NEJM â Collection Updates for Endocrinology

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Î±-Adrenergic Receptors and Type 2 Diabetes

Fiona M. Gribble, D.Phil., B.M., B.Ch. — Thu, 28Â JanÂ 2010 00:00:00 -05:00

The global epidemic of type 2 diabetes mellitus is undoubtedly driven by soaring levels of obesity. Although increased body fat impairs the action of insulin, the risk of type 2...

REVIEW ARTICLE: WilliamsâBeuren Syndrome

Barbara R. Pober, M.D. — Thu, 21Â JanÂ 2010 00:00:00 -05:00

WilliamsâBeuren syndrome (also known as Williams' syndrome; Online Mendelian Inheritance in Man [OMIM] number, 194050), a multisystem disorder, is caused by deletion of the WilliamsâBeuren syndrome chromosome region, spanning 1.5...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 1-2010 â A 75-Year-Old Man with Hypertension, Hyperglycemia, and Edema

Thu, 14Â JanÂ 2010 00:00:00 -05:00

Dr. Jennifer L. Lyons (Medicine): A 75-year-old man was admitted to this hospital because of the recent onset of hypertension, hyperglycemia, and edema.

The patient had been in his usual state...

IMAGES IN CLINICAL MEDICINE: Necrolytic Migratory Erythema

Laura McGevna, M.D. and Zarry Tavakkol, M.D. — Thu, 07Â JanÂ 2010 00:00:00 -05:00

A 63-year-old woman with a 4 1/2-year history of diabetes mellitus presented with an ulcerating rash, primarily on the shins, groin, and face (Panel A); cheilitis (Panel B); and glossitis....

CLINICAL PROBLEM-SOLVING: A Crisis in Late Pregnancy

Thu, 03Â DecÂ 2009 00:00:00 -05:00

A 31-year-old woman in the 37th week of an uncomplicated pregnancy presented to the emergency department with sudden onset of severe bitemporal headache and shortness of breath. Her medical history...

ORIGINAL ARTICLE: Normal Spermatogenesis in a Man with Mutant Luteinizing Hormone

Thu, 05Â NovÂ 2009 00:00:00 -05:00

Men with mutations in LHB, the gene encoding the beta subunit of luteinizing hormone (LHB), have azoospermia with absent or few fetal Leydig cells. We report a mutation in LHB...

CORRESPONDENCE: FOXP3 Forkhead Domain Mutation and Regulatory T Cells in the IPEX Syndrome

Thu, 22Â OctÂ 2009 00:00:00 -05:00

To the Editor: The immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome, a rare disorder characterized by multiorgan autoimmunity, often results in death in infancy.1 The IPEX syndrome results from mutations...

IMAGES IN CLINICAL MEDICINE: Cerebrospinal Fluid Leak

Theo Diekman, M.D., Ph.D. and Bram Mees, M.D. — Thu, 01Â OctÂ 2009 00:00:00 -05:00

A 50-year-old postmenopausal woman with asthma and allergic rhinitis presented with a 6-month history of unilateral rhinorrhea. When she woke in the mornings, she noticed a stain (halo) on her...

CORRESPONDENCE: Luteinizing HormoneâReleasing Hormone and Postmenopausal Flushing

Hans de Boer, M.D., Ph.D., Petri van Gastel, M.D., and Adriaan van Sorge, Ph.D. — Thu, 17Â SepÂ 2009 00:00:00 -05:00

To the Editor: The trigger for postmenopausal flushing is incompletely understood1; therefore, it has been difficult to develop new treatments. Neuroendocrine mechanisms, such as increased activity of luteinizing hormoneâreleasing hormone...

BOOK REVIEW:

Thu, 20Â AugÂ 2009 00:00:00 -05:00

Many fields in medicine intersect, thereby promoting clinical and research collaborations. Nephrology and endocrinology encompass a number of common topics; indeed, some departments and practices are joint ventures that include...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 24-2009 â A 26-Year-Old Woman with Painful Swelling of the Neck

John H. Stone, M.D., M.P.H., Paul A. Caruso, M.D., and Vikram Deshpande, M.D. — Thu, 30Â JulÂ 2009 00:00:00 -05:00

Dr. John H. Stone: A 26-year-old woman was seen in the rheumatology clinic of this hospital because of painful swelling on both sides of the neck.

She had been well until...

CORRESPONDENCE: Muscle Glycogenosis Due to Phosphoglucomutase 1 Deficiency

Thu, 23Â JulÂ 2009 00:00:00 -05:00

To the Editor: Muscle glycogen storage diseases are rare inborn diseases caused by errors of metabolism associated with either dynamic, exercise-related symptoms or permanent muscle weakness. The most common glycogen...

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...

CLINICAL PROBLEM-SOLVING: A Fragile Balance

Thu, 02Â JulÂ 2009 00:00:00 -05:00

A 31-year-old man presented to the emergency department with pain in the left shoulder. He had tripped over the shoulder strap of his backpack earlier in the day and noted...

ORIGINAL ARTICLE: Isolated Familial Hypogonadotropic Hypogonadism and a Mutation

Thu, 25Â JunÂ 2009 00:00:00 -05:00

We investigated whether mutations in the gene encoding gonadotropin-releasing hormone 1 (GNRH1) might be responsible for idiopathic hypogonadotropic hypogonadism (IHH) in humans. We identified a homozygous GNRH1 frameshift mutation, an...

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...

IMAGES IN CLINICAL MEDICINE: Graves' Hyperthyroidism

Gabriel Santino Iraci, M.D. and Carolina Fux-Otta, M.D. — Thu, 11Â JunÂ 2009 00:00:00 -05:00

A 42-year-old woman presented with palpitations, anxiety, tremor, and weight loss. She had received a diagnosis of Graves' disease 15 years previously but had discontinued therapy more than 5 years...

REVIEW ARTICLE: Predisposing Factors for Adrenal Insufficiency

Stefan R. Bornstein, M.D. — Thu, 28Â MayÂ 2009 00:00:00 -05:00

Adrenal insufficiency â the clinical manifestation of deficient production or action of glucocorticoids â is a life-threatening disorder that may result from either primary adrenal failure or secondary adrenal disease...

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....

CLINICAL IMPLICATIONS OF BASIC RESEARCH: The Origins of Brown Adipose Tissue

Sven EnerbÃ¤ck, M.D., Ph.D. — Thu, 07Â MayÂ 2009 00:00:00 -05:00

What is the source of brown adipose tissue? During the formation of the three germ layers (gas trulation), the mesoderm fills the void between the outer shell (ectoderm) and the...

BOOK REVIEW:

Thu, 30Â AprÂ 2009 00:00:00 -05:00

Pancreatic Cancer is the latest title in the M.D. Anderson Solid Tumor Oncology Series from Springer. It is the second book in the series to address pancreatic cancer; the first...

BOOK REVIEW:

Thu, 16Â AprÂ 2009 00:00:00 -05:00

Clinical endocrine oncology is an unusual hybrid specialty in that it involves both oncology and endocrinology, two disciplines that differ vastly. In fact, endocrinologists seldom appear to be interested in...

BOOK REVIEW:

Thu, 16Â AprÂ 2009 00:00:00 -05:00

This excellent book is a comprehensive and succinct summary of the diagnosis and management of various types of pituitary disorders. The contributors, who are leaders in the field, have written...

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...

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...

EDITORIAL: Brown Adipose Tissue â When It Pays to Be Inefficient

Francesco S. Celi, M.D. — Thu, 09Â AprÂ 2009 00:00:00 -05:00

Obesity has reached epidemic proportions, and complications related to obesity contribute substantially to health care costs and mortality. Since the accumulation of fat is the net result of a prolonged...

ORIGINAL ARTICLE: Functional Brown Adipose Tissue in Healthy Adults

Thu, 09Â AprÂ 2009 00:00:00 -05:00

Using positron-emission tomography (PET), we found that cold-induced glucose uptake was increased by a factor of 15 in paracervical and supraclavicular adipose tissue in five healthy subjects. We obtained biopsy...

ORIGINAL ARTICLE: Identification and Importance of Brown Adipose Tissue in Adult Humans

Thu, 09Â AprÂ 2009 00:00:00 -05:00

Background: Obesity results from an imbalance between energy intake and expenditure. In rodents and newborn humans, brown adipose tissue helps regulate energy expenditure by thermogenesis mediated by the expression of...

ORIGINAL ARTICLE: Cold-Activated Brown Adipose Tissue in Healthy Men

Thu, 09Â AprÂ 2009 00:00:00 -05:00

Background: Studies in animals indicate that brown adipose tissue is important in the regulation of body weight, and it is possible that individual variation in adaptive thermogenesis can be attributed...

IMAGES IN CLINICAL MEDICINE: Neonatal Breast Enlargement

Ahdi Amer, M.D. and Howard Fischer, M.D. — Thu, 02Â AprÂ 2009 00:00:00 -05:00

An 8-day-old girl presented with bilateral breast enlargement. She was born at term; the 1-minute and 5-minute Apgar scores were 8 and 9, respectively. She was feeding well and showed...

IMAGES IN CLINICAL MEDICINE: Mediastinal Extension of a Goiter

Giuseppe Mercante, M.D. and Verter Barbieri, M.D. — Thu, 26Â MarÂ 2009 00:00:00 -05:00

A 67-year-old woman presented with a 6-month history of dyspnea and dysphagia. Physical examination revealed a small cervical goiter, but the lower poles of the thyroid were not palpable. The...

ORIGINAL ARTICLE: Mutations in Associated with Ovarian Insufficiency

Thu, 19Â MarÂ 2009 00:00:00 -05:00

Background: The genetic causes of nonsyndromic ovarian insufficiency are largely unknown. A nuclear receptor, NR5A1 (also called steroidogenic factor 1), is a key transcriptional regulator of genes involved in the...

CLINICAL PRACTICE: Graves' Ophthalmopathy

Luigi Bartalena, M.D., and Maria Laura Tanda, M.D. — Thu, 05Â MarÂ 2009 00:00:00 -05:00

A 40-year-old woman who recently received a diagnosis of Graves' disease comes for a follow-up visit. She has been taking methimazole, at a dose of 10 mg daily, and is...

CLINICAL PROBLEM-SOLVING: When Past Is Prologue

Thu, 05Â MarÂ 2009 00:00:00 -05:00

A 36-year-old man presented to the emergency department with a 2-week history of lower-extremity edema, progressive fatigue, and exertional dyspnea. The patient's medical history was notable for a fall from...

REVIEW ARTICLE: Preservation of Fertility in Patients with Cancer

Jacqueline S. Jeruss, M.D., Ph.D., and Teresa K. Woodruff, Ph.D. — Thu, 26Â FebÂ 2009 00:00:00 -05:00

The unique duality involved in confronting a life-threatening diagnosis while simultaneously considering the deeply human desire to have a child presents a struggle both for patients with cancer and for...

CORRESPONDENCE: A Family with Complete Resistance to Thyrotropin-Releasing Hormone

Thu, 12Â FebÂ 2009 00:00:00 -05:00

To the Editor: We report on a family with recessive resistance to the action of thyrotropin-releasing hormone (TRH) due to a nonsense homozygous mutation in the TRH receptor (TRHR) gene...

CLINICAL PROBLEM-SOLVING: Needle in a Haystack

Thu, 05Â FebÂ 2009 00:00:00 -05:00

A 50-year-old woman reported multiple loose bowel movements associated with mild, cramping abdominal pain. She had been well until 2 months earlier, when her bowel habits changed from one formed...

CLINICAL PRACTICE: Primary Ovarian Insufficiency

Lawrence M. Nelson, M.D. — Thu, 05Â FebÂ 2009 00:00:00 -05:00

A 30-year-old woman presents with a history of no menses since she stopped taking oral contraceptives 6 months ago in order to conceive. She had undergone puberty that was normal...

CORRESPONDENCE: Excess Iodine from an Unexpected Source

Seth M. Arum, M.D., Xuemei He, M.D., and Lewis E. Braverman, M.D. — Thu, 22Â JanÂ 2009 00:00:00 -05:00

To the Editor: It is standard practice to induce a decrease in the body's iodine stores before treatment with radioiodine for thyroid cancer. Such patients should avoid an iodine-rich diet,12...

IMAGES IN CLINICAL MEDICINE: Hypogonadism Due to Pituicytoma in an Identical Twin

Harvey H. Newnham, M.B., B.S. and Livia M. Rivera-Woll, M.B., B.S. — Thu, 25Â DecÂ 2008 00:00:00 -05:00

A 43-year-old man, who is an identical twin, presented with a 2-year history of lethargy, weakness, and headaches and a several-year history of gradual loss of body hair and declining...

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...

EDITORIAL: Treating Low Sexual Desire â New Findings for Testosterone in Women

Julia R. Heiman, Ph.D. — Thu, 06Â NovÂ 2008 00:00:00 -05:00

Decreased or low sexual desire is commonly reported by women from late adolescence through the seventh decade across a number of cultures. Estimates of prevalence range from 25 to 53%.123...

ORIGINAL ARTICLE: Testosterone for Low Libido in Postmenopausal Women Not Taking Estrogen

Thu, 06Â NovÂ 2008 00:00:00 -05:00

Background: The efficacy and safety of testosterone treatment for hypoactive sexual desire disorder in postmenopausal women not receiving estrogen therapy are unknown.

Methods: We conducted a double-blind, placebo-controlled, 52-week trial in...

CORRESPONDENCE: Problems Associated with the Use of Thyrogen in Patients with a Thyroid Gland

Thu, 16Â OctÂ 2008 00:00:00 -05:00

To the Editor: Juweid et al. (September 18 issue)1 describe the administration of thyrotropin alfa (Thyrogen) to a patient with thyroid cancer who still has a thyroid gland. Genzyme, the...

CORRESPONDENCE: Diagnosis of Poorly Differentiated Thyroid Cancer with Radioiodine Scanning after Thyrotropin Alfa Stimulation

Malik Juweid, M.D., Thomas O'Dorisio, M.D., and Mohammed Milhem, M.D. — Thu, 18Â SepÂ 2008 00:00:00 -05:00

To the Editor: Poorly differentiated thyroid cancers are rare and can be difficult for surgeons, endocrinologists, and pathologists to identify.1 These cancers fall into two main histologic categories: insular and...

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,...

CORRESPONDENCE: Mutation with Primary Hyperparathyroidism and Undetectable Intact PTH

Thu, 11Â SepÂ 2008 00:00:00 -05:00

To the Editor: We describe a case of hypercalcemia associated with the secretion of an abnormally truncated parathyroid hormone (PTH) from a parathyroid adenoma. A 59-year-old postmenopausal woman presenting with...

EDITORIAL: Renal PhosphateâTransporter Regulatory Proteins and Nephrolithiasis

Moshe Levi, M.D., and Sophia Breusegem, Ph.D. — Thu, 11Â SepÂ 2008 00:00:00 -05:00

Nephrolithiasis is a common disorder, and idiopathic hypercalciuria is the most frequent metabolic disorder associated with nephrolithiasis.1 Several studies have shown that subjects with idiopathic hypercalciuria have phosphaturia or renal...

IMAGES IN CLINICAL MEDICINE: Extracapsular Hemorrhage from a Parathyroid Adenoma

M. Carola Zillikens, M.D. and Anke Wijbenga, M.D., Ph.D. — Thu, 11Â SepÂ 2008 00:00:00 -05:00

A healthy 34-year-old woman presented with an acute, painful swelling and bluish discoloration of the anterior neck with no associated trauma. She reported having fatigue, anorexia, and depression during the...

CLINICAL THERAPEUTICS: Gonadotropin-Releasing Hormone Agonists for Endometriosis

David L. Olive, M.D. — Thu, 11Â SepÂ 2008 00:00:00 -05:00

A 36-year-old woman presents with long-standing pelvic pain, including dysmenorrhea and pain with intercourse. She has previously received oral contraceptives and depot medroxyprogesterone for presumed endometriosis. This regimen has not...

ORIGINAL ARTICLE: Mutations and Responsiveness of Renal Parathyroid Hormone

Thu, 11Â SepÂ 2008 00:00:00 -05:00

Impaired renal phosphate reabsorption, as measured by dividing the tubular maximal reabsorption of phosphate by the glomerular filtration rate (TmP/GFR), increases the risks of nephrolithiasis and bone demineralization. Data from...

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,...

CORRESPONDENCE: Age, Renal Tubular Phosphate Reabsorption, and Serum Phosphate Levels in Adults

Massimo Cirillo, M.D., Carolina Ciacci, M.D., and Natale G. De Santo, M.D. — Thu, 21Â AugÂ 2008 00:00:00 -05:00

To the Editor: A relation between age and serum phosphate levels in adults has been recognized since 1969.1 We used data from the Gubbio Population Study to analyze serum phosphate...

CORRESPONDENCE: Glucagon-like Peptide 1âReceptor Scans to Localize Occult Insulinomas

Thu, 14Â AugÂ 2008 00:00:00 -05:00

To the Editor: The precise localization of some insulinomas (islet-cell adenomas that secrete insulin) with the use of conventional imaging techniques, such as computed tomography (CT), magnetic resonance imaging (MRI),...

EDITORIAL: Tibolone and the Promise of Ideal Hormone-Replacement Therapy

Ghada El-Hajj Fuleihan, M.D., M.P.H. — Thu, 14Â AugÂ 2008 00:00:00 -05:00

Since the mean life expectancy at menopause is 32.7 years, women spend more than one third of their lives in the estrogen-deficient postmenopausal state.1 Of the majority of menopausal women...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 25-2008 â A 43-Year-Old Man with Fatigue and Lesions in the Pituitary and Cerebellum

John A. Mills, M.D., R. Gilberto Gonzalez, M.D., Ph.D., and Ronald Jaffe, M.D. — Thu, 14Â AugÂ 2008 00:00:00 -05:00

Dr. Flavia V. Castelino (Rheumatology, Allergy, and Immunology): A 43-year-old man was seen in the neuro-oncology clinic of this hospital because of fatigue and lesions in the pituitary and cerebellum...

ORIGINAL ARTICLE: The Effects of Tibolone in Older Postmenopausal Women

Thu, 14Â AugÂ 2008 00:00:00 -05:00

Background: Tibolone has estrogenic, progestogenic, and androgenic effects. Although tibolone prevents bone loss, its effects on fractures, breast cancer, and cardiovascular disease are uncertain.

Methods: In this randomized study, we assigned...

EDITORIAL: FGF-23 and Outcomes Research â When Physiology Meets Epidemiology

Chi-yuan Hsu, M.D., M.Sc. — Thu, 07Â AugÂ 2008 00:00:00 -05:00

In this issue of the Journal, GutiÃ©rrez and colleagues report the results of a study, conducted with an efficient nested caseâcohort design, that showed that among patients who were beginning...

ORIGINAL ARTICLE: Fibroblast Growth Factor 23 and Mortality among Patients Undergoing Hemodialysis

Thu, 07Â AugÂ 2008 00:00:00 -05:00

Background: Fibroblast growth factor 23 (FGF-23) is a hormone that increases the rate of urinary excretion of phosphate and inhibits renal production of 1,25-dihydroxyvitamin D, thus helping to mitigate hyperphosphatemia...

CLINICAL PRACTICE: Hypoparathyroidism

Dolores Shoback, M.D. — Thu, 24Â JulÂ 2008 00:00:00 -05:00

A 58-year-old man is found on laboratory testing to have a serum calcium level of 6.0 mg per deciliter (1.5 mmol per liter) (normal range, 8.5 to 10.5 mg per...

BOOK REVIEW:

Thu, 17Â JulÂ 2008 00:00:00 -05:00

According to population studies, the prevalence of the metabolic syndrome in the United States among people over 60 years of age is approximately 45%, and the newest surveys suggest that...

IMAGES IN CLINICAL MEDICINE: Renal Osteodystrophy of the Palate

Yahya Daneshbod, M.D. — Thu, 03Â JulÂ 2008 00:00:00 -05:00

A 20-year-old man who had had idiopathic end-stage renal failure since he was 12 years of age and who was undergoing dialysis once weekly (with stable serum electrolyte, blood urea...

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...

ORIGINAL ARTICLE: Motesanib Diphosphate in Progressive Differentiated Thyroid Cancer

Thu, 03Â JulÂ 2008 00:00:00 -05:00

Background: The expression of vascular endothelial growth factor (VEGF) is characteristic of differentiated thyroid cancer and is associated with aggressive tumor behavior and a poor clinical outcome. Motesanib diphosphate (AMG...

CLINICAL PRACTICE: Graves' Disease

Gregory A. Brent, M.D. — Thu, 12Â JunÂ 2008 00:00:00 -05:00

A 23-year-old woman presents with palpitations. Over the past 6 months, she has reported loose stools, a 10-lb (4.5-kg) weight loss despite a good appetite and food intake, and increased...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Metabolism and Therapeutic Angiogenesis

Peter Carmeliet, M.D., Ph.D., and Myriam Baes, Ph.D. — Thu, 05Â JunÂ 2008 00:00:00 -05:00

Despite great expectations, therapeutic angiogenesis with the vascular endothelial growth factor (VEGF) and related molecules for the treatment of ischemic heart and peripheral arterial occlusive disease has thus far failed...

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...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 16-2008 â A 46-Year-Old Woman with Bone Pain

Marie B. Demay, M.D., Daniel I. Rosenthal, M.D., and Vikram Deshpande, M.D. — Thu, 22Â MayÂ 2008 00:00:00 -05:00

A 46-year-old woman was seen in the orthopedic oncology clinic of this hospital because of pain in the right hip and leg. Several months earlier, pain had developed in her...

CLINICAL PROBLEM-SOLVING: Back to Basics

Ilan Gabriely, M.D., James P. Leu, M.D., and Uriel S. Barzel, M.D. — Thu, 01Â MayÂ 2008 00:00:00 -05:00

A 41-year-old woman was brought by her husband to the emergency department with a history of 72 hours of epigastric pain, nausea, repeated vomiting, and altered mental status. Her blood...

EDITORIAL: Reduce, Recycle, Reuse â Iodotyrosine Deiodinase in Thyroid Iodide Metabolism

Peter A. Kopp, M.D. — Thu, 24Â AprÂ 2008 00:00:00 -05:00

Thyroid hormones are essential for normal brain development, growth, and metabolism. Their synthesis is dependent on a normally developed thyroid gland, an adequate nutritional intake of iodide, and a series...

ORIGINAL ARTICLE: Mutations in the Iodotyrosine Deiodinase Gene and Hypothyroidism

Thu, 24Â AprÂ 2008 00:00:00 -05:00

DEHAL1 has been identified as the gene encoding iodotyrosine deiodinase in the thyroid, where it controls the reuse of iodide for thyroid hormone synthesis. We screened patients with hypothyroidism who...

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...

ORIGINAL ARTICLE: Gene Identification for the cblD Defect of Vitamin B Metabolism

Thu, 03Â AprÂ 2008 00:00:00 -05:00

Background: Vitamin B12 (cobalamin) is an essential cofactor in several metabolic pathways. Intracellular conversion of cobalamin to its two coenzymes, adenosylcobalamin in mitochondria and methylcobalamin in the cytoplasm, is necessary...

ORIGINAL ARTICLE: Simvastatin with or without Ezetimibe in Familial Hypercholesterolemia

Thu, 03Â AprÂ 2008 00:00:00 -05:00

Background: Ezetimibe, a cholesterol-absorption inhibitor, reduces levels of low-density lipoprotein (LDL) cholesterol when added to statin treatment. However, the effect of ezetimibe on the progression of atherosclerosis remains unknown.

Methods: We...

ORIGINAL ARTICLE: Autoimmune Polyendocrine Syndrome Type 1 and NALP5, a Parathyroid Autoantigen

Thu, 06Â MarÂ 2008 00:00:00 -05:00

Background: Autoimmune polyendocrine syndrome type 1 (APS-1) is a multiorgan autoimmune disorder caused by mutations in AIRE, the autoimmune regulator gene. Though recent studies concerning AIRE deficiency have begun to...

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: Intensive Insulin Therapy and Pentastarch Resuscitation in Severe Sepsis

Thu, 10Â JanÂ 2008 00:00:00 -05:00

Background: The role of intensive insulin therapy in patients with severe sepsis is uncertain. Fluid resuscitation improves survival among patients with septic shock, but evidence is lacking to support the...

CLINICAL THERAPEUTICS: Metformin for the Treatment of the Polycystic Ovary Syndrome

John E. Nestler, M.D. — Thu, 03Â JanÂ 2008 00:00:00 -05:00

A 23-year-old woman with known polycystic ovary syndrome visits her family physician. She has taken oral contraceptive pills in the past but did not tolerate them and is not currently...

IMAGES IN CLINICAL MEDICINE: Calcinosis Associated with Renal Failure

Robert A. Cohen, M.D. and Samir M. Parikh, M.D. — Thu, 20Â DecÂ 2007 00:00:00 -05:00

A 36-year-old woman presented with painless, subcutaneous nodules that had developed during the previous 5 months on her buttocks, shoulders, and left wrist (Panel A). Her medical history was notable...

CLINICAL PRACTICE: Erectile Dysfunction

Kevin T. McVary, M.D. — Thu, 13Â DecÂ 2007 00:00:00 -05:00

A 65-year-old man presents to an outpatient clinic, reporting that he can no longer maintain an erection sufficient for intercourse. His medical history includes well-controlled hypertension and stable coronary artery...

EDITORIAL: Manipulation of the Growth Hormone Axis in Patients with HIV Infection

Marc R. Blackman, M.D. — Thu, 06Â DecÂ 2007 00:00:00 -05:00

Patients with human immunodeficiency virus (HIV) infection often have a reduced mass of skeletal muscle and bone and a maldistribution of body fat. Loss of muscle mass, strength, and function...

ORIGINAL ARTICLE: Metabolic Effects of a Growth HormoneâReleasing Factor in Patients with HIV

Thu, 06Â DecÂ 2007 00:00:00 -05:00

Background: Visceral adipose tissue accumulates during antiretroviral therapy in many patients who are infected with the human immunodeficiency virus (HIV); this process is associated with an increased cardiovascular risk. We...

IMAGES IN CLINICAL MEDICINE: Calciphylaxis with Arterial Calcification

Jonathan Z. Li, M.D. and William Huen, M.D. — Thu, 27Â SepÂ 2007 00:00:00 -05:00

A 53-year-old woman with diabetes mellitus and end-stage renal disease who was undergoing hemodialysis presented with fever, painful finger necrosis, and nonhealing ulceration of the legs. Hand radiographs showed no...

ORIGINAL ARTICLE: Evidence of MEN-2 in the Original Description of Classic Pheochromocytoma

Thu, 27Â SepÂ 2007 00:00:00 -05:00

The first description of pheochromocytoma in 1886 has been attributed to Felix FrÃ¤nkel, who described an 18-year-old woman with bilateral adrenal âsarcoma and angio-sarcoma.â We reviewed the publication and then...

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...

IMAGES IN CLINICAL MEDICINE: Lipemia Retinalis Associated with Secondary Hyperlipidemia

Young-Hoon Park, M.D. and Young-Chun Lee, M.D. — Thu, 06Â SepÂ 2007 00:00:00 -05:00

A 26-year-old man with a 6-month history of type 1 diabetes mellitus presented with weight loss and poor glycemic control. Physical examination revealed no xanthomas; all vessels in the posterior...

CLINICAL PRACTICE: Hypertriglyceridemia

John D. Brunzell, M.D. — Thu, 06Â SepÂ 2007 00:00:00 -05:00

Hypertriglyceridemia is a common form of dyslipidemia1 that is frequently associated with premature coronary artery disease,2345 which is generally defined by the occurrence of a myocardial infarction or the need...

EDITORIAL: Experiments of Nature â A Glimpse into the Mysteries of the Pubertal Clock

Shalender Bhasin, M.D. — Thu, 30Â AugÂ 2007 00:00:00 -05:00

In an extraordinary display of nature's myriad intricacies, in higher mammals the gonadotropin-releasing hormone (GnRH) pulse generator, which drives the pulsatile secretion of gonadotropin and sex steroids, is kept in...

ORIGINAL ARTICLE: Luteinizing Hormone Beta Mutation and Hypogonadism in Men and Women

Thu, 30Â AugÂ 2007 00:00:00 -05:00

Selective luteinizing hormone deficiency due to mutations in the luteinizing hormone beta-subunit gene (LHB) is a rare cause of hypogonadism. We describe the clinical features of a consanguineous family in...

ORIGINAL ARTICLE: Reversal of Idiopathic Hypogonadotropic Hypogonadism

Thu, 30Â AugÂ 2007 00:00:00 -05:00

Background: Idiopathic hypogonadotropic hypogonadism, which may be associated with anosmia (the Kallmann syndrome) or with a normal sense of smell, is a treatable form of male infertility caused by a...

REVIEW ARTICLE: Vitamin D Deficiency

Michael F. Holick, M.D., Ph.D. — Thu, 19Â JulÂ 2007 00:00:00 -05:00

Once foods were fortified with vitamin d and rickets appeared to have been conquered, many health care professionals thought the major health problems resulting from vitamin D deficiency had been...

IMAGES IN CLINICAL MEDICINE: Endemic Multinodular Goiter

Daniel Cuevas-Ramos, M.D. and Bernardo PÃ©rez-EnrÃquez, M.D. — Thu, 28Â JunÂ 2007 00:00:00 -05:00

A 72-year-old woman presented for evaluation of dyspnea. thirty-five years earlier, she had noticed a painless, slowly enlarging anterior neck mass. Since she was otherwise asymptomatic and many in her...

EDITORIAL: HRT and the Young at Heart

Michael E. Mendelsohn, M.D., and Richard H. Karas, M.D., Ph.D. — Thu, 21Â JunÂ 2007 00:00:00 -05:00

For the past decade, women and health care providers have faced confusing information about the effects of hormone-replacement therapy, or HRT, on heart disease. Important practical questions have remained unresolved:...

ORIGINAL ARTICLE: Estrogen Therapy and Coronary-Artery Calcification

Thu, 21Â JunÂ 2007 00:00:00 -05:00

Background: Calcified plaque in the coronary arteries is a marker for atheromatous-plaque burden and is predictive of future risk of cardiovascular events. We examined the relationship between estrogen therapy and...

EDITORIAL: Adjuvant Mitotane Therapy of Adrenal Cancer â Use and Controversy

David E. Schteingart, M.D. — Thu, 07Â JunÂ 2007 00:00:00 -05:00

Adrenocortical carcinoma is a rare, highly malignant neoplasm with an incidence of 2 cases per 1 million population per year worldwide, representing 0.2% of all cases of cancer. Several treatment...

ORIGINAL ARTICLE: Adjuvant Mitotane Treatment for Adrenocortical Carcinoma

Thu, 07Â JunÂ 2007 00:00:00 -05:00

Background: Adrenocortical carcinoma is a rare neoplasm characterized by a high risk of recurrence after radical resection. Whether the use of mitotane is beneficial as an adjuvant treatment has been...

CLINICAL PRACTICE: The Syndrome of Inappropriate Antidiuresis

David H. Ellison, M.D., and Tomas Berl, M.D. — Thu, 17Â MayÂ 2007 00:00:00 -05:00

A 62-year-old woman noted an unpleasant, sweet taste in her mouth. She otherwise felt well and was taking no medications. Because dysgeusia is a rare manifestation of hyponatremia, her serum...

ORIGINAL ARTICLE: in Vitiligo-Associated Multiple Autoimmune Disease

Thu, 22Â MarÂ 2007 00:00:00 -05:00

Background: Autoimmune and autoinflammatory diseases involve interactions between genetic risk factors and environmental triggers. We searched for a gene on chromosome 17p13 that contributes to a group of epidemiologically associated...

CLINICAL PRACTICE: The Incidentally Discovered Adrenal Mass

William F. Young, Jr., M.D. — Thu, 08Â FebÂ 2007 00:00:00 -05:00

A 68-year-old woman is incidentally found to have a left adrenal mass, 2.8 cm in diameter, on abdominal computed tomography that was ordered to evaluate right lower abdominal discomfort (which...

ORIGINAL ARTICLE: Prepubertal Gynecomastia Linked to Lavender and Tea Tree Oils

Thu, 01Â FebÂ 2007 00:00:00 -05:00

Most cases of male prepubertal gynecomastia are classified as idiopathic. We investigated possible causes of gynecomastia in three prepubertal boys who were otherwise healthy and had normal serum concentrations of...

CLINICAL PRACTICE: In Vitro Fertilization

Bradley J. Van Voorhis, M.D. — Thu, 25Â JanÂ 2007 00:00:00 -05:00

A 37-year-old woman who has never been pregnant and her 40-year-old husband have been attempting to conceive a child for the past 3 years. An infertility evaluation has shown no...

REVIEW ARTICLE: Acromegaly

Shlomo Melmed, M.B., Ch.B. — Thu, 14Â DecÂ 2006 00:00:00 -05:00

Pituitary tumors account for about 15% of primary intracranial neoplasms.1 Given the critical location of the gland, expanding tumors cause compressive symptoms. Furthermore, as pituitary cells secrete hormones, the proliferation...

IMAGES IN CLINICAL MEDICINE: Bisphosphonate-Associated Contact Stomatitis

Pietro Rubegni, M.D. and Michele Fimiani, M.D. — Thu, 30Â NovÂ 2006 00:00:00 -05:00

A 68-year-old woman with severe postmenopausal osteoporosis was referred because of a 6-month history of erosive mucositis of the hard palate, accompanied by intense pain and dysphagia. The patient had...

IMAGES IN CLINICAL MEDICINE: Bisphosphonate-Associated Osteonecrosis of the Jaw

Nathaniel Treister, D.M.D., D.M.Sc. and Sook-Bin Woo, D.M.D. — Thu, 30Â NovÂ 2006 00:00:00 -05:00

A 54-year-old man with a 3-year history of IgG myeloma, complicated by hypercalcemia, who was being treated with dexamethasone, lenalidomide, filgrastim, darbepoetin alfa, and pamidronate, presented with a 1-month history...

CLINICAL PRACTICE: Management of Menopausal Symptoms

Deborah Grady, M.D., M.P.H. — Thu, 30Â NovÂ 2006 00:00:00 -05:00

A 51-year-old woman has frequent and distressing hot flushes that interfere with her work and sleep, and vaginal dryness that makes sexual intercourse with her husband uncomfortable. She is otherwise...

PERSPECTIVE: Osteonecrosis of the Jaw â Do Bisphosphonates Pose a Risk?

Thu, 30Â NovÂ 2006 00:00:00 -05:00

Nitrogen-containing bisphosphonates are used widely for the management of metastatic cancer in bone (intravenous zoledronic acid or pamidronate), for the prevention and treatment of osteoporosis (oral alendronate, risedronate, and ibandronate...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 36-2006 â A 35-Year-Old Pregnant Woman with New Hypertension

Thu, 23Â NovÂ 2006 00:00:00 -05:00

Dr. Andrea L. Utz (Endocrinology): A 35-year-old pregnant woman (gravida 2, para 1) was admitted to this hospital at 19 weeks and 6 days of gestation because of the recent...

EDITORIAL: Vasopressin Antagonists â Progress and Promise

Thu, 16Â NovÂ 2006 00:00:00 -05:00

Hyponatremia is the most common in-hospital electrolyte disorder that a physician encounters. Its management has been the subject of numerous studies and reviews. In patients with chronic heart failure or...

ORIGINAL ARTICLE: Tolvaptan, a Selective Oral Vasopressin V-Receptor Antagonist, for Hyponatremia

Thu, 16Â NovÂ 2006 00:00:00 -05:00

Background: Hyponatremia (serum sodium concentration, <135 mmol per liter) is a predictor of death among patients with chronic heart failure and cirrhosis. At present, therapy for acute and chronic hyponatremia...

CLINICAL PRACTICE: Management of Hyperglycemia in the Hospital Setting

Silvio E. Inzucchi, M.D. — Thu, 02Â NovÂ 2006 00:00:00 -05:00

A 53-year-old woman with asthma and multilobar pneumonia is admitted with respiratory failure. She is intubated and treated with antibiotics, albuterol, and methylprednisolone. A random blood glucose measurement obtained on...

EDITORIAL: Aging and Fountain-of-Youth Hormones

Paul M. Stewart, M.D. — Thu, 19Â OctÂ 2006 00:00:00 -05:00

The search for eternal youth is vibrant in North America. In this search, it is all too easy to ascribe the aging process to endocrines. Although the secretion of growth...

ORIGINAL ARTICLE: DHEA in Elderly Women and DHEA or Testosterone in Elderly Men

Thu, 19Â OctÂ 2006 00:00:00 -05:00

Background: Dehydroepiandrosterone (DHEA) and testosterone are widely promoted as antiaging supplements, but the long-term benefits, as compared with potential harm, are unknown.

Methods: We performed a 2-year, placebo-controlled, randomized, double-blind study...

CLINICAL PRACTICE: Paget's Disease of Bone

Michael P. Whyte, M.D. — Thu, 10Â AugÂ 2006 00:00:00 -05:00

A 69-year-old man reports increasing pain in the right leg. Physical examination reveals warmth and anterolateral bowing of the right shin. The serum alkaline phosphatase level is 260 U per...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 23-2006 â A 36-Year-Old Man with Numbness in the Right Hand and Hypertension

Thu, 27Â JulÂ 2006 00:00:00 -05:00

Dr. Joanna J. Wykrzykowska (Medicine): A 36-year-old man was admitted to the hospital because of a mass in the right adrenal gland. The patient had been well until seven months...

ORIGINAL ARTICLE: Mutant Neurogenin-3 in Congenital Malabsorptive Diarrhea

Thu, 20Â JulÂ 2006 00:00:00 -05:00

Background: Neurogenin-3 (NEUROG3) is expressed in endocrine progenitor cells and is required for endocrine-cell development in the pancreas and intestine. The NEUROG3 gene (NEUROG3) is therefore a candidate for the...

EDITORIAL: RiskâBenefit Profiles of Raloxifene for Women

Marcia L. Stefanick, Ph.D. — Thu, 13Â JulÂ 2006 00:00:00 -05:00

During the past decade, considerable scientific, clinical, and public interest in the benefits and risks of postmenopausal estrogen therapy has focused attention on selective estrogen-receptor modulators (SERMs). These act as...

ORIGINAL ARTICLE: Effects of Raloxifene on Cardiovascular Events and Breast Cancer in Postmenopausal Women

Thu, 13Â JulÂ 2006 00:00:00 -05:00

Background: The effect of raloxifene, a selective estrogen-receptor modulator, on coronary heart disease (CHD) and breast cancer is not established.

Methods: We randomly assigned 10,101 postmenopausal women (mean age, 67.5 years)...

CLINICAL PROBLEM-SOLVING: Heading Down the Wrong Path

Thu, 06Â JulÂ 2006 00:00:00 -05:00

A 52-year-old woman who had undergone renal transplantation 13 years earlier was brought by her family to the emergency department of a community hospital. Five days earlier, she had suddenly...

IMAGES IN CLINICAL MEDICINE: Frey's Syndrome

Francesco Prattico, M.D. and Paola Perfetti, M.D. — Thu, 06Â JulÂ 2006 00:00:00 -05:00

A 30-year-old man had a pleomorphic adenoma removed from his left parotid gland. His postoperative course was uncomplicated. Two months later, he noted that his left cheek became wet while...

IMAGES IN CLINICAL MEDICINE: Hypothyroid-Induced Hyporeflexia

Patrick R. Sosnay, M.D. and Scott Kim, M.D. — Thu, 29Â JunÂ 2006 00:00:00 -05:00

A 48-year-old woman was brought to the hospital by her family because of diffuse, nonspecific pain. She was unable to provide her medical history. During the first hospital day, hypothermia...

EDITORIAL: Iodine Nutrition â More Is Better

Robert D. Utiger, M.D. — Thu, 29Â JunÂ 2006 00:00:00 -05:00

In normal adults, the daily production rate of the two biologically active thyroid hormones, tetraiodothyronine (which is better known as thyroxine and has four iodine atoms) and triiodothyronine (which has...

ORIGINAL ARTICLE: Effect of Iodine Intake on Thyroid Diseases in China

Thu, 29Â JunÂ 2006 00:00:00 -05:00

Background: Iodine is an essential component of thyroid hormones; either low or high intake may lead to thyroid disease. We observed an increase in the prevalence of overt hypothyroidism, subclinical...

IMAGES IN CLINICAL MEDICINE: Acquired Hypertrichosis Lanuginosa

Gerard Lorette, M.D. and Annabel Maruani, M.D. — Thu, 22Â JunÂ 2006 00:00:00 -05:00

A 68-year-old woman requested medical attention for a sudden increase in hair growth that she had noticed 11 months earlier. She had lost more than 30 kg during this same...

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...

IMAGES IN CLINICAL MEDICINE: Sudden Enlargement of the Neck

Stephen McDonnell, M.B., B.S. and Matthew Clark, M.B., B.S. — Thu, 27Â AprÂ 2006 00:00:00 -05:00

A 63-year-old man presented with a sudden enlargement of his neck. During the previous 10 years, his neck had grown progressively larger until he was no longer able to button...

ORIGINAL ARTICLE: Thyroxine in Goiter, Infection, and Chronic Gastritis

Thu, 27Â AprÂ 2006 00:00:00 -05:00

Background: Malabsorption of thyroxine has been described in patients treated with drugs that modify an acidic environment. We determined whether there is an increased need for thyroxine in patients with...

IMAGES IN CLINICAL MEDICINE: Pseudoclubbing

Afshin Farzaneh-Far, M.D., Ph.D. — Thu, 13Â AprÂ 2006 00:00:00 -05:00

A 36-year-old man was referred for further evaluation after having changes in his fingers that were initially thought to be due to clubbing (Panels A and B). He had a...

REVIEW ARTICLE: The Biology of Human Sex Differences

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

Everyone in medicine and related fields understands that there are marked sex-based differences in the epidemiology, clinical manifestations, course, and therapy of disease.1 Although very few of these differences are...

EDITORIAL: Serious Adverse Drug Effects â Seeing the Trees through the Forest

Jerry H. Gurwitz, M.D. — Thu, 30Â MarÂ 2006 00:00:00 -05:00

The medical community and the public have been buffeted by a steady stream of news linking the use of widely prescribed medications with serious health risks. The latest in this...

REVIEW ARTICLE: Hereditary Proteinuria Syndromes and Mechanisms of Proteinuria

Thu, 30Â MarÂ 2006 00:00:00 -05:00

The inherited forms of proteinuria comprise a heterogeneous group of rare renal diseases in which glomerular dysfunction and proteinuria are prominent. Despite the rarity of hereditary proteinuria syndromes, genetic, biochemical,...

ORIGINAL ARTICLE: Outpatient Gatifloxacin Therapy and Dysglycemia in Older Adults

Thu, 30Â MarÂ 2006 00:00:00 -05:00

Background: Gatifloxacin has been associated with both hypoglycemia and hyperglycemia. We examined dysglycemia-related health outcomes associated with various antibiotics in a population of approximately 1.4 million Ontario, Canada, residents 66...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 4-2006 â A 79-Year-Old Woman with Myalgias, Fatigue, and Shortness of Breath

Jonathan Kay, M.D., David S. Finn, M.D., and James R. Stone, M.D., Ph.D. — Thu, 09Â FebÂ 2006 00:00:00 -05:00

Dr. Celine R. Gounder (Department of Medicine): A 79-year-old woman was admitted to the hospital because of myalgias, fatigue, and shortness of breath.

Hyperlipidemia had been diagnosed six years earlier and...

EDITORIAL: Intensive Insulin in Intensive Care

Atul Malhotra, M.D. — Thu, 02Â FebÂ 2006 00:00:00 -05:00

Among the critically ill, elevations in blood glucose, a marker previously ignored or described as adaptive, became a major therapeutic target after a 2001 study indicated a mortality benefit of...

ORIGINAL ARTICLE: Intensive Insulin Therapy in the Medical ICU

Thu, 02Â FebÂ 2006 00:00:00 -05:00

Background: Intensive insulin therapy reduces morbidity and mortality in patients in surgical intensive care units (ICUs), but its role in patients in medical ICUs is unknown.

Methods: In a prospective, randomized,...

CLINICAL PRACTICE: Hirsutism

Robert L. Rosenfield, M.D. — Thu, 15Â DecÂ 2005 00:00:00 -05:00

A 19-year-old woman seeks care for slowly progressive hair growth. Since high school, she has shaved her upper lip weekly and waxed her abdomen and thighs monthly. Her menstrual periods...

REVIEW ARTICLE: Retinoid X Receptor Heterodimers in the Metabolic Syndrome

Andrew I. Shulman, Ph.D., and David J. Mangelsdorf, Ph.D. — Thu, 11Â AugÂ 2005 00:00:00 -05:00

The metabolic syndrome, also known as syndrome X, is characterized by abdominal obesity, atherogenic dyslipidemia, hypertension, insulin resistance, inflammation, and prothrombotic states.1 05081128.xml 05081128.xml 05081128.xml 05081128.xml Diagnostic of the metabolic...

IMAGES IN CLINICAL MEDICINE: Xanthomatous Pseudospectacles in Familial Hypercholesterolemia

M.P. Girish, M.D. and Mohit Dayal Gupta, M.D. — Thu, 09Â JunÂ 2005 00:00:00 -05:00

A 40-year-old woman presented with exertional chest pain and was found to have familial hypercholesterolemia (type IIa) and severe three-vessel coronary artery disease. Xanthoma palpebrarum was noted on physical examination,...

ORIGINAL ARTICLE: Independent Clonal Origins of Distinct Tumor Foci in Multifocal Papillary Thyroid Carcinoma

Thu, 09Â JunÂ 2005 00:00:00 -05:00

Background: Papillary thyroid carcinoma is frequently multifocal. We investigated whether noncontiguous tumor foci arise from intraglandular metastases from a single primary tumor or originate as unrelated clones derived from independent...

PERSPECTIVE: The Multiplicity of Thyroid Nodules and Carcinomas

Robert D. Utiger, M.D. — Thu, 09Â JunÂ 2005 00:00:00 -05:00

About 5 percent of adults in the United States have palpable thyroid nodules. Most of these nodules are at least 2 cm in their longest dimension, and their frequency doesn't...

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,...

ORIGINAL ARTICLE: Nephrogenic Syndrome of Inappropriate Antidiuresis

Thu, 05Â MayÂ 2005 00:00:00 -05:00

The syndrome of inappropriate antidiuretic hormone secretion (SIADH) is a common cause of hyponatremia. We describe two infants whose clinical and laboratory evaluations were consistent with the presence of SIADH,...

PERSPECTIVE: Hyperactive Vasopressin Receptors and Disturbed Water Homeostasis

Nine V.A.M. Knoers, M.D., Ph.D. — Thu, 05Â MayÂ 2005 00:00:00 -05:00

Regulation of water homeostasis is of vital importance for all terrestrial organisms. In most mammals, including humans, the maintenance of water balance is critically dependent on water intake, the sensation...

EDITORIAL: Low-Density Lipoprotein Cholesterol in Patients with Stable Coronary Heart Disease â Is It Time to Shift Our Goals?

Bertram Pitt, M.D. — Thu, 07Â AprÂ 2005 00:00:00 -05:00

The evidence of the effectiveness of statins in the treatment of stable coronary heart disease (CHD) continues to grow. Large-scale, randomized, secondary-prevention trials involving patients with CHD have shown that...

ORIGINAL ARTICLE: Intensive Lipid Lowering with Atorvastatin in Patients with Stable Coronary Disease

Thu, 07Â AprÂ 2005 00:00:00 -05:00

Background: Previous trials have demonstrated that lowering low-density lipoprotein (LDL) cholesterol levels below currently recommended levels is beneficial in patients with acute coronary syndromes. We prospectively assessed the efficacy and...

REVIEW ARTICLE: Polycystic Ovary Syndrome

David A. Ehrmann, M.D. — Thu, 24Â MarÂ 2005 00:00:00 -05:00

In 1935, Stein and Leventhal published a paper on their findings in seven women with amenorrhea, hirsutism, obesity, and a characteristic polycystic appearance to their ovaries â one of the...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 7-2005 â A 59-Year-Old Woman with an Incidentally Discovered Adrenal Nodule

Robert G. Dluhy, M.D., Michael M. Maher, M.D., and Chin-Lee Wu, M.D. — Thu, 10Â MarÂ 2005 00:00:00 -05:00

A 59-year-old woman was seen in the clinic for evaluation of an adrenal mass.

Two years earlier, she had undergone her first physical examination since childhood, having sought medical attention because...

IMAGES IN CLINICAL MEDICINE: Dermopathy of Graves' Disease

Shih-Ping Cheng, M.D. and Chien-Liang Liu, M.D. — Thu, 03Â MarÂ 2005 00:00:00 -05:00

A 51-year-old woman presented with weight loss (despite good appetite), palpitations, tremor, and heat intolerance. On examination, she had typical features of Graves' disease, including a diffusely enlarged thyroid, periorbital...

REVIEW ARTICLE: Antithyroid Drugs

David S. Cooper, M.D. — Thu, 03Â MarÂ 2005 00:00:00 -05:00

Antithyroid drugs, which have been in use for more than half a century, remain cornerstones in the management of hyperthyroidism, especially for patients with Graves' disease. Surveys of thyroidologists from...

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: Hypercalcemia Associated with Cancer

Andrew F. Stewart, M.D. — Thu, 27Â JanÂ 2005 00:00:00 -05:00

A 47-year-old woman with a history of breast cancer presents with confusion and dehydration. The serum calcium level is 18.0 mg per deciliter (4.5 mmol per liter). She has postural...

IMAGES IN CLINICAL MEDICINE: A Medical Mystery

Hans Hartmann, M.D. and Urs Schumacher, M.D. — Thu, 20Â JanÂ 2005 00:00:00 -05:00

A male infant was born at 38 weeks and 1 day of gestation with a weight of 3430 g. When the child was five months old, his mother noticed an...

ORIGINAL ARTICLE: Risk of Fracture after Androgen Deprivation for Prostate Cancer

Thu, 13Â JanÂ 2005 00:00:00 -05:00

Background: The use of androgen-deprivation therapy for prostate cancer has increased substantially over the past 15 years. This treatment is associated with a loss of bone-mineral density, but the risk...

IMAGES IN CLINICAL MEDICINE: Obstructive Lingual Thyroid

Andreas Barthel, M.D. and Stefan R. Bornstein, M.D., Ph.D. — Thu, 06Â JanÂ 2005 00:00:00 -05:00

A 43-year-old woman who was undergoing surgery could not be intubated for the administration of anesthesia because of an unusually narrow oropharynx. Ultrasonography showed a round, solid tumor about 3...

REVIEW ARTICLE: Cardiovascular Risk and Body-Fat Abnormalities in HIV-Infected Adults

Steven Grinspoon, M.D., and Andrew Carr, M.D. — Thu, 06Â JanÂ 2005 00:00:00 -05:00

Metabolic complications, including dyslipidemia, insulin resistance, and altered fat distribution (loss of subcutaneous fat and a relative increase in central fat), are common in adults infected with the human immunodeficiency...

ORIGINAL ARTICLE: Hypogonadism in a Patient with a Mutation in the Luteinizing Hormone Beta-Subunit Gene

Thu, 16Â DecÂ 2004 00:00:00 -05:00

A 30-year-old man who presented with delayed puberty and infertility was found to have hypogonadism associated with an absence of circulating luteinizing hormone. The patient had a homozygous missense mutation...

CLINICAL PRACTICE: The Thyroid Nodule

Laszlo HegedÃ¼s, M.D. — Thu, 21Â OctÂ 2004 00:00:00 -05:00

A 42-year-old woman presents with a palpable mass on the left side of her neck. She has no neck pain and no symptoms of thyroid dysfunction. Physical examination reveals a...

ORIGINAL ARTICLE: Recombinant Human Leptin in Women with Hypothalamic Amenorrhea

Thu, 02Â SepÂ 2004 00:00:00 -05:00

Background: Disruptions in hypothalamicâgonadal and other endocrine axes due to energy deficits are associated with low levels of the adipocyte-secreted hormone leptin and may result in hypothalamic amenorrhea. We hypothesized...

PERSPECTIVE: Body Fat, Leptin, and Hypothalamic Amenorrhea

Rexford S. Ahima, M.D., Ph.D. — Thu, 02Â SepÂ 2004 00:00:00 -05:00

Hypothalamic amenorrhea can be defined as the cessation of menstruation due to a dysfunction of hypothalamic signals to the pituitary gland, resulting in a failure of ovulation. Typically, young women...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: No Pancreatic Endocrine Stem Cells?

Fred Levine, M.D., Ph.D., and Mark Mercola, Ph.D. — Thu, 02Â SepÂ 2004 00:00:00 -05:00

Advances in clinical islet transplantation have raised the hope that a definitive treatment for diabetes is close. However, widespread application of islet transplantation has been limited by a shortage of...

IMAGES IN CLINICAL MEDICINE: VIPoma

Stuart M. Lubinski, M.D. and Thomas Hendrix, M.D. — Thu, 19Â AugÂ 2004 00:00:00 -05:00

A 50-year-old woman presented with a five-month history of fatigue, weakness, and diarrhea. Her electrocardiogram disclosed prominent U waves and ST-segment depression. The serum potassium level was 1.6 mmol per...

ORIGINAL ARTICLE: Acquired Hypocalciuric Hypercalcemia Due to Autoantibodies against the Calcium-Sensing Receptor

Thu, 22Â JulÂ 2004 00:00:00 -05:00

A complex homeostatic system involving the interplay of the bones, the kidneys, and the intestines has evolved to maintain extracellular calcium concentrations within a relatively narrow range.1 The primary regulator...

PERSPECTIVE: Translational Implications of the Parathyroid Calcium Receptor

Andrew F. Stewart, M.D. — Thu, 22Â JulÂ 2004 00:00:00 -05:00

We live in the era of the Human Genome Project, human and mouse genetics, proteomics, evidence-based medicine, intelligent drug design, and a new âroad mapâ for the National Institutes of...

EDITORIAL: Increased Levothyroxine Requirements in Pregnancy â Why, When, and How Much?

Anthony Toft, M.D. — Thu, 15Â JulÂ 2004 00:00:00 -05:00

Those with sufficiently long memories must be somewhat bemused by the successive controversies surrounding the treatment of primary hypothyroidism, a condition viewed by many as simple, satisfying to manage, and...

ORIGINAL ARTICLE: Timing and Magnitude of Increases in Levothyroxine Requirements during Pregnancy in Women with Hypothyroidism

Thu, 15Â JulÂ 2004 00:00:00 -05:00

Background: Hypothyroidism during pregnancy has been associated with impaired cognitive development and increased fetal mortality. During pregnancy, maternal thyroid hormone requirements increase. Although it is known that women with hypothyroidism...

PERSPECTIVE: Aldosterone â Villain or Bystander?

Robert G. Dluhy, M.D., and Gordon H. Williams, M.D. — Thu, 01Â JulÂ 2004 00:00:00 -05:00

The two primary regulators of aldosterone secretion are potassium and the reninâangiotensin system. The latter is involved in volume homeostasis, with high salt intake suppressing the reninâangiotensin system and aldosterone...

IMAGES IN CLINICAL MEDICINE: A Medical Mystery â Which Twin Is the Patient?

Willy-Anne Nieuwlaat, M.D. and Gerlach Pieters, M.D., Ph.D. — Thu, 01Â JulÂ 2004 00:00:00 -05:00

Which of these 50-year-old identical twins is the patient? And what is the diagnosis?...

ORIGINAL ARTICLE: Serum Aldosterone and the Incidence of Hypertension in Nonhypertensive Persons

Thu, 01Â JulÂ 2004 00:00:00 -05:00

Background: Primary hyperaldosteronism is a well-recognized cause of secondary hypertension. It is unknown whether serum aldosterone levels within the physiologic range influence the risk of hypertension.

Methods: We investigated the relation...

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 PRACTICE: Asymptomatic Primary Hyperparathyroidism

John P. Bilezikian, M.D., and Shonni J. Silverberg, M.D. — Thu, 22Â AprÂ 2004 00:00:00 -05:00

A 60-year-old woman is noted incidentally to have a calcium level of 10.8 mg per deciliter (2.70 mmol per liter; normal range, 8.4 to 10.2 mg per deciliter [2.10 to...

ORIGINAL ARTICLE: Measurements of Serum Free Cortisol in Critically Ill Patients

Amir H. Hamrahian, M.D., Tawakalitu S. Oseni, M.D., and Baha M. Arafah, M.D. — Thu, 15Â AprÂ 2004 00:00:00 -05:00

Background: Because more than 90 percent of circulating cortisol in human serum is protein-bound, changes in the binding proteins can alter measured serum total cortisol concentrations without influencing free concentrations...

PERSPECTIVE: Glucocorticoid Therapy in the Intensive Care Unit

Lynn Loriaux, M.D. — Thu, 15Â AprÂ 2004 00:00:00 -05:00

Acute adrenal insufficiency, a rare cause of shock, is manifested as shock that is poorly responsive to fluid resuscitation and pressors, not unlike cardiogenic or septic shock. It is almost...

EDITORIAL: Fooling the Parathyroid Gland â Will There Be Health Benefits?

Gary Curhan, M.D., Sc.D. — Thu, 08Â AprÂ 2004 00:00:00 -05:00

In people with a reduced glomerular filtration rate, the serum parathyroid hormone level is often secondarily elevated as a result of reduced serum levels of calcium and 1,25-dihydroxyvitamin D3 and...

ORIGINAL ARTICLE: Cinacalcet for Secondary Hyperparathyroidism in Patients Receiving Hemodialysis

Thu, 08Â AprÂ 2004 00:00:00 -05:00

Background: Treatment of secondary hyperparathyroidism with vitamin D and calcium in patients receiving dialysis is often complicated by hypercalcemia and hyperphosphatemia, which may contribute to cardiovascular disease and adverse clinical...

IMAGES IN CLINICAL MEDICINE: Pemberton's Sign

Shehzad Basaria, M.D. and Roberto Salvatori, M.D. — Thu, 25Â MarÂ 2004 00:00:00 -05:00

A 58-year-old woman with a 20-year history of goiter presented with a two-month history of progressive dyspnea on exertion, occasional stridor, and a choking sensation while supine. She had previously...

REVIEW ARTICLE: Acquired and Inherited Lipodystrophies

Abhimanyu Garg, M.D. — Thu, 18Â MarÂ 2004 00:00:00 -05:00

Lipodystrophies are clinically heterogeneous acquired or inherited disorders characterized by the selective loss of adipose tissue. Affected patients are predisposed to insulin resistance and its attendant complications, including diabetes mellitus,...

ORIGINAL ARTICLE: Circulating Angiogenic Factors and the Risk of Preeclampsia

Thu, 12Â FebÂ 2004 00:00:00 -05:00

Background: The cause of preeclampsia remains unclear. Limited data suggest that excess circulating soluble fms-like tyrosine kinase 1 (sFlt-1), which binds placental growth factor (PlGF) and vascular endothelial growth factor...

PERSPECTIVE: Preeclampsia â Searching for the Cause

Caren G. Solomon, M.D., M.P.H., and Ellen W. Seely, M.D. — Thu, 12Â FebÂ 2004 00:00:00 -05:00

Three to 5 percent of pregnancies in the United States are complicated by preeclampsia, a multisystem disorder characterized by hypertension and proteinuria that occurs after 20 weeks of pregnancy. Preeclampsia...

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...

REVIEW ARTICLE: Risks of Testosterone-Replacement Therapy and Recommendations for Monitoring

Ernani Luis Rhoden, M.D., and Abraham Morgentaler, M.D. — Thu, 29Â JanÂ 2004 00:00:00 -05:00

Hypogonadism is a clinical condition in which low levels of serum testosterone are found in association with specific signs and symptoms, including diminished libido and sense of vitality, erectile dysfunction,...

PERSPECTIVE: Hypogonadism in Elderly Men â What to Do Until the Evidence Comes

Peter J. Snyder, M.D. — Thu, 29Â JanÂ 2004 00:00:00 -05:00

Testosterone treatment for elderly men made the news again recently, when the Institute of Medicine Committee on Assessing the Need for Clinical Trials of Testosterone Replacement Therapy issued its long-awaited...

IMAGES IN CLINICAL MEDICINE: True Hermaphroditism

Jose A. Karam, M.D. and Linda A. Baker, M.D. — Thu, 22Â JanÂ 2004 00:00:00 -05:00

A 17-day-old black neonate presented with ambiguous genitalia. A sharp line separated hypopigmented skin on the left side of the abdomen from normally pigmented skin on the right side. A...

REVIEW ARTICLE: Sex Determination and Differentiation

David T. MacLaughlin, Ph.D., and Patricia K. Donahoe, M.D. — Thu, 22Â JanÂ 2004 00:00:00 -05:00

Sex determination, which depends on the sex-chromosome complement of the embryo, is established by multiple molecular events that direct the development of germ cells, their migration to the urogenital ridge,...

PERSPECTIVE: Three Facets of Sexual Differentiation

Daniel D. Federman, M.D. — Thu, 22Â JanÂ 2004 00:00:00 -05:00

Sexual reproduction requires three types of differentiation: gonadal for the production of gametes, genital for the conveyance of gametes to a point of fertilization, and behavioral for the urge to...

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: Hypokalemia

Adam Nosworthy, M.B., B.Ch. — Thu, 27Â NovÂ 2003 00:00:00 -05:00

A 42-year-old man was hospitalized because he had had confusion, weakness, and lethargy for approximately a week. Hypertension had recently been diagnosed, and the patient had begun therapy with a...

CLINICAL PRACTICE: Prolactinoma

Janet A. Schlechte, M.D. — Thu, 20Â NovÂ 2003 00:00:00 -05:00

A 22-year-old woman who wants to become pregnant has had no menses since she discontinued the use of an oral contraceptive one year ago, and recently, galactorrhea developed. She takes...

IMAGES IN CLINICAL MEDICINE: Pituitary Apoplexy

Leonard C. Glass, M.D. — Thu, 20Â NovÂ 2003 00:00:00 -05:00

Acute hypotension developed in a 63-year-old man during coronary-artery bypass graft surgery. On awakening from surgery, he reported a severe headache and lightheadedness. Four hours later, he was noted to...

ORIGINAL ARTICLE: Withdrawal of Long-Term Cabergoline Therapy for Tumoral and Nontumoral Hyperprolactinemia

Thu, 20Â NovÂ 2003 00:00:00 -05:00

Background: Whether the withdrawal of treatment in patients with nontumoral hyperprolactinemia, microprolactinomas, or macroprolactinomas is safe and effective has been unclear. We performed an observational, prospective study of cabergoline (a...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Nerves, Fat, and Insulin Resistance

Guenther Boden, M.D., and Robert D. Hoeldtke, Ph.D., M.D. — Thu, 13Â NovÂ 2003 00:00:00 -05:00

Free fatty acids, the chief constituents of fat, are the main fuel for the heart and skeletal muscle under normal circumstances. Under special circumstances â such as exercise or exposure...

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...

ORIGINAL ARTICLE: Somatic and Germ-Line Mutations of the Gene in Sporadic Parathyroid Carcinoma

Thu, 30Â OctÂ 2003 00:00:00 -05:00

Background: We looked for mutations of the HRPT2 gene, which encodes the parafibromin protein, in sporadic parathyroid carcinoma because germ-line inactivating HRPT2 mutations have been found in a type of...

PERSPECTIVE: a Marker of Parathyroid Cancer

Lee S. Weinstein, M.D., and William F. Simonds, M.D. — Thu, 30Â OctÂ 2003 00:00:00 -05:00

Primary hyperparathyroidism is a state of excess secretion of parathyroid hormone and hypercalcemia, often with skeletal and renal complications. The disease usually results from a single parathyroid adenoma. Less commonly,...

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: Lessons Learned from the Management of a Rare Genetic Cancer

Gilbert J. Cote, Ph.D., and Robert F. Gagel, M.D. — Thu, 16Â OctÂ 2003 00:00:00 -05:00

Less than a decade after the identification of activating mutations of the rearranged during transfection (RET) proto-oncogene in multiple endocrine neoplasia type 2 (MEN-2), a clinical syndrome characterized by medullary...

ORIGINAL ARTICLE: Early Malignant Progression of Hereditary Medullary Thyroid Cancer

Thu, 16Â OctÂ 2003 00:00:00 -05:00

Background: An age-related progression from C-cell hyperplasia to medullary thyroid carcinoma is associated with various germ-line mutations in the rearranged during transfection (RET) proto-oncogene that could be used to identify...

IMAGES IN CLINICAL MEDICINE: Epidural Lipomatosis Causing Spinal Cord Compression

Moahad Saeed Dar, M.D. and Shadi Daoud, M.D. — Thu, 09Â OctÂ 2003 00:00:00 -05:00

A 68-year-old woman with corticosteroid-dependent chronic obstructive pulmonary disease was admitted to the hospital for worsening respiratory symptoms and for new-onset weakness in both legs. Physical examination revealed poor air...

CLINICAL PROBLEM-SOLVING: Mind the Gap

Alexander Turchin, M.D., Julian L. Seifter, M.D., and Ellen W. Seely, M.D. — Thu, 09Â OctÂ 2003 00:00:00 -05:00

A 62-year-old woman was admitted to the hospital because of abnormal liver-function tests. She had a history of acute myelogenous leukemia and had undergone transplantation of T-cellâdepleted allogeneic bone marrow...

CLINICAL PRACTICE: Combination EstrogenâProgestin Oral Contraceptives

Diana B. Petitti, M.D., M.P.H. — Thu, 09Â OctÂ 2003 00:00:00 -05:00

A healthy, sexually active, 35-year-old woman presents for advice about the use of oral contraceptives. She does not smoke cigarettes and has no personal or family history of venous thromboembolism,...

EDITORIAL: Parathyroid Hormone plus Alendronate â A Combination That Does Not Add Up

Sundeep Khosla, M.D. — Thu, 25Â SepÂ 2003 00:00:00 -05:00

The past 10 years have witnessed tremendous advances in our ability to treat osteoporosis. The old mainstays of therapy, estrogen and calcitonin, have fallen out of favor because of concern...

IMAGES IN CLINICAL MEDICINE: A Medical Mystery

Nicholas Magee, M.R.C.P. and Rory Convery, M.R.C.P. — Thu, 25Â SepÂ 2003 00:00:00 -05:00

A 48-year-old woman had a 30-year history of dermatomyositis treated with prednisolone. She was admitted for depression and nausea. Examination revealed multiple craggy skin nodules, shown on the right flank...

ORIGINAL ARTICLE: The Effects of Parathyroid Hormone, Alendronate, or Both in Men with Osteoporosis

Thu, 25Â SepÂ 2003 00:00:00 -05:00

Background: Because parathyroid hormone increases both bone formation and bone resorption, it is possible that combining parathyroid hormone with an antiresorptive agent will enhance its effect on bone mineral density.

Methods:...

ORIGINAL ARTICLE: The Effects of Parathyroid Hormone and Alendronate Alone or in Combination in Postmenopausal Osteoporosis

Thu, 25Â SepÂ 2003 00:00:00 -05:00

Background: Parathyroid hormone increases bone strength primarily by stimulating bone formation, whereas antiresorptive drugs reduce bone resorption. We conducted a randomized, double-blind clinical study of parathyroid hormone and alendronate to...

IMAGES IN CLINICAL MEDICINE: Ectopic Calcinosis

Yoshiaki Nishioka, M.D. and Masanobu Miyazaki, M.D. — Thu, 18Â SepÂ 2003 00:00:00 -05:00

A 52-year-old man who had been receiving hemodialysis for 11 years had severe secondary hyperparathyroidism. His serum level of intact parathyroid hormone was 1489 pg per milliliter. His bone mineral...

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: Chondroid Clival Chordoma

Andreas M. Stark, M.D. and H. Maximilian Mehdorn, M.D., Ph.D. — Thu, 04Â SepÂ 2003 00:00:00 -05:00

A 54-year-old man presented with a history of several weeks of fatigue and weight loss. He also reported atrophy of the testes and loss of secondary hair that had begun...

ORIGINAL ARTICLE: Inhibition of Food Intake in Obese Subjects by Peptide YY

Thu, 04Â SepÂ 2003 00:00:00 -05:00

Background: The gut hormone fragment peptide YY3â36 (PYY) reduces appetite and food intake when infused into subjects of normal weight. In common with the adipocyte hormone leptin, PYY reduces food...

PERSPECTIVE: Prescription-Drug Coverage for Medicare Beneficiaries

John K. Iglehart — Thu, 04Â SepÂ 2003 00:00:00 -05:00

The Congressional Budget Office has estimated that all spending for prescription drugs by and on behalf of Medicare's eligible population will total $1.8 trillion during the period from 2004 through...

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: Ovarian Hyperstimulation Syndrome Due to a Mutation in the Follicle-Stimulating Hormone Receptor

Thu, 21Â AugÂ 2003 00:00:00 -05:00

The ovarian hyperstimulation syndrome most often occurs as an iatrogenic complication of ovarian-stimulation treatments for in vitro fertilization (the incidence of severe forms ranges from 0.5 to 5 percent).1 The...

ORIGINAL ARTICLE: A Chorionic GonadotropinâSensitive Mutation in the Follicle-Stimulating Hormone Receptor as a Cause of Familial Gestational Spontaneous Ovarian Hyperstimulation Syndrome

Thu, 21Â AugÂ 2003 00:00:00 -05:00

Spontaneous ovarian hyperstimulation syndrome is a rare event, in contrast to iatrogenic ovarian hyperstimulation syndrome, which is induced with the use of gonadotropins in assisted reproductive medicine. The pathological features...

PERSPECTIVE: The Pathogenesis of the Ovarian Hyperstimulation Syndrome

Ursula Brigitte Kaiser, M.D. — Thu, 21Â AugÂ 2003 00:00:00 -05:00

The ovarian hyperstimulation syndrome is an iatrogenic complication of ovulation-induction therapy. In its most severe form, this syndrome involves massive ovarian enlargement and the formation of multiple ovarian cysts, fluid...

PERSPECTIVE: Hormone-Replacement Therapy and Cardiovascular Diseases

John Bailar, M.D., Ph.D. — Thu, 07Â AugÂ 2003 00:00:00 -05:00

Eighteen years ago, the Journal published two observational studies12 of the relations between postmenopausal hormone-replacement therapy and cardiovascular diseases. One found that women who used such therapy had about half...

PERSPECTIVE: From Presumed Benefit to Potential Harm â Hormone Therapy and Heart Disease

David M. Herrington, M.D., M.H.S., and Timothy D. Howard, Ph.D. — Thu, 07Â AugÂ 2003 00:00:00 -05:00

During the past decade, postmenopausal hormone therapy became one of the most frequently prescribed therapies in the United States, with a highly diversified portfolio of presumed benefits for postmenopausal women....

EDITORIAL: Paricalcitol as Compared with Calcitriol in Patients Undergoing Hemodialysis

Tilman B. DrÃ¼eke, M.D., and David A. McCarron, M.D. — Thu, 31Â JulÂ 2003 00:00:00 -05:00

Secondary hyperparathyroidism, a common consequence of chronic kidney disease, results from abnormal regulation of calcium and phosphate homeostasis. Three factors are central to its development. The first, reduced renal synthesis...

IMAGES IN CLINICAL MEDICINE: Massive Calcification in Pseudohypoparathyroidism

Tominaga Shimizu, M.D. and Teiji Takeda, M.D. — Thu, 31Â JulÂ 2003 00:00:00 -05:00

A 55-year-old man with a 24-year history of pseudohypoparathyroidism type 2 presented with a mass in the right buttock and reported difficulty walking. At the time of diagnosis, he had...

ORIGINAL ARTICLE: Survival of Patients Undergoing Hemodialysis with Paricalcitol or Calcitriol Therapy

Thu, 31Â JulÂ 2003 00:00:00 -05:00

Background: Elevated calcium and phosphorus levels after therapy with injectable vitamin D for secondary hyperparathyroidism may accelerate vascular disease and hasten death in patients undergoing long-term hemodialysis. Paricalcitol, a new...

REVIEW ARTICLE: Thyroiditis

Elizabeth N. Pearce, M.D., Alan P. Farwell, M.D., and Lewis E. Braverman, M.D. — Thu, 26Â JunÂ 2003 00:00:00 -05:00

The term thyroiditis encompasses many relatively common thyroid disorders, which have been classified according to various schemes (Table 1). In this article we review the diagnosis and treatment of the...

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: Effects of Estrogen plus Progestin on Health-Related Quality of Life

Thu, 08Â MayÂ 2003 00:00:00 -05:00

Background: The Women's Health Initiative (WHI) and other clinical trials indicate that significant health risks are associated with combination hormone use. Less is known about the effect of hormone therapy...

PERSPECTIVE: Postmenopausal Hormones â Therapy for Symptoms Only

Deborah Grady, M.D., M.P.H. — Thu, 08Â MayÂ 2003 00:00:00 -05:00

Over the past two decades, multiple observational studies have suggested that postmenopausal hormone therapy reduces the risks of osteoporotic fractures and coronary heart disease. On the basis of this evidence,...

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...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Hemoglobin and the Paracrine and Endocrine Functions of Nitric Oxide

Alan N. Schechter, M.D., and Mark T. Gladwin, M.D. — Thu, 10Â AprÂ 2003 00:00:00 -05:00

Nitric oxide is a highly reactive molecule with many biologic effects. Its function as the âendothelium-derived relaxing factorâ that regulates vascular tone is a central feature of its role as...

EDITORIAL: Aldosterone Blockade and Heart Failure

Mariell Jessup, M.D. — Thu, 03Â AprÂ 2003 00:00:00 -05:00

In the fall of 1999, a multicenter, randomized trial examining the effect of spironolactone on morbidity and mortality among patients with severe heart failure was published in the Journal.1 The...

ORIGINAL ARTICLE: Eplerenone, a Selective Aldosterone Blocker, in Patients with Left Ventricular Dysfunction after Myocardial Infarction

Thu, 03Â AprÂ 2003 00:00:00 -05:00

Background: Aldosterone blockade reduces mortality and morbidity among patients with severe heart failure. 03040329.xml We conducted a double-blind, placebo-controlled study evaluating the effect of eplerenone, a selective aldosterone blocker, on...

EDITORIAL: Defective Melanocortin 4 Receptors in Hyperphagia and Morbid Obesity

James F. List, M.D., Ph.D., and Joel F. Habener, M.D. — Thu, 20Â MarÂ 2003 00:00:00 -05:00

There has been a dramatic increase in the worldwide prevalence of obesity, a disorder with complex genetic traits that is influenced by environmental factors and that predisposes affected persons to...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 9-2003 â An 18-Year-Old Man with Back and Leg Pain and a Nondiagnostic Biopsy Specimen

Thu, 20Â MarÂ 2003 00:00:00 -05:00

An 18-year-old man was admitted to the hospital because of back and leg pain and a nondiagnostic biopsy specimen from a paravertebral mass.

The patient had been well until eight months...

REVIEW ARTICLE: The ChromograninâSecretogranin Family

Laurent Taupenot, Ph.D., Kimberly L. Harper, M.D., and Daniel T. O'Connor, M.D. — Thu, 20Â MarÂ 2003 00:00:00 -05:00

Neurons and neuroendocrine cells contain membrane-delimited pools of peptide hormones, biogenic amines, and neurotransmitters with a characteristic electron-dense appearance on transmission electron microscopy (Figure 1). These vesicles, which are present...

ORIGINAL ARTICLE: Clinical Spectrum of Obesity and Mutations in the Melanocortin 4 Receptor Gene

Thu, 20Â MarÂ 2003 00:00:00 -05:00

Background: Melanocortin 4 receptor (MC4R) deficiency is the commonest monogenic form of obesity. However, the clinical spectrum and mode of inheritance have not been defined, pathophysiological mechanisms leading to obesity...

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....

PERSPECTIVE: Can Growth Hormone Prevent Aging?

Mary Lee Vance, M.D. — Thu, 27Â FebÂ 2003 00:00:00 -05:00

Does growth hormone prevent aging? An article by Rudman et al. that appeared in the Journal in 19901 reported the effect on body composition of administering human growth hormone for...

PERSPECTIVE: Inappropriate Advertising of Dietary Supplements

Jeffrey M. Drazen, M.D. — Thu, 27Â FebÂ 2003 00:00:00 -05:00

Information about health is worthwhile only if it is accurate and fairly communicated. Recently, a number of advertisements on the Internet have contained statements about dietary supplements that could lead...

REVIEW ARTICLE: Corticosteroid Insufficiency in Acutely Ill Patients

Mark S. Cooper, M.D., and Paul M. Stewart, M.D. — Thu, 20Â FebÂ 2003 00:00:00 -05:00

An increase in tissue corticosteroid levels during acute illness is an important protective response. Many diseases and their treatments interfere with the normal corticosteroid response to illness and thus induce...

PERSPECTIVE: Cardiovascular Risk Factors and Antiretroviral Therapy

Daniel R. Kuritzkes, M.D., and Judith Currier, M.D. — Thu, 20Â FebÂ 2003 00:00:00 -05:00

The advent of potent combination antiretroviral therapy has led to a profound decrease in the rate of illness related to the acquired immunodeficiency syndrome (AIDS) and has significantly improved survival...

SOUNDING BOARD: Understanding the Divergent Data on Postmenopausal Hormone Therapy

Thu, 13Â FebÂ 2003 00:00:00 -05:00

Recent findings from clinical trials examining the relation between postmenopausal hormone therapy and coronary heart disease12 have appeared to be widely divergent from the results of earlier observational studies. In...

REVIEW ARTICLE: Selective Estrogen-Receptor Modulators â Mechanisms of Action and Application to Clinical Practice

B. Lawrence Riggs, M.D., and Lynn C. Hartmann, M.D. — Thu, 13Â FebÂ 2003 00:00:00 -05:00

The selective estrogen-receptor modulators (SERMs) represent a major therapeutic advance for clinical practice. Unlike estrogens, which are uniformly agonists, and antiestrogens, which are uniformly antagonists, the SERMs exert selective agonist...

PERSPECTIVE: Rethinking Postmenopausal Hormone Therapy

Caren G. Solomon, M.D., and Robert G. Dluhy, M.D. — Thu, 13Â FebÂ 2003 00:00:00 -05:00

In May 2002, the Women's Health Initiative (WHI) trial of daily combined therapy with estrogen and progestin was terminated early. The reason for stopping was an increased risk of breast...

ORIGINAL ARTICLE: Ectopic Luteinizing Hormone Secretion and Anovulation

Thu, 23Â JanÂ 2003 00:00:00 -05:00

In patients with amenorrhea and infertility, increased levels of serum luteinizing hormone and follicle-stimulating hormone are usually due to primary gonadal failure, whereas a selective increase in luteinizing hormone can...

CLINICAL PROBLEM-SOLVING: Easy to See but Hard to Find

Brendan M. Reilly, M.D., Peter Clarke, M.D., and Petros Nikolinakos, M.D. — Thu, 02Â JanÂ 2003 00:00:00 -05:00

A 46-year-old woman from the Philippines with no history of serious medical conditions was hospitalized with fatigue, body aches, and a weight loss of 4.5 kg (10 lb) over the...

ORIGINAL ARTICLE: Normal Vision despite Narrowing of the Optic Canal in Fibrous Dysplasia

Thu, 21Â NovÂ 2002 00:00:00 -05:00

Background: Fibrous dysplasia of bone frequently involves the anterior base of the cranium and results in encasement of the optic-nerve canals. It has been assumed that such encasement leads to...

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...

EDITORIAL: Genetic Control of Bone Remodeling â Insights from a Rare Disease

Thu, 18Â JulÂ 2002 00:00:00 -05:00

Over the past decade, we have learned a great deal about how skeletal development and remodeling are controlled.1 Some of our knowledge has been derived from genetic analysis of diseases...

PERSPECTIVE: B-Type Natriuretic Peptide â A Window to the Heart

Thu, 18Â JulÂ 2002 00:00:00 -05:00

Left ventricular dysfunction, regardless of its cause, results in characteristic hemodynamic derangements and a process of neurohormonal activation. Among the neuronal and hormonal pathways activated are the sympathetic nervous system,...

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...

EDITORIAL: The Stomach Speaks â Ghrelin and Weight Regulation

Thu, 23Â MayÂ 2002 00:00:00 -05:00

Recent years have witnessed explosive growth in knowledge of the physiologic factors regulating appetite and body weight.1 Among these are neural, metabolic, and endocrine signals from the periphery that reflect...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 11-2002 â A 27-Year-Old Woman with Two Intracardiac Masses and a History of Endocrinopathy

Thu, 11Â AprÂ 2002 00:00:00 -05:00

A 27-year-old woman was admitted to the hospital because of syncope and two intracardiac masses.

The patient had been well until about three years before admission, when she had an episode...

EDITORIAL: Individualized Hormone-Replacement Therapy?

Thu, 28Â MarÂ 2002 00:00:00 -05:00

For many years, the conventional wisdom, backed by observational epidemiologic studies, has held that âreplacementâ of estrogen after menopause would restore the relative protection from cardiovascular disease enjoyed by premenopausal...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 7-2002 â A 47-Year-Old Woman with Late Recurrent Hyperparathyroidism

Arthur E. Broadus, M.D., and Kristina M. Braaten, M.D. — Thu, 28Â FebÂ 2002 00:00:00 -05:00

A 47-year-old woman was admitted to the hospital because of recurrent hypercalcemia.

The patient had been admitted 14 years earlier because of hyperparathyroidism, with an increased level of calcium (12.3 mg...

ORIGINAL ARTICLE: Leptin-Replacement Therapy for Lipodystrophy

Thu, 21Â FebÂ 2002 00:00:00 -05:00

Background: The adipocyte hormone leptin is important in regulating energy homeostasis. Since severe lipodystrophy is associated with leptin deficiency, insulin resistance, hypertriglyceridemia, and hepatic steatosis, we assessed whether leptin replacement...

REVIEW ARTICLE: Production and Actions of Estrogens

Thu, 31Â JanÂ 2002 00:00:00 -05:00

Estrogens have widespread biologic actions, and there are naturally occurring phytoestrogens that mimic some of the actions of endogenously produced estrogens. In this review, we will focus on new biochemical...

ORIGINAL ARTICLE: Octreotide Therapy for Tumor-Induced Osteomalacia

Thu, 27Â DecÂ 2001 00:00:00 -05:00

Tumor-induced osteomalacia (also known as oncogenic osteomalacia)1 is a rare disorder characterized by phosphaturia, hypophosphatemia, and osteomalacia mimicking the clinical phenotype of either X-linked2 or autosomal dominant3 hereditary hypophosphatemic rickets....

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...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 35-2001 â An 81-Year-Old Man with Hypothermia, Bradycardia, and Confusion

Steven K. Grinspoon and E. Tessa Hedley-White — Thu, 15Â NovÂ 2001 00:00:00 -05:00

An 81-year-old man was transferred to the hospital because of bradycardia, hypothermia, and increasing confusion.

A platelet count of 85,000 per cubic millimeter had been discovered five years before admission, but...

ORIGINAL ARTICLE: Sperm Morphology, Motility, and Concentration in Fertile and Infertile Men

Thu, 08Â NovÂ 2001 00:00:00 -05:00

Background: Although semen analysis is routinely used to evaluate the male partner in infertile couples, sperm measurements that discriminate between fertile and infertile men are not well defined.

Methods: We evaluated...

ORIGINAL ARTICLE: Intensive Insulin Therapy in Critically Ill Patients

Thu, 08Â NovÂ 2001 00:00:00 -05:00

Background: Hyperglycemia and insulin resistance are common in critically ill patients, even if they have not previously had diabetes. Whether the normalization of blood glucose levels with insulin therapy improves...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 31-2001 â A 70-Year-Old Woman with End-Stage Renal Disease and Cutaneous Ulcers

Daniel T. Baran, M.D., and Gary St.A. Letts, M.D. — Thu, 11Â OctÂ 2001 00:00:00 -05:00

A 70-year-old woman was admitted to the hospital because of dyspnea and multiple cutaneous ulcers.

The patient had a history of long-standing type 2 diabetes mellitus as well as an old...

ORIGINAL ARTICLE: The Prognostic Value of B-Type Natriuretic Peptide in Patients with Acute Coronary Syndromes

Thu, 04Â OctÂ 2001 00:00:00 -05:00

Background: Brain (B-type) natriuretic peptide is a neurohormone synthesized predominantly in ventricular myocardium. Although the circulating level of this neurohormone has been shown to provide independent prognostic information in patients...

CLINICAL PRACTICE: Subclinical Hyperthyroidism

Anthony D. Toft, M.D. — Thu, 16Â AugÂ 2001 00:00:00 -05:00

A 67-year-old woman presents with palpitations and is found to be in atrial fibrillation at a rate of 120 beats per minute. The only other finding on physical examination...

CLINICAL PRACTICE: Subclinical Hypothyroidism

David S. Cooper, M.D. — Thu, 26Â JulÂ 2001 00:00:00 -05:00

A 59-year-old woman is found to have a serum thyrotropin level of 7 mU per liter on routine screening. Her only symptoms are mild fatigue, which has been present for...

CLINICAL PRACTICE: Postmenopausal Hormone-Replacement Therapy

JoAnn E. Manson, M.D., Dr.P.H., and Kathryn A. Martin, M.D. — Thu, 05Â JulÂ 2001 00:00:00 -05:00

Patient 1 is a 52-year-old woman at average risk for breast cancer and osteoporosis who is having menopause-related hot flashes and disturbed sleep. Patient 2 is a 58-year-old woman with...

EDITORIAL: Estrogen, Thyroxine Binding in Serum, and Thyroxine Therapy

Thu, 07Â JunÂ 2001 00:00:00 -05:00

In this issue of the Journal, Arafah presents data indicating that some women with hypothyroidism receiving thyroxine therapy needed more thyroxine when they received estrogen therapy â a change caused...

ORIGINAL ARTICLE: Increased Need for Thyroxine in Women with Hypothyroidism during Estrogen Therapy

Baha M. Arafah, M.D. — Thu, 07Â JunÂ 2001 00:00:00 -05:00

Background: Women with hypothyroidism that is being treated with thyroxine often need higher doses when they are pregnant. Whether this need can be attributed solely to estrogen-induced increases in serum...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 15-2001 â A 72-Year-Old Man with Persistent Fever and Hypotension

Ursula B. Kaiser, E. Tessa Hedley-Whyte — Thu, 17Â MayÂ 2001 00:00:00 -05:00

A 72-year-old man was admitted to the hospital because of persistent fever and hypotension.

The patient had been well until eight days earlier, when nausea and vomiting developed, with lower abdominal...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 13-2001 â A 19-Year-Old Man with Bouts of Hypertension and Severe Headaches

Paul R. Conlin, William C. Faquin — Thu, 26Â AprÂ 2001 00:00:00 -05:00

A 19-year-old man was admitted to the hospital because of headaches and hypertension.

The patient had been well until two years earlier, when he began to have episodic mild headaches that...

REVIEW ARTICLE: Thyroid Hormone and the Cardiovascular System

Irwin Klein, M.D., and Kaie Ojamaa, Ph.D. — Thu, 15Â FebÂ 2001 00:00:00 -05:00

Thyroid hormone has many effects on the heart and vascular system.1 Many of the clinical manifestations of hyperthyroidism are due to the ability of thyroid hormone to alter cardiovascular hemodynamics.2...

ORIGINAL ARTICLE: Botulinum Toxin A for Axillary Hyperhidrosis (Excessive Sweating)

Thu, 15Â FebÂ 2001 00:00:00 -05:00

Background: Treatment of primary focal hyperhidrosis is often unsatisfactory. Botulinum toxin A can stop excessive sweating by blocking the release of acetylcholine, which mediates sympathetic neurotransmission in the sweat glands.

Methods:...

ORIGINAL ARTICLE: Malabsorption Due to Cholecystokinin Deficiency in a Patient with Autoimmune Polyglandular Syndrome Type I

Thu, 25Â JanÂ 2001 00:00:00 -05:00

Autoimmune polyglandular syndrome type I is an autosomal recessive inherited disease caused by mutations in the autoimmune regulator gene.1 Its hallmarks are the failure of multiple endocrine glands due to...

REVIEW ARTICLE: Hyperparathyroid and Hypoparathyroid Disorders

Stephen J. Marx, M.D. — Thu, 21Â DecÂ 2000 00:00:00 -05:00

The four parathyroid glands, through the secretion of parathyroid hormone, regulate serum calcium concentrations and bone metabolism.1 In turn, serum calcium concentrations regulate parathyroid hormone secretion; high concentrations inhibit secretion...

IMAGES IN CLINICAL MEDICINE: Trousseau's Sign

Michael E. Meininger, M.D. and Jason S. Kendler, M.D. — Thu, 21Â DecÂ 2000 00:00:00 -05:00

Figure 1. A 51-year-old woman with mild hypertension for which she was taking a beta-adrenergic antagonist and a thiazide diuretic was sent to the emergency department because of the discovery...

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: Graves' Disease

Anthony P. Weetman, M.D., D.Sc. — Thu, 26Â OctÂ 2000 00:00:00 -05:00

Robert Graves first identified the association of goiter, palpitations, and exophthalmos in 1835, although Caleb Parry had published details of a case 10 years earlier. The discovery of a thyroid-stimulating...

EDITORIAL: Melatonin, Circadian Rhythms, and Sleep

Thu, 12Â OctÂ 2000 00:00:00 -05:00

Disturbances in circadian rhythms often result in disturbances in sleep. Examples include syndromes in which sleep time is delayed or advanced, the sleeping problems associated with jet lag and shift...

ORIGINAL ARTICLE: Entrainment of Free-Running Circadian Rhythms by Melatonin in Blind People

Thu, 12Â OctÂ 2000 00:00:00 -05:00

Background: Most totally blind people have circadian rhythms that are âfree-runningâ (i.e., that are not synchronized to environmental time cues and that oscillate on a cycle slightly longer than 24...

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...

EDITORIAL: A Novel Explanation for Resistance to Androgens

Thu, 21Â SepÂ 2000 00:00:00 -05:00

Clinical disorders that arise as a result of resistance to the intracellular action of a hormone are being characterized with increasing frequency. Perhaps the most notable example is the testicular...

ORIGINAL ARTICLE: Androgen-Insensitivity Syndrome as a Possible Coactivator Disease

Thu, 21Â SepÂ 2000 00:00:00 -05:00

Androgen-insensitivity syndromes in 46,XY fetuses result in various degrees of impairment in genital virilization.1 These syndromes are caused by mutations in the androgen receptor gene that result in decreased binding...

EDITORIAL: Sex, Hormones, and Hysterectomies

Thu, 07Â SepÂ 2000 00:00:00 -05:00

In this issue of the Journal, Shifren and coworkers1 postulate that the reduction in androgens resulting from bilateral oophorectomy is associated with sexual dysfunction in some women and that testosterone...

ORIGINAL ARTICLE: Transdermal Testosterone Treatment in Women with Impaired Sexual Function after Oophorectomy

Thu, 07Â SepÂ 2000 00:00:00 -05:00

Background: The ovaries provide approximately half the circulating testosterone in premenopausal women. After bilateral oophorectomy, many women report impaired sexual functioning despite estrogen replacement. We evaluated the effects of transdermal...

ORIGINAL ARTICLE: Alendronate for the Treatment of Osteoporosis in Men

Thu, 31Â AugÂ 2000 00:00:00 -05:00

Background: Despite its association with disability, death, and increased medical costs, osteoporosis in men has been relatively neglected as a subject of study. There have been no large, controlled trials...

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,...

ORIGINAL ARTICLE: Acromegaly Caused by Secretion of Growth Hormone by a Non-Hodgkin's Lymphoma

Thu, 22Â JunÂ 2000 00:00:00 -05:00

Acromegaly is a systemic disorder caused by sustained hypersecretion of growth hormone. The typical features include thickening of the skin, enlargement of the hands, feet, and mandible, and visceromegaly.12 Active...

REVIEW ARTICLE: Erectile Dysfunction

Tom F. Lue, M.D. — Thu, 15Â JunÂ 2000 00:00:00 -05:00

Erectile dysfunction is defined as the inability to achieve and maintain an erection sufficient to permit satisfactory sexual intercourse.1 It has been estimated to affect 20 million to 30 million...

ORIGINAL ARTICLE: A Preliminary Study of Growth Hormone Therapy for Crohn's Disease

Thu, 01Â JunÂ 2000 00:00:00 -05:00

Background: Crohn's disease is a chronic inflammatory disorder of the bowel. In a preliminary study, we evaluated whether the administration of growth hormone (somatropin) as well as a high-protein diet...

EDITORIAL: Treatment of Acromegaly

Thu, 20Â AprÂ 2000 00:00:00 -05:00

Acromegaly is the clinical expression of chronic secretion of excessive amounts of growth hormone. Its cardinal clinical manifestation is thickening of the skin, soft tissue, and bones of the hands...

ORIGINAL ARTICLE: Treatment of Acromegaly with the Growth HormoneâReceptor Antagonist Pegvisomant

Thu, 20Â AprÂ 2000 00:00:00 -05:00

Background: Patients with acromegaly are treated with surgery, radiation therapy, and drugs to reduce hypersecretion of growth hormone, but the treatments may be ineffective and have adverse effects. Pegvisomant is...

REVIEW ARTICLE: Medical Termination of Pregnancy

Thu, 30Â MarÂ 2000 00:00:00 -05:00

Termination of pregnancy has been practiced since antiquity. Although many societies accept this practice, some reject it, and it is sometimes even considered a crime. The most widely used methods...

IMAGES IN CLINICAL MEDICINE: Pituitary Apoplexy

Latha Dulipsingh, M.D. and M. Nathan Lassman, M.D. — Thu, 24Â FebÂ 2000 00:00:00 -05:00

Figure 1. A 70-year-old man presented with a five-day history of worsening headaches associated with vomiting and confusion on the day of admission. He was being treated with warfarin for...

REVIEW ARTICLE: The Physiology of Parathyroid HormoneâRelated Protein

Gordon J. Strewler, M.D. — Thu, 20Â JanÂ 2000 00:00:00 -05:00

Parathyroid hormoneârelated protein was identified in the 1980s as a tumor product that had the ability to activate parathyroid hormone receptors and cause hypercalcemia.1 Parathyroid hormoneârelated protein resembles parathyroid hormone...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Getting a Handle on âGoodâ Cholesterol with the High-Density Lipoprotein Receptor

Thu, 23Â DecÂ 1999 00:00:00 -05:00

Plasma lipoproteins transport fats (cholesterol and triglyceride) and hence have a key role in atherosclerotic cardiovascular disease. Low-density lipoprotein (LDL) cholesterol promotes atherogenesis, and high-density lipoprotein (HDL) cholesterol inhibits it....

EDITORIAL: Precocious Puberty in Boys

Thu, 02Â DecÂ 1999 00:00:00 -05:00

Normal puberty starts when pulsatile release of gonadotropin-releasing hormone (GnRH) from the hypothalamus begins. This triggers the release of luteinizing hormone and follicle-stimulating hormone from the pituitary. In both sexes,...

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...

IMAGES IN CLINICAL MEDICINE: Brown Tumor in End-Stage Renal Disease

George M. Nassar, M.D. and Juan Carlos Ayus, M.D. — Thu, 25Â NovÂ 1999 00:00:00 -05:00

Figure 1. A 62-year-old man with end-stage renal disease from hypertensive nephrosclerosis that had been treated by hemodialysis for 14 years presented to the emergency room with a productive cough...

ORIGINAL ARTICLE: Leuprolide Acetate Therapy in Luteinizing HormoneâDependent Cushing's Syndrome

Thu, 18Â NovÂ 1999 00:00:00 -05:00

Corticotropin-independent Cushing's syndrome is usually caused by cortisol-secreting adrenal adenomas, carcinomas, or (rarely) bilateral adrenal hyperplasia. In some patients with this syndrome, the excess secretion of cortisol is caused by...

EDITORIAL: Treatment of Primary Hyperparathyroidism

Thu, 21Â OctÂ 1999 00:00:00 -05:00

Hypercalcemia, and subsequently primary hyperparathyroidism, is discovered in two ways. Most commonly, hypercalcemia is discovered when serum calcium is measured as a screening test or in patients who have fatigue,...

ORIGINAL ARTICLE: A 10-Year Prospective Study of Primary Hyperparathyroidism with or without Parathyroid Surgery

Thu, 21Â OctÂ 1999 00:00:00 -05:00

Background: In the United States, most patients with primary hyperparathyroidism have few or no symptoms. The need for parathyroidectomy to treat all patients with this disorder has therefore been questioned.

Methods:...

REVIEW ARTICLE: Growth Hormone Therapy in Adults and Children

Mary Lee Vance, M.D., and Nelly Mauras, M.D. — Thu, 14Â OctÂ 1999 00:00:00 -05:00

Growth hormone is produced by the somatotroph cells of the anterior pituitary. Its secretion is stimulated by growth hormoneâreleasing hormone and inhibited by somatostatin, which are both produced by the...

EDITORIAL: Dehydroepiandrosterone for Adrenal Insufficiency

Thu, 30Â SepÂ 1999 00:00:00 -05:00

The human adrenal cortex secretes not only the essential hormones cortisol and aldosterone, but also dehydroepiandrosterone and large amounts of dehydroepiandrosterone sulfate. The serum concentration of dehydroepiandrosterone sulfate is 20...

ORIGINAL ARTICLE: Dehydroepiandrosterone Replacement in Women with Adrenal Insufficiency

Thu, 30Â SepÂ 1999 00:00:00 -05:00

Background: The physiologic role of dehydroepiandrosterone in humans is still unclear. Adrenal insufficiency leads to a deficiency of dehydroepiandrosterone; we therefore investigated the effects of dehydroepiandrosterone replacement in patients with...

EDITORIAL: The Role of Leptin in Human Physiology

Thu, 16Â SepÂ 1999 00:00:00 -05:00

In humans, the relative long-term constancy of body weight, the difficulty of successfully sustaining intentional weight loss, and the metabolic and behavioral alterations that accompany weight change provide strong evidence...

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: Growth Hormone Therapy in Critically Ill Patients

Thu, 09Â SepÂ 1999 00:00:00 -05:00

In this issue of the Journal, Takala et al.1 report an increase in mortality among critically ill patients treated with growth hormone. This finding is of major importance and will...

ORIGINAL ARTICLE: Increased Mortality Associated with Growth Hormone Treatment in Critically Ill Adults

Thu, 09Â SepÂ 1999 00:00:00 -05:00

Background: The administration of growth hormone can attenuate the catabolic response to injury, surgery, and sepsis. However, the effect of high doses of growth hormone on the length of stay...

EDITORIAL: Tumor-Induced Hypoglycemia

Thu, 02Â SepÂ 1999 00:00:00 -05:00

Hypoglycemia is a common medical emergency. Among hospitalized patients, it is most common in those with diabetes mellitus, but it also occurs in patients with renal insufficiency, liver disease, malnutrition,...

ORIGINAL ARTICLE: Hypoglycemia Due to an Insulin-Secreting Small-Cell Carcinoma of the Cervix

Thu, 02Â SepÂ 1999 00:00:00 -05:00

Hypoglycemia is a condition commonly seen in the emergency department and is usually caused by insulin or sulfonylurea therapy for diabetes mellitus. Tumor-induced hypoglycemia occurs more rarely and can involve...

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...

EDITORIAL: Maternal Hypothyroidism and Fetal Development

Thu, 19Â AugÂ 1999 00:00:00 -05:00

Thyroid deficiency during the latter two thirds of gestation and the first months after delivery can result in mental retardation and sometimes neurologic deficits. Whether thyroid hormone is needed during...

REVIEW ARTICLE: Hormones and Hemodynamics in Heart Failure

Robert W. Schrier, M.D., and William T. Abraham, M.D. — Thu, 19Â AugÂ 1999 00:00:00 -05:00

Heart failure is a major cause of cardiovascular mortality and morbidity, resulting in more than 1 million hospitalizations per year, and is the most common hospital-discharge diagnosis among patients older...

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...

ORIGINAL ARTICLE: A Comparison of Calcium, Vitamin D, or Both for Nutritional Rickets in Nigerian Children

Thu, 19Â AugÂ 1999 00:00:00 -05:00

Background: Nutritional rickets remains prevalent in many tropical countries despite the fact that such countries have ample sunlight. Some postulate that a deficiency of dietary calcium, rather than vitamin D,...

ORIGINAL ARTICLE: Maternal Thyroid Deficiency during Pregnancy and Subsequent Neuropsychological Development of the Child

Thu, 19Â AugÂ 1999 00:00:00 -05:00

Background: When thyroid deficiency occurs simultaneously in a pregnant woman and her fetus, the child's neuropsychological development is adversely affected. Whether developmental problems occur when only the mother has hypothyroidism...

REVIEW ARTICLE: Drug Treatment of Lipid Disorders

Robert H. Knopp, M.D. — Thu, 12Â AugÂ 1999 00:00:00 -05:00

Arteriosclerosis of the coronary and peripheral vasculature is the leading cause of death among men and women in the United States1 and worldwide.2 In 1992, for example, cardiovascular disease accounted...

SOUNDING BOARD: Trans Fatty Acids and Coronary Heart Disease

Thu, 24Â JunÂ 1999 00:00:00 -05:00

Trans unsaturated fatty acids are produced commercially in large quantities by heating vegetable oils in the presence of metal catalysts and hydrogen to form shortening and margarine.1 They are so...

ORIGINAL ARTICLE: Effects of Different Forms of Dietary Hydrogenated Fats on Serum Lipoprotein Cholesterol Levels

Thu, 24Â JunÂ 1999 00:00:00 -05:00

Background: Metabolic studies suggest that fatty acids containing at least one double bond in the trans configuration, which are found in hydrogenated fat, have a detrimental effect on serum lipoprotein...

ORIGINAL ARTICLE: Plasma Normetanephrine and Metanephrine for Detecting Pheochromocytoma in von HippelâLindau Disease and Multiple Endocrine Neoplasia Type 2

Thu, 17Â JunÂ 1999 00:00:00 -05:00

Background: The detection of pheochromocytomas in patients at risk for these tumors, such as patients with von HippelâLindau disease or multiple endocrine neoplasia type 2 (MEN-2), is hindered by the...

REVIEW ARTICLE: Treatments for Wasting in Patients with the Acquired Immunodeficiency Syndrome

Colleen Corcoran, N.P., and Steven Grinspoon, M.D. — Thu, 03Â JunÂ 1999 00:00:00 -05:00

Wasting was an early identifying characteristic of human immunodeficiency virus (HIV) infection, originally termed âslim diseaseâ in Africa.1 As defined by the Centers for Disease Control and Prevention (CDC), wasting...

ORIGINAL ARTICLE: Ovulatory and Metabolic Effects of d--Inositol in the Polycystic Ovary Syndrome

Thu, 29Â AprÂ 1999 00:00:00 -05:00

Background: Women with the polycystic ovary syndrome have insulin resistance and hyperinsulinemia, possibly because of a deficiency of a d-chiro-inositolâcontaining phosphoglycan that mediates the action of insulin. We hypothesized that...

ORIGINAL ARTICLE: Hypogonadotropic Hypogonadism in a Female Caused by an X-Linked Recessive Mutation in the Gene

Thu, 22Â AprÂ 1999 00:00:00 -05:00

Adrenal hypoplasia congenita is a rare X-linked disorder characterized by primary adrenal insufficiency and hypogonadotropic hypogonadism.1 All patients described to date have been male, and female carriers have had no...

EDITORIAL: The Causes of Neonatal Hypoglycemia

Thu, 15Â AprÂ 1999 00:00:00 -05:00

The risk of hypoglycemia in neonates is high. If their first feeding is delayed for as little as three to six hours after birth, 10 percent of normal neonates cannot...

REVIEW ARTICLE: Genetic Disorders of Renal Electrolyte Transport

Thu, 15Â AprÂ 1999 00:00:00 -05:00

In the 1950s and 1960s, several inherited disorders of fluid and electrolyte metabolism were described in which the principal disturbance appeared to be a specific functional defect in the renal...

ORIGINAL ARTICLE: Clinical Features of 52 Neonates with Hyperinsulinism

Thu, 15Â AprÂ 1999 00:00:00 -05:00

Background: Neonatal hyperinsulinemic hypoglycemia is often resistant to medical therapy and is often treated with near-total pancreatectomy. However, the pancreatic lesions may be focal and treatable by partial pancreatic resection.

Methods:...

ORIGINAL ARTICLE: Central Hypothyroidism Associated with Retinoid X ReceptorâSelective Ligands

Thu, 08Â AprÂ 1999 00:00:00 -05:00

Background: The occurrence of symptomatic central hypothyroidism (characterized by low serum thyrotropin and thyroxine concentrations) in a patient with cutaneous T-cell lymphoma during therapy with the retinoid X receptorâselective ligand...

REVIEW ARTICLE: The Expanding Spectrum of G Protein Diseases

Zvi Farfel, M.D., Henry R. Bourne, M.D., and Taroh Iiri, M.D., Ph.D. — Thu, 01Â AprÂ 1999 00:00:00 -05:00

Disease-causing mutations often reveal key pathways of physiologic regulation and their underlying molecular mechanisms. Mutations in the trimeric guanine nucleotide-binding proteins (G proteins), which relay signals initiated by photons, odorants,...

ORIGINAL ARTICLE: Impaired Counterregulation of Glucose in a Patient with Hypothalamic Sarcoidosis

Thu, 18Â MarÂ 1999 00:00:00 -05:00

Hypoglycemia stimulates rapid increases in the secretion of several hormones, including catecholamines, glucagon, cortisol, and growth hormone, that act in concert to increase the plasma glucose concentration. The chief role...

REVIEW ARTICLE: Single-Gene Mutations Resulting in Reproductive Dysfunction in Women

Eli Y. Adashi, M.D., and Jon D. Hennebold, Ph.D. — Thu, 04Â MarÂ 1999 00:00:00 -05:00

Fertility in women is regulated by a series of highly coordinated and synchronized interactions in the hypothalamicâpituitaryâovarian axis (Figure 1A). The central regulator of the axis is the group of...

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...

IMAGES IN CLINICAL MEDICINE: Pituitary Gigantism

Robert F. Gagel, M.D. and Ian E. McCutcheon, M.D. — Thu, 18Â FebÂ 1999 00:00:00 -05:00

Figure 1. A 22-year-old man with gigantism due to excess growth hormone is shown to the left of his identical twin. The increased height (Panel A) and enlarged hand (Panel...

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: Thyroid Hormone Replacement â One Hormone or Two?

Thu, 11Â FebÂ 1999 00:00:00 -05:00

Extracts of animal thyroid tissue, first used in 1892, contained both thyroxine and triiodothyronine and were the only available treatment for hypothyroidism for some 50 years. Because of concern about...

ORIGINAL ARTICLE: Effects of Thyroxine as Compared with Thyroxine plus Triiodothyronine in Patients with Hypothyroidism

Thu, 11Â FebÂ 1999 00:00:00 -05:00

Background: Patients with hypothyroidism are usually treated with thyroxine (levothyroxine) only, although both thyroxine and triiodothyronine are secreted by the normal thyroid gland. Whether thyroid secretion of triiodothyronine is physiologically...

REVIEW ARTICLE: Primary Aldosteronism

Arunabha Ganguly, M.D. — Thu, 17Â DecÂ 1998 00:00:00 -05:00

Primary aldosteronism resulting from an adrenocortical adenoma (aldosteronoma), as described by Conn,1 is one of a few potentially curable forms of hypertension. Aldosteronoma is the most common cause of primary...

ORIGINAL ARTICLE: Familial Gestational Hyperthyroidism Caused by a Mutant Thyrotropin Receptor Hypersensitive to Human Chorionic Gonadotropin

Thu, 17Â DecÂ 1998 00:00:00 -05:00

Some degree of stimulation of the thyroid gland by human chorionic gonadotropin is common during early pregnancy.123 When serum chorionic gonadotropin concentrations are abnormally high â for example, in women...

REVIEW ARTICLE: Tamoxifen in the Treatment of Breast Cancer

C. Kent Osborne, M.D. — Thu, 26Â NovÂ 1998 00:00:00 -05:00

Breast cancer is the most common cancer in women in the Western world. Because breast cancer is estrogen-dependent, reducing estrogen secretion by oophorectomy, hypophysectomy, or adrenalectomy can cause the cancer...

IMAGES IN CLINICAL MEDICINE: Tumors of the Pituitary, Pancreas, and Parathyroid Glands in a Patient with Multiple Endocrine Neoplasia Type 1

Kenneth J. Simcic, M.D. and Albert J. Moreno, M.D. — Thu, 26Â NovÂ 1998 00:00:00 -05:00

Figure 1. In 1992, a T1-weighted magnetic resonance imaging study detected a macroadenoma of the pituitary (arrowhead in Panel A) in a 41-year-old man with transient right-sided weakness. He had...

EDITORIAL: KashinâBeck Disease â Expanding the Spectrum of Iodine-Deficiency Disorders

Thu, 15Â OctÂ 1998 00:00:00 -05:00

Selenium, as selenocysteine, is an integral component of two important enzymes â glutathione peroxidase and iodothyronine deiodinase â that are present in many tissues, including the thyroid gland. Glutathione peroxidase...

IMAGES IN CLINICAL MEDICINE: Mediastinal Goiter

Silvio E. Inzucchi, M.D. and Cristina M. Brunet, M.D. — Thu, 15Â OctÂ 1998 00:00:00 -05:00

Figure 1. A 51-year-old woman presented for evaluation of a mediastinal mass. Eleven years earlier, she had undergone subtotal thyroidectomy for a large, multinodular goiter. The mediastinal mass was first...

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: Estrogen and the Risk of Fracture â New Data, New Questions

Thu, 10Â SepÂ 1998 00:00:00 -05:00

The central role of estrogen deficiency in postmenopausal bone loss was demonstrated many years ago.1 We know estrogen can inhibit bone resorption and decrease bone turnover, but the precise pathways...

ORIGINAL ARTICLE: Aberrant Interleukin-1 Receptors in a Cortisol-Secreting Adrenal Adenoma Causing Cushing's Syndrome

Thu, 02Â JulÂ 1998 00:00:00 -05:00

Cortisol-secreting adrenal adenomas are an uncommon cause of Cushing's syndrome. Little is known about the events leading to the formation of these tumors, but molecular defects, including activating mutations of...

ORIGINAL ARTICLE: Effects of Metformin on Spontaneous and Clomiphene-Induced Ovulation in the Polycystic Ovary Syndrome

Thu, 25Â JunÂ 1998 00:00:00 -05:00

Background: Obese women with the polycystic ovary syndrome are relatively unresponsive to the induction of ovulation by clomiphene. We hypothesized that reducing insulin secretion by administering metformin would increase the...

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: Decreased Epinephrine Responses to Hypoglycemia during Sleep

Thu, 04Â JunÂ 1998 00:00:00 -05:00

Background: In patients with type I diabetes mellitus, hypoglycemia occurs commonly during sleep and is frequently asymptomatic. This raises the question of whether sleep is associated with reduced counterregulatory-hormone responses...

IMAGES IN CLINICAL MEDICINE: Achilles'-Tendon Xanthoma in Familial Hypercholesterolemia

Harrie C.M. van den Bosch, M.D. and Louwerens D. Vos, M.D. — Thu, 28Â MayÂ 1998 00:00:00 -05:00

Figure 1. A 22-year-old woman with a family history of hypercholesterolemia had had a soft-tissue swelling on the back of her left ankle for several years. Examination revealed a fusiform...

EDITORIAL: A Pill for Impotence

Thu, 14Â MayÂ 1998 00:00:00 -05:00

Impotence â the preferred term is now erectile dysfunction â is a common problem, especially among older men,1 and if it is not caused by psychological problems it certainly can...

REVIEW ARTICLE: Treatment of Benign Nodular Thyroid Disease

Ad R. Hermus, M.D., and Dyde A. Huysmans, M.D. — Thu, 14Â MayÂ 1998 00:00:00 -05:00

Nodular thyroid disease is common. Palpable thyroid nodules were detected in 0.8 percent of adult men and 5.3 percent of adult women in Whickham, northeast England,1 and in 1.5 percent...

IMAGES IN CLINICAL MEDICINE: Calcified Mass in a Patient on Long-Term Hemodialysis

Piotr Lazowski, M.D. and David S. Goldfarb, M.D. — Thu, 14Â MayÂ 1998 00:00:00 -05:00

Figure 1. A 46-year-old man with end-stage renal disease due to diabetic nephropathy had been undergoing hemodialysis for almost two years. Fourteen months after beginning dialysis, he reported pain below...

ORIGINAL ARTICLE: Hyperinsulinism and Hyperammonemia in Infants with Regulatory Mutations of the Glutamate Dehydrogenase Gene

Thu, 07Â MayÂ 1998 00:00:00 -05:00

Background: A new form of congenital hyperinsulinism characterized by hypoglycemia and hyperammonemia was described recently. We hypothesized that this syndrome of hyperinsulinism and hyperammonemia was caused by excessive activity of...

EDITORIAL: The Need for More Vitamin D

Thu, 19Â MarÂ 1998 00:00:00 -05:00

Vitamin D is an essential precursor of 1,25-dihydroxyvitamin D, the steroid hormone required not only for bone development and growth in children and maintenance of bone in adults, but also...

ORIGINAL ARTICLE: Hypovitaminosis D in Medical Inpatients

Thu, 19Â MarÂ 1998 00:00:00 -05:00

Background: Vitamin D deficiency is a major risk factor for bone loss and fracture. Although hypovitaminosis D has been detected frequently in elderly and housebound people, the prevalence of vitamin...

REVIEW ARTICLE: Treatment of Postmenopausal Osteoporosis

Richard Eastell, M.D. — Thu, 12Â MarÂ 1998 00:00:00 -05:00

Osteoporosis affects an estimated 75 million people in Europe, the United States, and Japan.1 It is a preventable and treatable condition, yet many people with osteoporosis remain unrecognized and untreated....

ORIGINAL ARTICLE: Mortality after the Treatment of Hyperthyroidism with Radioactive Iodine

Thu, 12Â MarÂ 1998 00:00:00 -05:00

Background: Hyperthyroidism affects many organ systems, but the effects are usually considered reversible. The long-term effects of hyperthyroidism on mortality are not known.

Methods: We conducted a population-based study of mortality...

EDITORIAL: The Many Faces of Rickets

Thu, 05Â MarÂ 1998 00:00:00 -05:00

The typical clinical picture of rickets, first clearly described in the 17th century, includes growth-plate abnormalities and delayed growth, weakening and bowing of weight-bearing bones, hypoplasia of tooth enamel, and...

ORIGINAL ARTICLE: Inactivating Mutations in the 25-Hydroxyvitamin D 1Î±-Hydroxylase Gene in Patients with Pseudovitamin DâDeficiency Rickets

Thu, 05Â MarÂ 1998 00:00:00 -05:00

Background: Pseudovitamin Dâdeficiency rickets is characterized by the early onset of rickets with hypocalcemia and is thought to be caused by a deficit in renal 25-hydroxyvitamin D3 1Î±-hydroxylase, the key...

ORIGINAL ARTICLE: Antenatal Thyrotropin-Releasing Hormone to Prevent Lung Disease in Preterm Infants

Thu, 19Â FebÂ 1998 00:00:00 -05:00

Background: Pulmonary disease is common in preterm infants, despite antenatal glucocorticoid therapy. The addition of antenatal thyrotropin-releasing hormone therapy has been reported to decrease pulmonary morbidity in these infants.

Methods: We...

ORIGINAL ARTICLE: Treatment of Men with Paraphilia with a Long-Acting Analogue of Gonadotropin-Releasing Hormone

Ariel RÃ¶sler, M.D., and Eliezer Witztum, M.D. — Thu, 12Â FebÂ 1998 00:00:00 -05:00

Background: Men with deviant sexual behavior, or paraphilia, are usually treated with psychotherapy, antidepressant drugs, progestins, and antiandrogens, but these treatments are often ineffective. Selective inhibition of pituitaryâgonadal function with...

REVIEW ARTICLE: Papillary and Follicular Thyroid Carcinoma

Martin Jean Schlumberger, M.D. — Thu, 29Â JanÂ 1998 00:00:00 -05:00

Papillary and follicular (differentiated) thyroid carcinomas are among the most curable cancers. However, some patients are at high risk for recurrent disease or even death. Most of these patients can...

ORIGINAL ARTICLE: Familial Hyperinsulinism Caused by an Activating Glucokinase Mutation

Thu, 22Â JanÂ 1998 00:00:00 -05:00

Spontaneous hyperinsulinemic hypoglycemia in adults is most frequently caused by sporadic, solitary pancreatic beta-cell tumors, whereas hyperinsulinemic hypoglycemia in childhood is commonly caused by generalized beta-cell dysfunction.1 Mutations in the...

ORIGINAL ARTICLE: Differential Behavioral Effects of Gonadal Steroids in Women with and in Those without Premenstrual Syndrome

Thu, 22Â JanÂ 1998 00:00:00 -05:00

Background: The symptoms of women with premenstrual syndrome improve in response to suppression of ovarian function, although these women have no evidence of ovarian dysfunction. We undertook a study to...

ORIGINAL ARTICLE: Relation between Therapy for Hyperthyroidism and the Course of Graves' Ophthalmopathy

Thu, 08Â JanÂ 1998 00:00:00 -05:00

Background: The chief clinical characteristics of Graves' disease are hyperthyroidism and ophthalmopathy. The relation between the two and the effect of treatment for hyperthyroidism on ophthalmopathy are unclear.

Methods: We studied...

EDITORIAL: Preventing Graves' Ophthalmopathy

Thu, 08Â JanÂ 1998 00:00:00 -05:00

Graves' ophthalmopathy and Graves' hyperthyroidism are closely linked, but the precise relation between these different expressions of the same disease is poorly understood. One much-debated issue is whether ophthalmopathy develops...

REVIEW ARTICLE: The Thyrotropin Receptor in Thyroid Diseases

Ralf Paschke, M.D., and Marian Ludgate, Ph.D. — Thu, 04Â DecÂ 1997 00:00:00 -05:00

The growth and function of the thyroid are controlled by thyrotropin1 through the activation of its receptor, which belongs to the large family of G proteinâcoupled receptors. Despite the extreme...

IMAGES IN CLINICAL MEDICINE: A Medical Mystery

Richard Frothingham, M.D. — Thu, 04Â DecÂ 1997 00:00:00 -05:00

Figure 1. Which of these 43-year-old identical twins is the patient, and what is her diagnosis?...

ORIGINAL ARTICLE: A Family with Hypogonadotropic Hypogonadism and Mutations in the Gonadotropin-Releasing Hormone Receptor

Thu, 27Â NovÂ 1997 00:00:00 -05:00

Hypogonadotropic hypogonadism is often associated with anosmia in a condition known as Kallmann's syndrome. The gene for the X-linked form of Kallmann's syndrome has been mapped to chromosome Xp22.3,1 and...

ORIGINAL ARTICLE: Short-Term Inhibition of Parathyroid Hormone Secretion by a Calcium-Receptor Agonist in Patients with Primary Hyperparathyroidism

Thu, 20Â NovÂ 1997 00:00:00 -05:00

Background: Surgery is the usual therapy for patients with primary hyperparathyroidism. We investigated the ability of a calcimimetic drug that inhibits parathyroid hormone secretion in vitro to decrease serum parathyroid...

ORIGINAL ARTICLE: Propranolol Therapy for Ectopic Î²-Adrenergic Receptors in Adrenal Cushing's Syndrome

Thu, 13Â NovÂ 1997 00:00:00 -05:00

Most patients with corticotropin-independent Cushing's syndrome have an adrenal adenoma or carcinoma,1 but a few have bilateral adrenal hyperplasia. Three patients with Cushing's syndrome and corticotropin-independent bilateral adrenal hyperplasia234 and...

REVIEW ARTICLE: Corticosteroid Therapy in Severe Illness

Steven W.J. Lamberts, Hajo A. Bruining, and Frank H. de Jong — Thu, 30Â OctÂ 1997 00:00:00 -05:00

Severe illnesses, trauma, anesthesia, and surgery are accompanied by activation of the hypothalamicâpituitaryâadrenal axis, as demonstrated by increased serum corticotropin and cortisol concentrations.1234567 This activation is an essential component of...

EDITORIAL: Follow-up of Patients with Thyroid Carcinoma

Thu, 25Â SepÂ 1997 00:00:00 -05:00

There are about 14,000 new cases of thyroid carcinoma in the United States each year, about 90 percent of them differentiated â papillary and follicular â carcinomas, and about 1100...

ORIGINAL ARTICLE: Comparison of Administration of Recombinant Human Thyrotropin with Withdrawal of Thyroid Hormone for Radioactive Iodine Scanning in Patients with Thyroid Carcinoma

Thu, 25Â SepÂ 1997 00:00:00 -05:00

Background: To detect recurrent disease in patients who have had differentiated thyroid cancer, periodic withdrawal of thyroid hormone therapy may be required to raise serum thyrotropin concentrations to stimulate thyroid...

ORIGINAL ARTICLE: Delayed Puberty and Hypogonadism Caused by Mutations in the Follicle-Stimulating Hormone Î²-Subunit Gene

Thu, 28Â AugÂ 1997 00:00:00 -05:00

The pituitary gonadotropins luteinizing hormone and follicle-stimulating hormone (FSH) regulate the production of sex steroids necessary for pubertal development and fertility. Inherited genetic defects that cause hypogonadism have been identified...

ORIGINAL ARTICLE: Delayed Puberty and Hypogonadism Caused by Mutations in the Follicle-Stimulating Hormone Î²-Subunit Gene

Thu, 28Â AugÂ 1997 00:00:00 -05:00

ORIGINAL ARTICLE: Effect of Testosterone and Estradiol in a Man with Aromatase Deficiency

Thu, 10Â JulÂ 1997 00:00:00 -05:00

Recent reports of disruptive mutations of the genes for the estrogen receptor or for cytochrome P-450 aromatase123456 have shed new light on the role of estrogen. In females the lack...

REVIEW ARTICLE: Neuroendocrine Responses to Starvation and Weight Loss

Michael W. Schwartz, M.D., and Randy J. Seeley, Ph.D. — Thu, 19Â JunÂ 1997 00:00:00 -05:00

The inability to consume enough calories to meet energy requirements is a common threat to survival. To meet this danger, animals retard their fuel depletion by lowering their metabolic rate...

EDITORIAL: Serum MÃ¼llerian Inhibiting Substance Assay â A New Diagnostic Test for Disorders of Gonadal Development

Thu, 22Â MayÂ 1997 00:00:00 -05:00

Half a century has passed since Alfred Jost1 postulated that male sexual differentiation is mediated by two discrete substances produced by the fetal testis and that, in their absence, sexual...

ORIGINAL ARTICLE: Measurements of Serum MÃ¼llerian Inhibiting Substance in the Evaluation of Children with Nonpalpable Gonads

Thu, 22Â MayÂ 1997 00:00:00 -05:00

Background: MÃ¼llerian inhibiting substance, produced constitutively by the prepubertal testes, promotes involution of the mÃ¼llerian ducts during normal male sexual differentiation. In children with virilization and nonpalpable gonads, only those...

EDITORIAL: The Y Chromosome and Spermatogenesis

Thu, 20Â FebÂ 1997 00:00:00 -05:00

Infertility affects approximately 1 man in 25. In about 30 to 40 percent of affected men, the results of semen analyses are abnormal but no cause of the infertility can...

REVIEW ARTICLE: The Management of Paget's Disease of Bone

Pierre D. Delmas, M.D., Ph.D. and Pierre J. Meunier, M.D. — Thu, 20Â FebÂ 1997 00:00:00 -05:00

Paget's disease of bone is a localized, monostotic or polyostotic disease characterized by increased bone remodeling, bone hypertrophy, and abnormal bone structure that leads in symptomatic patients to pain and...

ORIGINAL ARTICLE: Microdeletions in the Y Chromosome of Infertile Men

Thu, 20Â FebÂ 1997 00:00:00 -05:00

Background: Some infertile men with azoospermia or severe oligospermia have small deletions in regions of the Y chromosome. However, the frequency of such microdeletions among men with infertility in general...

ORIGINAL ARTICLE: Adult-Onset Idiopathic Hypogonadotropic Hypogonadism â A Treatable Form of Male Infertility

Thu, 06Â FebÂ 1997 00:00:00 -05:00

Background: Men with isolated gonadotropin-releasing hormone (GnRH) deficiency typically present with an absence of pubertal development. We describe an adult-onset form of idiopathic hypogonadotropic hypogonadism that develops after puberty.

Methods: We...

ORIGINAL ARTICLE: Successful Treatment of a Patient with a Thymoma and Pure Red-Cell Aplasia with Octreotide and Prednisone

Thu, 23Â JanÂ 1997 00:00:00 -05:00

Thymomas are rare epithelial neoplasms frequently associated with myasthenia gravis, hypogammaglobulinemia, and pure red-cell aplasia.1234567891011 In pure red-cell aplasia, autoantibodies against early and late erythroid-cell progenitors or erythropoietin, as well...

EDITORIAL: Treatment, and Retreatment, of Cushing's Disease

Thu, 16Â JanÂ 1997 00:00:00 -05:00

The proximate cause of the clinical manifestations of Cushing's syndrome â obesity, facial plethora, hirsutism, hypertension, muscle weakness, lethargy, and depression â is excess cortisol secretion. Few of these clinical...

REVIEW ARTICLE: Melatonin in Humans

Amnon Brzezinski, M.D. — Thu, 16Â JanÂ 1997 00:00:00 -05:00

Three centuries ago, the French philosopher RenÃ© Descartes described the pineal gland as âthe seat of the soul,â but it was not until the late 1950s that melatonin, the principal...

ORIGINAL ARTICLE: The Long-Term Outcome of Pituitary Irradiation after Unsuccessful Transsphenoidal Surgery in Cushing's Disease

Thu, 16Â JanÂ 1997 00:00:00 -05:00

Background: Irradiation of the pituitary is widely considered the most appropriate treatment for patients with Cushing's disease in whom transsphenoidal microsurgery has been unsuccessful. However, there is little information about...

ORIGINAL ARTICLE: The Pathophysiology and Genetics of Congenital Lipoid Adrenal Hyperplasia

Thu, 19Â DecÂ 1996 00:00:00 -05:00

Background: Congenital lipoid adrenal hyperplasia results in severe impairment of steroid biosynthesis in the adrenal glands and gonads that is manifested both in utero and postnatally. We recently found mutations...

REVIEW ARTICLE: Turner's Syndrome

Paul Saenger, M.D. — Thu, 05Â DecÂ 1996 00:00:00 -05:00

Currently, there are 50,000 to 75,000 girls and women with Turner's syndrome in the United States alone. Turner's syndrome, first described in 1938,1 is the most common sex-chromosome abnormality in...

EDITORIAL: Insulin-Like Growth Factor I â An Important Intrauterine Growth Factor

Thu, 31Â OctÂ 1996 00:00:00 -05:00

The importance of insulin-like growth factor I (IGF-I) in regulating the effects of growth hormone on postnatal growth and development is well established, but its role during the prenatal period...

EDITORIAL: Insulin-Like Growth Factor I â An Important Intrauterine Growth Factor

Thu, 31Â OctÂ 1996 00:00:00 -05:00

The importance of insulin-like growth factor I (IGF-I) in regulating the effects of growth hormone on postnatal growth and development is well established, but its role during the prenatal period...

ORIGINAL ARTICLE: Intrauterine Growth Retardation and Postnatal Growth Failure Associated with Deletion of the Insulin-Like Growth Factor I Gene

Thu, 31Â OctÂ 1996 00:00:00 -05:00

Insulin-like growth factor I (IGF-I) mediates the majority of the growth-promoting effects of growth hormone (GH) after birth.1 In the prenatal period, GH does not appear to have a major...

ORIGINAL ARTICLE: Intrauterine Growth Retardation and Postnatal Growth Failure Associated with Deletion of the Insulin-Like Growth Factor I Gene

Thu, 31Â OctÂ 1996 00:00:00 -05:00

Insulin-like growth factor I (IGF-I) mediates the majority of the growth-promoting effects of growth hormone (GH) after birth.1 In the prenatal period, GH does not appear to have a major...

REVIEW ARTICLE: Adrenal Insufficiency

Wolfgang Oelkers, M.D. — Thu, 17Â OctÂ 1996 00:00:00 -05:00

The hypothalamicâpituitaryâadrenal axis has an important role in the body's ability to cope with stresses such as infections, hypotension, and surgery. The hypothalamus is subject to regulatory influences from other...

EDITORIAL: Familial Hypocalcemia â Not Hypoparathyroidism

Thu, 10Â OctÂ 1996 00:00:00 -05:00

Familial benign hypercalcemia, also known as familial hypocalciuric hypercalcemia, was first identified clinically when patients thought to have primary hyperparathyroidism whose condition did not improve after neck surgery were found...

ORIGINAL ARTICLE: A Familial Syndrome of Hypocalcemia with Hypercalciuria Due to Mutations in the Calcium-Sensing Receptor

Thu, 10Â OctÂ 1996 00:00:00 -05:00

Background: The calcium-sensing receptor regulates the secretion of parathyroid hormone in response to changes in extracellular calcium concentrations, and mutations that result in a loss of function of the receptor...

REVIEW ARTICLE: The Proto-Oncogene in Multiple Endocrine Neoplasia Type 2 and Hirschsprung's Disease

Charis Eng, M.D., Ph.D. — Thu, 26Â SepÂ 1996 00:00:00 -05:00

Recent years have brought major advances in our understanding of the molecular genetic basis of both types of multiple endocrine neoplasia (MEN), an autosomal dominant cancer syndrome. MEN type 1...

ORIGINAL ARTICLE: Premature Atherosclerosis in Patients with Familial Chylomicronemia Caused by Mutations in the Lipoprotein Lipase Gene

Thu, 19Â SepÂ 1996 00:00:00 -05:00

Background: Patients with lipoprotein lipase deficiency usually present with chylomicronemia in childhood. The syndrome has been considered nonatherogenic primarily because of the low levels of low-density lipoprotein (LDL) cholesterol. We...

EDITORIAL: Actions of Parathyroid HormoneâRelated Peptide and Its Receptors

Thu, 05Â SepÂ 1996 00:00:00 -05:00

Building on the insight gained from their initial identification of a mutation in the receptor for parathyroid hormone (PTH) and parathyroid hormoneârelated peptide (PTHrP) in a patient with Jansen's metaphyseal...

ORIGINAL ARTICLE: Constitutively Activated Receptors for Parathyroid Hormone and Parathyroid HormoneâRelated Peptide in Jansen's Metaphyseal Chondrodysplasia

Thu, 05Â SepÂ 1996 00:00:00 -05:00

Background: An activating mutation of the receptor for parathyroid hormone (PTH) and parathyroid hormoneârelated peptide (PTHrP) was recently found in a patient with Jansen's metaphyseal chondrodysplasia, a rare form of...

EDITORIAL: Insulin and the Polycystic Ovary Syndrome

Thu, 29Â AugÂ 1996 00:00:00 -05:00

The cardinal clinical manifestations of the polycystic ovary syndrome are hirsutism and anovulation, oligomenorrhea, or amenorrhea. About half the women with this syndrome are obese, and some have diabetes mellitus....

ORIGINAL ARTICLE: Decreases in Ovarian Cytochrome P450c17Î± Activity and Serum Free Testosterone after Reduction of Insulin Secretion in Polycystic Ovary Syndrome

John E. Nestler, M.D., and Daniela J. Jakubowicz, M.D. — Thu, 29Â AugÂ 1996 00:00:00 -05:00

Background: Insulin resistance and increased ovarian cytochrome P450c17Î± activity are both features of the polycystic ovary syndrome. P450c17Î±, which is involved in androgen biosynthesis, has both 17Î±-hydroxylase and 17,20-lyase activities....

SOUNDING BOARD: Physical and Emotional Problems of Elite Female Gymnasts

Thu, 25Â JulÂ 1996 00:00:00 -05:00

Within the past five years, two U.S. female gymnasts at the Olympic level, Christy Henrich and Julissa Gomez, died from medical problems related to their sport. Christy died from complications...

REVIEW ARTICLE: Chronic Autoimmune Thyroiditis

Colin M. Dayan, M.D., Ph.D., and Gilbert H. Daniels, M.D. — Thu, 11Â JulÂ 1996 00:00:00 -05:00

In 1912, Hashimoto described four women in whom the thyroid gland was enlarged and appeared to have been transformed into lymphoid tissue (âstruma lymphomatosaâ).1 Although the patients were not initially...

EDITORIAL: The Anabolic Action of Testosterone

Thu, 04Â JulÂ 1996 00:00:00 -05:00

The paper by Bhasin et al.1 in this issue of the Journal shows that a dose of testosterone enanthate (600 mg weekly for 10 weeks) that produces supraphysiologic serum concentrations...

ORIGINAL ARTICLE: The Effects of Supraphysiologic Doses of Testosterone on Muscle Size and Strength in Normal Men

Thu, 04Â JulÂ 1996 00:00:00 -05:00

Background: Athletes often take androgenic steroids in an attempt to increase their strength. The efficacy of these substances for this purpose is unsubstantiated, however.

Methods: We randomly assigned 43 normal men...

REVIEW ARTICLE: Angiotensin Receptors and Their Antagonists

Theodore L. Goodfriend, M.D., Mary E. Elliott, Ph.D., and Kevin J. Catt, M.D. — Thu, 20Â JunÂ 1996 00:00:00 -05:00

Angiotensins are peptide hormones derived from the protein precursor angiotensinogen by the sequential actions of proteolytic enzymes (Figure 1). The classic pathway of angiotensin synthesis includes a reaction catalyzed by...

ORIGINAL ARTICLE: Hyperinsulinemia as an Independent Risk Factor for Ischemic Heart Disease

Thu, 11Â AprÂ 1996 00:00:00 -05:00

Background: Prospective studies suggest that hyperinsulinemia may be an important risk factor for ischemic heart disease. However, it has not been determined whether plasma insulin levels are independently related to...

EDITORIAL: Prematurity-Associated Neurologic and Developmental Abnormalities and Neonatal Thyroid Function

Thu, 28Â MarÂ 1996 00:00:00 -05:00

The first complete description of transient hypothyroxinemia in low-birth-weight infants dates from 1981,1 and there has since been much speculation about the causes of the phenomenon. The hypothyroxinemia may be...

ORIGINAL ARTICLE: The Relation of Transient Hypothyroxinemia in Preterm Infants to Neurologic Development at Two Years of Age

Thu, 28Â MarÂ 1996 00:00:00 -05:00

Background: Transient hypothyroxinemia, a common finding in premature infants, is not thought to have long-term sequelae or to require treatment. We investigated whether hypothyroxinemia in premature infants is a cause...

REVIEW ARTICLE: Androgens in Men â Uses and Abuses

Carrie J. Bagatell, M.D., and William J. Bremner, M.D., Ph.D. — Thu, 14Â MarÂ 1996 00:00:00 -05:00

The female steroid hormones, estrogen and progesterone, are prescribed widely by physicians, and their risks and benefits have been studied extensively. Although androgen preparations have been available for many years,...

REVIEW ARTICLE: Adrenergic Receptors â Evolving Concepts and Clinical Implications

Paul A. Insel, M.D. — Thu, 29Â FebÂ 1996 00:00:00 -05:00

The endogenous catecholamines â norepinephrine and epinephrine â are involved in the regulation of virtually every organ system. Norepinephrine acts as a neurotransmitter at certain sites in the central nervous...

ORIGINAL ARTICLE: Testicular and Ovarian Resistance to Luteinizing Hormone Caused by Inactivating Mutations of the Luteinizing HormoneâReceptor Gene

Thu, 22Â FebÂ 1996 00:00:00 -05:00

In normal males, luteinizing hormone (LH) regulates the function of Leydig cells and, hence, male sexual differentiation, pubertal androgenization, male sexual function, and fertility. Abnormalities in the function of Leydig...

ORIGINAL ARTICLE: Short Stature Due to Genetic Defects Affecting Growth Hormone Activity

Thu, 15Â FebÂ 1996 00:00:00 -05:00

Postnatal growth and, to a much lesser degree, intrauterine growth are critically dependent on the expression of the pituitary growth hormone GH-1 gene and the consequent generation of insulin-like growth...

ORIGINAL ARTICLE: Short Stature Caused by a Mutant Growth Hormone

Thu, 15Â FebÂ 1996 00:00:00 -05:00

The causes of growth hormoneâdependent short stature are primary pituitary disease, pituitary deficiency due to hypothalamic dysfunction, and, less often, insensitivity to growth hormone. The prototypical syndrome of growth hormone...

REVIEW ARTICLE: Hypertension and Associated Metabolic Abnormalities â The Role of Insulin Resistance and the Sympathoadrenal System

Gerald M. Reaven, M.D., Hans Lithell, M.D., and Lewis Landsberg, M.D. — Thu, 08Â FebÂ 1996 00:00:00 -05:00

Abnormalities of glucose, insulin, and lipoprotein metabolism are common in patients with hypertension. These changes can also be discerned in normotensive first-degree relatives of hypertensive patients. They are not present...

EDITORIAL: Obesity, Leptin, and the Brain

Thu, 01Â FebÂ 1996 00:00:00 -05:00

The notion that genetic abnormalities contribute to obesity gained important support with the identification of the ob gene and its protein product in 1994.1 The ob protein, termed âleptinâ from...

ORIGINAL ARTICLE: Serum Immunoreactive-Leptin Concentrations in Normal-Weight and Obese Humans

Thu, 01Â FebÂ 1996 00:00:00 -05:00

Background: Leptin, the product of the ob gene, is a hormone secreted by adipocytes. Animals with mutations in the ob gene are obese and lose weight when given leptin, but...

EDITORIAL: Immunosuppression of Graves' Hyperthyroidism â Still an Elusive Goal

Thu, 25Â JanÂ 1996 00:00:00 -05:00

Fifty years have passed since radioactive iodine and antithyroid drugs became available for the treatment of hyperthyroidism caused by Graves' disease. Despite the efficacy of both treatments, opinions still diverge...

REVIEW ARTICLE: Octreotide

Thu, 25Â JanÂ 1996 00:00:00 -05:00

A peptide inhibiting the release of growth hormone was originally detected accidentally during studies of the distribution of growth hormoneâreleasing factor in the hypothalamus of rats.1 This peptide, called somatostatin,...

ORIGINAL ARTICLE: Lack of Effect of Thyroxine in Patients with Graves' Hyperthyroidism Who Are Treated with an Antithyroid Drug

Thu, 25Â JanÂ 1996 00:00:00 -05:00

Background: Antithyroid drugs are effective in patients with hyperthyroidism due to Graves' disease, but the rate of recurrence after treatment is high. In a recent Japanese study, adjunctive treatment with...

NEJM â Collection Updates for Endocrinology

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Î±-Adrenergic Receptors and Type 2 Diabetes

REVIEW ARTICLE: WilliamsâBeuren Syndrome

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 1-2010 â A 75-Year-Old Man with Hypertension, Hyperglycemia, and Edema

IMAGES IN CLINICAL MEDICINE: Necrolytic Migratory Erythema

CLINICAL PROBLEM-SOLVING: A Crisis in Late Pregnancy

ORIGINAL ARTICLE: Normal Spermatogenesis in a Man with Mutant Luteinizing Hormone

CORRESPONDENCE: FOXP3 Forkhead Domain Mutation and Regulatory T Cells in the IPEX Syndrome

IMAGES IN CLINICAL MEDICINE: Cerebrospinal Fluid Leak

CORRESPONDENCE: Luteinizing HormoneâReleasing Hormone and Postmenopausal Flushing

BOOK REVIEW:

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 24-2009 â A 26-Year-Old Woman with Painful Swelling of the Neck

CORRESPONDENCE: Muscle Glycogenosis Due to Phosphoglucomutase 1 Deficiency

BOOK REVIEW:

CLINICAL PROBLEM-SOLVING: A Fragile Balance

ORIGINAL ARTICLE: Isolated Familial Hypogonadotropic Hypogonadism and a Mutation

BOOK REVIEW:

BOOK REVIEW:

IMAGES IN CLINICAL MEDICINE: Graves' Hyperthyroidism

REVIEW ARTICLE: Predisposing Factors for Adrenal Insufficiency

ORIGINAL ARTICLE: Inactivating Mutations in a Patient with Premature Pubarche

BOOK REVIEW:

CLINICAL IMPLICATIONS OF BASIC RESEARCH: The Origins of Brown Adipose Tissue

BOOK REVIEW:

BOOK REVIEW:

BOOK REVIEW:

BOOK REVIEW:

CORRESPONDENCE: Ending Propylthiouracil-Induced Liver Failure in Children

EDITORIAL: Brown Adipose Tissue â When It Pays to Be Inefficient

ORIGINAL ARTICLE: Functional Brown Adipose Tissue in Healthy Adults

ORIGINAL ARTICLE: Identification and Importance of Brown Adipose Tissue in Adult Humans

ORIGINAL ARTICLE: Cold-Activated Brown Adipose Tissue in Healthy Men

IMAGES IN CLINICAL MEDICINE: Neonatal Breast Enlargement

IMAGES IN CLINICAL MEDICINE: Mediastinal Extension of a Goiter

ORIGINAL ARTICLE: Mutations in Associated with Ovarian Insufficiency

CLINICAL PRACTICE: Graves' Ophthalmopathy

CLINICAL PROBLEM-SOLVING: When Past Is Prologue

REVIEW ARTICLE: Preservation of Fertility in Patients with Cancer

CORRESPONDENCE: A Family with Complete Resistance to Thyrotropin-Releasing Hormone

CLINICAL PROBLEM-SOLVING: Needle in a Haystack

CLINICAL PRACTICE: Primary Ovarian Insufficiency

CORRESPONDENCE: Excess Iodine from an Unexpected Source

IMAGES IN CLINICAL MEDICINE: Hypogonadism Due to Pituicytoma in an Identical Twin

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

EDITORIAL: Treating Low Sexual Desire â New Findings for Testosterone in Women

ORIGINAL ARTICLE: Testosterone for Low Libido in Postmenopausal Women Not Taking Estrogen

CORRESPONDENCE: Problems Associated with the Use of Thyrogen in Patients with a Thyroid Gland

CORRESPONDENCE: Diagnosis of Poorly Differentiated Thyroid Cancer with Radioiodine Scanning after Thyrotropin Alfa Stimulation

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

CORRESPONDENCE: Mutation with Primary Hyperparathyroidism and Undetectable Intact PTH

EDITORIAL: Renal PhosphateâTransporter Regulatory Proteins and Nephrolithiasis

IMAGES IN CLINICAL MEDICINE: Extracapsular Hemorrhage from a Parathyroid Adenoma

CLINICAL THERAPEUTICS: Gonadotropin-Releasing Hormone Agonists for Endometriosis

ORIGINAL ARTICLE: Mutations and Responsiveness of Renal Parathyroid Hormone

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

CORRESPONDENCE: Age, Renal Tubular Phosphate Reabsorption, and Serum Phosphate Levels in Adults

CORRESPONDENCE: Glucagon-like Peptide 1âReceptor Scans to Localize Occult Insulinomas

EDITORIAL: Tibolone and the Promise of Ideal Hormone-Replacement Therapy

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 25-2008 â A 43-Year-Old Man with Fatigue and Lesions in the Pituitary and Cerebellum

ORIGINAL ARTICLE: The Effects of Tibolone in Older Postmenopausal Women

EDITORIAL: FGF-23 and Outcomes Research â When Physiology Meets Epidemiology

ORIGINAL ARTICLE: Fibroblast Growth Factor 23 and Mortality among Patients Undergoing Hemodialysis

CLINICAL PRACTICE: Hypoparathyroidism

BOOK REVIEW:

IMAGES IN CLINICAL MEDICINE: Renal Osteodystrophy of the Palate

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

ORIGINAL ARTICLE: Motesanib Diphosphate in Progressive Differentiated Thyroid Cancer

CLINICAL PRACTICE: Graves' Disease

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Metabolism and Therapeutic Angiogenesis

CLINICAL PRACTICE: Precocious Puberty

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 16-2008 â A 46-Year-Old Woman with Bone Pain

CLINICAL PROBLEM-SOLVING: Back to Basics

EDITORIAL: Reduce, Recycle, Reuse â Iodotyrosine Deiodinase in Thyroid Iodide Metabolism

ORIGINAL ARTICLE: Mutations in the Iodotyrosine Deiodinase Gene and Hypothyroidism

IMAGES IN CLINICAL MEDICINE: Lingual Thyroid

ORIGINAL ARTICLE: Gene Identification for the cblD Defect of Vitamin B Metabolism

ORIGINAL ARTICLE: Simvastatin with or without Ezetimibe in Familial Hypercholesterolemia

ORIGINAL ARTICLE: Autoimmune Polyendocrine Syndrome Type 1 and NALP5, a Parathyroid Autoantigen

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

ORIGINAL ARTICLE: Intensive Insulin Therapy and Pentastarch Resuscitation in Severe Sepsis

NEJM â Collection Updates for Endocrinology

REVIEW ARTICLE: WilliamsâBeuren Syndrome

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 1-2010 â A 75-Year-Old Man with Hypertension, Hyperglycemia, and Edema

CORRESPONDENCE: Luteinizing HormoneâReleasing Hormone and Postmenopausal Flushing

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 24-2009 â A 26-Year-Old Woman with Painful Swelling of the Neck

EDITORIAL: Brown Adipose Tissue â When It Pays to Be Inefficient

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

EDITORIAL: Treating Low Sexual Desire â New Findings for Testosterone in Women

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

EDITORIAL: Renal PhosphateâTransporter Regulatory Proteins and Nephrolithiasis

CORRESPONDENCE: Glucagon-like Peptide 1âReceptor Scans to Localize Occult Insulinomas

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 25-2008 â A 43-Year-Old Man with Fatigue and Lesions in the Pituitary and Cerebellum

EDITORIAL: FGF-23 and Outcomes Research â When Physiology Meets Epidemiology

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 16-2008 â A 46-Year-Old Woman with Bone Pain

EDITORIAL: Reduce, Recycle, Reuse â Iodotyrosine Deiodinase in Thyroid Iodide Metabolism

ORIGINAL ARTICLE: Metabolic Effects of a Growth HormoneâReleasing Factor in Patients with HIV

EDITORIAL: Experiments of Nature â A Glimpse into the Mysteries of the Pubertal Clock

EDITORIAL: Adjuvant Mitotane Therapy of Adrenal Cancer â Use and Controversy

PERSPECTIVE: Osteonecrosis of the Jaw â Do Bisphosphonates Pose a Risk?

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 36-2006 â A 35-Year-Old Pregnant Woman with New Hypertension

EDITORIAL: Vasopressin Antagonists â Progress and Promise

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 23-2006 â A 36-Year-Old Man with Numbness in the Right Hand and Hypertension

EDITORIAL: RiskâBenefit Profiles of Raloxifene for Women

EDITORIAL: Iodine Nutrition â More Is Better

EDITORIAL: Serious Adverse Drug Effects â Seeing the Trees through the Forest

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 4-2006 â A 79-Year-Old Woman with Myalgias, Fatigue, and Shortness of Breath

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

EDITORIAL: Low-Density Lipoprotein Cholesterol in Patients with Stable Coronary Heart Disease â Is It Time to Shift Our Goals?

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 7-2005 â A 59-Year-Old Woman with an Incidentally Discovered Adrenal Nodule