- •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.
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.
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.
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%).
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.
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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- 2019 Annual data report.Cystic Fibrosis Foundation, Bethesda, Maryland2020
- Structure, gating, and regulation of the CFTR anion channel.Physiol Rev. 2019; 99: 707-738
- Cystic fibrosis.Nat Rev Dis Primers. 2015; 1: 15010
- Changing epidemiology and clinical issues arising in an ageing cystic fibrosis population.Ther Adv Respir Dis. 2011; 5: 105-119
- The prevalence of osteoporosis, osteopenia, and fractures among adults with cystic fibrosis: a systematic literature review with meta-analysis.Calcif Tissue Int. 2010; 86: 1-7
- Abnormal bone turnover in cystic fibrosis adults.Osteoporos Int. 2002; 13: 151-157
- The impact of dysglycaemia on bone mineral accrual in young people with cystic fibrosis.Clin Endocrinol (Oxf). 2013; 78: 36-42
- Estrogen, androgen, and the pathogenesis of bone fragility in women and men.Curr Osteoporos Rep. 2004; 2: 90-96
- Prevalence and correlates of vertebral fractures in adults with cystic fibrosis.Bone. 2004; 35: 771-776
- Osteoclast function, bone turnover and inflammatory cytokines during infective exacerbations of cystic fibrosis.J Cyst Fibros. 2010; 9: 93-98
- BMD and body composition in children and young patients affected by cystic fibrosis.J Bone Miner Res. 2006; 21: 388-396
- Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in human bone.Thorax. 2007; 62: 650-651
- Cystic fibrosis and bone disease: defective osteoblast maturation with the F508del mutation in cystic fibrosis transmembrane conductance regulator.Am J Respir Crit Care Med. 2014; 189: 746-748
- Osteoporosis after solid organ transplantation.J Clin Endocrinol Metab. 2005; 90: 2456-2465
- Osteoporosis in lung transplantation candidates with end-stage pulmonary disease.Am J Med. 1996; 101: 262-269
- Bone involvement in young adults with cystic fibrosis awaiting lung transplantation for end-stage respiratory failure.Osteoporos Int. 2019; 30: 1255-1263
- Efficacy of pamidronate for osteoporosis in patients with cystic fibrosis following lung transplantation.Am J Respir Crit Care Med. 2000; 162 (Pt 1): 941-946
- Bone density in heart or lung transplant recipients–a longitudinal study.Transplant Proc. 2013; 45: 2357-2365
- Executive summary of the 2019 ISCD position development conference on monitoring treatment, DXA cross-calibration and least significant change, spinal cord injury, peri-prosthetic and orthopedic bone health, transgender medicine, and pediatrics.J Clin Densitom. 2019; 22: 453-471
- Long-term bone mineral density changes and fractures in lung transplant recipients with cystic fibrosis.J Cyst Fibros. 2021; 20: 525-532
- Persistent osteopenia in adult cystic fibrosis transmembrane conductance regulator-deficient mice.Am J Respir Crit Care Med. 2008; 177: 309-315
- The F508del mutation in cystic fibrosis transmembrane conductance regulator gene impacts bone formation.Am J Pathol. 2012; 180: 2068-2075
- A roadmap to the brittle bones of cystic fibrosis.J Osteoporos. 2010; 2011926045
- Low bone mineral density in adults with cystic fibrosis.Thorax. 1999; 54: 961-967
- Impact of bone-active drugs and underlying disease on bone health after lung transplantation: a longitudinal study.J Cyst Fibros. 2021; 20: e100-e1e7
- The association of nephrolithiasis with cystic fibrosis.Am J Kidney Dis. 2003; 42: 1-11
- Urinary stone disease prevalence and associations in cystic fibrosis.Urolithiasis. 2021;
- Cystic fibrosis bone disease treatment: current knowledge and future directions.J Cyst Fibros. 2019; 18 (Suppl): S56-S65
- The effects of ivacaftor on bone density and microarchitecture in children and adults with cystic fibrosis.J Clin Endocrinol Metab. 2021; 106: e1248-e1e61
- Bone mineral density and fat-soluble vitamin status in adults with cystic fibrosis undergoing lung transplantation: a pilot study.Can J Diet Pract Res. 2016; 77: 199-202
Published online: January 18, 2023
Accepted: January 11, 2023
Received in revised form: December 22, 2022
Received: June 26, 2022
Publication stageIn Press Corrected Proof
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