Abstract • 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. (Am J Dis Child 132:241-243, 1978) References 1. Cornblath M, Schwartz R: Disorders of Carbohydrate Metabolism , ed 2. Philadelphia, WB Saunders Co, 1976, p 249. 2. Burr IM, O'Neill JA, Karzon DT: Comparison of the effects of total parenteral nutritions, continuous intragastric feeding . J Pediatr 85:792-795, 1974.Crossref 3. Greene HL, Slonim AE, O'Neill JA, et al: Continuous nocturnal intragastric feeding for management of type I glycogen storage disease . N Engl J Med 294:423-425, 1976.Crossref 4. Senior B, Loridan L: Functional differentiations of glycogenosis of the liver with respect to the use of glycerol . N Engl J Med 279:965-969, 1968.Crossref 5. Hohorst I, Hohorst J: Lactate , in Bergemeyer HU (ed): Methods of Enzymatic Analysis . New York, Academic Press Inc, 1965, p 266. 6. Ivy AC, Shapiro PF, Melnick P: The bleeding tendency in jaundice . Surg Gynecol Obstet 60:781-783, 1935. 7. Hardisty RM, Ingram GIC: Bleeding Disorders: Investigation and Management . London, Blackwell Scientific Publications, 1965. 8. Bowie EJ, Thompson JH, Didishein P, et al: Mayo Clinic Laboratory Manual of Hemostasis . Philadelphia, WB Saunders Co, 1971. 9. Folkman J, Philippart A, Tze WJ, et al: Portacaval shunt for glycogen storage disease: Value of prolonged intravenous hyperalimentation before surgery . Surgery 72:306-314, 1972. 10. Sadeghi-Nejad A, Presente E, Binkiewicz A, et al: Studies in type I glycogenosis of the liver: The genesis and disposition of lactate . J Pediatr 85:49-54, 1974. 11. Czapek EE, Deykin D, Salzman EW: Platelet dysfunction in glycogen storage disease type I . Blood 41:235-247, 1973. 12. Fine RN, Frosier SD, Donnell GN: Growth in glycogen storage disease type I . Am J Dis Child 117:169-177, 1969.
American Journal of Diseases of Children – American Medical Association
Published: Mar 1, 1978