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hereditary fructose intolerance

 

Definition: a metabolic error due to deficiency of hepatic fructose-1,6-bisphosphate aldolase B (which also acts on fructose 1-phosphate), which is the second enzyme in the specific fructose pathway. Vomiting and hypoglycemia follow intake of fructose; prolonged fructose ingestion in affected young children results in failure to thrive, jaundice, hepatomegaly, albuminuria, aminoaciduria, and sometimes cachexia and death; autosomal recessive inheritance caused by mutation in aldolase B gene (ALDOB) on chromo some 9 q.

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© Copyright 2017 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.
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