Journal of Inherited Metabolic Disease

, Volume 32, Supplement 1, pp 27–31

Adiponectin levels correlate with the severity of hypertriglyceridaemia in glycogen storage disease Ia

  • R. H. J. Bandsma
  • G. P. A. Smit
  • D.-J. Reijngoud
  • F. Kuipers
Short Report

DOI: 10.1007/s10545-009-0993-3

Cite this article as:
Bandsma, R.H.J., Smit, G.P.A., Reijngoud, DJ. et al. J Inherit Metab Dis (2009) 32(Suppl 1): 27. doi:10.1007/s10545-009-0993-3
  • 109 Downloads

Summary

Glycogen storage disease type Ia (GSD Ia) is characterized by severe hypercholesterolaemia and hypertriglyceridaemia. Little is known about the aetiology of the hyperlipidaemia in GSD Ia. Adipokines play an important regulatory role in lipid metabolism. We investigated whether adipokine concentrations were correlated with the degree of hyperlipidaemia in GSD Ia patients. Six patients with GSD Ia were studied in semi-fasted conditions. Adiponectin, but not leptin, correlated (r2 = −0.79, p = 0.02) with plasma triglyceride concentrations in the GSD Ia patients. Leptin correlated well with BMI (r2 = 0.59, p < 0.01). However, neither body mass index (BMI) nor homeostasis model assessment (HOMA), as a marker of insulin sensitivity, correlated with triglyceride concentrations. Although a small number of patients were studied, these results indicate that adiponectin concentrations are correlated with the degree of hypertriglyceridaemia in GSD Ia. Pharmacological treatment aimed at increasing adiponectin levels might improve the metabolic status of these patients.

Abbreviations

G6Pase-α

glucose-6-phosphatase

GSD

glycogen storage disease

HOMA

homeostasis model assessment

Copyright information

© Springer Science+Business Media B.V. 2009

Authors and Affiliations

  • R. H. J. Bandsma
    • 1
    • 3
  • G. P. A. Smit
    • 1
  • D.-J. Reijngoud
    • 2
  • F. Kuipers
    • 2
  1. 1.Center for Liver, Digestive and Metabolic Diseases, Department of PediatricsUniversity Medical Center GroningenGroningenThe Netherlands
  2. 2.Center for Liver, Digestive and Metabolic Diseases, Department of Laboratory MedicineUniversity Medical Center GroningenGroningenThe Netherlands
  3. 3.Center for Liver, Digestive and Metabolic DiseasesUniversity Medical Center GroningenGroningenThe Netherlands