<prompt> 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?Von Gierkes diseasePompes diseaseMcArdles diseaseCoris disease  <response>: Type Ia Glycogen Storage Disease-Von Gierkes DiseaseMost common Glycogen Storage Disorder in childhood.Autosomal recessive.The biochemical hallmarks are:HypoglycemiaLactic acidosisHyperlipidemiaHyperuricemiaClinical PresentationMost commonly present at 34 months of age with:Doll like facies with fat cheeksRelatively thin extremitiesShort stature, protuberant abdomenMassive hepatomegalyKidneys are also enlargedNo splenomegalyPlasma may be milky due to associated hypertriglyceridemiaReference- Harpers illustrated biochemistry. 30th edition page no: 179 