Clinical Affairs, Cystic Fibrosis Foundation, Bethesda, MD.
Clinical Affairs, Cystic Fibrosis Foundation, Bethesda, MD.
Ann Epidemiol. 2023 Oct;86:98-103.e5. doi: 10.1016/j.annepidem.2023.07.008. Epub 2023 Aug 6.
Deaths among those lost to follow-up (LTFU) in the Cystic Fibrosis Foundation Patient Registry (CFFPR) are critically important to the epidemiology of cystic fibrosis (CF). Unreported deaths could impact estimates of survival if LTFU is associated with disease trajectory.
We characterized the LTFU population (1986-2017) from the CFFPR and identified deaths via linkage with the National Death Index (NDI). Median predicted survival age and conditional survival were estimated with and without additional deaths and person-time from the NDI.
Of the 10,582 individuals LTFU in the CFFPR, 2,460 (23.2%) matched to an NDI death record. Individuals who died after LTFU with a CF diagnosis were 43% female, 91% White/non-Hispanic, 59% had advanced CF lung disease based on last CFFPR recorded forced expiratory volume in one second (FEV) %predicted <40 and 18% were post-lung transplant. Median predicted survival age during the most recent period available, 2013-2017, increased from 44.3 years (95% CI: 43.2, 45.7) to 45.8 years (95% CI 44.5, 47.1) with the inclusion of NDI data.
Inclusion of deaths and additional person-time among those LTFU changed the point estimate of median predicted survival for most time periods and increased the point estimate from 2009 onwards.
在囊性纤维化基金会患者注册中心（CFFPR）中，随访丢失（LTFU）人群的死亡情况对囊性纤维化（CF）的流行病学至关重要。如果 LTFU 与疾病轨迹相关，未报告的死亡可能会影响生存估计。
我们从 CFFPR 中描述了 LTFU 人群（1986-2017 年），并通过与国家死亡指数（NDI）的链接确定了死亡情况。在没有来自 NDI 的额外死亡和人时的情况下，估计了带有和不带有额外死亡和人时的中位预测生存年龄和条件生存。
在 CFFPR 中 LTFU 的 10582 人中，有 2460 人（23.2%）与 NDI 死亡记录相匹配。在 LTFU 后死亡且患有 CF 诊断的个体中，43%为女性，91%为白种人/非西班牙裔，59%的个体最后一次 CFFPR 记录的用力呼气量占预计值的百分比（FEV）%预测值<40%，18%为肺移植后。在最近可用的时间段，即 2013-2017 年，中位预测生存年龄从 44.3 岁（95%CI：43.2，45.7）增加到 45.8 岁（95%CI：44.5，47.1），包括了 NDI 数据。
纳入 LTFU 人群中的死亡和额外的人时数据改变了中位预测生存的点估计，并且从 2009 年开始增加了点估计。
