Disorders of Fructose Metabolism

  • M. Odièvre


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.


Pyloric Stenosis Renal Tubular Dysfunction Autosomal Recessive Trait Hereditary Fructose Intolerance Fructose Metabolism 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  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

There are no affiliations available

Personalised recommendations