total lipodystrophy

total lipodystrophy
a rare, autosomal recessive disorder seen mainly in infant girls, characterized by generalized loss of subcutaneous and extracutaneous adipose tissue with hepatomegaly, hypoglycemia and insulin-resistant nonketotic diabetes, hyperlipemia, elevation of the basal metabolic rate, accelerated somatic growth, advanced bone age, acanthosis nigricans, and hirsutism. Called also congenital generalized or generalized l., Berardinelli-Seip syndrome, and lipoatrophic diabetes.

Medical dictionary. 2011.

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  • Lipodystrophy — A disorder of adipose (fatty) tissue characterized by a selective loss of body fat. Patients with lipodystrophy have a tendency to develop insulin resistance, diabetes, a high triglyceride level (hypertriglyceridemia), and fatty liver. There are… …   Medical dictionary

  • Lipodystrophy syndrome — A disturbance of lipid (fat) metabolism that involves the partial or total absence of fat and often the abnormal deposition and distribution of fat in the body. There are a number of different lipodystrophy syndromes. Some of them are present at… …   Medical dictionary

  • generalized lipodystrophy — total l …   Medical dictionary

  • progressive congenital lipodystrophy — total l …   Medical dictionary

  • Congenital generalized lipodystrophy — Classification and external resources eMedicine article/1113171 Congenital generalized lipodystrophy (also known as Berardinelli–Seip syndrome) is a rare autosomal dominant skin condition, characterized by an extreme paucity of fat in the… …   Wikipedia

  • Syndrome, lipodystrophy — A disturbance of lipid (fat) metabolism that involves the partial or total absence of fat and often the abnormal deposition and distribution of fat in the body. There are a number of different lipodystrophy syndromes. Some of them are congenital… …   Medical dictionary

  • congenital generalized lipodystrophy — congenital progressive lipodystrophy total l …   Medical dictionary

  • lipoatrophic diabetes — total lipodystrophy …   Medical dictionary

  • Berardinelli-Seip syndrome — total lipodystrophy …   Medical dictionary

  • Lawrence-Seip syndrome — total lipodystrophy …   Medical dictionary

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