KEGG   DISEASE: Acquired generalized lipodystrophy
Entry
H01474                      Disease                                
Name
Acquired generalized lipodystrophy;
Lawrence syndrome
  Supergrp
Lipodystrophy [DS:H01475]
Description
Acquired generalized lipodystrophy (AGL), also called the Lawrence syndrome, is one subtype of acquired lipodystrophy caused by autoimmune disease, panniculitis or idiopathic. The disorder appears during the childhood and adolescence. Clinically, loss of subcutaneous fat occurs highly variable, exclusively at the face and extremities including legs, palms and some soles. In rare cases, intraabdomianl fat and bone marrow fat may also be spared. Similarly to congenitial generalized lipodystrophy, people with the disorder usually appear severe hepatic steatosis and fibrosis, diabetes, and hypertriglyceridemia. Metreleptin, as a recombinant analog of human leptin, has been approved for the treatment for the disorder as well as diet.
Category
Inherited metabolic disorder
Brite
Human diseases in ICD-11 classification [BR:br08403]
 05 Endocrine, nutritional or metabolic diseases
  Endocrine diseases
   Other disorders of glucose regulation or pancreatic internal secretion
    5A44  Insulin-resistance syndromes
     H01474  Acquired generalized lipodystrophy
Other DBs
ICD-11: 5A44
MeSH: D008060
Reference
  Authors
Garg A
  Title
Clinical review#: Lipodystrophies: genetic and acquired body fat disorders.
  Journal
J Clin Endocrinol Metab 96:3313-25 (2011)
DOI:10.1210/jc.2011-1159
Reference
  Authors
Tchang BG, Shukla AP, Aronne LJ
  Title
Metreleptin and generalized lipodystrophy and evolving therapeutic perspectives.
  Journal
Expert Opin Biol Ther 15:1061-75 (2015)
DOI:10.1517/14712598.2015.1052789
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