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 Gierke's disease
Anderson's disease
Pompe's disease
Mc Ardle's disease
Epinephrine- | blood glucose level by stimulating glycogenolysis and gluconeogenesis, mediated by glucose 6 phosphatase- conves Glucose-6-phosphate to Glucose Von Gierke disease- deficiency of Glucose 6 phosphatase enzyme Hence, epinephrine is not able to increase blood glucose in a child with Von Gierke disease, but lactate levels increase.
Disorders of Carbohydrate Metabolism