Table 1.

Metabolic disorders that are treatable with liver transplantation

Disorders that can be cured by LT	Organs/systems to be monitored after LT
Tyrosinemia type I	Kidney
Wilson disease	CNS**
Alpha 1 antitripsin deficiency	Lung
Acute intermittent porphyria (AIP)	Kidney
Maple syrup urine disease (MSUD)	CNS**
Urea cycle disorders (UCD)	CNS**
Ornithine transcarbamylase deficiency (OTC)
Citrullinemia
Carbamoyl phosphate synthetase I deficiency (CPSI)
Argininosuccinate lyase deficiency
Argininemia
Glycogen storage disease (GSD)     Type Ia, Ib, III and IV	Kidney, Blood/Bone marrow (type Ib), GI tract (type Ib)
Crigler-Najjar syndrome	CNS**
Primary hyperoxaluria	Kidney
Progressive familial intrahepatic cholestasis (PFIC)	Liver, GI tract
Disorders with continuous significant metabolic derangement after LT*
Familial hypercholesterolemia	Heart
Erythropoietic protoporphyria (EPP)	Liver
Organic acidemia	CNS**, Kidney, Heart
Methylmalonic acidemia (MMA)
Propionic acidemia (PA)
Mitochondrial disorders	CNS**, Kidney, Heart

^*General consideration of the requirement of post LT metabolic management by IEM specialists. Metabolic outcome of each patient varies and specific management plans including monitoring of metabolites and the frequency of follow up are at each IEM provider's discretion.

CNS: central nervous system

^**For patients who had neurological symptoms before transplant.