Diseases

Fructose Intolerance

An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity, resulting in accumulation of fructose-1-phosphate. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycemia following ingestion of fructose. Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. Patients develop a strong distaste for sweet food, and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity, resulting in accumulation of fructose-1-phosphate. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycemia following ingestion of fructose. Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. Patients develop a strong distaste for sweet food, and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet.

MeSH Record

Classification

Broader headings

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MeSH Record

See Also

MeSH Record

Synonyms

34 entry terms
  • ALDOB Deficiency
  • Aldolase B Deficiency
  • Fructose Aldolase B Deficiency
  • Fructose Intolerance, Hereditary
  • Fructose-1,6-Biphosphate Aldolase Deficiency
  • Fructose-1,6-Bisphosphate Aldolase B Deficiency
  • Fructose-1-Phosphate Aldolase Deficiency
  • Fructosemia
  • Hereditary Fructose Intolerance
  • ALDOB Deficiencies
  • Aldolase B Deficiencies
  • Aldolase Deficiencies, Fructose-1,6-Biphosphate
  • Aldolase Deficiencies, Fructose-1-Phosphate
  • Aldolase Deficiency, Fructose-1,6-Biphosphate
  • Aldolase Deficiency, Fructose-1-Phosphate
  • Deficiencies, ALDOB
  • Deficiencies, Aldolase B
  • Deficiencies, Fructose-1,6-Biphosphate Aldolase
  • Deficiencies, Fructose-1-Phosphate Aldolase
  • Deficiency, ALDOB
  • Deficiency, Aldolase B
  • Deficiency, Fructose-1,6-Biphosphate Aldolase
  • Deficiency, Fructose-1-Phosphate Aldolase
  • Fructose 1 Phosphate Aldolase Deficiency
  • Fructose 1,6 Biphosphate Aldolase Deficiency
  • Fructose 1,6 Bisphosphate Aldolase B Deficiency
  • Fructose Intolerances
  • Fructose Intolerances, Hereditary
  • Fructose-1,6-Biphosphate Aldolase Deficiencies
  • Fructose-1-Phosphate Aldolase Deficiencies
  • Fructosemias
  • Hereditary Fructose Intolerances
  • Intolerance, Fructose
  • Intolerances, Fructose

MeSH Record

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.

MeSH Record

Indexing Annotation

an inborn error of fructose metab; do not use /congen & do not coord with INFANT, NEWBORN, DISEASES

MeSH Record

History Note

1991(1975); use FRUCTOSE METABOLISM, INBORN ERRORS 1989-1990; use CARBOHYDRATE METABOLISM, INBORN ERRORS 1964-1988

MeSH Hierarchy

Tree Numbers

  1. C16w.320w.565w.202w.251.271w
  2. C18.452w.648w.202w.251.271w

AMA Style

References

  1. National Library of Medicine. Fructose Intolerance. Medical Subject Headings (MeSH). 2026. Unique ID D005633. http://id.nlm.nih.gov/mesh/2026/D005633
  2. Fructose Intolerance. In: Wikipedia. https://en.wikipedia.org/wiki/Hereditary_fructose_intolerance
  3. Fructose Intolerance. In: Wikidata. https://www.wikidata.org/wiki/Q1609755