About The Medical Condition Intolerances, Fructose
Find the definition of the medical term Intolerances, Fructose. Intolerances, Fructose defined and explained for easy understanding at the Medical Conditions from ClusterMed.info.
Intolerances, Fructose
| Intolerances, Fructose Medical Condition |
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What's The Definition Of The Medical Condition Intolerances, 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.
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