Cronkhite–Canada disease

From Wikipedia, the free encyclopedia

Cronkhite–Canada disease 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), and it is currently considered idiopathic (i.e. cause remains unknown).

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

Cronkhite-Canada syndrome is a rare disease affecting mainly patients between 50-60 years of age, characterised by skin changes and gastrointestinal distrubances. Patients present with epidermal changes such as hair loss, nail loss, and hyperpigmentation. There can also be varying degrees of malabsorption, malnutrition, hematochezia, and in some cases, colorectal cancer. About two-thirds of patients are of Japanese descent and the male to female ratio is 2:1.

1 See also
2 Cause
3 External links
4 References

[edit] See also

Other conditions consisting of multiple hamartomatous polyps of the digestive tract include Peutz-Jeghers syndrome, juvenile polyposis, and Cowden disease.

[edit] Cause

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.

[edit] External links

Cronkite-Canada syndrome.

[edit] References

PMID 16148564 "Is Cronkhite–Canada Syndrome necessarily a late-onset disease?", Eur J Gastroenterol Hepatol. 2005 Oct;17(10):1139–41
PMID 12868681 "Adenocarcinoma and multiple adenomas of the large intestine, associated with Cronkhite–Canada syndrome", Dig Liver Dis. 2003 Jun;35(6):434–8
PMID 11846261 "Medical management of Cronkhite–Canada syndrome", South Med J. 2002 Feb;95(2):272–4
PMID 11310396 "Cronkhite Canada syndrome", J Assoc Physicians India. 2000 Nov;48(11):1116–8
PMID 8076560 "The Cronkhite–Canada syndrome: a seldom recognized entity", Endoscopy. 1994 Mar;26(3):331–2
PMID 2552848 "Hepathology of Cronkhite–Canada polyps. A comparison to juvenile polyposis", Am J Surg Pathol. 1989 Nov;13(11):940–6
PMID 3745857 "Colon cancer in the Cronkhite–Canada syndrome", J Clin Gastroenterol. 1986
PMID 5022956 "Diffuse gastrointestinal polyposis with ectodermal changes: Cronkhite–Canada syndrome", Radiology. 1972 Jun;103(3):589–94
PMID 4099144 "Alopecia areata in association with intestinal polyposis. The relationship of two syndromes—Gardner's and Cronkhite–Canada", Acta Derm Venereol. 1970;50(5):381–4
PMID 4964743 "Case of cronkhite–Canada syndrome with associated jejunal diverticulosis", Br Med J. 1967 Dec 9;4(5579):601–2.