Cronkhite–Canada syndrome

Cronkhite–Canada syndrome
Other namesGastrointestinal polyposis-skin pigmentation-alopecia-fingernail changes syndrome
Cronkhite–Canada syndrome affects the digestive tract
SpecialtyGastroenterology Edit this on Wikidata

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 3:2.[3] It was characterized in 1955[4][5] by internal medicine physician Leonard Wolsey Cronkhite Jr. and radiologist Wilma Jeanne Canada.[6]

Signs and symptoms

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.[6] A biopsy will reveal them to be hamartomas; the possibility that they progress to cancer is generally considered to be low,[7] 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[3]

Causes

Diagnosis

There is no specific test to diagnose Cronkhite–Canada syndrome. Diagnosis is based on symptoms and features of the disease.[8]

Management

Nutritional support is fundamental, and may include dietary guidance, supplements, tube feeding, or intravenous solutions.[9] Treatments proposed include cromolyn sodium and prednisone,[10] as well as histamine (H2) receptor antagonists or proton pump inhibitors.[9]

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. ^ a b Yuan, Wei; Tian, Li; Ai, Fei-Yan; Liu, Shao-Jun; Shen, Shou-Rong; Wang, Xiao-Yan; Liu, Fen (June 2018). "Cronkhite-Canada syndrome: A case report". Oncology Letters. 15 (6): 8447–8453. doi:10.3892/ol.2018.8409. ISSN 1792-1074. PMC 5950510. PMID 29805581.
  4. ^ 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.
  5. ^ 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.
  6. ^ a b Samoha S, Arber N (2005). "Cronkhite-Canada Syndrome". Digestion. 71 (4): 199–200. doi:10.1159/000086134. PMID 15942206.
  7. ^ 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.
  8. ^ "Cronkhite-Canada Syndrome". NORD (National Organization for Rare Disorders). Retrieved 2019-05-19.
  9. ^ a b 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.
  10. ^ 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.
  • Rabinowitz, Simon S.; Ebigbo, Nonyelum Erica; et al. (2017-10-20). Cuffari, Carmen; Windle, Mary L.; Piccoli, David A. (eds.). "Pediatric Cronkite-Canada syndrome". Medscape. Retrieved 2024-01-30.

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