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儿童肝移植——红十字战争纪念儿童医院的经验

Liver transplantation in children--the Red Cross War Memorial Children's Hospital experience.

作者信息

Millar A J, Spearman C W, McCulloch M, Goddard E, Lopez T, Thomas J, Butt A, Rode H, Kahn D, Cywes S

机构信息

Departments of Paediatric Surgery, Paediatrics, Medicine and Surgery, Red Cross War Memorial Children's Hospital, Institute of Child Health and Medical Research Council Liver Research Centre, University of Cape Town.

出版信息

S Afr J Surg. 2000 Dec;38(4):91-7.

PMID:11424862
Abstract

Liver transplantation for infants and children has been available in South Africa for more than a decade. Current concerns have shifted from an initial target of early post-transplant survival to quality of life in the long term. Since 1985, 175 infants and children have been assessed, with 104 accepted for transplantation. Fifty have had orthotopic liver transplants (OLTx), 48 (3 retransplants) in Cape Town and 2 abroad. Biliary atresia was the most frequent diagnosis (52%) followed by acute liver failure (ALF) (16%). Waiting list mortality has remained high (15%), particularly for the ALF group (50%). Thirty-four patients survive 1 month--14 years post-transplant. Early post OLTx mortality was low (7%) but late morbidity and mortality (23%) were mainly due to viral infection: de novo hepatitis B (5 patients, 2 deaths), EBV-related post-transplantation lymphoproliferative disease (PTLPD) (6 patients, 4 deaths) and CMV disease (9 patients, 4 deaths). Tuberculosis prophylaxis, required in 6 cases, resulted in major morbidity in 1. Hypertension requiring medication along with some compromise of renal function has been present in all but 2 patients. However, all those of school-going age (20) attend school normally and remain in good health and only 3 of the survivors have abnormal liver function tests. Successful liver transplantation is possible in a developing country with limited resources. Scarcity of virus-free donors (HBV and HIV) leading to waiting list mortality and infrequent retransplantation along with long-term consequences of immunosuppression (infection, lymphoma and renal toxicity) remain problems.

摘要

在南非,婴儿和儿童肝脏移植已有十多年的历史。目前关注的焦点已从移植后早期存活这一最初目标转向长期生活质量。自1985年以来,对175名婴儿和儿童进行了评估,其中104名被接受移植。50名接受了原位肝移植(OLTx),48名(3名再次移植)在开普敦,2名在国外。胆道闭锁是最常见的诊断(52%),其次是急性肝衰竭(ALF)(16%)。等待名单上的死亡率一直很高(15%),尤其是ALF组(50%)。34名患者在移植后1个月至14年存活。OLTx术后早期死亡率较低(7%),但晚期发病率和死亡率(23%)主要归因于病毒感染:新发乙型肝炎(5例,2例死亡)、EB病毒相关的移植后淋巴细胞增生性疾病(PTLPD)(6例,4例死亡)和巨细胞病毒病(9例,4例死亡)。6例需要进行结核病预防,其中1例出现严重发病情况。除2名患者外,所有患者均出现需要药物治疗的高血压以及一定程度的肾功能损害。然而,所有达到上学年龄的患者(20名)都正常上学且身体健康,只有3名幸存者肝功能检查异常。在资源有限的发展中国家,成功进行肝脏移植是可行的。无病毒供体(乙肝病毒和艾滋病毒)稀缺导致等待名单上的死亡率和再次移植不常见,以及免疫抑制的长期后果(感染、淋巴瘤和肾毒性)仍然是问题。

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