Zahmatkeshan M, Bahador A, Geramizade B, Emadmarvasti V, Malekhosseini S A
Department of pediatrics, school of medicine, Shiraz University of Medical Sciences, Shiraz, Iran.
Department of organ transplantation, school of medicine, Shiraz University of Medical Sciences, Shiraz, Iran.
Int J Organ Transplant Med. 2012;3(4):189-91.
Caroli disease is a rare congenital disorder characterized by multifocal, segmental dilatation of intrahepatic bile ducts. Patients with Caroli disease who have recurrent bouts of biliary infection, particularly those who also have complications related to portal hypertension may require liver transplantation. In liver transplant ward of Shiraz University of Medical Science we had 4 patients with Caroli disease who were transplanted. Herein, we describe the demographic characteristics and post-transplant course of the patients. These patients presented with liver failure, recurrent cholangitis and portal hypertension sequelae unresponsive to medical treatment. The mean age of patients was 24.5 (range: 18-36) years, the mean MELD score was 17.5 (range: 11-23), three patients were female; one was male. All of the patients had good post-transplantation course except for one patient who developed post-operative biliary stricture for whom biliary reconstruction was done.
卡罗里病是一种罕见的先天性疾病，其特征为肝内胆管多灶性、节段性扩张。患有反复胆道感染的卡罗里病患者，尤其是那些同时伴有门静脉高压相关并发症的患者，可能需要进行肝移植。在设拉子医科大学的肝移植病房，我们有4例卡罗里病患者接受了移植手术。在此，我们描述这些患者的人口统计学特征及移植后的病程。这些患者表现为肝衰竭、复发性胆管炎和门静脉高压后遗症，内科治疗无效。患者的平均年龄为24.5岁（范围：18 - 36岁），平均终末期肝病模型（MELD）评分是17.5（范围：11 - 23），3例为女性，1例为男性。除1例术后发生胆道狭窄并接受胆道重建的患者外，所有患者移植后病程良好。
