MeSH Browser

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

Allowable Qualifiers: blood (BL)

cerebrospinal fluid (CF)

chemically induced (CI)

classification (CL)

complications (CO)

diagnosis (DI)

diagnostic imaging (DG)

diet therapy (DH)

drug therapy (DT)

economics (EC)

embryology (EM)

enzymology (EN)

epidemiology (EP)

ethnology (EH)

etiology (ET)

genetics (GE)

history (HI)

immunology (IM)

metabolism (ME)

microbiology (MI)

mortality (MO)

nursing (NU)

parasitology (PS)

pathology (PA)

physiopathology (PP)

prevention & control (PC)

psychology (PX)

radiotherapy (RT)

rehabilitation (RH)

surgery (SU)

therapy (TH)

urine (UR)

veterinary (VE)

virology (VI)

Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
- Genetic Diseases, Inborn [C16.320]
  - Metabolism, Inborn Errors [C16.320.565]
    - Carbohydrate Metabolism, Inborn Errors [C16.320.565.202]
      - Fructose Metabolism, Inborn Errors [C16.320.565.202.251]
        
        Fructose-1,6-Diphosphatase Deficiency [C16.320.565.202.251.221]
        
        Fructose Intolerance [C16.320.565.202.251.271]

Nutritional and Metabolic Diseases [C18]
- Metabolic Diseases [C18.452]
  - Metabolism, Inborn Errors [C18.452.648]
    - Carbohydrate Metabolism, Inborn Errors [C18.452.648.202]
      - Fructose Metabolism, Inborn Errors [C18.452.648.202.251]
        
        Fructose-1,6-Diphosphatase Deficiency [C18.452.648.202.251.221]
        
        Fructose Intolerance [C18.452.648.202.251.271]

Fructose Intolerance Preferred

Concept UI: M0008856
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.
Terms: Fructose Intolerance Preferred Term
Term UI T017036
Date01/01/1999
LexicalTag NON
ThesaurusID; Aldolase B Deficiency
Term UI T811543
Date11/15/2011
LexicalTag NON
ThesaurusID; Fructose Aldolase B Deficiency
Term UI T750973
Date05/12/2009
LexicalTag NON
ThesaurusID; Fructose Intolerance, Hereditary
Term UI T811545
Date11/15/2011
LexicalTag NON
ThesaurusID OMIM (2013); Fructose-1,6-Biphosphate Aldolase Deficiency
Term UI T750972
Date05/12/2009
LexicalTag NON
ThesaurusID; Fructose-1,6-Bisphosphate Aldolase B Deficiency
Term UI T811546
Date11/15/2011
LexicalTag NON
ThesaurusID OMIM (2013); Fructose-1-Phosphate Aldolase Deficiency
Term UI T750974
Date05/12/2009
LexicalTag NON
ThesaurusID; Fructosemia
Term UI T811547
Date11/15/2011
LexicalTag NON
ThesaurusID; Hereditary Fructose Intolerance
Term UI T750971
Date05/12/2009
LexicalTag NON
ThesaurusID; ALDOB Deficiency
Term UI T817356
Date02/06/2012
LexicalTag ABX
ThesaurusID

Fructose Intolerance MeSH Descriptor Data 2025