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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]
Inborn errors of metabolism form a large class of genetic diseases involving congenital disorders of enzyme activities. [1] The majority are due to defects of single genes that code for enzymes that facilitate conversion of various substances into others . In most of the disorders, problems arise due to accumulation of substances which are ...
If fructose is ingested, the enzymatic block at aldolase B causes an accumulation of fructose-1-phosphate which, over time, results in the death of liver cells. [1] This accumulation has downstream effects on gluconeogenesis and regeneration of adenosine triphosphate (ATP). [ 1 ]
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 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
This enzyme deficiency results in an accumulation of fructose-1-phosphate, which inhibits the production of glucose and results in diminished regeneration of adenosine triphosphate. Clinically, patients with hereditary fructose intolerance are much more severely affected than those with essential fructosuria, with elevated uric acid , growth ...
It is symptomatic resulting in severe hypoglycemia, abdominal pain, vomiting, hemorrhage, jaundice, hepatomegaly, and hyperuricemia eventually leading to liver and/or kidney failure and death. The incidence varies throughout the world, but it is estimated at 1:55,000 (range 1:10,000 to 1:100,000) live births.
The activation of HL occurs in two steps. First, HDL that makes its way to the liver, binds to HL thereby removing the heparan sulfate proteoglycan and freeing up the hepatic lipase into the bloodstream, but HL is still inactive due to the proteins on the surface of the lipoprotein. Second, HDL unbinds from HL to activate HL enzymes in the ...