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Fructose Intolerance (Intolerance, Fructose)

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.
Also Known As:
Intolerance, Fructose; 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; Intolerances, Fructose
Networked: 295 relevant articles (4 outcomes, 17 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Galactosemias (Galactosemia)
2. Gastrointestinal Diseases (Functional Gastrointestinal Disorders)
3. Pain (Aches)
4. Abdominal Pain (Pain, Abdominal)
5. Congenital Disorders of Glycosylation

Experts

1. Tolan, Dean R: 8 articles (05/2020 - 12/2002)
2. Brouwers, Martijn C G J: 4 articles (01/2022 - 11/2019)
3. Debray, François-Guillaume: 4 articles (01/2021 - 01/2018)
4. Esposito, Gabriella: 4 articles (10/2019 - 11/2002)
5. Michelakakis, Helen: 3 articles (01/2022 - 03/2009)
6. Moraitou, Marina: 3 articles (01/2022 - 03/2009)
7. Tran, Christel: 3 articles (01/2021 - 04/2017)
8. Schalkwijk, Casper G: 3 articles (06/2020 - 11/2019)
9. Stehouwer, Coen D A: 3 articles (06/2020 - 11/2019)
10. Parenti, Giancarlo: 3 articles (10/2019 - 12/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Fructose Intolerance:
1. FructoseIBA
2. LactoseFDA LinkGeneric
3. CarbohydratesIBA
4. Fructose-Bisphosphate Aldolase (Aldolase)IBA
5. SugarsIBA
6. EnzymesIBA
7. Phosphates (Orthophosphate)IBA
8. FructosuriaIBA
9. Uric Acid (Urate)IBA
10. HydrogenIBA

Therapies and Procedures

1. Diet Therapy (Therapy, Diet)
2. Therapeutics
3. Electronic Nose
4. Intravenous Administration
5. Gluten-Free Diet