<prompt> Von Geirkes disease occurs due to deficiency ofGlucose-6-PhosphataseLiver PhosphorylaseMuscle PhosphorylaseDebranching enzyme  <response>: Ans. a Glucose-6-Phosphatase Ref. Harper 28th160; 298; Table 19-2Von Gierke disease type i is a Autosomal recessive disease that occurs due to Glucose-6-phosphatase and characterized by Severe fasting hypoglycemia, glycogen in liver, blood lactate, hepatomegaly. Treatment: frequent oral giucosecornstarch; avoidance of fructose and galactose.Glycogen storage disordersTypeNameEnzyme DeficiencyClinical Features0--Glycogen synthaseHypoglycemia; hyperketonemia; early deathIVon Gierkes diseaseGlucose 6-phosphataseGlycogen accumulation in liver and renal tubule cells; Hyperlipemia, Hyperuricemia Hypoglycemia; Lactic acidemia; ketosis;IIPompes diseaseLysosomal-1-4 and 1-6 glucosidase acid maltaseAccumulation of glycogen in lysosomes: juvenile onset variant, muscle hypotonia, death from heart failure by age 2;Q adult onset variant, muscle dystrophyIIILimit dextrinosis, Forbes or Coris diseaseDebranching enzyme QFasting hypoglycemia; hepatomegalyinfancy; accumulation of characteristic branched polysaccharideIVAmylopectinosis, Andersens diseaseBranching enzymeHepatosplenomegaly;accumulation-of polysaccharide with few branch points; death from heart or liver failure in first year of lifeVMyophosphorylase defici McArdles syndrome0Muscle phosphorylasePoor exercise tolerance; muscle glycogen abnormally high 2.5-4 Q; blood lactate very low after exerciseVIHers diseaseLiver phosphorylaseHepatomegaly; accumulation of glycogen in liver; mild hypoglycemia; generally good prognosisVIITaruis diseaseMuscle and erythrocyte phosphofructokinase 1Poor exercise tolerance; muscle glycogen abnormally high 2.5-4; blood lactate very low after exercise; also hemolytic anemiaVIII Liver phosphorylase kinaseHepatomegaly; accumulation of glycogen in liver; mild hypoglycemia; good prognosisIX Liver and muscle phosphorylase kinaseHepatomegaly; accumulation of glycogen in liver and muscle; mild hypoglycemia; generally good prognosisX cAMP-dependent protein kinase AHepatomegaly; accumulation of glycogen in liver 