A child presents with hepatomegaly and hypoglycemia. There is no improvement in blood sugar even after administration of epinephrine. What is the likely diagnosis -
Von girke's disease
Anderson's disease
Pompe's disease
Mc Ardle's disease
Ans. is 'a' i.e., Von Girke's disease o Amongst the given options only Von Girke's disease presents with hepatomegaly and hypoglycemia. "A diagnosis of type I glycogen storage disease (Von Girke's disease) should be suspected whenever there is hepatomegaly with hypoglycemia that is unresponsive to glucagon or epinephrine".