What is the MOST common indication for pediatric liver transplantation?
In the pediatric population, biliary atresia is the most common indication for pediatric liver transplantation. It accounts for about half of pediatric liver transplants worldwide and about a third in the United States (Figure).
Biliary atresia presents within the first few weeks of life and is characterized by inflammatory destruction of intra- and extrahepatic bile ducts. The presenting signs and symptoms are persistent jaundice, pale stools, dark urine, failure to thrive, and coagulopathy that is unresponsive to vitamin K administration. Later signs of biliary atresia include hepatosplenomegaly, ascites, and progressive cirrhosis. In the absence of a liver transplant, survival beyond age 24 to 36 months is very unusual.
Metabolic diseases that may also be an indication for pediatric liver transplantation include cystic fibrosis, Wilson disease, tyrosinemia, urea cycle disorders, and α1-antitrypsin (A1AT) deficiency. A1AT deficiency is...
