Child of Vasanthi was weaned from breast milk on the 5th day and was given sugarcane juice the child developed hypoglycemia and hepatomegaly biochemical examination showed hypophosphatemia and enzyme deficiencies–reducing substances in urine. The child is probably suffering from which of the following enzyme deficiencies –
Correct Answer: Aldolase B
Description: HEREDITARY FRUCTOSE INTOLERANCE
Symptoms of hypoglycemia, hepatomegaly develping after injection of excessive fructose intake (sugar cane juice contains the major dietary source of fructose-Sucrose suggest the diagnosis of hereditary fructose intolerance.
Clinical disorders related to fructose metabolism can result from excessive fructose consumption that exceed's the body's ability to efficiently convert the sugar into metabolic intermediates.
In normal persons fructose is metabolized in the following way
Category:
Pediatrics
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