Department of Pediatrics, University of California, San Francisco, California.
Department of Epidemiology and Biostatistics, University of California San Francisco, San Francisco, California.
Pediatr Pulmonol. 2017 Dec;52(12):1550-1557. doi: 10.1002/ppul.23884. Epub 2017 Oct 30.
Hispanic patients with cystic fibrosis (CF) have decreased life expectancy compared to non-Hispanic white patients. Pulmonary function is a main predictor of life expectancy in CF. Ethnic differences in pulmonary function in CF have been understudied. The objective was to compare longitudinal pulmonary function between Hispanic and non-Hispanic white patients with CF.
This cohort study of 15 018 6-25 years old patients in the CF Foundation Patient Registry from 2008 to 2013 compared FEV percent predicted and longitudinal change in FEV percent predicted in Hispanic to non-Hispanic white patients. We used linear mixed effects models with patient-specific slopes and intercepts, adjusting for 14 demographic and clinical variables. We did sub-analyses by CFTR class, F508del copies, and PERT use.
Hispanic patients had lower FEV percent predicted (79.9%) compared with non-Hispanic white patients (85.6%); (-5.8%, 95%CI -6.7% to -4.8%, P < 0.001), however, there was no difference in FEV decline over time. Patients on PERT had a larger difference between Hispanic and non-Hispanic white patients in FEV percent predicted than patients not on PERT (-6.0% vs -4.1%, P = 0.02). The ethnic difference in FEV percent predicted was not statistically significant between CFTR classes (Class I-III: -6.1%, Class IV-V: -5.9%, Unclassified: -5.7%, P > 0.05) or between F508del copies (None: -7.6%, Heterozygotes: -5.6%, Homozygotes: -5.3%, P > 0.05).
Disparities in pulmonary function exist in Hispanic patients with CF early in life and then persist without improving or worsening over time. It is valuable to investigate the factors contributing to pulmonary function in Hispanic patients with CF.
与非西班牙裔白人患者相比，患有囊性纤维化 (CF) 的西班牙裔患者的预期寿命较短。肺功能是 CF 患者预期寿命的主要预测指标。CF 中肺功能的种族差异研究不足。本研究的目的是比较 CF 中西班牙裔和非西班牙裔白种人患者的纵向肺功能。
本研究对 2008 年至 2013 年期间在 CF 基金会患者注册中心登记的 15018 名 6-25 岁患者进行了队列研究，比较了西班牙裔和非西班牙裔白种人患者的 FEV%预测值和 FEV%预测值的纵向变化。我们使用具有患者特定斜率和截距的线性混合效应模型，调整了 14 个人口统计学和临床变量。我们根据 CFTR 分类、F508del 拷贝数和 PERT 使用情况进行了亚分析。
与非西班牙裔白种人患者（85.6%）相比，西班牙裔患者的 FEV%预测值较低（79.9%）；（-5.8%，95%CI -6.7%至-4.8%，P<0.001），但在时间上的 FEV 下降没有差异。使用 PERT 的患者的 FEV%预测值在西班牙裔和非西班牙裔白种人患者之间的差异大于未使用 PERT 的患者（-6.0%比-4.1%，P=0.02）。在 CFTR 分类（I-III 类：-6.1%，IV-V 类：-5.9%，未分类：-5.7%，P>0.05）或 F508del 拷贝数（无：-7.6%，杂合子：-5.6%，纯合子：-5.3%，P>0.05）之间，FEV%预测值的种族差异无统计学意义。
CF 中西班牙裔患者在生命早期就存在肺功能差异，且随着时间的推移，这些差异既没有改善也没有恶化。研究导致 CF 中西班牙裔患者肺功能差异的因素具有重要意义。
