Disorders of Fructose Metabolism

  • M. Odièvre

Summary

Three inborn errors of fructose metabolism are known. Two of these concern the fructose pathway: essential fructosuria and hereditary fructose intolerance (HFI). In the third, fructose-1,6-diphosphatase deficiency, gluconeogenesis is severely impaired; this defect will be described elsewhere.

In this chapter the two inherited abnormalities of fructose metabolism are described: deficiency of fructokinase and deficiency of fructose-1-phosphate aldolase. The first one is responsible for a benign condition, essential fructosuria, that does not necessitate any dietary restrictions. The second one is responsible for a severe disease, sometimes lethal, that necessitates a fructose-free diet as soon as possible. Both conditions are inherited as autosomal recessive traits.

Keywords

Sugar Sucrose Fructose Proteinuria Galactose 

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References

  1. 1.
    Van Den Berghe G (1975) Biochemical aspects of hereditary fructose intolerance. In: Hommes FA, Van Den Berg CJ (eds) Normal and pathological development of energy metabolism. Academic, New YorkGoogle Scholar
  2. 2.
    Odievre M. Gentil C, Gautier M, Alagille D (1978) Hereditary fructose intolerance in childhood. Diagnosis, management and course in 55 patients. Am J Dis Child 132: 605–608PubMedGoogle Scholar
  3. 3.
    Mock DM, Perman JA, Thaler MM, Morris RC (1983) Chronic fructose intoxication after infancy in children with hereditary fructose intolerance. A cause of growth retardation. N Engl J Med 309: 764–770PubMedCrossRefGoogle Scholar
  4. 4.
    Cornblath M, Schwartz R (1976) Hereditary fructose intolerance. In: Disorders of carbohydrate metabolism in infancy. Saunders, PhiladelphiaGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1990

Authors and Affiliations

  • M. Odièvre

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