No overt clinical or biochemical evidence of liver disease |
Death from complications of end-stage liver disease after age 5 years |
Biochemical abnormalities without symptoms of cholestasis |
Listed for or received a liver transplant after the age of 5 years |
Symptoms of cholestasis (e.g. pruritus) adequately managed with medications |
Cholestasis with significant complications (e.g. pathological bone fractures, pruritus requiring partial biliary diversion, etc.) |
|
Portal Hypertension (splenomegaly, esophageal or gastric varices, or ascites) |