Stampfl D A, Muñoz S J, Moritz M J, Rubin R, Armenti V T, Jarrell B E, Maddrey W C
Department of Medicine, Jefferson Medical College, Philadelphia, Pennsylvania.
Gastroenterology. 1990 Dec;99(6):1834-6. doi: 10.1016/0016-5085(90)90497-o.
Wilson's disease may present with severe acute hepatocellular failure. The only effective treatment for fulminant Wilson's disease is liver transplantation, which may lead to reversal of the underlying disease. Some patients with cirrhosis who are too ill to undergo orthotopic liver transplantation have been treated with heterotopic liver transplantation. However, use of heterotopic liver transplantation for fulminant hepatocellular failure has not been successful. This case study involves a patient in whom a heterotopic liver transplant was successfully used for treatment of Wilson's disease presenting with fulminant hepatocellular failure.
威尔逊氏病可能表现为严重的急性肝细胞衰竭。暴发性威尔逊氏病唯一有效的治疗方法是肝移植,这可能会使潜在疾病得到逆转。一些肝硬化患者病情过重无法进行原位肝移植,已接受异位肝移植治疗。然而,将异位肝移植用于暴发性肝细胞衰竭治疗尚未成功。本病例研究涉及一名患者,其异位肝移植成功用于治疗表现为暴发性肝细胞衰竭的威尔逊氏病。