Management General		Cirrhosis, a chronic diffuse liver disease characterized by irreversible, widespread hepatic fibrosis with regenerative nodule formation, represents the final common pathway to end-stage liver failure, no matter the original cause. Cholestatic liver diseases are the most common causes of pediatric cirrhosis. The progression to cirrhosis is highly variable - in biliary atresia it can occur as early as 12-16 weeks with liver failure at 24 weeks, whereas in cystic fibrosis there can be normal liver function for years before the development of portal hypertension in the second decade of life. As the fibrosis and regenerative nodule formation of cirrhosis progresses, liver architecture becomes distorted. This leads to altered hepatic blood flow and portal hypertension superimposed on the already compromised hepatic function, as well as the cholestasis and its consequences. All of this leads to the complications of cirrhosis and end-stage liver disease. As liver transplant has become a more successful option in the treatment of end-stage pediatric liver disease, the treatment focus has turned to aggressive management of these complications, in order to keep the child from becoming critically ill before transplant. Return to top of page