Huseman C, Johanson A, Varma M, Blizzard R M
J Pediatr. 1978 Aug;93(2):221-6. doi: 10.1016/s0022-3476(78)80500-9.
Three siblings with congenital lipodystrophy were studied extensively for endocrine abnormalities. A severe disturbance in carbohydrate metabolism was observed. Plasma concentrations of glucagon and insulin were markedly elevated both in the basal state and in response to provocative stimuli. In addition, marked resistance to exogenous insulin and a diabetic oral glucose tolerance test were demonstrated. Lipid metabolism, GH, and ACTH secretion were normal...
对三名患有先天性脂肪代谢障碍的兄弟姐妹进行了广泛的内分泌异常研究。观察到碳水化合物代谢严重紊乱。基础状态下以及对刺激的反应中，胰高血糖素和胰岛素的血浆浓度均显著升高。此外，还证实了对外源性胰岛素的明显抵抗以及糖尿病口服葡萄糖耐量试验结果。脂质代谢、生长激素（GH）和促肾上腺皮质激素（ACTH）分泌正常……
