Nocturnal gastric drip feeding in glucose-6-phosphatase deficient children

Six patients with glucose-6-phosphatase deficiency were treated for 12 hr at night with gastric drip feeding (GDF), containing soy milk plus glucose, dextrimaltose, and starch. An alarm system (thermistor), connected to the gastric tube, was devised in case of displacement of the tube by the child. The effects of GDF were as follows. Base deficit and lacticaciduria decreased, but did not normalize. Serum cholesterol levels tended to normalize, but serum triglycerides remained elevated. The hepatic and extrahepatic components of plasma lipoprotein lipase were measured separately 5 and 40 min after iv heparin injection. Hepatic triglyceride lipase activities which were subnormal to normal increased to the lower normal range. Extrahepatic lipoprotein lipase activities, though increasing after GDF, remained abnormally low. Catch-up growth occurred in all four growth-retarded children. The pronounced liver enlargement of the five patients, not previously treated with GDF, decreased markedly.

Nocturnal GDF in glucose-6-phosphatase deficient children, suppresses the tendency for hypoglycemia and acidosis which are causes for stress, catabolism, and anorexia. By suppressing these factors, GDF promotes anabolism and caloric intake, thus accounting for the catch-up growth seen in the patients with growth retardation.