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Inborn errors of fructose metabolism 

Inborn errors of fructose metabolism
Inborn errors of fructose metabolism

T.M. Cox



Hereditary fructose intolerance (fructosaemia)—increased emphasis on molecular methods for diagnosis, and reasons to avoid fructose/sucrose oral challenges. Expanded notes on prognosis.

Fructose diphosphatase deficiency—expanded discussion of clues to diagnosis.

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date: 23 October 2021

Most people in developed countries ingest 50 to 150 g fructose equivalents daily in their diet and the use of this sugar in food and drinks is increasing globally. Fructose is absorbed rapidly by a carrier mechanism that facilitates transport across the intestinal epithelium, metabolized (mainly in the liver) by the enzymes ketohexokinase (fructokinase), aldolase B, and triokinase, and eventually converted into glucose or glycogen. Dietary sugar has particular effects on those whose capacity to metabolise fructose is limited. Fructose occurs either as a free monosaccharide, as a component of sucrose, a disaccharide from which it is released by digestion; fructose may also be derived from the metabolism of the sugar alcohol, sorbitol....

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