A 12 year old boy rapidly develops hypoglycemia after moderate activity. On physical examination, kidney and liver are found to be enlarged. Histopathology of liver shows deposits of glycogen in an excess amount. Blood examination reveals raised ketone bodies, lactic acid and triglycerides. What is the diagnosis?
Type Ia Glycogen Storage Disease-Von Gierke’s Disease

Most common Glycogen Storage Disorder in childhood.
Autosomal recessive.

The biochemical hallmarks are:

Hypoglycemia
Lactic acidosis
Hyperlipidemia
Hyperuricemia

Clinical Presentation
Most commonly present at 3–4 months of age with:

Doll like facies with fat cheeks
Relatively thin extremities
Short stature, protuberant abdomen
Massive hepatomegaly
Kidneys are also enlarged
No splenomegaly
Plasma may be milky due to associated hypertriglyceridemia

Reference- Harper’s illustrated biochemistry. 30th edition page no: 179
Von Gierke's disease
Pompe's disease
McArdle's disease
Cori's disease