NEJM â Collection Updates for Child Health

CORRESPONDENCE: Responses to 2009 H1N1 Vaccine in Children 3 to 17 Years of Age

Adriano Arguedas, M.D., Carolina Soley, M.D., and Kelly Lindert, M.D. — Thu, 28Â JanÂ 2010 00:00:00 -05:00

To the Editor: The current 2009 pandemic influenza A (H1N1) virus is associated with substantial morbidity in children, with 45% of hospitalizations occurring in patients under 18 years of age.1...

EDITORIAL: Rotavirus Vaccine â A Powerful Tool to Combat Deaths from Diarrhea

Mathuram Santosham, M.D., M.P.H. — Thu, 28Â JanÂ 2010 00:00:00 -05:00

Rotavirus infection, the leading cause of severe childhood diarrhea in both developed and developing countries, results in over half a million deaths each year.1 Currently, two rotavirus vaccines (Rotarix [GlaxoSmithKline...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 3-2010 â A 5-Month-Old Boy with Developmental Delay and Irritability

Thu, 28Â JanÂ 2010 00:00:00 -05:00

A 4.5-month-old boy was seen in the pediatric neurology clinic of this hospital because of developmental delay and an abnormal neurologic examination.

The patient had been born at full-term by normal...

ORIGINAL ARTICLE: Vaccine-Acquired Rotavirus in Infants with Severe Combined Immunodeficiency

Thu, 28Â JanÂ 2010 00:00:00 -05:00

Live pentavalent humanâbovine reassortant rotavirus vaccine is recommended in the United States for routine immunization of infants. We describe three infants, two with failure to thrive, who had dehydration and...

ORIGINAL ARTICLE: Effect of Rotavirus Vaccination on Death from Childhood Diarrhea in Mexico

Thu, 28Â JanÂ 2010 00:00:00 -05:00

Background: A phased introduction of a monovalent rotavirus vaccine occurred in Mexico from February 2006 through May 2007. We assessed the effect of vaccination on deaths from diarrhea in Mexican...

ORIGINAL ARTICLE: Effect of Human Rotavirus Vaccine on Severe Diarrhea in African Infants

Thu, 28Â JanÂ 2010 00:00:00 -05:00

Background: Rotavirus is the most common cause of severe gastroenteritis among young children worldwide. Data are needed to assess the efficacy of the rotavirus vaccine in African children.

Methods: We conducted...

ORIGINAL ARTICLE: Pediatric Hospitalizations Associated with 2009 Pandemic Influenza A (H1N1) in Argentina

Thu, 07Â JanÂ 2010 00:00:00 -05:00

Background: While the Northern Hemisphere experiences the effects of the 2009 pandemic influenza A (H1N1) virus, data from the recent influenza season in the Southern Hemisphere can provide important information...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 41-2009 â A 16-Year-Old Boy with Hypothermia and Frostbite

Thu, 31Â DecÂ 2009 00:00:00 -05:00

Dr. Benjamin Sandefur (Emergency Medicine): A 16-year-old boy was admitted to the hospital after being found unconscious in a snow bank at 6 a.m. on New Year's Day. He had...

OTHER POINTS OF VIEW: When to Consider the Use of Antibiotics in the Treatment of 2009 H1N1 InfluenzaâAssociated Pneumonia

Thu, 10Â DecÂ 2009 00:00:00 -05:00

We are now facing a pandemic caused by an epidemiologically distinct, novel virus, the 2009 pandemic influenza A (H1N1) virus (swine flu), against which few persons born since 1970 have...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 38-2009 â A 16-Year-Old Boy with Paroxysmal Headaches and Visual Changes

Thu, 10Â DecÂ 2009 00:00:00 -05:00

A 16-year-old boy was seen in the pediatric neurology clinic of this hospital because of paroxysmal headaches and visual changes.

The patient had been well until approximately 4 months earlier, when...

PERSPECTIVE: Defeating Rotavirus? The Global Recommendation for Rotavirus Vaccination

Thu, 12Â NovÂ 2009 00:00:00 -05:00

This past April, the World Health Organization (WHO) Strategic Advisory Group of Experts (SAGE) on Immunization announced a global recommendation that rotavirus vaccines be included in national immunization programs. The...

CORRESPONDENCE: Risk of Confusion in Dosing Tamiflu Oral Suspension in Children

Thu, 05Â NovÂ 2009 00:00:00 -05:00

To the Editor: The medical community should be made aware of the serious potential for dosing errors in children prescribed Tamiflu (oseltamivir) oral suspension, as illustrated in the case described...

EDITORIAL: Antimicrobial Prophylaxis for Urinary Tract Infection in Children

Alejandro Hoberman, M.D., and Ron Keren, M.D., M.P.H. — Thu, 29Â OctÂ 2009 00:00:00 -05:00

Approximately one third of children who have a urinary tract infection â the most frequent serious bacterial infection in young febrile children â have vesicoureteral reflux, a congenital condition in...

ORIGINAL ARTICLE: Antibiotic Prophylaxis and Recurrent Urinary Tract Infection in Children

Thu, 29Â OctÂ 2009 00:00:00 -05:00

Background: Antibiotics are widely administered to children with the intention of preventing urinary tract infection, but adequately powered, placebo-controlled trials regarding efficacy are lacking. This study from four Australian centers...

PERSPECTIVE: How Health Care Reform Can Benefit Children and Adolescents

Thu, 22Â OctÂ 2009 00:00:00 -05:00

Medical and scientific advances have reduced the rates of acute childhood illnesses and childhood mortality and increased the positive outcomes of a wide variety of serious childhood illnesses; nevertheless, children's...

EDITORIAL: Blood-Pressure Control and Delay in Progression of Kidney Disease in Children

Julie R. Ingelfinger, M.D. — Thu, 22Â OctÂ 2009 00:00:00 -05:00

Many children with chronic kidney disease, even those in whom the disease is discovered very early, ultimately lose renal function; some ultimately progress to stage 5 chronic kidney disease (end-stage...

REVIEW ARTICLE: Atypical HemolyticâUremic Syndrome

Marina Noris, Ph.D., and Giuseppe Remuzzi, M.D. — Thu, 22Â OctÂ 2009 00:00:00 -05:00

The hemolyticâuremic syndrome is characterized by nonimmune hemolytic anemia, thrombocytopenia, and renal impairment.1 The disorder occurs most frequently in children under the age of 5 years, with an annual incidence...

ORIGINAL ARTICLE: Cisplatin versus Cisplatin plus Doxorubicin for Standard-Risk Hepatoblastoma

Thu, 22Â OctÂ 2009 00:00:00 -05:00

Background: Preoperative cisplatin alone may be as effective as cisplatin plus doxorubicin in standard-risk hepatoblastoma (a tumor involving three or fewer sectors of the liver that is associated with an...

ORIGINAL ARTICLE: Strict Blood-Pressure Control and Progression of Renal Failure in Children

The ESCAPE Trial Group — Thu, 22Â OctÂ 2009 00:00:00 -05:00

Background: Although inhibition of the reninâangiotensin system delays the progression of renal failure in adults with chronic kidney disease, the blood-pressure target for optimal renal protection is controversial. We assessed...

PERSPECTIVE: When Diseases Disappear â The Case of Familial Dysautonomia

Thu, 22Â OctÂ 2009 00:00:00 -05:00

The success of genetic-screening programs raises an intriguing possibility: some dread diseases of the 20th century may soon become history. A representative example is familial dysautonomia, a severe neurologic condition,...

IMAGES IN CLINICAL MEDICINE: Living with One Hemisphere â A Large Porencephalic Cyst

Antonio Trabacca, M.D. and Franca DiCuonzo, M.D. — Thu, 15Â OctÂ 2009 00:00:00 -05:00

A 2-year-old girl presented with gait disturbance and difficulty using her right hand. She had been born by normal spontaneous delivery at 38 weeks of gestation after an uncomplicated pregnancy....

ORIGINAL ARTICLE: Recurrence of Bile Salt Export Pump Deficiency after Liver Transplantation

Thu, 01Â OctÂ 2009 00:00:00 -05:00

Severe bile salt export pump (BSEP) deficiency is a hereditary cholestatic condition that starts in infancy and leads to end-stage liver disease. Three children who underwent orthotopic liver transplantation for...

ORIGINAL ARTICLE: Treatment of Medulloblastoma with Hedgehog Pathway Inhibitor GDC-0449

Thu, 17Â SepÂ 2009 00:00:00 -05:00

Medulloblastoma is the most common malignant brain tumor in children. Aberrant activation of the hedgehog signaling pathway is strongly implicated in the development of some cases of medulloblastoma. A 26-year-old...

BOOK REVIEW:

Thu, 20Â AugÂ 2009 00:00:00 -05:00

The publication of this book, just months after the death of Alvin Goldfarb â one of the founders of the North American Society for Pediatric and Adolescent Gynecology â prompts...

CORRESPONDENCE: Codeine, Ultrarapid-Metabolism Genotype, and Postoperative Death

Thu, 20Â AugÂ 2009 00:00:00 -05:00

To the Editor: Obstructive sleep apnea is not rare in children with hypertrophic tonsils, and the common curative procedure is adenotonsillectomy.1 Codeine is commonly prescribed for pain after adenotonsillectomy.2 The...

REVIEW ARTICLE: The Sudden Infant Death Syndrome

Hannah C. Kinney, M.D., and Bradley T. Thach, M.D. — Thu, 20Â AugÂ 2009 00:00:00 -05:00

The sudden infant death syndrome (SIDS), which is characterized by the sudden death of a seemingly healthy infant during a sleep period, has long been considered one of the most...

CORRESPONDENCE: Vision 1 Year after Gene Therapy for Leber's Congenital Amaurosis

Thu, 13Â AugÂ 2009 00:00:00 -05:00

To the Editor: Leber's congenital amaurosis, a common cause of blindness in infants and children,1 recently became the first human genetic retinal disease to show improved vision in response to...

IMAGES IN CLINICAL MEDICINE: Testicular Torsion in a Hydrocele

Eda Okatan, M.D. and Richard Keijzer, M.D., Ph.D. — Thu, 13Â AugÂ 2009 00:00:00 -05:00

A 6-week-old male infant with congenital bilateral hydroceles presented with a reddish, painful, and swollen left scrotum of 2 days' duration. There were no signs of inguinal hernia or fever....

BOOK REVIEW:

Thu, 30Â JulÂ 2009 00:00:00 -05:00

Medicating Children is an account of the past 100 years of the diagnosis and treatment of attention deficitâhyperactivity disorder (ADHD). The three authors come from different fields: Rick Mayes is...

ORIGINAL ARTICLE: AGC1 Deficiency Associated with Global Cerebral Hypomyelination

Thu, 30Â JulÂ 2009 00:00:00 -05:00

The mitochondrial aspartateâglutamate carrier isoform 1 (AGC1), specific to neurons and muscle, supplies aspartate to the cytosol and, as a component of the malateâaspartate shuttle, enables mitochondrial oxidation of cytosolic...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 23-2009 â A 13-Year-Old Boy with Headache, Nausea, Seizures, and Hypertension

Thu, 23Â JulÂ 2009 00:00:00 -05:00

A 13-year-old boy was admitted to the hospital because of headache, nausea, seizures, renal failure, and hypertension.

The patient had been well until approximately 3 weeks earlier, when intermittent left-sided headaches...

BOOK REVIEW:

Thu, 16Â JulÂ 2009 00:00:00 -05:00

Pediatric Endocrinology and Inborn Errors of Metabolism was designed for visual learners and features many tables and schematic presentations that are easy to consult. Its structure resembles the central intersection...

BOOK REVIEW:

Thu, 25Â JunÂ 2009 00:00:00 -05:00

This is the second edition of a book that was well received when it was first published. It has been updated substantially and now has a focus on problems of...

BOOK REVIEW:

Thu, 25Â JunÂ 2009 00:00:00 -05:00

The title and subtitle of this book promise a comprehensive examination of an important public health problem. However, the editors immediately disabuse readers of this notion by stating that the...

IMAGES IN CLINICAL MEDICINE: Ingested Magnets

Luigi Avolio, M.D. and Giuseppe Martucciello, M.D. — Thu, 25Â JunÂ 2009 00:00:00 -05:00

A 9-year-old boy ingested 23 magnets (Panel A). Four days later, he had clinical and surgical evidence of intestinal perforation and peritonitis due to pressure necrosis of the bowel. In...

ORIGINAL ARTICLE: Treating Childhood Acute Lymphoblastic Leukemia without Cranial Irradiation

Thu, 25Â JunÂ 2009 00:00:00 -05:00

Background: Prophylactic cranial irradiation has been a standard treatment in children with acute lymphoblastic leukemia (ALL) who are at high risk for central nervous system (CNS) relapse.

Methods: We conducted a...

BOOK REVIEW:

Thu, 18Â JunÂ 2009 00:00:00 -05:00

Susan Cohen and Christine Cosgrove, the authors of this compelling book, tell the story of how doctors treat tall and short children, and of how such treatment has evolved during...

BOOK REVIEW:

Thu, 18Â JunÂ 2009 00:00:00 -05:00

Normal fetal growth is determined by a number of factors. These include genetic potential, nutritional status of the mother, placental function and transfer of nutrients, and intrauterine hormones and growth...

PERSPECTIVE: Litigation, Regulation, and Education â Protecting the Public's Health through Childhood Immunization

Thu, 11Â JunÂ 2009 00:00:00 -05:00

Recently, three special masters of the U.S. Court of Federal Claims issued carefully reasoned, strongly worded opinions rejecting claims that medical and scientific evidence could demonstrate causal links between thimerosal-containing...

PERSPECTIVE: When Vaccine Injury Claims Go to Court

Thu, 11Â JunÂ 2009 00:00:00 -05:00

In February 2009, the National Vaccine Injury Compensation Program (VICP) released decisions for the first three test cases heard under the program's Omnibus Autism Proceeding. In each of the cases...

BOOK REVIEW:

Thu, 04Â JunÂ 2009 00:00:00 -05:00

The mystery and complexity of autism lead many to use metaphor in their attempts to describe it. Bear with me as I do the same. These two books lead the...

ORIGINAL ARTICLE: An Autoinflammatory Disease Due to Homozygous Deletion of the Locus

Thu, 04Â JunÂ 2009 00:00:00 -05:00

We describe a patient with an autoinflammatory disease in which the main clinical features are pustular rash, marked osteopenia, lytic bone lesions, respiratory insufficiency, and thrombosis. Genetic studies revealed a...

BOOK REVIEW:

Thu, 28Â MayÂ 2009 00:00:00 -05:00

A search in PubMed for publications that address pediatric nephrology, pediatric critical care, acute kidney injury, and acute dialysis yields more than 1000 publications in the past 15 years, with...

ORIGINAL ARTICLE: Inactivating Mutations in a Patient with Premature Pubarche

Thu, 28Â MayÂ 2009 00:00:00 -05:00

Dehydroepiandrosterone (DHEA) sulfotransferase, known as SULT2A1, converts the androgen precursor DHEA to its inactive sulfate ester, DHEAS, thereby preventing the conversion of DHEA to an active androgen. SULT2A1 requires 3â²-phosphoadenosine-5â²-phosphosulfate...

BOOK REVIEW:

Thu, 14Â MayÂ 2009 00:00:00 -05:00

This timely book describes the pediatric precursors of adult disease, with a focus on the metabolic syndrome and diabetes. The editors weave together three topic areas: epidemiology, pathophysiology, and treatment....

EDITORIAL: The Challenge of Managing Wheezing in Infants

Urs Frey, M.D., Ph.D., and Erika von Mutius, M.D., M.Sc. — Thu, 14Â MayÂ 2009 00:00:00 -05:00

Around the world, thousands of times a day, an infant 6 to 8 months old with a runny nose, a cough, and wheezing arrives in a pediatrician's office or an...

ORIGINAL ARTICLE: Antivenom for Critically Ill Children with Neurotoxicity from Scorpion Stings

Thu, 14Â MayÂ 2009 00:00:00 -05:00

Background: Clinically significant scorpion envenomation by Centruroides sculpturatus produces a dramatic neuromotor syndrome and respiratory insufficiency that often necessitate intensive supportive care. We hypothesized that a scorpion-specific F(abâ²)2 antivenom would...

ORIGINAL ARTICLE: Epinephrine and Dexamethasone in Children with Bronchiolitis

Thu, 14Â MayÂ 2009 00:00:00 -05:00

Background: Although numerous studies have explored the benefit of using nebulized epinephrine or corticosteroids alone to treat infants with bronchiolitis, the effectiveness of combining these medications is not well established.

Methods:...

SPECIAL ARTICLE: Vaccine Refusal, Mandatory Immunization, and the Risks of Vaccine-Preventable Diseases

Thu, 07Â MayÂ 2009 00:00:00 -05:00

Vaccines are among the most effective prevention tools available to clinicians. However, the success of an immunization program depends on high rates of acceptance and coverage. There is evidence of...

BOOK REVIEW:

Thu, 23Â AprÂ 2009 00:00:00 -05:00

The field of pediatric nephrology emerged in the early 1950s, largely because of the remarkable success of glucocorticoid therapy for childhood nephrotic syndrome and the use of percutaneous renal biopsies...

BOOK REVIEW:

Thu, 16Â AprÂ 2009 00:00:00 -05:00

More than 50 years ago, John Money and colleagues proposed that gender identity was shaped by rearing and social influence. They also argued that sexual reassignment was possible during the...

EDITORIAL: Which Drug for the Pregnant Woman with Epilepsy?

TorbjÃ¶rn Tomson, M.D. — Thu, 16Â AprÂ 2009 00:00:00 -05:00

Approximately 25,000 children are born in the United States each year to mothers with epilepsy.1 Most of these women need to continue taking medication during pregnancy, since uncontrolled seizures may...

ORIGINAL ARTICLE: Cognitive Function at 3 Years of Age after Fetal Exposure to Antiepileptic Drugs

Thu, 16Â AprÂ 2009 00:00:00 -05:00

Background: Fetal exposure of animals to antiepileptic drugs at doses lower than those required to produce congenital malformations can produce cognitive and behavioral abnormalities, but cognitive effects of fetal exposure...

IMAGES IN CLINICAL MEDICINE: Gingival and Periungual Vasculopathy of Juvenile Dermatomyositis

Lisa G. Rider, M.D. and Jane C. Atkinson, D.D.S. — Thu, 09Â AprÂ 2009 00:00:00 -05:00

A 16-year-old girl with long-standing juvenile dermatomyositis had an illness marked by intermittent clinical flares, including increased muscle weakness, joint contractures, photosensitive rashes, and dysphagia, despite treatment with multiple immunosuppressive...

CORRESPONDENCE: Ending Propylthiouracil-Induced Liver Failure in Children

Scott A. Rivkees, M.D. and Donald R. Mattison, M.D. — Thu, 09Â AprÂ 2009 00:00:00 -05:00

To the Editor: Graves' disease is treated with antithyroid drugs, radioactive iodine, or surgery.12 Propylthiouracil and methimazole are widely used in children as first-line therapy.12 Over the past 60 years...

CLINICAL PRACTICE: Evaluation and Management of Enuresis

W. Lane M. Robson, M.D. — Thu, 02Â AprÂ 2009 00:00:00 -05:00

The parents of an 8-year-old boy bring him to the pediatrician's office for evaluation of nightly bed-wetting. On half the nights, his urine soaks through a pair of absorbent disposable...

ORIGINAL ARTICLE: Genetic Compensation in a Human Genomic Disorder

Thu, 19Â MarÂ 2009 00:00:00 -05:00

Cytogenetic studies of the parents of a girl with the DiGeorge (or velocardiofacial) syndrome, who carried a deletion at 22q11.2, revealed an unexpected rearrangement of both 22q11.2 regions in the...

BOOK REVIEW:

Thu, 12Â MarÂ 2009 00:00:00 -05:00

In recent years, the public has been increasingly concerned about adverse events that have been attributed to vaccines. Although such safety concerns have existed since the days of Edward Jenner,...

CORRESPONDENCE: Melamine and Nephrolithiasis in Children in Taiwan

Thu, 12Â MarÂ 2009 00:00:00 -05:00

To the Editor: The adverse renal effects of melamine-tainted formula have raised international concern. To address public concerns about melamine, the department of health in Taiwan initiated services to screen...

CORRESPONDENCE: Ultrasonographic Evaluation of Melamine-Exposed Children in Hong Kong

Thu, 12Â MarÂ 2009 00:00:00 -05:00

To the Editor: The recent melamine contamination of infant formula and milk products in China placed thousands of children in China and neighboring regions at risk for renal-stone formation and...

EDITORIAL: Melamine, Powdered Milk, and Nephrolithiasis in Chinese Infants

Craig B. Langman, M.D. — Thu, 12Â MarÂ 2009 00:00:00 -05:00

Melamine, a synthetic nitrogenous product found in many industrial goods and even in fertilizer destined for use in growing crops for human consumption, was recently found to have been added...

ORIGINAL ARTICLE: Melamine-Contaminated Powdered Formula and Urolithiasis in Young Children

Thu, 12Â MarÂ 2009 00:00:00 -05:00

Background: A recent epidemic of melamine contamination of baby formula in China has been associated with the development of urinary tract stones, though the clinical manifestations and predisposing factors are...

BOOK REVIEW:

Thu, 05Â MarÂ 2009 00:00:00 -05:00

This book for pediatric surgeons, fellows, residents, and pediatric oncologists reflects the extensive experience of its editors and authors in caring for children with cancer. It is well written, well...

PERSPECTIVE: Expanding Coverage for Children â The Democrats' Power and SCHIP Reauthorization

Thu, 26Â FebÂ 2009 00:00:00 -05:00

In the first demonstration of Democratic dominance on health care issues since Barack Obama captured the White House, the new Congress quickly reauthorized the State Children's Health Insurance Program (SCHIP),...

PERSPECTIVE: Investing in Health Care Reform

Thu, 26Â FebÂ 2009 00:00:00 -05:00

Indicating that health care reform is integral to his policy agenda, President Barack Obama has moved swiftly to incorporate elements of reform into his economic recovery bill and signed reauthorization...

IMAGES IN CLINICAL MEDICINE: A Tuba Player with Air in the Parotid Gland

Deepa Mukundan, M.D. and Oliver Jenkins, M.D. — Thu, 12Â FebÂ 2009 00:00:00 -05:00

A 13-year-old boy who had recently begun playing the tuba presented with a 2-day history of facial swelling on the left side and pain without fever or ear or nasal...

CORRESPONDENCE: Prevalence of Mitochondrial 1555AâG Mutation in European Children

Thu, 05Â FebÂ 2009 00:00:00 -05:00

To the Editor: Aminoglycoside antibiotics are used worldwide to treat gram-negative sepsis. Since these drugs are ototoxic and nephrotoxic, drug levels are closely monitored. However, their effect on patients with...

ORIGINAL ARTICLE: Mutations in in Autosomal Nonsyndromic Mental Retardation

Thu, 05Â FebÂ 2009 00:00:00 -05:00

Although autosomal forms of nonsyndromic mental retardation account for the majority of cases of mental retardation, the genes that are involved remain largely unknown. We sequenced the autosomal gene SYNGAP1,...

ORIGINAL ARTICLE: The Burden of Respiratory Syncytial Virus Infection in Young Children

Thu, 05Â FebÂ 2009 00:00:00 -05:00

Background: The primary role of respiratory syncytial virus (RSV) in causing infant hospitalizations is well recognized, but the total burden of RSV infection among young children remains poorly defined.

Methods: We...

CORRESPONDENCE: Eculizumab for Congenital Atypical HemolyticâUremic Syndrome

Ralph A. Gruppo, M.D. and Russell P. Rother, Ph.D. — Thu, 29Â JanÂ 2009 00:00:00 -05:00

To the Editor: Atypical hemolyticâuremic syndrome of infancy is a rare disorder that is associated with thrombotic microangiopathy and acute renal failure. It often involves complement dysregulation.12 Plasma infusions have...

EDITORIAL: Gene Therapy Fulfilling Its Promise

Donald B. Kohn, M.D., and Fabio Candotti, M.D. — Thu, 29Â JanÂ 2009 00:00:00 -05:00

From its earliest conception, gene therapy held the promise of correcting inherited diseases by inserting a normal copy of the relevant gene into somatic cells.1 Common monogenic diseases of blood...

CLINICAL THERAPEUTICS: Behavioral Management for Anorexia Nervosa

Evelyn Attia, M.D., and B. Timothy Walsh, M.D. — Thu, 29Â JanÂ 2009 00:00:00 -05:00

A 23-year-old woman with anorexia nervosa visits her internist. Her height is 5 ft 6 in. (168 cm), and her weight is 104 lb (47 kg), with a body-mass index...

ORIGINAL ARTICLE: Gene Therapy for Immunodeficiency Due to Adenosine Deaminase Deficiency

Thu, 29Â JanÂ 2009 00:00:00 -05:00

Background: We investigated the long-term outcome of gene therapy for severe combined immunodeficiency (SCID) due to the lack of adenosine deaminase (ADA), a fatal disorder of purine metabolism and immunodeficiency.

Methods:...

EDITORIAL: Practice Imperfect â Treatment for Wheezing in Preschoolers

Andrew Bush, M.D. — Thu, 22Â JanÂ 2009 00:00:00 -05:00

Nearly one third of preschool children (4 years of age or younger) have intermittent wheezing, a condition that many of them outgrow. The most common early trigger is a respiratory...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 3-2009 â A 9-Month-Old Boy with Seizures

Michael F. Holick, M.D., Ph.D., Ruth Lim, M.D., and Anand S. Dighe, M.D., Ph.D. — Thu, 22Â JanÂ 2009 00:00:00 -05:00

Dr. Bronwen C. Carroll (Pediatrics): A 9-month-old boy was admitted to this hospital because of a generalized seizure and a bulging fontanelle.

The patient was reportedly well until 3 days before...

ORIGINAL ARTICLE: Preemptive Use of High-Dose Fluticasone for Virus-Induced Wheezing in Young Children

Thu, 22Â JanÂ 2009 00:00:00 -05:00

Background: Although virus-induced wheezing is common in preschool-age children, optimal management remains elusive. We examined the efficacy and safety of preemptive treatment with high-dose fluticasone in reducing the severity of...

ORIGINAL ARTICLE: Oral Prednisolone for Preschool Children with Acute Virus-Induced Wheezing

Thu, 22Â JanÂ 2009 00:00:00 -05:00

Background: Attacks of wheezing induced by upper respiratory viral infections are common in preschool children between the ages of 10 months and 6 years. A short course of oral prednisolone...

ORIGINAL ARTICLE: Effect of Pneumococcal Conjugate Vaccine on Pneumococcal Meningitis

Thu, 15Â JanÂ 2009 00:00:00 -05:00

Background: Invasive pneumococcal disease declined among children and adults after the introduction of the pediatric heptavalent pneumococcal conjugate vaccine (PCV7) in 2000, but its effect on pneumococcal meningitis is unclear.

Methods:...

BOOK REVIEW:

Thu, 08Â JanÂ 2009 00:00:00 -05:00

Asperger's Disorder is a fascinating compilation of scientific papers that concern a condition about which few people know anything. The book is a page turner â I could hardly put...

BOOK REVIEW:

Thu, 01Â JanÂ 2009 00:00:00 -05:00

Millions of children worldwide suffer from allergic diseases such as asthma, atopic dermatitis, allergic rhinitis, or food allergy. The diagnosis and treatment of these children require special attention to the...

BOOK REVIEW:

Thu, 01Â JanÂ 2009 00:00:00 -05:00

It is the year 1908. In the United States, more than 150,000 people will die from tuberculosis. If you contract tuberculosis, your best hope is to be shipped off to...

BOOK REVIEW:

Thu, 01Â JanÂ 2009 00:00:00 -05:00

Hope and Suffering is an apt title for this dense, encyclopedic, and riveting book. It includes narratives from patients and their family members that detail the hope, suffering, and despair...

ORIGINAL ARTICLE: A Syndrome with Congenital Neutropenia and Mutations in

Thu, 01Â JanÂ 2009 00:00:00 -05:00

Background: The main features of severe congenital neutropenia are the onset of severe bacterial infections early in life, a paucity of mature neutrophils, and an increased risk of leukemia. In...

PERSPECTIVE: The Many Causes of Severe Congenital Neutropenia

Thu, 01Â JanÂ 2009 00:00:00 -05:00

In 1956, Rolf Kostmann, a Swedish pediatrician, described an autosomal recessive disorder that he called infantile genetic agranulocytosis â which is now called severe congenital neutropenia. The Kostmann form of...

EDITORIAL: Pediatric Anxiety â Underrecognized and Undertreated

Graham J. Emslie, M.D. — Thu, 25Â DecÂ 2008 00:00:00 -05:00

Generalized anxiety disorder, separation anxiety disorder, and social phobia are relatively prevalent disorders that affect 6 to 20% of children and adolescents.1 However, these disorders frequently go unrecognized by medical...

ORIGINAL ARTICLE: Cognitive Behavioral Therapy, Sertraline, or a Combination in Childhood Anxiety

Thu, 25Â DecÂ 2008 00:00:00 -05:00

Background: Anxiety disorders are common psychiatric conditions affecting children and adolescents. Although cognitive behavioral therapy and selective serotonin-reuptake inhibitors have shown efficacy in treating these disorders, little is known about...

PERSPECTIVE: Melamine and the Global Implications of Food Contamination

Thu, 25Â DecÂ 2008 00:00:00 -05:00

Food contamination, whether accidental or intentional, has been a sad, recurrent theme throughout recorded history, going back some 8000 years and described in the Old Testament. However, a new dimension...

IMAGES IN CLINICAL MEDICINE: Subungual Exostosis

Alexandre Campanelli, M.D. and Luca Borradori, M.D. — Thu, 18Â DecÂ 2008 00:00:00 -05:00

A 12-year-old girl with an unremarkable history was evaluated for a nodule of the lateral nail fold on the right hallux that had been present for 3 months (Panel A)....

BOOK REVIEW:

Thu, 18Â DecÂ 2008 00:00:00 -05:00

Dyslexia, an unexpected and unexplained difficulty in learning to read when cognitive function is otherwise normal, is a major disability. Much of the educational process in schools is based on...

BOOK REVIEW:

Thu, 11Â DecÂ 2008 00:00:00 -05:00

The focus of this book is not limited to sleep difficulties in children and adolescents with psychiatric disorders. About half of the 28 chapters deal with such problems as autism,...

EDITORIAL: Energy In, Energy Out, and the Effects of Obesity-Related Genes

Rudolph L. Leibel, M.D. — Thu, 11Â DecÂ 2008 00:00:00 -05:00

More than 100 genes have been implicated in the determination of body weight. These genes, acting primarily in or through the central nervous system (primarily the hypothalamus and brain stem),...

EDITORIAL: A Hopeful Beginning for Malaria Vaccines

William E. Collins, Ph.D., and John W. Barnwell, M.P.H., Ph.D. — Thu, 11Â DecÂ 2008 00:00:00 -05:00

An effective human malaria vaccine has been sought for over 70 years, with little success.1 A successful malaria vaccine used in conjunction with other control interventions would help reduce and...

ORIGINAL ARTICLE: An Obesity-Associated Gene Variant and Increased Energy Intake in Children

Thu, 11Â DecÂ 2008 00:00:00 -05:00

Background: Variation in the fat mass and obesityâassociated (FTO) gene has provided the most robust associations with common obesity to date. However, the role of FTO variants in modulating specific...

ORIGINAL ARTICLE: A Trial of Combination Antimalarial Therapies in Children from Papua New Guinea

Thu, 11Â DecÂ 2008 00:00:00 -05:00

Background: Malaria control is difficult where there is intense year-round transmission of multiple plasmodium species, such as in Papua New Guinea.

Methods: Between April 2005 and July 2007, we conducted an...

ORIGINAL ARTICLE: Safety and Immunogenicity of RTS,S/AS02D Malaria Vaccine in Infants

Thu, 11Â DecÂ 2008 00:00:00 -05:00

Background: The RTS,S/AS malaria vaccine is being developed for delivery through the World Health Organization's Expanded Program on Immunization (EPI). We assessed the feasibility of integrating RTS,S/AS02D into a standard...

ORIGINAL ARTICLE: Efficacy of RTS,S/AS01E Vaccine against Malaria in Children 5 to 17 Months of Age

Thu, 11Â DecÂ 2008 00:00:00 -05:00

Background: Plasmodium falciparum malaria is a pressing global health problem. A previous study of the malaria vaccine RTS,S (which targets the circumsporozoite protein), given with an adjuvant system (AS02A), showed...

BOOK REVIEW:

Thu, 04Â DecÂ 2008 00:00:00 -05:00

William Carlos Williams (1883â1963) was a pediatrician who made house calls from Rutherford to Paterson, New Jersey. Despite the daily grind of working as a physician, he was posthumously awarded...

EDITORIAL: The Genetics of Speech and Language Impairments

Karin Stromswold, M.D., Ph.D. — Thu, 27Â NovÂ 2008 00:00:00 -05:00

Without instruction, most children master the complexities of spoken language by the age of 6 or 7 years. About 5% of apparently healthy children, however, struggle to acquire basic competence...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 37-2008 â A 17-Year-Old Boy with a Pituitary Tumor and Skull Abnormalities

Thu, 27Â NovÂ 2008 00:00:00 -05:00

A 17-year-old boy was seen at this hospital because of a pituitary tumor and radiologic evidence of bony abnormalities in the skull. The patient was well until approximately 12 years...

ORIGINAL ARTICLE: A Functional Genetic Link between Distinct Developmental Language Disorders

Thu, 27Â NovÂ 2008 00:00:00 -05:00

Background: Rare mutations affecting the FOXP2 transcription factor cause a monogenic speech and language disorder. We hypothesized that neural pathways downstream of FOXP2 influence more common phenotypes, such as specific...

EDITORIAL: 17q21 Variants and Asthma â Questions and Answers

John W. Holloway, Ph.D., and Gerard H. Koppelman, M.D., Ph.D. — Thu, 06Â NovÂ 2008 00:00:00 -05:00

It has long been established that many common diseases are the result of interactions between genetic susceptibility and environmental exposure. The identification of genetic variants that underlie susceptibility to diseases...

ORIGINAL ARTICLE: Effect of 17q21 Variants and Smoking Exposure in Early-Onset Asthma

Thu, 06Â NovÂ 2008 00:00:00 -05:00

Background: A genomewide association study has shown an association between variants at chromosome 17q21 and an increased risk of asthma. To elucidate the relationship between this locus and disease, we...

EDITORIAL: Immunotherapy on Trial for New-Onset Type 1 Diabetes

Denise L. Faustman, M.D., Ph.D. — Thu, 30Â OctÂ 2008 00:00:00 -05:00

Type 1 diabetes mellitus is an autoimmune disease in which pathologic, autoreactive T cells of the immune system attack the insulin-secreting pancreatic islets of Langerhans. Cytotoxic T cells, which bear...

ORIGINAL ARTICLE: GAD Treatment and Insulin Secretion in Recent-Onset Type 1 Diabetes

Thu, 30Â OctÂ 2008 00:00:00 -05:00

Background: The 65-kD isoform of glutamic acid decarboxylase (GAD) is a major autoantigen in patients with type 1 diabetes mellitus. This trial assessed the ability of alum-formulated GAD (GAD-alum) to...

BOOK REVIEW:

Thu, 16Â OctÂ 2008 00:00:00 -05:00

Adolescent Health Care is a comprehensive book that will be of considerable value to clinicians working with teenagers and young adults. This useful book begins with a solid overview of...

EDITORIAL: Monovalent Oral Poliovirus Vaccines â A Good Tool but Not a Total Solution

Ellie Ehrenfeld, Ph.D., and Konstantin Chumakov, Ph.D. — Thu, 16Â OctÂ 2008 00:00:00 -05:00

The polio eradication campaign initiated in 1988 by the World Health Organization (WHO) led to an impressive decline in cases of paralytic poliomyelitis around the world. The eradication strategy involved...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 32-2008 â A 10-Year-Old Girl with Recurrent Oral Lesions and Cutaneous Bullae

James G.H. Dinulos, M.D., and Artur Zembowicz, M.D. — Thu, 16Â OctÂ 2008 00:00:00 -05:00

Dr. Melissa M. Burnett (Pediatric Dermatology): A 10.8-year-old girl was seen in the Pediatric Dermatology Clinic of this hospital because of recurrent oral ulcers and cutaneous bullae.

The patient was well...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 32-2008 â A 10-Year-Old Girl with Recurrent Oral Lesions and Cutaneous Bullae

James G.H. Dinulos, M.D., and Artur Zembowicz, M.D. — Thu, 16Â OctÂ 2008 00:00:00 -05:00

ORIGINAL ARTICLE: Recurrent Rearrangements of Chromosome 1q21.1 and Variable Pediatric Phenotypes

Thu, 16Â OctÂ 2008 00:00:00 -05:00

Background: Duplications and deletions in the human genome can cause disease or predispose persons to disease. Advances in technologies to detect these changes allow for the routine identification of submicroscopic...

ORIGINAL ARTICLE: Effectiveness of Immunization against Paralytic Poliomyelitis in Nigeria

Thu, 16Â OctÂ 2008 00:00:00 -05:00

Background: The number of cases of paralytic poliomyelitis has declined in Nigeria since the introduction of newly licensed monovalent oral poliovirus vaccines and new techniques of vaccine delivery. Understanding the...

PERSPECTIVE: Storm over Statins â The Controversy Surrounding Pharmacologic Treatment of Children

Thu, 25Â SepÂ 2008 00:00:00 -05:00

In July of this year, the American Academy of Pediatrics (AAP) released revised recommendations for the management of hypercholesterolemia in children (see table).1 Within days after publication, the new policy...

BOOK REVIEW:

Thu, 18Â SepÂ 2008 00:00:00 -05:00

The earliest symptoms of autism spectrum disorders often appear before a child's second birthday, but most children with autism are not diagnosed until they are in preschool or elementary school....

BOOK REVIEW:

Thu, 18Â SepÂ 2008 00:00:00 -05:00

Gastrointestinal disease in children is often the orphan of clinical inquiry and scientific investigation. This book provides a thorough update of the pathophysiology and clinical features of gastrointestinal disease in...

BOOK REVIEW:

Thu, 18Â SepÂ 2008 00:00:00 -05:00

Pediatric rheumatology has a quality that sets it apart from other pediatric subspecialties. In some respects, this quality is determined by the limited number of board-certified practitioners caring for an...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 29-2008 â A 19-Year-Old Man with Weight Loss and Abdominal Pain

Thu, 18Â SepÂ 2008 00:00:00 -05:00

Dr. Jennifer Broder (Pediatrics): A 19-year-old man was admitted to this hospital because of acute abdominal pain.

He had been in his usual state of health until the evening before admission,...

CLINICAL PRACTICE: Food Allergy

Gideon Lack, M.D. — Thu, 18Â SepÂ 2008 00:00:00 -05:00

A 3-year-old boy with a recent history of hives and wheezing after eating chocolate mousse is evaluated. At 18 months of age, contact urticaria developed after he put his hand...

PERSPECTIVE: Close Calls

Thu, 04Â SepÂ 2008 00:00:00 -05:00

I didn't realize I was on call that night until we were at Fenway Park and the ball game was well under way. Call started at 9 p.m., and frankly,...

ORIGINAL ARTICLE: Brain-Derived Neurotrophic Factor and Obesity in the WAGR Syndrome

Thu, 28Â AugÂ 2008 00:00:00 -05:00

Background: Brain-derived neurotrophic factor (BDNF) has been found to be important in energy homeostasis in animal models, but little is known about its role in energy balance in humans. Heterozygous,...

EDITORIAL: Human Papillomavirus Vaccination â Reasons for Caution

Charlotte J. Haug, M.D., Ph.D. — Thu, 21Â AugÂ 2008 00:00:00 -05:00

Despite great expectations and promising results of clinical trials, we still lack sufficient evidence of an effective vaccine against cervical cancer. Several strains of human papillomavirus (HPV) can cause cervical...

SPECIAL ARTICLE: Health and Economic Implications of HPV Vaccination in the United States

Jane J. Kim, Ph.D., and Sue J. Goldie, M.D., M.P.H. — Thu, 21Â AugÂ 2008 00:00:00 -05:00

Background: The cost-effectiveness of prophylactic vaccination against human papillomavirus types 16 (HPV-16) and 18 (HPV-18) is an important consideration for guidelines for immunization in the United States.

Methods: We synthesized epidemiologic...

ORIGINAL ARTICLE: Adalimumab with or without Methotrexate in Juvenile Rheumatoid Arthritis

Thu, 21Â AugÂ 2008 00:00:00 -05:00

Background: Tumor necrosis factor (TNF) has a pathogenic role in juvenile rheumatoid arthritis. We evaluated the efficacy and safety of adalimumab, a fully human monoclonal anti-TNF antibody, in children with...

EDITORIAL: Cardiac Transplantation in Infants

Gregory D. Curfman, M.D., Stephen Morrissey, Ph.D., and Jeffrey M. Drazen, M.D. — Thu, 14Â AugÂ 2008 00:00:00 -05:00

For children born with inoperable congenital heart disease or advanced cardiomyopathy, cardiac transplantation is the only therapeutic option. Each year in the United States, approximately 400 heart-transplantation procedures are performed...

ORIGINAL ARTICLE: Pediatric Heart Transplantation after Declaration of Cardiocirculatory Death

Thu, 14Â AugÂ 2008 00:00:00 -05:00

In three infants awaiting orthotopic cardiac transplantation, transplantation was successfully performed with the use of organs from donors who had died from cardiocirculatory causes. The three recipients had blood group...

IMAGES IN CLINICAL MEDICINE: Horizontal Pendular Nystagmus in a Patient with Ocular Albinism

Ariel D. Teitel, M.D. and Josh Rubin, M.D. — Thu, 31Â JulÂ 2008 00:00:00 -05:00

A 32-year-old Hispanic woman with ocular albinism presented with a congenital horizontal pendular nystagmus (see video). Other than being fair-skinned, the patient had no cutaneous manifestations of albinism, and pigmentation...

ORIGINAL ARTICLE: Long-Term Medical and Social Consequences of Preterm Birth

Dag Moster, M.D., Ph.D., Rolv Terje Lie, Ph.D., and Trond Markestad, M.D., Ph.D. — Thu, 17Â JulÂ 2008 00:00:00 -05:00

Background: Advances in perinatal care have increased the number of premature babies who survive. There are concerns, however, about the ability of these children to cope with the demands of...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 21-2008 â An 11-Month-Old Boy with Fever and Pulmonary Infiltrates

Thu, 10Â JulÂ 2008 00:00:00 -05:00

An 11-month-old boy was admitted to this hospital because of fevers and pulmonary infiltrates. One month before admission, a month after beginning a visit to India with his parents, daily...

REVIEW ARTICLE: Effect of In Utero and Early-Life Conditions on Adult Health and Disease

Thu, 03Â JulÂ 2008 00:00:00 -05:00

A long latency period between an environmental trigger and the onset of subsequent disease is widely recognized in the etiology of certain cancers, yet this phenomenon is not generally considered...

EDITORIAL: Neuroblastoma â Linking a Common Allele to a Rare Disease

Brian H. Kushner, M.D., and Nai-Kong V. Cheung, M.D., Ph.D. — Thu, 12Â JunÂ 2008 00:00:00 -05:00

Neuroblastoma has a notorious reputation among solid tumors of childhood because of its frequently massive and widespread tumor burden. Yet stage for stage, this embryonal neoplasm of the sympathetic nervous...

ORIGINAL ARTICLE: Chromosome 6p22 Locus Associated with Clinically Aggressive Neuroblastoma

Thu, 12Â JunÂ 2008 00:00:00 -05:00

Background: Neuroblastoma is a malignant condition of the developing sympathetic nervous system that most commonly affects young children and is often lethal. Its cause is not known.

Methods: We performed a...

ORIGINAL ARTICLE: Hypothermia Therapy after Traumatic Brain Injury in Children

Thu, 05Â JunÂ 2008 00:00:00 -05:00

Background: Hypothermia therapy improves survival and the neurologic outcome in animal models of traumatic brain injury. However, the effect of hypothermia therapy on the neurologic outcome and mortality among children...

CLINICAL PRACTICE: Precocious Puberty

Jean-Claude Carel, M.D., and Juliane LÃ©ger, M.D. — Thu, 29Â MayÂ 2008 00:00:00 -05:00

The parents of a 6-year-old girl bring her to a pediatrician because of breast development. Her medical history is unremarkable. The parents are of average height, and the mother reports...

PERSPECTIVE: The Moral of the Story

Thu, 29Â MayÂ 2008 00:00:00 -05:00

I came home the other night clutching a scrap of paper towel with a mother's cell-phone number scribbled on it. I had been precepting in the residents' pediatric primary care...

EDITORIAL: Preliminary Results of Gene Therapy for Retinal Degeneration

Joan W. Miller, M.D. — Thu, 22Â MayÂ 2008 00:00:00 -05:00

In this issue of the Journal, two groups of investigators â Bainbridge et al.1 and Maguire et al.2 â describe the first results of separate clinical trials investigating the short-term...

IMAGES IN CLINICAL MEDICINE: Ocular Trauma Due to a Water-Bottle Cap

Shiyoung Roh, M.D. and Mark E. Patron, M.D. — Thu, 22Â MayÂ 2008 00:00:00 -05:00

A 14-year-old girl presented with a traumatic injury to the right eye from a water-bottle cap, caused by the increasingly popular adolescent activity of twisting and crushing an empty plastic...

ORIGINAL ARTICLE: Safety and Efficacy of Gene Transfer for Leber's Congenital Amaurosis

Thu, 22Â MayÂ 2008 00:00:00 -05:00

Leber's congenital amaurosis (LCA) is a group of inherited blinding diseases with onset during childhood. One form of the disease, LCA2, is caused by mutations in the retinal pigment epitheliumâspecific...

ORIGINAL ARTICLE: Effect of Gene Therapy on Visual Function in Leber's Congenital Amaurosis

Thu, 22Â MayÂ 2008 00:00:00 -05:00

Early-onset, severe retinal dystrophy caused by mutations in the gene encoding retinal pigment epitheliumâspecific 65-kD protein (RPE65) is associated with poor vision at birth and complete loss of vision in...

PERSPECTIVE: Vaccines and Autism Revisited â The Hannah Poling Case

Thu, 15Â MayÂ 2008 00:00:00 -05:00

On April 11, 2008, the National Vaccine Advisory Committee took an unusual step: in the name of transparency, trust, and collaboration, it asked members of the public to help set...

ORIGINAL ARTICLE: An Immunodeficiency Disease with Mutations and Granulomas

Thu, 08Â MayÂ 2008 00:00:00 -05:00

We describe three unrelated girls who had an immunodeficiency disease with granulomas in the skin, mucous membranes, and internal organs. All three girls had severe complications after viral infections, including...

IMAGES IN CLINICAL MEDICINE: Nevus Sebaceus of Jadassohn

Sherrif F. Ibrahim, M.D., Ph.D. and John H. Tu, M.D. — Thu, 01Â MayÂ 2008 00:00:00 -05:00

A 14-year-old girl came to the hospital with her mother, stating that a small birthmark on the scalp had recently started growing rather rapidly. It was increasingly pruritic and caused...

ORIGINAL ARTICLE: Shared Genetic Causes of Cardiac Hypertrophy in Children and Adults

Thu, 01Â MayÂ 2008 00:00:00 -05:00

Background: The childhood onset of idiopathic cardiac hypertrophy that occurs without a family history of cardiomyopathy can portend a poor prognosis. Despite morphologic similarities to genetic cardiomyopathies of adulthood, the...

IMAGES IN CLINICAL MEDICINE: Lingual Thyroid

Mohammad Hassan Moaddab, M.D. and Mansour Siavash, M.D. — Thu, 17Â AprÂ 2008 00:00:00 -05:00

A healthy girl, 4 1/2 years of age, was brought to our clinic for an assessment of her growth. A general physical examination was normal except for an incidental mass...

REVIEW ARTICLE: Management and Outcomes of Very Low Birth Weight

Eric C. Eichenwald, M.D., and Ann R. Stark, M.D. — Thu, 17Â AprÂ 2008 00:00:00 -05:00

Approximately 12.5% of births in the United States are preterm (occurring before 37 weeks of gestation). 08041729.xml 08041729.xml 08041729.xml 08041729.xml Preterm infants with âvery lowâ birth weight are those who...

ORIGINAL ARTICLE: Recent Resurgence of Mumps in the United States

Thu, 10Â AprÂ 2008 00:00:00 -05:00

Background: The widespread use of a second dose of mumps vaccine among U.S. schoolchildren beginning in 1990 was followed by historically low reports of mumps cases. A 2010 elimination goal...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 10-2008 â A 10-Year-Old Girl with Dyspnea on Exertion

Thu, 27Â MarÂ 2008 00:00:00 -05:00

A 10-year-old girl was seen in the multidisciplinary Airway, Voice, and Swallowing Center for Children at the Massachusetts Eye and Ear Infirmary, associated with this hospital, because of dyspnea and...

PERSPECTIVE: Taking Your Child's Breath Away â The Extension of Asthma's Global Reach

Thu, 20Â MarÂ 2008 00:00:00 -05:00

On a clear summer day, as Michael runs through the fields playing with his friends, the view from his farm is spectacular. You can look past the hills where his...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 7-2008 â A 17-Year-Old Girl with Chest Pain and Hemoptysis

Thu, 28Â FebÂ 2008 00:00:00 -05:00

Dr. Mary Shannon Fracchia (Pediatrics): A 17-year-old girl was transferred to this hospital because of chest pain and hemoptysis. She had been well except for mild asthma until 4 months...

ORIGINAL ARTICLE: Severe Anemia in Malawian Children

Thu, 28Â FebÂ 2008 00:00:00 -05:00

Background: Severe anemia is a major cause of sickness and death in African children, yet the causes of anemia in this population have been inadequately studied.

Methods: We conducted a caseâcontrol...

PERSPECTIVE: The Proxy War â SCHIP and the Government's Role in Health Care Reform

Thu, 28Â FebÂ 2008 00:00:00 -05:00

The conflagration over the reauthorization of the State Children's Health Insurance Program (SCHIP) offers a compelling example of Washington's current inability to address even seemingly uncontroversial matters such as improved...

EDITORIAL: A Hot Spot of Genetic Instability in Autism

Evan E. Eichler, Ph.D., and Andrew W. Zimmerman, M.D. — Thu, 14Â FebÂ 2008 00:00:00 -05:00

Sixty-five years after Leo Kanner first described autism,1 we are beginning to move beyond description of this clinically heterogeneous neurobehavioral syndrome toward a deeper understanding of its biologic complexity. In...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 5-2008 â An 18-Month-Old Girl with an Advanced Neck Contracture after a Burn

Thu, 14Â FebÂ 2008 00:00:00 -05:00

An 18-month-old girl was transferred from a hospital in China to the Shriners Hospital for Children in Boston for management of a life-threatening neck contracture.

The child had been well until...

ORIGINAL ARTICLE: A -Activating Mutation in a Patient with Central Precocious Puberty

Thu, 14Â FebÂ 2008 00:00:00 -05:00

Gonadotropin-dependent, or central, precocious puberty is caused by early maturation of the hypothalamicâpituitaryâgonadal axis. In girls, this condition is most often idiopathic. Recently, a G proteinâcoupled receptor, GPR54, and its...

ORIGINAL ARTICLE: Association between Microdeletion and Microduplication at 16p11.2 and Autism

Thu, 14Â FebÂ 2008 00:00:00 -05:00

Background: Autism spectrum disorder is a heritable developmental disorder in which chromosomal abnormalities are thought to play a role.

Methods: As a first component of a genomewide association study of families...

ORIGINAL ARTICLE: Phenotype and Course of HutchinsonâGilford Progeria Syndrome

Thu, 07Â FebÂ 2008 00:00:00 -05:00

Background: HutchinsonâGilford progeria syndrome is a rare, sporadic, autosomal dominant syndrome that involves premature aging, generally leading to death at approximately 13 years of age due to myocardial infarction or...

PERSPECTIVE: HutchinsonâGilford Progeria Syndrome, Aging, and the Nuclear Lamina

Thu, 07Â FebÂ 2008 00:00:00 -05:00

We are living in a time that will probably be remembered as a golden age of discovery in human genetics. Most of the recent excitement has focused on the identification...

CLINICAL PRACTICE: Croup

James D. Cherry, M.D., M.Sc. — Thu, 24Â JanÂ 2008 00:00:00 -05:00

Crouplike symptoms develop in a previously healthy 2-year-old girl at 11 p.m. She is seen in an emergency department 2 hours later with a barking cough and, when upset, inspiratory...

ORIGINAL ARTICLE: Etanercept Treatment for Children and Adolescents with Plaque Psoriasis

Thu, 17Â JanÂ 2008 00:00:00 -05:00

Background: Etanercept, a soluble tumor necrosis factor receptor, has been shown to lessen disease severity in adult patients with psoriasis. We assessed the efficacy and safety of etanercept in children...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 39-2007 â A 5-Month-Old Girl with Skin Lesions

Melissa M. Burnett, M.D., Mary S. Huang, M.D., and Rania M. Seliem, M.D. — Thu, 20Â DecÂ 2007 00:00:00 -05:00

Dr. Ruth Ann Vleugels (Dermatology): A 5-month-old girl was seen in the pediatric dermatology clinic because of a rash. She had been well until 3 months of age, when her...

ORIGINAL ARTICLE: Dexamethasone in Vietnamese Adolescents and Adults with Bacterial Meningitis

Thu, 13Â DecÂ 2007 00:00:00 -05:00

Background: It is uncertain whether all adults with bacterial meningitis benefit from treatment with adjunctive dexamethasone.

Methods: We conducted a randomized, double-blind, placebo-controlled trial of dexamethasone in 435 patients over the...

CLINICAL THERAPEUTICS: Cochlear Implants for Children with Severe-to-Profound Hearing Loss

Blake C. Papsin, M.D., and Karen A. Gordon, Ph.D. — Thu, 06Â DecÂ 2007 00:00:00 -05:00

A 4-month-old baby girl whose parents and 6-year-old brother have severe-to-profound sensorineural hearing loss is referred for consideration for cochlear implantation. Each of the baby's parents had previously received a...

SPECIAL ARTICLE: Adolescent Overweight and Future Adult Coronary Heart Disease

Thu, 06Â DecÂ 2007 00:00:00 -05:00

Background: The effect of adolescent overweight on future adult coronary heart disease (CHD) is not known.

Methods: We estimated the prevalence of obese 35-year-olds in 2020 on the basis of adolescent...

ORIGINAL ARTICLE: Childhood Body-Mass Index and the Risk of Coronary Heart Disease in Adulthood

Thu, 06Â DecÂ 2007 00:00:00 -05:00

Background: The worldwide epidemic of childhood obesity is progressing at an alarming rate. Risk factors for coronary heart disease (CHD) are already identifiable in overweight children. The severity of the...

PERSPECTIVE: Childhood Obesity â The Shape of Things to Come

Thu, 06Â DecÂ 2007 00:00:00 -05:00

Last week, I met with the G. family in the Optimal Weight for Life (OWL) clinic at my hospital. One of the parents was overweight, and the other was obese....

PERSPECTIVE: Over the Counter but No Longer under the Radar â Pediatric Cough and Cold Medications

Thu, 06Â DecÂ 2007 00:00:00 -05:00

In recent weeks, over-the-counter cough and cold medications for children have received unprecedented attention from regulators, physicians, the media, and parents. This scrutiny represents a long-overdue reassessment of products that...

EDITORIAL: Lung Transplantation in Cystic Fibrosis â Primum Non Nocere?

Julian Allen, M.D., and Gary Visner, D.O. — Thu, 22Â NovÂ 2007 00:00:00 -05:00

Cystic fibrosis is one of the most common autosomal recessive diseases. It causes intestinal, pancreatic, and progressive lung dysfunction, which is ultimately lethal. In 1989, the first specific cystic fibrosis...

ORIGINAL ARTICLE: Lung Transplantation and Survival in Children with Cystic Fibrosis

Thu, 22Â NovÂ 2007 00:00:00 -05:00

Background: The effects of lung transplantation on the survival and quality of life in children with cystic fibrosis are uncertain.

Methods: We used data from the U.S. Cystic Fibrosis Foundation Patient...

PERSPECTIVE: The Fate of SCHIP â Surrogate Marker for Health Care Ideology?

Thu, 22Â NovÂ 2007 00:00:00 -05:00

To appreciate the power of the U.S. presidency â even when its current occupant's approval rating is only 31% â one need look no further than the political brawl over...

REVIEW ARTICLE: Chronic Lung Disease after Premature Birth

Eugenio Baraldi, M.D., and Marco Filippone, M.D. — Thu, 08Â NovÂ 2007 00:00:00 -05:00

In 1967, Northway et al. first described a new chronic respiratory disease, bronchopulmonary dysplasia, that developed in premature infants exposed to mechanical ventilation and oxygen supplementation.1 Two decades later, the...

IMAGES IN CLINICAL MEDICINE: Tracheal Bronchus

Baris Bakir, M.D. and Ege Terzibasioglu, M.D. — Thu, 25Â OctÂ 2007 00:00:00 -05:00

A 9-year-old girl with recurrent respiratory infection presented for evaluation for possible active tuberculosis. Multislice computed tomography of the chest showed no evidence of active tuberculosis, but there was an...

EDITORIAL: The Quality of Children's Health Care Matters â Time to Pay Attention

James M. Perrin, M.D., and Charles J. Homer, M.D., M.P.H. — Thu, 11Â OctÂ 2007 00:00:00 -05:00

High-quality health care matters for all children â and is critically important for some. In many ways, health care for children serves the same function as health care for adults....

EDITORIAL: Of Attraction and Rejection â Asthma and the Microbial World

Erika von Mutius, M.D., M.Sc. — Thu, 11Â OctÂ 2007 00:00:00 -05:00

In the first half of the past century, it was thought that asthma was precipitated or prolonged by infection and that infection with several bacteria, including Streptococcus pneumoniae and Haemophilus...

SPECIAL ARTICLE: The Quality of Ambulatory Care Delivered to Children in the United States

Thu, 11Â OctÂ 2007 00:00:00 -05:00

Background: Little is known about the magnitude of deficits in the quality of care delivered to children, since comprehensive studies have been lacking.

Methods: We assessed the extent to which care...

ORIGINAL ARTICLE: Cardiomyopathy and Exercise Intolerance in Muscle Glycogen Storage Disease 0

Thu, 11Â OctÂ 2007 00:00:00 -05:00

Storage of glycogen is essential for glucose homeostasis and for energy supply during bursts of activity and sustained muscle work. We describe three siblings with profound muscle and heart glycogen...

ORIGINAL ARTICLE: Childhood Asthma after Bacterial Colonization of the Airway in Neonates

Thu, 11Â OctÂ 2007 00:00:00 -05:00

Background: Pathological features of the airway in young children with severe recurrent wheeze suggest an association between bacterial colonization and the initiating events of early asthma. We conducted a study...

ORIGINAL ARTICLE: Early Thimerosal Exposure and Neuropsychological Outcomes at 7 to 10 Years

Thu, 27Â SepÂ 2007 00:00:00 -05:00

Background: It has been hypothesized that early exposure to thimerosal, a mercury-containing preservative used in vaccines and immune globulin preparations, is associated with neuropsychological deficits in children.

Methods: We enrolled 1047...

PERSPECTIVE: Thimerosal and Vaccines â A Cautionary Tale

Thu, 27Â SepÂ 2007 00:00:00 -05:00

In 1997, Frank Pallone, a U.S. congressman from New Jersey, attached a simple, 133-word amendment to a Food and Drug Administration (FDA) reauthorization bill. This amendment gave the FDA 2...

PERSPECTIVE: Cases in Vaccine Court â Legal Battles over Vaccines and Autism

Thu, 27Â SepÂ 2007 00:00:00 -05:00

Do childhood vaccines cause autism? This scientific question has now become a legal one â perhaps inevitable in our society. Some families with autistic children are pursuing legal channels in...

CLINICAL PRACTICE: Gynecomastia

Glenn D. Braunstein, M.D. — Thu, 20Â SepÂ 2007 00:00:00 -05:00

During an evaluation for low back pain, a 67-year-old man is found to have gynecomastia on the right side that is nontender on palpation. Other than a body-mass index (the...

PERSPECTIVE: The Battle over SCHIP

Thu, 06Â SepÂ 2007 00:00:00 -05:00

Reauthorization of the State Children's Health Insurance Program (SCHIP), which was considered a routine matter until recently because of the program's success in expanding coverage to children of the working...

EDITORIAL: No Child Left Uncovered

Gregory D. Curfman, M.D., Stephen Morrissey, Ph.D., and Jeffrey M. Drazen, M.D. — Thu, 06Â SepÂ 2007 00:00:00 -05:00

In the grand tradition of bare-knuckle American politics, the reauthorization of the State Children's Health Insurance Program (SCHIP), which provides health coverage for low-income children, has become part of a...

EDITORIAL: Prophylactic Treatment for Prevention of Joint Disease in Hemophilia â Cost versus Benefit

Goris Roosendaal, M.D., Ph.D., and Floris Lafeber, Ph.D. — Thu, 09Â AugÂ 2007 00:00:00 -05:00

Patients with severe hemophilia number only around 400,000 worldwide, but their lifespan has increased because of improved treatment, and with that the prevalence of the disease is increasing. Despite the...

ORIGINAL ARTICLE: Prophylaxis versus Episodic Treatment to Prevent Joint Disease in Boys with Severe Hemophilia

Thu, 09Â AugÂ 2007 00:00:00 -05:00

Background: Effective ways to prevent arthropathy in severe hemophilia are unknown.

Methods: We randomly assigned young boys with severe hemophilia A to regular infusions of recombinant factor VIII (prophylaxis) or to...

ORIGINAL ARTICLE: Prevalence of Rheumatic Heart Disease Detected by Echocardiographic Screening

Thu, 02Â AugÂ 2007 00:00:00 -05:00

Background: Epidemiologic studies of the prevalence of rheumatic heart disease have used clinical screening with echocardiographic confirmation of suspected cases. We hypothesized that echocardiographic screening of all surveyed children would...

PERSPECTIVE: Rheumatic Heart Disease in Developing Countries

Thu, 02Â AugÂ 2007 00:00:00 -05:00

Only 30 or 40 years ago, rheumatic fever was a common topic in the Journal. A PubMed search for articles on rheumatic fever published between 1967 and 1976 returned 55...

EDITORIAL: Therapy for Bronchiolitis: When Some Become None

Caroline Breese Hall, M.D. — Thu, 26Â JulÂ 2007 00:00:00 -05:00

In past centuries, infant mortality in the first year of life was described as the greatest plague.1 Infants in the 21st century continue to be plagued by high rates of...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 23-2007 â A 9-Year-Old Boy with Bone Pain, Rash, and Gingival Hypertrophy

Thu, 26Â JulÂ 2007 00:00:00 -05:00

Dr. Laura Chapman (Pediatrics): A 9-year-old boy with autism was admitted to this hospital because of pain in the hip, refusal to walk, and the recent onset of a rash...

ORIGINAL ARTICLE: A Multicenter, Randomized, Controlled Trial of Dexamethasone for Bronchiolitis

Thu, 26Â JulÂ 2007 00:00:00 -05:00

Background: Bronchiolitis, the most common infection of the lower respiratory tract in infants, is a leading cause of hospitalization in childhood. Corticosteroids are commonly used to treat bronchiolitis, but evidence...

HEALTH POLICY REPORT: Insuring All Children â The New Political Imperative

John K. Iglehart — Thu, 05Â JulÂ 2007 00:00:00 -05:00

Efforts by the 110th Congress, which is commanded by Democrats eager to reduce the record number of people without health insurance, coupled with other federal and state initiatives,123 have thrust...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 20-2007 â An 11-Year-Old Boy with a Calcified Mass in the Nose

Thu, 28Â JunÂ 2007 00:00:00 -05:00

Dr. Margo McKenna Benoit (Otolaryngology): An 11-year-old boy was referred to a pediatric otolaryngologist at the Massachusetts Eye and Ear Infirmary because of headaches and a right nasal mass. Bifrontal...

ORIGINAL ARTICLE: Allogeneic Bone Marrow Transplantation in Mevalonic Aciduria

Thu, 28Â JunÂ 2007 00:00:00 -05:00

Mevalonic aciduria is a rare, inborn error of isoprene biosynthesis characterized by severe, periodic attacks of fever and inflammation, developmental delay, ataxia, and dysmorphic features. This autosomal recessive disease is...

PERSPECTIVE: Mevalonate Kinase Deficiency and Autoinflammatory Disorders

Thu, 28Â JunÂ 2007 00:00:00 -05:00

A deficiency of mevalonate kinase resulting in mevalonic aciduria was the first inherited defect in cholesterol and nonsterol isoprene biosynthesis to be recognized.1 Nine other enzyme deficiencies have since been...

ORIGINAL ARTICLE: Natural History of Multiple Sclerosis with Childhood Onset

Thu, 21Â JunÂ 2007 00:00:00 -05:00

Background: The course and prognosis of childhood-onset multiple sclerosis have not been well described.

Methods: We used data from 13 adult neurology departments affiliated with the European Database for Multiple Sclerosis...

ORIGINAL ARTICLE: Survival after Treatment with Phenylacetate and Benzoate for Urea-Cycle Disorders

Thu, 31Â MayÂ 2007 00:00:00 -05:00

Background: The combination of intravenous sodium phenylacetate and sodium benzoate has been shown to lower plasma ammonium levels and improve survival in small cohorts of patients with historically lethal urea-cycle...

EDITORIAL: Prematurity and the Legacy of Intrauterine Stress

Julie R. Ingelfinger, M.D. — Thu, 17Â MayÂ 2007 00:00:00 -05:00

In 1960, a neonate weighing 1000 g had a 95% risk of death; today, a neonate with the same birth weight has a 95% chance of surviving. This progress in...

ORIGINAL ARTICLE: Glucose Regulation in Young Adults with Very Low Birth Weight

Thu, 17Â MayÂ 2007 00:00:00 -05:00

Background: The association between small size at birth and impaired glucose regulation later in life is well established in persons born at term. Preterm birth with very low birth weight...

PERSPECTIVE: Introducing HPV Vaccine in Developing Countries â Key Challenges and Issues

Thu, 10Â MayÂ 2007 00:00:00 -05:00

More than any other cancer, cervical cancer reflects striking global health inequity. It is the second most common cancer among women worldwide, with about 493,000 new cases diagnosed annually (see...

PERSPECTIVE: Politics, Parents, and Prophylaxis â Mandating HPV Vaccination in the United States

Thu, 10Â MayÂ 2007 00:00:00 -05:00

Cancer prevention has fallen victim to the culture wars. Throughout the United States, state legislatures are scrambling to respond to the availability of Merck's human papillomavirus (HPV) vaccine, Gardasil, and...

ORIGINAL ARTICLE: Age at Surgery for Undescended Testis and Risk of Testicular Cancer

Thu, 03Â MayÂ 2007 00:00:00 -05:00

Background: Undescended testis, which is a risk factor for testicular cancer, is usually treated surgically, but whether the age at treatment has any effect on the risk is unclear. We...

ORIGINAL ARTICLE: A Lethal Defect of Mitochondrial and Peroxisomal Fission

Thu, 26Â AprÂ 2007 00:00:00 -05:00

We report on a newborn girl with microcephaly, abnormal brain development, optic atrophy and hypoplasia, persistent lactic acidemia, and a mildly elevated plasma concentration of very-long-chain fatty acids. We found...

EDITORIAL: Blood Transfusion â When Is More Really Less?

Howard L. Corwin, M.D., and Jeffrey L. Carson, M.D. — Thu, 19Â AprÂ 2007 00:00:00 -05:00

Blood transfusion has for years been considered to have obvious clinical benefits and to be a relatively low-risk procedure. Not until the early 1980s did transfusion practices begin to come...

ORIGINAL ARTICLE: Transfusion Strategies for Patients in Pediatric Intensive Care Units

Thu, 19Â AprÂ 2007 00:00:00 -05:00

Background: The optimal hemoglobin threshold for erythrocyte transfusions in critically ill children is unknown. We hypothesized that a restrictive transfusion strategy of using packed red cells that were leukocyte-reduced before...

PERSPECTIVE: Making Motherhood Safe in Developing Countries

Thu, 05Â AprÂ 2007 00:00:00 -05:00

This year marks the 20th anniversary of the Safe Motherhood Conference in Nairobi, an event that launched a global initiative to reduce maternal mortality in developing countries. At that time,...

ORIGINAL ARTICLE: Neurodevelopment and Cognition in Children after Enterovirus 71 Infection

Thu, 22Â MarÂ 2007 00:00:00 -05:00

Background: Enterovirus 71 is a common cause of hand, foot, and mouth disease and encephalitis in Asia and elsewhere. The long-term neurologic and psychiatric effects of this viral infection on...

ORIGINAL ARTICLE: Loss of Vaccine-Induced Immunity to Varicella over Time

Thu, 15Â MarÂ 2007 00:00:00 -05:00

Background: The introduction of universal varicella vaccination in 1995 has substantially reduced varicella-related morbidity and mortality in the United States. However, it remains unclear whether vaccine-induced immunity wanes over time,...

CLINICAL PRACTICE: Pediatric Strabismus

Sean P. Donahue, M.D., Ph.D. — Thu, 08Â MarÂ 2007 00:00:00 -05:00

A healthy 3-year-old boy presents with a 6-month history of strabismus in his left eye. The visible inward deviation of the eye began intermittently but is now constant. His visual...

IMAGES IN CLINICAL MEDICINE: Bone within Bone â Chronic Osteomyelitis

Avigdor Hevroni, M.D. and Benjamin Z. Koplewitz, M.D. — Thu, 22Â FebÂ 2007 00:00:00 -05:00

An afebrile 8-year-old Ethiopian girl presented with a limp. Two years earlier, she had had mild trauma followed by âbone infectionâ and had received short courses of oral antibiotics. Examination...

EDITORIAL: Inactivated and Live Attenuated Influenza Vaccines in Young Children â How Do They Compare?

Nancy J. Cox, Ph.D., and Carolyn Buxton Bridges, M.D. — Thu, 15Â FebÂ 2007 00:00:00 -05:00

Two quite different influenza vaccines are now licensed for use in the United States â a trivalent inactivated influenza vaccine that is administered intramuscularly and a live attenuated influenza vaccine...

ORIGINAL ARTICLE: Live Attenuated versus Inactivated Influenza Vaccine in Infants and Young Children

Thu, 15Â FebÂ 2007 00:00:00 -05:00

Background: Universal vaccination of children 6 to 59 months of age with trivalent inactivated influenza vaccine has recently been recommended by U.S. advisory bodies. To evaluate alternative vaccine approaches, we...

ORIGINAL ARTICLE: Randomized Trial of Pulsed Corticosteroid Therapy for Primary Treatment of Kawasaki Disease

Thu, 15Â FebÂ 2007 00:00:00 -05:00

Background: Treatment of acute Kawasaki disease with intravenous immune globulin and aspirin reduces the risk of coronary-artery abnormalities and systemic inflammation, but despite intravenous immune globulin therapy, coronary-artery abnormalities develop...

PERSPECTIVE: The Riddle of Kawasaki Disease

Thu, 15Â FebÂ 2007 00:00:00 -05:00

Two generations of clinicians and researchers have been stumped by the riddle of Kawasaki disease. How can an illness look like an infectious disease but not have a recoverable agent;...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 3-2007 â A 3-Year-Old Boy with Recurrent Episodes of Respiratory Insufficiency

Thu, 25Â JanÂ 2007 00:00:00 -05:00

A 3-year-old boy was admitted to the hospital because of recurrent episodes of respiratory insufficiency, pulmonary infiltrates, and anemia. He was treated with antibiotics and levalbuterol and budesonide by inhalation...

EDITORIAL: The End of an Era in Otitis Research

Stephen Berman, M.D. — Thu, 18Â JanÂ 2007 00:00:00 -05:00

Retrospective studies carried out in the 1950s, 1960s, and 1970s suggested an association between otitis media early in life and subsequent developmental impairments in children. Although these studies were not...

ORIGINAL ARTICLE: Tympanostomy Tubes and Developmental Outcomes at 9 to 11 Years of Age

Thu, 18Â JanÂ 2007 00:00:00 -05:00

Background: Developmental impairments in children have been attributed to persistent middle-ear effusion in their early years of life. Previously, we reported that among children younger than 3 years of age...

ORIGINAL ARTICLE: Clinical and Molecular Genetic Spectrum of Congenital Deficiency of the Leptin Receptor

Thu, 18Â JanÂ 2007 00:00:00 -05:00

Background: A single family has been described in which obesity results from a mutation in the leptin-receptor gene (LEPR), but the prevalence of such mutations in severe, early-onset obesity has...

IMAGES IN CLINICAL MEDICINE: Adults with Osteogenesis Imperfecta

Janet Reeder, M.S., P.A.-C. and Eric Orwoll, M.D. — Thu, 28Â DecÂ 2006 00:00:00 -05:00

Osteogenesis imperfecta is a heritable disorder caused by mutations in the gene for type I collagen. The Sillence classification of osteogenesis imperfecta (types I through IV) is based on clinical...

ORIGINAL ARTICLE: Deficiency of Cartilage-Associated Protein in Recessive Lethal Osteogenesis Imperfecta

Thu, 28Â DecÂ 2006 00:00:00 -05:00

Classic osteogenesis imperfecta, an autosomal dominant disorder associated with osteoporosis and bone fragility, is caused by mutations in the genes for type I collagen. A recessive form of the disorder...

PERSPECTIVE: The Antidepressant Quandary â Considering Suicide Risk When Treating Adolescent Depression

Thu, 28Â DecÂ 2006 00:00:00 -05:00

In March 2004, the Food and Drug Administration (FDA) issued a public health advisory regarding worsening depression and suicidal thoughts and behavior in patients treated with the newer antidepressant drugs...

PERSPECTIVE: Familial Pathways to Suicidal Behavior â Understanding and Preventing Suicide among Adolescents

Thu, 28Â DecÂ 2006 00:00:00 -05:00

A 16-year-old boy whose brother recently committed suicide is seen in the emergency room after slashing his wrists. He reports having felt severely depressed and hopeless since his brother died...

PERSPECTIVE: Uncovering an Epidemic â Screening for Mental Illness in Teens

Thu, 28Â DecÂ 2006 00:00:00 -05:00

Courtney, a 15-year-old from Portland, Oregon, always knew she was different from the other kids. âI had a sense that something was going on, but I was afraid to say...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 38-2006 â A 5-Year-Old Boy with Headache and Abdominal Pain

Thu, 14Â DecÂ 2006 00:00:00 -05:00

Dr. Kerstin Zanger (Pediatrics): A 5-year-old boy was admitted to the hospital because of headache, abdominal pain that had increased in severity and frequency, increasing abdominal girth, and a rash.

The...

ORIGINAL ARTICLE: Effectiveness of School-Based Influenza Vaccination

Thu, 14Â DecÂ 2006 00:00:00 -05:00

Background: Vaccination of children in school is one strategy to reduce the spread of influenza in households and communities.

Methods: We identified 11 demographically similar clusters of elementary schools in four...

PERSPECTIVE: The Eradication of Polio â Progress and Challenges

Thu, 14Â DecÂ 2006 00:00:00 -05:00

Six years after the original 2000 target date for the global eradication of polio, public health workers are encountering several stumbling blocks. Poliovirus circulation persists in countries where the virus...

PERSPECTIVE: The Ethics and Politics of Compulsory HPV Vaccination

Thu, 07Â DecÂ 2006 00:00:00 -05:00

On September 12, 2006, 3 months after the Food and Drug Administration licensed a vaccine against human papillomavirus (HPV), Michigan lawmakers became the first in the United States to propose...

REVIEW ARTICLE: The Asthma Epidemic

Waltraud Eder, M.D., Markus J. Ege, M.D., M.P.H., and Erika von Mutius, M.D. — Thu, 23Â NovÂ 2006 00:00:00 -05:00

The prevalence and incidence of asthma are very high in the Western world. There is widespread concern that the prevalence of asthma is still rising in developed countries, but the...

ORIGINAL ARTICLE: The Genotype of the Original Wiskott Phenotype

Thu, 26Â OctÂ 2006 00:00:00 -05:00

The WiskottâAldrich syndrome is an X-linked hereditary disorder associated with combined immunodeficiency, thrombocytopenia, small platelets, eczema, and increased susceptibility to autoimmune disorders and cancers. It is caused by mutations in...

PERSPECTIVE: Lessons from the WiskottâAldrich Syndrome

Thu, 26Â OctÂ 2006 00:00:00 -05:00

The WiskottâAldrich syndrome is a well-recognized triad of eczema, bleeding diathesis, and recurrent infections that occurs in boys. Although it is rare (with an estimated incidence of less than 1...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 32-2006 â A 3-Year-Old Girl with Fever after a Visit to Africa

Thu, 19Â OctÂ 2006 00:00:00 -05:00

Dr. Christine M. Cserti: A 3-year-old girl was admitted to this hospital because of fever and parasites in the peripheral blood.

Three weeks earlier, the patient and her 5-year-old brother and...

ORIGINAL ARTICLE: Reduced Lung Function at Birth and the Risk of Asthma at 10 Years of Age

Thu, 19Â OctÂ 2006 00:00:00 -05:00

Background: Reduced lung function in early infancy has been associated with later obstructive airway diseases. We assessed whether reduced lung function shortly after birth predicts asthma 10 years later.

Methods: We...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 31-2006 â A 15-Year-Old Girl with Severe Obesity

Thu, 12Â OctÂ 2006 00:00:00 -05:00

A 15 1/2-year-old girl was seen in the outpatient Weight Center of this hospital for the evaluation of severe obesity. She had had a normal gestation without complications and had...

SPECIAL ARTICLE: Chronic Health Conditions in Adult Survivors of Childhood Cancer

Thu, 12Â OctÂ 2006 00:00:00 -05:00

Background: Only a few small studies have assessed the long-term morbidity that follows the treatment of childhood cancer. We determined the incidence and severity of chronic health conditions in adult...

PERSPECTIVE: The Two-Edged Sword of Curing Childhood Cancer

Thu, 12Â OctÂ 2006 00:00:00 -05:00

In the June 3, 1948, issue of the Journal, Sydney Farber and his colleagues reported that they had successfully used aminopterin to induce temporary remission in children with lymphoid leukemia....

REVIEW ARTICLE: The Tuberous Sclerosis Complex

Thu, 28Â SepÂ 2006 00:00:00 -05:00

The tuberous sclerosis complex (TSC), a multisystem, autosomal dominant disorder affecting children and adults, results from mutations in one of two genes, TSC1 (encoding hamartin) or TSC2 (encoding tuberin) (see...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 27-2006 â A 17-Year-Old Boy with Fever and Lesions in the Liver and Spleen

Stephen I. Pelton, M.D., John Y. Kim, M.D., and Richard L. Kradin, M.D. — Thu, 31Â AugÂ 2006 00:00:00 -05:00

Dr. Melissa Sue Lee (Pediatrics): A 17-year-old boy was admitted to this hospital because of fever and nodules in the liver and spleen. The patient had been well until two...

IMAGES IN CLINICAL MEDICINE: Salt-and-Pepper Retinopathy of Rubella

Rahul N. Khurana, M.D. and Srinivas R. Sadda, M.D. — Thu, 03Â AugÂ 2006 00:00:00 -05:00

A 39-year-old woman presented at a clinic for a second opinion regarding a diagnosis of age-related macular degeneration, which had been noted on routine ophthalmologic examination. She reported having no...

EDITORIAL: Air Pollution and Children â An Unhealthy Mix

W. James Gauderman, Ph.D. — Thu, 06Â JulÂ 2006 00:00:00 -05:00

Residents in urban areas may be worried about the air they breathe. In recent reports, air pollution has been linked to increased mortality, an increased risk of asthma, and decreased...

ORIGINAL ARTICLE: The Underrecognized Burden of Influenza in Young Children

Thu, 06Â JulÂ 2006 00:00:00 -05:00

Background: The disease burden of influenza infection among children is not well established. We conducted a population-based surveillance of medical visits associated with laboratory-confirmed influenza.

Methods: Eligible children were younger than...

ORIGINAL ARTICLE: Carbon in Airway Macrophages and Lung Function in Children

Thu, 06Â JulÂ 2006 00:00:00 -05:00

Background: Epidemiologic studies indirectly suggest that the inhalation of carbonaceous particulate matter impairs lung function in children. Using the carbon content of airway macrophages as a marker of individual exposure...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 19-2006 â A 22-Month-Old Boy with the Rapid Growth of Subcutaneous Nodules

J. Patrick Whelan, M.D., Ph.D., and Artur Zembowicz, M.D., Ph.D. — Thu, 22Â JunÂ 2006 00:00:00 -05:00

Dr. Richard Chou (Rheumatology): A 22-month-old boy was seen in the pediatric rheumatology clinic because of enlarging subcutaneous nodules on the head, legs, and right hand. The patient had been...

CLINICAL THERAPEUTICS: Omalizumab for Asthma

Robert C. Strunk, M.D., and Gordon R. Bloomberg, M.D. — Thu, 22Â JunÂ 2006 00:00:00 -05:00

A 16-year-old boy with a 15-year history of asthma from the age of one year and two visits to the emergency department in the past year presents for evaluation. He...

CLINICAL PRACTICE: Idiopathic Short Stature

Mary M. Lee, M.D. — Thu, 15Â JunÂ 2006 00:00:00 -05:00

A healthy, active 12-year-old boy is in the first percentile for height (133.0 cm [52.4 in.]; 2.25 SD below the mean for age and sex) and in the third percentile...

PERSPECTIVE: Food Marketing and Childhood Obesity â A Matter of Policy

Thu, 15Â JunÂ 2006 00:00:00 -05:00

Everyone knows that American children are becoming fatter, but not everyone agrees on the cause. Many of today's children routinely consume more calories than they expend in physical activity, but...

ORIGINAL ARTICLE: Outcomes of In-Hospital Ventricular Fibrillation in Children

Thu, 01Â JunÂ 2006 00:00:00 -05:00

Background: Ventricular fibrillation and ventricular tachycardia are less common causes of cardiac arrest in children than in adults. These tachyarrhythmias can also begin during cardiopulmonary resuscitation (CPR), presumably as reperfusion...

REVIEW ARTICLE: Newborn Hearing Screening â A Silent Revolution

Cynthia C. Morton, Ph.D., and Walter E. Nance, M.D., Ph.D. — Thu, 18Â MayÂ 2006 00:00:00 -05:00

Hearing loss is an etiologically heterogeneous trait with many known genetic and environmental causes.1 Historically, some environmental causes of hearing loss, such as rubella embryopathy, have been epidemic in nature,...

ORIGINAL ARTICLE: Language Ability after Early Detection of Permanent Childhood Hearing Impairment

Thu, 18Â MayÂ 2006 00:00:00 -05:00

Background: Children with bilateral permanent hearing impairment often have impaired language and speech abilities. However, the effects of universal newborn screening for permanent bilateral childhood hearing impairment and the effects...

EDITORIAL: Inhaled Corticosteroids for Young Children with Wheezing

Diane R. Gold, M.D., M.P.H., and Anne L. Fuhlbrigge, M.D. — Thu, 11Â MayÂ 2006 00:00:00 -05:00

In the Childhood Asthma Management Program study of school-aged children with established mild-to-moderate persistent asthma, twice-daily therapy with inhaled corticosteroids controlled symptoms of asthma but slightly reduced height growth and...

ORIGINAL ARTICLE: Intermittent Inhaled Corticosteroids in Infants with Episodic Wheezing

Thu, 11Â MayÂ 2006 00:00:00 -05:00

Background: We hypothesized that asthma is preceded by a stage of recurrent episodes of wheezing during the first years of life and that inhaled corticosteroid therapy during symptomatic episodes in...

ORIGINAL ARTICLE: Long-Term Inhaled Corticosteroids in Preschool Children at High Risk for Asthma

Thu, 11Â MayÂ 2006 00:00:00 -05:00

Background: It is unknown whether inhaled corticosteroids can modify the subsequent development of asthma in preschool children at high risk for asthma.

Methods: We randomly assigned 285 participants two or three...

IMAGES IN CLINICAL MEDICINE: Cat Scratch Disease Lymphadenopathy

Tal Eidlitz-Markus, M.D. and Avraham Zeharia, M.D. — Thu, 27Â AprÂ 2006 00:00:00 -05:00

An otherwise healthy, 7 1/2-year-old girl had a two-week history of a swelling behind her left ear. She had no systemic illness and noted no improvement with a course of...

ORIGINAL ARTICLE: Oral Ondansetron for Gastroenteritis in a Pediatric Emergency Department

Thu, 20Â AprÂ 2006 00:00:00 -05:00

Background: Vomiting limits the success of oral rehydration in children with gastroenteritis. We conducted a double-blind trial to determine whether a single oral dose of ondansetron, an antiemetic, would improve...

EDITORIAL: Pneumococcal Vaccine â Direct and Indirect (âHerdâ) Effects

Daniel M. Musher, M.D. — Thu, 06Â AprÂ 2006 00:00:00 -05:00

A nationwide vaccination program may have direct or indirect health effects that may be anticipated or unanticipated; although these effects are, of course, intended to be beneficial, some potentially adverse...

ORIGINAL ARTICLE: Effect of Introduction of the Pneumococcal Conjugate Vaccine on Drug-Resistant

Thu, 06Â AprÂ 2006 00:00:00 -05:00

Background: Five of seven serotypes in the pneumococcal conjugate vaccine, introduced for infants in the United States in 2000, are responsible for most penicillin-resistant infections. We examined the effect of...

PERSPECTIVE: The Changing Face of Teenage Drug Abuse â The Trend toward Prescription Drugs

Thu, 06Â AprÂ 2006 00:00:00 -05:00

When Eric, an 18-year-old who lives in San Francisco, wants to get some Vicodin (hydrocodoneâacetaminophen), it's a simple matter. âI can get prescription drugs from different places and don't ever...

PERSPECTIVE: ADHD Drugs and Cardiovascular Risk

Thu, 06Â AprÂ 2006 00:00:00 -05:00

On February 9, 2006, the Drug Safety and Risk Management Advisory Committee of the Food and Drug Administration (FDA) voted by a narrow margin â eight to seven â to...

ORIGINAL ARTICLE: Recessive Symptomatic Focal Epilepsy and Mutant Contactin-Associated Protein-like 2

Thu, 30Â MarÂ 2006 00:00:00 -05:00

Contactin-associated protein-like 2 (CASPR2) is encoded by CNTNAP2 and clusters voltage-gated potassium channels (Kv1.1) at the nodes of Ranvier. We report a homozygous mutation of CNTNAP2 in Old Order Amish...

REVIEW ARTICLE: Pregnancy in Recipients of Solid Organs â Effects on Mother and Child

Dianne B. McKay, M.D., and Michelle A. Josephson, M.D. — Thu, 23Â MarÂ 2006 00:00:00 -05:00

The first child born to a transplant recipient turned 48 years old on March 10, 2006.1 When the field of transplantation was first developing, physicians worried about the teratogenicity of...

SPECIAL ARTICLE: Changes in Abortions and Births and the Texas Parental Notification Law

Theodore Joyce, Ph.D., Robert Kaestner, Ph.D., and Silvie Colman, B.B.A. — Thu, 09Â MarÂ 2006 00:00:00 -05:00

Background: On January 1, 2000, Texas began enforcement of a law that requires physicians to notify a parent of a minor child seeking an abortion at least 48 hours before...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 5-2006 â An 11-Year-Old Girl with Loss of Vision in the Right Eye

Thu, 16Â FebÂ 2006 00:00:00 -05:00

Dr. Torsten Wiegand (Massachusetts Eye and Ear Infirmary): An 11-year-old girl was evaluated at our institution because of a loss of vision in the right eye.

One month earlier, she had...

IMAGES IN CLINICAL MEDICINE: Koplik's Spots

Lawrence M. Tierney, Jr., M.D. and Kevin C. Wang, M.D., Ph.D. — Thu, 16Â FebÂ 2006 00:00:00 -05:00

A 16-year-old, previously healthy boy came to the hospital with a history of fever for several days, nonproductive cough, and coryza. On examination he appeared to be acutely ill; his...

ORIGINAL ARTICLE: Human Botulism Immune Globulin for the Treatment of Infant Botulism

Thu, 02Â FebÂ 2006 00:00:00 -05:00

Background: We created the orphan drug Human Botulism Immune Globulin Intravenous (Human) (BIG-IV), which neutralizes botulinum toxin, and evaluated its safety and efficacy in treating infant botulism, the intestinal-toxemia form...

PERSPECTIVE: Noma â The Ulcer of Extreme Poverty

Thu, 19Â JanÂ 2006 00:00:00 -05:00

In 2001, my research team was visiting a farming community in northwestern Nigeria, where word quickly spread to neighboring villages that foreign doctors were examining the mouths of sick children....

EDITORIAL: The Promise of New Rotavirus Vaccines

Roger I. Glass, M.D., Ph.D. and Umesh D. Parashar, M.B., B.S., M.P.H. — Thu, 05Â JanÂ 2006 00:00:00 -05:00

Rotavirus disease kills approximately half a million children annually in developing countries and accounts for one third of hospitalizations for diarrhea worldwide.1 In 1999, global efforts to control the tremendous...

ORIGINAL ARTICLE: Safety and Efficacy of a Pentavalent HumanâBovine (WC3) Reassortant Rotavirus Vaccine

Thu, 05Â JanÂ 2006 00:00:00 -05:00

Background: Rotavirus is a leading cause of childhood gastroenteritis and death worldwide.

Methods: We studied healthy infants approximately 6 to 12 weeks old who were randomly assigned to receive three oral...

ORIGINAL ARTICLE: Safety and Efficacy of an Attenuated Vaccine against Severe Rotavirus Gastroenteritis

Thu, 05Â JanÂ 2006 00:00:00 -05:00

Background: The safety and efficacy of an attenuated G1P[8] human rotavirus (HRV) vaccine were tested in a randomized, double-blind, phase 3 trial.

Methods: We studied 63,225 healthy infants from 11 Latin...

ORIGINAL ARTICLE: Discontinuing Prophylactic Transfusions Used to Prevent Stroke in Sickle Cell Disease

Thu, 29Â DecÂ 2005 00:00:00 -05:00

Background: Prophylactic transfusion prevents strokes in children with sickle cell anemia who have abnormalities on transcranial Doppler ultrasonographic examination. However, it is not known how long transfusion should be continued...

PERSPECTIVE: Preventing Stroke in Sickle Cell Anemia

Thu, 29Â DecÂ 2005 00:00:00 -05:00

In the late 1930s, William Bosworth Castle and his colleague Thomas Hale Ham were studying blood samples obtained from patients with sickle cell anemia and found that the viscosity of...

ORIGINAL ARTICLE: Influenza-Associated Deaths among Children in the United States, 2003â2004

Thu, 15Â DecÂ 2005 00:00:00 -05:00

Background: Although influenza is common among children, pediatric mortality related to laboratory-confirmed influenza has not been assessed nationally.

Methods: During the 2003â2004 influenza season, we requested that state health departments report...

PERSPECTIVE: Influenza â Interpandemic as Well as Pandemic Disease

Thu, 15Â DecÂ 2005 00:00:00 -05:00

Influenza occurs in both pandemic and interpandemic forms. Fortunately, pandemics, defined as worldwide outbreaks of severe disease, occur infrequently. Interpandemic influenza, although less extensive in its impact, occurs virtually every...

ORIGINAL ARTICLE: Chromosome 1p and 11q Deletions and Outcome in Neuroblastoma

Thu, 24Â NovÂ 2005 00:00:00 -05:00

Background: Neuroblastoma is a childhood cancer with considerable morbidity and mortality. Tumor-derived biomarkers may improve risk stratification.

Methods: We screened 915 samples of neuroblastoma for loss of heterozygosity (LOH) at chromosome...

PERSPECTIVE: Neuroblastoma â from Genetic Profiles to Clinical Challenge

Thu, 24Â NovÂ 2005 00:00:00 -05:00

Neuroblastoma stands out among childhood cancers because of its relative frequency, enigmatic natural history, intriguing biologic features, and daunting therapeutic challenges. Much that is novel and on the cutting edge...

PERSPECTIVE: Betting on a Malaria Vaccine

Susan Okie, M.D. — Thu, 03Â NovÂ 2005 00:00:00 -05:00

Sometime within the next two years, clinical researchers are expected to begin inoculating at least 2000 African infants in the largest trial ever undertaken of an experimental vaccine for malaria....

EDITORIAL: Developmental Origins of Health and Disease

Matthew W. Gillman, M.D. — Thu, 27Â OctÂ 2005 00:00:00 -05:00

At first glance, it may seem implausible that your mother's exposure to stress or toxins while she was pregnant with you, how she fed you when you were an infant,...

ORIGINAL ARTICLE: Trajectories of Growth among Children Who Have Coronary Events as Adults

Thu, 27Â OctÂ 2005 00:00:00 -05:00

Background: Low birth weight is a risk factor for coronary heart disease. It is uncertain how postnatal growth affects disease risk.

Methods: We studied 8760 people born in Helsinki from 1934...

PERSPECTIVE: On Genes, Speech, and Language

Simon E. Fisher, D.Phil. — Thu, 20Â OctÂ 2005 00:00:00 -05:00

Learning to talk is one of the most important milestones in human development, but we still have only a limited understanding of the way in which the process occurs. It...

CLINICAL PRACTICE: Anorexia Nervosa

Joel Yager, M.D., and Arnold E. Andersen, M.D. — Thu, 06Â OctÂ 2005 00:00:00 -05:00

A 17-year-old girl is taken to her physician by worried parents. Never overweight, in the past six months she became determined to reduce from her baseline weight of 59.1 kg...

EDITORIAL: Prophylactic Thyroidectomy in MEN-2A â A Stitch in Time?

Francis D. Moore, M.D., and Robert G. Dluhy, M.D. — Thu, 15Â SepÂ 2005 00:00:00 -05:00

Multiple endocrine neoplasia (MEN) type 2A (MEN-2A) and type 2B (MEN-2B) illustrate the remarkable pace of knowledge that has been acquired about a group of heritable disorders. In 1961, Sipple...

ORIGINAL ARTICLE: Prophylactic Thyroidectomy in Multiple Endocrine Neoplasia Type 2A

Thu, 15Â SepÂ 2005 00:00:00 -05:00

Background: Medullary thyroid carcinoma is the most common cause of death in patients with multiple endocrine neoplasia (MEN) type 2A (MEN-2A) or type 2B or familial medullary thyroid carcinoma. We...

EDITORIAL: Intravenous Fluids â Getting the Balance Right

Thu, 01Â SepÂ 2005 00:00:00 -05:00

Acute infections are a major cause of illness and death in children worldwide. In Africa, 50 percent of in-hospital deaths of children occur within 24 hours of admission, and shock...

ORIGINAL ARTICLE: Recombinant Osteoprotegerin for Juvenile Paget's Disease

Thu, 01Â SepÂ 2005 00:00:00 -05:00

Juvenile Paget's disease, a genetic bone disease characterized by accelerated bone turnover, results from inactivating mutations in the gene encoding osteoprotegerin â a key regulator of osteoclastogenesis. The effects of...

ORIGINAL ARTICLE: Comparison of Three Fluid Solutions for Resuscitation in Dengue Shock Syndrome

Thu, 01Â SepÂ 2005 00:00:00 -05:00

Background: Dengue shock syndrome is characterized by severe vascular leakage and disordered hemostasis and progresses to death in 1 to 5 percent of cases. Although volume replacement is recognized as...

ORIGINAL ARTICLE: Developmental Outcomes after Early or Delayed Insertion of Tympanostomy Tubes

Thu, 11Â AugÂ 2005 00:00:00 -05:00

Background: To prevent later developmental impairments, myringotomy with the insertion of tympanostomy tubes has often been undertaken in young children who have persistent otitis media with effusion. We previously reported...

SPECIAL ARTICLE: Children in the United States with Discontinuous Health Insurance Coverage

Lynn M. Olson, Ph.D., Suk-fong S. Tang, Ph.D., and Paul W. Newacheck, Dr.P.H. — Thu, 28Â JulÂ 2005 00:00:00 -05:00

Background: Estimates of the number of uninsured people in the United States usually exclude those with discontinuous coverage. The effects of gaps in insurance coverage for children on access to...

IMAGES IN CLINICAL MEDICINE: Small-Bowel Prolapse through a Persistent Omphalomesenteric Duct

Daniel F. Saad, M.D. and Kenneth W. Gow, M.D. — Thu, 07Â JulÂ 2005 00:00:00 -05:00

A 23-day-old term male infant presented to the emergency room with acute small-bowel evisceration through the umbilicus (Panel A). His parents had noted periumbilical erythema and mucus-containing umbilical drainage before...

ORIGINAL ARTICLE: Ovarian Transplantation between Monozygotic Twins Discordant for Premature Ovarian Failure

Thu, 07Â JulÂ 2005 00:00:00 -05:00

Monozygotic 24-year-old twins presented with discordant ovarian function. One had had premature ovarian failure at the age of 14 years, whereas her sister had normal ovaries and three naturally conceived...

EDITORIAL: Gestational Diabetes Mellitus â Time to Treat

Michael F. Greene, M.D., and Caren G. Solomon, M.D., M.P.H. — Thu, 16Â JunÂ 2005 00:00:00 -05:00

Gestational diabetes mellitus, broadly defined as carbohydrate intolerance beginning or first recognized during pregnancy,12 was originally described decades ago and has since been the subject of extensive research. Yet the...

ORIGINAL ARTICLE: Survival after Treatment of Rabies with Induction of Coma

Thu, 16Â JunÂ 2005 00:00:00 -05:00

We report the survival of a 15-year-old girl in whom clinical rabies developed one month after she was bitten by a bat. Treatment included induction of coma while a native...

ORIGINAL ARTICLE: Effect of Treatment of Gestational Diabetes Mellitus on Pregnancy Outcomes

Thu, 16Â JunÂ 2005 00:00:00 -05:00

Background: We conducted a randomized clinical trial to determine whether treatment of women with gestational diabetes mellitus reduced the risk of perinatal complications.

Methods: We randomly assigned women between 24 and...

CLINICAL PRACTICE: Atopic Dermatitis

Hywel C. Williams, Ph.D. — Thu, 02Â JunÂ 2005 00:00:00 -05:00

A 10-year-old girl with atopic dermatitis reports itching that has recently become relentless, resulting in sleep loss. Her mother has been reluctant to treat the girl with topical corticosteroids, because...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 16-2005 â A Nine-Year-Old Girl with Headaches and Hypertension

Thu, 26Â MayÂ 2005 00:00:00 -05:00

A nine-year-old girl was admitted to this hospital because of headaches, enuresis, recent visual changes, and vomiting.

Intermittent frontal headaches began 18 months before admission, occurring most often during physical activity,...

PERSPECTIVE: Saving the Children â Improving Childhood Cancer Treatment in Developing Countries

Raul C. Ribeiro, M.D., and Ching-Hon Pui, M.D. — Thu, 26Â MayÂ 2005 00:00:00 -05:00

Unprecedented gains have been made in the cure rates for childhood cancer during the past four decades. This progress reflects steady improvement in treatment protocols, a multidisciplinary approach to patient...

IMAGES IN CLINICAL MEDICINE: Asphyxia Due to an Inhaled Foreign Body

Neda Zarrin-Khameh, M.D., M.P.H. and Robert E. Lyon, D.O. — Thu, 19Â MayÂ 2005 00:00:00 -05:00

A three-year-old boy was playing with a small plastic ball while riding in the backseat of a motor vehicle driven by his father. The father heard a gasp, saw that...

CLINICAL PRACTICE: Overweight Children and Adolescents

William H. Dietz, M.D., Ph.D., and Thomas N. Robinson, M.D., M.P.H. — Thu, 19Â MayÂ 2005 00:00:00 -05:00

A seven-year-old girl is 130 cm tall (51 in., the 90th percentile for girls of the same age) and weighs 34.6 kg (76 lb, above the 95th percentile), with a...

ORIGINAL ARTICLE: Transplantation of Umbilical-Cord Blood in Babies with Infantile Krabbe's Disease

Thu, 19Â MayÂ 2005 00:00:00 -05:00

Background: Infantile Krabbe's disease produces progressive neurologic deterioration and death in early childhood. We hypothesized that transplantation of umbilical-cord blood from unrelated donors before the development of symptoms would favorably...

EDITORIAL: The Fate of Circulating Osteoblasts

Ernesto Canalis, M.D. — Thu, 12Â MayÂ 2005 00:00:00 -05:00

Bone remodeling is a temporally regulated process resulting in the coordinated resorption and formation of skeletal tissue carried out in basic multicellular units throughout life.1 Signals determining the fate, function,...

REVIEW ARTICLE: Cystic Fibrosis

Steven M. Rowe, M.D., Stacey Miller, B.S., and Eric J. Sorscher, M.D. — Thu, 12Â MayÂ 2005 00:00:00 -05:00

Advances in the care of patients with cystic fibrosis have improved survival, and as a result, patients with the disease now often live beyond the third decade.1 In addition, developments...

ORIGINAL ARTICLE: Autoantibodies to Folate Receptors in the Cerebral Folate Deficiency Syndrome

Thu, 12Â MayÂ 2005 00:00:00 -05:00

In infantile-onset cerebral folate deficiency, 5-methyltetrahydrofolate (5MTHF) levels in the cerebrospinal fluid are low, but folate levels in the serum and erythrocytes are normal. We examined serum specimens from 28...

ORIGINAL ARTICLE: Circulating Osteoblast-Lineage Cells in Humans

Thu, 12Â MayÂ 2005 00:00:00 -05:00

Background: Although current evidence suggests that only a minuscule number of osteoblast-lineage cells are present in peripheral blood, we hypothesized that such cells circulate but that their concentration has been...

PERSPECTIVE: Autoimmune Folate Deficiency and the Rise and Fall of âHorror Autotoxicusâ

Robert S. Schwartz, M.D. — Thu, 12Â MayÂ 2005 00:00:00 -05:00

In 1904, a little more than a century ago, Julius Donath, a senior physician working in the Vienna Merchants' Hospital, and Karl Landsteiner, the chair of the pathology department at...

ORIGINAL ARTICLE: Leflunomide or Methotrexate for Juvenile Rheumatoid Arthritis

Thu, 21Â AprÂ 2005 00:00:00 -05:00

Background: We compared the safety and efficacy of leflunomide with that of methotrexate in the treatment of polyarticular juvenile rheumatoid arthritis in a multinational, randomized, controlled trial.

Methods: Patients 3 to...

ORIGINAL ARTICLE: Methicillin-Resistant Disease in Three Communities

Thu, 07Â AprÂ 2005 00:00:00 -05:00

Background: Methicillin-resistant Staphylococcus aureus (MRSA) infection has emerged in patients who do not have the established risk factors. The national burden and clinical effect of this novel presentation of MRSA...

PERSPECTIVE: The Cutter Incident, 50 Years Later

Paul A. Offit, M.D. — Thu, 07Â AprÂ 2005 00:00:00 -05:00

On April 12, 1955, Jonas Salk's polio vaccine â made by inactivating poliovirus with formaldehyde â was declared to be safe and effective. The trial of Salk's vaccine had included...

PERSPECTIVE: April 12, 1955 â Tommy Francis and the Salk Vaccine

Howard Markel, M.D., Ph.D. — Thu, 07Â AprÂ 2005 00:00:00 -05:00

April 12, 1955, was supposed to be Tommy Francis's day. At 10:20 a.m., the distinguished epidemiologist was scheduled to conduct an international press conference in Rackham Auditorium at the University...

CLINICAL PRACTICE: Pertussis â Not Just for Kids

Erik L. Hewlett, M.D., and Kathryn M. Edwards, M.D. — Thu, 24Â MarÂ 2005 00:00:00 -05:00

Six weeks ago, a 45-year-old woman noticed a scratchy feeling in her throat that has now progressed to more than 20 episodes of severe, spasmodic coughing per day. Her coughing...

ORIGINAL ARTICLE: Hospitalization for Mental Illness among Parents after the Death of a Child

Thu, 24Â MarÂ 2005 00:00:00 -05:00

Background: The loss of a child is considered one of the most stressful events in the life of a parent. We hypothesized that parental bereavement increases the risk of hospital...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 8-2005 â A 10-Year-Old Boy with Pain in the Right Thigh

Mark C. Gebhardt, M.D., Daniel I. Rosenthal, M.D., and Paula M. Arnell, M.D. — Thu, 17Â MarÂ 2005 00:00:00 -05:00

A 10-year-old boy was seen in the clinic because of pain in the upper portion of his right thigh. The pain had begun six months earlier and was made worse...

ORIGINAL ARTICLE: Treatment of Early Childhood Medulloblastoma by Postoperative Chemotherapy Alone

Thu, 10Â MarÂ 2005 00:00:00 -05:00

Background: The prognosis for young children with medulloblastoma is poor, and survivors are at high risk for cognitive deficits. We conducted a trial of the treatment of this brain tumor...

EDITORIAL: Chemotherapy for Brain Tumors â A New Beginning

Lisa M. DeAngelis, M.D. — Thu, 10Â MarÂ 2005 00:00:00 -05:00

Primary brain tumors often resist treatment; the most common brain tumor in adults, glioblastoma, kills patients within a median of a year after diagnosis, even with aggressive surgical resection and...

ORIGINAL ARTICLE: A Population-Based Study of Primary Human Herpesvirus 6 Infection

Thu, 24Â FebÂ 2005 00:00:00 -05:00

Background: Serologic studies indicate that human herpesvirus 6 (HHV-6) infects 90 percent of children by two years of age. Little is known about the acquisition, virologic course, and clinical manifestations...

ORIGINAL ARTICLE: Fatal Avian Influenza A (H5N1) in a Child Presenting with Diarrhea Followed by Coma

Thu, 17Â FebÂ 2005 00:00:00 -05:00

In southern Vietnam, a four-year-old boy presented with severe diarrhea, followed by seizures, coma, and death. The cerebrospinal fluid contained 1 white cell per cubic millimeter, normal glucose levels, and...

ORIGINAL ARTICLE: Decline in Mortality Due to Varicella after Implementation of Varicella Vaccination in the United States

Thu, 03Â FebÂ 2005 00:00:00 -05:00

Background: Varicella disease has been preventable in the United States since 1995. Starting in 1999, active and passive surveillance data showed sharp decreases in varicella disease. We reviewed national death...

PERSPECTIVE: Varicella Vaccine and Infection with VaricellaâZoster Virus

Marietta VÃ¡zquez, M.D., and Eugene D. Shapiro, M.D. — Thu, 03Â FebÂ 2005 00:00:00 -05:00

Varicellaâzoster virus is the cause of both varicella (chickenpox) and herpes zoster (shingles). A live attenuated varicella vaccine was developed in Japan in 1974, and in 1995 it was approved...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 3-2005 â A 14-Year-Old Boy with Recent Slowing of Growth and Delayed Puberty

Thu, 27Â JanÂ 2005 00:00:00 -05:00

A 14-year-old boy was evaluated in the pediatric endocrinology clinic because of decreased endurance, slow growth, and delayed onset of puberty.

Ten weeks before his presentation at the clinic, he saw...

CLINICAL PRACTICE: Attention DeficitâHyperactivity Disorder

Marsha D. Rappley, M.D. — Thu, 13Â JanÂ 2005 00:00:00 -05:00

A mother brings in her eight-year-old son for evaluation after he is suspended from riding the school bus for jumping out of his seat, teasing other children, and not following...

ORIGINAL ARTICLE: Neurologic and Developmental Disability at Six Years of Age after Extremely Preterm Birth

Thu, 06Â JanÂ 2005 00:00:00 -05:00

Background: Birth before 26 weeks of gestation is associated with a high prevalence of neurologic and developmental disabilities in the infant during the first two years of life.

Methods: We studied...

EDITORIAL: Bacterial Infections â A Major Cause of Death among Children in Africa

E. Kim Mulholland, M.D., and Richard A. Adegbola, Ph.D. — Thu, 06Â JanÂ 2005 00:00:00 -05:00

For the past 25 years, since the United Nations Children's Fund (UNICEF) has been publishing estimates of mortality among children worldwide, the international medical community has been aware of the...

EDITORIAL: Extreme Prematurity â The Continuing Dilemma

Betty R. Vohr, M.D., and Marilee Allen, M.D. — Thu, 06Â JanÂ 2005 00:00:00 -05:00

The tremendous advances in perinatal and neonatal care that were made beginning in the 1970s and through the 1990s have contributed to the survival of infants as immature as 22...

PERSPECTIVE: Shifting Sands

Perri Klass, M.D. — Thu, 06Â JanÂ 2005 00:00:00 -05:00

For drama's sake, this story should start, âIt was the coldest night of the year in Boston.â Poetic license aside, it was a frigid January night, and I decided to...

ORIGINAL ARTICLE: Bacteremia among Children Admitted to a Rural Hospital in Kenya

Thu, 06Â JanÂ 2005 00:00:00 -05:00

Background: There are few epidemiologic data on invasive bacterial infections among children in sub-Saharan Africa. We studied every acute pediatric admission to a rural district hospital in Kenya to examine...

REVIEW ARTICLE: Osteopetrosis

Jakub Tolar, M.D., Ph.D., Steven L. Teitelbaum, M.D., and Paul J. Orchard, M.D. — Thu, 30Â DecÂ 2004 00:00:00 -05:00

Bone is a dynamic tissue in which osteoblasts synthesize bone matrix while osteoclasts resorb bone. Therefore, bone density is dependent on the relative function of these two types of cells....

PERSPECTIVE: America's Gulag Archipelago

Norman Fost, M.D., M.P.H. — Thu, 02Â DecÂ 2004 00:00:00 -05:00

If you were to meet Fred Boyce today, you might think he was a normal person, aside from the fact that he is better looking and more charming than most...

IMAGES IN CLINICAL MEDICINE: Double Aortic Arch Diagnosed by Magnetic Resonance Imaging

Joachim Lotz, M.D. and Paolo Macchiarini, M.D. — Thu, 25Â NovÂ 2004 00:00:00 -05:00

A six-month-old boy who had recurrent respiratory difficulties was admitted to the hospital with acute respiratory failure that required mechanical ventilation. Echocardiography suggested the presence of a double aortic arch....

EDITORIAL: Prematurity â A Window of Opportunity?

Mark A. Sperling, M.D. — Thu, 18Â NovÂ 2004 00:00:00 -05:00

Infants born with evidence of restricted fetal growth â those who are small for gestational age â appear to be at increased risk for metabolic disorders (type 2 diabetes mellitus...

ORIGINAL ARTICLE: Premature Birth and Later Insulin Resistance

Thu, 18Â NovÂ 2004 00:00:00 -05:00

Background: Term infants who are small for gestational age appear prone to the development of insulin resistance during childhood. We hypothesized that insulin resistance, a marker of type 2 diabetes...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 31-2004 â A Four-Year-Old Boy with Hypoxemia

T. Bernard Kinane, M.D., and Sjirk J. Westra, M.D. — Thu, 14Â OctÂ 2004 00:00:00 -05:00

A four-year-old boy was transferred to this hospital because of hypoxemia of unknown origin. Five days before admission, he had been taken to the emergency room of another hospital with...

ORIGINAL ARTICLE: Growth Patterns and the Risk of Breast Cancer in Women

Thu, 14Â OctÂ 2004 00:00:00 -05:00

Background: Adult height and body-mass index influence the risk of breast cancer in women. Whether these associations reflect growth patterns of the fetus or growth during childhood and adolescence is...

PERSPECTIVE: Antidepressants and Pediatric Depression â The Risk of Doing Nothing

David A. Brent, M.D. — Thu, 14Â OctÂ 2004 00:00:00 -05:00

There is great concern that antidepressants used in children and adolescents may paradoxically increase their risk of suicidal thoughts and behavior. Is this concern valid, and if so, how should...

PERSPECTIVE: A Black-Box Warning for Antidepressants in Children?

Thomas B. Newman, M.D., M.P.H. — Thu, 14Â OctÂ 2004 00:00:00 -05:00

On September 14, 2004, a Food and Drug Administration (FDA) joint advisory committee voted 15 to 8 to recommend that a âblack-boxâ warning label be required for antidepressant drugs, indicating...

ORIGINAL ARTICLE: Hemoglobin Jamaica Plain â A Sickling Hemoglobin with Reduced Oxygen Affinity

Thu, 07Â OctÂ 2004 00:00:00 -05:00

A baby girl presented with symptomatic sickle cell disease exacerbated by mild hypoxemia, despite a newborn-screening diagnosis of sickle cell trait. DNA sequencing of the Î² globin gene revealed that...

PERSPECTIVE: Genotypes and Phenotypes â Another Lesson from the Hemoglobinopathies

Edward J. Benz, Jr., M.D. — Thu, 07Â OctÂ 2004 00:00:00 -05:00

The human genome project has ignited considerable excitement about the future of medicine by revealing insights into diseases that seemed impossible to unravel only a few years ago. But knowledge...

ORIGINAL ARTICLE: Autoimmune Lymphoproliferative Syndrome with Somatic Mutations

Thu, 30Â SepÂ 2004 00:00:00 -05:00

Background: Impaired Fas-induced apoptosis of lymphocytes in vitro is a principal feature of the autoimmune lymphoproliferative syndrome (ALPS). We studied six children with ALPS whose lymphocytes had normal sensitivity to...

ORIGINAL ARTICLE: A Randomized Trial of a Single Dose of Oral Dexamethasone for Mild Croup

Thu, 23Â SepÂ 2004 00:00:00 -05:00

Background: The benefits of dexamethasone treatment for moderate-to-severe croup are well established. However, most children with croup have mild symptoms, and it is unknown whether they would derive the same...

PERSPECTIVE: Croup â The Bark Is Worse Than the Bite

Perri Klass, M.D. — Thu, 23Â SepÂ 2004 00:00:00 -05:00

It is a clichÃ© of primary care pediatrics: on call in the winter, you find yourself on the telephone with a worried parent who is anxiously trying to describe a...

EDITORIAL: Should Parents Speak with a Dying Child about Impending Death?

Lawrence Wolfe, M.D. — Thu, 16Â SepÂ 2004 00:00:00 -05:00

In 1985, a reading of Patrick Irvine's essay in the Journal about his participation in the funerals of his patients1 eased concerns of my own about attending the funerals of...

REVIEW ARTICLE: Turner's Syndrome

Virginia P. Sybert, M.D., and Elizabeth McCauley, Ph.D. — Thu, 16Â SepÂ 2004 00:00:00 -05:00

Turner's syndrome, a disorder in females characterized by the absence of all or part of a normal second sex chromosome, leads to a constellation of physical findings that often includes...

ORIGINAL ARTICLE: Radiofrequency Ablation in Children with Asymptomatic WolffâParkinsonâWhite Syndrome

Thu, 16Â SepÂ 2004 00:00:00 -05:00

Background: Ventricular fibrillation can be the presenting arrhythmia in children with asymptomatic WolffâParkinsonâWhite syndrome. Deaths due to this arrhythmia are potentially preventable.

Methods: We performed a randomized study in which prophylactic...

ORIGINAL ARTICLE: Talking about Death with Children Who Have Severe Malignant Disease

Thu, 16Â SepÂ 2004 00:00:00 -05:00

Background: One of the questions faced by the parents of a child who is terminally ill with a malignant disease is whether or not they should talk about death with...

PERSPECTIVE: Catheter Ablation for Cardiac Arrhythmias

Hein J.J. Wellens, M.D. — Thu, 16Â SepÂ 2004 00:00:00 -05:00

Cardiac arrhythmias are common; some are life-threatening, others merely a nuisance. They are caused by abnormalities in impulse formation or conduction that lead to slow or fast, regular or irregular...

EDITORIAL: Allergen Avoidance to Reduce Asthma-Related Morbidity

Albert L. Sheffer, M.D. — Thu, 09Â SepÂ 2004 00:00:00 -05:00

Central to the reduction of the severity of allergic disease is a decrease in â and preferably the removal of â the offending environmental allergen. Such allergen avoidance is particularly...

EDITORIAL: Air Pollution and Health â Good News and Bad

C. Arden Pope III, Ph.D. — Thu, 09Â SepÂ 2004 00:00:00 -05:00

Early concerns regarding the health-related effects of air pollution originated from severe episodes in Meuse Valley, Belgium, in 1930; Donora, Pennsylvania, in 1948; and London, in 1952. Although the overall...

ORIGINAL ARTICLE: Elevated Plasma Factor VIII and D-Dimer Levels as Predictors of Poor Outcomes of Thrombosis in Children

Thu, 09Â SepÂ 2004 00:00:00 -05:00

Background: Elevated levels of plasma factor VIII and D-dimer predict recurrent venous thromboembolism in adults. We sought to determine whether an elevation of factor VIII, D-dimer, or both at diagnosis...

ORIGINAL ARTICLE: Results of a Home-Based Environmental Intervention among Urban Children with Asthma

Thu, 09Â SepÂ 2004 00:00:00 -05:00

Background: Children with asthma who live in the inner city are exposed to multiple indoor allergens and environmental tobacco smoke in their homes. Reductions in these triggers of asthma have...

ORIGINAL ARTICLE: The Effect of Air Pollution on Lung Development from 10 to 18 Years of Age

Thu, 09Â SepÂ 2004 00:00:00 -05:00

Background: Whether exposure to air pollution adversely affects the growth of lung function during the period of rapid lung development that occurs between the ages of 10 and 18 years...

PERSPECTIVE: Factor VIII, D-Dimer, and Thromboembolism in Children

Ulrike Nowak-GÃ¶ttl, M.D., and Andrea Kosch, M.D. — Thu, 09Â SepÂ 2004 00:00:00 -05:00

Symptomatic venous and arterial thromboses are being diagnosed with increasing frequency in neonates, infants, and children. Because of the special properties of the hemostatic system in children, thrombotic manifestations are...

EDITORIAL: Bare-Bones Fact â Children Are Not Small Adults

Laura K. Bachrach, M.D. — Thu, 26Â AugÂ 2004 00:00:00 -05:00

Diagnostic and therapeutic guidelines used for children are commonly extrapolated from studies conducted in adults. There are potential dangers in assuming that children will have the same response to disease...

ORIGINAL ARTICLE: Long-Term, High-Dose Glucocorticoids and Bone Mineral Content in Childhood Glucocorticoid-Sensitive Nephrotic Syndrome

Thu, 26Â AugÂ 2004 00:00:00 -05:00

Background: Glucocorticoids suppress bone formation, impair growth, and induce obesity. We determined the effects of long-term treatment with glucocorticoids on bone mineral content in children with glucocorticoid-sensitive nephrotic syndrome, a...

IMAGES IN CLINICAL MEDICINE: Rotationplasty for Ewing's Sarcoma of the Distal Femur

John P. Dormans, M.D. and Sumeet Garg, M.D. — Thu, 19Â AugÂ 2004 00:00:00 -05:00

A five-year-old girl received a diagnosis of Ewing's sarcoma of the distal femur after she noticed a painless mass behind her left knee. The tumor had not metastasized at the...

EDITORIAL: Finding Needles in Haystacks â Gene Variants in Isolated Cleft Lip or Cleft Palate

Aravinda Chakravarti, Ph.D. — Thu, 19Â AugÂ 2004 00:00:00 -05:00

Human geneticists have been on a long joyride. The power of contemporary genetics and genomics has allowed us to identify the specific causal gene in a dizzying variety of mendelian...

ORIGINAL ARTICLE: Interferon Regulatory Factor 6 () Gene Variants and the Risk of Isolated Cleft Lip or Palate

Thu, 19Â AugÂ 2004 00:00:00 -05:00

Background: Cleft lip or palate (or the two in combination) is a common birth defect that results from a mixture of genetic and environmental factors. We searched for a specific...

PERSPECTIVE: The Changing Faces of Children with Cleft Lip and Palate

John B. Mulliken, M.D. — Thu, 19Â AugÂ 2004 00:00:00 -05:00

Many expectant parents worry, consciously or unconsciously, that their baby will be born with a malformation. Cleft lip with or without cleft palate is one of the most common birth...

IMAGES IN CLINICAL MEDICINE: Imperforate Hymen with Hematocolpometra

Sean M. Stone, M.D. and John L. Alexander, M.D. — Thu, 12Â AugÂ 2004 00:00:00 -05:00

An 11-year-old girl presumed to be premenarchal presented to the emergency department with a 24-hour history of constant periumbilical pain that radiated to both flanks. She also noted nausea, urinary...

EDITORIAL: Immunotherapy in Allergy to Insect Stings in Children

Rebecca S. Gruchalla, M.D., Ph.D. — Thu, 12Â AugÂ 2004 00:00:00 -05:00

The earliest reported death from an allergic reaction to a stinging insect was that of King Menes of Egypt, who died in 2641 B.C., purportedly as the result of a...

ORIGINAL ARTICLE: Outcomes of Allergy to Insect Stings in Children, with and without Venom Immunotherapy

Thu, 12Â AugÂ 2004 00:00:00 -05:00

Background: Children are thought to âoutgrowâ the allergy to insect stings, but there are no reports documenting the natural history of this reaction. We studied the outcome of allergic reactions...

EDITORIAL: Childhood Leukemia â New Advances and Challenges

Naomi J. Winick, M.D., William L. Carroll, M.D., and Stephen P. Hunger, M.D. — Thu, 05Â AugÂ 2004 00:00:00 -05:00

Since 1970, the rate of cure of acute lymphoblastic leukemia (ALL) in children has increased dramatically, from less than 30 percent to approximately 80 percent. This remarkable improvement has resulted...

ORIGINAL ARTICLE: Loss of Smad3 in Acute T-Cell Lymphoblastic Leukemia

Thu, 05Â AugÂ 2004 00:00:00 -05:00

Background: The receptors for transforming growth factor Î² (TGF-Î²) and their signaling intermediates make up an important tumor-suppressor pathway. The role of one of these intermediates â Smad3 â in...

IMAGES IN CLINICAL MEDICINE: Circumpapillary Retinal Ridge in the Shaken-Baby Syndrome

Camille Hylton, M.D. and Morton F. Goldberg, M.D. — Thu, 08Â JulÂ 2004 00:00:00 -05:00

A five-month-old girl was admitted to the hospital because of uncontrollable seizures. Her father had shaken her vigorously, allegedly in an attempt to rescue her from a choking spell. A...

ORIGINAL ARTICLE: The Effect of Dexrazoxane on Myocardial Injury in Doxorubicin-Treated Children with Acute Lymphoblastic Leukemia

Thu, 08Â JulÂ 2004 00:00:00 -05:00

Background: Doxorubicin chemotherapy is very effective in children with acute lymphoblastic leukemia (ALL) but also injures myocardial cells. Dexrazoxane, a free-radical scavenger, may protect the heart from doxorubicin-associated damage.

Methods: To...

PERSPECTIVE: Anthracycline Cardiotoxicity in Children

Leontine C.M. Kremer, M.D., Ph.D., and Huib N. Caron, M.D., Ph.D. — Thu, 08Â JulÂ 2004 00:00:00 -05:00

More than 70 percent of children who are treated for childhood cancer can be cured. For long-term survivors, possible late effects of treatment and their consequences for the quality of...

IMAGES IN CLINICAL MEDICINE: Homozygous Hemoglobin C Disease

Rick M. Fairhurst, M.D., Ph.D. and James F. Casella, M.D. — Thu, 24Â JunÂ 2004 00:00:00 -05:00

A Ghanaian child was found on routine screening to have a hemoglobin level of 9.0 g per deciliter, a hematocrit of 24.3 percent, a mean corpuscular volume of 53.8 Î¼m3,...

ORIGINAL ARTICLE: Myostatin Mutation Associated with Gross Muscle Hypertrophy in a Child

Thu, 24Â JunÂ 2004 00:00:00 -05:00

Muscle wasting and weakness are among the most common inherited and acquired disorders and include the muscular dystrophies, cachexia, and age-related wasting. Since there is no generally accepted treatment to...

ORIGINAL ARTICLE: Long-Term Survival of Children with End-Stage Renal Disease

Thu, 24Â JunÂ 2004 00:00:00 -05:00

Background: Although renal-replacement therapy for children with end-stage renal disease has been used for several decades, data on patients' long-term survival are sparse.

Methods: We examined the long-term survival of all...

PERSPECTIVE: Powerful Genes â Myostatin Regulation of Human Muscle Mass

Elizabeth M. McNally, M.D., Ph.D. — Thu, 24Â JunÂ 2004 00:00:00 -05:00

In this issue of the Journal, Schuelke et al. (pages 2682â2688) describe a child with substantial muscle hypertrophy and a splice-site mutation in the gene encoding myostatin. Myostatin is a...

PERSPECTIVE: Pediatric Renal-Replacement Therapy â Coming of Age

Dawn S. Milliner, M.D. — Thu, 24Â JunÂ 2004 00:00:00 -05:00

Before the middle of the 20th century, the prospect that a child with end-stage renal disease (ESRD) would reach adulthood was essentially nil. Once dialysis and transplantation became available, that...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 19-2004 â A 12-Year-Old Boy with Fatigue and Eosinophilia

Mary S. Huang, M.D., and Robert P. Hasserjian, M.D. — Thu, 17Â JunÂ 2004 00:00:00 -05:00

A 12-year-old boy was evaluated in the hematology clinic of this hospital because of fatigue and peripheral-blood eosinophilia. For one week, he had had fatigue that had caused him to...

IMAGES IN CLINICAL MEDICINE: Gas Gangrene

Stephen M. Schexnayder, M.D. and Sarah G. Klein, M.D. — Thu, 17Â JunÂ 2004 00:00:00 -05:00

A 12-year-old boy with acute myelogenous leukemia that had been diagnosed two months earlier presented to a hospital with fever and leg pain. He had received intravenous cytarabine two weeks...

ORIGINAL ARTICLE: A Trial of Three Antiretroviral Regimens in HIV-1âInfected Children

Thu, 10Â JunÂ 2004 00:00:00 -05:00

Background: Depletion of CD4 T-cell counts or progression of human immunodeficiency virus (HIV) disease occurs rapidly in children, but few data address the efficacy of aggressive therapy for HIV-infected children.

Methods:...

ORIGINAL ARTICLE: Obesity and the Metabolic Syndrome in Children and Adolescents

Thu, 03Â JunÂ 2004 00:00:00 -05:00

Background: The prevalence and magnitude of childhood obesity are increasing dramatically. We examined the effect of varying degrees of obesity on the prevalence of the metabolic syndrome and its relation...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Targeting Gene Therapy for Osteogenesis Imperfecta

Darwin J. Prockop, M.D., Ph.D. — Thu, 27Â MayÂ 2004 00:00:00 -05:00

Two shadows have recently been cast over the exciting field of gene therapy. One was cast by the unfortunate death of an 18-year-old man at the University of Pennsylvania in...

PERSPECTIVE: Torment

Danielle Ofri, M.D., Ph.D. — Thu, 27Â MayÂ 2004 00:00:00 -05:00

I groan when I catch sight of her name on the patient roster. Nazma Uddin. Not again! She is in my clinic office almost every month. I dread her visits,...

PERSPECTIVE: Pediatric Antecedents of Adult Cardiovascular Disease â Awareness and Intervention

Julie R. Ingelfinger, M.D. — Thu, 20Â MayÂ 2004 00:00:00 -05:00

Hypertension, which is common among adults in the United States, has for several decades been recognized as a cardiovascular risk factor. Since blood pressure tends to âtrackâ along the same...

PERSPECTIVE: Index Case

Perri Klass, M.D. — Thu, 13Â MayÂ 2004 00:00:00 -05:00

Because of my extensive training â four years of medical school, three years of pediatric residency, a two-year fellowship in pediatric infectious diseases â and because of my years of...

ORIGINAL ARTICLE: Cord-Blood Transplants from Unrelated Donors in Patients with Hurler's Syndrome

Thu, 06Â MayÂ 2004 00:00:00 -05:00

Background: Hurler's syndrome (the most severe form of mucopolysaccharidosis type I) causes progressive deterioration of the central nervous system and death in childhood. Allogeneic bone marrow transplantation before the age...

PERSPECTIVE: Advances in the Treatment of Mucopolysaccharidosis Type I

Joseph Muenzer, M.D., Ph.D., and Amy Fisher, M.S. — Thu, 06Â MayÂ 2004 00:00:00 -05:00

The mucopolysaccharidoses are a group of lysosomal storage diseases caused by a deficiency of enzymes that degrade glycosaminoglycans.1 Mucopolysaccharidosis type I, an autosomal recessive disorder caused by a deficiency of...

ORIGINAL ARTICLE: Activating Mutations in the Gene Encoding the ATP-Sensitive Potassium-Channel Subunit Kir6.2 and Permanent Neonatal Diabetes

Thu, 29Â AprÂ 2004 00:00:00 -05:00

Background: Patients with permanent neonatal diabetes usually present within the first three months of life and require insulin treatment. In most, the cause is unknown. Because ATP-sensitive potassium (KATP) channels...

PERSPECTIVE: Open to Control â New Hope for Patients with Neonatal Diabetes

Fiona M. Gribble, B.M., B.Ch., Ph.D., and Frank Reimann, Ph.D. — Thu, 29Â AprÂ 2004 00:00:00 -05:00

Pancreatic beta cells, the insulin-releasing cells of the islets of Langerhans, are electrically excitable cells. They sense fluctuations in the plasma glucose concentration and convert the signal into a change...

REVIEW ARTICLE: Pediatric Palliative Care

Thu, 22Â AprÂ 2004 00:00:00 -05:00

Each year in the United States, approximately 50,000 children die and 500,000 children cope with life-threatening conditions. Worldwide these numbers are in the millions.12 Such children and their families require...

ORIGINAL ARTICLE: A Comparison of High-Dose and Standard-Dose Epinephrine in Children with Cardiac Arrest

Thu, 22Â AprÂ 2004 00:00:00 -05:00

Background: When efforts to resuscitate a child after cardiac arrest are unsuccessful despite the administration of an initial dose of epinephrine, it is unclear whether the next dose of epinephrine...

PERSPECTIVE: Can Resuscitation Jeopardize Survival?

George Lister, M.D., and J. Julio PÃ©rez FontÃ¡n, M.D. — Thu, 22Â AprÂ 2004 00:00:00 -05:00

Resuscitations are events that are often clouded by desperation and frenzy and directed by persons with limited experience, who may feel a great burden of responsibility for the circumstances precipitating...

PERSPECTIVE: Antidepressant Medications in Children

Benedetto Vitiello, M.D., and Susan Swedo, M.D. — Thu, 08Â AprÂ 2004 00:00:00 -05:00

In June 2003, the Medicine and Healthcare Products Regulatory Agency of the British Department of Health warned physicians to avoid the off-label use of paroxetine, a selective serotonin-reuptake inhibitor (SSRI)...

ORIGINAL ARTICLE: Childhood Vaccination and Type 1 Diabetes

Thu, 01Â AprÂ 2004 00:00:00 -05:00

Background: A link between childhood vaccinations and the development of type 1 diabetes has been proposed.

Methods: We evaluated a cohort comprising all children born in Denmark from January 1, 1990,...

PERSPECTIVE: Childhood Immunizations and Chronic Illness

Lynne L. Levitsky, M.D. — Thu, 01Â AprÂ 2004 00:00:00 -05:00

These hopeful words of Edward Jenner (see Figure) were prescient, although their promise remained unfulfilled for almost 200 years. One hundred years ago, more than 48,000 people contracted smallpox annually...

ORIGINAL ARTICLE: Salt Wasting and Deafness Resulting from Mutations in Two Chloride Channels

Thu, 25Â MarÂ 2004 00:00:00 -05:00

Inherited kidney disorders associated with salt wasting, hypokalemia, and metabolic alkalosis (Bartter's syndrome) are clinically classified into several variants1: classic Bartter's syndrome,2 Gitelman's variant of Bartter's syndrome,3 and antenatal Bartter's...

ORIGINAL ARTICLE: Outcomes at School Age after Postnatal Dexamethasone Therapy for Lung Disease of Prematurity

Thu, 25Â MarÂ 2004 00:00:00 -05:00

Background: We studied the outcomes at school age in children who had participated in a double-blind, placebo-controlled trial of early postnatal dexamethasone therapy (initiated within 12 hours after birth) for...

CLINICAL PROBLEM-SOLVING: A Pain in the Neck

Sandra J. Bliss, M.D., Scott A. Flanders, M.D., and Sanjay Saint, M.D., M.P.H. — Thu, 04Â MarÂ 2004 00:00:00 -05:00

A previously healthy 16-year-old girl presented to her physician because of a two-day history of sore throat, fatigue, fever, headache, and vomiting; she had not had rhinorrhea or a cough....

ORIGINAL ARTICLE: Relation of Serial Changes in Childhood Body-Mass Index to Impaired Glucose Tolerance in Young Adulthood

Thu, 26Â FebÂ 2004 00:00:00 -05:00

Background: The risk of type 2 diabetes mellitus is increased in people who have low birth weights and who subsequently become obese as adults. Whether their obesity originates in childhood...

PERSPECTIVE: Overweight in Childhood and Adolescence

William H. Dietz, M.D., Ph.D. — Thu, 26Â FebÂ 2004 00:00:00 -05:00

The prevalence of overweight doubled among children 6 to 11 years of age and tripled among those 12 to 17 years of age between the second National Health and Nutrition...

IMAGES IN CLINICAL MEDICINE: Congenital Lymphedema

Jeffrey S. Upperman, M.D. and Edward M. Barksdale, Jr., M.D. — Thu, 05Â FebÂ 2004 00:00:00 -05:00

A full-term infant born to a woman (gravida 1, para 1) with a history of gestational diabetes presented with edema of the right leg (Panel A). When the infant was...

ORIGINAL ARTICLE: Deficiency of the Circulating Insulin-like Growth Factor System Associated with Inactivation of the Acid-Labile Subunit Gene

Thu, 05Â FebÂ 2004 00:00:00 -05:00

The growth-promoting actions of growth hormone were originally hypothesized to be mediated through a circulating liver-generated sulfation factor that later came to be known as insulin-like growth factor I (IGF-I).1...

IMAGES IN CLINICAL MEDICINE: Hypoglossal-Nerve Palsy

Dimitrios I. Zafeiriou,, M.D., Ph.D. and Evangelos Pavlou, M.D. — Thu, 29Â JanÂ 2004 00:00:00 -05:00

A six-year-old boy was brought to the emergency department because of acute speech difficulties. Five days previously, a low-grade fever, sore throat, and malaise had developed. The day before presentation,...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 4-2004 â A Nine-Month-Old Boy with an Orbital Rhabdomyosarcoma

Thu, 29Â JanÂ 2004 00:00:00 -05:00

Dr. Torunn Yock (Radiation Oncology): A nine-month-old male infant was referred to this hospital for treatment of an orbital rhabdomyosarcoma. The patient had been well until the age of...

ORIGINAL ARTICLE: Human Metapneumovirus and Lower Respiratory Tract Disease in Otherwise Healthy Infants and Children

Thu, 29Â JanÂ 2004 00:00:00 -05:00

Background: We sought to determine the role of human metapneumovirus in lower respiratory tract illness in previously healthy infants and children.

Methods: We tested nasal-wash specimens, obtained over a 25-year period...

PERSPECTIVE: Human Metapneumovirus â An Important New Respiratory Virus

Kenneth McIntosh, M.D., and Alexander J. McAdam, M.D., Ph.D. — Thu, 29Â JanÂ 2004 00:00:00 -05:00

Physicians who have an interest in infectious diseases know well that many infections cannot be assigned a microbial cause. This gap between what we observe and what we can explain...

ORIGINAL ARTICLE: Discordant Sexual Identity in Some Genetic Males with Cloacal Exstrophy Assigned to Female Sex at Birth

William G. Reiner, M.D., and John P. Gearhart, M.D. — Thu, 22Â JanÂ 2004 00:00:00 -05:00

Background: Cloacal exstrophy is a rare, complex defect of the entire pelvis and its contents that occurs during embryogenesis and is associated with severe phallic inadequacy or phallic absence in...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 40-2003 â A 14-Month-Old Boy with Recurrent Abdominal Distention and Diarrhea

Benjamin D. Gold, M.D., Sjirk J. Westra, M.D., and Fiona M. Graeme-Cook, M.D. — Thu, 25Â DecÂ 2003 00:00:00 -05:00

A 14-month-old boy was admitted to the hospital because of recurrent abdominal distention, vomiting, and diarrhea.

The boy had been born at another hospital at 34 weeks' gestation and weighed 2.56...

ORIGINAL ARTICLE: The Effect of Oral Sucrose on Exercise Tolerance in Patients with McArdle's Disease

John Vissing, M.D., Ph.D., and Ronald G. Haller, M.D. — Thu, 25Â DecÂ 2003 00:00:00 -05:00

Background: Energy metabolism in muscles relies predominantly on the breakdown of glycogen early in exercise. In patients with McArdle's disease, blocked glycogenolysis in muscles results in low exercise tolerance and...

PERSPECTIVE: Sweet Success â A Treatment for McArdle's Disease

Anthony A. Amato, M.D. — Thu, 25Â DecÂ 2003 00:00:00 -05:00

Carbohydrates are often stored as glycogen in the body. Pathways for the synthesis and breakdown of glycogen (see Figure) permit the maintenance of a steady blood glucose level and provide...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 38-2003 â A 12-Year-Old Girl with Fever and Coma

H. Shaw Warren, Jr., M.D., R. Gilberto Gonzalez, M.D., and Di Tian, M.D., Ph.D. — Thu, 11Â DecÂ 2003 00:00:00 -05:00

A 12-year-old girl was admitted to the hospital because of fever, vomiting, and an abrupt onset of unresponsiveness.

She had been well until one week earlier, when a cough, sore throat,...

ORIGINAL ARTICLE: IGF-I Receptor Mutations Resulting in Intrauterine and Postnatal Growth Retardation

Thu, 04Â DecÂ 2003 00:00:00 -05:00

Background: Approximately 10 percent of infants with intrauterine growth retardation remain small, and the causes of their growth deficits are often unclear. We postulated that mutations in the gene for...

PERSPECTIVE: Insulin-like Growth Factors and the Basis of Growth

Ron G. Rosenfeld, M.D. — Thu, 04Â DecÂ 2003 00:00:00 -05:00

Growth in any species is an extraordinarily complex process, but growth in humans is characterized by a number of unique features. These include dramatic fetal growth (the most rapid phase...

IMAGES IN CLINICAL MEDICINE: Congenital Pulmonary Lymphangiectasia

Cornelia Hagmann, M.D. and Thomas M. Berger, M.D. — Thu, 27Â NovÂ 2003 00:00:00 -05:00

A female infant was born at 40 weeks and 3 days of gestation, weighing 2970 g. The Apgar scores were 8, 10, and 10 at 1, 5, and 10 minutes,...

ORIGINAL ARTICLE: Inhaled Nitric Oxide in Premature Infants with the Respiratory Distress Syndrome

Thu, 27Â NovÂ 2003 00:00:00 -05:00

Background: Inhaled nitric oxide improves gas exchange, decreases pulmonary vascular lability, and reduces pulmonary inflammation. We hypothesized that the use of inhaled nitric oxide would decrease the incidence of chronic...

IMAGES IN CLINICAL MEDICINE: Transillumination of a Cystic Lymphatic Malformation

Hanspaul S. Makkar, M.D. and Ilona J. Frieden, M.D. — Thu, 06Â NovÂ 2003 00:00:00 -05:00

A four-week-old, full-term male infant presented with a mass at the base of his neck (Panel A). The growth of the lesion, evident since his birth after an uncomplicated pregnancy,...

ORIGINAL ARTICLE: Effect of CD3Î´ Deficiency on Maturation of Î±/Î² and Î³/Î´ T-Cell Lineages in Severe Combined Immunodeficiency

Harjit K. Dadi, Ph.D., Amos J. Simon, Ph.D., and Chaim M. Roifman, M.D. — Thu, 06Â NovÂ 2003 00:00:00 -05:00

The T-cellâreceptor complex consists of the Î± and Î² or Î³ and Î´ variant chains, paired as mutually exclusive heterodimers in association with the invariant chains CD3Î³, Î´, Îµ, and...

IMAGES IN CLINICAL MEDICINE: Bony Ghosts â Residual Effects of Severe Growth Arrest

JÃ¶rg PfÃ¶rtner, M.D. and Matthias HÃ¶vel, M.D. — Thu, 30Â OctÂ 2003 00:00:00 -05:00

A 16-year-old boy reported a two-to-three-month history of back pain during sports activities. The pain radiated to the buttocks, but without signs of sciatica. The patient had no history of...

SOUNDING BOARD: Can We Prevent Cerebral Palsy?

Karin B. Nelson, M.D. — Thu, 30Â OctÂ 2003 00:00:00 -05:00

Improvements in obstetrical and neonatal care have led to marked declines in rates of injury and death in mothers and infants. The risk of neurologic disability in infants and children...

REVIEW ARTICLE: The Toxicology of Mercury â Current Exposures and Clinical Manifestations

Thomas W. Clarkson, Ph.D., Laszlo Magos, M.D., and Gary J. Myers, M.D. — Thu, 30Â OctÂ 2003 00:00:00 -05:00

Mercury has been used commercially and medically for centuries. In the past it was a common constituent of many medications. It is still used in hospitals in thermometers and blood-pressure...

ORIGINAL ARTICLE: The Gene as a Regulator of Puberty

Thu, 23Â OctÂ 2003 00:00:00 -05:00

Background: Puberty, a complex biologic process involving sexual development, accelerated linear growth, and adrenal maturation, is initiated when gonadotropin-releasing hormone begins to be secreted by the hypothalamus. We conducted studies...

PERSPECTIVE: Bench and Bedside â The G ProteinâCoupled Receptor GPR54 and Puberty

David R. Beier, Ph.D., M.D., and Robert G. Dluhy, M.D. — Thu, 23Â OctÂ 2003 00:00:00 -05:00

The ontogeny of human gonadotropin secretion is remarkable for surges in pulsatile secretion during the neonatal period and in early childhood, followed by a period of quiescence (the so-called juvenile...

EDITORIAL: Toward Asthma Prevention â Does All That Really Matters Happen before We Learn to Read?

Fernando D. Martinez, M.D. — Thu, 09Â OctÂ 2003 00:00:00 -05:00

Patients with asthma and their caregivers are currently faced with a remarkable paradox. Considerable progress has been made during the past 20 years in the pharmacotherapy, educational tools, and environmental...

ORIGINAL ARTICLE: Gentamicin-Induced Correction of CFTR Function in Patients with Cystic Fibrosis and Stop Mutations

Thu, 09Â OctÂ 2003 00:00:00 -05:00

Background: Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene containing a premature termination signal cause a deficiency or absence of functional chloride-channel activity. Aminoglycoside antibiotics can suppress premature...

ORIGINAL ARTICLE: A Longitudinal, Population-Based, Cohort Study of Childhood Asthma Followed to Adulthood

Thu, 09Â OctÂ 2003 00:00:00 -05:00

Background: The outcome of childhood asthma in adults has been described in high-risk cohorts, but few population-based studies have reported the risk factors for persistence and relapse.

Methods: We assessed children...

PERSPECTIVE: Pharmacologic Approaches to Correcting the Basic Defect in Cystic Fibrosis

Gergely L. Lukacs, M.D., Ph.D., and Peter R. Durie, M.D. — Thu, 09Â OctÂ 2003 00:00:00 -05:00

Cystic fibrosis is a heterogeneous condition caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.1 The gene product, a regulated chloride channel, is expressed in the apical...

ORIGINAL ARTICLE: A Trial of a 9-Valent Pneumococcal Conjugate Vaccine in Children with and Those without HIV Infection

Thu, 02Â OctÂ 2003 00:00:00 -05:00

Background: Acute respiratory tract infections caused by Streptococcus pneumoniae are a leading cause of morbidity and mortality in young children. We evaluated the efficacy of a 9-valent pneumococcal conjugate vaccine...

PERSPECTIVE: Great Expectations for a New Vaccine

Kathryn M. Edwards, M.D., and Marie R. Griffin, M.D., M.P.H. — Thu, 02Â OctÂ 2003 00:00:00 -05:00

Despite the fact that a licensed pneumococcal capsular polysaccharide vaccine has been available for more than 30 years, Streptococcus pneumoniae continues to sicken and kill children and adults throughout the...

REVIEW ARTICLE: Epilepsy

Bernard S. Chang, M.D., and Daniel H. Lowenstein, M.D. — Thu, 25Â SepÂ 2003 00:00:00 -05:00

Epilepsy is one of the most common neurologic problems worldwide. Approximately 2 million persons in the United States have epilepsy, and 3 percent of persons in the general population will...

REVIEW ARTICLE: Developmental Pharmacology â Drug Disposition, Action, and Therapy in Infants and Children

Thu, 18Â SepÂ 2003 00:00:00 -05:00

Infants and children are far different from adults in terms of societal, psychosocial, behavioral, and medical perspectives. More than 100 years ago Dr. Abraham Jacobi, the father of American pediatrics,...

ORIGINAL ARTICLE: Growth Hormone Insensitivity Associated with a Mutation

Thu, 18Â SepÂ 2003 00:00:00 -05:00

The syndrome of growth hormone insensitivity is characterized by phenotypic features consistent with the presence of a growth hormone deficiency, but with normal-to-elevated circulating concentrations of growth hormone and resistance...

PERSPECTIVE: New Revelations about the Role of STATs in Stature

Erica A. Eugster, M.D., and Ora Hirsch Pescovitz, M.D. — Thu, 18Â SepÂ 2003 00:00:00 -05:00

In this issue of the Journal, Kofoed et al. (pages 1139â1147) describe a novel mechanism for impaired growth in the form of a mutation in the gene for the intracytoplasmic...

IMAGES IN CLINICAL MEDICINE: Human Bites versus Dog Bites

Howard Fischer, M.D., Pamela W. Hammel, D.D.S., and L.J. Dragovic, M.D. — Thu, 11Â SepÂ 2003 00:00:00 -05:00

A six-year-old boy was admitted to the hospital with an acute abdomen. He had a rectal perforation and two groups of lesions on his right leg. Neither he nor his...

REVIEW ARTICLE: Sudden Death in Young Athletes

Barry J. Maron, M.D. — Thu, 11Â SepÂ 2003 00:00:00 -05:00

Young athletes have come to be regarded as a special part of society, owing to their unique lifestyle and the widely held perception that they epitomize health and invulnerability, capable...

ORIGINAL ARTICLE: Oxygen-Saturation Targets and Outcomes in Extremely Preterm Infants

Thu, 04Â SepÂ 2003 00:00:00 -05:00

Background: Physiological studies have shown that chronic hypoxemia may occur in preterm infants who require supplemental oxygen for extended periods and that this hypoxemia may contribute to poor growth and...

REVIEW ARTICLE: Laryngology and Phonosurgery

Steven M. Zeitels, M.D., and Gerald B. Healy, M.D. — Thu, 28Â AugÂ 2003 00:00:00 -05:00

It has been more than 20 years since Vaughan1 reviewed the state of the art in human laryngology and voice disorders. During that time, an improved understanding of the physiology...

REVIEW ARTICLE: Congenital Adrenal Hyperplasia

Phyllis W. Speiser, M.D., and Perrin C. White, M.D. — Thu, 21Â AugÂ 2003 00:00:00 -05:00

Congenital adrenal hyperplasia is a group of autosomal recessive disorders resulting from the deficiency of one of the five enzymes required for the synthesis of cortisol in the adrenal cortex....

ORIGINAL ARTICLE: Psychiatric Hospitalizations among Survivors of Cancer in Childhood or Adolescence

Thu, 14Â AugÂ 2003 00:00:00 -05:00

Background: We investigated whether children and adolescents who survive cancer are at increased risk for psychiatric hospitalization.

Methods: In a nationwide, population-based, retrospective cohort study, 3710 persons who survived at least...

ORIGINAL ARTICLE: Extended Follow-up of Long-Term Survivors of Childhood Acute Lymphoblastic Leukemia

Thu, 14Â AugÂ 2003 00:00:00 -05:00

Background: Children who survive acute lymphoblastic leukemia are at risk for leukemia-related or treatment-related complications, which can adversely affect survival and socioeconomic status. We determined the long-term survival and the...

PERSPECTIVE: Childhood Leukemia â Successes and Challenges for Survivors

Joseph V. Simone, M.D. — Thu, 14Â AugÂ 2003 00:00:00 -05:00

I was a brand-new fellow in pediatric hematologyâoncology, in July 1963, when I first cared for a child with acute leukemia. By then, with chemotherapy we were able to induce...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 24-2003 â A 10-Year-Old Girl with Recurrent Bouts of Abdominal Pain

Dawn M. McGee, M.D., Susan A. Connolly, M.D., and Robert H. Young, M.D. — Thu, 31Â JulÂ 2003 00:00:00 -05:00

A 10-year-old girl was admitted to the hospital because of abdominal pain, vomiting, and fever.

She had been in excellent health until about 18 months earlier, when she had the first...

ORIGINAL ARTICLE: Bisphosphonate-Induced Osteopetrosis

Thu, 31Â JulÂ 2003 00:00:00 -05:00

Bisphosphonates, synthetic analogues of inorganic pyrophosphate, potently inhibit skeletal resorption by suppressing the recruitment and activity of osteoclasts and shortening their life span.1 Consequently, several bisphosphonates were developed to treat...

ORIGINAL ARTICLE: Risk of Bacterial Meningitis in Children with Cochlear Implants

Thu, 31Â JulÂ 2003 00:00:00 -05:00

Background: In June 2002, the Food and Drug Administration received reports of bacterial meningitis in patients with cochlear implants for treatment of hearing loss. Implants that included a positioner (a...

PERSPECTIVE: Do Bisphosphonates Make Children's Bones Better or Brittle?

Joan C. Marini, M.D., Ph.D. — Thu, 31Â JulÂ 2003 00:00:00 -05:00

Bisphosphonates such as alendronate (Fosamax, Merck) and pamidronate (Aredia, CibaâGeigy) are synthetic analogues of inorganic pyrophosphate. The main effect of bisphosphonates, which are deposited on the surface of bone and...

PERSPECTIVE: Cochlear Implants

George A. Gates, M.D., and Richard T. Miyamoto, M.D. — Thu, 31Â JulÂ 2003 00:00:00 -05:00

Currently, a cochlear implant is the standard treatment for people whose hearing ability is so poor that well-fit hearing aids fail to permit effective oral communication. The implant is a...

IMAGES IN CLINICAL MEDICINE: Raccoon Eyes and Neuroblastoma

Robert Timmerman, M.D. — Thu, 24Â JulÂ 2003 00:00:00 -05:00

A nine-month-old girl was seen for failure to thrive and bruising around the eyes. On physical examination, she was noted to have periorbital ecchymosis, dysconjugate gaze, dilated and fixed pupils,...

IMAGES IN CLINICAL MEDICINE: A Quarter-Century of Fibrous Dysplasia

Herberto De Jesus, M.D. — Thu, 10Â JulÂ 2003 00:00:00 -05:00

Radiographs of the right humerus obtained at 4 years of age (Panel A), 24 years of age (Panel B), and 30 years of age (Panel C) in a patient with...

EDITORIAL: Treatment of Acute Bronchiolitis

Mary Ellen B. Wohl, M.D., and Victor Chernick, M.D. — Thu, 03Â JulÂ 2003 00:00:00 -05:00

In this issue of the Journal, Wainwright et al.1 provide data from a clinical trial in which 194 infants hospitalized with bronchiolitis were treated with inhaled epinephrine or placebo. In...

ORIGINAL ARTICLE: A Multicenter, Randomized, Double-Blind, Controlled Trial of Nebulized Epinephrine in Infants with Acute Bronchiolitis

Thu, 03Â JulÂ 2003 00:00:00 -05:00

Background: The treatment of infants with bronchiolitis is largely supportive. The role of bronchodilators is controversial. Most studies of the use of bronchodilators have enrolled small numbers of subjects and...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 20-2003 â A Nine-Year-Old Girl with Hepatosplenomegaly and Pain in the Thigh

Eric C. Larsen, M.D., Susan A. Connolly, M.D., and Andrew E. Rosenberg, M.D. — Thu, 26Â JunÂ 2003 00:00:00 -05:00

A nine-year-old girl was admitted to the hospital because of hepatosplenomegaly and pain in the thigh.

The patient had been well until three years earlier, when she began to have frequent...

EDITORIAL: Celiac Disease â How to Handle a Clinical Chameleon

Alessio Fasano, M.D. — Thu, 19Â JunÂ 2003 00:00:00 -05:00

Celiac disease is an immune-mediated enteropathy triggered by the ingestion of gluten-containing grains (including wheat, rye, and barley) in genetically susceptible persons. The disease is associated with HLA-DQ2 in 90...

ORIGINAL ARTICLE: Prevalence of Celiac Disease among Children in Finland

Thu, 19Â JunÂ 2003 00:00:00 -05:00

Background: Wheat, rye, and barley proteins induce celiac disease, an autoimmune type of gastrointestinal disorder, in genetically susceptible persons. Because the disease may be underdiagnosed, we estimated the prevalence of...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 18-2003 â A 15-Year-Old Girl with Pain in the Left Leg and Back, Pruritus, and Thoracic Lymphadenopathy

Leslie E. Lehmann, M.D., Sudha Anupindi, M.D., and Nancy Lee Harris, M.D. — Thu, 12Â JunÂ 2003 00:00:00 -05:00

A 15-year-old girl was admitted to the hospital because of pain in the left leg and back, pruritus, thoracic lymphadenopathy, and multiple pulmonary nodules.

The patient had been well until 11...

ORIGINAL ARTICLE: Estrogen Excess Associated with Novel Gain-of-Function Mutations Affecting the Aromatase Gene

Thu, 08Â MayÂ 2003 00:00:00 -05:00

Background: Gynecomastia of prepubertal onset may result from increased estrogen owing to excessive aromatase activity in extraglandular tissues. A gene in chromosome 15q21.2 encodes aromatase, the key enzyme for estrogen...

ORIGINAL ARTICLE: Decline in Invasive Pneumococcal Disease after the Introduction of ProteinâPolysaccharide Conjugate Vaccine

Thu, 01Â MayÂ 2003 00:00:00 -05:00

Background: In early 2000, a proteinâpolysaccharide conjugate vaccine targeting seven pneumococcal serotypes was licensed in the United States for use in young children.

Methods: We examined population-based data from the Active...

EDITORIAL: Pediatric Cardiomyopathy â A Long Way to Go

Arnold Strauss, M.D., and James E. Lock, M.D. — Thu, 24Â AprÂ 2003 00:00:00 -05:00

Improvements in the treatment of congenital heart disease are among the most impressive medical achievements of the second half of the 20th century. In 1950, patent ductus arteriosus and aortic...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 13-2003 â A 14-Month-Old Boy with Hepatomegaly, Perianal Lesions, and a Bony Lump on the Forehead

G. Naheed Usmani, M.D., Sjirk J. Westra, M.D., and Souhad Younes, M.D. — Thu, 24Â AprÂ 2003 00:00:00 -05:00

A 14-month-old boy was admitted to the hospital because of hepatomegaly, left-sided ptosis, and a bony lump on the forehead.

The patient had been constipated for several months, passing one hard...

ORIGINAL ARTICLE: The Incidence of Pediatric Cardiomyopathy in Two Regions of the United States

Thu, 24Â AprÂ 2003 00:00:00 -05:00

Background: Population-based data on the incidence of pediatric cardiomyopathy are rare because of the lack of large, prospective studies.

Methods: Since 1996 the Pediatric Cardiomyopathy Registry sponsored by the National Heart,...

ORIGINAL ARTICLE: The Epidemiology of Childhood Cardiomyopathy in Australia

Thu, 24Â AprÂ 2003 00:00:00 -05:00

Background: The incidence and age distribution of primary cardiomyopathy in children are not well defined. We undertook a population-based, retrospective cohort study in Australia to document the epidemiology of childhood...

ORIGINAL ARTICLE: Blood Lead Concentration and Delayed Puberty in Girls

Thu, 17Â AprÂ 2003 00:00:00 -05:00

Background: Environmental lead exposure has been linked to alterations in growth and endocrine function. It is not known whether such exposure affects pubertal development.

Methods: We analyzed the relations between blood...

ORIGINAL ARTICLE: Intellectual Impairment in Children with Blood Lead Concentrations below 10 Î¼g per Deciliter

Thu, 17Â AprÂ 2003 00:00:00 -05:00

Background: Despite dramatic declines in children's blood lead concentrations and a lowering of the Centers for Disease Control and Prevention's level of concern to 10 Î¼g per deciliter (0.483 Î¼mol...

PERSPECTIVE: Exposure to Lead in Children â How Low Is Low Enough?

Walter J. Rogan, M.D., and James H. Ware, Ph.D. — Thu, 17Â AprÂ 2003 00:00:00 -05:00

Encephalopathy in childhood due to lead poisoning was described more than 100 years ago, and cognitive sequelae after recovery were reported in 1943. During the past three decades, epidemiologic studies...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 11-2003 â A 14-Year-Old Boy with Ulcerative Colitis, Primary Sclerosing Cholangitis, and Partial Duodenal Obstruction

Albert M. Ross IV, M.D., Sudha A. Anupindi, M.D., and Ulysses J. Balis, M.D. — Thu, 10Â AprÂ 2003 00:00:00 -05:00

A 14-year-old boy with a history of ulcerative colitis was admitted to the hospital because of vomiting and abdominal pain.

Ulcerative colitis had been diagnosed at the age of three years...

IMAGES IN CLINICAL MEDICINE: Trichobezoar in a Four-Year-Old Girl

John L. Lanoue, M.D. and Marc S. Arkovitz, M.D. — Thu, 27Â MarÂ 2003 00:00:00 -05:00

A four-year-old girl was brought to her pediatrician by her parents because of intermittent bilious emesis, which had started two weeks earlier. There were no other changes in her bowel...

ORIGINAL ARTICLE: Factors Associated with Progression of Carcinoid Heart Disease

Thu, 13Â MarÂ 2003 00:00:00 -05:00

Background: By releasing vasoactive substances into the circulation, carcinoid tumors can cause right-sided valvular heart disease. Factors associated with the progression of carcinoid heart disease are poorly understood. We conducted...

EDITORIAL: Is Treatment with a Luteinizing HormoneâReleasing Hormone Agonist Justified in Short Adolescents?

Mary M. Lee, M.D. — Thu, 06Â MarÂ 2003 00:00:00 -05:00

In a 1953 editorial entitled âThe Need for an Inhibitor of Gonadotropin,â Lawson Wilkins noted that a âmethod of suppressing the secretion of pituitary gonadotropins . . . might lead...

ORIGINAL ARTICLE: Treatment with a Luteinizing HormoneâReleasing Hormone Agonist in Adolescents with Short Stature

Thu, 06Â MarÂ 2003 00:00:00 -05:00

Background: Treatment with a luteinizing hormoneâreleasing hormone (LHRH) agonist increases adult height in children with LHRH-dependent precocious puberty and is prescribed by some practitioners to augment height in short adolescents....

EDITORIAL: Therapies for Cancer in Children â Past Successes, Future Challenges

Robert E. Wittes, M.D. — Thu, 20Â FebÂ 2003 00:00:00 -05:00

Of the many things that physicians do, participating in cooperative clinical trials is among the strangest. Relatively undervalued in the typical academic promotion-and-tenure process, often inadequately reimbursed by government funding...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 6-2003 â A Nine-Year-Old Girl with Progressive Weakness and Areflexia

Howard W. Sander, M.D., and E. Tessa Hedley-Whyte, M.D. — Thu, 20Â FebÂ 2003 00:00:00 -05:00

A nine-year-old right-handed girl from the United Arab Emirates was admitted to the hospital because of progressive weakness and areflexia.

The patient had been well until three months earlier, when a...

ORIGINAL ARTICLE: Addition of Ifosfamide and Etoposide to Standard Chemotherapy for Ewing's Sarcoma and Primitive Neuroectodermal Tumor of Bone

Thu, 20Â FebÂ 2003 00:00:00 -05:00

Background: Ewing's sarcoma and primitive neuroectodermal tumor of bone are closely related, highly malignant tumors of children, adolescents, and young adults. A new drug combination, ifosfamide and etoposide, was highly...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 5-2003 â A 16-Year-Old Girl with a Rash and Chest Pain

Ivor Caro, M.D., and Artur Zembowicz, M.D. — Thu, 13Â FebÂ 2003 00:00:00 -05:00

A 16-year-old girl was admitted to the hospital because of pleuritic chest pain.

Bilateral imbrication of the hip capsules had been performed 15 months earlier at this hospital because of recurrent...

EDITORIAL: Increased Body Lead Burden â Cause or Consequence of Chronic Renal Insufficiency?

Philip A. Marsden, M.D. — Thu, 23Â JanÂ 2003 00:00:00 -05:00

Greek physicians first recognized the association between exposure to lead and disease over two millennia ago. The sources of lead have changed since ancient times, but exposure is still largely...

EDITORIAL: Imaging Studies for Childhood Urinary Infections

F. Bruder Stapleton, M.D. — Thu, 16Â JanÂ 2003 00:00:00 -05:00

The question of whether to obtain imaging studies after a urinary tract infection in a child is a challenging one. The potential long-term morbidity associated with repeated bouts of pyelonephritis...

ORIGINAL ARTICLE: Imaging Studies after a First Febrile Urinary Tract Infection in Young Children

Thu, 16Â JanÂ 2003 00:00:00 -05:00

Background: Guidelines from the American Academy of Pediatrics recommend obtaining a voiding cystourethrogram and a renal ultrasonogram for young children after a first urinary tract infection; renal scanning with technetium-99mâlabeled...

EDITORIAL: The Global Implications of Influenza in Hong Kong

Thu, 26Â DecÂ 2002 00:00:00 -05:00

Was the disappearance of brooding storks in Germany during the latter half of the last century really responsible for the precipitous decline in the number of newborn babies there? This...

ORIGINAL ARTICLE: Tetrahydrobiopterin as an Alternative Treatment for Mild Phenylketonuria

Thu, 26Â DecÂ 2002 00:00:00 -05:00

Background: Hyperphenylalaninemia is a common inherited metabolic disease that is due to phenylalanine hydroxylase deficiency, and at least half the affected patients have mild clinical phenotypes. Treatment with a low-phenylalanine...

ORIGINAL ARTICLE: Influenza-Related Hospitalizations among Children in Hong Kong

Thu, 26Â DecÂ 2002 00:00:00 -05:00

Background: It has been difficult to define the burden of influenza in children because of confounding by the cocirculation of respiratory syncytial virus (RSV). In Hong Kong, China, the influenza...

PERSPECTIVE: Tetrahydrobiopterin and Dietary Restriction in Mild Phenylketonuria

Thu, 26Â DecÂ 2002 00:00:00 -05:00

Inherited disorders of intermediary metabolism are the prototypes for the treatment of genetic disorders. In each condition, accumulation of a metabolite results from a genetically determined abnormality in a metabolic...

EDITORIAL: Varicella Vaccine â Are Two Doses Better Than One?

Thu, 12Â DecÂ 2002 00:00:00 -05:00

The live attenuated varicella vaccine was approved by the Food and Drug Administration in 1995 for routine use in healthy persons older than one year of age who are susceptible...

ORIGINAL ARTICLE: Outbreak of Varicella at a Day-Care Center despite Vaccination

Thu, 12Â DecÂ 2002 00:00:00 -05:00

Background: In seven studies of the effectiveness of the varicella vaccine conducted since it was licensed, the effectiveness was 71 to 100 percent against disease of any severity and 95...

CLINICAL PROBLEM-SOLVING: The Unusual Suspect

Thu, 05Â DecÂ 2002 00:00:00 -05:00

A previously healthy 17-year-old boy awoke with left-sided pleuritic chest pain. He also noticed mild dyspnea on exertion during track-and-field practice but reported no sputum production, fever, chills, or recent...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 35-2002 â A Nine-Year-Old Girl with Cold Intolerance, Visual-Field Defects, and a Suprasellar Tumor

R. Michael Scott and E. Tessa Hedley-Whyte — Thu, 14Â NovÂ 2002 00:00:00 -05:00

A nine-year-old right-handed girl was admitted to the hospital because of a suprasellar mass.

The girl had been well, with normal development, until about six months earlier, when her mother observed...

ORIGINAL ARTICLE: A Population-Based Study of Measles, Mumps, and Rubella Vaccination and Autism

Thu, 07Â NovÂ 2002 00:00:00 -05:00

Background: It has been suggested that vaccination against measles, mumps, and rubella (MMR) is a cause of autism.

Methods: We conducted a retrospective cohort study of all children born in Denmark...

PERSPECTIVE: Suspicions about the Safety of Vaccines

Thu, 07Â NovÂ 2002 00:00:00 -05:00

Vaccines represent one of the most effective interventions in medicine. They can protect whole populations from potentially dangerous diseases. Because vaccines are usually given to healthy people, especially children, any...

HEALTH POLICY REPORT: Testing Medications in Children

Robert Steinbrook, M.D. — Thu, 31Â OctÂ 2002 00:00:00 -05:00

When sulfanilamide became available in the 1930s, a liquid preparation that could be taken by small children was needed. Because the sulfa drug did not dissolve easily in water, a...

IMAGES IN CLINICAL MEDICINE: Commotio Cordis

Janette F. Strasburger, M.D. and Barry J. Maron, M.D. — Thu, 17Â OctÂ 2002 00:00:00 -05:00

Figure 1. A 13-year-old boy in good health was a batter in a Little League baseball game. He pivoted his body toward the field to avoid an inside pitch, was...

CLINICAL PRACTICE: Otitis Media

J. Owen Hendley, M.D. — Thu, 10Â OctÂ 2002 00:00:00 -05:00

An otherwise healthy 17-month-old boy had a cold accompanied by two days of rhinorrhea, cough, and fever (temperature of up to 38.8Â°C [102Â°F]). On day 5 he became fussy and...

REVIEW ARTICLE: Analgesics for the Treatment of Pain in Children

Charles B. Berde, M.D., Ph.D., and Navil F. Sethna, M.B., Ch.B. — Thu, 03Â OctÂ 2002 00:00:00 -05:00

Treatment of pain and suffering should be a priority for all clinicians. Previous reviews1 decried inadequate treatment of pain in infants and children. Surveys in the 1970s and 1980s2 reported...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 30-2002 â An Eight-Year-Old Girl with Fever, Hemoptysis, and Pulmonary Consolidations

Brian P. O'Sullivan, Lori A. Erickson, and John L. Niles — Thu, 26Â SepÂ 2002 00:00:00 -05:00

An eight-year-old girl was admitted to the hospital because of fever, cough, hemoptysis, and pulmonary consolidations.

She had been well until six days before admission, when she was visiting El Salvador....

EDITORIAL: Eat Dirt â The Hygiene Hypothesis and Allergic Diseases

Thu, 19Â SepÂ 2002 00:00:00 -05:00

There has been an epidemic of both autoimmune diseases (in which the immune response is dominated by type 1 helper T [Th1] cells, such as type 1 diabetes, Crohn's disease,...

REVIEW ARTICLE: The Effect of Infections on Susceptibility to Autoimmune and Allergic Diseases

Jean-FranÃ§ois Bach, M.D., D.Sc. — Thu, 19Â SepÂ 2002 00:00:00 -05:00

Infectious agents can induce autoimmune diseases in several experimental settings, some of which have clinical counterparts. A variety of mechanisms have been invoked to explain these observations, including molecular mimicry...

ORIGINAL ARTICLE: Environmental Exposure to Endotoxin and Its Relation to Asthma in School-Age Children

Thu, 19Â SepÂ 2002 00:00:00 -05:00

Background: In early life, the innate immune system can recognize both viable and nonviable parts of microorganisms. Immune activation may direct the immune response, thus conferring tolerance to allergens such...

IMAGES IN CLINICAL MEDICINE: Severe Abdominal Pain in a Girl

Carrie A. Czarnecki, M.D. and D. Fergus O'Coclain, M.D. — Thu, 05Â SepÂ 2002 00:00:00 -05:00

Figure 1. A 17-year-old girl with a history of bulimia and binge eating presented to the emergency room with severe, diffuse abdominal pain six hours after ingesting massive amounts of...

ORIGINAL ARTICLE: Decline in Physical Activity in Black Girls and White Girls during Adolescence

Thu, 05Â SepÂ 2002 00:00:00 -05:00

Background: Physical activity declines during adolescence, but the underlying reasons remain unknown.

Methods: We prospectively followed 1213 black girls and 1166 white girls enrolled in the National Heart, Lung, and Blood...

PERSPECTIVE: Physical Inactivity among Young People

Thu, 05Â SepÂ 2002 00:00:00 -05:00

It is widely recognized that the increasing proportion of overweight children and adolescents in the United States is a serious public health issue. Fifteen percent of young Americans are overweight...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 27-2002 â A 5 1/2-Year-Old Boy with Seizures and Progressive Deterioration of Cognitive and Motor Function

Robert S. Rust and Diane Karluk — Thu, 29Â AugÂ 2002 00:00:00 -05:00

A 5 1/2-year-old boy was admitted for video electroencephalographic monitoring to investigate his seizures.

The boy had been born to a 21-year-old woman by spontaneous vaginal delivery after an uncomplicated, full-term,...

CLINICAL PRACTICE: Adolescent Depression

David A. Brent, M.D., and Boris Birmaher, M.D. — Thu, 29Â AugÂ 2002 00:00:00 -05:00

A 16-year-old boy is brought by his parents to his primary care physician because of a decline in school performance, which began at least three years earlier and has become...

EDITORIAL: O157:H7 â Piecing Together the Jigsaw Puzzle

Thu, 22Â AugÂ 2002 00:00:00 -05:00

Nineteen years ago, the first report of two outbreaks of hemorrhagic colitis linked with Escherichia coli O157:H7 infection was published in the Journal.1 Both outbreaks were associated with hamburger consumption,...

ORIGINAL ARTICLE: An Outbreak of O157:H7 Infections among Visitors to a Dairy Farm

Thu, 22Â AugÂ 2002 00:00:00 -05:00

Background: Outbreaks of Escherichia coli O157:H7 infections have involved direct transmission from animals and their environment to humans. We describe an outbreak among visitors to a Pennsylvania dairy and petting...

ORIGINAL ARTICLE: Risperidone in Children with Autism and Serious Behavioral Problems

Thu, 01Â AugÂ 2002 00:00:00 -05:00

Background: Atypical antipsychotic agents, which block postsynaptic dopamine and serotonin receptors, have advantages over traditional antipsychotic medications in the treatment of adults with schizophrenia and may be beneficial in children...

PERSPECTIVE: The Autistic-Spectrum Disorders

Thu, 01Â AugÂ 2002 00:00:00 -05:00

Any person, talented or handicapped, whose social skills have been severely deficient since very early childhood, who started to talk late or whose communicative use of language is inadequate, and...

ORIGINAL ARTICLE: Dermoid Inclusion Cysts and Early Spinal Cord Tethering after Fetal Surgery for Myelomeningocele

Thu, 25Â JulÂ 2002 00:00:00 -05:00

02072523.xml Myelomeningocele occurs in approximately 1 of every 2000 live births.1 The prenatal incidence is higher, because many parents elect to terminate the pregnancy, once the diagnosis has been...

ORIGINAL ARTICLE: Osteoprotegerin Deficiency and Juvenile Paget's Disease

Thu, 18Â JulÂ 2002 00:00:00 -05:00

Background: Juvenile Paget's disease, an autosomal recessive osteopathy, is characterized by rapidly remodeling woven bone, osteopenia, fractures, and progressive skeletal deformity. The molecular basis is not known. Osteoprotegerin deficiency could...

ORIGINAL ARTICLE: Inactivating Mutations in the Gene for Thyroid Oxidase 2 () and Congenital Hypothyroidism

Thu, 11Â JulÂ 2002 00:00:00 -05:00

Background: Several genetic defects are associated with permanent congenital hypothyroidism. Immunologic, environmental, and iatrogenic (but not genetic) factors are known to induce transient congenital hypothyroidism, which spontaneously resolves within the...

REVIEW ARTICLE: Cystinosis

William A. Gahl, M.D., Ph.D., Jess G. Thoene, M.D., and Jerry A. Schneider, M.D. — Thu, 11Â JulÂ 2002 00:00:00 -05:00

Cystinosis is an autosomal recessive disorder with an estimated incidence of 1 case per 100,000 to 200,000 live births. The gene for cystinosis, CTNS, was mapped to chromosome 17p13 in...

ORIGINAL ARTICLE: Comparative Efficacy of Insect Repellents against Mosquito Bites

Mark S. Fradin, M.D., and John F. Day, Ph.D. — Thu, 04Â JulÂ 2002 00:00:00 -05:00

Background: The worldwide threat of arthropod-transmitted diseases, with their associated morbidity and mortality, underscores the need for effective insect repellents. Multiple chemical, botanical, and âalternativeâ repellent products are marketed to...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 19-2002 â A 13-Year-Old Girl with a Mass in the Left Parotid Gland and Regional Lymph Nodes

Trevor J.I. McGill and C.-L. Wu — Thu, 20Â JunÂ 2002 00:00:00 -05:00

A 13-year-old girl was admitted to the Massachusetts Eye and Ear Infirmary because of a mass in the left parotid gland.

The patient had been well until four or five months...

PERSPECTIVE: Preventing HIV Infection in Children

Thu, 13Â JunÂ 2002 00:00:00 -05:00

For a child, the risk of becoming infected with the human immunodeficiency virus (HIV) is a stark example of the difference between being born in a rich country and being...

ORIGINAL ARTICLE: Clinical Trial of Lamivudine in Children with Chronic Hepatitis B

Thu, 30Â MayÂ 2002 00:00:00 -05:00

Background: Lamivudine therapy is effective for chronic hepatitis B infection in adults. We evaluated the efficacy and tolerability of lamivudine as a treatment for chronic infection with hepatitis B virus...

CLINICAL PRACTICE: Head Lice

Richard J. Roberts, M.B., B.S., D.C.H., M.P.H. — Thu, 23Â MayÂ 2002 00:00:00 -05:00

An eight-year-old girl is sent home after the school nurse detects head lice. She will not be permitted to return to school until the absence of infestation is documented. What...

EDITORIAL: Continuing Medical Education in the

Thu, 18Â AprÂ 2002 00:00:00 -05:00

In this issue of the Journal, readers will find a quiz based on the article entitled âNonalcoholic Fatty Liver Disease,â by Angulo. This marks the beginning of a new continuing-medical-education...

ORIGINAL ARTICLE: Erythromycin-Resistant Group A Streptococci in Schoolchildren in Pittsburgh

Thu, 18Â AprÂ 2002 00:00:00 -05:00

Background: Resistance to erythromycin has been very uncommon among group A streptococci in the United States.

Methods: As part of a longitudinal study, we obtained surveillance throat cultures twice monthly and...

EDITORIAL: The Science and Politics of Screening Newborns

Thu, 04Â AprÂ 2002 00:00:00 -05:00

A major accomplishment of the public health system has been the development of programs of screening newborns for inherited and congenital metabolic disorders. This effort began in response to the...

ORIGINAL ARTICLE: Neuroblastoma Screening at One Year of Age

Thu, 04Â AprÂ 2002 00:00:00 -05:00

Background: Neuroblastoma is the second most common type of childhood tumor. It is not known whether screening for neuroblastoma at one year of age reduces the incidence of metastatic disease...

ORIGINAL ARTICLE: Screening of Infants and Mortality Due to Neuroblastoma

Thu, 04Â AprÂ 2002 00:00:00 -05:00

Background: Neuroblastoma, the most common extracranial solid tumor that occurs in early childhood, can be identified in the preclinical stages by the detection of catecholamines in the urine. However, it...

EDITORIAL: Childhood Obesity and a Diabetes Epidemic

Thu, 14Â MarÂ 2002 00:00:00 -05:00

Childhood obesity has reached epidemic proportions; worldwide, approximately 22 million children under five years of age are overweight.1 During the past three decades, the number of overweight children in the...

ORIGINAL ARTICLE: Prevalence of Impaired Glucose Tolerance among Children and Adolescents with Marked Obesity

Thu, 14Â MarÂ 2002 00:00:00 -05:00

Background: Childhood obesity, epidemic in the United States, has been accompanied by an increase in the prevalence of type 2 diabetes among children and adolescents. We determined the prevalence of...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 5-2002 â A 15-Year-Old Boy with a Retro-orbital Mass and Impaired Vision

Craig A. Hurwitz, M.D.,and William C. Faquin, M.D., Ph.D. — Thu, 14Â FebÂ 2002 00:00:00 -05:00

A 15-year-old boy was admitted to the hospital because of a retro-orbital mass.

The patient had been well until three weeks earlier, when he began to have right retro-orbital and frontal...

REVIEW ARTICLE: Community-Acquired Pneumonia in Children

Kenneth McIntosh, M.D. — Thu, 07Â FebÂ 2002 00:00:00 -05:00

Community-acquired pneumonia is a common and potentially serious infection that afflicts children throughout the world; it is fundamentally different in children and in adults. The annual incidence of pneumonia in...

ORIGINAL ARTICLE: Expression of p53 and Prognosis in Children with Malignant Gliomas

Thu, 07Â FebÂ 2002 00:00:00 -05:00

Background: The prognosis of children with high-grade gliomas is uncertain, even when clinical and histologic findings are considered. We investigated whether mutations in the TP53 gene or the degree of...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 3-2002 â A 17-Year-Old Boy with Biliary Obstruction

M. Susan Moyer and Fiona M. Graeme-Cook — Thu, 24Â JanÂ 2002 00:00:00 -05:00

A 17-year-old boy was admitted to the hospital because of evidence of biliary obstruction.

Seventeen months before admission, the patient was admitted to another hospital because of bloody diarrhea. The serum...

EDITORIAL: Premature Infants Grow Up

Thu, 17Â JanÂ 2002 00:00:00 -05:00

Infants of very low birth weight (less than 1500 g) account for 1 percent of the annual births in the United States (about 40,000 births per year) and for the...

ORIGINAL ARTICLE: Outcomes in Young Adulthood for Very-Low-Birth-Weight Infants

Thu, 17Â JanÂ 2002 00:00:00 -05:00

Background: Very-low-birth-weight infants (those weighing less than 1500 g) born during the initial years of neonatal intensive care have now reached young adulthood.

Methods: We compared a cohort of 242 survivors...

PERSPECTIVE: Outcomes of Very Low Birth Weight in Young Adults

Thu, 17Â JanÂ 2002 00:00:00 -05:00

Preterm delivery remains the primary public health challenge in obstetrics. Infants who weigh less than 2500 g account for 11 percent of all births in the United States but for...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 40-2001 â An Eight-Year-Old Boy with Fever, Headache, and Vertigo Two Days after Aural Trauma

Cliff A. Megerian and Tessa A. Hadlock — Thu, 27Â DecÂ 2001 00:00:00 -05:00

An eight-year-old boy was admitted to the Massachusetts Eye and Ear Infirmary because of fever, headache, vertigo, and vomiting.

The child had been well until two days before admission, when he...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 39-2001 â A Newborn Girl with Seizures and Persistent Hypoglycemia

Ab Sadeghi-Nejad and Fiona M. Graeme-Cook — Thu, 20Â DecÂ 2001 00:00:00 -05:00

A two-day-old girl was admitted to the hospital because of persistent hypoglycemia.

The infant had been born at 34 weeks' gestation to a 35-year-old mother after an uneventful pregnancy. The birth...

EDITORIAL: The Changing Face of Pediatric HIV-1 Infection

Thu, 22Â NovÂ 2001 00:00:00 -05:00

It has been nearly 20 years since the first cases of AIDS were reported in children in the United States, and the face of pediatric human immunodeficiency virus type 1...

ORIGINAL ARTICLE: Effect of Combination Therapy Including Protease Inhibitors on Mortality among Children and Adolescents Infected with HIV-1

Thu, 22Â NovÂ 2001 00:00:00 -05:00

Background: Combination therapy including protease inhibitors has been shown to be effective in treating adults infected with human immunodeficiency virus type 1 (HIV-1), but there are only limited data regarding...

IMAGES IN CLINICAL MEDICINE: Atloido-Occipital Dislocation in a Small Child after Air-Bag Deployment

Carlos A. Angel, M.D. and Richard A. Ehlers, M.D. — Thu, 25Â OctÂ 2001 00:00:00 -05:00

Figure 1. A two-year-old girl riding in an automobile was involved in a low-speed collision in which the air bags deployed. At the time of the crash she was sitting...

ORIGINAL ARTICLE: Reversal of Catabolism by Beta-Blockade after Severe Burns

Thu, 25Â OctÂ 2001 00:00:00 -05:00

Background: The catecholamine-mediated hypermetabolic response to severe burns causes increased energy expenditure and muscle-protein catabolism. We hypothesized that blockade of Î²-adrenergic stimulation with propranolol would decrease resting energy expenditure and...

REVIEW ARTICLE: Tourette's Syndrome

Joseph Jankovic, M.D. — Thu, 18Â OctÂ 2001 00:00:00 -05:00

Tourette's syndrome is a neurologic disorder named after the French neurologist Georges Gilles de la Tourette, who, in 1885, described nine patients with childhood-onset tics, accompanied in some by uncontrollable...

REVIEW ARTICLE: The Prevention of Pneumococcal Disease in Children

G. Scott Giebink, M.D. — Thu, 18Â OctÂ 2001 00:00:00 -05:00

Otitis media in children leads to more than 20 million visits to physicians annually in the United States.123 By three years of age, 80 percent of all children in the...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 29-2001 â A 14-Year-Old Boy with Abnormal Bones and a Sacral Mass

Richard M. Terek, M.D., and G. Petur Nielsen, M.D. — Thu, 20Â SepÂ 2001 00:00:00 -05:00

A 14-year-old boy was admitted to the hospital because of bone pain and skeletal abnormalities.

The patient had been vigorously active until one year before admission, when he began to have...

ORIGINAL ARTICLE: Cerebral Sinovenous Thrombosis in Children

Thu, 09Â AugÂ 2001 00:00:00 -05:00

Background: Cerebral sinovenous thrombosis in children is a serious disorder, and information is needed about its prevention and treatment.

Methods: The Canadian Pediatric Ischemic Stroke Registry was initiated in 1992 at...

ORIGINAL ARTICLE: The Pseudomonas Hot-Foot Syndrome

Thu, 02Â AugÂ 2001 00:00:00 -05:00

Background: Between March and May 1998, there was an outbreak of a clinically distinct skin eruption on the soles of the feet of children who used a community wading pool.

Methods:...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 23-2001 â An 18-Month-Old Girl with Persistent Diarrhea and Malnutrition

Christopher P. Duggan, M.D., M.P.H., and Joseph Misdraji, M.D. — Thu, 26Â JulÂ 2001 00:00:00 -05:00

An 18-month-old girl was admitted to the hospital because of diarrhea with intermittent constipation and vomiting.

The child had been born by cesarean section, performed because of a breech presentation, at...

REVIEW ARTICLE: Respiratory Syncytial Virus and Parainfluenza Virus

Caroline Breese Hall, M.D. — Thu, 21Â JunÂ 2001 00:00:00 -05:00

Respiratory syncytial virus (RSV), originally recovered from a colony of chimpanzees with coryza and designated chimpanzee coryza agent,12 and human parainfluenza virus types 1, 2, 3, and 4 have been...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 18-2001 â A 19-Month-Old Boy with Fever and Soft-Tissue Masses

William Jerry Durbin, Eugene J. Mark — Thu, 14Â JunÂ 2001 00:00:00 -05:00

A 19-month-old boy was admitted to the hospital because of a febrile illness and refusal to walk.

The child's immunizations were up to date. His mother was Portuguese, and his father...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 16-2001 â A 17-Year-Old Girl with Worsening Abdominal Pain, Fever, and Diarrhea after a Recent Cesarean Section

J. Thomas LaMont, Fiona M. Graeme-Cook — Thu, 24Â MayÂ 2001 00:00:00 -05:00

A 17-year-old girl (gravida 1, para 1) was admitted to the hospital because of abdominal pain, fever, and diarrhea.

The patient had been in good health before admission. Three weeks earlier,...

ORIGINAL ARTICLE: Association of Viral Genome with Graft Loss in Children after Cardiac Transplantation

Thu, 17Â MayÂ 2001 00:00:00 -05:00

Background: The survival of recipients of cardiac allografts is limited by rejection and coronary vasculopathy. The purpose of this study in children who had received heart transplants was to evaluate...

EDITORIAL: Primary Prevention of Childhood Lead Poisoning â The Only Solution

Thu, 10Â MayÂ 2001 00:00:00 -05:00

Lead poisoning in children was first recognized in its severe acute form, known as lead encephalopathy.1 This condition is characterized by seizures, coma, and â not infrequently â death, and...

ORIGINAL ARTICLE: The Effect of Chelation Therapy with Succimer on Neuropsychological Development in Children Exposed to Lead

Thu, 10Â MayÂ 2001 00:00:00 -05:00

Background: Thousands of children, especially poor children living in deteriorated urban housing, are exposed to enough lead to produce cognitive impairment. It is not known whether treatment to reduce blood...

EDITORIAL: Drug Treatment of Anxiety Disorders in Children

Thu, 26Â AprÂ 2001 00:00:00 -05:00

Anxiety disorders represent the most common group of serious psychiatric conditions affecting children and adolescents. In a community-based study, approximately 3 percent of 9-to-17-year-olds had a diagnosable anxiety disorder that...

EDITORIAL: Polysaccharide Conjugate Typhoid Vaccine

Thu, 26Â AprÂ 2001 00:00:00 -05:00

There is a growing appreciation of the huge health threat posed by increasingly resistant infectious diseases, especially in tropical areas of the developing world. Only with improved water, sanitation, housing,...

ORIGINAL ARTICLE: Fluvoxamine for the Treatment of Anxiety Disorders in Children and Adolescents

Thu, 26Â AprÂ 2001 00:00:00 -05:00

Background: Drugs that selectively inhibit serotonin reuptake are effective treatments for adults with mood and anxiety disorders, but limited data are available on the safety and efficacy of serotonin-reuptake inhibitors...

ORIGINAL ARTICLE: The Efficacy of a Vi Conjugate Vaccine in Two-to-Five-Year-Old Children

Thu, 26Â AprÂ 2001 00:00:00 -05:00

Background: Typhoid fever is common in developing countries. The licensed typhoid vaccines confer only about 70 percent immunity, do not protect young children, and are not used for routine vaccination....

EDITORIAL: Should We Operate on Children with Fluid in the Middle Ear?

Thu, 19Â AprÂ 2001 00:00:00 -05:00

A number of studies suggest that persistent middle-ear effusion in children has long-term effects on speech and language development, as well as development in general, although the studies vary in...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 12-2001 â A 16-Year-Old Boy with an Altered Mental Status and Muscle Rigidity

Mark H. Libenson, Jane M. Yang — Thu, 19Â AprÂ 2001 00:00:00 -05:00

A 16-year-old boy was admitted to the hospital because of an altered mental and emotional status.

At an uncertain interval before admission, the patient began to have diarrhea. One week before...

ORIGINAL ARTICLE: The Role of Adjuvant Adenoidectomy and Tonsillectomy in the Outcome of the Insertion of Tympanostomy Tubes

Thu, 19Â AprÂ 2001 00:00:00 -05:00

Background: Otitis media is the most common medical problem in young children. The usual surgical treatment is myringotomy with insertion of tympanostomy tubes. There is debate about the usefulness of...

ORIGINAL ARTICLE: Effect of Early or Delayed Insertion of Tympanostomy Tubes for Persistent Otitis Media on Developmental Outcomes at the Age of Three Years

Thu, 19Â AprÂ 2001 00:00:00 -05:00

Background: A main indication for the insertion of tympanostomy tubes in infants and young children is persistent otitis media with effusion, reflecting concern that this condition may cause lasting impairments...

ORIGINAL ARTICLE: The Effectiveness of the Varicella Vaccine in Clinical Practice

Thu, 29Â MarÂ 2001 00:00:00 -05:00

Background: A live attenuated varicella vaccine was approved for use in the United States in March 1995 and is recommended for all susceptible persons 12 months of age or older.

Methods:...

EDITORIAL: Varicella Vaccine â The First Six Years

Thu, 29Â MarÂ 2001 00:00:00 -05:00

The live attenuated varicella vaccine is the only vaccine licensed for the prevention of a disease caused by a human herpesvirus. The origin of the vaccine can be traced back...

ORIGINAL ARTICLE: The Japanese Experience with Vaccinating Schoolchildren against Influenza

Thu, 22Â MarÂ 2001 00:00:00 -05:00

Background: Influenza epidemics lead to increased mortality, principally among elderly persons and others at high risk, and in most developed countries, influenza-control efforts focus on the vaccination of this group....

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 7-2001 â A Male Infant with a Right Maxillary Mass

Mark S. Volk, G. Petur Nielsen — Thu, 08Â MarÂ 2001 00:00:00 -05:00

A 10-week-old boy was evaluated at Massachusetts Eye and Ear Infirmary because of progressive swelling in the region of the right side of the upper lip of two weeks' duration.

The...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 6-2001 â A 17-Year-Old Girl with Marked Jaundice and Weight Loss

Maureen M. Jonas, Fiona M. Graeme-Cook — Thu, 22Â FebÂ 2001 00:00:00 -05:00

A 17-year-old girl was admitted to the hospital because of progressive jaundice and weight loss.

The patient had been well until four months earlier, when she experienced the onset of malaise,...

EDITORIAL: The Apgar Score in the 21st Century

Thu, 15Â FebÂ 2001 00:00:00 -05:00

In 1952, Dr. Virginia Apgar developed a scoring system to evaluate the condition of neonates born at the Sloane Hospital for Women in New York City.1 Dr. Apgar, an obstetrical...

ORIGINAL ARTICLE: The Continuing Value of the Apgar Score for the Assessment of Newborn Infants

Brian M. Casey, M.D., Donald D. McIntire, Ph.D., and Kenneth J. Leveno, M.D. — Thu, 15Â FebÂ 2001 00:00:00 -05:00

Background: The 10-point Apgar score has been used to assess the condition and prognosis of newborn infants throughout the world for almost 50 years. Some investigators have proposed that measurement...

ORIGINAL ARTICLE: Efficacy of a Pneumococcal Conjugate Vaccine against Acute Otitis Media

Thu, 08Â FebÂ 2001 00:00:00 -05:00

Background: Ear infections are a common cause of illness during the first two years of life. New conjugate vaccines may be able to prevent a substantial portion of cases of...

EDITORIAL: Alarming Facts about the Health and Nutrition of Children in Tibet

Thu, 01Â FebÂ 2001 00:00:00 -05:00

Many people view Tibet as an exotic region where people live in rugged circumstances yet perpetuate values that are noble and mysterious. The study reported by Harris et al. in...

ORIGINAL ARTICLE: Nutritional and Health Status of Tibetan Children Living at High Altitudes

Thu, 01Â FebÂ 2001 00:00:00 -05:00

Background: Children living at high altitudes often have delayed growth, but whether growth retardation is related to altitude or to other factors is not known.

Methods: A multicultural health care team...

EDITORIAL: Predicting Cerebral Edema during Diabetic Ketoacidosis

Thu, 25Â JanÂ 2001 00:00:00 -05:00

Cerebral edema is a devastating complication of diabetic ketoacidosis and remains the leading cause of serious illness and death in children with diabetes mellitus.1 The causes of cerebral edema are...

ORIGINAL ARTICLE: Risk Factors for Cerebral Edema in Children with Diabetic Ketoacidosis

Thu, 25Â JanÂ 2001 00:00:00 -05:00

Background: Cerebral edema is an uncommon but devastating complication of diabetic ketoacidosis in children. Risk factors for this complication have not been clearly defined.

Methods: In this multicenter study, we identified...

ORIGINAL ARTICLE: Adrenomedullary Dysplasia and Hypofunction in Patients with Classic 21-Hydroxylase Deficiency

Thu, 09Â NovÂ 2000 00:00:00 -05:00

Background: Glucocorticoids are essential for the normal development and functioning of the adrenal medulla. Whether adrenomedullary structure and function are normal in patients with congenital adrenal hyperplasia is not known.

Methods:...

REVIEW ARTICLE: Primary Immunodeficiency Diseases Due to Defects in Lymphocytes

Rebecca H. Buckley, M.D. — Thu, 02Â NovÂ 2000 00:00:00 -05:00

The recognition of impaired immunity in children five decades ago12 spurred an exponential increase in knowledge of the functions of the immune system. More than 95 inherited immunodeficiency disorders have...

ORIGINAL ARTICLE: Central Diabetes Insipidus in Children and Young Adults

Thu, 05Â OctÂ 2000 00:00:00 -05:00

Background: Central diabetes insipidus is rare in children and young adults, and up to 50 percent of cases are idiopathic. The clinical presentation and the long-term course of this disorder...

ORIGINAL ARTICLE: Effect of Growth Hormone Treatment on the Adult Height of Children with Chronic Renal Failure

Thu, 28Â SepÂ 2000 00:00:00 -05:00

Background: Growth hormone treatment stimulates growth in short children with chronic renal failure. However, the extent to which this therapy increases final adult height is not known.

Methods: We followed 38...

IMAGES IN CLINICAL MEDICINE: Childhood Idiopathic Pulmonary Hemosiderosis

Charles Keller, M.D. and Claire Langston, M.D. — Thu, 14Â SepÂ 2000 00:00:00 -05:00

Figure 1. A three-year-old girl with a declining growth rate and recurrent iron-deficiency anemia presented with a five-day history of nonproductive cough, fever, and anorexia. In association with an upper...

ORIGINAL ARTICLE: Trial of a Supplemental Dose of Four Poliovirus Vaccines

Thu, 14Â SepÂ 2000 00:00:00 -05:00

Background: The immunogenicity of oral poliovirus vaccine (OPV), particularly the type 3 component, is lower in infants in most developing countries than in infants in industrialized countries. We conducted a...

ORIGINAL ARTICLE: Absence of Cardiac Toxicity of Zidovudine in Infants

Thu, 14Â SepÂ 2000 00:00:00 -05:00

Background: Some evidence suggests that perinatal exposure to zidovudine may cause cardiac abnormalities in infants. We prospectively studied left ventricular structure and function in infants born to mothers infected with...

ORIGINAL ARTICLE: Genetic and Clinical Features of Hemoglobin H Disease in Chinese Patients

Thu, 24Â AugÂ 2000 00:00:00 -05:00

Background: Normally, one pair of each of the two Î±-globin genes, Î±1 and Î±2, resides on each copy of chromosome 16. In hemoglobin H disease, three of these four Î±-globin...

ORIGINAL ARTICLE: Racecadotril in the Treatment of Acute Watery Diarrhea in Children

Thu, 17Â AugÂ 2000 00:00:00 -05:00

Background: Racecadotril (acetorphan), an enkephalinase inhibitor with antisecretory and antidiarrheal actions, is an effective and safe treatment for acute diarrhea in adults and children. Whether treatment with racecadotril and oral...

ORIGINAL ARTICLE: A Molecular Link between the Sudden Infant Death Syndrome and the Long-QT Syndrome

Thu, 27Â JulÂ 2000 00:00:00 -05:00

The sudden infant death syndrome (SIDS) remains the leading cause of death in the first year of life and has a devastating impact on the affected families.1234 Despite the fact...

ORIGINAL ARTICLE: Severe Hypothyroidism Caused by Type 3 Iodothyronine Deiodinase in Infantile Hemangiomas

Thu, 20Â JulÂ 2000 00:00:00 -05:00

Hemangiomas are the most common tumors of infancy, with a prevalence of 5 to 10 percent among one-year-olds. They are characterized by rapid growth in the first year of life,...

EDITORIAL: Antibiotics, and the HemolyticâUremic Syndrome

Thu, 29Â JunÂ 2000 00:00:00 -05:00

The hemolyticâuremic syndrome is characterized by hemolytic anemia, thrombocytopenia, and acute renal insufficiency. There are at least four major causes of the syndrome: bacterial and viral infections, which are responsible...

ORIGINAL ARTICLE: The Risk of the HemolyticâUremic Syndrome after Antibiotic Treatment of O157:H7 Infections

Thu, 29Â JunÂ 2000 00:00:00 -05:00

Background: Children with gastrointestinal infections caused by Escherichia coli O157:H7 are at risk for the hemolyticâuremic syndrome. Whether antibiotics alter this risk is unknown.

Methods: We conducted a prospective cohort study...

IMAGES IN CLINICAL MEDICINE: Bacterial Perianal Dermatitis

Heikki Peltola, M.D. — Thu, 22Â JunÂ 2000 00:00:00 -05:00

Figure 1. A four-year-old boy was seen by the pediatrician because of difficulty sleeping and intense pruritus in the anal region, with a three-day history of local redness in the...

ORIGINAL ARTICLE: Causes and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease

Thu, 22Â JunÂ 2000 00:00:00 -05:00

Background: The acute chest syndrome is the leading cause of death among patients with sickle cell disease. Since its cause is largely unknown, therapy is supportive. Pilot studies with improved...

ORIGINAL ARTICLE: Graft-Versus-Host Disease in Children Who Have Received a Cord-Blood or Bone Marrow Transplant from an HLA-Identical Sibling

Thu, 22Â JunÂ 2000 00:00:00 -05:00

Background: Umbilical-cord blood as an alternative to bone marrow for hematopoietic stem-cell transplantation may lower the risk of graft-versus-host disease (GVHD).

Methods: We studied the records of 113 recipients of cord...

REVIEW ARTICLE: Pediatric Surgery â First of Two Parts

N. Scott Adzick, M.D., and Michael L. Nance, M.D. — Thu, 01Â JunÂ 2000 00:00:00 -05:00

The scope of pediatric surgery is broad, encompassing the care of patients from before birth through the adolescent years and addressing a wide range of conditions, including congenital malformations, cancer,...

ORIGINAL ARTICLE: Mortality from Pneumonia in Children in the United States, 1939 through 1996

Thu, 11Â MayÂ 2000 00:00:00 -05:00

Background: and Methods: Pneumonia remains an important cause of childhood deaths throughout the world, but in developed countries, the mortality rate is decreasing. We reviewed death records for children in...

ORIGINAL ARTICLE: Ceftriaxone-Resistant Salmonella Infection Acquired by a Child from Cattle

Thu, 27Â AprÂ 2000 00:00:00 -05:00

Background: The emergence of resistance to antimicrobial agents within the salmonellae is a worldwide problem that has been associated with the use of antibiotics in livestock. Resistance to ceftriaxone and...

ORIGINAL ARTICLE: Outcome of Treatment in Children with Philadelphia ChromosomeâPositive Acute Lymphoblastic Leukemia

Thu, 06Â AprÂ 2000 00:00:00 -05:00

Background: Children with Philadelphia chromosomeâpositive acute lymphoblastic leukemia (Ph-positive ALL) have a poor prognosis, and there is no consensus on the optimal treatment for this variant of ALL.

Methods: We reviewed...

REVIEW ARTICLE: Sedation and Analgesia for Procedures in Children

Baruch Krauss, M.D., and Steven M. Green, M.D. — Thu, 30Â MarÂ 2000 00:00:00 -05:00

The management of acute pain and anxiety in children undergoing therapeutic and diagnostic procedures outside the operating room has developed substantially in the past 15 years. The widespread availability of...

EDITORIAL: Growth in Children with Chronic Lung Disease

Thu, 23Â MarÂ 2000 00:00:00 -05:00

In patients with cystic fibrosis, the inflammatory response in the airways to bacterial infection is excessive and deleterious. Thus, interrupting the inflammatory response is a goal of therapy. In a...

ORIGINAL ARTICLE: Risk of Persistent Growth Impairment after Alternate-Day Prednisone Treatment in Children with Cystic Fibrosis

Thu, 23Â MarÂ 2000 00:00:00 -05:00

Background: It is uncertain whether the growth impairment that occurs in children during long-term treatment with glucocorticoids persists after the medication is discontinued and ultimately affects adult height.

Methods: We evaluated...

ORIGINAL ARTICLE: Etanercept in Children with Polyarticular Juvenile Rheumatoid Arthritis

Thu, 16Â MarÂ 2000 00:00:00 -05:00

Background: We evaluated the safety and efficacy of etanercept, a soluble tumor necrosis factor receptor (p75):Fc fusion protein, in children with polyarticular juvenile rheumatoid arthritis who did not tolerate or...

ORIGINAL ARTICLE: Clinical and Genetic Features of EhlersâDanlos Syndrome Type IV, the Vascular Type

Thu, 09Â MarÂ 2000 00:00:00 -05:00

Background: EhlersâDanlos syndrome type IV, the vascular type, results from mutations in the gene for type III procollagen (COL3A1). Affected patients are at risk for arterial, bowel, and uterine rupture,...

IMAGES IN CLINICAL MEDICINE: Varicella Gangrenosa

Javier Tello, M.D. and Juan Bossano, M.D. — Thu, 02Â MarÂ 2000 00:00:00 -05:00

Figure 1. Four days after the onset of symptoms of varicella in a 16-month-old girl, violaceous lesions developed on her neck and trunk that became necrotic and had an erythematous...

EDITORIAL: Dieting and Exercise in Overweight, Lactating Women

Thu, 17Â FebÂ 2000 00:00:00 -05:00

In this issue of the Journal, Lovelady et al.1 report the results of a 10-week intervention trial in which 40 overweight, lactating women were randomly assigned either to a diet-and-exercise...

ORIGINAL ARTICLE: The Effect of Weight Loss in Overweight, Lactating Women on the Growth of Their Infants

Thu, 17Â FebÂ 2000 00:00:00 -05:00

Background: The retention of weight gained during pregnancy may contribute to obesity. Lactation should promote weight loss, but weight loss is highly variable among lactating women. The risks associated with...

IMAGES IN CLINICAL MEDICINE: Internal Ventricular Defibrillation

Arthur J. Moss, M.D. and James P. Daubert, M.D. — Thu, 10Â FebÂ 2000 00:00:00 -05:00

Figure 1. A 12-year-old boy with the familial long-QT syndrome had an automatic cardioverterâdefibrillator implanted after resuscitation from cardiac arrest. The device was programmed to wait 7.5 seconds before discharging...

EDITORIAL: Care of Children Who Are Dying of Cancer

Thu, 03Â FebÂ 2000 00:00:00 -05:00

Each year, about 12,400 children in the United States are given a diagnosis of cancer. Over the past 30 years, scientific and technological advances, the advent of specialized pediatric cancer...

SPECIAL ARTICLE: Symptoms and Suffering at the End of Life in Children with Cancer

Thu, 03Â FebÂ 2000 00:00:00 -05:00

Background: Cancer is the second leading cause of death in children, after accidents. Little is known, however, about the symptoms and suffering at the end of life in children with...

EDITORIAL: Is It Time to Give Influenza Vaccine to Healthy Infants?

Thu, 27Â JanÂ 2000 00:00:00 -05:00

Among the many respiratory viruses, influenza-virus has always stood out as different. Certainly, in adults, it is the most pathogenic. Not only did it cause in 1918â1919 one of the...

ORIGINAL ARTICLE: Influenza and the Rates of Hospitalization for Respiratory Disease among Infants and Young Children

Thu, 27Â JanÂ 2000 00:00:00 -05:00

Background: Young children may be at increased risk for serious complications from influenzavirus infection. However, in population-based studies it has been difficult to separate the effects of influenzavirus from those...

ORIGINAL ARTICLE: The Effect of Influenza on Hospitalizations, Outpatient Visits, and Courses of Antibiotics in Children

Thu, 27Â JanÂ 2000 00:00:00 -05:00

Background: Despite high annual rates of influenza in children, influenza vaccines are given to children infrequently. We measured the disease burden of influenza in a large cohort of healthy children...

REVIEW ARTICLE: Ingestion of Toxic Substances by Children

Michael Shannon, M.D., M.P.H. — Thu, 20Â JanÂ 2000 00:00:00 -05:00

Ingestion of a harmful substance is among the most common causes of injury to children less than six years of age. Fortunately, in most cases, the ingested agent has minimal...

ORIGINAL ARTICLE: Prediction of Adverse Outcomes in Children with Sickle Cell Disease

Thu, 13Â JanÂ 2000 00:00:00 -05:00

Background: The ability to identify infants with sickle cell anemia who are likely to have severe complications later in life would permit accurate prognostication and tailoring of therapy to match...

REVIEW ARTICLE: Disorders of Iron Metabolism

Nancy C. Andrews, M.D., Ph.D. — Thu, 23Â DecÂ 1999 00:00:00 -05:00

Iron has the capacity to accept and donate electrons readily, interconverting between ferric (Fe2+) and ferrous (Fe3+) forms. This capability makes it a useful component of cytochromes, oxygen-binding molecules (i.e.,...

ORIGINAL ARTICLE: Combination Therapy with Efavirenz, Nelfinavir, and Nucleoside Reverse-Transcriptase Inhibitors in Children Infected with Human Immunodeficiency Virus Type 1

Thu, 16Â DecÂ 1999 00:00:00 -05:00

Background: Consistent long-term viral suppression has been difficult to achieve in children with human immunodeficiency virus type 1 (HIV-1) infection. We tested the safety and antiviral efficacy of a novel...

EDITORIAL: Lessons from Secretin

Thu, 09Â DecÂ 1999 00:00:00 -05:00

In this issue of the Journal, Sandler and colleagues report the negative results of a double-blind, placebo-controlled trial of a single intravenous dose of synthetic human secretin in children with...

EDITORIAL: Lessons from Secretin

Thu, 09Â DecÂ 1999 00:00:00 -05:00

ORIGINAL ARTICLE: Lack of Benefit of a Single Dose of Synthetic Human Secretin in the Treatment of Autism and Pervasive Developmental Disorder

Thu, 09Â DecÂ 1999 00:00:00 -05:00

Background: Secretin is a peptide hormone that stimulates pancreatic secretion. After recent publicity about a child with autism whose condition markedly improved after a single dose of secretin, thousands of...

ORIGINAL ARTICLE: Lack of Benefit of a Single Dose of Synthetic Human Secretin in the Treatment of Autism and Pervasive Developmental Disorder

Thu, 09Â DecÂ 1999 00:00:00 -05:00

ORIGINAL ARTICLE: Leydig-Cell Tumors Caused by an Activating Mutation of the Gene Encoding the Luteinizing Hormone Receptor

Thu, 02Â DecÂ 1999 00:00:00 -05:00

The receptor for luteinizing hormone and chorionic gonadotropin plays a major part in normal and abnormal reproductive function.1234 In males, activation of the receptor regulates the development and function of...

EDITORIAL: Caring for Internationally Adopted Children

Thu, 11Â NovÂ 1999 00:00:00 -05:00

Since 1986, families in the United States have adopted more than 125,000 children from other countries. These children, who have usually been in institutional care before adoption, come from countries...

ORIGINAL ARTICLE: Extensive Transmission of from a Child

Thu, 11Â NovÂ 1999 00:00:00 -05:00

Background: and Methods: Young children rarely transmit tuberculosis. In July 1998, infectious tuberculosis was identified in a nine-year-old boy in North Dakota who was screened because extrapulmonary tuberculosis had been...

ORIGINAL ARTICLE: Transplantation of Thymus Tissue in Complete DiGeorge Syndrome

Thu, 14Â OctÂ 1999 00:00:00 -05:00

Background: The DiGeorge syndrome is a congenital disorder that affects the heart, parathyroid glands, and thymus. In complete DiGeorge syndrome, patients have severely reduced T-cell function.

Methods: We treated five infants...

EDITORIAL: Hepatitis C Infection in Children

Thu, 16Â SepÂ 1999 00:00:00 -05:00

Recent data indicate that an estimated 2.7 million people in the United States are chronically infected with hepatitis C virus (HCV).1 Hepatitis C has become the most common reason for...

ORIGINAL ARTICLE: Effects of Recombinant Leptin Therapy in a Child with Congenital Leptin Deficiency

Thu, 16Â SepÂ 1999 00:00:00 -05:00

Severely obese (ob/ob) mice are deficient in the adipocyte-derived hormone leptin, which acts on the hypothalamus to control appetite and energy expenditure.1 The administration of leptin to these mice corrects...

EDITORIAL: Rickets Today â Children Still Need Milk and Sunshine

Thu, 19Â AugÂ 1999 00:00:00 -05:00

Rickets has been a childhood scourge for centuries. Before the Industrial Revolution in England it was a disease of the affluent, because their style of clothing and the fact that...

IMAGES IN CLINICAL MEDICINE: Nutritional Rickets

Tom D. Thacher, M.D. — Thu, 19Â AugÂ 1999 00:00:00 -05:00

Figure 1. A 30-month-old girl had had progressive bowing of the legs since she began walking at the age of 11 months. She reported bilateral thigh pain. Physical examination revealed...

REVIEW ARTICLE: Common Musculoskeletal Tumors of Childhood and Adolescence

Carola A.S. Arndt, M.D., and William M. Crist, M.D. — Thu, 29Â JulÂ 1999 00:00:00 -05:00

Common malignant tumors of the musculoskeletal system in children, adolescents, and young adults (<30 years old) include rhabdomyosarcoma, osteosarcoma, and Ewing's sarcoma. Together they constitute about 10 percent of newly...

REVIEW ARTICLE: Hemangiomas in Children

Beth A. Drolet, M.D., Nancy B. Esterly, M.D., and Ilona J. Frieden, M.D. — Thu, 15Â JulÂ 1999 00:00:00 -05:00

Hemangiomas are the most common soft-tissue tumors of infancy, occurring in approximately 5 to 10 percent of one-year-old children. Despite the frequency of these tumors, their pathogenesis is not completely...

EDITORIAL: Predicting Outcome in Neuroblastoma

Thu, 24Â JunÂ 1999 00:00:00 -05:00

Neuroblastoma is a malignant tumor in children that arises from primordial neural-crest cells destined to populate the adrenal gland and sympathetic ganglia, hence its varied clinical presentation and course. It...

EDITORIAL: The Disappearance of Reye's Syndrome â A Public Health Triumph

Thu, 06Â MayÂ 1999 00:00:00 -05:00

Today, few young parents in the United States recognize the name Reye's syndrome or react strongly to it. A short time ago the response was far different. In the 1970s...

ORIGINAL ARTICLE: Reye's Syndrome in the United States from 1981 through 1997

Thu, 06Â MayÂ 1999 00:00:00 -05:00

Background: Reye's syndrome is characterized by encephalopathy and fatty degeneration of the liver, usually after influenza or varicella. Beginning in 1980, warnings were issued about the use of salicylates in...

REVIEW ARTICLE: Treatment of Attention-DeficitâHyperactivity Disorder

Josephine Elia, M.D., Paul J. Ambrosini, M.D., and Judith L. Rapoport, M.D. — Thu, 11Â MarÂ 1999 00:00:00 -05:00

Attention-deficitâhyperactivity disorder (ADHD) is a common but controversial syndrome characterized by developmentally inappropriate hyperactivity, impulsivity, and inattention.1 The disorder, which continues throughout life,234567 usually begins in childhood and is characterized...

EDITORIAL: Thirty Years of Bone Marrow Transplantation for Severe Combined Immunodeficiency

Thu, 18Â FebÂ 1999 00:00:00 -05:00

It has been 30 years since the first patient with combined T-cell and B-cell immunodeficiency was successfully treated with transplantation of bone marrow from an HLA-identical sibling.1 Since then, hundreds...

EDITORIAL: Growth Hormone Use in Normal, Short Children â A Plea for Reason

Thu, 18Â FebÂ 1999 00:00:00 -05:00

In the United States, recombinant growth hormone is approved for use in children for three conditions: growth hormone deficiency, Turner's syndrome, and chronic renal insufficiency before transplantation. Because the supply...

ORIGINAL ARTICLE: Effect of Growth Hormone Treatment on Adult Height of Children with Idiopathic Short Stature

Thu, 18Â FebÂ 1999 00:00:00 -05:00

Background: Short-term administration of growth hormone to children with idiopathic short stature results in increases in growth rate and standard-deviation scores for height. However, the effect of long-term growth hormone...

EDITORIAL: Respiratory Viruses in Acute Otitis Media

Thu, 28Â JanÂ 1999 00:00:00 -05:00

Acute otitis media is a common disease whose cause is complex. Recent estimates suggest that more than 12 million episodes of acute otitis media are treated annually in the United...

ORIGINAL ARTICLE: Prevalence of Various Respiratory Viruses in the Middle Ear during Acute Otitis Media

Terho Heikkinen, M.D., Monica Thint, M.D., and Tasnee Chonmaitree, M.D. — Thu, 28Â JanÂ 1999 00:00:00 -05:00

Background: Vaccines against respiratory viruses may be able to reduce the frequency of acute otitis media. Although the role of respiratory viruses in the pathogenesis of acute otitis media is...

REVIEW ARTICLE: Problems in the Management of Attention-DeficitâHyperactivity Disorder

Alan J. Zametkin, M.D., and Monique Ernst, M.D., Ph.D. — Thu, 07Â JanÂ 1999 00:00:00 -05:00

Despite several name changes over the past 50 years, the current diagnosis of attention-deficitâhyperactivity disorder (ADHD) shares the core group of symptoms â impulsivity, inattention, and motor restlessness â with...

ORIGINAL ARTICLE: A Defect in the Transport of Long-Chain Fatty Acids Associated with Acute Liver Failure

Thu, 10Â DecÂ 1998 00:00:00 -05:00

Fatty-acid oxidation has a major role in energy production during periods of fasting. When body glucose is depleted, fatty acids are mobilized from adipose tissue, taken up by the liver,...

ORIGINAL ARTICLE: Therapy with a Purified Plasminogen Concentrate in an Infant with Ligneous Conjunctivitis and Homozygous Plasminogen Deficiency

Thu, 03Â DecÂ 1998 00:00:00 -05:00

Ligneous conjunctivitis is a rare disease characterized by acute or chronic recurrent conjunctivitis in which the conjunctival membranes acquire a wood-like consistency, due primarily to deposits of fibrin.12 Corneal involvement...

SOUNDING BOARD: Competitive Athletes with Cardiovascular Disease â The Case of Nicholas Knapp

Thu, 26Â NovÂ 1998 00:00:00 -05:00

A variety of congenital cardiovascular abnormalities are the principal causes of sudden death in young competitive athletes,12345 and some of these tragedies have been widely publicized.6 The identification of important...

ORIGINAL ARTICLE: Mutations in the Connexin 26 Gene () among Ashkenazi Jews with Nonsyndromic Recessive Deafness

Thu, 19Â NovÂ 1998 00:00:00 -05:00

Background: Mutations in the GJB2 gene cause one form of nonsyndromic recessive deafness. Among Mediterranean Europeans, more than 80 percent of cases of nonsyndromic recessive deafness result from inheritance of...

EDITORIAL: Infanticide

Thu, 22Â OctÂ 1998 00:00:00 -05:00

Accounts of parents who kill their infants date to the beginning of recorded history.1 As recently as the early 1800s in Europe, up to a third of live-born infants were...

SPECIAL ARTICLE: Risk Factors for Infant Homicide in the United States

Thu, 22Â OctÂ 1998 00:00:00 -05:00

Background: Homicide is the leading cause of infant deaths due to injury. More than 80 percent of infant homicides are considered to be fatal child abuse. This study assessed the...

ORIGINAL ARTICLE: Myocardial Bridging in Children with Hypertrophic Cardiomyopathy â A Risk Factor for Sudden Death

Thu, 22Â OctÂ 1998 00:00:00 -05:00

Background: Myocardial bridging may cause compression of a coronary artery, and it has been suggested that myocardial ischemia may result. The clinical significance and prognostic value of myocardial bridging of...

EDITORIAL: Constipation in Children

Thu, 15Â OctÂ 1998 00:00:00 -05:00

Constipation is a frequent problem among infants and children. For example, in a U.S. study, 16 percent of parents of 22-month-olds reported that their children had constipation.1 In the United...

ORIGINAL ARTICLE: KashinâBeck Osteoarthropathy in Rural Tibet in Relation to Selenium and Iodine Status

Thu, 15Â OctÂ 1998 00:00:00 -05:00

Background: and Methods: KashinâBeck disease is a degenerative osteoarticular disorder that is endemic to certain areas of Tibet, where selenium deficiency is also endemic. Because selenium is involved in thyroid...

ORIGINAL ARTICLE: Intolerance of Cow's Milk and Chronic Constipation in Children

Thu, 15Â OctÂ 1998 00:00:00 -05:00

Background: Chronic diarrhea is the most common gastrointestinal symptom of intolerance of cow's milk among children. On the basis of a prior open study, we hypothesized that intolerance of cow's...

EDITORIAL: Osteogenesis Imperfecta â Managing Brittle Bones

Thu, 01Â OctÂ 1998 00:00:00 -05:00

Osteogenesis imperfecta is an autosomal dominant disorder of the connective tissue, which is also known as âbrittle-bone diseaseâ because it renders those affected susceptible to fractures after minimal trauma. Clinically...

ORIGINAL ARTICLE: Cyclic Administration of Pamidronate in Children with Severe Osteogenesis Imperfecta

Thu, 01Â OctÂ 1998 00:00:00 -05:00

Background: Severe osteogenesis imperfecta is a disorder characterized by osteopenia, frequent fractures, progressive deformity, loss of mobility, and chronic bone pain. There is no effective therapy for the disorder. We...

ORIGINAL ARTICLE: Cushing's Syndrome Caused by Corticotropin Secretion by Pulmonary Tumorlets

Thu, 24Â SepÂ 1998 00:00:00 -05:00

The term âpulmonary tumorletsâ describes multiple microscopic nests of neuroendocrine cells in the lungs.1 Like bronchial carcinoid tumors and small-cell carcinomas, pulmonary tumorlets arise from Kulchitsky's cells. In the lung...

EDITORIAL: Regulation of Firearms

Thu, 17Â SepÂ 1998 00:00:00 -05:00

In the United States, the rates of injury and death due to firearms and the rate of crimes committed with firearms are far higher than those in any other industrialized...

SPECIAL ARTICLE: Support for New Policies to Regulate Firearms â Results of Two National Surveys

Thu, 17Â SepÂ 1998 00:00:00 -05:00

Background: New policy options are emerging in the debate regarding the regulation of firearms in the United States. These options include the treatment of firearms as consumer products, the design...

EDITORIAL: Pancreatitis and Mutations of the Cystic Fibrosis Gene

Thu, 03Â SepÂ 1998 00:00:00 -05:00

In patients with cystic fibrosis, the inability to maintain the luminal hydration of ducts that contain or secrete large molecules may affect multiple organs. The protein product of the gene...

ORIGINAL ARTICLE: Increased Bone Mass as a Result of Estrogen Therapy in a Man with Aromatase Deficiency

Thu, 27Â AugÂ 1998 00:00:00 -05:00

During childhood and young adulthood, the skeleton accrues virtually all the bone mineral it will ever have.1 Since the aging process is associated with bone loss, the more bone mass...

ORIGINAL ARTICLE: Clinical Significance of Minimal Residual Disease in Childhood Acute Lymphoblastic Leukemia

Thu, 27Â AugÂ 1998 00:00:00 -05:00

Background: and Methods: The implications of the detection of residual disease after treatment of acute lymphoblastic leukemia (ALL) are unclear. We conducted a prospective study at 11 centers to determine...

EDITORIAL: The Treatment of Croup with Glucocorticoids

Thu, 20Â AugÂ 1998 00:00:00 -05:00

In this issue of the Journal, Johnson et al. report that in children with moderately severe croup, a single dose of intramuscular dexamethasone (0.6 mg per kilogram of body weight)...

ORIGINAL ARTICLE: A Comparison of Nebulized Budesonide, Intramuscular Dexamethasone, and Placebo for Moderately Severe Croup

Thu, 20Â AugÂ 1998 00:00:00 -05:00

Background: In children with croup, treatment with nebulized budesonide decreases symptoms, but it is uncertain how budesonide compares with dexamethasone, the conventional therapy for croup, and whether either reduces the...

ORIGINAL ARTICLE: Screening for Hypertrophic Cardiomyopathy in Young Athletes

Thu, 06Â AugÂ 1998 00:00:00 -05:00

Background: For more than 20 years in Italy, young athletes have been screened before participating in competitive sports. We assessed whether this strategy results in the prevention of sudden death...

ORIGINAL ARTICLE: Prevention of a First Stroke by Transfusions in Children with Sickle Cell Anemia and Abnormal Results on Transcranial Doppler Ultrasonography

Thu, 02Â JulÂ 1998 00:00:00 -05:00

Background: Blood transfusions prevent recurrent stroke in children with sickle cell anemia, but the value of transfusions in preventing a first stroke is unknown. We used transcranial Doppler ultrasonography to...

EDITORIAL: Sickle Cell Disease â New Treatments, New Questions

Thu, 02Â JulÂ 1998 00:00:00 -05:00

The history of sickle cell disease shows how tortuous the road can be from the discovery of the mechanism of a disease to improvement in the care of affected patients....

EDITORIAL: Gaining a Perspective on Childhood Seizures

Thu, 25Â JunÂ 1998 00:00:00 -05:00

Three articles in this and the June 11 issue of the Journal give us an opportunity to reexamine issues that are critical to how we perceive and respond to seizures...

REVIEW ARTICLE: Nonaccidental Head Injury in Infants â The âShaken-Baby Syndromeâ

Thu, 18Â JunÂ 1998 00:00:00 -05:00

Trauma is the most common cause of death in childhood, and inflicted head injury is the most common cause of traumatic death in infancy.123 Beginning with the classic descriptions of...

ORIGINAL ARTICLE: An Experimental Model of Sudden Death Due to Low-Energy Chest-Wall Impact (Commotio Cordis)

Thu, 18Â JunÂ 1998 00:00:00 -05:00

Background: The syndrome of sudden death due to low-energy trauma to the chest wall (commotio cordis) has been described in young sports participants, but the mechanism is unknown.

Methods: We developed...

EDITORIAL: Prolongation of the QT Interval and the Sudden Infant Death Syndrome

Thu, 11Â JunÂ 1998 00:00:00 -05:00

The unexpected and unexplained death of an infant is a tragic occurrence. When the infant is less than one year of age and the cause remains unknown despite a thorough...

ORIGINAL ARTICLE: Male Hypogonadism Due to a Mutation in the Gene for the Î²-Subunit of Follicle-Stimulating Hormone

Thu, 11Â JunÂ 1998 00:00:00 -05:00

Normal pubertal development and fertility depend on the intricate interplay of hypothalamic, pituitary, and gonadal factors. Crucial in this respect are normal secretory patterns of follicle-stimulating hormone and luteinizing hormone....

ORIGINAL ARTICLE: Long-Term Intellectual and Behavioral Outcomes of Children with Febrile Convulsions

Thu, 11Â JunÂ 1998 00:00:00 -05:00

Background: Hospital-based studies have reported that children with febrile convulsions have subsequent mental retardation and behavior problems. In contrast, population-based studies have reported a better outcome.

Methods: We identified 398 children...

ORIGINAL ARTICLE: Long-Term Prognosis of Seizures with Onset in Childhood

Thu, 11Â JunÂ 1998 00:00:00 -05:00

Background: The long-term prognosis of seizures that begin in childhood is uncertain.

Methods: We prospectively studied 245 children from the catchment area of Turku University Hospital in Turku, Finland, who had...

ORIGINAL ARTICLE: Prolongation of the QT Interval and the Sudden Infant Death Syndrome

Thu, 11Â JunÂ 1998 00:00:00 -05:00

Background: The sudden infant death syndrome (SIDS) is multifactorial in origin, but its causes remain unknown. We previously proposed that prolongation of the QT interval on the electrocardiogram, possibly resulting...

EDITORIAL: When Should Heart Disease Prevention Begin?

Thu, 04Â JunÂ 1998 00:00:00 -05:00

Early in the next century, cardiovascular disease will probably be the number-one killer worldwide.1 Despite important successes in the past 30 years, unfavorable trends in some coronary risk factors may...

NEJM â Collection Updates for Child Health

CORRESPONDENCE: Responses to 2009 H1N1 Vaccine in Children 3 to 17 Years of Age

EDITORIAL: Rotavirus Vaccine â A Powerful Tool to Combat Deaths from Diarrhea

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 3-2010 â A 5-Month-Old Boy with Developmental Delay and Irritability

ORIGINAL ARTICLE: Vaccine-Acquired Rotavirus in Infants with Severe Combined Immunodeficiency

ORIGINAL ARTICLE: Effect of Rotavirus Vaccination on Death from Childhood Diarrhea in Mexico

ORIGINAL ARTICLE: Effect of Human Rotavirus Vaccine on Severe Diarrhea in African Infants

ORIGINAL ARTICLE: Pediatric Hospitalizations Associated with 2009 Pandemic Influenza A (H1N1) in Argentina

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 41-2009 â A 16-Year-Old Boy with Hypothermia and Frostbite

OTHER POINTS OF VIEW: When to Consider the Use of Antibiotics in the Treatment of 2009 H1N1 InfluenzaâAssociated Pneumonia

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 38-2009 â A 16-Year-Old Boy with Paroxysmal Headaches and Visual Changes

PERSPECTIVE: Defeating Rotavirus? The Global Recommendation for Rotavirus Vaccination

CORRESPONDENCE: Risk of Confusion in Dosing Tamiflu Oral Suspension in Children

EDITORIAL: Antimicrobial Prophylaxis for Urinary Tract Infection in Children

ORIGINAL ARTICLE: Antibiotic Prophylaxis and Recurrent Urinary Tract Infection in Children

PERSPECTIVE: How Health Care Reform Can Benefit Children and Adolescents

EDITORIAL: Blood-Pressure Control and Delay in Progression of Kidney Disease in Children

REVIEW ARTICLE: Atypical HemolyticâUremic Syndrome

ORIGINAL ARTICLE: Cisplatin versus Cisplatin plus Doxorubicin for Standard-Risk Hepatoblastoma

ORIGINAL ARTICLE: Strict Blood-Pressure Control and Progression of Renal Failure in Children

PERSPECTIVE: When Diseases Disappear â The Case of Familial Dysautonomia

IMAGES IN CLINICAL MEDICINE: Living with One Hemisphere â A Large Porencephalic Cyst

ORIGINAL ARTICLE: Recurrence of Bile Salt Export Pump Deficiency after Liver Transplantation

ORIGINAL ARTICLE: Treatment of Medulloblastoma with Hedgehog Pathway Inhibitor GDC-0449

BOOK REVIEW:

CORRESPONDENCE: Codeine, Ultrarapid-Metabolism Genotype, and Postoperative Death

REVIEW ARTICLE: The Sudden Infant Death Syndrome

CORRESPONDENCE: Vision 1 Year after Gene Therapy for Leber's Congenital Amaurosis

IMAGES IN CLINICAL MEDICINE: Testicular Torsion in a Hydrocele

BOOK REVIEW:

ORIGINAL ARTICLE: AGC1 Deficiency Associated with Global Cerebral Hypomyelination

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 23-2009 â A 13-Year-Old Boy with Headache, Nausea, Seizures, and Hypertension

BOOK REVIEW:

BOOK REVIEW:

BOOK REVIEW:

IMAGES IN CLINICAL MEDICINE: Ingested Magnets

ORIGINAL ARTICLE: Treating Childhood Acute Lymphoblastic Leukemia without Cranial Irradiation

BOOK REVIEW:

BOOK REVIEW:

PERSPECTIVE: Litigation, Regulation, and Education â Protecting the Public's Health through Childhood Immunization

PERSPECTIVE: When Vaccine Injury Claims Go to Court

BOOK REVIEW:

ORIGINAL ARTICLE: An Autoinflammatory Disease Due to Homozygous Deletion of the Locus

BOOK REVIEW:

ORIGINAL ARTICLE: Inactivating Mutations in a Patient with Premature Pubarche

BOOK REVIEW:

EDITORIAL: The Challenge of Managing Wheezing in Infants

ORIGINAL ARTICLE: Antivenom for Critically Ill Children with Neurotoxicity from Scorpion Stings

ORIGINAL ARTICLE: Epinephrine and Dexamethasone in Children with Bronchiolitis

SPECIAL ARTICLE: Vaccine Refusal, Mandatory Immunization, and the Risks of Vaccine-Preventable Diseases

BOOK REVIEW:

BOOK REVIEW:

EDITORIAL: Which Drug for the Pregnant Woman with Epilepsy?

ORIGINAL ARTICLE: Cognitive Function at 3 Years of Age after Fetal Exposure to Antiepileptic Drugs

IMAGES IN CLINICAL MEDICINE: Gingival and Periungual Vasculopathy of Juvenile Dermatomyositis

CORRESPONDENCE: Ending Propylthiouracil-Induced Liver Failure in Children

CLINICAL PRACTICE: Evaluation and Management of Enuresis

ORIGINAL ARTICLE: Genetic Compensation in a Human Genomic Disorder

BOOK REVIEW:

CORRESPONDENCE: Melamine and Nephrolithiasis in Children in Taiwan

CORRESPONDENCE: Ultrasonographic Evaluation of Melamine-Exposed Children in Hong Kong

EDITORIAL: Melamine, Powdered Milk, and Nephrolithiasis in Chinese Infants

ORIGINAL ARTICLE: Melamine-Contaminated Powdered Formula and Urolithiasis in Young Children

BOOK REVIEW:

PERSPECTIVE: Expanding Coverage for Children â The Democrats' Power and SCHIP Reauthorization

PERSPECTIVE: Investing in Health Care Reform

IMAGES IN CLINICAL MEDICINE: A Tuba Player with Air in the Parotid Gland

CORRESPONDENCE: Prevalence of Mitochondrial 1555AâG Mutation in European Children

ORIGINAL ARTICLE: Mutations in in Autosomal Nonsyndromic Mental Retardation

ORIGINAL ARTICLE: The Burden of Respiratory Syncytial Virus Infection in Young Children

CORRESPONDENCE: Eculizumab for Congenital Atypical HemolyticâUremic Syndrome

EDITORIAL: Gene Therapy Fulfilling Its Promise

CLINICAL THERAPEUTICS: Behavioral Management for Anorexia Nervosa

ORIGINAL ARTICLE: Gene Therapy for Immunodeficiency Due to Adenosine Deaminase Deficiency

EDITORIAL: Practice Imperfect â Treatment for Wheezing in Preschoolers

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 3-2009 â A 9-Month-Old Boy with Seizures

ORIGINAL ARTICLE: Preemptive Use of High-Dose Fluticasone for Virus-Induced Wheezing in Young Children

ORIGINAL ARTICLE: Oral Prednisolone for Preschool Children with Acute Virus-Induced Wheezing

ORIGINAL ARTICLE: Effect of Pneumococcal Conjugate Vaccine on Pneumococcal Meningitis

BOOK REVIEW:

NEJM â Collection Updates for Child Health

EDITORIAL: Rotavirus Vaccine â A Powerful Tool to Combat Deaths from Diarrhea

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 3-2010 â A 5-Month-Old Boy with Developmental Delay and Irritability

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 41-2009 â A 16-Year-Old Boy with Hypothermia and Frostbite

OTHER POINTS OF VIEW: When to Consider the Use of Antibiotics in the Treatment of 2009 H1N1 InfluenzaâAssociated Pneumonia

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 38-2009 â A 16-Year-Old Boy with Paroxysmal Headaches and Visual Changes

REVIEW ARTICLE: Atypical HemolyticâUremic Syndrome

PERSPECTIVE: When Diseases Disappear â The Case of Familial Dysautonomia

IMAGES IN CLINICAL MEDICINE: Living with One Hemisphere â A Large Porencephalic Cyst

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 23-2009 â A 13-Year-Old Boy with Headache, Nausea, Seizures, and Hypertension

PERSPECTIVE: Litigation, Regulation, and Education â Protecting the Public's Health through Childhood Immunization

PERSPECTIVE: Expanding Coverage for Children â The Democrats' Power and SCHIP Reauthorization

CORRESPONDENCE: Prevalence of Mitochondrial 1555AâG Mutation in European Children

CORRESPONDENCE: Eculizumab for Congenital Atypical HemolyticâUremic Syndrome

EDITORIAL: Practice Imperfect â Treatment for Wheezing in Preschoolers

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 3-2009 â A 9-Month-Old Boy with Seizures

EDITORIAL: Pediatric Anxiety â Underrecognized and Undertreated

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 37-2008 â A 17-Year-Old Boy with a Pituitary Tumor and Skull Abnormalities

EDITORIAL: 17q21 Variants and Asthma â Questions and Answers

EDITORIAL: Monovalent Oral Poliovirus Vaccines â A Good Tool but Not a Total Solution

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 32-2008 â A 10-Year-Old Girl with Recurrent Oral Lesions and Cutaneous Bullae

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 32-2008 â A 10-Year-Old Girl with Recurrent Oral Lesions and Cutaneous Bullae

PERSPECTIVE: Storm over Statins â The Controversy Surrounding Pharmacologic Treatment of Children

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 29-2008 â A 19-Year-Old Man with Weight Loss and Abdominal Pain

EDITORIAL: Human Papillomavirus Vaccination â Reasons for Caution

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 21-2008 â An 11-Month-Old Boy with Fever and Pulmonary Infiltrates

EDITORIAL: Neuroblastoma â Linking a Common Allele to a Rare Disease

PERSPECTIVE: Vaccines and Autism Revisited â The Hannah Poling Case

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 10-2008 â A 10-Year-Old Girl with Dyspnea on Exertion

PERSPECTIVE: Taking Your Child's Breath Away â The Extension of Asthma's Global Reach

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 7-2008 â A 17-Year-Old Girl with Chest Pain and Hemoptysis

PERSPECTIVE: The Proxy War â SCHIP and the Government's Role in Health Care Reform