David A. Weinstein

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The anemia of chronic disease is a prevalent, poorly understood condition that afflicts patients with a wide variety of diseases, including infections, malignancies, and rheumatologic disorders. It is characterized by a blunted erythropoietin response by erythroid precursors, decreased red blood cell survival, and a defect in iron absorption and macrophage(More)
Joseph I. Wolfsdorf1 and David A. Weinstein2 1Senior Associate in Medicine, Director, Diabetes Program, Division of Endocrinology; Chief, Charles A. Janeway Medical Firm, Children’s Hospital Boston; Associate Professor of Pediatrics, Harvard Medical School, Boston 02115, MA, USA; 2Assistant in Medicine (Endocrinology), Children’s Hospital Boston; Instructor(More)
PURPOSE Glycogen storage disease type III is a rare disease of variable clinical severity affecting primarily the liver, heart, and skeletal muscle. It is caused by deficient activity of glycogen debranching enzyme, which is a key enzyme in glycogen degradation. Glycogen storage disease type III manifests a wide clinical spectrum. Individuals with glycogen(More)
The glycogen storage diseases comprise several inherited diseases caused by abnormalities of enzymes that regulate the synthesis or degradation of glycogen. In contrast to the classic hepatic glycogen storage diseases that are characterized by fasting hypoglycemia and hepatomegaly, the liver is not enlarged in GSD0. Patients with GSD0 typically have fasting(More)
BACKGROUND Type I glycogen storage disease (GSD) is caused by a deficiency of glucose-6-phosphatase resulting in severe fasting hypoglycemia. OBJECTIVE We compared the efficacy of a new modified starch with the currently used cornstarch therapy in patients with type Ia and Ib GSD. DESIGN This was a randomized, 2-d, double-blinded, crossover pilot study(More)
Dual-photon absorptiometry characterized bone loss in males aged less than 40 years after complete traumatic paraplegic and quadriplegic spinal cord injury. Total bone mass of various regions and bone mineral density (BMD) of the knee were measured in 55 subjects. Three different populations were partitioned into four groups: 10 controls (healthy, age(More)
We present a series of 8 patients (6 males, 2 females) with hepatocellular carcinoma (HCC) and glycogen storage disease type Ia (GSD Ia). In this group, the age at which treatment was initiated ranged from birth to 39 years (mean 9.9 years). All patients but one were noncompliant with treatment. Hepatic masses were first detected at an age range of 13–45(More)
To assess the role of magnetic resonance imaging (MRI) in diagnosing osteomyelitis in diabetic foot infections, 47 diabetic patients with clinical suspicion of osteomyelitis, nonhealing foot ulcer, or soft tissue infection of the foot were examined prospectively by MRI and plain radiographs. Pathological confirmation of diagnosis was obtained in 62 bones(More)
To evaluate the effects of uncooked cornstarch (UCS) on metabolic control, growth, and complications of pubertal and postpubertal subjects with type 1a glycogen storage disease, we studied 26 subjects (16 males), mean age 20.8±5.1 years, in whom continuous glucose therapy with cornstarch began at 6.8±4.3 years. At the time of this analysis, subjects had(More)
PURPOSE Glycogen storage disease type I (GSD I) is a rare disease of variable clinical severity that primarily affects the liver and kidney. It is caused by deficient activity of the glucose 6-phosphatase enzyme (GSD Ia) or a deficiency in the microsomal transport proteins for glucose 6-phosphate (GSD Ib), resulting in excessive accumulation of glycogen and(More)