目的 提高临床医师对Caroli病的认识.方法 回顾性总结解放军总医院2005年1月至2010年10月24例Caroli病患者的一般资料、临床特点以及治疗方法.结果 本组患者男女比例1:2.4；平均发病年龄20.1岁；临床表现以上腹痛、发热、黄疸、消化道出血为主；胆管结石是最常见的合并症；影像学检查发现21例(87.5％)及胎儿1例；20例行外科手术治疗,其中肝移植2例,随访4个月至5年一般情况良好；2例行内镜下食管胃底静脉曲张硬化剂、组织胶治疗,分别随访3个月、1年无再发出血.结论 Caroli病患者起病早,临床表现多样,诊断主要依靠临床表现、影像学检查,外科手术及内镜下治疗是有效的治疗方式.%Objective To enhance the clinical understanding of Caroli's disease. Methods The clinical characteristics and therapeutic methods of 24 patients with Caroli's disease were reviewed retrospectively who had been treated from January 2005 to October 2010 in our hospital. Results In this series,the male to female ratio was 1: 2. 4, and the average age of onset was 20. 1 years old. Common clinical manifestations were upper abdominal pain,fever,jaundice,and GI bleeding. Bile duct stones were the most common complications. Twenty-one cases,including one fetus,were diagnosed by image findings (87. 5% ). Surgical operations were carried out in 20 cases, with liver transplantation in two of them. Follow-ups of 4 months to 5 years showed good general conditions in them. In the two patients with esophageal and gastric varices undergoing endoscopic injection of sclerotherapy ( EIS) and cyanoacrylate injection, follow-ups at 3 months and one year found no rebleeding. Conclusion The onset of Caroli's disease usually occurs early, with various clinical manifestations. Its diagnosis predominantly depends on clinical manifestations and image findings. Surgery or/and therapeutic endoscopy are effective therapies.
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