KEGG   DISEASE: Cronkhite-Canada syndrome
Entry
H01874                      Disease                                
Name
Cronkhite-Canada syndrome
Description
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.
Category
Digestive system disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
 20 Developmental anomalies
  Multiple developmental anomalies or syndromes
   LD27  Syndromes with skin or mucosal anomalies as a major feature
    H01874  Cronkhite-Canada syndrome
Other DBs
ICD-11: LD27.01
MeSH: D044483
OMIM: 175500
Reference
  Authors
Sweetser S, Boardman LA
  Title
Cronkhite-Canada syndrome: an acquired condition of gastrointestinal polyposis and dermatologic abnormalities.
  Journal
Gastroenterol Hepatol (N Y) 8:201-3 (2012)
Reference
  Authors
Wen XH, Wang L, Wang YX, Qian JM
  Title
Cronkhite-Canada syndrome: report of six cases and review of literature.
  Journal
World J Gastroenterol 20:7518-22 (2014)
DOI:10.3748/wjg.v20.i23.7518
LinkDB

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