Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care. Liver Transpl 2017 Jan;23(1):96-109
Date
09/22/2016Pubmed ID
27650268Pubmed Central ID
PMC5177506DOI
10.1002/lt.24640Scopus ID
2-s2.0-85006494243 (requires institutional sign-in at Scopus site) 165 CitationsAbstract
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.
Author List
Sundaram SS, Mack CL, Feldman AG, Sokol RJAuthor
Cara Lynn Mack MD Chief, Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Biliary AtresiaChild
Emotional Adjustment
End Stage Liver Disease
Family Relations
Health Policy
Health Services Accessibility
Hepatopulmonary Syndrome
Humans
Hypertension, Portal
Infant
Liver Transplantation
Portoenterostomy, Hepatic
Preoperative Care
Severity of Illness Index
Survival Rate
Time Factors
Waiting Lists
