KEGG   DISEASE: クロンカイト・カナダ症候群
エントリ  
H01874                                                             
名称    
クロンカイト・カナダ症候群
概要    
Cronkhite-Canada syndrome (CCS) is a rare noninherited condition characterized by gastrointestinal hamartomatous polyposis, alopecia, onychodystrophy, hyperpigmentation, and diarrhea. CCS is reported worldwide, with 75% of cases in Japan. The average age of onset is 60 years and the male to female ratio is 3:2. At present, the pathogenesis of CCS is unknown, but the factors that include immune abnormalities, infection, and allergies, may be relevant. There are no evidence-based therapies. Numerous treatments have been attempted in CCS patients, with varying degrees of success. These treatments include glucocorticoids, nutritional support, antibiotics, and polypectomy.
カテゴリ  
消化器系疾患
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 20 発達異常
  多発性の発達異常または症候群
   LD27  主な特徴として皮膚または粘膜の異常を伴う症候群
    H01874  クロンカイト・カナダ症候群
指定難病 [jp08407.html]
 H01874
リンク   
ICD-11: LD27.01
MeSH: D044483
OMIM: 175500
文献    
  著者
Sweetser S, Boardman LA
  タイトル
Cronkhite-Canada syndrome: an acquired condition of gastrointestinal polyposis and dermatologic abnormalities.
  雑誌
Gastroenterol Hepatol (N Y) 8:201-3 (2012)
文献    
  著者
Wen XH, Wang L, Wang YX, Qian JM
  タイトル
Cronkhite-Canada syndrome: report of six cases and review of literature.
  雑誌
World J Gastroenterol 20:7518-22 (2014)
DOI:10.3748/wjg.v20.i23.7518
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