A child presents with massive hepatomegaly and hypoglycemia. There is no improvement in blood glucose on administration of glucagon. The probable diagnosis is -

Ans. is 'a' i.e., Von Gierke disease Liver glycogenoses (Liver glycogen storage disease) Presence of hepatomegaly and hypoglycemia alongwith absence of an increase in blood glucose upon administration of glucagon nuggets the diagnosis of Von Gierke disease. In Von Gierke disease, administration of glucagon or epinephrin results in little or no rise in blood glucose. Mc Ardle disease is a Muscle glycogenoses and does not characteristically present with hepatomegaly and hypoglycemia. Mc Ardle's disease can hence be excluded. Cori and Forbesare liver glycogenoses grouped under glycogen storage disease type III. Although they present with hepatomegaly and hypoglycemia, the administration of glucagon in these conditions provokes a normal increase in blood glucose (Nelson 18th/e 605). The child in question does not show an improvement in hypoglycemia after administration of glucagon and hence is unlikely to have Cori or Forbes type III GSD.