Lamireau T, Martin S, Lallier M, Marcotte J E, Alvarez F
Pediatric Gastroenterology, Hôpital Sainte-Justine, Montreal, Quebec, Canada.
Can J Gastroenterol. 2006 Jul;20(7):475-8. doi: 10.1155/2006/539345.
Liver disease is the third most common cause of death in children with cystic fibrosis (CF). Liver transplantation is an effective treatment in children with hepatic failure.
The objective of the present study was to review the indications and postoperative course of hepatic transplantation in a cystic fibrosis population.
Five children with CF, at a mean age of 16.5 years, underwent liver transplantation.
All patients showed cirrhosis, portal hypertension and hepatic failure. The main postoperative complication was ascites refractory to treatment in two patients. No significant deterioration of the pulmonary function was noted. Two patients died, one of Hodgkin lymphoma and the other of progressive pulmonary failure.
Liver transplantation was indicated in children with CF when hepatic failure and/or severe portal hypertension was present with well-preserved pulmonary function.
肝脏疾病是囊性纤维化(CF)患儿第三大常见死因。肝移植是治疗肝衰竭患儿的有效方法。
本研究的目的是回顾囊性纤维化患者肝移植的适应证及术后病程。
5例CF患儿接受了肝移植,平均年龄16.5岁。
所有患者均表现为肝硬化、门静脉高压和肝衰竭。主要术后并发症是2例患者出现难治性腹水。未观察到肺功能显著恶化。2例患者死亡,1例死于霍奇金淋巴瘤,另1例死于进行性肺衰竭。
当存在肝衰竭和/或严重门静脉高压且肺功能良好时,CF患儿适合进行肝移植。