Question

An 18 yr old is admitted to the hospital after he passed out in his dorm room. He recovers after intravenous administration of glucose. Medical history reveals that this is a common occurrence for him when he neglects to eat for over 10 hours. The attending physician thinks that the patient has a cellular (storage) glycogen metabolism disorder.

(a) Is the attending physician likely to be correct in her diagnosis? Explain why.

(b) Blood tests reveal that the patient has high levels of liver glucose 1 phosphate, but low levels of blood glucose. Which enzyme is this patient deficient in and why?

Answer 1

1.Physician is likely to he correct as patient has glycogen storage disease, it is a metabolic disorder which is caused by deficiencies of enzymes which affects the glycogen synthesis or glycolysis- glycogen breakdown which keads to low blood sugar level when we are fasting for more than 3 hours.
2. The patient is deficient in glucose pyrophosphorylase and glycogen synthase which forms glucose and also can be deficient in glucagon, is secreted by the α cells of the pancreas in response to a lo w blood sugar level in the fasting state.

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