Nutritional Goals in Glycogen Storage Disease

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Abstract
Three decades ago the pioneering work of Cori and Cori demonstrated the first hepatic enzyme deficiency in humans, that of glucose-6-phosphatase, and elucidated the biochemical bases of several glycogen storage diseases.1 In the ensuing years, inherited abnormalities of most of the enzymes of glycogen metabolism have been correlated with specific clinical entities. Although the incidence of the glycogen storage diseases is low (≤1 per 100,000 live births for each disorder), an understanding of these disorders has had an important influence on our knowledge of normal metabolism and the biochemical adaptations available to the human organism. Glucose-6-phosphatase deficiency (glycogen storage disease . . .