Hyperlipoproteinemia in Glycogen Storage Disease Type I: Effect of Nocturnal Intragastric Feeding

  • E. Levy
  • E. Seidman
  • J. Letarte
  • L. Thibault
  • G. Lepage
  • C. C. Roy
Conference paper
Part of the NATO ASI Series book series (NSSA, volume 150)

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.

Keywords

Cholesterol Phosphorus Phenol Carbohydrate Lactate 

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Copyright information

© Plenum Press, New York 1988

Authors and Affiliations

  • E. Levy
    • 1
  • E. Seidman
    • 1
  • J. Letarte
    • 1
  • L. Thibault
    • 1
  • G. Lepage
    • 1
  • C. C. Roy
    • 1
  1. 1.Centre de Recherche, Hôpital Sainte-Justine Departments of Pediatrics, Nutrition and GastroenterologyUniversité de MontréalQuébecCanada

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