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The key identifying feature of HFI is the appearance of symptoms with the introduction of fructose to the diet. [ 5 ] [ 6 ] Affected individuals are asymptomatic and healthy, provided they do not ingest foods containing fructose or any of its common precursors, sucrose and sorbitol .
Hereditary fructose intolerance (HFI) results in poor feeding, failure to thrive, chronic liver disease and chronic kidney disease, and death. HFI is caused by a deficiency of fructose 1,6-biphosphate aldolase in the liver, kidney cortex and small intestine. Infants and adults are asymptomatic unless they ingest fructose or sucrose. [citation ...
Fructose malabsorption, formerly named dietary fructose intolerance (DFI), is a digestive disorder [1] in which absorption of fructose is impaired by deficient fructose carriers in the small intestine's enterocytes. This results in an increased concentration of fructose. Intolerance to fructose was first identified and reported in 1956. [2]
The lack of two important enzymes in fructose metabolism results in the development of two inborn errors in carbohydrate metabolism – essential fructosuria and hereditary fructose intolerance. In addition, reduced phosphorylation potential within hepatocytes can occur with intravenous infusion of fructose.
The amount of fructose routinely lost in urine is quite small. [6] Other errors in fructose metabolism have greater clinical significance. Hereditary fructose intolerance, or the presence of fructose in the blood (fructosemia), is caused by a deficiency of aldolase B, the second enzyme involved in the metabolism of fructose. [citation needed]
Fructose intolerance may refer to: Fructose malabsorption , a digestive disorder of the small intestine in which the fructose carrier in enterocytes is deficient Hereditary fructose intolerance , a hereditary condition caused by a deficiency of liver enzymes that metabolise fructose
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