This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Metabolic disorders, Subject name: Pediatrics
[{'content': 'A 6 month child presents with episosdes of vomitting after ingesting fruit juice. Which of following enzyme deficiency is likely ? \nSelect and explain. \nA. Aldolase B, B. Fructokinase, C. Glucose 6 phosphatase, D. Hexokinase', 'role': 'user'}, {'content': 'Answer: A \nExplanation: The condition is hereditary fructose intolerance,an autosomal recessive inborn error of metabolism,due to defect in aldolase-B or fructose-1-phosphate aldolase.Hence,fructose -1-phosphate cannot be metabilised.This is seen when sucrose containing fructose(eg.,fruit juices) is introduced in the diet of infants,usually around 6 months of age.Accumulation of fructose-1-phosphate will inhibit glycogen phosphorylase.This leads to accumulation of glycogen in liver and associated hypoglycemia.Vomiting and loss of appetite are seen.The infants often fail to thrive.Hepatomegaly & jaundice may occur.If liver damage progresses,death will occur.Withdrawal of fructose(fruit juices)from the diet will immediately relieve the symptoms. Reference:Textbook of biochemistry-DM Vasudevan,6 th edition,page no:120.', 'role': 'assistant'}]