Nocturnal Intragastric Infusion of Glucose in Management of Defective Gluconeogenesis With Hypoglycemia

Abstract

\s=b\ Three children with defective gluconeogenesis and hypoglycemia were treated with frequent daytime feeding and continuous intragastric infusion of glucose at night. By this technique, the blood glucose level was maintained at or slightly above the physiological range. Secondary lacticacidemia, hyperlipidemia, hyperuricacidemia, and coagulation defects all improved. Weight and height velocity increased dramatically. Strength and sense of well-being improved. Nocturnal intragastric infusion of glucose is now the management of choice for children with defective gluconeogenesis and hypoglycemia.

Glycogen storage disease Type I. Results of treatment with frequent daytime feeding, combined with nocturnal intragastric feeding and with administration of an ?-glucosidase inhibitor

“…There is no doubt that frequent daytime feeding combined with nocturnal intragastric feeding leads to a distinct improve- [2,4,5,6,8,9,12,13]. On the other hand the blood lactate levels and triglycerides remained above the upper range of normal.…”

Effect of Continuous Glucose Therapy Begun in Infancy on the Long‐Term Clinical Course of Patients with Type I Glycogen Storage Disease

“…When hypoglycemia is prevented by providing an appropriate amount of dietary glucose throughout the day and night, the biochemical abnormalities are ameliorated, and growth improves (4‐9). Investigators in several studies have shown that glucose can be supplied continuously by a variety of methods at a rate sufficient to satisfy glucose requirements in the postprandial state: intravenously (5,10), through the gastrointestinal tract by intragastric infusion (by nasogastric or gastrostomy tube) (4‐7,11), or by use of low‐glycemic‐index foods (uncooked cornstarch [UCS] has the most suitable properties described to date) (8,12‐15). Theoretically, the fasting endogenous glucose production rate (16) defines the minimum “appropriate” amount.…”

Clinical and Biochemical Findings Before and After Portacaval Shunt in a Girl with Type Ib Glycogen Storage Disease

“…Recovery was uneventful apart from transitory ketotic hypoglycemia which was corrected by infusion of high amounts of glucose and bicarbonate. Such episodes have been observed after discontinuation of intragastric glucose drips (8,16). The patient was discharged 15 days after the surgical procedure with a regimen of frequent meals, including one night feeding.…”