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Cronkhite–Canada syndrome

From Wikipedia, the free encyclopedia
Medical condition
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 raresyndrome characterized by multiplepolyps of thedigestive tract. It is sporadic (i.e. it does not seem to be ahereditary disease),[1] and it is currently considered acquired[2] andidiopathic (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

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Polyps are found throughout the GI tract (most frequently in thestomach andlarge intestine, followed by thesmall intestine) though typically avoid theesophagus.[6] Abiopsy will reveal them to behamartomas; the possibility that they progress to cancer is generally considered to be low,[7] although it has been reported multiple times in the past. Chronicdiarrhea andprotein-losing enteropathy are often observed. Possible collateral features include variable anomalies ofectodermal tissues, such asalopecia,atrophy of thenails, or skinpigmentation[3]

Causes

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Diagnosis

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

Management

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

References

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  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. ^abYuan, 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. ^abSamoha 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). Retrieved2019-05-19.
  9. ^abWard 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.

External links

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  • 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. Retrieved2024-01-30.
Classification
External resources
GI tract
Upper
Esophagus
Stomach
Lower
Small intestine
Appendix
Colon/rectum
Anus
Upper and/or lower
Accessory
Liver
Biliary tract
Pancreas
Peritoneum
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