Cronkhite–Canada syndrome

Cronkhite–Canada syndrome
Other names: Gastrointestinal polyposis-skin pigmentation-alopecia-fingernail changes syndrome
Cronkhite–Canada syndrome affects the digestive tract

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] by internal medicine physician Leonard Wolsey Cronkhite Jr. and radiologist Wilma Jeanne Canada.[5]

Signs and symptoms

a)Hyperpigmentation, onychodystrophy b) alopecia

Polyps are found throughout the GI tract (most frequently in the stomach and large intestine, followed by the small intestine) though typically avoid the esophagus.[5]

A biopsy will reveal them to be hamartomas; the possibility that they progress to cancer is generally considered to be low,[6] 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

Causes

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.

Diagnosis

There is no specific test to diagnose Cronkhite–Canada syndrome. Diagnosis is based on symptoms and features of the disease.[7]

Management

Nutritional support is fundamental, and may include dietary guidance, supplements, tube feeding, or intravenous solutions.[8] Treatments proposed include cromolyn sodium and prednisone,[9] as well as histamine (H2) receptor antagonists or proton pump inhibitors.[8]

References

  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. 1 2 Samoha S, Arber N (2005). "Cronkhite-Canada Syndrome". Digestion. 71 (4): 199–200. doi:10.1159/000086134. PMID 15942206. Archived from the original on 2021-09-01. Retrieved 2020-12-07.
  6. ↑ 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.
  7. ↑ "Cronkhite-Canada Syndrome". NORD (National Organization for Rare Disorders). Archived from the original on 2018-02-12. Retrieved 2019-05-19.
  8. 1 2 Ward EM, Wolfsen HC (2003). "Pharmacological management of Cronkhite-Canada syndrome". Expert Opin. Pharmacother. 4 (3): 385–389. doi:10.1517/14656566.4.3.385. PMID 12614190. S2CID 36982546.
  9. ↑ 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.
Classification
External resources
This article is issued from Offline. The text is licensed under Creative Commons - Attribution - Sharealike. Additional terms may apply for the media files.