Intrahepatic biliary dysplasia

Intrahepatic biliary dysplasia

Intrahepatic biliary dysplasia is caused by a rare autosomal dominant trait. Liver problems are caused by structural changes that impair bile duct function. Children with intrahepatic biliary dysplasia often have other anomalies that affect cardiac function. Infants with intrahepatic biliary dysplasia often have cholestatic jaundice in the neonatal period, often accompanied by failure to thrive, prematurity, or small size for gestational age.

Treatment consists of symptomatic and supportive care, and good nutrition. The only surgical treatment is liver transplantation. An infant who receives a transplanted liver requires lifelong cyclosporin and prednisone therapy to prevent liver rejection. The quality of life and life expectancy for patients with intrahepatic biliary dysplasia depends primarily on the extent of heart disease and on long term liver status. Some patients experience a gradual remission of cholestatis, while others progress to cirrhosis.

Columbia University has a comprehensive liver disease resource. Please click here, and investigate Liver Transplantation.

Then answer the following question: What is the average success rate 5 years after liver transplanation?