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目的探讨国人 Cronkhite-Canada 综合征(CCS)临床与诊治特点,提高对该病的认识。方法回顾性分析并总结近20年国人 CCS 共32例临床资料。结果患者以胃肠道多发息肉伴外胚层三联征为主要表现,慢性腹泻、腹痛、体重下降、贫血、水肿等症状最为常见,2例患者合并癌变。综合治疗可使部分患者症状得到不同程度“缓解”。该病呈现一定的种族及地区聚集性,但常被忽视。胃肠核素显像、双气囊小肠镜和胶囊内镜等新技术的应用为了解该病提供了新的信息。结论 CCS 是较少见消化道疾病,国人与英、日文献报道病例的临床特点基本相近。新技术的应用、报道项目的规范化以及数据共享有助于对该罕见病的深入了解。
Objective To investigate the clinical and diagnosis and treatment characteristics of Chinese Cronkhite-Canada syndrome (CCS) and to raise awareness of the disease. Methods The clinical data of 32 Chinese patients with CCS in recent 20 years were retrospectively analyzed and summarized. Results Patients with gastrointestinal multiple polyps with ectoderm triad as the main performance, chronic diarrhea, abdominal pain, weight loss, anemia, edema and other symptoms most common, 2 patients with cancer. Comprehensive treatment can make some patients symptoms have different degrees “ease ”. The disease presents a certain racial and regional aggregation, but is often overlooked. The application of new technologies such as gastrointestinal radiography, double balloon enteroscopy and capsule endoscopy provides new information for understanding the disease. Conclusions CCS is a rare gastrointestinal disease. The clinical characteristics of the cases reported by Chinese and English and Japanese literature are basically similar. The application of new technologies, the normalization of reporting programs, and data sharing will help to gain insight into this rare disease.