Jad Reem, Ma Xiayi, Stanojevic Sanja, Illango Abarnaa, Tullis Elizabeth, Gilmour Julie, Goss Christopher H, Strug Lisa J, Stephenson Anne L
Division of Respirology, St. Michael's Hospital, University of Toronto, Toronto, ON M5B 1W8, Canada.
Department of Community Health and Epidemiology, Dalhousie University, Halifax, NS B3H 1V7, Canada.
J Bone Miner Res. 2024 Nov 29;39(12):1716-1721. doi: 10.1093/jbmr/zjae139.
Improved survival in people with cystic fibrosis (pwCF) presents new complexities of care, including CF-related bone disease, a common complication in older pwCF. The trajectory of bone loss with age in this population remains unclear. The objective of this study was to estimate the average rate of change in BMD in adults with CF. This retrospective study included adults with CF, aged 25-48 yr, followed between January 2000 and December 2021. Subjects with at least one DXA scan were included. Scans obtained posttransplantation, after the initiation of bisphosphonates or cystic fibrosis transmembrane conductance regulator modulator therapy was excluded. The primary outcome was BMD (g/cm2) at the LS and FN. A linear mixed-effects model with both random intercept and random slope terms was used to estimate the average annual change in BMD. A total of 1502 DXA scans in 500 adults (average age 28.4 y) were included. There was a statistically significant annual decline in BMD of -0.008 gm/cm2/yr (95% CI, -0.009 to -0.007) at the FN and -0.006 gm/cm2/yr (95% CI, -0.007 to -0.004) at the LS. Relative to BMD at age 25, there was a 18.8% decline at the FN by age 48 yr and a 11% decline at the LS. Pancreatic insufficient subjects had a faster rate of decline in BMD compared with pancreatic sufficient subjects. After adjusting for markers of disease severity, the annual rate of decline remained significant. Individuals with CF experience bone loss at an age when it is not anticipated, thereby entering early adulthood, where further bone loss is inevitable especially with the decrease in estrogen during menopause, with suboptimal BMD. As the CF population ages, it will become very important to consider interventions to maximize bone health.
囊性纤维化患者（pwCF）生存率的提高带来了新的护理复杂性，包括与CF相关的骨病，这是老年pwCF患者的常见并发症。该人群中骨量随年龄的流失轨迹仍不清楚。本研究的目的是估计成年CF患者骨密度（BMD）的平均变化率。这项回顾性研究纳入了2000年1月至2021年12月期间随访的25至48岁成年CF患者。纳入至少进行过一次双能X线吸收法（DXA）扫描的受试者。排除移植后、开始使用双膦酸盐或囊性纤维化跨膜传导调节因子调节剂治疗后获得的扫描结果。主要结局是腰椎（LS）和股骨颈（FN）的骨密度（g/cm²）。使用具有随机截距和随机斜率项的线性混合效应模型来估计骨密度的平均年变化。共纳入了500名成年人（平均年龄28.4岁）的1502次DXA扫描。FN处骨密度的年下降具有统计学意义，为-0.008 g/cm²/年（95%置信区间，-0.009至-0.007），LS处为-0.006 g/cm²/年（95%置信区间，-0.007至-0.004）。相对于25岁时的骨密度，48岁时FN处骨密度下降了18.8%，LS处下降了11%。胰腺功能不全的受试者骨密度下降速度比胰腺功能正常的受试者更快。在调整疾病严重程度指标后，年下降率仍然显著。CF患者在未预期的年龄出现骨质流失，从而进入成年早期，在此期间进一步的骨质流失不可避免，尤其是在绝经期间雌激素减少且骨密度不理想的情况下。随着CF患者群体的老龄化，考虑采取干预措施以最大化骨骼健康将变得非常重要。
