What is Von Gierke's disease?
Glucose 6 phosphatase deficiency in liver, kidneys, intestines
(G6P -> free glucose + Pi), so catalyses the terminal reaction of glycogenolysis and gluconeogenesis
result in impaired glucose export & high levels of G6P in liver & kidney
hypoglycaemia that does not respond to glucagon
symptoms: intervals between feeds increase, infants sleep through night, illness prevents normal feeding routine