The case of a boy affected by type III glycogen storage disease and total GH deficiency is reported. Substitutive treatment with hGH caused an extreme elevation of blood lipids. His lipid profile returned near to basal values 1 month after treatment was discontinued. The association of growth hormone and amylo-1-6-glucosidase deficiencies is unusual and difficult to treat; however growth hormone deficiency should be considered in patients with hepatic glycogenoses and severe growth retardation
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