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Review and Summary

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Review and Summary
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Molecular Basis of G-6-PD Deficiency
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General Features (Glycogen Storage Diseases)
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        Similar to Sphingolipidoses

              1.     Diseases of Degradation (all except one)

              2.     Accumulation of Glycogen

                     a.     abnormal branching (too many, too little)
                     b.     normal amount (too much, too little)

              3.     Liver Enlargement (Hepatomegaly)

              4.     Fasting Hypoglycemia

              5.     Treatment requires continual glucose infusions (IV)

								
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