TY - JOUR
AU - Herman, Robert H.
AB - Abstract A 2½-year-old girl who fulfilled certain of the diagnostic criteria of "ketotic hypoglycemia" was found to have intolerance to glycerol and fructose. Intestinal and hepatic biopsy tissues were assayed for pyruvate kinase, fructose-1-phosphate aldolase, fructose-1,6-diphosphate aldolase, and fructose-1,6-diphosphatase (FDPase) activities. All enzyme activities were normal except FDPase which was less than normal in both tissues. Since pharmacologic doses of folic acid cause an increase in jejunal and hepatic FDPase, the patient was treated with 30 mg of folic acid per day. Hepatic FDPase activity increased by 60% and tolerance to glycerol, fructose, and alanine improved. Hypoglycemia no longer occurred with the ketosis produced by a ketogenic diet. References 1. Baker L, Winegrad AI: Fasting hypoglycemia and metabolic acidosis associated with deficiency of hepatic fructose-1,6-diphosphatase activity . Lancet 2:13-16, 1970.Crossref 2. David M: Hypoglycemic et acidose metabolique avec deficience en fructose 1-6 diphosphatase . Pediatrie 24:223-225, 1971. 3. Pagliara AS, Karl I, Keating JP, et al: Hepatic fructose-1,6-diphosphatase deficiency: A cause of lactic acidosis and hypoglycemia in infancy , abstracted. Clin Res 19:481, 1971. 4. Greene HL, Stifel FB, Herman RH: Hypoglycemia due to fructose-1,6-diphosphatase deficiency and the treatment of two patients with folate, abstracted. Read before the annual meeting of the Society for Pediatric Research, Washington, DC, 1972. 5. Rosensweig NS, Herman RH, Stifel FB: Dietary regulation of glycolytic enzymes: VI. Effect of dietary sugars and oral folic acid on human jejunal pyruvate kinase, phosphofructokinase and fructosediphosphatase activities . Biochim Biophys Acta 208:373-380, 1970.Crossref 6. Colle E, Ulstrom RA: Ketotic hypoglycemia . J Pediat 64:632-641, 1964.Crossref 7. Sauls HS Jr, Ulstrom RA: Hypoglycemia , in Kelly VC (ed): Brennemans' Practice of Pediatrics . Hagerstown, Md, WF Prior Co Inc, 1965. 8. Rosensweig NS, Stifel FB, Herman RH: The dietary regulation of glycolytic enzymes: II. Adaptive changes in human jejunum . Biochim Biophys Acta 170:228-234, 1968.Crossref 9. Stifel FB, Herman RH, Rosensweig NS: Dietary regulation of glycolytic enzymes: III. Adaptive changes in rat jejunal pyruvate kinase, phosphofructokinase, fructose diphosphatase and glycerol-3-phosphate dehydrogenase . Biochim Biophys Acta 184:29-34, 1969.Crossref 10. Bucher T, Pfleiderer G: Pyruvate kinase from muscle , in Colowick SP, Kaplan NO (eds): Methods in Enzymology . New York, Academic Press Inc, 1955, vol 1, p 435. 11. Spolter PD, Adelman RC, Weinhouse S: Distinctive properties of native and carboxypeptidase-treated aldolases of rabbit muscle and liver . J Biol Chem 240:1327-1337, 1965. 12. Lowry OH, Rosebrough NJ, Farr AL: Protein measurements with Folin's phenol reagent . J Biol Chem 193:265-269, 1951. 13. Rosensweig NS, Herman RH, Stifel FB: Regulation of human jejunal glycolytic enzymes by folic acid . J Clin Invest 48:2038-2045, 1969.Crossref 14. Cornblath M, Schwartz R: Disorders of Carbohydrate Metabolism . Philadelphia, WB Saunders Co, 1966, p 237. 15. Bondy PK: Disorders of carbohydrate metabolism , in Bondy PK, Rosenberg L (eds): Diseases of Metabolism , ed 6. Philadelphia, WB Saunders Co, 1969, p 272. 16. Stifel FB, Herman RH, Rosensweig NS: Dietary regulation of glycolytic enzymes: XI. Effect of inhibitors of protein synthesis on the adaptation of certain jejunal glycolytic and folate-metabolizing enzymes to diet and sex hormones . Biochim Biophys Acta 277:484-489, 1971.Crossref 17. Herman RH, Rosensweig NS: The initiation of protein synthesis . Amer J Clin Nutr 22:806-812, 1969. 18. Pagliara A, Karl I, Devine D, et al: Hypoalaninemia: The cause of ketotic hypoglycemia of childhood , abstracted. J Clin Invest 50:73a, 1971. 19. Koffler H, Schubert WK, Hug G: Sporadic hypoglycemia: Abnormal epinephrine response to the ketogenic diet or to insulin . J Pediat 78:448-453, 1971.Crossref
TI - Ketotic Hypoglycemia Due to Hepatic Fructose-1,6-Diphosphatase Deficiency: Treatment With Folic Acid
JF - American Journal of Diseases of Children
DO - 10.1001/archpedi.1972.02110150113019
DA - 1972-09-01
UR - https://www.deepdyve.com/lp/american-medical-association/ketotic-hypoglycemia-due-to-hepatic-fructose-1-6-diphosphatase-V36CeZBzhY
SP - 415
EP - 418
VL - 124
IS - 3
DP - DeepDyve
ER -