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目的分析中国人 Cronkhite-Canada 综合征(CCS)临床特点。方法在 PubMed 和中国医学文献数据库中检索1985至2006年中国人 CCS 临床资料并进行回顾性分析。结果发现35例中国人病例,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。结论中国人病例与英、日文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人了解。
Objective To analyze the clinical features of Chinese Cronkhite-Canada syndrome (CCS). Methods The clinical data of Chinese CCS from 1985 to 2006 were retrieved from PubMed and Chinese medical literature database and analyzed retrospectively. The results showed that 35 cases of Chinese patients, patients with gastrointestinal polyp polyp ectodermal 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 nuclide imaging, double balloon enteroscopy and capsule endoscopy provides new information for understanding the disease. Conclusion The clinical characteristics of Chinese cases and English and Japanese cases are basically similar. The application of new technologies, the normalization of reporting programs, and data sharing will help to gain a deep understanding of this rare disease.