姐妹同患Caroli综合征并ARPKD 2例  

Sisters suffering from the same Caroli's syndrome and ARPKD in 2 cases

作　　者：董九伟[1] 姜晓华[1] 张伟强[1] 朱文灵[1] 孙玉晶[1] 王玲玲[1] 李凤臣[1] 慕昭梅 

机构地区：[1]解放军107中心医院小儿科,山东烟台264000

出　　处：《中国优生与遗传杂志》2014年第9期139-141,143,共4页Chinese Journal of Birth Health & Heredity

摘　　要：目的探讨Caroli综合征的临床特征,提高对该病的认识。方法对我院先后收治的2例均患Caroli综合征姐妹的临床资料进行回顾性分析,并复习文献。结果本组2例患儿均表现上消化道出血、肝脏纤维化、脾肿大、重度贫血并多囊肾,均给予输血、抑酸等对症治疗。结论 Caroli综合征早期缺乏特异性临床表现,症状单一,起病隐匿,早期难于发现。肝脏移植是唯一的根治方法。Objective： To investigate the clinical characteristics of Caroli＇s syndrome, raise awareness of the disease. Methods： Our hospital has treated two patients were suffering from clinical data sisters Caroli syndrome were retrospectively analyzed, and review of the literature. Results： The group 2 patients showed upper gastrointestinal bleeding, liver fibrosis, splenomegaly, severe anemia and polycystic kidney disease, were given blood transfusions, acid suppression and other symptomatic treatment. Conclusion： Clinical manifestations of early single, the lack of specific clinical manifestations, early difficult to find, easy to cause misdiagnosis. Liver transplantation is the only cure.

关 键 词：Caroli综合征 多囊肾 临床表现 肝脏移植 

分 类 号：R725.9[医药卫生—儿科] R726[医药卫生—临床医学]