A 5-month old baby presents to emergency with history of vomiting, irritability & jaundice. The baby was previously healthy. History of introducing fruit juices into the diet one week back. USG revealed hepatomegaly and liver function test were abnormal. The infants most likely has deficiency of which of the following enzymes?
Correct Answer: Aldolase B
Description: b. Aldolase B(Ref: Nelson 20/e p 720-28)The given history of symptoms, on introduction of fruit juices, including hepatic dysfunction, points to a diagnosis of Hereditary fructose intolerance, which is due to deficiency of Aldolase B.
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Pediatrics
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