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Children and adolescents with cystic fibrosis display moderate bone microarchitecture abnormalities: data from high-resolution peripheral quantitative computed tomography

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Abstract

Summary

We investigated whether bone microstructure assessed by high-resolution peripheral quantitative tomography (HR-pQCT) could be altered in children and teenagers with cystic fibrosis (CF). In comparison to their healthy counterparts, bone microstructure was mildly affected at the tibial level only.

Introduction

Cystic fibrosis-related bone disease (CFBD) may alter bone health, ultimately predisposing patients to bone fractures. Our aim was to assess bone microstructure using high-resolution peripheral quantitative tomography (HR-pQCT) in a cohort of children and teenagers with CF in comparison to age-, puberty-, and gender-matched healthy volunteers (HVs).

Methods

In this single-center, prospective, cross-sectional study, we evaluated the HR-pQCT bone parameters of CF patients and compared them to those of the healthy volunteers.

Results

At a median age of 15.4 [range, 10.5–17.9] years, 37 CF patients (21 boys) with 91% [range, 46–138%] median forced expiratory volume in 1 s were included. At the ultradistal tibia, CF patients had a smaller bone cross-sectional area (579 [range, 399–1087] mm2) than HVs (655 [range, 445–981] mm2) (p = 0.027), related to a decreased trabecular area, without any significant differences for height. No other differences were found (trabecular number, separation, thickness, or distribution) at the radial or tibial levels. Bone structure was different in patients receiving ursodeoxycholic acid and those bearing two F508del mutations.

Conclusion

In our cohort of children and teenagers with good nutritional and lung function status, bone microstructure evaluated with HR-pQCT was not severely affected. Minimal microstructure abnormalities observed at the tibial level may be related to the cystic fibrosis transmembrane conductance regulator defect alone; the long-term consequences of such impairment will require further evaluation.

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Acknowledgements

The authors would like to thank the patients and families for their enthusiastic and active participation in this study. We also thank Marie Perceval for setting up and managing the database and Véronique Delaup for managing the patients and families.

Author information

Authors and Affiliations

  1. Pediatric Cystic Fibrosis Center, Hospices Civils de Lyon, Lyon, France

    C. Braun & P. Reix

  2. University Claude-Bernard Lyon 1, Lyon, France

    C. Braun, J. Bacchetta, P. Braillon, R. Chapurlat & P. Reix

  3. Centre de Ressources et de Compétences de la Mucoviscidose, Hôpital Femme Mère Enfant, 69677, Bron, France

    C. Braun

  4. INSERM UMR 1033, Lyon, France

    J. Bacchetta & R. Chapurlat

  5. Rare Renal Diseases Reference Center Néphrogones, Hospices Civils de Lyon, Lyon, France

    J. Bacchetta

  6. Pediatric Radiology Department, Hospices Civils de Lyon, Lyon, France

    P. Braillon & R. Chapurlat

  7. Rheumatology Department, Hospices Civils de Lyon, Lyon, France

    J. Drai

  8. Biochemistry Department, Hospices Civils de Lyon, Lyon, France

    P. Reix

  9. UMR 5558 (EMET). CNRS, LBBE, Université de Lyon, Villeurbanne, France

    P. Reix

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  1. C. Braun
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Corresponding author

Correspondence to C. Braun.

Ethics declarations

The study was approved by the local institutional review board (Comité de Protection des Personnes Lyon Sud Est II, #2012-029-2). Written informed consent was obtained from all parents and patients before enrollment.

Funding

Institutional funding for this study was provided by l’Appel D’Offre Jeune Chercheur des Hospices Civils de Lyon (P. Reix, 2011). Institutional funding for the VITADOS cohort was provided by the Programme Hospitalier de Recherche Clinique (PHRC) Interrégional (J. Bacchetta, 2011).

Conflicts of interest

None.

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Braun, C., Bacchetta, J., Braillon, P. et al. Children and adolescents with cystic fibrosis display moderate bone microarchitecture abnormalities: data from high-resolution peripheral quantitative computed tomography. Osteoporos Int 28, 3179–3188 (2017). https://doi.org/10.1007/s00198-017-4179-9

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  • DOI: https://doi.org/10.1007/s00198-017-4179-9

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