patients is also included. Tables describe the diagnostic criteria of each personality disorder. Figures illustrate the prevalence of personality disorders in the general and psychiatric populations; schizotypal personality disorder in the community, general population, and clinical population; childhood trauma in individuals with personality disorder; and comorbid disorders in individuals with borderline personality disorder. A model of brain processing in borderline personality disorder is also featured.

      This chapter contains 5 highly rendered figures, 10 tables, 230 references, and 5 MCQs.

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    • 11

      Sleep Disorders

      By Sudhansu Chokroverty, MD, FRCP, FACP
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      Sleep Disorders

      • SUDHANSU CHOKROVERTY, MD, FRCP, FACPProfessor and Director of Sleep Research, Medical Director of Devry Technology Training Program, Co-Chair Emeritus of Neurology, Department of Neurology, JFK Neuroscience Institute, Edison, NJ, Professor of Neuroscience, Seton Hall University, South Orange, NJ, Clinical Professor of Neurology, Robert Wood Johnson Medical School, New Brunswick, NJ

      Recent research has generated an enormous fund of knowledge about the neurobiology of sleep and wakefulness. Sleeping and waking brain circuits can now be studied by sophisticated neuroimaging techniques that map different areas of the brain during different sleep states and stages. Although the exact biologic functions of sleep are not known, sleep is essential, and sleep deprivation leads to impaired attention and decreased performance. Sleep is also believed to have restorative, conservative, adaptive, thermoregulatory, and consolidative functions. This review discusses the physiology of sleep, including its two independent states, rapid eye movement (REM) and non–rapid eye movement (NREM) sleep, as well as functional neuroanatomy, physiologic changes during sleep, and circadian rhythms. The classification and diagnosis of sleep disorders are discussed generally. The diagnosis and treatment of the following disorders are described: obstructive sleep apnea syndrome, narcolepsy-cataplexy sydrome, idiopathic hypersomnia, restless legs syndrome (RLS) and periodic limb movements in sleep, circadian rhythm sleep disorders, insomnias, nocturnal frontal lobe epilepsy, and parasomnias. Sleep-related movement disorders and the relationship between sleep and psychiatric disorders are also discussed. Tables describe behavioral and physiologic characteristics of states of awareness, the international classification of sleep disorders, common sleep complaints, comorbid insomnia disorders, causes of excessive daytime somnolence, laboratory tests to assess sleep disorders, essential diagnostic criteria for RLS and Willis-Ekbom disease, and drug therapy for insomnia. Figures include polysomnographic recording showing wakefulness in an adult; stage 1, 2, and 3 NREM sleep in an adult; REM sleep in an adult; a patient with sleep apnea syndrome; a patient with Cheyne-Stokes breathing; a patient with RLS; and a patient with dream-enacting behavior; schematic sagittal section of the brainstem of the cat; schematic diagram of the McCarley-Hobson model of REM sleep mechanism; the Lu-Saper “flip-flop” model; the Luppi model to explain REM sleep mechanism; and a wrist actigraph from a man with bipolar disorder.

      This review contains 14 highly rendered figures, 8 tables, 115 references, and 5 MCQs.

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    • 12

      Acute Psychosis

      By James Kimo Takayesu, MD, MS; Suzanne Bird, MD
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      Acute Psychosis

      • JAMES KIMO TAKAYESU, MD, MSAssistant Residency Director, Department of Emergency Medicine, Massachusetts General Hospital, Boston, MA
      • SUZANNE BIRD, MDAcute Psychiatric Service, Massachusetts General Hospital, Boston, MA

      Acute psychosis can be a true emergency, and the primary goal in the evaluation of an acutely psychotic patient should be to maintain safety and prevent harm to the patient and staff. The defining symptoms of psychosis include hallucinations, delusions, disorganized thought or speech, abnormal motor behavior, and negative symptoms. This review covers the approach to the patient, and definitive treatment, disposition and outcomes for patients experiencing acute psychosis. The figure shows an interview setting in a triangular arrangement, allowing for safe egress. Tables list goals in the evaluation of the acutely psychotic patient; causes of secondary psychosis; common medication classes causing mental status change; four key questions for assessing psychotic behavior; screening assessment for psychosis; clinical features of dementia, delirium, and psychiatric illness; brief mental status examination; common medications for the treatment of acute psychosis and chemical sedation; QT-prolonging effects of commonly used antipsychotic medications; and documentation required in the use of chemical and/or physical restraints.

      This review contains 1 highly rendered figure, 10 tables, and 57 references.

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  • Pulmonary & Critical Care Medicine
    • Pulmonary
      • 1

        Approach to the Patient With Cough

        By Christopher H. Fanta, MD
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        Approach to the Patient With Cough

        • CHRISTOPHER H. FANTA, MDDirector, Partners Asthma Center; Member, Pulmonary and Critical Care Medicine Division, Department of Medicine, Brigham and Women’s Hospital; and Professor of Medicine, Harvard Medical School, Boston, MA

        The cough reflex is critically important in the clearance of abnormal airway secretions and protection of the lower respiratory tract from aspirated foreign matter. A weak or ineffective cough can lead to respiratory compromise from even a relatively minor bronchial infection. Persistent cough is often one of a constellation of symptoms indicative of respiratory disease—a potential clue in the differential diagnosis of the patient’s illness. Given the widespread distribution of sensory nerve endings of the cough reflex throughout the upper and lower respiratory tract, it is not surprising that myriad respiratory diseases, involving lung parenchyma and airways, can manifest with cough. Sometimes cough is the sole or predominant symptom in a patient who is otherwise well. Evaluating and treating the patient with persistent cough who has few, if any, other respiratory symptoms is a common challenge for the practicing physician. This review covers the normal cough mechanism, impaired cough, pathologic cough, cough suppressant therapy, and new developments. Figures show a flow-volume loop during cough, a posteroanterior chest x-ray in a patient presenting with chronic cough, flow-volume curves and spirograms documenting expiratory airflow obstruction, and the approach to the patient with chronic cough. The table lists selected examples of extrapulmonary physical findings of potential importance in the assessment of cough.

        This review contains 4 highly rendered figures, 1 table, and 94 references.

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      • 2

        Approach to the Patient With Respiratory Symptoms

        By John Hansen-Flaschen, MD
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        Approach to the Patient With Respiratory Symptoms

        • JOHN HANSEN-FLASCHEN, MDProfessor of Medicine, Chief, Pulmonary, Allergy and Critical Care Division, Department of Medicine, University of Pennsylvania. Medical Director, Penn Lung Center, Hospital of the University of Pennsylvania, Philadelphia, Pa.

        The respiratory system comprises two organs: the lungs and the respiratory chest pump, the latter of which is just as vital to survival as the heart. Chronic lower respiratory diseases ranked as the fourth highest cause of death in the United States in 2006, and as third highest the world over. Two of the most common of all medical complaints are dyspnea and cough. More than 15 distinct sensations fall under the current definition of dyspnea. For clinical assessment, dyspnea can be divided into three broad categories: dyspnea on exertion, paroxysmal dyspnea, and sustained dyspnea at rest. Patients most often visit their physician because of a cough. There are at least two distinct types of cough mediated by at least two types of vagal afferent nerve fibers: aspiration-induced or "defensive" reflex coughing, and cough initiated by an itchy, urge-to-cough sensation. Acute cough is thought to be triggered by an acute viral respiratory infection, but other conditions come to medical attention shortly after the onset of a cough. An unexplained, troubling cough that lasts longer than 8 weeks calls for a posterior-anterior and lateral chest radiograph plus a complete history and physical examination. This chapter contains 40 references.

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      • 3

        Pulmonary Edema

        By Annette Esper, MD; Greg S. Martin, MD, MSc, FACP; Gerald W. Staton Jr, MD, FACP
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        Pulmonary Edema

        • ANNETTE ESPER, MDAssistant Professor of Medicine, Division of Pulmonary, Allergy and Critical Care, Emory University School of Medicine, 615 Michael Street, Whitehead Building 205, Atlanta, GA 30322
        • GREG S. MARTIN, MD, MSC, FACPAssociate Professor of Medicine, Division of Pulmonary, Allergy and Critical Care, Emory University School of Medicine, 615 Michael Street, Whitehead Building 205, Atlanta, GA 30322
        • GERALD W. STATON JR, MD, FACPProfessor of Medicine, Division of Pulmonary, Allergy and Critical Care, Emory University School of Medicine, 615 Michael Street, Whitehead Building 205, Atlanta, GA 30322

        There are two categories of pulmonary edema: edema caused by increased capillary pressure (hydrostatic or cardiogenic edema) and edema caused by increased capillary permeability (noncardiogenic pulmonary edema, or acute respiratory distress syndrome [ARDS]). This chapter discusses the pathogenesis of pulmonary edema and describes the general approach to patients with suspected pulmonary edema as well as approaches to patients with cardiogenic pulmonary edema and patients with noncardiogenic pulmonary edema (a.k.a. ARDS). The pathogenesis, diagnosis, treatment, and outcome of cardiogenic and noncardiogenic pulmonary edema are reviewed. Miscellaneous causes of pulmonary edema are discussed, including neurologic insults, exposure to high altitude, re-expansion of a collapsed lung, lung transplant, upper airway obstruction, drugs, and lung resection. Figures comprise chest scans showing pulmonary edema and noncardiogenic pulmonary edema, an illustration of the differences between cardiogenic and noncardiogenic edema, and a chart comparing lung mechanics and other variables in experimental models of cardiogenic pulmonary edema and noncardiogenic edema. Tables show the differences between cardiogenic and noncardiogenic pulmonary edema, treatments for cardiogenic pulmonary edema, causes of ARDS, and treatments for ARDS that do not involve ventilation.

        This review contains 4 highly rendered figures, 5 tables, and 87 references.

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      • 4

        Pulmonary Function Testing

        By Maryl Kreider, MD, MS
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        Pulmonary Function Testing

        • MARYL KREIDER, MD, MSAssistant Professor of Medicine, University of Pennsylvania School of Medicine, Medical Director, Pulmonary Diagnostics Laboratory, Hospital of the University of Pennsylvania, Philadelphia, PA

        Intelligent management of pulmonary disease requires a fundamental understanding of the complex interrelationships between multiple elements that interact to maintain homeostasis in the respiratory system. This chapter discusses the physiologic basis for pulmonary function testing and the use of these tests to diagnose disease, quantitate functional impairments, and follow the effects of treatment. Figures illustrate the relationships between lung volume, airway conductance, and airway resistance; the relationship between forced expiratory volume and time (spirogram); the relationship between spirometry and maximum expiratory flow volume; dynamic airway compression; patterns of abnormalities seen on flow-volume curves; lung volumes and capacities; pressure-volume relationships in health and disease; and the clinical assessment of lung function. Tables list the capacities and volumes of gas contained in the lungs during various breathing maneuvers, conditions associated with alterations in diffusing capacity, and recommendations for tests for various clinical scenarios.

        This review contains 7 highly rendered figures, 3 tables, and 86 references.

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      • 5

        Imaging of Lung Disease

        By Eugene A. Berkowitz, MD, PhD; Phuong-Anh T. Duong, MD; Gerald W. Staton Jr, MD, FACP
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        Imaging of Lung Disease

        • EUGENE A. BERKOWITZ, MD, PHDAssistant Professor of Radiology, Emory University School of Medicine, Grady Health System, Department of Radiology and Imaging Sciences, 1365 Clifton RD NE AT 508, Atlanta, GA 30322
        • PHUONG-ANH T. DUONG, MDAssistant Professor of Radiology, Emory University School of Medicine, Grady Health System, Department of Radiology and Imaging Sciences, 1365 Clifton RD NE AT 501, Atlanta, GA 30322
        • GERALD W. STATON JR, MD, FACPProfessor of Medicine, Division of Pulmonary, Allergy and Critical Care, Emory University School of Medicine, 615 Michael Street, Whitehead Building 205, Atlanta, GA 30322

        Currently, a variety of techniques are available for imaging the chest. With recent advances in computed tomography (CT), magnetic resonance imaging (MRI), and ultrasonography (US), indications for the use of each modality have continued to evolve. At times, selection of the best test can be intimidating. Consultation with a radiologist is the best way to ensure that the best technique is used. Also, the American College of Radiology provides guidelines for the appropriateness and relative radiation risk of imaging modalities for a number of indications in the ACR Appropriateness Criteria. As the radiation risks of medical imaging have become an increasing concern in the medical community as well as the general population, choosing appropriate imaging, including whether or not to image, requires careful consideration.

        This chapter discusses conventional chest radiography CT, MRI, nuclear medicine, US, and digital subtraction pulmonary angiography and how these modalities are used in the approach to diagnosis of lung disease. Parenchymal lung diseases are classified into eight categories on the basis of chest radiography and CT: (1) focal pulmonary infiltrates, (2) multifocal pulmonary infiltrates, (3) true segmental infiltrates, (4) interstitial infiltrates, (5) cavitary infiltrates, (6) single small nodules, (7) large masses, and (8) multiple nodules. Tables list the major causes of these findings, as well as typical radiation doses of routine chest diagnostic studies. Figures illustrate the use of chest imaging modalities in the diagnosis of disorders in each of the eight categories. In this chapter, a simple approach is taken to divide causes of pulmonary abnormalities into three broad categories: infectious, neoplastic, and noninfectious/nonneoplastic.

        This review contains 38 figures covering all imaging techniques, 12 tables, and 56 references.

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      • 6

        Invasive Diagnostic and Therapeutic Techniques in Lung Disease

        By Raphael Bueno, MD; David J. Sugarbaker, MD
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        Invasive Diagnostic and Therapeutic Techniques in Lung Disease

        • RAPHAEL BUENO, MDAssociate Chief, Division of Thoracic Surgery, Brigham and Women’s Hospital, Boston, MA
        • DAVID J. SUGARBAKER, MDChief, Division of Thoracic Surgery, Brigham and Women’s Hospital, Boston, MA

        Invasive diagnostic and therapeutic techniques in lung disease include a broad range of procedures. This chapter provides a description of many of these procedures, as well as the specific indications, contraindications, risks, and diagnostic yield associated with their use. Special attention is given to transthoracic needle aspiration and biopsy, bronchoscopy, thoracentesis, and tube thoracostomy, because these procedures are most commonly used by internists and pulmonologists. Figures provide images of transthoracic needle aspiration and biopsy with CT guidance; a side-by-side comparison of a flexible bronchoscope and a rigid bronchoscope; and photographs of common airway stents. Tables provide a comparison of diagnostic techniques used in diseases of the lung and mediastinum; the most common indications for bronchoscopy; the accessibility of mediastinal lymph node stations by various diagnostic nodes; the complications of diagnostic bronchoscopy as performed with a flexible bronchoscope; the types of pleural effusions for which a definitive diagnosis can be achieved by pleural fluid analysis; the relative values of various bronchoscopic modalities for the treatment of airway obstruction; the complications of therapeutic bronchoscopy; and criteria for chest tube drainage of parapneumonic effusions. There are 33 references in this chapter.

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      • 7

        Asthma

        By Kohei Hasegawa, MD, MPH
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        Asthma

        • KOHEI HASEGAWA, MD, MPHAssistant Professor, Department of Emergency Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA

        Asthma is one of the most common diseases in developed nations. A pathognomonic feature of asthma is episodic aggravations of the disease; these exacerbations can be life-threatening and contribute to a significant proportion of the public health burden of asthma. In the emergency department, successful management of asthma exacerbations requires early recognition and intervention before they become severe and potentially fatal. This review summarizes the pathophysiology, stabilization and assessment, diagnosis and treatment, and disposition and outcomes for asthma. Figures show the management of asthma exacerbations in the emergency department and hospital, pooled odds ratio comparing inhaled corticosteroids and oral corticosteroids with oral corticosteroids alone following emergency department discharge, and an asthma discharge plan at the emergency department. Tables list current asthma prevalence among selected demographic groups in the United States, risk factors for fatal asthma exacerbations, differential diagnosis of asthma exacerbations, and dosages of drugs for asthma exacerbations.

        This review contains 3 highly rendered figures, 4 tables, and 117 references.

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      • 8

        Chronic Obstructive Pulmonary Disease

        By Robert M. Senior, MD; Edwin K. Silverman, MD, PhD
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        Chronic Obstructive Pulmonary Disease

        • ROBERT M. SENIOR, MDDorothy R. and Hubert C. Moog Professor of Pulmonary Diseases in Medicine, and Professor of Cell Biology and Physiology, Washington University School of Medicine, St. Louis, MO
        • EDWIN K. SILVERMAN, MD, PHDAssociate Professor of Medicine, Channing Laboratory and Division of Pulmonary and Critical Care Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA

        Chronic obstructive pulmonary disease (COPD) is a disease state characterized by airflow obstruction that is not fully reversible. The airflow obstruction is usually both progressive and associated with an inflammatory response of the lungs to noxious particles and gases. In most cases, the noxious particles and gases derive from tobacco smoke. This definition emphasizes the importance of airflow obstruction, which is typically determined using spirometry, in the diagnosis of COPD. Unlike earlier definitions of COPD, the current definition does not mention emphysema or chronic bronchitis. It also differs from earlier definitions in that it includes inflammation, which is the presumed major pathogenetic mechanism of COPD. This chapter discusses the epidemiology, etiology, pathogenesis, pathophysiology, diagnosis, treatment, complications, and prognosis for COPD. Figures illustrate the difference in spirometry results between a healthy person and one with severe COPD; mean forced expiratory volume in one second (FEV1), expressed as a percentage of the predicted value, as related to smoking intensity in a general population sample; stages of COPD, as classified by the Global Initiative for Chronic Obstructive Lung Disease (GOLD); the pathogenesis of emphysema from smoking; pathologic lesions in small airways in COPD; and the acinar structure of normal lungs, as compared with that of lungs in patients with centriacinar (centrilobular) emphysema or panacinar (panlobular) emphysema. Tables describe the differential diagnosis of COPD and the inhaled bronchodilators commonly used in the treatment of COPD.

        This review contains 6 highly rendered figures, 2 tables, and 178 references.

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      • 9

        Sarcoidosis and Immunologic Lung Disease

        By Manju P. Paul, MBBS; Michael C. Iannuzzi, MD, MBA, FACP
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        Sarcoidosis and Immunologic Lung Disease

        • MANJU P. PAUL, MBBSFellow, Division of Pulmonary and Critical Care Medicine, SUNY Upstate Medical University, Syracuse, NY
        • MICHAEL C. IANNUZZI, MD, MBA, FACPEdward C. Reifenstein Professor and Chair, Department of Medicine, SUNY Upstate Medical University, Syracuse, NY

        The first portion of the chapter is devoted to sarcoidosis, a systemic disease characterized by the presence of noncaseating granulomas, which accumulate in affected organs. The incidence, the organs involved, and disease severity depend on environmental exposures and host factors. The cause of sarcoidosis remains unknown. Any organ can be affected; however, involvement of the lung, heart, and nervous system contributes most to morbidity and mortality. The discussion of sarcoidosis includes the epidemiology, pathogenesis, diagnosis, and management of the disease. The second portion of the chapter covers a range of interstitial lung diseases of immunologic origin, including lung disease caused by collagen vascular diseases (systemic lupus erythematosus, rheumatoid arthritis, Sjögren syndrome, polymyositis and dermatomyositis, and mixed connective tissue disease), diffuse alveolar hemorrhage, hypersensitivity pneumonitis, lymphangioleiomyomatosis, idiopathic eosinophilic pneumonia, Langerhans cell histiocytosis, pulmonary alveolar proteinosis, and drug-induced infiltrative lung disease. Discussion of each of these diseases covers etiology, pathogenesis, diagnosis, differential diagnosis, management, complications, and prognosis. Tables outline the initial evaluation used for patients suspected of having sarcoidosis; extrapulmonary manifestations, imaging, and initial treatment of sarcoidosis; causes of granulomatous diseases; diseases causing diffuse alveolar hemorrhage; characteristics of hypersensitivity pneumonitis and interstitial inflammatory disorders; and causes and clinical manifestations of drug-induced lung diseases. Figures include the pathogenesis of sarcoidosis, radiographic stages of sarcoidosis, and imaging studies and histologic findings in various disorders described in the chapter. This chapter contains 166 references.

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      • 10

        Cystic Fibrosis and Non-cystic Fibrosis Bronchiectasis

        By Michael J. Stephen, MD
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        Cystic Fibrosis and Non-cystic Fibrosis Bronchiectasis

        • MICHAEL J. STEPHEN, MDAssistant Professor of Medicine, Drexel University School of Medicine, Philadelphia, PA

        Cystic fibrosis (CF) is an autosomal recessive disease characterized by an elevated sweat chloride level, diffuse bronchiectasis, and pancreatic exocrine deficiency. It is the most common lethal inherited disease in whites. Most patients present at birth or early childhood, although later diagnoses are not infrequent. Once CF was uniformly fatal at an early age, but advances in nutrition, airway clearance, and infection management have led to an average survival of 37 years. The newest aspect of care is the advent of protein modulators, which may increase life expectancy even further. This chapter discusses the epidemiology, genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, and treatment of CF. The definition, epidemiology, etiology, pathogenesis, diagnosis, management, and prognosis of non-CF bronchiectasis are also covered. Figures illustrate normal and abnormal CF transmembrane conductance regulators, the vicious cycle hypothesis of lung injury, rates of respiratory germs by age, the diagnosis of CF, the therapeutics pipeline for CF, forced expiratory volume in 1 second lung function percent predicted versus body mass index, and the median predicted survival age of patients with CF. A chest x-ray and chest computed tomographic scan of CF are also provided. Tables outline the most common CF mutations in 2011, class mutations of CF, a mnemonic for acute exacerbations of CF, the diagnosis of CF-related diabetes in a stable patient, sweat test values, and the differential diagnosis of bronchiectasis.
        This chapter contains 9 highly rendered figures, 6 tables, 143 references, 1 teaching slide set, and 5 MCQs.

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      • 11

        Interstitial and Fibrotic Lung Diseases

        By Lake D. Morrison, MD; Paul W. Noble, MD
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        Interstitial and Fibrotic Lung Diseases

        • LAKE D. MORRISON, MDDirector, Interstitial Lung Disease Clinic, Duke University Medical Center, Durham, NC
        • PAUL W. NOBLE, MDProfessor of Medicine, Chief, Division of Pulmonary, Allergy, and Critical Care Medicine, Duke University Medical Center, Durham, NC

        Interstitial lung disease refers to any of a number of conditions in which infiltration of the alveolar walls with an inflammatory and/or fibrotic process occurs, often leading to restriction of lung function and impairment of gas exchange. In interstitial lung diseases, proliferation of the fibroblasts and excessive collage deposition occur in response to some type of injury. Depending on a number of factors, including the cause and timing of injury, as well as factors unique to certain individuals, the inflammatory component of this response may be prominent or minor, and anti-inflammatory medications may or may not be effective. Because airways disease and other lung diseases also may affect the interstitium of the lung, and interstitial lung diseases can affect the airways, the term diffuse parenchymal lung disease (DPLD) is often preferred. This chapter outlines an approach to the patient with diffuse parenchymal lung disease, as well as describes the salient features of the individual idiopathic interstitial pneumonias. Figures illustrate several diagnostic techniques and tables present the classification and categorization of diffuse parenchymal lung diseases and summarizes the key features of the idiopathic interstitial pneumonias. This chapter contains 209 references.

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      • 12

        Occupational and Environmental Lung Diseases

        By Timothy J. Bedient, MD; David A. Schwartz, MD
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        Occupational and Environmental Lung Diseases

        • TIMOTHY J. BEDIENT, MDFellow Physician, Department of Pulmonary Sciences and Critical Care Medicine, University of Colorado Denver, Denver, CO
        • DAVID A. SCHWARTZ, MD

        The lungs are the major interface between humans and their environment, and consequently are a common site of environmentally induced disease. Thousands of environmental toxins and commercial chemicals are now in use, which may become aerosolized or airborne as fibers, fumes, mists, or dusts. People may be exposed to these agents in their occupation and in their workplace environment. Despite such exposures, pulmonary function in most persons is rarely affected as the lungs are equipped with a complex system that minimizes inhaled substances, potentially harmful effects, preserving the alveolar surface’s sensitive gas mechanisms. Nevertheless, lung diseases due to occupational and environmental exposures are both common and diverse. This chapter provides an organized approach to identifying inhalation exposures that can cause lung disease and discusses several specific occupational and environmentally induced lung diseases. Diseases discussed include occupational asthma, occupation-related chronic obstructive pulmonary disease, byssinosis, occupational parenchymal diseases, asbestosis and asbestos-induced pleural disease, pleural disease, silicosis, coal worker’s pneumoconiosis, kaolin pneumoconiosis, talc pneumoconiosis, mica pneumoconiosis, and other mineral silicate and miscellaneous pneumoconioses. Mixed obstructive and parenchymal diseases are covered, including hypersensitivity pneumonitis, inhalational bronchiolitis, and berylliosis, as well as occupational lung neoplasms such as bronchogenic carcinomas and mesothelioma. New figures include various photomicrographs, chest radiographs, and computed tomographic scans. Tables list occupational pulmonary disorders, minimum exposure history details that should be obtained from all patients, known and suspected causes of occupational asthma, plus occupations and industries associated with asbestosis and silicosis, toxic inhalations associated with bronchiolitis, agents listed as carcinogens with their respective occupation/industry, and estimated relative risks of occupational lung carcinogens. This chapter contains 271 references.

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      • 13

        Pulmonary Hypertension, Cor Pulmonale, and Other Pulmonary Vascular Conditions

        By Matthew Moll, MD; Mayank Sardana, MBBS; Harrison W. Farber, MD, FAHA, FCCP
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        Pulmonary Hypertension, Cor Pulmonale, and Other Pulmonary Vascular Conditions

        • MATTHEW MOLL, MDResident, Department of Internal Medicine, Boston Medical Center, Boston, MA
        • MAYANK SARDANA, MBBSFellow, Division of Cardiology, University of Massachusetts Medical School, Worcester, MA
        • HARRISON W. FARBER, MD, FAHA, FCCPProfessor of Medicine, Pulmonary Center, Boston University School of ­Medicine, Boston, MA

        This review covers the diseases that affect the pulmonary vasculature directly. These conditions include pulmonary hypertension; pulmonary arterial hypertension; chronic thromboembolic pulmonary hypertension; pulmonary hypertension attributed to left heart disease, lung disease and/or hypoxemia, and other disorders; cor pulmonale; pulmonary atriovenous malformations; and pulmonary aneurysms. Figures show changes in the pulmonary vasculature in pulmonary hypertension, pathways involved in the development of pulmonary hypertension, general guidelines for the evaluation of suspected pulmonary hypertension, enlarged proximal pulmonary arteries with pruning of distal pulmonary vasculature (typical of advanced pulmonary arterial hypertension), the remodeling of the heart and continuous-wave Doppler study results observed with chronic pulmonary hypertension, ventilation and perfusion scans of  the lungs with results typical of chronic thromboembolic pulmonary hypertension, and a general approach to the treatment of patients with pulmonary arterial hypertension. Tables list the revised nomenclature and classification of pulmonary hypertension, the World Health Organization classification of functional capacity in patients with pulmonary hypertension, advanced vascular medications for pulmonary artery hypertension, and perioperative management of pulmonary arterial hypertension.

        This review contains 8 highly rendered figures, 4 tables, and 118 references.

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      • 14

        Disorders of the Pleura, Mediastinum, and Hilum

        By Andrew D. Lerner, MD; David Feller-Kopman, MD
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        Disorders of the Pleura, Mediastinum, and Hilum

        • ANDREW D. LERNER, MDAssistant in Medicine, Interventional Pulmonology, Division of Pulmonary and Critical Care Medicine, Johns Hopkins Hospital, Baltimore, MD
        • DAVID FELLER-KOPMAN, MDDirector, Bronchoscopy and Interventional Pulmonology, Associate Professor of Medicine, Otolaryngology–Head and Neck Surgery, Interventional Pulmonology, Division of Pulmonary and Critical Care Medicine, Johns Hopkins Hospital, Baltimore, MD

        The pleura are a composition of two serous membranes: the visceral pleura lining the lungs and the parietal pleura lining the inner chest wall, diaphragm, and mediastinum. The pulmonary hilum is composed of blood vessels, airways, nerves, and lymph nodes and denotes the meeting point between the mediastinum and the pleural cavities. This review covers disorders of the pleura, mediastinum, and hilum. Figures show a schematic diagram of normal filtration/resorption of fluid in the pleural space; comparison of a simultaneously obtained chest x-ray (CXR) and a chest computed tomographic (CT) scan of the same patient; an algorithm for evaluation of patients with pleural effusion (PE); a three-compartment model of mediastinal anatomy; photographs of a resected, well-encapsulated thymoma and a benign, multiloculated thymic cyst that were completely removed by sternotomy, a resected esophageal duplication cyst, and an esophageal leiomyoma being removed by means of a right thoracotomy; CT scans demonstrating the characteristic appearance of an invasive thymoma, an extragonadal germ cell tumor, a primary mediastinal B cell lymphoma, a goiter with extension behind the trachea, right paratracheal adenopathy in the middle mediastinal compartment, a middle mediastinal cystic mass, a large subcarinal bronchogenic cyst, a tracheal chondrosarcoma, gas in the mediastinum due to esophageal perforation, and mediastinal fibrosis; coronal imaging showing a large, smooth muscle tumor associated with the distal esophagus; a barium swallow showing a large esophageal perforation that resulted in soilage of the middle mediastinum; and posteroanterior and lateral CXRs of a posterior mediastinal neurogenic tumor accompanied by a CT scan showing the posterior mediastinal neurogenic tumor visualized in the posteroanterior radiograph. Tables list physical characteristics of pleural fluid that suggest a diagnosis, common tests in the analysis of a PE, common discordant exudates, incidence and range of pleural fluid glucose in diseases with low glucose concentration, PEs with associated cell count differentials, differentiation between a chylothorax and a cholesterol PE, unilateral and bilateral PE, rapid reaccumulation following thoracentesis, spectrum of parapneumonic effusions, commonly encountered benign and malignant lesions of the middle mediastinum, clinical manifestations of granulomatous and fibrosing mediastinitis, origins and causes of pneumomediastinum, and neurogenic tumors of the posterior mediastinum.

         

        This review contains 19 highly rendered figures, 13 tables, 1 video, and 82 references.

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      • 15

        Chest Wall and Neuromuscular Disease

        By Başak Çoruh, MD; Joshua O. Benditt, MD
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        Chest Wall and Neuromuscular Disease

        • BAŞAK ÇORUH, MDSenior Fellow, Division of Pulmonary and Critical Care Medicine, University of Washington, Seattle, WA
        • JOSHUA O. BENDITT, MDProfessor, Division of Pulmonary and Critical Care Medicine, University of Washington, Seattle, WA

        Chest wall and neuromuscular diseases encompass a broad spectrum of illnesses that affect the mechanics of breathing. This chapter reviews the physiology of the respiratory system and the impact of these diseases. A brief discussion of various chest wall and neuromuscular diseases is included. The approach to the evaluation of a patient with suspected chest wall or neuromuscular disease, including key aspects of the history, physical examination, and diagnostic testing, is discussed. Respiratory care, including ventilation, cough, swallowing, and sleep, is described. Tables outline neuromuscular diseases affecting the respiratory system, causes of diaphragm weakness and paralysis, and a comprehensive approach to respiratory care in chest wall and neuromuscular disease. Figures include illustrations of the anatomy of the respiratory system and contraction of the diaphragm, pressure measures above and below the diaphragm, radiographic images of chest wall diseases and measurement of the Cobb angle, a computed tomographic scan of a patient with pectus excavatum, a graph showing patterns of pulmonary function testing in chest wall and neuromuscular disease, and photos of mouthpiece ventilation and a mechanical insufflator-exsufflator device. Videos show a fluoroscopic sniff test demonstrating unilateral diaphragm paralysis and the use of a mechanical insufflator-exsufflator device.

        This review contains 9 highly rendered figures, 2 videos, 3 tables, and 40 references.

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      • 16

        Lung Transplantation

        By Hilary J. Goldberg, MD, MPH
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        Lung Transplantation

        • HILARY J. GOLDBERG, MD, MPHProfessor of Medicine, Harvard Medical School, Boston, MA

        Lung transplantation is a potential therapeutic option for select candidates with advanced lung disease who have exhausted other therapeutic interventions, and in whom survival and/or quality of life are threatened by progression of disease. This chapter provides an overview of, and the indications for, lung transplantation (chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, cystic fibrosis, primary pulmonary hypertension, and 1-antitrypsin deficiency emphysema) and discusses candidate selection and evaluation, timing of transplantation (general and disease-specific guidelines), and types of organ donors (i.e., brain-dead donors, non–heart-beating donors, and living donors). Also discussed are types of transplant procedures (i.e., single-lung transplantation, bilateral lung transplantation, heart-lung transplantation, living-donor lobar transplantation), transplantation outcomes, posttransplantation management, and complications (i.e., primary graft dysfunction, airway complications, allograft rejection [hyperacute, acute, and chronic, humoral], and posttransplantation infection. Figures depict indications for transplantation, procurement rates, bilateral lung transplantation, and 5-year survival rates. Also shown are examples of primary graft dysfunction and airway stenosis, plus pathologic manifestations of acute rejection and chronic rejection. Tables show contraindications to transplantation, disease-specific guidelines for referral and transplantation, predictors of poor prognosis in cystic fibrosis patients, donor criteria, causes of death after lung transplantation, classification and histologic features of allograft rejection, the staging system for bronchiolitis obliterans syndrome, and complications of lung transplantation and posttransplantation immunosuppression. A sidebar lists Internet resources and registries. The chapter has 118 references.

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    • Critical Care
      • 1

        Approach to the Patient With Shock

        By David C. Mackenzie, MD, CM
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        Approach to the Patient With Shock

        • DAVID C. MACKENZIE, MD, CMDirector of Emergency Ultrasound, Maine Medicine Medical Center, Portland ME, Assistant Professor of Emergency Medicine, Tufts University School of Medicine, Boston, MA

        There are four main categories of shock: hypovolemic, distributive, cardiogenic, and obstructive. Although the main end point (i.e., inadequate delivery of oxygenated blood to the body’s tissues and organs) of each of these categories of shock is the same, the pathophysiologic mechanisms differ. This review covers the pathophysiology, stabilization and assessment, diagnosis and treatment, and disposition and outcomes for patients with shock. Figures show the Frank-Starling relationship illustrating changes in distributive and cardiogenic shock, the FAST examination, lung ultrasonography in pulmonary edema, pericardial effusion, apical four-chamber view of the heart with right ventricular enlargement, and a parasternal short-axis view of the heart. Tables list representative historical information associated with specific categories of shock; point-of-care ultrasound applications for the evaluation of shock; criteria for severe sepsis and septic shock; vasopressor dosages, mechanisms of action, clinical effects, and indications; anaphylaxis diagnostic criteria; and causes of cardiogenic shock.

        This review contains 6 highly rendered figures, 6 tables, and 32 references.

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      • 2

        Acute Respiratory Failure

        By Patricia Kritek, MD
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        Acute Respiratory Failure

        • PATRICIA KRITEK, MDAssociate Professor, Division of Pulmonary and Critical Care Medicine, University of Washington, Seattle, Washington

        Acute respiratory failure is due to a failure of one or more components of the respiratory system (the central nervous system; the peripheral nerves, the diaphragm, the ribcage and chest wall; the accessory muscles of breathing; and/or the lungs and the airways. The chapter begins by describing acute hypoxemic respiratory failure and then acute hypercarbic respiratory failure. The acute hypoxemic respiratory failure section discusses the mechanisms of hypoxemia, ventilation-perfusion mismatch, alveolar hypoventilation, low mixed venous oxygen concentration, and diffusion impairment. The acute hypercarbic respiratory failure section discusses physiology, diagnostic evaluation, treatment, non-invasive mechanical support, and invasive mechanical ventilation. Specific conditions covered include acute respiratory distress syndrome, obstructive lung disease, chronic obstructive pulmonary disease, and asthma. Separate sections cover complications of, and discontinuation of, mechanical ventilation. Tables include clinical causes of hypoxemic respiratory failure, indications for noninvasive positive pressure support, common causes of abnormal respiratory mechanics, and common causes of acute respiratory distress syndrome. One figure is a pressure-time curve from a volume-targeted breath showing the peak inspiratory pressure, and a second figure is a pressure-time curve from a volume-targeted breath showing autoPEEP (positive end-expiratory pressure). This chapter contains 64 references.

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      • 3

        Hemodynamic Monitoring in the ICU

        By Sunny Lim, MD; Andrew M. Luks, MD
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        Hemodynamic Monitoring in the ICU

        • SUNNY LIM, MDSenior Fellow, Division of Pulmonary and Critical Care Medicine, University of Washington, Seattle, WA
        • ANDREW M. LUKS, MDAssociate Professor, Division of Pulmonary and Critical Care Medicine, University of Washington, Seattle, WA

        This review examines the most commonly used hemodynamic monitoring devices in the intensive care unit. After a brief review of some important issues in hemodynamic monitoring, a variety of monitoring systems are considered, including arterial catheters, pulmonary artery catheters (PACs), less invasive hemodynamic monitors, central venous oxygen saturation (ScvO2) monitors, and point-of-care (POC) echocardiography. For each system, the basic operating principles, indications and limitations of use, complications, key issues in data interpretation, and evidence regarding utility in patient care are reviewed. Figures depict the distinction between correlation and agreement, a representative Bland-Altman plot, a square wave test, PAC waveforms, principles of pulmonary artery occlusion pressure measurement, measuring pulmonary artery occlusion pressure at end-exhalation, right atrial and ventricular pressure waveform, pulmonary artery pressure waveform, potential pulmonary artery occlusion waveforms, west zones of the lung and the pulmonary artery occlusion pressure measurements, and POC echocardiography. Tables outline landmarks during insertion of the PAC from the internal jugular and subclavian vein insertion sites; indications, contraindications, and complications of PAC insertion; a comparison of less invasive hemodynamic monitors; reasons for decreased mixed or ScvO2; and indications and contraindications for transesophageal echocardiography.

        This review contains 12 highly rendered figures, 7 tables, and 95 references.

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      • 4

        Cardiac Arrest and Resuscitation

        By Charles N. Pozner, MD; Jennifer L. Martindale, MD
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        Cardiac Arrest and Resuscitation

        • CHARLES N. POZNER, MDMedical Director, Neil and Elise Wallace STRATUS Center for Medical Simulation, Brigham and Women’s Hospital, Associate Professor of Medicine (Emergency Medicine), Harvard Medical School, Boston, MA
        • JENNIFER L. MARTINDALE, MDHarvard Affiliated Emergency Medicine Residency Program, Brigham and Women’s Hospital, 75 Francis Street, Neville House, Boston, MA

        The most effective treatment for cardiac arrest is the administration of high-quality chest compressions and early defibrillation; once spontaneous circulation is restored, post–cardiac arrest care is essential to support full return of neurologic function. This review summarizes the pathophysiology, stabilization and assessment, diagnosis and treatment, and disposition and outcomes of cardiac arrest and resuscitation. Figures show the foundations of cardiac resuscitation, ventricular arrhythmias, coronary perfusion pressure as a function of time, an algorithm for initial treatment of cardiac arrest, sample capnographs, and the electrocardiographic appearance of varying degrees of hyperkalemia. Tables include components of suboptimal cardiac resuscitation and corrective actions, recommended doses of medications commonly used in cardiac resuscitation, causes of pulseless electrical activity/asystolic arrest to consider, immediate post–return of spontaneous circulation checklist, and resuscitation goals during post–cardiac arrest care.

        This review contains 6 highly rendered figures, 5 tables, and 142 references.

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      • 5

        Approach to Shock

        By Jeremy B. Richards, MD, MA; Jakob I. McSparron, MD
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        Approach to Shock

        • JEREMY B. RICHARDS, MD, MAInstructor in Medicine, Harvard Medical School and Beth Israel Deaconess Medical Center, Boston, MA
        • JAKOB I. MCSPARRON, MDPulmonary and Critical Care Fellow, Harvard Combined Fellowship Program, Boston, MA

        Shock is due to cardiovascular or circulatory impairment resulting in inadequate delivery of oxygenated blood to the body’s organs and tissues. There are four clinically relevant categories of shock: distributive, cardiogenic, hypovolemic, and obstructive shock. This chapter discusses the clinical assessment and cardiovascular monitoring of undifferentiated shock and the pathophysiology, causes, history and physical examination, diagnostic tests, and initial management of the specific types of shock. Figures depict Starling curves in distributive and cardiogenic shock, telemetry tracings of complete heart block and ventricular fibrillation, focused assessment sonography in trauma (FAST) examination, tension pneumothorax, and cardiac tamponade. Tables outline representative historical information associated with specific categories of shock; vasopressor dosages, mechanisms of action, clinical effects, and indications; early goal-directed therapy for patients in shock; representative causes of cardiogenic shock; an example of a massive transfusion protocol; and causes of obstructive shock.

        This review contains 5 highly rendered figures, 6 tables, and 28 references.

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  • Rheumatology
    • 1

      Introduction to the Patient With Rheumatic Disease

      By David A. Fox, MD
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      Introduction to the Patient With Rheumatic Disease

      • DAVID A. FOX, MDProfessor, Division of Rheumatology, Department of Internal Medicine, University of Michigan Medical School and Health System, Ann Arbor, MI

      The rheumatic diseases encompass a broad spectrum of conditions that include inflammatory, metabolic, and structural diseases of the joints and adjacent musculoskeletal structures, chronic musculoskeletal pain syndromes, and a wide range of systemic autoimmune and autoinflammatory diseases that may or may not have articular manifestations. One in seven patients who visit a physician’s office has a complaint regarding the musculoskeletal system. Although many of these patients have benign, self-limited conditions that respond to simple remedies, some patients have serious, complex problems for which timely intervention may be crucial for a successful outcome. This review covers the approach to diagnosis, history, physical examination, laboratory studies, clinical presentation and initial approach, and prognosis of rheumatic diseases. Figures show variations in the temporal course and severity of pain in the rheumatic diseases, the approach to laboratory and imaging tests in the patient with joint pain, the initial approach to the patient with symptoms of monoarticular disease, and the initial approach to the patient with symptoms of polyarticular disease. Tables list common rheumatic diseases, symptoms and signs of rheumatologic emergencies, diagnostic clues from the review of systems, factors other than inflammation that influence the erythrocyte sedimentation rate, and differential diagnosis of diffuse aches and pains.

       

      This review contains 4 highly rendered figures, 5 tables, and 25 references.

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    • 2

      Rheumatoid Arthritis: Etiology and Pathogenesis

      By Anna-Karin H. Ekwall, MD, PhD; Gary S. Firestein, MD
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      Rheumatoid Arthritis: Etiology and Pathogenesis

      • ANNA-KARIN H. EKWALL, MD, PHDSpecialist in Rheumatology, University of California San Diego School of Medicine, La Jolla, CA, Researcher/Postdoc, The Sahlgrenska Academy at Gothenburg University, Gothenburg, Sweden
      • GARY S. FIRESTEIN, MDProfessor of Medicine, Dean and Associate Vice Chancellor of Translational Medicine, UC San Diego School of Medicine, La Jolla, California

      Rheumatoid arthritis (RA) is among the most common forms of chronic inflammatory arthritis. It affects approximately 1% of adults and is two to three times more prevalent in women than in men. There are no specific laboratory tests for RA; diagnosis depends on a constellation of signs and symptoms that can be supported by serology and radiographs. The disease evolves over many years as a consequence of repeated environmental stress causing inflammation and immune activation followed by a breakdown of tolerance in individuals with a specific genetic background. This review describes the definition of RA; its etiology, including genetics, infections, the role of smoking and citrullination of proteins, and epigenetic mechanisms; and its pathogenesis, including synovial histopathology, bone and cartilage damage, adaptive and innate immunity, and the role of cytokines and intracellular signaling. Tables include the 1987 American Rheumatism Association criteria for the classification of RA and the 2010 American College of Rheumatology/European League Against Rheumatism classification for RA. Figures show citrullinated proteins in airway cells, a section of a proliferative synovium from a patient with a classic RA, and scalloped regions of erosion at the junction between a proliferative inflamed rheumatoid synovium and the bone.

      This review contains 3 highly rendered figures, 2 tables, and 71 references.

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    • 3

      Rheumatoid Arthritis: Clinical Manifestations and Diagnosis

      By Anna-Karin H. Ekwall, MD, PhD; Gary S. Firestein, MD
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      Rheumatoid Arthritis: Clinical Manifestations and Diagnosis

      • ANNA-KARIN H. EKWALL, MD, PHDSpecialist in Rheumatology, University of California San Diego School of Medicine, La Jolla, CA, Researcher/Postdoc, The Sahlgrenska Academy at Gothenburg University, Gothenburg, Sweden
      • GARY S. FIRESTEIN, MDProfessor of Medicine, Dean and Associate Vice Chancellor of Translational Medicine, UC San Diego School of Medicine, La Jolla, California

      The onset and course of rheumatoid arthritis (RA) can be highly variable, and the lack of a specific biologic marker can make diagnosis difficult in early disease. A careful history and physical examination and the integration of clinical and laboratory data are often required. The presence of anti-citrullinated peptide antibodies (ACPAs) is one of the best predictors of progression to establish RA. This review describes the clinical manifestations used to diagnose RA, such as physical examination of the hands and wrists, elbows and shoulders, hips, knees, ankles and feet, and cervical spine. Extra-articular manifestations related to rheumatoid nodules, eyes, lungs, heart, blood and blood vessels, and neuromuscular involvement are reviewed. Laboratory tests and differential diagnosis are also discussed. Figures show synovitis in the hand and wrist, rheumatoid nodules near the extensor surface of the elbow, a pelvic radiograph of a patient with classic seropositive RA, erosions in the metatarsal heads and phalanges of the foot of a patient with classic seropositive RA, the anterior edge of the odontoid process, and a typical rheumatoid nodule.

      This review contains 6 highly rendered figures and 20 references.

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    • 4

      Rheumatoid Arthritis: Treatment

      By Anna-Karin H. Ekwall, MD, PhD; Gary S. Firestein, MD
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      Rheumatoid Arthritis: Treatment

      • ANNA-KARIN H. EKWALL, MD, PHDSpecialist in Rheumatology, University of California San Diego School of Medicine, La Jolla, CA, Researcher/Postdoc, The Sahlgrenska Academy at Gothenburg University, Gothenburg, Sweden
      • GARY S. FIRESTEIN, MDProfessor of Medicine, Dean and Associate Vice Chancellor of Translational Medicine, UC San Diego School of Medicine, La Jolla, California

      The main goal of treatment of rheumatoid arthritis (RA) has evolved from modest improvement to low disease activity and will soon be complete remission. To reach this goal, the rheumatologist and patient should define the goal and treatment strategy together. Disease activity should be measured regularly using validated composite measures such as disease activity score, simple disease activity index, and clinical disease activity index. Management involves efforts to relieve pain and discomfort, preserve strength and joint function, and prevent structural deformities. Surgical intervention is important for replacing destroyed joints and for restoring function and preventing further damage. This review discusses the role of drug therapy, including nonsteroidal antiinflammatory drugs, methotrexate, antimalarial drugs, sulfasalazine, leflunomide, tofacitinib, biologic drugs, T cell– and B cell–targeted therapy, glucocorticoids, and other immunosuppressive agents. Nonmedical therapy, surgery, and prognosis are also detailed. Tables include the American College of Rheumatology definition of improvement of RA and comparisons of various antirheumatic treatments using small-molecule and biologic drugs. Figures include an algorithm for pharmacologic management of RA and a graph showing mean disease activity scores.

      This review contains 2 highly rendered figures, 3 tables, and 54 references.

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    • 5

      Seronegative Spondyloarthritis

      By Walter P. Maksymowych, MB, ChB, FRCP (UK), FRCPC, FACP
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      Seronegative Spondyloarthritis

      • WALTER P. MAKSYMOWYCH, MB, CHB, FRCP (UK), FRCPC, FACPProfessor of Medicine, Department of Medicine, University of Alberta, Medical Scientist, Alberta Innovates-Health Solutions, Edmonton, AB

      The term spondyloarthritis encompasses a family of clinically, epidemiologically, and genetically related inflammatory diseases that primarily affect spinal and peripheral joints. The prototypic disease known as ankylosing spondylitis is characterized by progressive ankylosis in the joints of the axial skeleton that is readily evident on radiography and culminates in a permanently stooped spinal posture. Disease may not progress to ankylosis and may initially affect the peripheral joints and entheses, so the term spondyloarthritis is now considered the more appropriate term for this group of arthritides. This chapter provides a comprehensive overview of the classification, epidemiology, pathogenesis (including genetic factors, effector cytokines, bacteria and intestinal inflammation, and the link between inflammation and spinal ankylosis), pathology, diagnosis, management (including patient education, physical modalities, symptom-modifying antirheumatic drugs, second-line agents, biologic therapies, antibiotics, and surgery), and prognosis of spondyloarthritis. Graphs, algorithms, illustrations, and radiographic images are provided. Tables outline the modified New York criteria for ankylosing spondylitis, disease associations with genes outside the HLA locus in ankylosing spondylitis and overlap with Crohn disease and psoriasis, Assessments in SpondyloArthritis international Society (ASAS)/European League against Rheumatism (EULAR) recommendations for the management of ankylosing spondylitis, 2010 recommendations for the use of anti–tumor necrosis factor agents in patients with axial spondyloarthritis, and the ASAS core set for clinical record keeping. 

      This review contains 15 highly rendered figures, 5 tables, and 176 references.

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    • 6

      Systemic Lupus Erythematosus

      By Kyriakos A. Kirou, MD; Michael D. Lockshin, MD
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      Systemic Lupus Erythematosus

      • KYRIAKOS A. KIROU, MDAssistant Professor of Clinical Medicine, Weill Medical College of Cornell University, Co-director, Mary Kirkland Center for Lupus Care, Hospital for Special Surgery, 535 E 70th street, New York, NY 10021, Tel: 212-606-1728; Fax: 212-606-1012
      • MICHAEL D. LOCKSHIN, MDProfessor of Medicine and Obstetrics-Gynecology, Weill Medical College of Cornell University, Director, Barbara Volcker Center, Hospital for Special Surgery, 535 E 70th St, New York, NY 10021, Tel 212-606-1461; Fax 212-774-2374

      Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune illness characterized by autoantibodies directed at nuclear antigens that cause clinical and laboratory abnormalities, such as rash, arthritis, leukopenia and thrombocytopenia, alopecia, fever, nephritis, and neurologic disease. Most or all of the symptoms of acute lupus are attributable to immunologic attack on the affected organs. Many complications of long-term disease are attributable to both the disease and its treatment. Intense sun exposure, drug reactions, and infections are circumstances that induce flare; the aim of treatment is to induce remission. This chapter is divided into sections dealing with SLE’s definitions; epidemiology; pathogenesis; disease classification, diagnosis, and differential diagnosis; and treatment. The section about pathogenesis discusses autoantibodies, abnormal innate and adaptive immunity, genetic susceptibility, infections, female predominance, and the complications of chronic illness. The discussion of disease classification, diagnosis, and differential diagnosis takes into account systemic signs and symptoms, skin and mucosal involvement, pulmonary involvement, cardiovascular involvement, musculoskeletal involvement, gastrointestinal and hepatic involvement, hematologic and ontologic involvement, neuropsychiatric involvement, renal involvement, special presentations, and indices of disease activity and damage. The discussion of treatment covers active disease, treatment of antiphospholipid syndrome (APS), and SLE during pregnancy. Figures illustrate important features of the pathogenesis of systemic SLE, malar rash, rash of subacute cutaneous lupus erythematosus annular type, discoid lupus rashes, lesions of discoid lupus erythematosus, oral ulcers, pleural effusion due to SLE, gray matter transverse myelitis involving the conus medularis, and selected kidney biopsy histology in lupus nephritis. A graph shows the incidence of SLE based on sex, age, and race. Tables detail types of human lupus erythematosus other than SLE, subtypes of drug-induced lupus erythematosus, American College of Rheumatology revised criteria for the classification of SLE, clinical and immunologic criteria in the Systemic Lupus International Collaborating Clinics classification criteria, neuropsychiatric SLE attributed to SLE, the International Society of Nephrology/Renal Pathology Society classification of lupus nephritis, revised classification criteria for APS syndrome, SLE management goals, “standard of care” therapies for SLE, and management of proliferative lupus nephritis and nephrotic membranous lupus nephritis.

      This review contains 10 highly rendered figures, 10 tables, and 194 references.

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    • 7

      Scleroderma and Related Disorders

      By Kristine Phillips, MD, PhD
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      Scleroderma and Related Disorders

      • KRISTINE PHILLIPS, MD, PHDScleroderma Program, Division of Rheumatology, University of Michigan, Ann Arbor, MI

      Scleroderma spectrum diseases are a heterogeneous group of disorders that are distinguished by abnormalities of the connective tissue in the skin and, in some cases, other organs. Each disorder may be characterized by the extent of cutaneous and internal involvement, as well as histopathologic features of skin biopsy. Scleroderma spectrum diseases include systemic scleroderma, localized scleroderma, and eosinophilic fasciitis. This chapter reviews the classification, epidemiology, etiology, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, outcome measures, management, and clinical course of scleroderma as well as the definition and classification, etiology/genetics, differential diagnosis, and treatment of localized scleroderma. Also discussed are the definition and classification, epidemiology, etiology/genetics/pathogenesis, diagnosis, differential diagnosis, and treatment of eosinophilic fasciitis. Tables review the classification of—and antinuclear antibodies in—scleroderma as well as the key assessments and interventions in scleroderma management. Figures illustrate the disease's presentation and clinical manifestations, including several images of scleroderma of the hands; face, palmar, and buccal telangiectasias in a patient with scleroderma; a radiograph demonstrating calcinosis of the elbow; Raynaud’s phenomenon; high-resolution computed tomographic images of diffuse cutaneous scleroderma, scleroderma and severe pulmonary hypertension, and limited cutaneous scleroderma; plus an esophagram demonstrating hypomotility.

      This review contains 11 highly rendered figures, 3 tables, and 72 references.

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    • 8

      Idiopathic Inflammatory Myopathies

      By Adam Schiffenbauer, MD; Frederick W. Miller, MD, PhD
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      Idiopathic Inflammatory Myopathies

      • ADAM SCHIFFENBAUER, MD
      • FREDERICK W. MILLER, MD, PHDChief, Environmental Autoimmunity Group, Office of Clinical Research, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD

      The idiopathic inflammatory myopathies (IIMs), also known as myositis syndromes, are a collection of heterogeneous disorders that share the common feature of chronic muscle inflammation of unknown cause. These disorders may occur in adults or children and are sometimes associated with other connective tissue disorders and a variety of cancers. A combined clinical, laboratory, and pathologic evaluation is needed to establish the diagnosis of these acquired systemic connective tissue diseases to rule out the many disorders that mimic IIMs. This module reviews the classification of IIMs, including polymyositis, dermatomyositis, inclusion body myositis, myositis associated with other connective tissue diseases and cancer, and antisynthetase syndrome. The epidemiology; etiology, genetics, and environmental factors; pathophysiology and pathogenesis; diagnosis; differential diagnosis; treatment; and prognosis of IIMs are discussed. Tables describe the criteria for polymyositis, dermatomyositis, and inclusion body myositis; well-characterized subgroups of the IIMs in adults and children; presentation of polymyositis; differential diagnosis of muscle weakness or pain; features that assist in discriminating IIMs from other myopathies; goals for managing IIMs; and key factors for achieving adequate corticosteroid response in IIMs. Figures demonstrate skin findings in IIMs, muscle pathology of IIMs, magnetic resonance imaging of three patients with different IIMs, and treatment approaches to the management of myositis patients.

      This review contains 4 highly rendered figures, 8 tables, and 80 references.

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    • 9

      Systemic Vasculitis Syndromes

      By Alexandra Villa-Forte, MD, MPH; Brian F. Mandell, MD, PhD, FACP
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      Systemic Vasculitis Syndromes

      • ALEXANDRA VILLA-FORTE, MD, MPHStaff Physician, Center for Vasculitis Care and Research, Orthopedic and Rheumatologic Institute, Cleveland Clinic, Cleveland, OH
      • BRIAN F. MANDELL, MD, PHD, FACPProfessor of Medicine, Department of Rheumatic Immunologic Disease, Vice Chairman of Medicine, Cleveland Clinic, Lerner College of Medicine, Case Western Reserve University, Cleveland, OH

      Vasculitis is defined by histologic evidence of inflammation that involves the blood vessels. The diagnosis of a specific primary vasculitic disorder depends on the pattern of organ involvement, the histopathology, the size of affected blood vessels, and the exclusion of diseases that can cause “secondary” vasculitis. This review presents an approach to the patient suspected of having vasculitis, and goes on to discuss small vessel vasculitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, polyarteritis nodosa, Kawasaki disease, large vessel arteritis, and Behçet disease. Figures show classification of the systemic vasculitis syndromes, the relationships among the causes of small vessel (“hypersensitivity”) vasculitis, palpable purpura of the distal extremities, saddle nose deformity, the nodular infiltrates of the lung in granulomatosis with polyangiitis shown on plain radiograph as well as computed tomography, necrotizing scleritis, livedo reticularis, and angiograms of a patient with Takayasu arteritis. Tables list selected laboratory tests for patients with multisystem disease and possible vasculitis, practical comments on immunosuppressive therapies for vasculitis, features of vasculitis, diagnostic criteria for Kawasaki disease, and giant cell arteritis.

      This review contains 8 highly rendered figures, 5 tables, and 59 references.

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    • 10

      Crystal-induced Joint Disease

      By N. Lawrence Edwards, MD, FACP, FACR
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      Crystal-induced Joint Disease

      • N. LAWRENCE EDWARDS, MD, FACP, FACRProfessor and Program Director, Vice Chairman, Department of Medicine, University of Florida College of Medicine, Gainesville, FL

      The destructive potential of intracellular crystals has been recognized for over a century. The mechanisms by which crystals induce inflammation and bone and cartilage destruction have been elucidated over the past decade. The three most common crystal-induced arthropathies are caused by precipitation of monosodium urate monohydrate, calcium pyrophosphate dihydrate (CPP) and basic calcium phosphate. The definition, epidemiology, pathogenesis and etiology, diagnosis, and treatment of gout and CPP crystal deposition are reviewed, as well as the clinical stages of gout (i.e., acute gouty arthritis, intercritical gout, advanced gout, nonclassic presentations of gout, and other conditions associated with gout). Also reviewed are the clinical manifestations of calcium pyrophosphate dihydrate deposition disease (CPPD), such as asymptomatic CPPD, osteoarthritis with CPPD, acute CPP crystal arthritis, and chronic CPP crystal inflammatory arthritis. Figures illustrate renal transport of urate, monosodium urate crystals, acute gouty flare, advanced gouty arthritis, gouty synovial fluid, radiographic changes of advanced gout, ultrasound appearance of the femoral intercondylar cartilage, pharmacologic management of gout, the effect of gender and age on knee chondrocalcinosis, radiographs of chondrocalcinosis, and compensated polarized microscopy of CPPD. Tables present the major factors responsible for hyperuricemia, characteristics of classic gouty flares, antiinflammatory therapy for gout, and urate-lowering therapy. This chapter contains 90 references.

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    • 11

      Osteoarthritis

      By Christopher Wise, MD, FACP
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      Osteoarthritis

      • CHRISTOPHER WISE, MD, FACPRobert Irby Professor of Medicine, Division of Rheumatology, Allergy, and Immunology, Department of Internal Medicine, Virginia Commonwealth University, Medical College of Virginia, Richmond, VA

      Osteoarthritis is a common form of arthritis characterized by degeneration of articular cartilage and pathologic changes in surrounding bone and periarticular tissue. The disease process results in pain and dysfunction of affected joints and is a major cause of disability in the general population. Prognosis is variable; greater muscle strength, mental health, self-efficacy, social support, and aerobic exercise are associated with better outcomes. This review outlines the classification of osteoarthritis (primary and secondary) and its epidemiology and etiologic factors, including risk factors, normal articular cartilage, and pathologic changes. Diagnosis is reviewed in terms of general considerations and specific joint involvement and related complications. The differential diagnosis is discussed. Management of osteoarthritis includes nonpharmacologic measures, pharmacologic therapy, surgery, and disease-modifying or chondroprotective therapy. Tables describe causes of secondary osteoarthritis, risk factors for osteoarthritis, and treatment of osteoarthritis. Figures demonstrate the microscopic appearance of normal and osteoarthritic articular cartilage, the diagnostic process for osteoarthritis, the hands of a patient with typical primary osteoarthritis, destructive changes in the interphalangeal joints, knee radiographs, and an osteoarthritic hip joint.

      This review contains 6 highly rendered figures, 3 tables, and 113 references.

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    • 12

      Back Pain and Common Musculoskeletal Problems

      By Christopher M. Wise, MD; Huzaefah Syed, MD
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      Back Pain and Common Musculoskeletal Problems

      • CHRISTOPHER M. WISE, MDW. Robert Irby Professor of Medicine, Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Virginia Commonwealth University Health System, Richmond, VA
      • HUZAEFAH SYED, MDAssistant Professor of Medicine, Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Virginia Commonwealth University Health System, Richmond, VA

      Knowledge of the common nonarticular regional rheumatic disorders is important because of their high prevalence in primary care practice, the dependence on clinical findings for diagnosis, and the high cost that can result from unnecessary laboratory evaluations. The ability to recognize important patterns of pain and associated physical signs is essential to making a correct diagnosis; in most cases, radiographic and laboratory studies are not needed. This review covers the common causes of pain in the neck, back, shoulder, chest wall, elbow, hand and wrist, hip girdle, knee and lower leg, and ankle and foot. Figures show magnetic resonance imaging of the lumbar spine showing multilevel stenosis of the spinal canal due to degenerative osteophytes, causes of shoulder pain, the anatomy of the elbow, carpal tunnel syndrome, and causes of foot pain. Tables list indications of underlying conditions and suggested imaging in patients with acute low back pain, and the differential diagnosis of hip girdle pain.

      This review contains 5 highly rendered figures, 2 tables, and 100 references.

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    • 13

      Fibromyalgia

      By Daniel Joseph Clauw, MD
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      Fibromyalgia

      • DANIEL JOSEPH CLAUW, MDProfessor of Anesthesiology, Medicine and Psychiatry, Director, Chronic Pain and Fatigue Research Center, University of Michigan, Ann Arbor, MI

      Clinicians often encounter individuals who present with pain that they cannot adequately explain based on the degree of damage or inflammation noted in peripheral tissues. This typically prompts an evaluation looking for a cause of the pain. If no cause is found, these individuals are often given a diagnostic label that merely connotes that the patient has chronic pain in a region of the body, without an underlying mechanistic cause. Fibromyalgia (FM) is merely the current term for widespread musculoskeletal pain for which no alternative cause can be identified. This review covers the epidemiology, etiology/genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, and complications and prognosis of FM. Figures show underlying mechanisms that can cause chronic pain; an individual’s “set point” or “volume control setting” for pain as set by a variety of factors, including the levels of neurotransmitters that either facilitate pain or reduce pain transmission; the 2011 Fibromyalgia Survey Criteria; symptoms and syndromes frequently seen in individuals with FM; the distribution of the 2011 Fibromyalgia Survey scores in a large cohort of individuals undergoing joint replacement surgery; and an algorithm showing the importance of dually focused treatment for FM and other chronic pain conditions. Tables list clinical characteristics of centralized pain, pharmacologic therapies for FM, and nonpharmacologic therapies for FM.

      This review contains 6 highly rendered figures, 3 tables, and 99 references.

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    • 14

      Autoinflammatory Syndromes

      By Arturo Diaz, MD
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      Autoinflammatory Syndromes

      • ARTURO DIAZ, MDInstructor of Medicine, Harvard Medical School, Division of Rheumatology, Beth Israel Deaconess Medical Center, Boston, MA

      The autoinflammatory syndromes are a group of diseases characterized by apparently spontaneous episodes of fever and inflammatory manifestations in several organs. Some of these conditions, such as familial Mediterranean fever (FMF), have been known for over a century, but others have only recently been defined. The discovery of the genetic defects underlying the pathophysiology of these diseases has been critical for their understanding and eventual grouping in a new category of diseases. Despite their rarity, the monogenic autoinflammatory syndromes are relevant because identification of the causative genetic variants has greatly expanded our understanding of the inflammatory process and the innate immune system and defined new diseases and their treatment. In addition, the autoinflammatory syndromes have been incorporated in the differential diagnosis of fever of unknown origin. In this chapter, the following selected syndromes are reviewed: FMF; cryopyrin-associated periodic syndromes; tumor necrosis factor–associated periodic fever syndrome; hyper-IgD syndrome; pyogenic sterile arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome; Blau syndrome; deficiency of the interleukin-1 receptor antagonist; Majeed syndrome; cherubism; joint contractures, muscular atrophy, macrocytic anemia, and panniculitis-associated lipodystrophy (JMP) syndrome; CANDLE syndrome; systemic-onset juvenile idiopathic arthritis; and adult-onset Still disease. Each syndrome is broken down by epidemiology, etiology, pathogenesis, diagnosis, differential diagnosis, management, complications, and prognosis.

      This review contains 4 highly rendered figures, 5 tables, and 96 references.

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    • 15

      Primary Sjögren Syndrome

      By Melissa A. Wells, MD; E. William St. Clair, MD
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      Primary Sjögren Syndrome

      • MELISSA A. WELLS, MDFellow, Division of Rheumatology and Immunology, Duke University Medical Center, Durham, NC
      • E. WILLIAM ST. CLAIR, MDProfessor of Medicine and Immunology, Division of Rheumatology and Immunology, Duke University Medical Center, Durham, NC

      This review focuses on the primary category of Sjögren syndrome (SS), a chronic inflammatory condition that is defined by the presence of dry eyes (keratoconjunctivitis sicca) or dry mouth (xerostomia) in the absence of other rheumatologic diseases. SS may also have extraglandular manifestations in the form of pulmonary, renal, gastrointestinal, and neurologic diseases that can cause significant morbidity and increased mortality and is distinct from other connective tissue diseases.

      Although the etiology of primary SS is unknown, genome-wide association studies are continuing to reveal that susceptibility to the disease is based on genetic predisposition; patients with primary SS have been identified with several non–major histocompatibility complex genetic polymorphisms that are statistically associated with increased disease susceptibility. In a recent study, blood CD4+ T cells from patients with primary SS were shown to differ in their patterns of DNA methylation compared with healthy controls and demonstrated that many genes involved in lymphocyte activation and the immune response were poised for transcription.

      Treatment of primary SS mainly involves relief of symptoms and prevention of long-term disease complications. Although biologic therapies have been studied, the results so far have been either negative or inconclusive.

      This review contains 5 highly rendered figures, 5 tables, and 89 references.

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    • 16

      Septic Arthritis, Septic Bursitis, and Osteomyelitis

      By Cameron Ashbaugh, MD
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      Septic Arthritis, Septic Bursitis, and Osteomyelitis

      • CAMERON ASHBAUGH, MDAssistant Professor of Medicine, Harvard Medical School, Associate Physician, Division of Infectious Diseases, Brigham and Women's Hospital, Boston, MA

      Infections of joints and bones are important causes of morbidity due to the potential for permanent injury to structures necessary for mechanical support and useful motion. The spectrum of disease is broad, with host factors, pathogen, site of infection, and comorbidities all influencing outcome. In some cases of bone infection, cure may not be possible, and the therapeutic goal becomes control. This review details the epidemiology, pathogenesis, diagnosis, differential diagnosis, treatment, and prognosis of septic arthritis, septic bursitis, vertebral body osteomyelitis, pedal osteomyelitis in association with diabetes, and chronic posttraumatic osteomyelitis with union or malunion.

      Figures include algorithms for the initial evaluation and management of a suspected septic joint, suspected vertebral body osteomyelitis, osteomyelitis in diabetic patients with neuropathic ulcers, and chronic posttraumatic osteomyelitis; pathophysiology of bone infection; magnetic resonance imaging (MRI) of vertebral body osteomyelitis; probe-to-bone test for diagnosing osteomyelitis in the diabetic foot; MRI of diabetic foot infection with osteomyelitis; nonsurgical treatment of osteomyelitis of the foot in a patient with diabetes; cutaneous sinus; a healed tibial fracture with malunion and chronic osteomyelitis; and a sequestrum associated with chronic tibial osteomyelitis. Tables describe representative studies with likelihood ratios for diagnostic tests used in the evaluation of native joint septic arthritis and diabetic pedal osteomyelitis; risk factors for the development of septic arthritis in patients with underlying joint disease; microbiology in septic arthritis, vertebral body osteomyelitis, and diabetic pedal osteomyelitis; empirical antibiotic therapy for septic arthritis; suggested tests and test frequencies to monitor for antibiotic toxicity; the two most commonly referenced classification schemes for osteomyelitis; supportive findings for diagnosis of chronic osteomyelitis; antibiotic therapy for vertebral osteomyelitis awaiting culture results and with unknown or established microbiology; International Working Group on the Diabetic Foot approach to diagnosis and management of diabetic pedal osteomyelitis; antibiotic therapy for diabetic pedal osteomyelitis with unknown or established microbiology; and antibiotic duration for diabetic pedal osteomyelitis. 

      This review contains 13 highly rendered figures, 15 tables, and 187 references.

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  • Women's Health
    • 1

      Primary and Preventive Care of Women

      By Janet B. Henrich, MD
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      Primary and Preventive Care of Women

      • JANET B. HENRICH, MDAssociate Professor of Medicine and Obstetrics and Gynecology, Yale University, New Haven, CT

      Women’s health can be defined as diseases or conditions that are unique to women or that involve gender differences that are particularly important to women. This definition acknowledges the increasing scientific evidence supporting a focus on sex and gender and expands the concept of women’s health beyond the traditional focus on reproductive organs and their function. Over time, the definition has come to include an appreciation of wellness and prevention, the interdisciplinary and holistic nature of women’s health, the diversity of women and their health needs over the life span, and the central role of women as patients and as active participants in their health care. This broader interdisciplinary perspective has important implications for clinicians providing care to women. In addition to understanding basic female physiology and reproductive biology, clinicians need to appreciate the complex interaction between the environment and the biology and psychosocial development of women. When dealing with conditions that are not specific to women, clinicians need to be aware of those aspects of disease that are different in women or have important gender implications. The ability to apply this information requires that clinicians adopt attitudes and behavior that are culturally and gender sensitive. Figures visualize female life expectancy, age-adjusted death rates, female breast cancer incidence and death rates, trends in female cigarette smoking, and the U.S. Preventive Services Task Force guidelines for preventive primary care in women. 
      This chapter contains 5 highly rendered figures, 52 references, 5 MCQs, and 1 teaching slide set.

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    • Menses and Fertility
      • 1

        Normal and Abnormal Menstruation

        By Janet E. Hall, MD
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        Normal and Abnormal Menstruation

        • JANET E. HALL, MDProfessor of Medicine, Harvard Medical School, Associate Chief, Reproductive Endocrine Unit, Massachusetts General Hospital, Boston, MA

        Normal reproductive function requires precise integration of hormonal events involving the hypothalamus, the pituitary, and the ovary, with the uterus, vagina, and breast acting as key end organs for ovarian steroid effects. This chapter discusses the physiology of the reproductive system in women; the assessment of reproductive function; and the epidemiology, etiology, diagnosis, and treatment of primary and secondary amenorrhea, abnormal vaginal bleeding—including menorrhagia, menometrorrhagia, and hypomenorrhea—and dysmenorrhea. Figures illustrate the relationship between the hypothalamus, pituitary, and ovaries in reproductive function and normal menstrual cycle function; an algorithm depicts the evaluation of amenorrhea. Tables list the relative frequency of the causes of amenorrhea and the neuroanatomic causes of hypogonadotropic hypogonadism. This chapter has 42 references.

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      • 2

        Premenstrual Syndrome

        By Sarah L. Berga, MD; Jessica B. Spencer, MD, MSc
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        Premenstrual Syndrome

        • SARAH L. BERGA, MDJames Robert McCord Professor and Chairman, Department of Gynecology and Obstetrics, Emory University School of Medicine, Atlanta, GA
        • JESSICA B. SPENCER, MD, MSCAssistant Professor, Department of Gynecology and Obstetrics, Emory University School of Medicine, Altlanta, GA

        Premenstrual syndrome (PMS) is a recurrent constellation of affective and physical symptoms that begin during the luteal phase of the menstrual cycle and resolve completely or almost completely during the follicular phase. Symptoms range in severity from mild to severe. The pathophysiology of PMS is discussed in this chapter, and potential causes are listed in a table. The diagnosis and differential diagnosis are reviewed. To warrant medical attention, evaluation, and intervention, premenstrual symptoms must be recurrent and sufficiently severe to interfere with daily work and social activities. Mild cases of PMS can be treated with lifestyle modification (e.g., good sleep patterns, regular exercise) and nonpharmacologic therapy (e.g., bright-light therapy, stress management, behavioral therapy). More severe cases warrant aggressive intervention, with pharmacologic therapy and even surgery in women who respond very well to a gonadotropin-releasing hormone (GnRH) agonist and have completed childbearing. This chapter contains 49 references.

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      • 3

        Contraception

        By Sarah L. Berga, MD; Carrie Cwiak, MD, MPH
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        Contraception

        • SARAH L. BERGA, MDJames Robert McCord Professor and Chairman, Department of Gynecology and Obstetrics, Emory University School of Medicine, Atlanta, GA
        • CARRIE CWIAK, MD, MPHAssociate Professor, Director of the Division of Family Planning, Director of the Fellowship in Family Planning, Department of Gynecology and Obstetrics, Emory University School of Medicine, Medical Director, Family Planning Clinic, Grady Health System, Atlanta, GA

        Although contraception is meant to prevent implantation and conception, most methods of contraception also reduce the risk of acquiring sexually transmitted infections (STIs). Contraceptive methods are categorized as reversible (e.g., barrier methods, oral contraceptives) or irreversible (i.e., surgical sterilization), and this chapter evaluates their effectiveness. Most methods are designed for use by women. The methods of contraception discussed in this chapter include combined estrogen-progestin contraceptives, progestin-only contraceptives, intrauterine devices, barrier methods, periodic abstinence, sterilization, and emergency contraception, including Plan B One-Step, which, since 2013, is available for men and women over the counter without age restrictions. The benefits, drawbacks, and side effects of each method are reviewed. The decision regarding which form of contraception to choose can be influenced by age, motivation, marital status, partner attitude, perceived risk of pregnancy, frequency of intercourse, medical conditions, costs, cultural considerations, and religious beliefs. Figures include graphs comparing the risk of unintended pregnancy using no, oral, and intrauterine contraception and graphs illustrating pregnancy rates with long-acting reversible contraceptives, short-acting contraceptives, and emergency contraceptives. The chapter also provides illustrations of the chemical structure of progesterone, norethindrone, and spironolactone and the typical effectiveness of contraceptive methods. Tables list commercially available reversible contraceptives, contraindications to long-acting reversible contraception, noncontraceptive benefits of hormonal contraceptives methods, and contraindications to combined estrogen-progestin methods.

        This chapter contains 96 references, 4 tables, 6 highly rendered figures, and 5 MCQs.

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      • 4

        Infertility

        By Alan H. DeCherney, MD; Eric D. Levens, MD
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        Infertility

        • ALAN H. DECHERNEY, MDChief, Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD
        • ERIC D. LEVENS, MDShady Grove Fertility Reproductive Science Center, Rockville, MD

        Infertility is the inability to conceive after 1 year of regular sexual intercourse without the use of contraception. The incidence of infertility has been increasing over the past 40 years, and estimates are that nearly 12% of women aged 15 to 44 in the United States have used fertility services. This chapter discusses factors contributing to male and female infertility, including unexplained infertility, and describes the evaluation and treatment of the infertile couple. Figures illustrate the causes of infertility in couples and in women, the management of infertility and anovulatory infertility, ovulation induction with clomiphene citrate, and the development of the embryo. A video shows embryonic development from the two-cell stage to the hatched blastocyst stage. Tables list the fecundity of normal couples over time, the causes of infertility in men, semen analysis parameters, and the steps of in vitro fertilization. This chapter contains 40 references.

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      • 5

        Ectopic Pregnancy and Spontaneous Abortion

        By Alan H. DeCherney, MD; Eric D. Levens, MD
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        Ectopic Pregnancy and Spontaneous Abortion

        • ALAN H. DECHERNEY, MDChief, Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD
        • ERIC D. LEVENS, MDShady Grove Fertility Reproductive Science Center, Rockville, MD

        Ectopic pregnancy, the implantation of an embryo outside the endometrial cavity, is the leading cause of morbidity and mortality in the first trimester. The embryo may be implanted in the fallopian tubes, ovaries, abdomen, or cervix, with the fallopian tubes being the site of implantation in 95% of cases. If left untreated, ectopic pregnancy can result in rupture of the fallopian tube, which can lead to hemorrhagic shock and death. The signs and symptoms of ectopic pregnancy and diagnosis and treatment are detailed in the chapter. Spontaneous abortion, defined as a natural termination of a pregnancy before 20 weeks’ gestation, occurs in almost 30% of known pregnancies and an estimated 50% of all conceptions. Causes include genetic, environmental, endocrine, and immunologic factors; anatomic abnormalities; antiphospholipid syndrome; and polycystic ovary syndrome. Tables describe signs and symptoms of ectopic pregnancy, patient history consistent with suspected ectopic pregnancy, serologic measures that differentiate an extrauterine pregnancy or abnormal gestation from an intrauterine pregnancy, criteria and contraindications for methotrexate treatment, and factors associated with recurrent pregnancy loss. Figures include a graph depicting the number of ectopic pregnancies in the United States from 1970 to 1992, multicultural surveillance for ectopic pregnancy, and the incidence of ectopic pregnancy in adolescents versus adults. Algorithms outline the management of ectopic pregnancy and spontaneous abortion and metabolic workup during pregnancy.

        This review contains 6 highly rendered figures, 5 tables, and 48 references.

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      • 6

        Medical Complications in Pregnancy

        By Ellen W. Seely, MD; Jeffrey L. Ecker, MD
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        Medical Complications in Pregnancy

        • ELLEN W. SEELY, MDDirector of Clinical Research, Division of Endocrinology, Diabetes, and Hypertension, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA
        • JEFFREY L. ECKER, MDDirector, Fellowship in Maternal Fetal Medicine, Department of Obstetrics and Gynecology, Massachusetts General Hospital, Professor, Harvard Medical School, Boston, MA

        Medical complications and intercurrent disease have long presented challenges to obstetricians and other medical providers caring for pregnant women. Contemporary medical practice and treatments have only added to these challenges. Advances in disease management mean that patients with some conditions (e.g., cystic fibrosis) whose life expectancies in the past would have precluded pregnancy are now living to reproductive age. Furthermore, treatments to restore fertility allow the barrier of age, as well as anatomic and genetic barriers, to be surmounted. All of these advances emphasize the need for careful and considered collaboration between clinicians caring for women of reproductive age who are not pregnant and those who care for them during pregnancy. This review discusses pregnancy planning and counseling, principles of teratogenesis, physiologic changes in pregnancy, cardiovascular disease, diabetes mellitus, thyroid disease, thrombophilia, asthma, infectious diseases, renal disease, autoimmune diseases, cancer, neurologic diseases, substance use, intrahepatic cholestasis, and pregnancy-specific conditions. Tables list elements of preconception care and counseling, the Food and Drug Administration drug classification system for pregnancy, selected drugs with suspected or known teratogenic potential, and physiologic changes of pregnancy.

        This review contains 4 tables and 116 references.

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      • 7

        Polycystic Ovary Syndrome

        By Kimberly Keefe, MD; Lubna Pal, MBBS, MRCOG (UK), MS, FACOG
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        Polycystic Ovary Syndrome

        • KIMBERLY KEEFE, MDResident physician, Department of Obstetrics, Gynecology, and Reproductive Sciences; Yale School of Medicine, New Haven, CT USA
        • LUBNA PAL, MBBS, MRCOG (UK), MS, FACOGAssociate Professor, Director, Program for Polycystic Ovarian Syndrome, Director, Menopause Program, Associate Chair for Education, Department of Obstetrics, Gynecology & Reproductive Sciences, Yale University School of Medicine, New Haven, CT

        Polycystic ovary syndrome (PCOS) is a commonly encountered endocrine disorder that is characterized by a combination of hyperandrogenism, menstrual disturbances (predominantly oligomenorrhea), and a classic sonographic polycystic ovarian morphology. Additional sequelae associated with PCOS include infertility and enhanced risk of developing type 2 diabetes mellitus and cardiovascular disease. The prevalence of depressive disorders and obstructive sleep apnea is disproportionately higher in women with PCOS, and this population is at increased risk for endometrial hyperplasia and endometrial cancer. This chapter reviews the definition, epidemiology, etiology and genetics, pathophysiology, diagnosis (including history, physical examination, and laboratory tests), differential diagnosis, and management of PCOS. Syndromes and diseases associated with PCOS are discussed. Tables describe the three approaches to PCOS, conditions mimicking PCOS, diagnosis of the metabolic syndrome in women using the National Cholesterol Education Program criteria, workup for PCOS, comprehensive care for PCOS patients, and the multistep approach to treatment of PCOS-related anovulatory infertility. Figures include transvaginal ultrasound images of a normal ovary and a multifollicular ovary in a woman with PCOS, the Ferriman-Gallwey scoring system for quantifying hirsutism, acanthosis nigrans on the neck of a woman with PCOS and insulin resistance, the relationship between body mass index and basal luteinizing hormone levels, oral glucose challenge results in nonobese women with PCOS, and an algorithm for treatment decision making.

        This review contains 6 highly rendered figures, 6 tables, and 120 references.

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      • 8

        Hirsutism and Hyperandrogenism

        By Deborah E. Ikhena, MD; Lubna Pal, MBBS, MRCOG (UK), MS, FACOG
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        Hirsutism and Hyperandrogenism

        • DEBORAH E. IKHENA, MDResident Physician, Department of Obstetrics and Gynecology, University of Massachusetts Medical School, Worcester, MA
        • LUBNA PAL, MBBS, MRCOG (UK), MS, FACOGAssociate Professor, Director, Program for Polycystic Ovarian Syndrome, Director, Menopause Program, Associate Chair for Education, Department of Obstetrics, Gynecology & Reproductive Sciences, Yale University School of Medicine, New Haven, CT

        Hirsutism is defined as the presence in females of terminal, or dark, coarse hairs that grow in a pattern normally seen in males. The presence of hirsutism is commonly associated with excess androgen production (hyperandrogenism) and warrants further evaluation. In addition to hirsutism, hyperandrogenism may also present clinically as acne, androgenetic alopecia, and menstrual irregularities. Regardless of the severity, hirsutism can be a very disconcerting problem for women and should be addressed with concern and sympathy by clinicians. This chapter discusses the epidemiology, etiology, physiology, diagnosis, differential diagnosis, and treatment of hirsutism. Tables describe common iatrogenic causes of hyperandrogenism, laboratory evaluation, differential diagnosis of hirsutism and hyperandrogenism, and pharmacologic management options for symptomatic clinical hyperandrogenism. Figures include an outline of the hair cycle, a clinical photo of clitoromegaly, and a modified Ferriman-Gallwey scoring system.

        This chapter contains 3 highly rendered figures, 4 tables, 160 references, and 5 MCQs.

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      • 9

        Endometriosis

        By Matthew L. Macer, MD; Hugh S. Taylor, MD
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        Endometriosis

        • MATTHEW L. MACER, MDPhysician-House Staff, Department of Obstetrics, Gynecology, and Reproductive Sciences, Yale School of Medicine, New Haven, CT
        • HUGH S. TAYLOR, MDAnita O’Keeffe Young Professor and Chair, Department of Obstetrics, Gynecology and Reproductive Sciences, Yale School of Medicine, Chief, Department of Obstetrics and Gynecology, Yale-New Haven Hospital, New Haven, CT

        Endometriosis is an estrogen-dependent disease characterized by the presence of ectopic endometrial implants. The most common sites of endometriosis, in decreasing order, are the ovaries, anterior/posterior cul-de-sac, broad and uterosacral ligaments, uterine serosa, fallopian tubes, sigmoid colon, and appendix. Lesions undergo cycles of growth and bleeding in tandem with the menstrual cycle. Intraperitoneal bleeding and lesion growth elicit an inflammatory response in the pelvis that is associated with pain and infertility. This chapter discusses the epidemiology and risk factors, pathogenesis, diagnosis, and prognosis related to endometriosis. The relationship between endometriosis and infertility and the treatment of endometriosis-associated pelvic pain and infertility are also described. Tables review current theories of pathogenesis, hormone effects on endometrial tissue, proposed mechanisms of causing infertility, stepwise treatment of endometriosis pelvic pain, gonadotropin-releasing hormone (GnRH) agonists, add-back therapy for use with GnRH agonist therapy, and stepwise treatment of infertility in endometriosis. Figures include classic endometriosis implants, laparoscopic images of endometriosis on the uterosacral ligament and peritoneal surface posterior to the uterus, and excision of an implant.

        This review contains 3 highly rendered figures, 7 tables, and 87 references.


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      • 10

        Menopause

        By Susan D. Reed, MD, MPH; Eliza L. Sutton, MD, FACP
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        Menopause

        • SUSAN D. REED, MD, MPHProfessor, Department of Obstetrics and Gynecology, Department of Epidemiology, Director, Women’s Reproductive Health Research Program, University of Washington School of Medicine, Chief of Service, Obstetrics and Gynecology, Harborview Medical Center, Seattle, WA
        • ELIZA L. SUTTON, MD, FACPAssociate Professor, Division of General Internal Medicine, Department of Medicine, Medical Director, Women’s Health Care Center, University of Washington Medical Center, Seattle, WA

        The female reproductive system matures in a continuous, natural process from menarche to menopause as the finite numbers of oocytes produced during fetal development are gradually lost to ovulation and senescence. Menopause is defined as the permanent cessation of menses; by convention, the diagnosis of menopause is not made until the individual has had 12 months of amenorrhea. Menopause is thus characterized by the menstrual changes that reflect oocyte depletion and subsequent changes in ovarian hormone production. However, hormonal changes, rather than the cessation of menstruation itself, cause the manifestations that occur around the time of menopause. Therefore, a woman who has undergone a hysterectomy but who retains her ovaries can experience normal menopausal symptoms as oocyte depletion leads to changes in estrogen levels, even though cessation of menstruation occurred with surgery. This review covers definitions, natural menopause, menopausal transition and postmenopausal symptom management, and premature ovarian insufficiency. Figures show stages of reproductive aging, serum concentrations of hormones during menopausal transition and postmenopause, hormonal changes associated with reproductive aging, symptoms of menopausal transition and menopause, treatment algorithm(s), and Women’s Health Initiative findings: risks and benefits of estrogen alone and estrogen plus progestin by age group: 50 to 59, 60 to 69, and 70 to 79 years. Tables list target tissues, physical manifestations, and menopausal symptoms; selective estrogen receptor modulators used in postmenopausal women; differential diagnosis and evaluation of common menopausal symptoms; estrogen doses; progestogen dosing for endometrial protection; nonhormonal pharmaceutical hot flash therapies; and pharmacologic therapy for genitourinary atrophy.

        This review contains 6 highly rendered figures, 7 tables, and 119 references.

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    • Special Topics in Women's Health
      • 1

        Women With Disabilities

        By Lisa I. Iezzoni, MD, MSc
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        Women With Disabilities

        • LISA I. IEZZONI, MD, MSCProfessor of Medicine, Department of Medicine, Harvard Medical School, Director, Mongan Institute for Health Policy, Massachusetts General Hospital, Boston, MA

        Women with disabilities face substantial barriers to obtaining health care services. They are more likely than nondisabled women to smoke cigarettes, to be obese or overweight, to be physically inactive, and to report depressive symptoms, anxiety, and fears. Women with disabilities are often stigmatized as being asexual and experience higher rates of domestic partner and in-home abuse. Physical, communication, and attitudinal barriers within the health care system may prevent women with disabilities from receiving the care they need. This module discusses the definition of disability, prevalence and demographics of disability among US women, health conditions and risk factors, and general considerations in caring for women with disabilities. Selected health issues in caring for women with disabilities, including reproductive and parental rights, menstruation and fertility, contraception, preconception counseling, and menopause, are also discussed. Tables review components of the International Classification of Functioning, Health and Disability, examples of disability definitions, disability indicators using National Health Interview Survey data, health conditions causing chronic mobility disability for women ages 18 to 49, comorbid health conditions reported among women with and without disability, mental health conditions reported by women with and without disability, examples of women’s experiences with inaccessible medical equipment, examples of communication accommodations, and accessible labeling of prescription drug containers. Figures include the International Classification of Functioning, Health and Disability model of disability and graphs showing the percentage of women with basic action difficulties (BADs); with one or more BADs; with complex action limitations; with BADs by racial and ethnic groups; with indicated level of education and income among women with and without BADs; with indicated level of self-reported overall health among women with BADs; by smoking status among women with and without BADs; in body mass index category among women with and without BADs; with indicated physical activity level among women with and without BADs; with indicated heath care service use and lacking health insurance among women with and without BADs; and receiving Pap smears, mammography screening, and colon cancer screening among women with and without BADs.

        This review contains 12 highly rendered figures, 9 tables, and 86 references.

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      • 2

        Female Sexuality: Assessing Satisfaction and Addressing Problems

        By Jennifer Potter, MD
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        Female Sexuality: Assessing Satisfaction and Addressing Problems

        • JENNIFER POTTER, MDAssociate Professor of Medicine, Department of Medicine, Harvard Medical School, Director, Women’s Health Center, Beth Israel Deaconess Medical Center, Boston, MA

        Sexuality is important to women of all ages. Although changes in sexual function occur with aging, hormonal transitions, illness, the use of medications, and disability, many women can maintain a satisfying sex life by making appropriate adaptations. Clinicians who take the time to obtain a complete and careful sexual history and perform a pertinent physical examination can help the majority of women who present with sexual complaints. Effective treatment must address the contribution of psychological, relationship, and biologic factors and often requires the collaboration of physicians and psychotherapists, as well as sex and physical therapists in many circumstances. Simply initiating a discussion about sexual concerns is frequently the most valuable aspect of treatment for women and their partners. Also useful are provision of basic education about normal female genital anatomy and sexual function across the lifespan; permission to explore masturbation, erotica, and versatile sexual techniques, as well as nongenital pleasuring; information about lubricants; and the prescription of estrogen in the setting of vulvovaginal atrophy. There are as yet no approved agents to treat the biologic component of hypoactive sexual desire. However, it may be appropriate to consider using androgen supplementation in patients with surgical menopause, as well as the addition of bupropion in patients taking selective serotonin reuptake inhibitors (SSRIs). This chapter discusses the epidemiology of female sexual disorders, the female sexual response and sexual behavior, and the diagnosis and management of specific sexual disorders, including desire, arousal, orgasm, and sexual-pain problems. Figures illustrate the biopsychosocial model of sexuality, models of human sexual response, and the neurobiology of female sexual response. Tables list medications with sexual side effects, classification of female sexual dysfunction, helpful questions to ask for the sexual history, and the genital examination in women with sexual difficulties. A sidebar lists selected resources for female sexuality. This chapter contains 132 references.

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      • 3

        Intimate-partner Violence

        By Jane S. Sillman, MD
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        Intimate-partner Violence

        • JANE S. SILLMAN, MDAssistant Professor of Medicine, Department of Medicine, Harvard Medical School, Senior Physician, Brigham and Women’s Hospital, Boston, MA

        Intimate-partner violence describes relationships characterized by intentional controlling or violent behavior by someone who is in an intimate relationship with the victim. The abuser’s controlling behavior may take many forms, including psychological abuse, physical abuse, sexual abuse, economic control, and social isolation. Abuse may ultimately lead to the death of the victim from homicide or suicide. Typically, an abusive relationship goes through cycles of violence. There are periods of calm, followed by increasing tension in the abuser, outbursts of violence, and return to periods of calm. These cycles often spiral toward increasing violence over time. The victims of intimate-partner violence are usually women, but intimate-partner violence is also a significant problem for gay couples and for the disabled and elderly of both sexes. This chapter discusses the epidemiology, diagnosis, treatment, outcomes, and prevention of intimate-partner violence. Risk factors for experiencing violence, risk factors for perpetrating violence, and consequences of abuse are also analyzed. Figures depict the prevalence of intimate-partner violence in the United States, types of abuse experienced as found in a 2004 study, intimate-partner violence among the elderly, an abuse assessment screen for women with disabilities, and a comparison of batterer intervention programs. Tables list the SAFE screen, Abuse Assessment Screen, American Medical Association screening questions for elder abuse, and the Massachusetts Medical Society’s RADAR.
        This chapter contains 5 highly rendered figures, 4 tables, 42 references, 1 teaching slide set, and 5 MCQs.

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      • 4

        Approach to the Patient With a Breast Mass

        By George Plitas, MD; Monica Morrow, MD, FACS
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        Approach to the Patient With a Breast Mass

        • GEORGE PLITAS, MDAssistant Attending, Breast Service, Memorial Sloan-Kettering Cancer Center, New York, NY, Assistant Professor of Surgery, Weill Cornell Medical College, New York, NY
        • MONICA MORROW, MD, FACSChief, Breast Service, Department of Surgery, Memorial Sloan-Kettering Cancer Center, Anne Burnett Windfohr Chair of Clinical Oncology, Professor of Surgery, Weill Cornell Medical College, New York, NY

        A breast mass is the most common presenting symptom among patients in a breast clinic. The presence of a breast mass can cause a great deal of anxiety in women, as well as their physicians. The differential diagnosis of a palpable breast abnormality is broad, although the majority of breast masses are benign. The responsibility of the physician who is evaluating a breast mass is to exclude the presence of malignancy. Once cancer is ruled out, the physician should then attempt to provide an accurate diagnosis, appropriate treatment, and reassurance to the patient. This chapter discusses the assessment of normal breast physiology, identification of a breast mass, evaluation of the various classifications of breast mass (e.g., dominant mass with clinically benign features and dominant mass with suspicious features), differential diagnosis and management of common benign breast masses (e.g., cysts, fibroadenomas, phyllodes tumors, hamartomas, fat necrosis), and the risk of breast cancer associated with benign breast lesions. The chapter also discusses the diagnosis and management of a breast mass in male patients. Tables out