Abstract：
BACKGROUND: The decline in pulmonary function is a predictor of disease progression in patients with cystic fibrosis (CF). This study aimed to determine the decline rate of percent predicted forced expiratory volume in 1 s (ppFEV1) based on the data of the CF Registry of Turkey. The secondary aim was to investigate the risk factors related to the decline in ppFEV1.
METHODS: A retrospective cohort study of CF patients over 6 years old, with pulmonary function data over at least 2 years of follow-up was extracted from the national CF registry for years 2017-2019. Patients were classified according to disease severity and age groups. Multivariate analysis was used to predict the decline in ppFEV1 and to investigate the associated risk factors.
RESULTS: A total of 1722 pulmonary function test results were available from 574 patients over the study period. Mean diagnostic age was older and weight for age, height for age, and body mass index z scores were significantly lower in the group of ppFEV1 < 40, while chronic Pseudomonas aeruginosa (p < .001) and mucoid P. aeruginosa colonization (p < .001) were significantly higher in this group (p < .001). Overall mean annual ppFEV1 decline was -0.97% (95% confidence interval [CI] = -0.02 to -1.92%). The mean change of ppFEV1 was significantly higher in the group with ppFEV1 ≥ 70 compared with the other (ppFEV1 < 40 and ppFEV1: 40-69) two groups (p = .004). Chronic P. aeruginosa colonization (odds ratio [OR] = 1.79 95% CI = 1.26-2.54; p = .01) and initial ppFEV1 ≥ 70 (OR = 2.98 95% CI = 1.06-8.36), p = .038) were associated with significant ppFEV1 decline in the whole cohort.
CONCLUSIONS: This data analysis recommends close follow-up of patients with normal initial ppFEV1 levels at baseline; advocates for early interventions for P. aeruginosa; and underlines the importance of nutritional interventions to slow down lung disease progression.
METHODS: A retrospective cohort study of CF patients over 6 years old, with pulmonary function data over at least 2 years of follow-up was extracted from the national CF registry for years 2017-2019. Patients were classified according to disease severity and age groups. Multivariate analysis was used to predict the decline in ppFEV1 and to investigate the associated risk factors.
RESULTS: A total of 1722 pulmonary function test results were available from 574 patients over the study period. Mean diagnostic age was older and weight for age, height for age, and body mass index z scores were significantly lower in the group of ppFEV1 < 40, while chronic Pseudomonas aeruginosa (p < .001) and mucoid P. aeruginosa colonization (p < .001) were significantly higher in this group (p < .001). Overall mean annual ppFEV1 decline was -0.97% (95% confidence interval [CI] = -0.02 to -1.92%). The mean change of ppFEV1 was significantly higher in the group with ppFEV1 ≥ 70 compared with the other (ppFEV1 < 40 and ppFEV1: 40-69) two groups (p = .004). Chronic P. aeruginosa colonization (odds ratio [OR] = 1.79 95% CI = 1.26-2.54; p = .01) and initial ppFEV1 ≥ 70 (OR = 2.98 95% CI = 1.06-8.36), p = .038) were associated with significant ppFEV1 decline in the whole cohort.
CONCLUSIONS: This data analysis recommends close follow-up of patients with normal initial ppFEV1 levels at baseline; advocates for early interventions for P. aeruginosa; and underlines the importance of nutritional interventions to slow down lung disease progression.
摘要：
背景：肺功能下降是囊性纤维化（CF）患者疾病进展的预测因子。本研究旨在根据土耳其CF登记处的数据确定1s内预测用力呼气量百分比的下降率（ppFEV1）。次要目的是调查与ppFEV1下降相关的危险因素。
方法：一项6岁以上CF患者的回顾性队列研究，从2017-2019年的国家CF登记处提取至少2年随访期间的肺功能数据.根据疾病严重程度和年龄组对患者进行分类。多因素分析用于预测ppFEV1下降并调查相关危险因素。
结果：在研究期间，574名患者共获得1722例肺功能测试结果。平均诊断年龄较大，体重随年龄变化，身高的年龄，在ppFEV1<40的组中，体重指数z评分明显较低，而慢性铜绿假单胞菌（p<.001）和黏液性铜绿假单胞菌定植（p<.001）则明显较高（p<.001）。ppFEV1总体平均年下降为-0.97%（95%置信区间[CI]=-0.02至-1.92%）。ppFEV1≥70组的ppFEV1的平均变化明显高于其他两组（ppFEV1<40和ppFEV1：40-69）（p=.004）。慢性铜绿假单胞菌定植（比值比[OR]=1.7995％CI=1.26-2.54；p=0.01）和初始ppFEV1≥70（OR=2.9895％CI=1.06-8.36），p=.038）与整个队列中ppFEV1的显着下降有关。
结论：本数据分析建议在基线时对初始ppFEV1水平正常的患者进行密切随访；提倡对铜绿假单胞菌进行早期干预；并强调营养干预对减缓肺部疾病进展的重要性。
方法：一项6岁以上CF患者的回顾性队列研究，从2017-2019年的国家CF登记处提取至少2年随访期间的肺功能数据.根据疾病严重程度和年龄组对患者进行分类。多因素分析用于预测ppFEV1下降并调查相关危险因素。
结果：在研究期间，574名患者共获得1722例肺功能测试结果。平均诊断年龄较大，体重随年龄变化，身高的年龄，在ppFEV1<40的组中，体重指数z评分明显较低，而慢性铜绿假单胞菌（p<.001）和黏液性铜绿假单胞菌定植（p<.001）则明显较高（p<.001）。ppFEV1总体平均年下降为-0.97%（95%置信区间[CI]=-0.02至-1.92%）。ppFEV1≥70组的ppFEV1的平均变化明显高于其他两组（ppFEV1<40和ppFEV1：40-69）（p=.004）。慢性铜绿假单胞菌定植（比值比[OR]=1.7995％CI=1.26-2.54；p=0.01）和初始ppFEV1≥70（OR=2.9895％CI=1.06-8.36），p=.038）与整个队列中ppFEV1的显着下降有关。
结论：本数据分析建议在基线时对初始ppFEV1水平正常的患者进行密切随访；提倡对铜绿假单胞菌进行早期干预；并强调营养干预对减缓肺部疾病进展的重要性。
