Continuous glucose for treatment of patients with type 1 glycogen-storage disease: comparison of the effects of dextrose and uncooked cornstarch on biochemical variables

Article Abstract:

In type 1 glycogen-storage disease (GSD-1), glucose production in the liver is severely impaired by a lack of activity of the liver enzyme glucose-6-phosphatase (a required enzyme for glucose synthesis). This impairment results in hypoglycemia (low blood glucose) if glucose intake is not maintained. Consequently, a constant source of dietary glucose is required to maintain normal blood glucose levels. The effects of uncooked corn starch (UCS), dextrose (Dex), or continuous overnight glucose feeding (COG) on blood glucose and insulin levels were determined in seven children with GSD-1. Administration of Dex, one milligram per kilogram of body weight, caused blood glucose and insulin to reach maximum levels within 45 to 60 minutes, and to decrease to hypoglycemic levels by 180 minutes. In contrast, UCS caused blood glucose and insulin levels to remain constant over the four-hour test period. Overnight, blood glucose and insulin levels remained constant during COG feeding by gastrostomy tube or intermittent UCS feeding. UCS appeared to act as an intestinal reservoir of glucose. The results demonstrate that providing a continuous source of glucose by COG or UCS can maintain blood glucose levels within the normal range. (Consumer Summary produced by Reliance Medical Information, Inc.)

Dextrose

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Efficacy of cornstarch therapy in type III glycogen-storage disease

Article Abstract:

Glycogen-storage disease type III (GSD-III) is a metabolic disorder characterized by a decrease in the enzyme amylo-1,6 glucosidase, which alters the structure of glycogen (a glucose compound). Patients with GSD-III develop hypoglycemia, an abnormally low level of glucose, because of their inability to release glucose from glycogen, which is stored in the liver. Symptoms of this disorder include impaired liver function, growth failure, and striated muscle disease. Continuous feeding with cornstarch at night through a tube inserted into the intestinal tract was shown to enhance growth and improve metabolic abnormalities associated with GSD-III. The effects of cornstarch given by mouth have not been previously assessed. The effectiveness of initial treatment with oral cornstarch was examined in three patients with GSD-III who showed growth failure, increased levels of the enzyme aminotransferase, and hypoglycemia. Initial treatment with cornstarch was associated with normal blood levels of glucose, an increased rate of growth, and decreased levels of aminotransferase. These findings suggest that initial treatment with oral cornstarch is effective in improving symptoms of patients with GSD-III. (Consumer Summary produced by Reliance Medical Information, Inc.)

Physiological aspects, Dosage and administration, Growth, Growth (Physiology)

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Physical growth and development of children with type 1 glycogen-storage disease: comparison of the effects of long-term use of dextrose and uncooked cornstarch

Article Abstract:

Continuous administration of glucose has been shown to correct the biochemical abnormalities resulting from type 1 glycogen-storage disease (GSD-1). Children with GSD-1 experienced clinical improvement and increased growth following a combination of continuous glucose infusion, daily feedings of dextrose and a healthy diet. Thirteen patients with GSD-1 were studied to determine the biochemical and growth effects of a two-year therapy program using uncooked cornstarch (UCS) or continuous overnight glucose (COG), supplemented with daily dextrose feeding. Over the two-year test period, both therapy programs produced height and weight increases similar to those of normal children of the same age. In addition, blood glucose and serum insulin levels were maintained within the normal range. The results indicate that intermittent feedings of UCS at four-hour intervals during the day and five-hour intervals at night provide a satisfactory substitute for the continuous administration of glucose. (Consumer Summary produced by Reliance Medical Information, Inc.)

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