[Long-term management of glycogenosis type I by nocturnal continuous intragastric glucose infusion].
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Two new patients of glucogenosis type I, fed with a special diet, are described. Various indicated treatments to correct metabolic alterations of the disease were revised (diet, drugs, surgery). Different forms of therapy are compared according with results obtained. Metabolic acidosis and hypoglycemia improved. Growing rate and hyperlypemia did not improved. Uricemic acid levels with alopurinol got normal. Possible implications of this results are discussed.
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