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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; Fructose Aldolase B Deficiency; Fructose-1,6-Biphosphate Aldolase Deficiency; Fructose-1-Phosphate Aldolase Deficiency; Hereditary Fructose Intolerance; Aldolase Deficiencies, Fructose-1,6-Biphosphate; Aldolase Deficiencies, Fructose-1-Phosphate; Aldolase Deficiency, Fructose-1,6-Biphosphate; Aldolase Deficiency, Fructose-1-Phosphate; Deficiencies, Fructose-1,6-Biphosphate Aldolase; Deficiencies, Fructose-1-Phosphate Aldolase; Deficiency, Fructose-1,6-Biphosphate Aldolase; Deficiency, Fructose-1-Phosphate Aldolase; Fructose 1 Phosphate Aldolase Deficiency; Fructose 1,6 Biphosphate Aldolase Deficiency; Fructose Intolerance, Hereditary; Fructose Intolerances; Fructose Intolerances, Hereditary; Fructose-1,6-Biphosphate Aldolase Deficiencies; Fructose-1-Phosphate Aldolase Deficiencies; Hereditary Fructose Intolerances; Intolerance, Hereditary Fructose; Intolerances, Fructose; Intolerances, Hereditary Fructose
Networked: 195 relevant articles (1 outcomes, 14 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Galactosemias (Galactosemia)
2. Abdominal Pain (Pain, Abdominal)
3. Congenital Disorders of Glycosylation
4. Parasitic Diseases (Parasitic Disease)
5. Renal Insufficiency (Renal Failure)

Experts

1. Tolan, Dean R: 5 articles (03/2015 - 12/2002)
2. Salvatore, Francesco: 3 articles (12/2010 - 11/2002)
3. Vitagliano, Luigi: 3 articles (12/2010 - 11/2002)
4. Zagari, Adriana: 3 articles (12/2010 - 11/2002)
5. Esposito, Gabriella: 3 articles (12/2010 - 11/2002)
6. Mavridou, Irene: 2 articles (10/2012 - 03/2009)
7. Moraitou, Marina: 2 articles (10/2012 - 03/2009)
8. Pollak, Agnieszka: 2 articles (10/2012 - 04/2006)
9. Dimitriou, Evangelia: 2 articles (10/2012 - 03/2009)
10. Michelakakis, Helen: 2 articles (10/2012 - 03/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Fructose Intolerance:
1. CarbohydratesIBA
2. Fructose-Bisphosphate Aldolase (Aldolase)IBA
3. FructoseIBA
4. Glucose (Dextrose)FDA LinkGeneric
5. Uric Acid (Urate)IBA
6. FructosuriaIBA
7. HydrogenIBA
8. Aldehyde-LyasesIBA
9. Immunoglobulins (Immunoglobulin)IBA
10. Genetic Markers (Genetic Marker)IBA

Therapies and Procedures

1. Diet Therapy (Therapy, Diet)
2. Nutrition Therapy (Medical Nutrition Therapy)
3. Protein-Restricted Diet (Diet, Protein Restricted)
4. Minor Surgical Procedures (Minor Surgery)
5. Nephrectomy