The Journal of Pediatrics
Volume 154, Issue 4 , Pages 588-591 , April 2009

Vascular Dysfunction in Glycogen Storage Disease Type I

  • Angelina V. Bernier, MD

      Affiliations

    • Division of Pediatric Endocrinology and Glycogen Storage Disease Program, Department of Pediatrics, University of Florida, Gainesville, FL
    • ,
  • Catherine E. Correia, BS

      Affiliations

    • Division of Pediatric Endocrinology and Glycogen Storage Disease Program, Department of Pediatrics, University of Florida, Gainesville, FL
    • ,
  • Michael J. Haller, MD

      Affiliations

    • Division of Pediatric Endocrinology and Glycogen Storage Disease Program, Department of Pediatrics, University of Florida, Gainesville, FL
    • ,
  • Douglas W. Theriaque, MS

      Affiliations

    • General Clinical Research Center, University of Florida, Gainesville, FL
    • Division of Biostatistics, Department of Epidemiology and Health Policy Research, University of Florida, Gainesville, FL
    • ,
  • Jonathan J. Shuster, PhD

      Affiliations

    • General Clinical Research Center, University of Florida, Gainesville, FL
    • Division of Biostatistics, Department of Epidemiology and Health Policy Research, University of Florida, Gainesville, FL
    • ,
  • David A. Weinstein, MD, MMSc

      Affiliations

    • Division of Pediatric Endocrinology and Glycogen Storage Disease Program, Department of Pediatrics, University of Florida, Gainesville, FL
    • Corresponding Author InformationReprint requests: David A. Weinstein, MD, MMSc, Division of Pediatric Endocrinology, University of Florida, PO Box 100296, Gainesville, FL 32610-0296

Received 13 August 2008 ,Revised 2 October 2008 ,Accepted 29 October 2008.

References 

  1. Wolfsdorf JI, Weinstein DA. Glycogen storage diseases. Rev Endocr Metab Disord. 2003;4:95–102
  2. Levy E, Letarte J, Lepage G, Thibault L, Roy CC. Plasma and lipoprotein fatty acid composition in glycogen storage disease type I. Lipids. 1987;22:381–385
  3. Ubels FL, Rake JP, Slaets JP, Smit GP, Smit AJ. Is glycogen storage disease 1a associated with atherosclerosis?. Eur J Pediatr. 2002;161:S62–S64
  4. Lee PJ, Celermajer DS, Robinson J, McCarthy SN, Betteridge DJ, Leonard JV. Hyperlipidaemia does not impair vascular endothelial function in glycogen storage disease type 1a. Atherosclerosis. 1994;110:95–100
  5. Nguyen AD, Pan CJ, Weinstein DA, Chou JY. Increased scavenger receptor class B type 1-mediated cellular cholesterol efflux and antioxidant capacity in the sera of glycogen storage disease type Ia patients. Mol Genet Metab. 2006;89:233–238
  6. Wittenstein B, Klein M, Finckh B, Ullrich K, Kohlschutter A. Plasma antioxidants in pediatric patients with glycogen storage disease, diabetes mellitus, and hypercholesterolemia. Free Radic Biol Med. 2002;33:103–110
  7. Muhlhausen C, Schneppenheim R, Budde U, Merkel M, Muschol N, Ullrich K, et al. Decreased plasma concentration of von Willebrand factor antigen (VWF:Ag) in patients with glycogen storage disease type Ia. J Inherit Metab Dis. 2005;28:945–950
  8. Russo G, Leopold JA, Loscalzo J. Vasoactive substances: nitric oxide and endothelial dysfunction in atherosclerosis. Vasc Pharmacol. 2002;38:259–269
  9. Fathi R, Marwick TH. Noninvasive tests of vascular function and structure: why and how to perform them. Am Heart J. 2001;141:694–703
  10. van Dam MJ, de Groot E, Clee SM, Hovingh GK, Roelants R, Brooks-Wilson A, et al. Association between increased arterial-wall thickness and impairment in ABCA1-driven cholesterol efflux: an observational study. Lancet. 2002;359:37–42
  11. Slyper AH. Clinical review 168: what vascular ultrasound testing has revealed about pediatric atherogenesis, and a potential clinical role for ultrasound in pediatric risk assessment. J Clin Endocrinol Metab. 2004;89:3089–3095
  12. Elpeleg ON. The molecular background of glycogen metabolism disorders. J Pediatr Endocrinol Metab. 1999;12:363–379
  13. Haller MJ, Samyn M, Nichols WW, Brusko T, Wasserfall C, Schwartz RF, et al. Radial artery tonometry demonstrates arterial stiffness in children with type 1 diabetes. Diabetes Care. 2004;27:2911–2917
  14. Lippi G, Montagnana M, Franchini M, Favaloro EJ, Targher G. The paradoxical relationship between serum uric acid and cardiovascular disease. Clin Chim Acta. 2008;392:1–7
  15. McDonald L, Edgill M. Changes in coagulability of the blood during various phases of ischaemic heart-disease. Lancet. 1959;1:1115–1118
  16. Marfaing-Koka A, Wolf M, Boyer-Neumann C, Meyer D, Odievre M, Labrune P. Increased levels of hemostatic proteins are independent of inflammation in glycogen storage disease type Ia. J Pediatr Gastroenterol Nutr. 2003;37:566–570
  17. Fielding CJ, Fielding PE. Cellular cholesterol efflux. Biochem Biophys Acta. 2001;1533:175–189
  18. Gordon T, Castelli WP, Hjortland MC, Kannel WB, Dawber TR. High density lipoprotein as a protective factor against coronary heart disease (The Framingham Study). Am J Med. 1977;62:707–714
  19. Bandsma RH, Prinsen BH, van Der Velden Mde S, Rake JP, Boer T, Smit GP, et al. Increased de novo lipogenesis and delayed conversion of large VLDL into IDL particles contribute to hyperlipidemia in Glycogen Storage Disease type 1a. Pediatr Res. 2008;63:702–707
  20. Shek A, Ferrill MJ. Statin-fibrate combination therapy. Ann Pharmacother. 2001;35:908–917

 Support for this project was provided by the Association for Glycogen Storage Disease, Scott Miller Glycogen Storage Disease Research Fund, the National Center for Research Resources General Clinical Research Center grant M01 RR 00082, National Institutes of Health, and NIH Mentored Career Award K23 RR 017560 (DAW). The authors declare no conflicts of interest.

PII: S0022-3476(08)00957-8

doi: 10.1016/j.jpeds.2008.10.048

The Journal of Pediatrics
Volume 154, Issue 4 , Pages 588-591 , April 2009

Article Tools

* Image Usage & Resolution