Cronkhite–Canada syndrome

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Cronkhite–Canada syndrome
Classification and external resources
ICD-10 K63.8, K63.5, K31.7
ICD-9 211.3
OMIM 175500
DiseasesDB 1924
eMedicine derm/729
MeSH D044483

Cronkhite–Canada syndrome is a rare syndrome characterised by multiple polyps of the digestive tract. It is sporadic (i.e. it does not seem to be a hereditary disease),[1] and it is currently considered acquired[2] and idiopathic (i.e. cause remains unknown).

About two-thirds of patients are of Japanese descent and the male to female ratio is 2:1.

It was characterized in 1955.[3][4]

Presentation[edit]

Polyps are most frequent in the stomach and large intestine, are also found in the small intestine, and are least frequent in the esophagus. A biopsy will reveal them to be hamartomas; the possibility that they progress to cancer is generally considered to be low,[5] although it has been reported multiple times in the past. Chronic diarrhea and protein-losing enteropathy are often observed. Possible collateral features include variable anomalies of ectodermal tissues, such as alopecia, atrophy of the nails, or skin pigmentation

Cause[edit]

The cause of the disease is unknown. It was originally thought that the epidermal changes were secondary to profound malnutrition as a result of protein-losing enteropathy. Recent findings have called this hypothesis into question; specifically, the hair and nail changes may not improve with improved nutrition.

Other conditions consisting of multiple hamartomatous polyps of the digestive tract include Peutz-Jeghers syndrome, juvenile polyposis, and Cowden disease. Related polyposis conditions are familial adenomatous polyposis, attenuated familial adenomatous polyposis, Birt–Hogg–Dubé syndrome and MUTYH.

Treatment[edit]

Treatments proposed include cromolyn sodium and prednisone.[6]

References[edit]

  1. ^ Vernia P, Marcheggiano A, Marinaro V, Morabito S, Guzzo I, Pierucci A (October 2005). "Is Cronkhite-Canada Syndrome necessarily a late-onset disease?". Eur J Gastroenterol Hepatol 17 (10): 1139–41. doi:10.1097/00042737-200510000-00022. PMID 16148564. 
  2. ^ Calva D, Howe JR (August 2008). "Hamartomatous polyposis syndromes". The Surgical clinics of North America 88 (4): 779–817, vii. doi:10.1016/j.suc.2008.05.002. PMC 2659506. PMID 18672141. 
  3. ^ Cronkhite LW, Canada WJ (June 1955). "Generalized gastrointestinal polyposis; an unusual syndrome of polyposis, pigmentation, alopecia and onychotrophia". N. Engl. J. Med. 252 (24): 1011–5. doi:10.1056/NEJM195506162522401. PMID 14383952. 
  4. ^ Junnarkar SP, Sloan JM, Johnston BT, Laird JD, Irwin ST (May 2001). "Cronkhite-Canada syndrome". The Ulster medical journal 70 (1): 56–8. PMC 2449205. PMID 11428328. 
  5. ^ Nagata J, Kijima H, Hasumi K, Suzuki T, Shirai T, Mine T (June 2003). "Adenocarcinoma and multiple adenomas of the large intestine, associated with Cronkhite-Canada syndrome". Dig Liver Dis 35 (6): 434–8. doi:10.1016/s1590-8658(03)00160-9. PMID 12868681. 
  6. ^ Ward E, Wolfsen HC, Ng C (February 2002). "Medical management of Cronkhite-Canada syndrome". South. Med. J. 95 (2): 272–4. doi:10.1097/00007611-200202000-00025. PMID 11846261. 

External links[edit]