Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care.
Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care.
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DOI：
10.1002/lt.24640
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发表时间：
2017-01
影响因子：
4.6
通讯作者：
Sokol, Ronald J.
中科院分区：
文献类型：
--
作者：
Sundaram, Shikha S.;Mack, Cara L.;Feldman, Amy G.;Sokol, Ronald J.
胆道闭锁是婴儿期肝内和肝外胆管的一种进行性纤维闭塞性疾病。大多数患病儿童最终会发展为终末期肝病并需要进行肝移植。胆道闭锁肝移植的指征包括葛西（Kasai）肝门肠吻合术失败、严重且顽固的营养不良、反复胆管炎以及门静脉高压的进行性表现。该疾病的肝外并发症，如肝肺综合征和门肺高压，也是肝移植的指征。对这些可能危及生命的并发症进行最佳的移植前管理以及最大限度地改善营养和生长，需要一个有胆道闭锁护理经验的多学科团队的专业知识。胆道闭锁移植的时机需要仔细考虑在疾病任何特定阶段移植的潜在风险与生存益处。胆道闭锁患儿通常需要长时间等待移植，除非因疾病严重程度而获得特殊加分。因此，家庭对这一艰难过程做好准备至关重要。
Biliary Atresia is a progressive, fibro-obliterative disorder of the intra and extrahepatic bile ducts in infancy. The majority of affected children will eventually develop end-stage liver disease and require liver transplantation. Indications for liver transplant in biliary atresia include failed Kasai portoenterostomy, significant and recalcitrant malnutrition, recurrent cholangitis, and the progressive manifestations of portal hypertension. Extra-hepatic complications of this disease, such as hepatopulmonary syndrome and portopulmonary hypertension, are also indications for liver transplantation. Optimal pre-transplant management of these potentially life threatening complications and maximizing nutrition and growth require the expertise of a multi-disciplinary team with experience caring for biliary atresia. The timing of transplant for biliary atresia requires careful consideration of the potential risk of transplant versus the survival benefit at any given stage of disease. Children with biliary atresia often experience long wait times for transplant unless exception points are granted to reflect severity of disease. Family preparedness for this arduous process is therefore critical.
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