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Original Article|Articles in Press

Bone health outcomes in post-lung transplant patients with cystic fibrosis

  • Triet Vincent M. Tran
    Affiliations
    Charles and Jane Pak Center for Mineral Metabolism and Clinical Research, UT Southwestern Medical Center, Dallas, TX, United States of America
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  • Xilong Li
    Affiliations
    Charles and Jane Pak Center for Mineral Metabolism and Clinical Research, UT Southwestern Medical Center, Dallas, TX, United States of America

    Department of Population and Data Sciences, UT Southwestern Medical Center, Dallas, TX, United States of America
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  • Naim M. Maalouf
    Correspondence
    Corresponding author at: 5323 Harry Hines Boulevard, Dallas, Texas 75390-8885, USA.
    Affiliations
    Charles and Jane Pak Center for Mineral Metabolism and Clinical Research, UT Southwestern Medical Center, Dallas, TX, United States of America

    Department of Internal Medicine, UT Southwestern Medical Center, Dallas, TX, United States of America
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Published:January 18, 2023DOI:https://doi.org/10.1016/j.jcf.2023.01.003

      Highlights

      • Data on bone health following lung transplantation in patients with cystic fibrosis is limited.
      • In this study, we compared bone mineral density changes and fracture incidence post-lung transplantation in patients with vs. without cystic fibrosis.
      • Despite lower pre-transplantation BMD, CF patients exhibit better BMD recovery post-transplantation.
      • Post-lung transplantation, fractures occurred in 30% and 34% of CF and non-CF patients, respectively.
      • Lower pre-transplantation BMD and FEV1% are associated with greater fracture incidence post-transplantation.

      Abstract

      Background

      Osteoporosis is a common comorbidity in patients with cystic fibrosis (CF). Although lung transplantation (LTx) improves quality of life of CF patients, there is little research examining long-term bone health outcomes following LTx in these patients.

      Methods

      Data were collected on 59 patients who underwent LTx between 2006 and 2019, including 30 with CF and 29 without CF. We compared baseline characteristics, long-term bone mineral density (BMD) trends, and fracture incidence between the two patient populations, and examined factors associated with post-LTx fractures in CF patients.

      Results

      Compared with non-CF patients, patients with CF were younger, had lower body mass index, and lower baseline BMD Z-scores at the lumbar spine, femoral neck, and total hip (all p<0.001). BMD at all sites declined in both groups in the first year post-LTx. In subsequent years, CF patients exhibited better BMD recovery relative to pre-transplantation, but continued to have lower BMD post-LTx. Post-transplant fractures occurred in 30% and 34% of CF and non-CF patients, respectively. CF patients who developed fractures after LTx had significantly lower BMD and lower pre-transplantation percent predicted forced expiratory volume in one second (FEV1%).

      Conclusions

      Although CF patients exhibit better BMD recovery following LTx compared to their non-CF counterparts, CF patients start with significantly lower pre-LTx BMD and experience a similarly high rate of post-LTx fractures. These findings highlight the unique contribution of the CF disease process to bone health, as well as a clear need for better prevention and treatment of osteoporosis in CF patients before and after LTx.

      Keywords

      Abbreviations:

      BMD (bone mineral density), CF (cystic fibrosis), CFTR (cystic fibrosis transmembrane conductance regulator), DXA (dual-energy x-ray absorptiometry), eGFR (estimated glomerular filtration rate), FEV1 (forced expiratory volume in one second), FEV1% (percent predicted forced expiratory volume in one second), FVC (forced vital capacity), LTx (lung transplantation), PFT (pulmonary function test), 6MWD (6-minute walk distance)
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