title: Adiponectin levels correlate with the severity of hypertriglyceridaemia in glycogen storage disease Ia.
abstract: 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.
authors.affiliation: Center for Liver, Digestive and Metabolic Diseases, Department of Pediatrics, University Medical Center Groningen, Groningen, The Netherlands. rbandsma@yahoo.com
authors.forename: R H J
authors.initials: RH
authors.lastname: Bandsma
authors.forename: G P A
authors.initials: GP
authors.lastname: Smit
authors.forename: D-J
authors.initials: DJ
authors.lastname: Reijngoud
authors.forename: F
authors.initials: F
authors.lastname: Kuipers
mesh_terms: D052242:Adiponectin / Q000097:blood*; D000293:Adolescent; D000328:Adult; D015992:Body Mass Index; D016022:Case-Control Studies; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000097:blood* / Q000150:complications / Q000473:pathology; D006801:Humans; D015228:Hypertriglyceridemia / Q000097:blood* / Q000188:drug therapy / Q000209:etiology; D007333:Insulin Resistance; D020738:Leptin / Q000097:blood; D008297:Male; D012720:Severity of Illness Index; D045162:Thiazolidinediones / Q000494:pharmacology; D014280:Triglycerides / Q000097:blood; D055815:Young Adult
chemical_list: C499918:ADIPOQ protein, human; D052242:Adiponectin; D020738:Leptin; D045162:Thiazolidinediones; D014280:Triglycerides