Question

a 3-year-old girl has been a fussy eater since being weaned, particularly when fruit is part of her diet. she would get cranky, sweat and display dizziness, and lethargy, after eating a meal with fruit. her mother noticed this correlation, and as long as fruit was withdrawn from her diet the child did not display such symptoms. the problems the girl exhibits when eating fruit is most likely due to which one of the following?

Answer 1

The problems the girl exhibits when eating fruit is most likely due to elevated levels of fructose-1-phosphate in liver cells

Define Hereditary fructose intolerance (HFI).

Fruit sugar fructose and its precursors cannot be digested in people with hereditary fructose intolerance (HFI). The accumulation of fructose-1-phosphate in the liver, kidney, and small intestine is caused by a lack of activity of the enzyme fructose-1-phosphate aldolase.

The patient has HFI, which is due to a mutation in aldolase B. Sucrose would still be cleaved by sucrase, thus it would not increase in the stool. Fructose would not be metabolized normally, therefore it would be elevated in the blood and urine. Aldolase B would not cleave fructose 1-phosphate, thus its levels would be elevated and the product, glyceraldehyde, would not be produced.

HFI patients frequently grow to dislike fruit and sweets intensely. After consuming fructose-containing foods, they may experience symptoms like excruciating stomach pain, vomiting, and low blood sugar.

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