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Cultured lymphocytes transformed by Epstein--Barr virus were employed to study insulin receptors from a patient with extreme insulin resistance associated with the syndrome of leprechaunism. With cultured lymphocytes from normal subjects, insulin binding to its receptor is exquisitely sensitive to changes in temperature and pH. In cells from normal(More)
Leprechaunism is a rare, heritable syndrome, associated with multiple dysmorphic and pathologic features, suggestive of an endocrine dysfunction. Few endocrine and metabolic studies have been obtained because of the rarity of the syndrome, and the small size and early demise of these infants. The authors present here the clinical, anatomic, and(More)
Postprandial and postabsorptive glucose metabolism was studied in a 3-yr-old girl with leprechaunism by substrate and hormonal measurements and by quantifying hepatic glucose output during continuous infusion of D-[6-6-2H2]-glucose. Hepatic glucogen content and the activity of glycogen synthase and phosphorylase were also measured in the post-prandial state(More)
Testicular Leydig cell hyperplasia was observed in two brothers presenting with progressive sexual precocity at 2 yr of age. Virilization was shown to result from increased secretion rather than decreased clearance of gonadal testosterone. Testosterone hypersecretion appeared to be gonadotropin independent, as basal and gonadotropin-releasing(More)
Glucocorticoids cause growth retardation in children. We have studied the effect of these hormones on serum somatomedin (Sm) levels in seven children with nephrosis. Intravenous administration of methylprednisolone sodium succinate, 2.2 mg/kg, causes a rapid fall in serum Sm activity. The activity remains suppressed during continuous therapy, but returns(More)
Cerebral gigantism is a syndrome consisting of characteristic dysmorphic features, accelerated growth in early childhood, and variable degrees of mental retardation. Its etiology and pathogenesis have not been defined. Three families are presented with multiple affected members. The vertical transmission of the trait and equal expression in both sexes in(More)
The growth hormone responses to arginine infusion and to insulin-induced hypoglycemia were studied in 13 patients with neoplastic disease after treatment with radiation and chemotherapy. Patients who received intensive cranial radiation (greater than 2,400 rads) had no response to either arginine or insulin; those who received moderate cranial radiation(More)