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Adults with Prader–Willi Syndrome have Weaker Bones: Effect of Treatment with GH and Sex Steroids

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Abstract

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 ± 8.6 years. 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.

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Acknowledgments

We are grateful to the patients with PWS and their families for their willingness to participate in this research. We are indebted with Dr Lan Nguyen for editing the english version of the manuscript.

Conflict of interest

Dr. Longhi, Dr. Grugni, Dr. Gatti, Dr. Spinozzi, Dr. Sartorio, Dr. Adami, Dr. Fanolla, and Dr. Radetti have no conflict of interest.

Human and Animal Rights and Informed Consent

Informed consent was obtained from the subjects, under parental guidance for PWS patients and the investigations had been approved by an institutional human research committee (Ethical Committee of Istituto Auxologico Italiano).

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Authors and Affiliations

  1. Department of Paediatrics, Regional Hospital Bolzano, L. Boehler 5, 39100, Bolzano, Italy

    Silvia Longhi & Giorgio Radetti

  2. Division of Auxology, Istituto Auxologico Italiano, Research Institute, Verbania, Italy

    Graziano Grugni & Alessandro Sartorio

  3. Department of Reumathology, University of Verona, Verona, Italy

    Davide Gatti & Silvano Adami

  4. Department of Radiology, Istituto Auxologico Italiano, Research Institute, Verbania, Italy

    Emiliano Spinozzi

  5. Department of Biostatistics, Regional Hospital Bolzano, Bolzano, Italy

    Antonio Fanolla

Authors
  1. Silvia Longhi
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  2. Graziano Grugni
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  3. Davide Gatti
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  4. Emiliano Spinozzi
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  5. Alessandro Sartorio
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  6. Silvano Adami
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  7. Antonio Fanolla
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  8. Giorgio Radetti
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Corresponding author

Correspondence to Giorgio Radetti.

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Longhi, S., Grugni, G., Gatti, D. et al. Adults with Prader–Willi Syndrome have Weaker Bones: Effect of Treatment with GH and Sex Steroids. Calcif Tissue Int 96, 160–166 (2015). https://doi.org/10.1007/s00223-014-9949-1

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  • DOI: https://doi.org/10.1007/s00223-014-9949-1

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