Abstract
Von Gierke’s disease is the most common form (Type I) of glycogen storage disease, resulting from the deficiency of the enzyme glucose-6- phosphatase. When poorly controlled, glycogen storage disease Type I (GSD-I) is characterized by hypoglycemia, metabolic acidosis, hyperlipidemia, failure to thrive and hepatomegaly (1). Attempts to maintain blood glucose homeostasis by frequent daytime meal coupled with nocturnal intragastric feeding (NF) has afforded a vastly improved control of clinical and biochemical parameters (2,3). Very limited information is available concerning the hyperlipidemia and lipoprotein profile of GSDI patients (4,5). In this symposium, we report our analysis of detailed plasma lipid and lipoprotein composition following chronic NF therapy. We have assessed the postheparin hepatic and lipoprotein lipase activities to examine the catabolism of triglycerides in these patients. In view of the known relationship between certain forms of hyperlipoproteinemia and atherosclerosis, special attention is focused on the potential risk factors for premature cardiovascular disease in GSD-I.
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References
R.R. Howell. The glycogen storage diseases. In The Metabolic Basis of Inherited Disease. J.B. Stanbury, J.B. Wyngaarden and D.S. Frederickson, editors. McGraw-Hill, New York, N.Y. (1978).
H.L. Greene, A.F. Slonim, J.A. O’Neill, and I.B. Burr. Continuous nocturnal feeding for management of type I glycogen storage disease. N. Engl. J. Med. 294:423 (1976).
H.L. Greene, A.E. Slonim, I.M. Burr, and J.R. Moran. Type I glycogen storage disease: five years of management with nocturnal intragastric feeding. J. Pediatr. 96:590 (1980).
J. Fernandes and N.A. Pikaar. Hyperlipemia in children with liver glycogen disease. Am. J. Clin. Nutr. 22:617 (1969).
S. Jacovcic, A.K. Khachadurian, and D.J.J. Hsia. The hyperlipidemia in glycogen storage disease. J. Lab. Clin. Med. 68:769 (1966).
E. Levy, L.A. Thibault, C.C. Roy, M. Bendayan, G. Lepage, and J. Letarte. Circulating lipids and lipoproteins in glycogen storage disease type I with nocturnal intragastric feeding. J. Lipid Res. (1987) in press.
O.H. Lowry, N.J. Rosebrough, A.L. Farr, and R.J. Randall. Protein measurement with the folin phenol reagent. J. Biol. Chem. 193:265 (1951).
G.R. Bartlett. Phosphorus assay in column chromatography. J. Biol. Chem. 234:466 (1959).
M.F. Lopez-Virella, P. Stone, S. Ellis, and J.A. Colwell. Cholesterol determination in high density lipoprotein separated by three different methods. Clin. Chem. 23:882 (1977).
B. Schriewer, W. Nolte, and G. Assmann. VLDL apolipoprotein B determination in blood serum following precipitation of LDL with polyvinylsulphate. J. Clin. Chem. Clin. Biochem. 23:349 (1985).
G. Lepage, and C.C. Roy. Direct transesterification of all classes of lipids in a one-step reaction. J. Lipid Res. 27:114 (1986).
C.A. Stanley, J.L. Mills, and L. Baker. Intragastric feeding in type I glycogen storage disease: factors affecting the control of lactic acidemia. Pediatr. Res. 15:1504 (1981).
J. Fernandes, H. Jansen, and T.C. Jansen. Nocturnal gastric drip feeding in glucose-6-phosphatase deficiency children. Pediatr. Res. 13:225 (1979).
H. Blackburn. Conference on the health effects of blood lipids: optimal distributions for populations. Prev. Med. 8:612 (1979).
T. Pearson. Coronary arteriography in the study of the epidemiology of coronary heart disease. Epidemiol. Rev. 6:140, 1984.
P.O. Kwiterowich. Biochemical, clinical, epidemiologic, genetic, and pathologic data in the pediatric age group relevant to the cholesterol hypothesis.
E. Levy, J. Letarte, G. Lepage, L. Thibault, and C.C. Roy. Plasma and lipoprotein fatty acid composition in glycogen storage disease type I. Lipids 22:381 (1987).
Recommanded Dietary Allowances. In National Academy of Science/National Research Council. Washington, D.C., p. 178 (1980).
W.C. Hülsmann, W.H.M. Eijebenboom, and J. Fernandes. Glucose-6-phosphatase deficiency and hyperlipemia. Clin. Chim. Acta 36:775 (1970).
A. Sadeghi-Nejad, E. Presente, A. Bienkiewicz, and B. Senior. Studies in type I glycogenesis of the liver. The genesis and disposition of lactate. J. Pediatr. 85:49 (1974).
A. Jonas, S.A. Sweeny, and P.N. Herbert. Discoidal complexes of A and C apolipoproteins with lipids and their reactions with lecithin-cholesterol acyltransferase. J. Biol. Chem. 259:6359 (1984).
G. Bengtsson, T. Olivercrona. Lipoprotein lipase: some effects of activator proteins. Eur. J. Biochem. 106:549 (1980).
A.J. Vergroesen. In Detection and Treatment of Lipid and Lipoprotein Disorders of Childhood. Alan R. Liss, New York, pp 161 (1985).
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© 1988 Plenum Press, New York
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Levy, E., Seidman, E., Letarte, J., Thibault, L., Lepage, G., Roy, C.C. (1988). Hyperlipoproteinemia in Glycogen Storage Disease Type I: Effect of Nocturnal Intragastric Feeding. In: Salvayre, R., Douste-Blazy, L., Gatt, S. (eds) Lipid Storage Disorders. NATO ASI Series, vol 150. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-1029-7_93
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DOI: https://doi.org/10.1007/978-1-4613-1029-7_93
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