Journal
LIVER TRANSPLANTATION
Volume 23, Issue 1, Pages 96-109Publisher
WILEY
DOI: 10.1002/lt.24640
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Categories
Funding
- National Institutes of Health [U01DK062453]
- National Institutes of Health/NCATS Colorado CTSA [UL1TR001082]
- Shire
- NATIONAL CENTER FOR ADVANCING TRANSLATIONAL SCIENCES [UL1TR001082] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASES [R01DK094937, U01DK062453] Funding Source: NIH RePORTER
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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.
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