(BQ) Part 1 book Nelson essentials of pediatrics presents the following contents: The profession of pediatrics, growth and development, behavioral disorders, psychiatric disorders, pediatric nutrition and nutritional disorders, fluids and electrolytes, metabolic disorders, fetal and neonatal medicine,... and other contents.
Nelson Essentials of Pediatrics th Edition Karen J Marcdante, MD Professor Department of Pediatrics Medical College of Wisconsin Children’s Hospital of Wisconsin Milwaukee, Wisconsin Robert M Kliegman, MD Professor and Chairman Emeritus Department of Pediatrics Medical College of Wisconsin Children’s Hospital of Wisconsin Milwaukee, Wisconsin 1600 John F Kennedy Blvd Ste 1800 Philadelphia, PA 19103-2899 NELSON ESSENTIALS OF PEDIATRICS, SEVENTH EDITION ISBN: 978-1-4557-5980-4 INTERNATIONAL EDITION ISBN: 978-0-323-22700-1 Copyright © 2015, 2011, 2006, 2002, 1998, 1994, 1990 by Saunders, an imprint of Elsevier Inc All rights reserved No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher Details on how to seek permission, further information about the Publisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/permissions This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may be noted herein) Notices Knowledge and best practice in this field are constantly changing As new research and experience broaden our understanding, changes in research methods, professional practices, or medical treatment may become necessary Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds, or experiments described herein In using such information or methods they should be mindful of their own safety and the safety of others, including parties for whom they have a professional responsibility With respect to any drug or pharmaceutical products identified, readers are advised to check the most current information provided (i) on procedures featured or (ii) by the manufacturer of each product to be administered, to verify the recommended dose or formula, the method and duration of administration, and contraindications It is the responsibility of practitioners, relying on their own experience and knowledge of their patients, to make diagnoses, to determine dosages and the best treatment for each individual patient, and to take all appropriate safety precautions To the fullest extent of the law, neither the Publisher nor the authors, contributors, or editors, assume any liability for any injury and/or damage to persons or property as a matter of products liability, negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the material herein Library of Congress Cataloging-in-Publication Data Nelson essentials of pediatrics / [edited by] Karen J Marcdante, Robert M Kliegman. Seventh edition p ; cm Essentials of pediatrics Includes bibliographical references and index ISBN 978-1-4557-5980-4 (paperback : alk paper) I Marcdante, Karen J., editor of compilation II Kliegman, Robert, editor of compilation III Title: Essentials of pediatrics [DNLM: Pediatrics WS 100] RJ45 618.92 dc23 2013044668 Senior Content Strategist: James Merritt Senior Content Development Specialist: Jennifer Shreiner Publishing Services Manager: Patricia Tannian Project Manager: Amanda Mincher Manager, Art and Design: Steven Stave Printed in the United States of America Last digit is the print number: 9 8 7 6 5 4 3 2 1 This book is dedicated to all of our colleagues (faculty, residents, and medical students) who demonstrate a passion for learning, a curiosity that drives advancement in the care of children, and an amazing dedication to the patients and families we are honored to serve Contributors Lisa M Allen, MD Associate Professor Department of Obstetrics and Gynecology University of Toronto The Hospital for Sick Children Mount Sinai Hospital Toronto, Ontario Canada Adolescent Medicine Warren P Bishop, MD Professor Department of Pediatrics University of Iowa Carver College of Medicine Director, Division of Gastroenterology University of Iowa Children’s Hospital Iowa City, Iowa The Digestive System Kim Blake, MD, MRCP, FRCPC Professor of General Pediatrics IWK Health Centre Division of Medical Education Dalhousie University Halifax, Nova Scotia Canada Adolescent Medicine Nathan J Blum, MD Professor Department of Pediatrics The Perelman School of Medicine at the University of Pennsylvania Director, Leadership Education in Neurodevelopmental Disabilities Program Director, Developmental-Behavioral Pediatrics Fellowship Program Division of Child Development and Metabolic Disease The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania Psychosocial Issues Raed Bou-Matar, MD Associate Staff Center for Pediatric Nephrology Cleveland Clinic Foundation Cleveland, Ohio Fluids and Electrolytes Scott J Brown, MD Developmental-Behavioral Pediatric Fellow Department of Pediatrics University of California, San Diego La Jolla, California Behavioral Disorders April O Buchanan, MD Associate Professor Department of Pediatrics Academic Director, Years and University of South Carolina School of Medicine Greenville Pediatric Hospitalist Children’s Hospital, Greenville Health System Greenville, South Carolina Pediatric Nutrition and Nutritional Disorders Asriani M Chiu, MD Associate Professor of Pediatrics Division of Pediatric Allergy and Immunology Director, Asthma and Allergy Director, Allergy and Immunology Fellowship Program Medical College of Wisconsin Milwaukee, Wisconsin Allergy Yvonne E Chiu, MD Assistant Professor Department of Dermatology Medical College of Wisconsin Milwaukee, Wisconsin Dermatology Cindy W Christian, MD Professor Department of Pediatrics The Perelman School of Medicine at the University of Pennsylvania Director, Safe Place The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania Psychosocial Issues David Dimmock, MD Assistant Professor Department of Pediatrics Division of Pediatric Genetics Medical College of Wisconsin Milwaukee, Wisconsin Metabolic Disorders ix x Contributors Dawn R Ebach, MD Clinical Associate Professor Department of Pediatrics University of Iowa Carver College of Medicine Iowa City, Iowa The Digestive System Sheila Gahagan, MD, MPH Professor and Chief Academic General Pediatrics, Child Development and C ommunity Health Martin Stein Endowed Chair, Developmental-Behavioral Pediatrics University of California, San Diego La Jolla, California Behavioral Disorders Clarence W Gowen, Jr., MD, FAAP Associate Professor and Interim Chair Department of Pediatrics Eastern Virginia Medical School Interim Senior Vice President for Academic Affairs Director of Medical Education Director of Pediatric Residency Program Children’s Hospital of The King’s Daughters Norfolk, Virginia Fetal and Neonatal Medicine Larry A Greenbaum, MD, PhD Marcus Professor of Pediatrics Director, Division of Pediatric Nephrology Emory University School of Medicine Chief, Pediatric Nephrology Emory-Children’s Center Atlanta, Georgia Fluids and Electrolytes Hilary M Haftel, MD, MHPE Clinical Associate Professor Departments of Pediatrics and Communicable Diseases and Internal Medicine Director of Pediatric Education Pediatric Residency Director University of Michigan Medical School Ann Arbor, Michigan Rheumatic Diseases of Childhood MaryKathleen Heneghan, MD Attending Physician Division of Pediatric Endocrinology Advocate Lutheran General Children’s Hospital Park Ridge, Illinois Endocrinology Matthew P Kronman, MD, MSCE Assistant Professor of Pediatrics University of Washington School of Medicine Division of Pediatric Infectious Diseases Seattle Children’s Hospital Seattle, Washington Infectious Diseases K Jane Lee, MD Assistant Professor of Pediatrics, Bioethics, and Medical Humanities Program Director, Pediatric Critical Care Fellowship Medical College of Wisconsin Institute for Health and Society Milwaukee, Wisconsin The Acutely Ill or Injured Child David A Levine, MD Professor Department of Pediatrics Chief, Division of Pre-doctoral Education Morehouse School of Medicine Atlanta, Georgia Growth and Development Paul A Levy, MD, FACMG Assistant Professor Departments of Pediatrics and Pathology Albert Einstein College of Medicine of Yeshiva University Attending Geneticist Children’s Hospital at Montefiore Bronx, New York Human Genetics and Dysmorphology Yi Hui Liu, MD, MPH Assistant Professor Department of Pediatrics University of California, San Diego La Jolla, California Behavioral Disorders John D Mahan, MD Professor, Department of Pediatrics Program Director, Pediatric Residency Program Program Director, Pediatric Nephrology Fellowship Program Vice-Chair for Education The Ohio State University College of Medicine Nationwide Children’s Hospital Columbus, Ohio Nephrology and Urology Robert W Marion, MD Professor Department of Pediatrics Department of Obstetrics and Gynecology and Women’s Health Ruth L Gottesman Chair in Developmental Pediatrics Chief, Section of Child Development Chief, Section of Genetics Department of Pediatrics Albert Einstein College of Medicine of Yeshiva University Bronx, New York Human Genetics and Dysmorphology Maria L Marquez, MD Associate Professor Department of Pediatrics Georgetown University School of Medicine Director, Medical Student Education Georgetown University Hospital Washington, DC Pediatric Nutrition and Nutritional Disorders Contributors xi Susan G Marshall, MD Professor Department of Pediatrics University of Washington School of Medicine Attending Physician Pulmonary Division Seattle Children’s Hospital Seattle, Washington The Respiratory System Thomas W McLean, MD Associate Professor Department of Pediatrics Wake Forest University Baptist Medical Center Winston-Salem, North Carolina Oncology Thida Ong, MD Assistant Professor Department of Pediatrics University of Washington School of Medicine Attending Physician Pulmonary Division Seattle Children’s Hospital Seattle, Washington The Respiratory System Julie A Panepinto, MD, MSPH Professor Department of Pediatrics Medical College of Wisconsin Division of Pediatric Hematology The Children’s Research Institute of the Children’s Hospital of Wisconsin Milwaukee, Wisconsin Hematology Hiren P Patel, MD Clinical Associate Professor Department of Pediatrics The Ohio State University College of Medicine Chief, Section of Nephrology Medical Director, Renal Dialysis Unit Nationwide Children’s Hospital Columbus, Ohio Nephrology and Urology Rowena C Punzalan, MD Assistant Professor Department of Pediatrics Medical College of Wisconsin Division of Pediatric Hematology The Children’s Research Institute of the Children’s Hospital of Wisconsin Milwaukee, Wisconsin Hematology Russell Scheffer, MD Chair and Professor Department of Psychiatry and Behavioral Sciences Professor Department of Pediatrics University of Kansas School of Medicine–Wichita Wichita, Kansas Psychiatric Disorders Jocelyn Huang Schiller, MD Clinical Assistant Professor Department of Pediatrics University of Michigan Medical School Division of Pediatric Neurology C.S Mott Children’s Hospital Ann Arbor, Michigan Neurology Daniel S Schneider, MD Associate Professor Department of Pediatrics University of Virginia School of Medicine Charlottesville, Virginia The Cardiovascular System J Paul Scott, MD Professor Department of Pediatrics Medical College of Wisconsin Medical Director, Wisconsin Sickle Cell Center The Children’s Research Institute of the Children’s Hospital of Wisconsin Milwaukee, Wisconsin Hematology Renée A Shellhaas, MD, MS Clinical Assistant Professor Department of Pediatrics University of Michigan Medical School Division of Pediatric Neurology C.S Mott Children’s Hospital Ann Arbor, Michigan Neurology Benjamin S Siegel, MD Director, Medical Student Education in Pediatrics Professor Department of Pediatrics Boston University School of Medicine Boston, Massachusetts The Profession of Pediatrics Paola A Palma Sisto, MD Associate Professor Department of Pediatrics University of Connecticut School of Medicine Director, Endocrinology Program Division of Pediatric Endocrinology Connecticut Children’s Medical Center Hartford, Connecticut Endocrinology Sherilyn Smith, MD Professor of Pediatrics Fellowship Director, Pediatric Infectious Disease University of Washington School of Medicine Associate Clerkship Director Seattle Children’s Hospital Seattle, Washington Infectious Diseases xii Contributors Amanda Striegl, MD, MS Assistant Professor Department of Pediatrics University of Washington School of Medicine Attending Physician Pulmonary Division Seattle Children’s Hospital Seattle, Washington James W Verbsky, MD, PhD Assistant Professor Department of Pediatrics Department of Microbiology and Molecular Genetics Division of Pediatric Rheumatology Medical College of Wisconsin Children’s Hospital of Wisconsin Milwaukee, Wisconsin J Channing Tassone, MD Associate Professor Departments of Orthopedic Surgery and Pediatrics Medical College of Wisconsin Division of Pediatric Orthopedic Surgery Children’s Hospital of Wisconsin Milwaukee, Wisconsin Kevin D Walter, MD, FAAP Assistant Professor Departments of Orthopedic Surgery and Pediatrics Medical College of Wisconsin Program Director, Primary Care Sports Medicine Children’s Hospital of Wisconsin Milwaukee, Wisconsin Aveekshit Tripathi, MD Senior Psychiatry Resident Department of Psychiatry and Behavioral Sciences University of Kansas School of Medicine–Wichita Wichita, Kansas Marcia M Wofford, MD Associate Professor Department of Pediatrics Wake Forest University Baptist Medical Center Winston-Salem, North Carolina The Respiratory System Orthopedics Psychiatric Disorders Immunology Orthopedics Oncology Preface Medicine and technology just don’t stop! The amazing advancements we hear about as our scientist colleagues further delineate the pathophysiology and mechanisms of diseases must eventually be translated to our daily care of patients Our goal, as the editors and authors of this textbook, is not only to provide the classic, foundational knowledge we use every day but to include these advances in a readable and concise text for medical students and residents This new edition has been updated with the advances that have occurred since the last edition We have also incorporated technology by linking this book to the second edition of Pediatric Decision Making Strategies by Pomeranz, Busey, Sabnis, and Kliegman This will allow you to read about the medical issues and then follow a link to an algorithm to facilitate efficient and effective evaluations We believe this integration will help you investigate the common and classic pediatric disorders in a time-honored, logical format to both acquire knowledge and apply knowledge to your patients We have also once again asked our colleagues who serve as clerkship directors to write many of the sections so that you can gain the knowledge and skills necessary to succeed both in caring for patients and in preparing for clerkship or in-service examinations We are honored to be part of the journey of thousands of learners who rotate through pediatrics as well as those who will become new providers of pediatric care in the years to come Karen J Marcdante, MD Robert M Kliegman, MD xiii Acknowledgments The editors could never have published this edition without the assistance and attention to detail of James Merritt and Jennifer Shreiner We also couldn’t have accomplished this without Carolyn Redman, whose prompting, organizing, and overseeing of the process helped us create this new edition xv CHARACTERISTIC JUVENILE IDIOPATHIC ARTHRITIS SYSTEMIC LUPUS ERYTHEMATOSUS RHEUMATIC FEVER LYME DISEASE LEUKEMIA KAWASAKI DISEASE GONOCOCCEMIA Sex F>M Type-dependent M=F M=F M=F F>M M=F Age 10–20 yr 1–16 yr 5–15 yr >5–20 yr 2–10 yr >12 yr 99%) Positive (50%) Negative Negative Negative Negative Negative Rheumatoid factor Positive or negative Positive (10%) (polyarticular) Negative Negative Negative Negative Negative Other laboratory results ↓ Complement, ↑antibodies to doublestranded DNA Anti-CCP antibody + in adult type RA ↑ASO anti-DNase B ↑Cryoglobulin, ↑immune complexes + Bone marrow + Culture for Neisseria gonorrhoeae Thrombocytosis, ↑immune complexes Erosive arthritis Rare Yes Rare Rare No Yes No Other clinical manifestations Proteinuria, serositis Fever, serositis (systemic onset) Carditis, nodules, chorea Carditis, neuropathy, meningitis Thrombocytopenia Sexual activity, menses Fever, lymphadenopathy, swollen hands/feet, mouth lesions Pathogenesis Autoimmune Autoimmune Group A streptococcus Borrelia burgdorferi Acute lymphoblastic leukemia N gonorrhoeae Unknown Treatment NSAIDs, corticosteroids, hydroxychloroquine, immunosuppressive agents NSAIDs, methotrexate, TNF blockers for resistant disease Penicillin prophylaxis, aspirin, corticosteroids Penicillin, doxycycline, ceftriaxone Corticosteroids, chemotherapy Ceftriaxone Intravenous immunoglobulin, aspirin pauciarticular ANA, Antinuclear antibody; ASO, antistreptolysin-O titer; CCP, cyclic citrullinated protein; NSAID, nonsteroidal anti-inflammatory drug; TNF, tumor necrosis factor; WBC, white blood cell 300 Section 15 u Rheumatic Diseases of Childhood Table 86-1 Differential Diagnosis of Pediatric Arthritis Syndromes Chapter 87 u Henoch-Schönlein Purpura 301 Table 86-2 Manifestations of Autoantibodies Coombs-positive hemolytic anemia Immune neutropenia Immune thrombocytopenia Thrombosis (anticardiolipin, antiphospholipid, lupus anticoagulant) Immune lymphopenia Antimitochondrial (primary biliary cirrhosis, SLE) Antimicrosomal (chronic active hepatitis, SLE) Antithyroid (thyroiditis, SLE) Antineutrophil cytoplasmic antibody (ANCA-cytoplasmic) (granulomatosis with polyangiitis) ANCA-perinuclear (microscopic polyangiitis) Anti-CCP (rheumatoid positive JIA) ANTINUCLEAR ANTIBODIES TO SPECIFIC NUCLEAR ANTIGENS AND ASSOCIATED MANIFESTATIONS Most rheumatologic diagnoses are established by clinical findings and fulfillment of classification criteria Laboratory testing should be judicious and based on a differential diagnosis rather than random screening in search of a diagnosis Laboratory tests confirm clinical diagnoses rather than develop them LABORATORY TESTING Evidence of an underlying systemic inflammation may be indicated by elevated acute phase reactants, especially the erythrocyte sedimentation rate, but also the white blood cell count, platelet count, and C-reactive protein The complete blood count may demonstrate a normochromic, normocytic anemia of chronic disease These laboratory findings are nonspecific for any particular rheumatologic diagnosis Certain laboratory tests may help confirm a diagnosis, such as autoantibody production in SLE or muscle enzyme elevation in JDM, or identify increased risk for complications, such as uveitis in a patient with JIA with a positive antinuclear antibody Single-stranded DNA (nonspecific, indicates inflammation) Double-stranded DNA (SLE, renal disease) DNA-histone (drug-induced SLE) Sm (Smith) (SLE, renal, CNS) RNP (ribonucleoprotein) (SLE, Sjögren syndrome, scleroderma, polymyositis, MCTD) Ro (Robert: SSA) (SLE, neonatal lupus-congenital heart block, Sjögren syndrome) La (Lane: SSB) (SLE, neonatal lupus [congenital heart block], Sjögren syndrome) Jo-1 (polymyositis, dermatomyositis) DIAGNOSTIC IMAGING Radiologic studies should focus on areas of concern identified by history or physical examination Radiography of joints in patients with arthritis on examination may be beneficial, but radiographic abnormalities may lag far behind the clinical examination Tests with greater sensitivity, such as bone scan, computed tomography scan, and magnetic resonance imaging, may be useful when trying to differentiate between synovitis and traumatic soft tissue injury Magnetic resonance imaging may also be useful to identify evidence of central nervous system involvement with SLE or for evidence of myositis with JDM Scl-70 (systemic sclerosis) Centromere (CREST; limited scleroderma) PM-Scl (scleroderma, UCTD) Adapted from Condemi J: The autoimmune disease, JAMA 268:2882–2892, 1992 ANCA, Antineutrophil cytoplasmic antibody; CCP, cyclic citrullinated protein; CNS, central nervous system; CREST syndrome, calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia; MCTD, mixed connective tissue disease; SLE, systemic lupus erythematosus; SSA, Sjögren syndrome antigen A; SSB, Sjögren syndrome antigen B; UCTD, undifferentiated connective tissue disease cytokines, and circulating antibodies This antibody production can be nonspecific, or it can be targeted against specific native proteins, leading to subsequent disease manifestations (Table 86-2) Although immune system hyperactivity may be self-limited, the hallmark of most rheumatic diseases of childhood is chronicity, or the perpetuation of the inflammatory process, which can lead to long-term disability INITIAL DIAGNOSTIC EVALUATION Although rheumatic diseases sometimes present with nonspecific symptoms, especially early in the course, over time a characteristic set of symptoms and physical findings can be elicited In conjunction with carefully chosen confirmatory laboratory tests, an appropriate differential diagnosis is made, and eventually the correct diagnosis and treatment plan is developed Chapter 87 HENOCH-SCHƯNLEIN PURPURA ETIOLOGY Henoch-Schưnlein purpura (HSP) is a vasculitis of unknown etiology characterized by inflammation of small blood vessels with leukocytic infiltration of tissue, hemorrhage, and ischemia The immune complexes associated with HSP are predominantly composed of IgA EPIDEMIOLOGY HSP is the most common systemic vasculitis of childhood and cause of nonthrombocytopenic purpura, with an incidence of 13 per 100,000 children It occurs primarily in children to 15 years of age, although it has been described in adults HSP is slightly more common in boys than girls and occurs more frequently in the winter than in the summer months 302 Section 15 u Rheumatic Diseases of Childhood CLINICAL MANIFESTATIONS Decision-Making Algorithms Available @ StudentConsult.com Red Urine and Hematuria Proteinuria Scrotal Pain Fever and Rash Petechiae/Purpura HSP is characterized by rash, arthritis, and, less frequently, gastrointestinal or renal vasculitis The hallmark of HSP is palpable purpura, caused by small vessel inflammation in the skin leading to extravasation of blood into the surrounding tissues, frequently with IgA deposition The rash is classically found in dependent areas: below the waist, on the buttocks, and lower extremities (Fig 87-1) The rash can begin as small macules or urticarial lesions but rapidly progresses to purpura with areas of ecchymosis The rash also can be accompanied by edema, particularly of the calves and dorsum of the feet, scalp, and scrotum or labia HSP occasionally is associated with encephalopathy, pancreatitis, and orchitis Arthritis occurs in 80% of patients with HSP and is most common in the lower extremities, particularly the ankles and knees The arthritis is acute and very painful with refusal to bear weight Joint swelling can be confused with peripheral edema seen with the rash of HSP Gastrointestinal involvement occurs in about one half of affected children and most typically presents as mild to moderate crampy abdominal pain, thought to be due to small vessel involvement of the gastrointestinal tract leading to ischemia Less commonly, significant abdominal distention, bloody diarrhea, intussusception, or abdominal perforation occurs and requires emergent intervention Gastrointestinal involvement is typically seen during the acute phase of the illness It may precede the onset of rash One third of children with HSP develop renal involvement, which can be acute or chronic Although renal involvement is mild in most cases, acute glomerulonephritis manifested by hematuria, hypertension, or acute renal failure can occur Most cases of glomerulonephritis occur within the first few months of presentation, but rarely patients develop late renal disease, which ultimately can lead to chronic renal disease, including renal failure LABORATORY AND IMAGING STUDIES Erythrocyte sedimentation rate, C-reactive protein, and white blood cell count are elevated in patients with HSP The platelet count is the most important test, because HSP is characterized by nonthrombocytopenic purpura with a normal, or even high, platelet count, differentiating HSP from other causes of purpura that are associated with thrombocytopenia such as autoimmune thrombocytopenia, systemic lupus erythematosus, or leukemia A urinalysis screens for evidence of hematuria A serum blood urea nitrogen and creatinine should be obtained to evaluate renal function Testing the stool for blood may identify evidence of gut ischemia Any question of gut perforation requires radiologic investigation DIFFERENTIAL DIAGNOSIS The diagnosis of HSP is based on the presence of two of four criteria (Table 87-1), which provides 87.1% sensitivity and 87.7% specificity for the disease The differential diagnosis includes other systemic vasculitides (Table 87-2) and diseases associated with thrombocytopenic purpura, such as idiopathic thrombocytopenic purpura and leukemia TREATMENT Therapy for HSP is supportive A short-term course of nonsteroidal anti-inflammatory drugs can be administered for the acute arthritis Systemic corticosteroids usually are reserved for children with gastrointestinal disease and provide significant relief of abdominal pain A typical dosing regimen is prednisone, mg/kg/day for to weeks, followed by a taper schedule Recurrence of abdominal pain as corticosteroids are weaned may necessitate a longer course of treatment Acute nephritis typically is treated with corticosteroids but may require more aggressive immunosuppressive therapy COMPLICATIONS Most cases of HSP are monophasic, lasting to weeks and resolving completely The rash can wax and wane, however, for year after HSP Parents should be warned regarding possible recurrences The arthritis of HSP does not leave any permanent joint damage; it does not typically recur Gastrointestinal involvement can lead to temporary abnormal peristalsis that poses a risk of intussusception, which may be followed by complete obstruction or infarction with bowel perforation Any child Table 87-1 Criteria for Diagnosis of Henoch-Schönlein Purpura* CRITERIA Figure 87-1 Rash of Henoch-Schönlein purpura on the lower extremities of a child Note evidence of both purpura and petechiae DEFINITION Palpable purpura Raised, palpable hemorrhagic skin lesions in the absence of thrombocytopenia Bowel angina Diffuse abdominal pain or the diagnosis of bowel ischemia Diagnostic biopsy Histologic changes showing granulocytes in the walls of arterioles or venules; IgA deposits in vessel wall Pediatric age group Age 40 °C) without an apparent source The onset of fever is followed by conjunctival erythema; mucosal changes, including dry, cracked lips and a strawberry tongue; cervical lymphadenopathy; and swelling of the hands and feet (Fig 88-1) Conjunctivitis is bilateral, bulbar, and nonsuppurative Cervical lymphadenopathy is found in 70% of children and should be greater than 1.5 cm in diameter for the purposes of diagnosis A rash, which can vary in appearance, occurs in 80% of children with KD and may be particularly accentuated in the inguinal area and on the chest Extreme irritability is prominent, especially in infants Abdominal pain and hydrops of the gallbladder, cerebrospinal fluid pleocytosis, sterile pyuria, and arthritis, particularly of medium-sized to large joints, may occur Carditis in the acute phase may be manifested by tachycardia, shortness of breath, or overt congestive heart failure Giant coronary artery aneurysms, which are rare but occur most commonly in very young children, can appear during this phase Subacute Phase The subacute phase, which lasts until about the fourth week, is characterized by gradual resolution of fever (if untreated) and other symptoms Desquamation of the skin, particularly of the fingers and toes, appears at this point The platelet count, previously normal or slightly depressed, increases to a significant degree (often >1 million/mm3) This phase heralds the onset of coronary artery aneurysms, which may also appear in the convalescent phase and pose the highest risk of morbidity and mortality Risk factors for development of coronary artery aneurysms include prolonged fever, prolonged elevation of inflammatory parameters such as the erythrocyte 304 Section 15 u Rheumatic Diseases of Childhood Figure 88-1 Facial features of Kawa- saki disease with (A) morbilliform rash and nonsuppurative conjunctivitis and (B) red, chapped lips A sedimentation rate (ESR), age younger than year or older than years, and male gender Convalescent Phase The convalescent phase begins with the disappearance of clinical symptoms and continues until the ESR returns to normal, usually to weeks after the onset of illness Beau lines of the fingernails may appear during this phase LABORATORY AND IMAGING STUDIES It is particularly important to exclude other causes of fever, notably infection It is appropriate to obtain blood and urine cultures and to perform a chest x-ray In the acute phase, inflammatory parameters are elevated, including white blood cell count, C-reactive protein, and the ESR, which can be profoundly elevated (often >80 mm/hr) Platelet counts may be inappropriately low or normal A lumbar puncture, if performed to exclude infection, may reveal pleocytosis Tests of hepatobiliary function may be abnormal Greatly elevated platelet counts develop during the subacute phase The development of coronary artery aneurysms is monitored by performing two-dimensional echocardiograms, usually during the acute phase, at to weeks, and again at to weeks More frequent echocardiograms and, potentially, coronary angiography are indicated for patients who develop coronary artery abnormalities B Table 88-1 Criteria for Diagnosis of Kawasaki Disease Fever of >5 days’ duration associated with at least four* of the following five changes: Bilateral nonsuppurative conjunctivitis One or more changes of the mucous membranes of the upper respiratory tract, including pharyngeal injection, dry fissured lips, injected lips, and “strawberry” tongue One or more changes of the extremities, including peripheral erythema, peripheral edema, periungual desquamation, and generalized desquamation Polymorphous rash, primarily truncal Cervical lymphadenopathy >1.5 cm in diameter Disease cannot be explained by some other known disease process *A diagnosis of Kawasaki disease can be made if fever and only three changes are present in conjunction with coronary artery disease documented by twodimensional echocardiography or coronary angiography Table 88-2 Differential Diagnosis of Kawasaki Disease INFECTIOUS Scarlet fever Epstein-Barr virus Adenovirus Meningococcemia DIFFERENTIAL DIAGNOSIS The diagnosis of KD is based on the presence of fever for more than days without an identifiable source and the presence of four of five other clinical criteria (Table 88-1) The diagnosis of incomplete (atypical) KD, which occurs more commonly in infants, is made when fever is present for at least days even if only two or three clinical criteria are present, particularly in the presence of coronary artery aneurysms The diagnosis of KD should be considered in infants younger than months of age with fever for at least days even if no other criteria are present Because many of the manifestations of KD are found in other illnesses, many diagnoses must be considered and excluded before the diagnosis of KD can be established (Table 88-2) TREATMENT Intravenous immunoglobulin (IVIG) is the mainstay of therapy for KD, although the mechanism of action is unknown Measles Rubella Roseola infantum Staphylococcal toxic shock syndrome Scalded skin syndrome Toxoplasmosis Leptospirosis Rocky Mountain spotted fever INFLAMMATORY Juvenile idiopathic arthritis (systemic onset) Polyarteritis nodosa Behỗet syndrome HYPERSENSITIVITY Drug reaction Stevens-Johnson syndrome (erythema multiforme) Chapter 89 u Juvenile Idiopathic Arthritis 305 Table 88-3 Complications of Kawasaki Disease Coronary artery thrombosis Peripheral artery aneurysm Coronary artery aneurysms Myocardial infarction Myopericarditis Chapter 89 JUVENILE IDIOPATHIC ARTHRITIS Heart failure Hydrops of gallbladder Aseptic meningitis Irritability Arthritis Sterile pyuria (urethritis) Thrombocytosis (late) Diarrhea Pancreatitis Peripheral gangrene A single dose of IVIG (2 g/kg over 12 hours) results in rapid defervescence and resolution of clinical illness in most patients and, more important, reduces the incidence of coronary artery aneurysms Aspirin is initially given in anti- inflammatory doses (80 to 100 mg/kg/day divided every hours) in the acute phase Once the fever resolves, aspirin is reduced to antithrombotic doses (3 to mg/kg/day as a single dose) and given through the subacute and convalescent phases, usually for to weeks, until follow-up echocardiography documents the absence or resolution of coronary artery aneurysms Up to 10% of children with KD initially fail to respond satisfactorily to IVIG therapy Most of these patients respond to retreatment with IVIG, but an alternative preparation of IVIG may be required Corticosteroids or infliximab are rarely used in KD, as opposed to other vasculitides, but may have a role during the acute phase if active carditis is apparent or for children with persistent fever after two doses of IVIG COMPLICATIONS Most cases resolve without sequelae Myocardial infarction has been documented, most likely caused by stenosis of a coronary artery at the site of an aneurysm Coronary artery aneurysms found on autopsy in older children following sudden cardiac death may have been due to past KD Other complications are listed in Table 88-3 PROGNOSIS IVIG reduces the prevalence of coronary artery disease from 20% to 25% in children treated with aspirin alone to 2% to 4% in children treated with IVIG and aspirin Other than the risk of persistent coronary artery aneurysms, KD has an excellent prognosis ETIOLOGY The chronic arthritides of childhood include several types, the most common of which is juvenile idiopathic arthritis (JIA), formerly called juvenile rheumatoid arthritis (JRA) The classification of JIA includes several other types of juvenile arthritis, such as enthesitis-related arthritis and psoriatic arthritis The etiology of this autoimmune disease is unknown The common underlying manifestation of this group of illnesses is the presence of chronic synovitis, or inflammation of the joint synovium The synovium becomes thickened and hypervascular with infiltration by lymphocytes, which also can be found in the synovial fluid along with inflammatory cytokines The inflammation leads to production and release of tissue proteases and collagenases If left untreated, the inflammation can lead to tissue destruction, particularly of the articular cartilage and, eventually, the underlying bony structures EPIDEMIOLOGY JIA is the most common chronic rheumatologic disease of childhood, with a prevalence of 1:1000 children The disease has two peaks, one at to years and one at to 12 years, but it can occur at any age Girls are affected more commonly than boys, particularly with the oligoarticular form of the illness CLINICAL PRESENTATION Decision-Making Algorithms Available @ StudentConsult.com Red Eye Limp Arthritis Knee Pain Extremity Pain JIA can be divided into several subtypes, depending on the number of joints involved (less than five versus five or more), the presence of sacroiliac involvement, and the presence of systemic features, each with particular disease characteristics (Table 89-1) Although the onset of the arthritis is slow, the actual joint swelling is often noticed acutely by the child or parent, such as after an accident or fall, and can be confused with trauma (even though traumatic effusions are rare in children) The child may develop pain and stiffness in the joint that limit use, but rarely refuses to use the joint at all Morning stiffness and gelling also can occur in the joint and, if present, can be followed in response to therapy On physical examination, signs of inflammation are present, including joint tenderness, erythema, and effusion (Fig 89-1) Joint range of motion may be limited because of pain, swelling, or contractures from lack of use In children, because of the 306 Section 15 u Rheumatic Diseases of Childhood Table 89-1 Features of Juvenile Idiopathic Arthritis Subgroups FEATURE OLIGOARTICULAR POLYARTICULAR SYSTEMIC ONSET SPONDYLOARTHROPATHIES No joints M (especially in younger children) F>M F=M M>F Systemic features None Some constitutional Prominent Some constitutional Eye disease +++ (uveitis) ++ (uveitis) + (uveitis) ++ (iritis) Extra-articular manifestations None None Systemic features Enthesopathy, psoriasis, bowel disease ANA positivity ++ + — — Variable, depends on extent of arthritis Variable RF positivity Outcomes + (in older children with early-onset RA) Excellent, >90% complete remission Good, >50% complete remission, some risk of disability ANA, Antinuclear antibody; RA, rheumatoid arthritis; RF, rheumatoid factor increased risk, the subgroup of children, particularly young girls, with oligoarticular (less than five affected joints) JIA and a positive antinuclear antibody are at highest risk, with an incidence of uveitis of 80% The uveitis associated with JIA can be asymptomatic until the point of visual loss, making it a primary treatable cause of blindness in children It is crucial for children with JIA to undergo regular ophthalmologic screening with a slit-lamp examination to identify anterior chamber inflammation and to initiate prompt treatment of any active disease Oligoarticular Juvenile Idiopathic Arthritis Figure 89-1 An affected knee in a patient with oligoarticular juvenile idiopathic arthritis Note sizeable effusion, bony proliferation, and flexion contracture presence of an active growth plate, it may be possible to find bony abnormalities of the surrounding bone, causing bony proliferation and localized growth disturbance In a lower extremity joint, a leg length discrepancy may be appreciable if the arthritis is asymmetric All children with chronic arthritis are at risk for chronic iridocyclitis or uveitis There is an association between human leukocyte antigens (HLAs) (HLA-DR5, HLA-DR6, and HLA-DR8) and uveitis The presence of a positive antinuclear antibody identifies children with arthritis who are at higher risk for chronic uveitis Although all children with JIA are at Oligoarticular JIA is defined as the presence of arthritis in fewer than five joints within months of diagnosis This is the most common form of JIA, accounting for approximately 50% of cases Oligoarticular JIA presents in young children, with a peak at to years and another peak at to 12 years The arthritis is found in medium-sized to large joints; the knee is the most common joint involved, followed by the ankle and the wrist It is unusual for small joints, such as the fingers or toes, to be involved, although this may occur Neck and hip involvement also is uncommon Children with oligoarticular JIA may be otherwise well without any evidence of systemic inflammation (fever, weight loss, or failure to thrive) or any laboratory evidence of systemic inflammation (elevated white blood cell count or erythrocyte sedimentation rate) A subset of these children later develops polyarticular disease (called extended oligoarthritis) Polyarticular Juvenile Idiopathic Arthritis Polyarticular JIA describes children with arthritis in five or more joints within the first months of diagnosis and accounts for about 40% of cases Children with polyarticular JIA tend to have symmetric arthritis, which can affect any joint but typically involves the small joints of the hands, feet, ankles, wrists, and knees The cervical spine can be involved, leading Chapter 89 u Juvenile Idiopathic Arthritis 307 Table 89-2 Comparison of Juvenile Idiopathic Arthritis and Spondyloarthropathies CLINICAL MANIFESTATIONS JIA JAS PSA IBD F M Equal Equal +++ + ++ + Back symptoms – +++ + ++ Family history – ++ ++ + ANA positivity ++ – – – – ++ – Gender predominance Peripheral arthritis HLA-B27 positivity RF positivity + (in late-onset JIA) – Extra-articular manifestations Systemic symptoms (systemic-onset JRA) Enthesopathy Eye disease Anterior uveitis iritis – – Psoriasis, nail changes Bowel symptoms Posterior uveitis Anterior uveitis ANA, Antinuclear antibody; IBD, inflammatory bowel disease; JAS, juvenile ankylosing spondylitis; JIA, juvenile idiopathic arthritis; PSA, poststreptococcal arthritis; RF, rheumatoid factor to fusion of the spine over time In contrast to oligoarticular JIA, children with polyarticular disease can present with evidence of systemic inflammation, including malaise, low-grade fever, growth retardation, anemia of chronic disease, and elevated markers of inflammation Polyarticular JIA can present at any age, although there is a peak in early childhood There is a second peak in adolescence, but these children differ by the presence of a positive rheumatoid factor (and anti-CCP antibody) and most likely represent a subgroup with true adult rheumatoid arthritis; the clinical course and prognosis are similar to the adult entity Systemic-Onset Juvenile Idiopathic Arthritis A small subgroup of patients (approximately 10%) with juvenile arthritis does not present with onset of arthritis but rather with preceding systemic inflammation This form of JIA, thought to be an autoinflammatory disease, manifests with a typical recurring, spiking fever, usually once or twice per day, which can occur for several weeks to months This is accompanied by a rash, typically morbilliform and salmon-colored The rash may be evanescent and occur at times of high fever only Rarely the rash can be urticarial in nature Internal organ involvement also occurs Serositis, such as pleuritis and pericarditis, occurs in 50% of children Pericardial tamponade rarely may occur Hepatosplenomegaly occurs in 70% of children Children with systemic-onset JIA appear sick; they have significant constitutional symptoms, including malaise and failure to thrive Laboratory findings show the inflammation, with elevated erythrocyte sedimentation rate, C-reactive protein, white blood cell count, and platelet counts and anemia The arthritis of JIA follows the systemic inflammation by weeks to months The arthritis is typically polyarticular in nature and can be extensive and resistant to treatment, placing these children at highest risk for long-term disability Spondyloarthropathies The spondyloarthropathies describe a group of arthritides that include inflammation of the axial skeleton and sacroiliac joints and enthesitis, or inflammation of tendinous insertions These include juvenile ankylosing spondylitis, psoriatic arthritis, and the arthritis of inflammatory bowel disease This group of diseases can also present with peripheral arthritis and can be initially classified in other subgroups It is only later, when the patient develops evidence of sacroiliac arthritis, psoriasis, or gastrointestinal disease, that the diagnosis becomes clear (Table 89-2) Other important features of this group include the frequent presence of HLA-B27 and the need for earlier treatment with tumor necrosis factor (TNF) blockers LABORATORY AND IMAGING STUDIES Most children with oligoarticular JIA have no laboratory abnormalities Children with polyarticular and systemic-onset disease commonly show elevated acute phase reactants and anemia of chronic disease In all pediatric patients with joint or bone pain, a complete blood count should be performed to exclude leukemia, which also can present with limb pain (see Chapter 155) All patients with oligoarticular JIA should have an antinuclear antibody test to help identify those at higher risk for uveitis Older children and adolescents with polyarticular disease should have a rheumatoid factor performed to identify children with early onset adult rheumatoid arthritis Diagnostic arthrocentesis may be necessary to exclude suppurative arthritis in children who present with acute onset of monarticular symptoms The synovial fluid white blood cell count is typically less than 50,000 to 100,000/mm3 and should be predominantly lymphocytes, rather than neutrophils seen with suppurative arthritis Gram stain and culture should be negative (see Chapter 118) The most common radiologic finding in the early stages of JIA is a normal bone x-ray Over time, periarticular osteopenia, resulting from decreased mineralization, is most commonly found Growth centers may be slow to develop, whereas there may be accelerated maturation of growth plates or evidence of bony proliferation Erosions of bony articular surfaces may be a late finding If the cervical spine is involved, fusion of C1-4 may occur, and atlantoaxial subluxation may be demonstrable DIFFERENTIAL DIAGNOSIS The diagnosis of JIA is established by the presence of arthritis, the duration of the disease for at least weeks, and exclusion of other possible diagnoses Although a presumptive diagnosis 308 Section 15 u Rheumatic Diseases of Childhood of systemic-onset JIA can be established for a child during the systemic phase, a definitive diagnosis is not possible until arthritis develops Children must be younger than 16 years of age at time of onset of disease; the diagnosis of JIA does not change when the child becomes an adult Because there are so many other causes of arthritis, these disorders need to be excluded before providing a definitive diagnosis of JIA (Table 89-3) The acute arthritides can affect the same joints as JIA but have a shorter time course Table 89-3 Differential Diagnosis of Juvenile Arthritis CONNECTIVE TISSUE DISEASES Juvenile idiopathic arthritis Systemic lupus erythematosus Juvenile dermatomyositis Scleroderma with arthritis INFECTIOUS ARTHRITIS Bacterial arthritis TREATMENT The treatment of JIA focuses on suppressing inflammation, preserving and maximizing function, preventing deformity, and preventing blindness Nonsteroidal anti-inflammatory drugs (NSAIDs) are the first choice in the treatment of JIA Naproxen, sulindac, ibuprofen, indomethacin, and others have been used successfully Systemic corticosteroid medications, such as prednisone and prednisolone, should be avoided in all but the most extreme circumstances, such as for severe systemic-onset JIA with internal organ involvement or for significant active arthritis leading to the inability to ambulate In this circumstance, the corticosteroids are used as bridging therapy until other medications take effect For patients with a few isolated inflamed joints, intra-articular corticosteroids may be helpful Second-line medications, such as hydroxychloroquine and sulfasalazine, have been used in patients whose arthritis is not completely controlled with NSAIDs alone Methotrexate, given either orally or subcutaneously, has become the drug of choice for polyarticular and systemic-onset JIA, which may not respond to baseline agents alone Methotrexate can cause bone marrow suppression and hepatotoxicity; regular monitoring can minimize these risks Leflunomide, with a similar adverse effect profile to methotrexate, has also been used Biologic agents that inhibit TNF-α and block the inflammatory cascade, including etanercept, infliximab, and adalimumab, are effective in the treatment of JIA The risks of these agents are greater, however, and include serious infection and, possibly, increased risk of malignancy Anakinra, an interleukin-1 receptor antagonist, is very beneficial in the treatment of the systemic features of systemic-onset JIA Viral arthritis Fungal arthritis Lyme disease REACTIVE ARTHRITIS Poststreptococcal arthritis Rheumatic fever Toxic synovitis Henoch-Schönlein purpura Reiter syndrome ORTHOPEDIC DISORDERS Traumatic arthritis Legg-Calve-Perthes disease Slipped capital femoral epiphysis Osteochondritis dissecans Chondromalacia patellae MUSCULOSKELETAL PAIN SYNDROMES Growing pains Hypermobility syndromes Myofascial pain syndromes/fibromyalgia Reflex sympathetic dystrophy HEMATOLOGIC/ONCOLOGIC DISORDERS Leukemia Lymphoma Sickle cell disease Thalassemia Malignant and benign tumors of bone, cartilage, or synovium COMPLICATIONS Complications with JIA result primarily from the loss of function of an involved joint secondary to contractures, bony fusion, or loss of joint space Physical and occupational therapies, professionally and through home programs, are crucial to preserve and maximize function More serious complications stem from associated uveitis; if left untreated, it can lead to serious visual loss or blindness PROGNOSIS The prognosis of JIA is excellent, with an overall 85% complete remission rate Children with oligoarticular JIA uniformly tend to well, whereas children with polyarticular disease and systemic-onset disease constitute most children with functional disability Systemic-onset disease, a positive Metastatic bone disease Hemophilia MISCELLANEOUS Rickets/metabolic bone disease Lysosomal storage diseases Heritable disorders of collagen rheumatoid factor, poor response to therapy, and the presence of erosions on x-ray all connote a poorer prognosis The importance of physical and occupational therapy cannot be overstated because when the disease remits, the physical limitations remain with the patient into adulthood Chapter 90 u Systemic Lupus Erythematosus 309 Chapter 90 SYSTEMIC LUPUS ERYTHEMATOSUS Table 90-1 Criteria for Diagnosis of Systemic Lupus Erythematosus* PHYSICAL SIGNS Malar butterfly rash Discoid lupus Photosensitivity Oral and nasopharyngeal ulcers ETIOLOGY Systemic lupus erythematosus (SLE) is a multisystem disorder of unknown etiology characterized by a production of large amounts of circulating autoantibodies This antibody production may be due to loss of T-lymphocyte control on B-lymphocyte activity, leading to hyperactivity of B lymphocytes, which leads to nonspecific and specific antibody and autoantibody production These antibodies form immune complexes that become trapped in the microvasculature, leading to inflammation and ischemia Nonerosive arthritis (more than two joints with effusion and tenderness) Pleuritis or pericarditis (serositis) Seizures or psychosis in absence of metabolic toxins or drugs LABORATORY DATA RENAL DISEASE (NEPHRITIS) Proteinuria (>500 mg/24 h) or Cellular casts (RBC, granular, or tubular) HEMATOLOGIC DISEASE Hemolytic anemia with reticulocytosis or EPIDEMIOLOGY Although SLE affects primarily women of childbearing age, approximately 5% of cases present in childhood, mainly around puberty SLE is rare in children younger than years of age Although there is a female predominance of this disease in adolescence and adulthood, there is an equal gender distribution in children The overall prevalence of SLE in the pediatric population is 10 to 25 cases per 100,000 children Leukopenia (10 degrees hyperextension of knee point each for right and left Touch palms to floor with knees straight point *>6 points defines hypermobility Figure 92-1 Hyperextension of the knees, an example of hypermobility a nighttime dose of acetaminophen or an analgesic dose of a nonsteroidal anti-inflammatory drug (NSAID) Occasionally nocturnal awakening has been of long duration, leading to disruptive behavior patterns In these cases, intervention must be aimed at decreasing the secondary gain associated with nighttime parental attention and should focus on sleep hygiene Other than the negative behavioral patterns that can occur, there are no significant complications Growing pains are not associated with other illnesses and resolve over time BENIGN HYPERMOBILITY Hypermobility syndromes are disorders of unknown etiology that cause musculoskeletal pain secondary to excessive mobility of joints These disorders most commonly present in children to 10 years of age Girls are more commonly affected than boys There is a familial predisposition to hypermobility syndromes Hypermobility can be isolated to a specific joint group or can present as a generalized disorder Symptoms vary depending on the joints involved The most consistent symptom is pain, which may occur during the day or night The discomfort may increase after exertion but rarely interferes with regular physical activity Children with hypermobility of the ankles or feet may complain of chronic leg or back pain Joint hypermobility may be quite marked Range of motion may be exaggerated with excessive flexion or extension at the metacarpophalangeal joints, wrists, elbows, or knees (genu recurvatum) (Fig 92-1) There may be excessive pronation of the ankles Hypermobility of the foot (flat foot; pes planus) is shown by the presence of a longitudinal arch of the foot that disappears with weight bearing and may be associated with a shortened Achilles tendon (see Chapter 200) These findings are rarely associated with tenderness on examination No laboratory test abnormalities are apparent, and radiographs of affected joints are normal The diagnosis of isolated hypermobility is made on the basis of physical examination with demonstration of exaggerated mobility of a joint Generalized hypermobility is diagnosed by the presence of sufficient criteria (Table 92-1) and the absence of evidence of other underlying disorders Excessive skin elasticity, easy bruisability, or mitral valve prolapse suggests Ehlers-Danlos syndrome or Marfan syndrome rather than benign hypermobility The treatment of hypermobility consists of reassurance and regular stretching, similar to treatment for other benign musculoskeletal disorders NSAIDs can be administered as needed but not need to be prescribed on a regular basis Arch supports can be helpful in children with symptomatic pes planus but are not indicated in the absence of symptoms Benign hypermobility tends to improve with increasing age and is not associated with long-term complications MYOFASCIAL PAIN SYNDROMES AND FIBROMYALGIA The myofascial pain syndromes are a group of noninflammatory disorders characterized by diffuse musculoskeletal pain, the presence of multiple tender points, fatigue, malaise, and poor sleep patterns The etiology of these disorders is unknown, although there seems to be a familial predisposition Although these disorders sometimes follow viral infection or trauma, no causal relationship has been shown The myofascial pain syndromes are most common in adults but can occur in children (particularly >12 years of age) The syndromes are more common in girls than in boys The prevalence of fibromyalgia in children has been reported to be 6% Patients with myofascial pain syndromes complain of long-standing diffuse pain in muscles and in the soft tissues around joints that can occur at any time of day, awaken the patient from sleep, and interfere with regular activities There is frequently a high degree of school absenteeism, despite maintaining adequate school performance A significant percentage of patients with myofascial pain syndromes exhibit symptoms consistent with depression An increased incidence of sexual abuse has been reported in children presenting with fibromyalgia Physical examination is typically unremarkable with the exception of the presence of specific points that are 314 Section 15 u Rheumatic Diseases of Childhood painful—not just tender—to digital palpation These points often are located on the neck, back, lateral epicondyles, greater trochanter, and knees There is no evidence of arthritis or muscular weakness Patients with myofascial pain syndromes frequently undergo extensive medical testing because of the concern for underlying inflammatory disease These tests are invariably normal Children may have a false-positive antinuclear antibody, which is found in 20% of the normal pediatric population The diagnosis of myofascial pain syndrome is based on the presence of multiple tender points in the absence of other illness To fulfill strict criteria for a diagnosis of fibromyalgia, the patient must have a history of diffuse pain for at least months and the presence of 11 of 18 specific tender points on examination It is important to exclude underlying inflammatory diseases, such as systemic lupus erythematosus, or the postinfectious fatigue that characteristically follows Epstein-Barr virus and influenza virus infection Mood and conversion disorders also should be considered Treatment consists of pain control, usually using NSAIDs, physical therapy, relaxation techniques, and education regarding sleep hygiene Patients may require low doses of medications, such as amitriptyline to regulate sleep or gabapentin to reduce pain sensitivity Education and reassurance are crucial Because of the disability associated with myofascial pain syndromes, patients and parents frequently believe that the child has a serious underlying condition and may be resistant to reassurance It should be emphasized that there is no simple cure, and time and perseverance are required The long-term outcomes in the myofascial pain syndromes vary Patients and families who focus on therapy and are positive in their approach tend to have better outcomes Patients who demand prolonged evaluations, especially from multiple health care providers, may more poorly Overall children with fibromyalgia and myofascial pain syndromes have better prognoses than their adult counterparts Suggested Reading Connelly M, Schanberg L: Latest developments in the assessment and management of chronic musculoskeletal pain syndromes in children, Curr Opin Rheumatol 18:496–502, 2006 Falcini F: Kawasaki disease, Curr Opin Rheumatol 18:33–38, 2006 Feldman BM, Rider LG, Reed AM, et al: Juvenile dermatomyosítis and other idiopathic inflammatory myopathies of childhood, Lancet 371:2201–2212, 2008 Gottlieb BS, Ilowite NT: Systemic lupus erythematosus in children and adolescents, Pediatr Rev 27:323–330, 2006 Ravelli A, Martini A: Juvenile idiopathic arthritis, Lancet 369:767–778, 2007 Tizard EJ, Hamilton-Ayres MJJ: Henoch Schonlein purpura, Arch Dis Child Ed Pract 93:1–8, 2008 ... percentiles 12 13 14 15 16 17 18 19 20 cm AGE (YEARS) in 76 19 0 18 5 18 0 17 5 17 0 in cm 62 60 58 16 5 10 11 16 0 16 0 15 5 15 5 15 0 15 0 54 52 50 48 46 44 42 70 68 66 64 62 60 14 0 10 5 230 13 5 10 0 220 13 0 12 5... 210 90 200 12 0 85 11 5 80 11 0 75 10 5 70 19 0 18 0 17 0 16 0 95 15 0 65 14 0 60 13 0 36 90 55 12 0 34 85 50 11 0 32 80 45 10 0 40 90 35 35 30 30 25 25 20 20 15 15 10 kg 10 AGE (YEARS) kg 10 11 12 13 14 15 ... 22 22 21 21 20 20 19 19 18 18 17 17 16 16 15 15 14 14 13 13 12 12 AGE (YEARS) kg/m Figure 5-2 Head circumference and weight-by-length percentiles for boys, birth to years of age Developed by the