<prompt> An infant has hepatorenomegaly, hypoglycemia, hyperlipidemia, acidosis and normal structured glycogen deposition in liver. What is the diagnosis ?Hers diseaseVon Gierkes diseaseCoris diseaseAndersons disease  <response>: Ans. is b i.e., Von Gierkes disease Von-Gierke disease Type I glvcogenosis o It is an autosomal recessive disorder. o It is due to absent or deficient activity of glucose-6-phosphatase in Liven Kidney, Intestinal mucosa o It can be divided into two subtypes ? i Type Ia glucose - 6 - phosphatase is defective ii Type Ib Translocase is defective translocase transpos glucose-6-phosphatase across microsomal membrane. 