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June 1978

Hereditary Fructose Intolerance in Childhood: Diagnosis, Management, and Course in 55 Patients

Author Affiliations

From the Unité de Recherche d'Hépatologie Infantile I.N.S.E.R.M. U 56 and Clinique de Pédiatrie, Université Paris-Sud, Hôpital d'Enfants, 94270 Bicêtre, France.

Am J Dis Child. 1978;132(6):605-608. doi:10.1001/archpedi.1978.02120310069014

• 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 human beings.

(Am J Dis Child 132:605-608, 1978)