ANS. AGlycogen-storage diseasesTypeDefective enzymeOrgan affectedGlycogen in the affected organClinical featuresI Von GierkeGlucose- 6-phosphataseLiver and kidneyIncreased amount; normal structureMassive enlargement of the liver.Failure to thrive.Severe hypoglycemia, ketosis, hyperuricemia, hyperlipemiaII Pompea-1,4-glucosidase (lysosomal)All organsMassive increase in amount; normal structureCardiorespiratory failure causes death, usually before age 2III CoriAmylo-1,6-glucosidase (debranching enzyme)Muscle and liverIncreased amount; short outer branchesLike type I but milder courseIV AndersenBranching enzyme (a-1, 4 - a-1, 6)Liver and spleenNormal amount; very long outer branchesProgressive cirrhosis of the liver. Liver failure causes death, usually before age 2V McArdlePhosphorylaseMuscleModerately increased amount; normal structureLimited ability to perform strenuous exercise because of painful muscle cramps.Otherwise patient is normal and well developedVI HersPhosphorylaseLiverIncreased amountLike type I, but milder courseVIIPhospho- fructokinaseMuscleIncreased amount; normal structureLike type VVIIIPhosphorylase kinaseLiverIncreased amount; normal structureMild liver enlargement.Mild hypoglycemia# Important points regarding glycogen storage disorders* Most common glycogen storage disorder: Von gierke's disease* Glycogen storage disorder with cardiomegaly:Pompe's* Glycogen storage disorder with liver cirrhosis:Anderson's disease* Burgundy colour urine after exercise:Mcardle disease* Features same as von gierke,but kidneys not enlarged:Cori's* Glycogen storage disorder which is also a lysosomal disorer: Pompe's disease.