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MBBS QUESTION #10352
Question 1
A child with CPT-I (carnitine palmitoyltransferase I) deficiency presents with hypoketotic hypoglycemia during prolonged fasting or intercurrent illness. This clinical picture is best explained by the fact that:
  • Fatty acids cannot enter mitochondria for beta-oxidation, impairing both fatty acid-derived energy production and the hepatic ketogenesis that normally accompanies prolonged fasting✔️
  • Glycogen stores are congenitally entirely absent
  • Insulin secretion becomes pathologically excessive during fasting
  • Glucagon secretion is impaired in this condition
Correct Answer Explanation
Without functional CPT-I, fatty acids cannot be transported into mitochondria for beta-oxidation, depriving the liver of the acetyl-CoA needed both for direct energy production and for hepatic ketogenesis; during fasting, when the body would normally shift to fat oxidation and ketone production to spare glucose, this pathway failure produces dangerous hypoglycemia without the expected compensatory ketosis.