| Descriptor English: | Fructose Intolerance | ||||
| Descriptor Spanish: |
Intolerancia a la Fructosa
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| Descriptor Portuguese: | Intolerância à Frutose | ||||
| Descriptor French: | Intolérance au fructose | ||||
| Entry term(s): |
ALDOB Deficiencies ALDOB Deficiency Aldolase B Deficiencies Aldolase B Deficiency 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 Aldolase B Deficiency Fructose Intolerance, Hereditary Fructose Intolerances Fructose Intolerances, Hereditary Fructose-1,6-Biphosphate Aldolase Deficiencies Fructose-1,6-Biphosphate Aldolase Deficiency Fructose-1,6-Bisphosphate Aldolase B Deficiency Fructose-1-Phosphate Aldolase Deficiencies Fructose-1-Phosphate Aldolase Deficiency Fructosemia Fructosemias Hereditary Fructose Intolerance Hereditary Fructose Intolerances Intolerance, Fructose Intolerances, Fructose |
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| Tree number(s): |
C16.320.565.202.251.271 C18.452.648.202.251.271 |
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| RDF Unique Identifier: | https://id.nlm.nih.gov/mesh/D005633 | ||||
| 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. |
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| Annotation: | an inborn error of fructose metab; do not use /congen & do not coord with INFANT, NEWBORN, DISEASES |
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| Allowable Qualifiers: |
BL blood CF cerebrospinal fluid CI chemically induced CL classification CO complications DG diagnostic imaging DH diet therapy DI diagnosis DT drug therapy EC economics EH ethnology EM embryology EN enzymology EP epidemiology ET etiology GE genetics HI history IM immunology ME metabolism MI microbiology MO mortality NU nursing PA pathology PC prevention & control PP physiopathology PS parasitology PX psychology RH rehabilitation RT radiotherapy SU surgery TH therapy UR urine VE veterinary VI virology |
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| Public MeSH Note: | 1991; see FRUCTOSE METABOLISM, INBORN ERRORS 1989-1990; see CARBOHYDRATE METABOLISM, INBORN ERRORS 1967-1988 |
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| History Note: | 1991(1975); use FRUCTOSE METABOLISM, INBORN ERRORS 1989-1990; use CARBOHYDRATE METABOLISM, INBORN ERRORS 1964-1988 |
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| Related: |
Fructose-Bisphosphate Aldolase
MeSH | ||||
| DeCS ID: | 24002 | ||||
| Unique ID: | D005633 | ||||
| Documents indexed in the Virtual Health Library (VHL): | Click here to access the VHL documents | ||||
| Date Established: | 1991/01/01 | ||||
| Date of Entry: | 1999/01/01 | ||||
| Revision Date: | 2013/07/08 |
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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. |
| Preferred term | Fructose Intolerance |
| Entry term(s) |
ALDOB Deficiencies ALDOB Deficiency Aldolase B Deficiencies Aldolase B Deficiency 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 Aldolase B Deficiency Fructose Intolerance, Hereditary Fructose Intolerances Fructose Intolerances, Hereditary Fructose-1,6-Biphosphate Aldolase Deficiencies Fructose-1,6-Biphosphate Aldolase Deficiency Fructose-1,6-Bisphosphate Aldolase B Deficiency Fructose-1-Phosphate Aldolase Deficiencies Fructose-1-Phosphate Aldolase Deficiency Fructosemia Fructosemias Hereditary Fructose Intolerance Hereditary Fructose Intolerances Intolerance, Fructose Intolerances, Fructose |
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