Azoulay D, Castaing D, Lemoine A, Samuel D, Majno P, Reynes M, Charpentier B, Bismuth H
Centre Hépato-Biliaire, Faculté de Médecine Paris-Sud, France.
Clin Nephrol. 1998 Aug;50(2):118-22.
Azathioprine-induced veno-occlusive disease of the liver mainly described after kidney transplantation is as rare as severe with a high mortality due to acute portal hypertension and liver failure. A kidney-transplanted patient with severe azathioprine-induced veno-occlusive disease of the liver and worsening despite drug discontinuation was treated by emergency transjugular intrahepatic portosystemic shunt. Whereas the veno-occlusive disease was controlled, the patient developed severe intractable portosystemic encephalopathy successfully treated by a stent reducer maintaining a certain degree of portal diversion. Twelve months after transjugular intrahepatic portosystemic shunt, liver function was normalized and the stent was thrombosed with a subnormal liver histology. Thirty-six months after transjugular intrahepatic portosystemic shunt the patient is alive with normal liver function tests and kidney graft function. Transjugular intrahepatic portosystemic shunt for treatment of severe veno-occlusive disease of the liver is an alternative to tide the patient over until recovery of liver function.
硫唑嘌呤诱发的肝静脉闭塞病主要在肾移植后被描述，极为罕见且病情严重，因急性门静脉高压和肝衰竭导致死亡率很高。一名肾移植患者发生了严重的硫唑嘌呤诱发的肝静脉闭塞病，尽管停用了药物病情仍在恶化，通过紧急经颈静脉肝内门体分流术进行了治疗。虽然肝静脉闭塞病得到了控制，但患者出现了严重的顽固性门体性脑病，通过维持一定程度门静脉分流的支架缩窄器成功治疗。经颈静脉肝内门体分流术后12个月，肝功能恢复正常，支架血栓形成，肝脏组织学检查结果低于正常水平。经颈静脉肝内门体分流术后36个月，患者存活，肝功能检查和肾移植功能正常。经颈静脉肝内门体分流术治疗严重肝静脉闭塞病是一种替代方法，可使患者度过难关直至肝功能恢复。
