Department of Surgery, Section of Transplantation and Hepatobiliary Surgery, University of Utah School of Medicine, Salt Lake City, UT 84132, USA.
J Pediatr Surg. 2011 Aug;46(8):1638-41. doi: 10.1016/j.jpedsurg.2011.04.011.
Caroli's disease (including Caroli's syndrome) is a rare autosomal recessive disorder of the liver characterized by diffuse cystic dilatation of the intrahepatic bile ducts. The disease may present at any age and is characterized by recurrent episodes of biliary obstruction, cholangitis, hepaticolithiasis, and liver abscesses. Caroli's syndrome is further associated with congenital hepatic fibrosis and portal hypertension. Patients with recurrent complications or cirrhosis may die because of recurrent infection, portal hypertension, liver failure, or cholangiocarcinoma. Liver transplantation is the treatment of choice for these complicated patients. Here we describe the youngest reported patient with Caroli's syndrome treated successfully using liver transplantation and review the recent literature.
卡罗利病（包括卡罗利综合征）是一种罕见的常染色体隐性遗传性肝脏疾病，其特征为肝内胆管弥漫性囊状扩张。该病可发生于任何年龄，其特征为反复发作的胆道梗阻、胆管炎、肝胆管结石和肝脓肿。卡罗利综合征进一步与先天性肝纤维化和门静脉高压相关。由于反复感染、门静脉高压、肝功能衰竭或胆管癌，反复发作并发症或肝硬化的患者可能会死亡。肝移植是这些复杂患者的治疗选择。在此，我们描述了经肝移植成功治疗的有报道的最年轻卡罗利综合征患者，并复习了近期文献。
