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 significant elevation of transaminases. The clinical course
may progress rapidly to include the development of coagulopathy,
profound hypoglycemia, and encephalopathy. In its fulminant course,
life-threatening extrahepatic complications, including coma with
cerebral edema, hepatorenal syndrome, and pulmonary hypertension,
can develop and are associated with a very high mortality without liver
transplantation.