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Adiponectin levels correlate with the severity of hypertriglyceridaemia in glycogen storage disease Ia
Author(s) -
Bandsma R. H. J.,
Smit G. P. A.,
Reijngoud D.J.,
Kuipers F.
Publication year - 2009
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/s10545-009-0993-3
Subject(s) - adiponectin , medicine , adipokine , endocrinology , triglyceride , leptin , body mass index , metabolic syndrome , insulin , insulin resistance , obesity , cholesterol
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 ( r 2 = −0.79, p = 0.02) with plasma triglyceride concentrations in the GSD Ia patients. Leptin correlated well with BMI ( r 2 = 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.