This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: None, Subject name: Biochemistry
[{'content': "A 10 month old male child patient comes with vomiting, lethargy and severe jaundice  when weaning was started with fruit juice. Laboratory diagnosis reveals prolonged  clotting time, hypoalbuminemia, elevation of bilirubin and transaminase levels. What  is the diagnosis? \nSelect and explain. \nA. Von Gierke's disease, B. Hereditary fructose intolerance, C. Benign fructosuria, D. Lactose intolerance", 'role': 'user'}, {'content': 'Answer: B \nExplanation: HEREDITARY FRUCTOSE INTOLERANCE (HFI):\nIt is an autosomal recessive condition.\nBiochemical defect\n\nDeficiency of fructose – 1,6 – bisphosphate Aldolase (Aldolase B).\nThe gene for aldolase B is on chromosome 9q22.3.\nDeficiency of this enzyme activity causes a rapid accumulation of fructose-1-phosphate and initiates severe toxic symptoms when exposed to fructose.\n\nClinical manifestations\n\nPatients with HFI are asymptomatic, until fructose or sucrose (table sugar) is ingested (usually from fruit, fruit juice\xa0or sweetened cereal).\nSymptoms may occur early in life, soon after birth if food or formulas containing these sugars are introduced into the diet.\nEarly clinical manifestations resemble galactosemia and include jaundice, hepatomegaly, vomiting, lethargy, irritability and convulsions, hypoglycemia.\nAcute fructose ingestion produces symptomatic hypoglycemia. If the intake of the fructose persists, hypoglycemic episodes recur\xa0and liver and kidney failure progress, eventually leading to death.\nChronic ingestion results in failure to thrive.\n\nLaboratory diagnosis\n\nProlonged\xa0clotting time, hypoalbuminemia, elevation of bilirubin and transaminase levels.\nProximal tubular dysfunction.\nDefinitive diagnosis is made by assay of fructaldolase B activity in the liver.\nGene-based diagnosis for the mutation.\n\nTreatment\n\nComplete exclusion of all sources of fructose.\n\nKey Concept:\nDeficiency of fructose – 1,6 – bisphosphate Aldolase (Aldolase B) is the cause of Hereditary fructose intolerance.\nReference- Harper’s illustrated biochemistry. 30th edition page -205', 'role': 'assistant'}]