2 MCQs in Biochemistry for NEET PG
A 9-month-old infant presents with severe hypoglycemia, vomiting, and hepatomegaly after introduction of fruit juice. Genetic testing confirms autosomal recessive deficiency of the enzyme marked **B** in the fructose metabolism diagram. Which of the following best explains the pathophysiology of this infant's acute presentation?
A 2-week-old neonate born to consanguineous parents presents with vomiting, jaundice, hepatomegaly, and failure to thrive after initiation of milk feeding. Laboratory investigations show elevated galactose-1-phosphate levels and reduced enzyme activity. Newborn screening confirms deficiency of the enzyme marked **B** in the galactose metabolism pathway diagram. Which of the following is the most appropriate long-term management for this patient?
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