Generalized lipodystrophy, congenital and acquired (lipoatrophy)
作者:
M Seip,
O Trygstad,
期刊:
Acta Pædiatrica
(WILEY Available online 1996)
卷期:
Volume 85,
issue 1
页码: 2-28
ISSN:0803-5253
年代: 1996
DOI:10.1111/j.1651-2227.1996.tb14262.x
出版商: Blackwell Publishing Ltd
关键词: Acanthosis nigricans. anabolic syndrome;generalized lipodystrophy;hypermetabolism;hypertrophic cardiomyopathy;lipoatrophic diabetes
数据来源: WILEY
摘要:
This review is based on longitudinal studies on our seven patients with congenital generalized lipodystrophy, our patient with acquired generalized lipodystrophy, and published papers on these subjects. An inability to store energy in adipose tissue is of pathogenetic importance. In congenital lipodystrophy, insulin resistance is present from birth, resulting in hyperinsulinaemia, dyslipidaemia, and insulin‐resistant diabetes with an anabolic syndrome worsened by a voracious appetite. Clinically, we observed increased height velocity in pre‐school age children, and organomegaly with hypertrophic cardiomyopathy, which seems to be lethal in early adulthood: three of our patients died at the ages of 24,32 and 37 years. The oldest alive, 39 years, suffers from stenocardia. Regarding treatment, it is most important to reduce energy consumption. The congenital form is recessively inherited. The aetiology may be related to insulin receptor or postreceptor mechanisms. Acquired generalized lipodystrophy seems to be an autoimmune disorder with secondary destruction of the adipose organ; the anabolic syndrome with insulin‐resistant diabetes is secondary. Our patient died when 24 years old from pneu
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