• The early manifestations of hereditary fructose intolerance are described in a series of 55
patients. Management of this metabolic disorder depends on the severity of liver impairment.
When the patients are given a fructose-free diet, the improvement is dramatic but liver
enlargement and fatty vacuolization of liver cells often persist. These hepatic findings were
also observed in the five homozygous infants who were given a fructosefree diet from birth;
this outcome may support the hypothesis that minimal amounts of fructose are essential for …