caa a22 4500 605521573 CHVBK 20210128100741.0 cr unu---uuuuu 210128e20150201xx s 000 0 eng 10.1007/s00223-014-9949-1 doi (NATIONALLICENCE)springer-10.1007/s00223-014-9949-1 Adults with Prader-Willi Syndrome have Weaker Bones: Effect of Treatment with GH and Sex Steroids [Elektronische Daten] [Silvia Longhi, Graziano Grugni, Davide Gatti, Emiliano Spinozzi, Alessandro Sartorio, Silvano Adami, Antonio Fanolla, Giorgio Radetti] Obesity has been considered to have a protective effect against the risk of fractures in adults. However, a high frequency of fracture is described in obese adults with Prader-Willi syndrome. To evaluate bone geometry, density and strength in a group of adult obese patients with Prader-Willi syndrome (PWS) and to examine the modulating effect on bone of treatment with growth hormone (GH) and sex steroids. This was a cross-sectional study performed in 41 (17 males, 24 females) obese subjects with genetically confirmed PWS, aged 29.4±8.6years. Forty-six healthy subjects (22 males and 24 females) served as controls. Digitalized X-rays were evaluated at the level of the 2nd metacarpal bone to assess bone geometry, i.e. cross-sectional area (CSA), cortical area (CA), medullary area (MA), metacarpal index (MI) and bone strength evaluated as bending breaking resistance index (BBRI). DEXA was also used to evaluate body composition and bone mineral density (total body, lumbar spine and femoral neck). PWS subjects, after adjusting for height and bone size, had a reduced CSA, CA and BBRI, while bone density was not different. GH treatment had a positive effect and sex steroids a negative effect on bone size and strength. PWS subjects showed a reduced bone size at the metacarpus leading to a reduced strength, while bone density was appropriate for size. GH treatment improves bone geometry but not bone density. Bone strength was significantly reduced in PWS patients who did not receive GH and had been treated with sex steroids. Springer Science+Business Media New York, 2015 Prader−Willi syndrome nationallicence Obesity nationallicence Bone geometry nationallicence Bone density nationallicence Longhi Silvia Department of Paediatrics, Regional Hospital Bolzano, L. Boehler 5, 39100, Bolzano, Italy aut Grugni Graziano Division of Auxology, Istituto Auxologico Italiano, Research Institute, Verbania, Italy aut Gatti Davide Department of Reumathology, University of Verona, Verona, Italy aut Spinozzi Emiliano Department of Radiology, Istituto Auxologico Italiano, Research Institute, Verbania, Italy aut Sartorio Alessandro Division of Auxology, Istituto Auxologico Italiano, Research Institute, Verbania, Italy aut Adami Silvano Department of Reumathology, University of Verona, Verona, Italy aut Fanolla Antonio Department of Biostatistics, Regional Hospital Bolzano, Bolzano, Italy aut Radetti Giorgio Department of Paediatrics, Regional Hospital Bolzano, L. Boehler 5, 39100, Bolzano, Italy aut Calcified Tissue International Springer US; http://www.springer-ny.com 96/2(2015-02-01), 160-166 0171-967X 96:2<160 2015 96 223 https://doi.org/10.1007/s00223-014-9949-1 text/html Onlinezugriff via DOI BK010053 XK010053 XK010000 Metadata rights reserved Springer special CC-BY-NC licence nationallicence 1 research-article jats NATIONALLICENCE NATIONALLICENCE NL-springer NATIONALLICENCE 856 40 https://doi.org/10.1007/s00223-014-9949-1 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Longhi Silvia Department of Paediatrics, Regional Hospital Bolzano, L. Boehler 5, 39100, Bolzano, Italy aut NATIONALLICENCE 700 1- Grugni Graziano Division of Auxology, Istituto Auxologico Italiano, Research Institute, Verbania, Italy aut NATIONALLICENCE 700 1- Gatti Davide Department of Reumathology, University of Verona, Verona, Italy aut NATIONALLICENCE 700 1- Spinozzi Emiliano Department of Radiology, Istituto Auxologico Italiano, Research Institute, Verbania, Italy aut NATIONALLICENCE 700 1- Sartorio Alessandro Division of Auxology, Istituto Auxologico Italiano, Research Institute, Verbania, Italy aut NATIONALLICENCE 700 1- Adami Silvano Department of Reumathology, University of Verona, Verona, Italy aut NATIONALLICENCE 700 1- Fanolla Antonio Department of Biostatistics, Regional Hospital Bolzano, Bolzano, Italy aut NATIONALLICENCE 700 1- Radetti Giorgio Department of Paediatrics, Regional Hospital Bolzano, L. Boehler 5, 39100, Bolzano, Italy aut NATIONALLICENCE 773 0- Calcified Tissue International Springer US; http://www.springer-ny.com 96/2(2015-02-01), 160-166 0171-967X 96:2<160 2015 96 223