Diseases

Inborn Errors Fructose Metabolism

Inherited abnormalities of fructose metabolism, which include three known autosomal recessive types: hepatic fructokinase deficiency (essential fructosuria), hereditary fructose intolerance, and hereditary fructose-1,6-diphosphatase deficiency. Essential fructosuria is a benign asymptomatic metabolic disorder caused by deficiency in fructokinase, leading to decreased conversion of fructose to fructose-1-phosphate and alimentary hyperfructosemia, but with no clinical dysfunction; may produce a false-positive diabetes test.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

Inherited abnormalities of fructose metabolism, which include three known autosomal recessive types: hepatic fructokinase deficiency (essential fructosuria), hereditary fructose intolerance, and hereditary fructose-1,6-diphosphatase deficiency. Essential fructosuria is a benign asymptomatic metabolic disorder caused by deficiency in fructokinase, leading to decreased conversion of fructose to fructose-1-phosphate and alimentary hyperfructosemia, but with no clinical dysfunction; may produce a false-positive diabetes test.

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Synonyms

1 entry terms
  • Fructose Metabolism, Inborn Errors

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Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

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History Note

89; FRUCTOSE INTOLERANCE was see under CARBOHYDRATE METABOLISM, INBORN ERRORS 1967-88 (Prov 1964-66)

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Previous Indexing

  • Carbohydrate Metabolism, Inborn Errors (1966-1988)
  • Fructose (1966-1988)

MeSH Hierarchy

Tree Numbers

  1. C16w.320w.565w.202w.251
  2. C18.452w.648w.202w.251

AMA Style

References

  1. National Library of Medicine. Inborn Errors Fructose Metabolism. Medical Subject Headings (MeSH). 2026. Unique ID D015318. http://id.nlm.nih.gov/mesh/2026/D015318
  2. Inborn Errors Fructose Metabolism. In: Wikidata. https://www.wikidata.org/wiki/Q19000492