In fructosemia, why would you be hypoglycemic if you couldn't break down fructose. Wouldn't it be hyperglycemi

Question

In fructosemia, why would you be hypoglycemic if you couldn't break down fructose. Wouldn't it be hyperglycemi

Answer 1

Hypoglycemic refers to the symptoms occurring when your cells do not have enough glucose. In fructosemia, the body cannot use the fructose, so it behaves like there is no sugar in the blood

Answer 2

Fructosemia simply means the presence of fructose in the blood.

You may be referring to hereditary fructose intolerance. While some forms are benign, the deficiency of the enzyme1-phosphofructaldolase (fructose 1-phosphate aldolase) can shortly after birth produce lethargy, vomiting, jaundice, enlarged liver and seizures. A deficiency of this enzyme results in the accumulation of fructose 1 phosphate in the hepatocytes which in turn results in the competitive inhibition of phosphorylase, the enzyme that promotes the conversion of glycogen to glucose, This inhibition  results in severe hypoglycemia.

Hope that answers your question.