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Cases Report the Cronkhite-Canada Syndrome: Improving the Prognosis.
Medicine (Baltimore). 2015 Dec; 94(52):e2356.M

Abstract

Cronkhite-Canada syndrome (CCS) is a rare nongenetic polyposis syndrome first reported by Cronkhite and Canada in 1955. Up to the present time, the literature consists of ∼400 cases of CCS with the majority being reported from Japan although 49 cases have been described in China.CCS is characterized by diffuse polyposis of the digestive tract in association with ectodermal changes, such as onychomadesis, alopecia, and cutaneous hyperpigmentation. The principal symptoms of CCS are diarrhea, weight loss, abdominal pain, and other gastrointestinal complications, such as protein-losing enteropathy and malnutrition.It has been traditional to consider that CCS is associated with a poor prognosis. This paper describes a relatively mild case and reviews the literature, which more recently, suggests that it may be a more benign condition that might actually be reversible with treatment.There is some evidence that infection or disturbed immunity may be involved in the pathophysiology and that targeting such abnormalities could have therapeutic potential.A strong case could be made for establishing an international case registry for this disease so that the pathophysiology, treatment, and prognosis could become much better understood.

Authors+Show Affiliations

From the Department of Gastroenterology, Dongfang Hospital, Beijing University of Chinese Medicine, Beijing, China (YQY, LHW, JXL, QC, JM); and Neurogastroenterology Unit, University Hospital of South Manchester, Manchester, UK (PJW).No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

26717374

Citation

Yu, Yi Qun, et al. "Cases Report the Cronkhite-Canada Syndrome: Improving the Prognosis." Medicine, vol. 94, no. 52, 2015, pp. e2356.
Yu YQ, Whorwell PJ, Wang LH, et al. Cases Report the Cronkhite-Canada Syndrome: Improving the Prognosis. Medicine (Baltimore). 2015;94(52):e2356.
Yu, Y. Q., Whorwell, P. J., Wang, L. H., Li, J. X., Chang, Q., & Meng, J. (2015). Cases Report the Cronkhite-Canada Syndrome: Improving the Prognosis. Medicine, 94(52), e2356. https://doi.org/10.1097/MD.0000000000002356
Yu YQ, et al. Cases Report the Cronkhite-Canada Syndrome: Improving the Prognosis. Medicine (Baltimore). 2015;94(52):e2356. PubMed PMID: 26717374.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cases Report the Cronkhite-Canada Syndrome: Improving the Prognosis. AU - Yu,Yi Qun, AU - Whorwell,Peter James, AU - Wang,Lin Heng, AU - Li,Jun Xiang, AU - Chang,Qing, AU - Meng,Jie, PY - 2015/12/31/entrez PY - 2015/12/31/pubmed PY - 2016/5/7/medline SP - e2356 EP - e2356 JF - Medicine JO - Medicine (Baltimore) VL - 94 IS - 52 N2 - Cronkhite-Canada syndrome (CCS) is a rare nongenetic polyposis syndrome first reported by Cronkhite and Canada in 1955. Up to the present time, the literature consists of ∼400 cases of CCS with the majority being reported from Japan although 49 cases have been described in China.CCS is characterized by diffuse polyposis of the digestive tract in association with ectodermal changes, such as onychomadesis, alopecia, and cutaneous hyperpigmentation. The principal symptoms of CCS are diarrhea, weight loss, abdominal pain, and other gastrointestinal complications, such as protein-losing enteropathy and malnutrition.It has been traditional to consider that CCS is associated with a poor prognosis. This paper describes a relatively mild case and reviews the literature, which more recently, suggests that it may be a more benign condition that might actually be reversible with treatment.There is some evidence that infection or disturbed immunity may be involved in the pathophysiology and that targeting such abnormalities could have therapeutic potential.A strong case could be made for establishing an international case registry for this disease so that the pathophysiology, treatment, and prognosis could become much better understood. SN - 1536-5964 UR - https://www.unboundmedicine.com/medline/citation/26717374/Cases_Report_the_Cronkhite_Canada_Syndrome:_Improving_the_Prognosis_ L2 - http://dx.doi.org/10.1097/MD.0000000000002356 DB - PRIME DP - Unbound Medicine ER -