Hereditary fructose intolerance in childhood. Diagnosis, management, and course in 55 patients
M. Odievre, C. Gentil, M. Gautier and D. Alagille
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 a 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
fructose-free diet from birth; this outcome may support the hypothesis that
minimal amounts of fructose are esential for human beings.
