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Fructose Intolerance MeSH Descriptor Data 2024


MeSH Heading
Fructose Intolerance
Tree Number(s)
C16.320.565.202.251.271
C18.452.648.202.251.271
Unique ID
D005633
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/D005633
Annotation
an inborn error of fructose metab; do not use /‌congen & do not coord with INFANT, NEWBORN, DISEASES
Scope Note
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.
Entry Term(s)
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
See Also
Fructose-Bisphosphate Aldolase
Public MeSH Note
1991; see FRUCTOSE METABOLISM, INBORN ERRORS 1989-1990; see CARBOHYDRATE METABOLISM, INBORN ERRORS 1967-1988
History Note
1991(1975); use FRUCTOSE METABOLISM, INBORN ERRORS 1989-1990; use CARBOHYDRATE METABOLISM, INBORN ERRORS 1964-1988
Date Established
1991/01/01
Date of Entry
1999/01/01
Revision Date
2013/07/08
Fructose Intolerance Preferred
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