1Case Report Of Cronhkite-Canada Sydrome And Review Of The Literatures

Cronkhite-Canada syndrome (CCS) is a rare nonhereditary polyposis syndrome, which is characterized by diffuse multiple polyps of the gastrointestinal tract and ectodermal symptoms including alopecia,dystrophic changes of fingernails, and cutaneous hyperpigmentation.Only around500cases have been reported worldwide, since the syndrome was first documented.The exact etiology of CCS is currently unknown,while an autoimmune inflammatory process is most commonly suspected.The most common symptoms consist of diarrhea, weight loss,abdominal pain, anorexia, hypoproteinemia,etc.The disease is very rare in the world,and early manifestations are nonspecific,which often leave the clinician perplexed.Diagnosis of CCS is based on history, physical examination,results of laboratory tests,endoscopy with finding of gastrointestinal polyposis, and histology.Many treatment strategies have been reported,including symptomatic and nutritional support therapy,Corticosteroids antibiotics,antihistamine receptor antagonist agents and cromolyn sodium,NSAIDs,acid inhibitor,cyclosporine,endoscopic mucosal resection,surgery. However,the optimum treatment of CCS is currently unknown.A58-year-old man who had suffered from chronic diarrhea and weakness for1month was admitted to Gastroenterology Department of our hospital in July2013.Ectodermal changes were observed2months after gastrointestinal symptoms had begun.Here we present the clinical manifestations of this rare case,as well as our treatment experience of the patient.In addition,we retrived relevant data of Chinese CCS patients from Pubmed,CNKI(National Knowledge Infrastructure),and WanFang Data to conduct a retrospective analysis based on epidemiological data,clinical manifestations,laboratory examinations and endoscopy findings, treatment,and prognosis.By investigating the clinical characteristics of Chinese patients with CCS, we expect to promote the undersanding of this rare sydrome,so as to provide domestic clinicians with a reference in future clinical diagnosis and medical management.