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What is A child presents with massive hepatomegaly and hypoglycemia. There is no improvement in blood glucose on administration of glucagon. The probable diagnosis is and explain in detail?
Clinical manifestations result, directly or indirectly, from inability to maintain an adequate blood glucose level during the post-absorptive hours of each day organ changes due to glycogen accumulation excessive lactic acid generation damage to tissue from hyperuricemia in GSD Ib, bleeding and infection risk from blood cell effects. Reference: GHAI Essential pediatrics, 8th edition