Cornstarch Therapy in Type I Glycogen-Storage Disease

TYPE I glycogen-storage disease, an inherited absence or deficiency of glucose-6-phosphatase activity in the liver, kidney, and intestines, is associated with the accumulation of glycogen in those organs. The lack of glucose-6-phosphatase activity in the liver rapidly leads to hypoglycemia in fasting, because of the inadequate release of glucose from glucose-6-phosphate through normal glycogenolysis and gluconeogenesis. Other clinical manifestations include growth retardation, hepatomegaly, lactic acidosis, hyperuricemia, and hyperlipidemia. Most of these abnormalities have been attributed to the hypoglycemia, since treatments that maintain normal glucose concentrations correct most of the metabolic abnormalities and growth retardation. So far, the various kinds of . . .