儿童肝移植术后的随访与治疗

Management of pediatric patients who underwent liver transplantation

目的评估复旦大学附属儿科医院肝移植患儿术后疗效。方法回顾性分析2002年10月至2005年5月期间8例小儿肝移植术后的临床效果和生活质量。本组男4例,女4例,肝移植年龄4～67个月,体重6～19 kg。6例亲体活体肝移植,1例劈裂式肝移植,1例减体积肝移植。结果围手术期死亡1例,手术存活率87.5%;手术成功患儿中1例于术后8个月死于消化道出血,1年生存率为75.0%,其他6例存活至今,最长生存期43个月。术后并发症:急性排斥反应2例;远期感染:巨细胞病毒感染2例,Epstein-Barr 病毒感染1例,乙型肝炎病毒感染1例;低蛋白血症3例。远期外科并发症:门静脉血栓1例,下腔静脉和肝静脉狭窄1例。他克莫司(FKS06)的副作用:高血压3例,肾脏损害1例,肝脏损害1例,腹泻1例。所有存活患儿无心理异常;1例术前体格发育落后者,术后发生了生长追赶。结论肝移植改善了小儿终末期肝病的预后。儿童肝移植后并发症多见。周密的术后随访与管理有助于发现各种内外科并发症和保证术后生活质量。

Objective To evaluate the outcome of pediatric patients who underwent liver transplantation between Oct. 2002 and May 2005 in the Pediatric Hospital Methods Eight cases aged from 4 to 67 months who underwent liver transplantation were analyzed retrospectively. Four of the patients were boys and 4 gids, whose body weight at the time of liver transplantation was 6 - 19 kg. The underlying diseases were biliary atresia, congenital cholostasis, drug-induced cholestatic cirrhosis and cryptogenic cirrhosis. These patients had been followed up for blood routine examinations, liver and renal function, serum electrolytes and blood concentration of tacrolimus for 16 to 43 months after liver transplatation. Results of serological studies for viral etiology, liver biopsy, growth and mental development were also recorded. Results One-year-survival-rate was 75.0% with the longest survival time of 43 months after transplantation. One patient died from renal failure due to postoperative bleeding 24 hours after the surgery and another case died of variceal hemorrhage 8 months after transplantation. Posttransplantation complications included acute cellular rejection, viral infection and hypoalbuminemia. Viral infections included cytomegalovirus infection in 3 cases, Esptein-Barr virus infection in 1 and hepatitis B virus infection in 1. Surgical complications of portal vein thrombosis and stenosis of inferior vena cava and hepatic vein occurred in 2 cases respectively. Side effects of tacrelimus including hypertension, renal damage, liver damage and diarrhea were observed. Significant growth-retardation was not often seen. A self-reported high quality of life was common. Conclusions Close follow-up and management of patients after liver transplantation may significantly increase the survival rate and improve quality of life in children with endstage liver diseases.