Disorders of Fructose and Galactose Metabolism — MCQs

Which amino acids accumulate in maple syrup urine disease?

History of dislike for sweet food items is typically present in:

Which enzyme is deficient in Galactosemia?

An 8-month-old infant is brought in with poor feeding, lethargy, hypotonia, and hepatomegaly. Labs reveal hypoglycemia and metabolic acidosis. Which condition is most likely?

A cataract formation in both eyes was discovered in a 1-year-old child during a routine well-child visit, with blood tests showing elevated galactose and galactitol levels. To determine which enzyme might be defective in the child, which intracellular metabolite should be measured?

Which carbohydrate is primarily metabolized by Aldolase-B?

What are the immediate metabolic products formed during the conversion of Fructose 1,6-bisphosphate to two molecules of pyruvate?

Fructose intolerance is due to deficiency of which enzyme?

Most Common enzyme deficient in galactosemics:

Congenital lactic acidosis is primarily due to a defect in which enzyme?