KEGG   DISEASE: 後天性全身性脂肪萎縮症
エントリ  
H01474                                                             
名称    
後天性全身性脂肪萎縮症;
Lawrence 症候群
  上位グループ
脂肪萎縮症 [DS:H01475]
概要    
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.
カテゴリ  
先天性代謝異常症
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 05 内分泌, 栄養, 代謝の疾患
  内分泌疾患
   その他のグルコース調節または膵内分泌の疾患
    5A44  インスリン抵抗性症候群
     H01474  後天性全身性脂肪萎縮症
リンク   
ICD-11: 5A44
MeSH: D008060
文献    
  著者
Garg A
  タイトル
Clinical review#: Lipodystrophies: genetic and acquired body fat disorders.
  雑誌
J Clin Endocrinol Metab 96:3313-25 (2011)
DOI:10.1210/jc.2011-1159
文献    
  著者
Tchang BG, Shukla AP, Aronne LJ
  タイトル
Metreleptin and generalized lipodystrophy and evolving therapeutic perspectives.
  雑誌
Expert Opin Biol Ther 15:1061-75 (2015)
DOI:10.1517/14712598.2015.1052789
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