Type IA and IB Glycogen Storage Diseases

Beside severe hypoglycemia, growth failure, and acidosis, they also carry a risk of liver adenoma, possibly complicated by intratumoral hemorrhage or liver cell carcinoma.

Wilson's Disease

This is usually treated successfully with copper-chelating agents; in a few cases, howover (e.g., acure liver failure with or without hemolysis, acute relapse after discontinuation of therapy),

Hereditary Tyrosinemia

For several years, liver transplantation has proven extremely useful in children with this otherwise lethal disease. The recenr introduction of the enzyme inhibitor NTBC (see chapter by Kvitington, Clayton, and Leonard, this volume), may diminish the need for liver transplantation.

Inborn Metabolic Disorders Treated by Liver Transplantation

In the European Liver Transplant Registry issued in October 1993 a metabolic disorder was recorder as a indication for transplantation in 18% of 1248 children treated by liver transplantation from May 1988 to June 1993. Two groups can be distinguished, as discussed below.