Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care

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• 作者：Shikha S. Sundaram ; Cara L. Mack ; Amy G. Feldman ; Ronald J. Sokol
• 刊名：Liver Transplantation
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：23
• 期：1
• 页码：96-109
• 全文大小：199.4KB
• ISSN：1527-6473

文摘

Biliary atresia (BA) is a progressive, fibro-obliterative disorder of the intrahepatic and extrahepatic bile ducts in infancy. The majority of affected children will eventually develop end-stage liver disease and require liver transplantation (LT). Indications for LT in BA include failed Kasai portoenterostomy, significant and recalcitrant malnutrition, recurrent cholangitis, and the progressive manifestations of portal hypertension. Extrahepatic complications of this disease, such as hepatopulmonary syndrome and portopulmonary hypertension, are also indications for LT. Optimal pretransplant management of these potentially life-threatening complications and maximizing nutrition and growth require the expertise of a multidisciplinary team with experience caring for BA. The timing of transplant for BA requires careful consideration of the potential risk of transplant versus the survival benefit at any given stage of disease. Children with BA 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. Liver Transplantation 23:96–109 2017 AASLD.