De Kerckhove Laurent, De Meyer Martine, Verbaandert Catherine, Mourad Michel, Sokal Etienne, Goffette Pierre, Geubel Andre, Karam Vincent, Adam Rene, Lerut Jan
Abdominal Transplantation Unit, Université Catholique de Louvain, UCL, Brussels, Belgium.
Transpl Int. 2006 May;19(5):381-8. doi: 10.1111/j.1432-2277.2006.00292.x.
Caroli's disease (CD) or syndrome (CS) are rare inherited disorders which may cause severe, life-threatening, cholangitis or which may lead to hepatobiliary degeneration. The typical cystic biliary anomalies are often associated to congenital hepatic fibrosis (CHF) and, less frequently, to cystic renal disease especially autosomic recessive polycystic kidney disease (ARPKD). The place of liver transplantation (LT) in the treatment of CD or CS is evaluated based on our own experience of three successfully transplanted patients, the literature review of 19 patients and the European experience with 110 patients collected in the European Liver Transplant Registry. LT should be proposed as a definitive therapeutic option once severe cholangitis or (suspicion of) malignant transformation is present. The frequently used radiological, endoscopical or surgical biliary drainage procedures carry a high morbidity and mortality rate. In case of concomitant symptomatic CHF and renal failure, combined or sequential hepatorenal transplantation should be carried out, dependent on the evolution of the hepatic and renal disease. In case of associated ARPKD, renal transplantation is often indicated early on because of the more rapid progression of the renal component of the disease.
卡罗里病（CD）或综合征（CS）是罕见的遗传性疾病，可导致严重的、危及生命的胆管炎，或导致肝胆退变。典型的胆管囊性异常常与先天性肝纤维化（CHF）相关，较少与囊性肾病相关，尤其是常染色体隐性多囊肾病（ARPKD）。基于我们成功进行肝移植的3例患者的经验、对19例患者的文献回顾以及欧洲肝脏移植登记处收集的110例患者的欧洲经验，对肝移植（LT）在CD或CS治疗中的地位进行了评估。一旦出现严重胆管炎或（疑似）恶变，应将肝移植作为一种确定性治疗选择。常用的放射学、内镜或手术胆管引流程序具有较高的发病率和死亡率。对于伴有症状性CHF和肾衰竭的情况，应根据肝脏和肾脏疾病的进展情况进行联合或序贯肝肾移植。对于合并ARPKD的情况，由于疾病的肾脏部分进展较快，通常早期就需要进行肾移植。
