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Molecular Architecture of the Goodpasture Autoantigen in Anti-GBM Nephritis

NEJM - Current Issue - Wed, 07/21/2010 - 14:05

This study compared the conformation of the antibody epitopes in Goodpasture's disease and Alport's post-transplantation nephritis in search of the pathogenesis of anti-basement membrane glomerulonephritis. The authors report evidence that the initiation of such disease depends on the conformation involving perturbation of the quaternary structure of basement-membrane collagen, eliciting an autoimmune response.

Efficacy of Gene Therapy for X-Linked Severe Combined Immunodeficiency

NEJM - Current Issue - Wed, 07/21/2010 - 14:05

In a long-term (up to 11 years) follow-up of nine patients with X-linked severe combined immunodeficiency treated with retroviral insertion of a normal common {gamma}-chain gene into hematopoietic progenitors, seven had durable T-cell reconstitution and long-term survival. B-cell immunity was not corrected. Acute leukemia developed in four patients. In patients without a matched marrow donor, gene therapy may be an option but is associated with serious risks.

Functional Hypothalamic Amenorrhea

NEJM - Current Issue - Wed, 07/21/2010 - 14:05

A 16-year-old girl presents for evaluation of secondary amenorrhea. Her menarche was at the age of 12 years. Her last menstrual period was 6 months ago. She has lost 2.3 kg (5 lb) over the past 3 months and reports a 2-week history of unilateral foot pain. She typically runs 10 km (6 mi) per day, at least five times per week. Her body-mass index is 19. How should her case be evaluated and managed?

Clinical Evaluation of the Knee

NEJM - Current Issue - Wed, 07/21/2010 - 14:05

Figure

Knee problems are one of the most common musculoskeletal symptoms evaluated by the primary care physician. This video shows how to perform a comprehensive examination of the knee.

Chloroquine-Induced Hair Hypopigmentation

NEJM - Current Issue - Wed, 07/21/2010 - 14:05

A 16-year-old, blonde-haired patient was evaluated for a new band of lighter-colored hair located approximately 3 cm from the scalp. The patient first noted the change in hair color 1 ...

Congenital Cholesteatoma of the Middle Ear

NEJM - Current Issue - Wed, 07/21/2010 - 14:05

A 9-year-old boy was referred to an otolaryngologist for subjective right-sided hearing loss. A hearing test revealed mild conductive hearing loss at low and middle frequencies. Otoendoscopy showed a mass ...

Case 22-2010 -- An 87-Year-Old Woman with Dementia and a Seizure

NEJM - Current Issue - Wed, 07/21/2010 - 14:05

An 87-year-old woman with a history of dementia was admitted to this hospital because of a seizure. A diagnosis of possible Alzheimer's disease had been made 4 years earlier; at baseline, she recognized only her husband and children. On examination, she was unresponsive. Magnetic resonance imaging scans of the brain revealed T2-weighted hyperintensities in the white matter involving both cerebral hemispheres, as well as innumerable small foci of susceptibility-weighted artifact at the cortical-subcortical junction. Despite a temporary improvement after treatment, she died of pneumonia a few days later. An autopsy was performed.

Continuous Glucose Monitoring -- Coming of Age

NEJM - Current Issue - Wed, 07/21/2010 - 14:05

Advances in glucose-monitoring technology have been an important driver for improvements in the treatment of type 1 diabetes. The introduction of fingerstick capillary blood glucose monitoring in the 1970s set ...

"Race Correction" in Pulmonary-Function Testing

NEJM - Current Issue - Wed, 07/21/2010 - 14:05

Tests of pulmonary function and radiographic imaging of the chest are the two key methods used in diagnostic evaluation of patients with pulmonary disease. Unlike blood pressure, acceptable normal values ...

Is Early Reconstruction Necessary for All Anterior Cruciate Ligament Tears?

NEJM - Current Issue - Wed, 07/21/2010 - 14:05

The anterior cruciate ligament (ACL) is necessary to prevent knee instability (giving way) during sports activities, in particular those that require planting and pivoting-type maneuvers. Episodes of giving way typically ...

Goodpasture's Disease -- New Secrets Revealed

NEJM - Current Issue - Wed, 07/21/2010 - 14:05

In this issue of the Journal, Pedchenko et al.1 increase our understanding of the pathogenesis of two forms of antibody-mediated glomerulonephritis. One, Goodpasture's disease, is an organ-specific autoimmune disease that ...

Lenient versus Strict Rate Control in Atrial Fibrillation

NEJM - Current Issue - Wed, 07/21/2010 - 14:05

To the Editor: In the report by Van Gelder et al. (April 15 issue),1 the median duration of atrial fibrillation ...

Epidural Analgesia for Labor and Delivery

NEJM - Current Issue - Wed, 07/21/2010 - 14:05

To the Editor: In her Clinical Therapeutics article, Hawkins (April 22 issue)1 outlines the risks of epidural analgesia for childbirth. ...

Case 10-2010: A Woman with Weakness and a Mass in the Brain

NEJM - Current Issue - Wed, 07/21/2010 - 14:05

To the Editor: Cahill et al. (April 8 issue)1 present the case of a woman with a pulmonary arteriovenous malformation ...

JAK2 Mutations and Coronary Ischemia

NEJM - Current Issue - Wed, 07/21/2010 - 14:05

To the Editor: The chronic myeloproliferative disorders are clonal marrow neoplasms that are often associated with arterial or venous thromboses ...

Patient-Specific Induced Pluripotent Stem-Cell Models for Long-QT Syndrome

NEJM - Current Issue - Wed, 07/21/2010 - 13:50

Background Long-QT syndromes are heritable diseases associated with prolongation of the QT interval on an electrocardiogram and a high risk of sudden cardiac death due to ventricular tachyarrhythmia. In long-QT ...

Revisiting the Rosiglitazone Story -- Lessons Learned

NEJM - Current Issue - Wed, 07/21/2010 - 13:50

(No abstract is available for this citation)

Walking the Tightrope of Health Insurance Reform between 2010 and 2014

NEJM - Current Issue - Wed, 07/21/2010 - 13:49

(No abstract is available for this citation)

Truth and Consequences -- Insurance-Premium Rate Regulation and the ACA

NEJM - Current Issue - Wed, 07/21/2010 - 13:49

(No abstract is available for this citation)

Health Reform, Primary Care, and Graduate Medical Education

NEJM - Current Issue - Wed, 07/21/2010 - 13:49

(No abstract is available for this citation)