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  • Title: Hereditary fructose intolerance in childhood. Diagnosis, management, and course in 55 patients.
    Author: Odièvre M, Gentil C, Gautier M, Alagille D.
    Journal: Am J Dis Child; 1978 Jun; 132(6):605-8. PubMed ID: 655145.
    Abstract:
    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.
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