<prompt> An infant presents with hepatosplenomegaly, hypoglycemia, hyperuricemia and lactic acidosis. Liver biopsy shows normal structured glycogen deposition. Enzyme deficient in this infant is ?Debranching enzymeBranching enzymeGlucose-6-phosphataseMuscle phosphorylase  <response>: Ans. is c i.e., Glucose-6-phosphataseThe infant is presenting with features of liver glycogenosis, i.e. glycogen storage disease which primarily affects liver.Among the given options a, b and c are liver glycogenoses. but normal glycogen structure rules out a and b options.Debranching enzyme deficiency Type III glycogenosis and branching enzyme deficiency type IV glycogenosis have depostion of abnormal glycogen.Glucose-6-phosphatase deficiency type I glycogenosis has deposition of normal glycogen. 