الفهرس 
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Abstract
Liver transplants have become a well-established procedure to treat
liver failure in pediatric patients. As a result of refinements in surgical
technique, the advent of new immunosuppressive agents and improvements
in critical care, patient survival at 1 year has improved from 20% in the
1970s to 90% currently. In several ways, liver transplants for pediatric
patients are quite similar to those for adults; however, several features make
pediatric patients unique.
The clinical indications for a pediatric liver transplant are similar to
those for adults. Endpoints that require a transplant include evidence of
portal hypertension as manifested by variceal bleeding and ascites,
significant jaundice, intractable pruritus, encephalopathy, failing synthetic
function (e.g., hypoalbuminemia or coagulopathy), poor quality of life, and
failure to thrive (as manifested by poor weight gain or poor height increase).
Biliary atresia is the most common indication for a pediatric liver
transplant. Once the diagnosis is established, a portoenterostomy, or Kasai
procedure, is indicated to drain microscopic ducts within the porta hepatis.
Successful bile flow can be achieved in 40 to 60% of patients whose Kasai
procedure takes place early in their life. However, even with a Kasai
procedure, 75% of children with biliary atresia eventually require a liver
transplant because of progressive cholestasis followed by cirrhosis. Other
cholestatic disorders that may eventually require a transplant include
Summary
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sclerosing cholangitis, familial cholestasis syndromes, and paucity of
intrahepatic bile ducts (as seen with Alagille syndrome).
Metabolic disorders probably account for the next largest group of
disorders that may require a liver transplant. Such disorders may directly
result in liver failure or may have mainly extrahepatic manifestations.
Alpha1-antitrypsin deficiency is the most common metabolic disorder that
may require a liver transplant. Another metabolic disorder resulting in liver
failure is tyrosinemia, a hereditary disorder characterized by deficiency of an
enzyme that degrades the metabolic products of tyrosine, resulting in
cirrhosis and a greatly increased risk for HCC.
FHF may be seen in children from similar causes as seen in adults. Of
note, younger children (<10 years old) with FHF have a poor prognosis for
spontaneous recovery of liver function without a transplant.