What is the MOST common indication for pediatric liver transplantation?
- □ (A) α1-antitrypsin deficiency
- □ (B) Biliary atresia
- □ (C) Hepatoblastoma
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 a rare genetic disease caused by abnormal production of the serine protease inhibitor α antitrypsin (AAT). This results in the irreversible inactivation of neutrophil elastase in the lung, increased levels of lung and liver inflammatory mediators, and the accumulation of insoluble abnormal proteins within liver hepatocytes. A1AT, while not the most common cause of pediatric liver transplantation, is the most common genetic cause of childhood liver disease.
Pediatric patients with liver tumors unresectable after chemotherapy may also undergo liver transplantation. Hepatoblastoma is the most common pediatric liver tumor. Hepatoblastoma, however, constitutes only 7.3% of pediatric liver transplants.
The one- and five-year survival rates in pediatric liver transplant recipients are 90% and 85%, respectively.
Answer B
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