Serum sex hormone-binding globulin levels are reduced and inversely associated with intrahepatic lipid content and saturated fatty acid fraction in adult patients with glycogen storage disease type 1a

Autor: Simons, P I H G, Valkenburg, O, Telgenkamp, I, van der Waaij, K M, de Groot, D M, Veeraiah, P, Bons, J A P, Derks, T G J, Schalkwijk, C G, Schrauwen-Hinderling, V B, Stehouwer, C D A, Brouwers, M C G J
Přispěvatelé: RS: Carim - V01 Vascular complications of diabetes and metabolic syndrome, Interne Geneeskunde, RS: GROW - R4 - Reproductive and Perinatal Medicine, Obstetrie & Gynaecologie, MUMC+: MA Medische Staf Obstetrie Gynaecologie (9), RS: CAPHRI - R3 - Functioning, Participating and Rehabilitation, Scannexus, RS: NUTRIM - R1 - Obesity, diabetes and cardiovascular health, Beeldvorming, MUMC+: DA CDL Algemeen (9), RS: NUTRIM - R3 - Respiratory & Age-related Health, Nutrition and Movement Sciences, MUMC+: DA BV Research (9), MUMC+: MA Interne Geneeskunde (3), MUMC+: Centrum voor Chronische Zieken (3), MUMC+: HVC Pieken Maastricht Studie (9), MUMC+: MA Endocrinologie (9), Center for Liver, Digestive and Metabolic Diseases (CLDM)
Jazyk: angličtina
Rok vydání: 2022
Předmět:
Zdroj: Journal of Endocrinological Investigation, 45(6), 1227-1234. Official Journal of Italian Society of Endocrinology (SIE)
Journal of endocrinological investigation, 45. SPRINGER
ISSN: 0391-4097
1720-8386
Popis: Purpose De novo lipogenesis has been inversely associated with serum sex hormone-binding globulin (SHBG) levels. However, the directionality of this association has remained uncertain. We, therefore, studied individuals with glycogen storage disease type 1a (GSD1a), who are characterized by a genetic defect in glucose-6-phosphatase resulting in increased rates of de novo lipogenesis, to assess the downstream effect on serum SHBG levels. Methods A case–control study comparing serum SHBG levels in patients with GSD1a (n = 10) and controls matched for age, sex, and BMI (n = 10). Intrahepatic lipid content and saturated fatty acid fraction were quantified by proton magnetic resonance spectroscopy. Results Serum SHBG levels were statistically significantly lower in patients with GSD1a compared to the controls (p = 0.041), while intrahepatic lipid content and intrahepatic saturated fatty acid fraction—a marker of de novo lipogenesis—were significantly higher in patients with GSD1a (p = 0.001 and p = 0.019, respectively). In addition, there was a statistically significant, inverse association of intrahepatic lipid content and saturated fatty acid fraction with serum SHBG levels in patients and controls combined (β: − 0.28, 95% CI: − 0.47;− 0.09 and β: − 0.02, 95% CI: − 0.04;− 0.01, respectively). Conclusion Patients with GSD1a, who are characterized by genetically determined higher rates of de novo lipogenesis, have lower serum SHBG levels than controls.
Databáze: OpenAIRE
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