Trygstad O, Seip M, Oseid S
Int J Obes. 1977;1(3):287-92.
Congenital generalized lipodystrophy is considered to be a diencephalic syndrome with disturbance of hypothalamic transmitters. After puberty and arrest of growth the patients develop a serious untreatable diabetes mellitus. One of our patients, a girl 15 years of age, developed a lipodystrophic diabetes with fasting blood glucose levels above 300 mg/100 ml, increased serum insulin with insulin resistance, and hyperlipidaemia. Daily administration of fenfluramine gave a dramatic improvement. The voracious hunger and profuse perspiration were reduced, the patient's serum lipids became normal, her blood glucose fell, and her sensitivity to exogenous insulin increased. A normalization of the urinary excretion of the serotonin metabolite, 5-OH-indole acetic acid, was observed.
先天性全身性脂肪营养不良被认为是一种伴有下丘脑递质紊乱的间脑综合征。青春期后生长停滞，患者会发展为严重的、无法治疗的糖尿病。我们的一名患者，一名15岁女孩，患了脂肪营养不良性糖尿病，空腹血糖水平高于300mg/100ml，血清胰岛素升高且伴有胰岛素抵抗，还有高脂血症。每日服用芬氟拉明后有显著改善。贪食和多汗症状减轻，患者的血脂恢复正常，血糖下降，对外源性胰岛素的敏感性增加。观察到血清素代谢产物5-羟基吲哚乙酸的尿排泄恢复正常。
