<prompt> A 5 year old boy presents with hepatomegaly, hypoglycaemia ketosis. The diagnosis isMucopolysaccharidosisGlycogen storage disorderLipopolysaccharidosisDiabetes mellitus  <response>: Patients with type I GSD may present in the neonatal period with hypoglycemia and lactic acidosis.These children often have doll-like faces with fat cheeks, relatively thin extremities, short stature and a protuberant abdomen that is due to massive epatomegaly; the kidneys are also enlarged, whereas the spleen and heart are normal.The biochemical hallmarks of the Type Ia GSD von Gierkes disease are hypoglycemia, lactic acidosis, hyperuricemia and hyperlipidemia. 