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Liver transplantation is now the accepted standard of care for the management of irreversible end-stage liver disease in children. Since the first successful liver transplant by Starzl in 1966, continuous improvements in patient selection, surgical technique and postoperative care, as well as advances in organ preservation and immunosuppression management have resulted in improved graft and patient survival. Earlier referral for transplantation, and advances in the clinical management of rejection, infection, and biliary and vascular complications have also contributed to improved outcomes.

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Each year, approximately 600 children have received a liver transplant in the United States.1 Of these, 15% have been living donor liver transplants. In 2007, approximately 400 children were waiting for a liver transplant on the United Network for Organ Sharing (UNOS) national waiting list. The risk of death while waiting for a transplant is related to age. Young children less than 1 year old have the highest death rate of all waiting list candidates, adult or pediatric, nearly 6 times the death rate for older children and adults.

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Indications for liver transplantation are listed in Table 130-1. Liver transplantation is indicated for life threatening failure of liver function due to either chronic liver conditions or acute hepatic necrosis. End-stage liver disease because of cholestatic liver disease represents the reason that the majority of children are referred for liver transplantation. Of these, biliary atresia is the most common single diagnosis, accounting for approximately 45% of all children undergoing liver transplant.2 Other common indications include metabolic liver diseases, acute liver failure, hepatic malignancy, and chronic hepatitis.

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Table 130-1. Indications for Liver Transplantation
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Children with advanced liver disease may develop cirrhosis and present clinically with decreased synthetic liver function, evidenced by jaundice, hypoalbuminemia, coagulopathy, or poor growth. Coagulopathy may be exacerbated by associated vitamin K deficiency because of poor intestinal absorption of fat-soluble vitamins. Hepatic cirrhosis is often associated with portal hypertension, presenting clinically with hepatosplenomegaly, ascites, increased risk for variceal hemorrhage, and encephalopathy. Acute hepatic necrosis typically presents within 8 weeks of a gastrointestinal illness, associated with jaundice, fever, and ...

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