Cronkhite–Canada syndrome
| Cronkhite–Canada syndrome | |
|---|---|
| Classification and external resources | |
| Specialty | gastroenterology |
| ICD-10 | K63.8, K63.5, K31.7 |
| ICD-9-CM | 211.3 |
| OMIM | 175500 |
| DiseasesDB | 1924 |
| eMedicine | derm/729 |
| MeSH | D044483 |
Cronkhite–Canada syndrome is a rare syndrome characterized 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
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
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
Treatments proposed include cromolyn sodium and prednisone.[6]
References
- ↑ 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.
- ↑ 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. - ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.