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红十字战争纪念儿童医院的肝移植手术。

Liver transplantation at Red Cross War Memorial Children's Hospital.

作者信息

Spearman C W N, McCulloch M, Millar A J W, Burger H, Numanoglu A, Goddard E, Gajjar P, Davies C, Muller E, McCurdie F, Kemm D, Cywes S, Rode H, Khan D

机构信息

School of Child and Adolescent Health, University of Cape Town and Red Cross War Memorial Children's Hospital, Rondebosch, Cape Town, South Africa.

出版信息

S Afr Med J. 2006 Sep;96(9 Pt 2):960-3.

Abstract

UNLABELLED

The liver transplant programme for infants and children at Red Cross War Memorial Children's Hospital is the only established paediatric service in sub-Saharan Africa. Referrals for liver transplant assessment come from most provinces within South Africa as well as neighbouring countries.

PATIENTS AND METHODS

Since 1987, 81 children (range 6 months-14 years) have had 84 liver transplants with biliary atresia being the most frequent diagnosis. The indications for transplantation include biliary atresia (48), metabolic (7), fulminant hepatic failure (10), redo transplants (3) and other (16). Four combined liver/kidney transplants have been performed. Fifty-three were reduced-size transplants with donor/recipient weight ratios ranging from 2:1 to 11:1 and 32 children weighed less than 10 kg.

RESULTS

Sixty patients (74%) survived 3 months-14 years post-transplant. Overall cumulative 1- and 5-year patient survival figures are 79% and 70% respectively. However, with the introduction of prophylactic intravenous ganciclovir and the exclusion of hepatitis B virus (HBV) IgG core Ab-positive donors, the 1-year patient survival is 90% and the projected 5-year paediatric survival is > 80%. Early (< 1 month) post-liver-transplant mortality was low. Causes include primary malfunction (1), inferior vena cava thrombosis (1), bleeding oesophageal ulcer (1), sepsis (1) and cerebral oedema (1). Late morbidity and mortality was mainly due to infections: de novo hepatitis B (5 patients, 2 deaths), Epstein-Barr virus (EBV)- related post-transplantation lymphoproliferative disease (12 patients, 7 deaths) and cytomegalovirus (CMV) disease (10 patients, 5 deaths). Tuberculosis (TB) treatment in 3 patients was complicated by chronic rejection (1) and TB-drug-induced subfulminant liver failure (1).

CONCLUSION

Despite limited resources, a successful paediatric programme has been established with good patient and graft survival figures and excellent quality of life. Shortage of donors because of infection with HBV and human immunodeficiency virus (HIV) leads to significant waiting-list mortality and infrequent transplantation.

摘要

未标注

红十字战争纪念儿童医院的婴幼儿和儿童肝移植项目是撒哈拉以南非洲唯一已确立的儿科服务项目。肝移植评估的转诊来自南非的大多数省份以及周边国家。

患者与方法

自1987年以来,81名儿童(年龄范围6个月至14岁)接受了84次肝移植,最常见的诊断是胆道闭锁。移植指征包括胆道闭锁(48例)、代谢性疾病(7例)、暴发性肝衰竭(10例)、再次移植(3例)和其他(16例)。已进行了4例肝肾联合移植。53例为减体积肝移植,供体/受体体重比为2:1至11:1,32名儿童体重不足10千克。

结果

60例患者(74%)在移植后3个月至14年存活。总体1年和5年患者累积生存率分别为79%和70%。然而,随着预防性静脉注射更昔洛韦的引入以及排除乙型肝炎病毒(HBV)IgG核心抗体阳性供体,1年患者生存率为90%,预计5年儿科生存率>80%。肝移植后早期(<1个月)死亡率较低。死因包括原发性功能障碍(1例)、下腔静脉血栓形成(1例)、食管溃疡出血(1例)、败血症(1例)和脑水肿(1例)。晚期发病率和死亡率主要归因于感染:新发乙型肝炎(5例患者,2例死亡)、爱泼斯坦-巴尔病毒(EBV)相关的移植后淋巴细胞增生性疾病(12例患者,7例死亡)和巨细胞病毒(CMV)疾病(10例患者,5例死亡)。3例患者的结核病治疗因慢性排斥反应(1例)和抗结核药物引起的亚暴发性肝衰竭(1例)而复杂化。

结论

尽管资源有限,但已建立了一个成功的儿科项目,患者和移植物生存率良好,生活质量优异。由于感染HBV和人类免疫缺陷病毒(HIV)导致供体短缺,导致等待名单上的死亡率显著增加且移植不频繁。

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