<prompt> An infant has hepatosplenomegaly, hypoglycemia, hyperlipidemia, acidosis and normally structured glycogen deposition in the liver. What is the diagnosisHers diseaseVon Gierkes diseaseCors diseaseAndersons disease  <response>: Von-Gierke disease Type I glycogenosisIt is an autosomal recessive disorder.It is due to absent or deficient activity of glucose-6-phosphatase in Liver, Kidney, Intestinal mucosaIt can be divided into two subtypes -Type la glucose - 6 - phosphatase is defectiveType lb Translocase is defective translocase transports glucose-6-phosphatase across the microsomal membrane.Clinical featuresHypoglycemiaSeizuresDoll-like faces with fat cheeks Thin extremitiesShort StatureProtrubent abdomenHepatomegaly and renontegalyNormal spleen heart sizeBiochemical findingsHypoglycemiaHyperuricemiaHyperlipidemiaLactic acidosis 