<prompt> G-6- Phosphatase deficiency is seen in: PGI Dec 2006Von Gierkes diseaseTaysachs diseasePompes diseaseAndersons disease  <response>: Arts: A Von Gierkes disease Von GierkesType I glycogen strorage disease Inheritance Deficient enzyme Organ affected Structure of glycogen Clinical features Autosomal recessiveQ G-6 phosphataseQ - Liver- Intestine- Kidney NormalQ But metabolically not available - HypoglycemiaQ- HyperlipidemiaQ- Ketosis acidosis-- - 2 Lactic acid- 2 Uric acidQ- HepatosplenogalyQTable Harper 28thl 60 : Glycogen Storage diseasesGlycogenosisNameCause of DisorderCharacteristicsType 0-Glycogen synthaseHyperglycemia, Hyperketonemia; Early deathType IVon Giekes diseseQDeficiency of glucose-6- phosphataseLiver cells and renal tubule cells loaded with glycogen. Hypoglycemia, lacticacidemia, ketosis, hyperlipemia.Type IIPompes diseaseDeficiency of lysosomal a-l - 4 and l-6 - glucosidase add malt- ageFatal accumulation of glycogen in lysosomes, heart failure.Type IIILimit dextrinosis., forbes or coris diseaseAbsence of debranching ezymeAccumulation of a characteristic branched polysachha- rideType IVAmyiopectinosis, Andersens diseaseAbsence of branching enzymeAccumulation of a polysaccharide having few branch points. Death due to cardiac or liver failure in first year of life.Type VMyophophorylase deficiency. McArdles syndromeAbsence of musde phosphorylaseDiminshed exercise tolerance; muscles have abnormally high glycogen content 2.5- 4.1. Little or no lactate in blood after exercise.Type VIHers diseaseDeficiency of phosphofructokinase in muscle and erythrocytesAs for type V but also possibility of hemolytic anemiaType VIIITaruis diseaseDeficiency of liver phophorylase kinaseAs for type ViIX Liver Musde Phosphorylase kinaseHepatomegaly; accumulation of glycogen in liver muscle; mild hypog; ycemia; generally good prognosisX C-AMP dependent protein kinaseHepatomegaly; accumulation of glycogen in liver 