Galactosemia

Galactosemia (galactose 1-phosphate uridytransferase deficiency) and hereditary fructosemia (fructose 1-phosphate aldolase deficiency) usually occur in infancy with acute or fulminant liver failure and kidney decompensation &. Dietary restriction with elimination of all milk products reverses the diseases. However, the ingestion of even small amount of galactose may lead to progressive liver injury and subsequent cirrhosis and hepatocellular carcinoma development. For this reason some patients have been treated with liver transplantation &.