[Glycogenosis type I. Therapy (author's transl)].
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.
D002675:Child, Preschool; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000178:diet therapy / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D006801:Humans; D007223:Infant
Glucogenosis tipo I. Actualización del tratamiento.