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Average rate of lung function decline in adults with cystic fibrosis in the United Kingdom: Data from the UK CF registry
Journal of Cystic Fibrosis ( IF 5.4 ) Pub Date : 2021-01-01 , DOI: 10.1016/j.jcf.2020.04.008
L Caley 1 , L Smith 2 , H White 3 , D G Peckham 4
Affiliation  

BACKGROUND Rate of change in lung function is used as a measure of disease progression and a predictor of mortality in individuals with cystic fibrosis (CF). The aim of this study was to determine the national rate of decline in percent predicted Forced Expiratory Volume in 1 second (ppFEV1) in adults in the UK accounting for age, sex and pancreatic status. METHODS Data on ppFEV1 for adults with CF, excluding those post lung transplantation, was extracted from the UK CF registry between 2015 and 2017. Multilevel modelling was conducted to calculate the annual rate of change in ppFEV1 accounting for age, sex and pancreatic status. RESULTS Overall annual ppFEV1 decline was -1.52% (95% CI: -1.66 to -1.38%) and -0.55% (95% CI: -0.86 to -0.23%) in pancreatic insufficient (PI) and sufficient (PS) adults respectively. In PI individuals, females had a greater rate of decline in ppFEV1. There were differences between age groups. The fastest rate of decline was observed in the 18-28 years group, declining -1.76% (95% CI: -2.06 to -1.46) and -1.61% (95% CI: -1.91 to -1.31) per year in PI females and males respectively. The pattern between the sexes and age categories was more inconsistent in the PS group. CONCLUSIONS The average annual rates of decline in lung function in adults with CF in the UK are similar to reports from other large international cohorts. Pancreatic status has a marked impact on average rate of decline. Younger adults, especially females, have a faster rate of decline and need close monitoring.

中文翻译:

英国囊性纤维化成人肺功能平均下降率:来自英国 CF 登记处的数据

背景肺功能的变化率被用作患有囊性纤维化(CF)的个体的疾病进展的量度和死亡率的预测因子。本研究的目的是确定英国成年人在考虑年龄、性别和胰腺状况的情况下,1 秒内预测用力呼气量 (ppFEV1) 的全国下降率。方法 从 2015 年至 2017 年期间从英国 CF 登记处提取 CF 成人(肺移植后患者除外)的 ppFEV1 数据。进行多级建模以计算 ppFEV1 的年变化率,考虑年龄、性别和胰腺状况。结果 胰腺功能不足 (PI) 和功能充足 (PS) 成人患者的 ppFEV1 总体年下降率分别为 -1.52%(95% CI:-1.66 至 -1.38%)和 -0.55%(95% CI:-0.86 至 -0.23%) . 在 PI 个体中,女性的 ppFEV1 下降速度更快。年龄组之间存在差异。在 18-28 岁组中观察到的下降速度最快,PI 女性每年下降 -1.76%(95% CI:-2.06 至 -1.46)和 -1.61%(95% CI:-1.91 至 -1.31)和男性分别。在 PS 组中,性别和年龄类别之间的模式更加不一致。结论 英国 CF 成人肺功能的平均年下降率与其他大型国际队列的报告相似。胰腺状况对平均下降速度有显着影响。年轻的成年人,尤其是女性,下降速度更快,需要密切监测。06 至 -1.46) 和 -1.61%(95% CI:-1.91 至 -1.31)在 PI 女性和男性中分别为每年。在 PS 组中,性别和年龄类别之间的模式更加不一致。结论 英国 CF 成人肺功能的平均年下降率与其他大型国际队列的报告相似。胰腺状况对平均下降速度有显着影响。年轻的成年人,尤其是女性,下降速度更快,需要密切监测。06 至 -1.46) 和 -1.61%(95% CI:-1.91 至 -1.31)在 PI 女性和男性中分别为每年。在 PS 组中,性别和年龄类别之间的模式更加不一致。结论 英国 CF 成人肺功能的平均年下降率与其他大型国际队列的报告相似。胰腺状况对平均下降速度有显着影响。年轻的成年人,尤其是女性,下降速度更快,需要密切监测。胰腺状况对平均下降速度有显着影响。年轻的成年人,尤其是女性,下降速度更快,需要密切监测。胰腺状况对平均下降速度有显着影响。年轻的成年人,尤其是女性,下降速度更快,需要密切监测。
更新日期:2021-01-01
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