Hereditary fructose intolerance
Definition: An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 126.96.36.199) 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.
Synonyms (terms occurring on more labels are shown first): fructose intolerance
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