Cases Report the Cronkhite-Canada Syndrome: Improving the Prognosis

Yi Qun Yu, Peter James Whorwell, Lin Heng Wang, Jun Xiang Li, Qing Chang, Jie Meng

    Research output: Contribution to journalArticlepeer-review

    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.

    Original languageEnglish
    Pages (from-to)e2356
    JournalMedicine (Print)
    Volume94
    Issue number52
    DOIs
    Publication statusPublished - Dec 2015

    Keywords

    • Drugs, Chinese Herbal
    • Esomeprazole
    • Female
    • Gastrointestinal Tract
    • Humans
    • Intestinal Polyposis
    • Middle Aged
    • Prognosis
    • Proton Pump Inhibitors
    • Treatment Outcome

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