[Hereditary fructose intolerance]

Abstract

Two cases of hereditary fructose intolerance are reported. In the first one the symtomatology has started with an acute hepatic failure; the second one has come to our observation with a diagnosis of intrahepatic biliary duct atresia. It is underlined the difficulty of a differential diagnosis, in infants with serious hepatic failure, between infectious, metabolic and others illnesses.