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Fructose Intolerance

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Definition: 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.  an inborn error of fructose metab; do not use /congen & do not coord with INFANT, NEWBORN, DISEASES   
See Also Fructose-Bisphosphate Aldolase 		Other names Intolerances, Hereditary Fructose; Intolerances, Fructose; Intolerance, Hereditary Fructose; Intolerance, Fructose; Hereditary Fructose Intolerances; Fructose-1-Phosphate Aldolase Deficiencies; Fructose-1,6-Biphosphate Aldolase Deficiencies; Fructose Intolerances, Hereditary; Fructose Intolerances; Fructose Intolerance, Hereditary; Fructose 1,6 Biphosphate Aldolase Deficiency; Fructose 1 Phosphate Aldolase Deficiency; Deficiency, Fructose-1-Phosphate Aldolase; Deficiency, Fructose-1,6-Biphosphate Aldolase; Deficiencies, Fructose-1-Phosphate Aldolase; Deficiencies, Fructose-1,6-Biphosphate Aldolase; Aldolase Deficiency, Fructose-1-Phosphate; Aldolase Deficiency, Fructose-1,6-Biphosphate; Aldolase Deficiencies, Fructose-1-Phosphate; Aldolase Deficiencies, Fructose-1,6-Biphosphate
 
SubstanceCAS Registry & nameCategoriesSource
Aldolase B deficiency  0   *Fructose Intolerance.
acetolactate decarboxylase  EC 4.1.1.5   *Carboxy-Lyases Fructose Intolerance Ketones Lactates. Meth Enzymol 41:526;1975

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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