Body composition, adipokines, bone mineral density and bone remodeling markers in relation to IGF-1 levels in adults with Prader-Willi syndrome.

Autor: van Nieuwpoort IC; 1Department of Internal Medicine, Section Endocrinology, VU University Medical Center and Amsterdam Neuroscience, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands., Twisk JWR; 2Department of Clinical Epidemiology and Biostatistics and EMGO Institute, VU University Medical Center and Institute of Health Sciences, VU University, Amsterdam, The Netherlands., Curfs LMG; 3Department of Clinical Genetics, Maastricht University Medical Center, Maastricht, The Netherlands., Lips P; 1Department of Internal Medicine, Section Endocrinology, VU University Medical Center and Amsterdam Neuroscience, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands., Drent ML; 1Department of Internal Medicine, Section Endocrinology, VU University Medical Center and Amsterdam Neuroscience, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands.
Jazyk: angličtina
Zdroj: International journal of pediatric endocrinology [Int J Pediatr Endocrinol] 2018; Vol. 2018, pp. 1. Date of Electronic Publication: 2018 Jan 16.
DOI: 10.1186/s13633-018-0055-4
Abstrakt: Background: In patients with Prader-Willi syndrome (PWS) body composition is abnormal and alterations in appetite regulating factors, bone mineral density and insulin-like growth factor-1 (IGF-1) levels have been described. Studies in PWS adults are limited. In this study, we investigated body composition, appetite regulating peptides, bone mineral density and markers of bone remodeling in an adult PWS population. Furthermore, we investigated the association between these different parameters and IGF-1 levels because of the described similarities with growth hormone deficient patients.
Methods: In this cross-sectional observational cohort study in a university hospital setting we studied fifteen adult PWS patients. Anthropometric and metabolic parameters, IGF-1 levels, bone mineral density and bone metabolism were evaluated. The homeostasis model assessment of insulin resistance (HOMA2-IR) was calculated. Fourteen healthy siblings served as a control group for part of the measurements.
Results: In the adult PWS patients, height, fat free mass, IGF-1 and bone mineral content were significantly lower when compared to controls; body mass index (BMI), waist, waist-to-hip ratio and fat mass were higher. There was a high prevalence of osteopenia and osteoporosis in the PWS patients. Also, appetite regulating peptides and bone remodelling markers were aberrant when compared to reference values. Measurements of body composition were significantly correlated to appetite regulating peptides and high-sensitive C-reactive protein (hs-CRP), furthermore HOMA was correlated to BMI and adipokines.
Conclusion: In adults with Prader-Willi syndrome alterations in body composition, adipokines, hs-CRP and bone mineral density were demonstrated but these were not associated with IGF-1 levels. Further investigations are warranted to gain more insight into the exact pathophysiology and the role of these alterations in the metabolic and cardiovascular complications seen in PWS, so these complications can be prevented or treated as early as possible.
Competing Interests: This study was a cross-sectional observational study which was approved by the Medical Ethics Review Committee of the VU University Medical Center and was conducted according to the principles of the Helsinki Declaration.Not applicable.The authors declare that they have no competing interests.Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Databáze: MEDLINE
Externí odkaz:
Nepřihlášeným uživatelům se plný text nezobrazuje