Cronkhite-Canada syndrome with steroid dependency: A case report
作者机构:Department of GastroenterologyThe First Affiliated Hospital of Guangxi Medical UniversityNanning 530021Guangxi Zhuang Autonomous RegionChina
出 版 物:《World Journal of Clinical Cases》 (世界临床病例杂志)
年 卷 期:2021年第9卷第14期
页 面:3466-3471页
核心收录:
学科分类:1002[医学-临床医学] 100201[医学-内科学(含:心血管病、血液病、呼吸系病、消化系病、内分泌与代谢病、肾病、风湿病、传染病)] 10[医学]
主 题:Cronkhite-Canada syndrome Endoscopy Prednisone Relapse Maintenance therapy Case report
摘 要:BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare nonhereditary disease characterized by chronic diarrhoea,diffuse gastrointestinal polyposis and ectodermal *** lethality of CCS can be up to 50%if it is untreated or if treatment is delayed or *** than 35%of the patients do not achieve long-term clinical remission after corticosteroid administration,with relapse occurring during or after the cessation of glucocorticoid *** optimal strategy of maintenance therapy of this disease is *** SUMMARY A 47-year-old man presented to the hospital with a 3-mo history of frequent watery diarrhoea,accompanied by macular skin pigmentation that included the palms and soles,and onychodystrophy of the fingernails and *** and colonoscopy revealed numerous polyps in the stomach and *** other possibilities were ruled out by a series of examinations,CCS was diagnosed and treated with *** patient took prednisone for more than 1 year before achieving complete resolution of his symptoms and endoscopic *** patient was then given prednisone 5 mg/d for 6 mo of maintenance *** clinical improvement and polyp regression,prednisone was *** mo after the discontinuation of prednisone,the diarrhoea and gastrointestinal polyps ***,the patient was given the same dose of prednisone,and complete remission was achieved *** It is necessary to extend the duration of prednisone maintenance therapy for *** is still effective when readministered after *** endoscopy at intervals of 1 year or less is recommended to assess mucosal disease activity.