Metabolic and endocrine studies in a case of lipoatrophic diabetes

摘要: A 20-yr-old female with congenital lipoatrophic diabetes was studied, the following findings: (1) Serum insulin levels increased after both oral glucose and intravenous arginine administration; there no growth hormone response to latter. (2) The infusion of (0.1 units 0.5 units/kg) during fed state a 110-hr fast produced only minimal changes various fuels measured, exception decrease in branched-chain amino acids. (3) There production ketones fast. (4) Metabolic expenditure markedly postabsorptive (65–75 kcal/hr/sq m); it fell into normal range (31–35 m). (5) Following meals, patient experienced complaints ranging from cold shivering feeling hot gross diaphoresis. These findings were associated intermittent lability her skin temperature, which varied 1°–2°F 3-hr period. (6) Progressive increases doses regular before each meal resulted up total 9000 units/day being required blood achieved. (7) 2-wk therapeutic trial pimozide provided significant variety hormones basal or perturbations. (8) pituitary target organ studied hypothyroid (Hashimoto's thyroiditis) euthyroid (following thyroid replacement). All responses except that did not rise slow wave sleep either state.