在美国囊性纤维化基金会患者登记中,囊性纤维化护理的空白与肺功能下降有关。

IF 6.8 2区 医学 Q1 RESPIRATORY SYSTEM Annals of the American Thoracic Society Pub Date : 2023-09-01 DOI:10.1513/AnnalsATS.202211-951OC
Edmund H Sears, Alexandra C Hinton, Sara Lopez-Pintado, Christine W Lary, Jonathan B Zuckerman
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引用次数: 2

摘要

理由:囊性纤维化(CF)是一种遗传性疾病,可导致进行性肺功能丧失和早期死亡。许多临床和人口统计学变量与肺功能下降有关,但对长期错过护理的影响知之甚少。目的:确定囊性纤维化基金会患者登记(CFFPR)中遗漏的护理是否与随访时肺功能下降有关。方法:分析2004-2016年确定的CFFPR数据,感兴趣的暴露在CFFPR数据中大于或等于12个月的差距。我们采用纵向半参数模型,采用自然三次样条对年龄(分位数)和受试者特异性随机效应进行建模,对性别和CFTR(囊性纤维化跨膜传导调节因子)基因型、种族和民族进行调整,并纳入时变协变量,如护理差距、保险类型、体重不足体重指数、cf相关糖尿病状态和慢性感染。结果:在cfpr中,1,082,899例遭遇的24,328例个体符合纳入标准。在队列中,8,413(35%)个人至少有一次大于或等于12个月的不连续性发作,而15,915(65%)有持续护理。在间隔12个月之前的接触中,75.8%发生在18岁及以上的患者中。与连续护理的患者相比,有间断护理事件的患者在指标访视时预测1秒用力呼气量的随访百分比较低(-0.81%;95%置信区间,-1.00,-0.61)。这种差异的幅度要大得多(-2.1%;95%可信区间,-1.5,-2.7)。结论:在cfpr中记录的护理中有大于或等于12个月的间隔率,特别是在成人中。cfpr中发现的间断护理与肺功能下降密切相关,特别是在F508del CFTR突变纯合子的青少年和年轻人中。这可能对识别和治疗长时间护理间隔的人有影响,也可能对CFF护理建议有影响。
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Gaps in Cystic Fibrosis Care Are Associated with Reduced Lung Function in the U.S. Cystic Fibrosis Foundation Patient Registry.

Rationale: Cystic fibrosis (CF) is a genetic disease leading to progressive lung function loss and early mortality. Many clinical and demographic variables are associated with lung function decline, but little is known about the effects of prolonged periods of missed care. Objectives: To determine if missed care in the Cystic Fibrosis Foundation Patient Registry (CFFPR) is associated with decreased lung function at follow-up visits. Methods: Deidentified CFFPR data for 2004-2016 were analyzed, with the exposure of interest being ⩾12-month gap in CFFPR data. We modeled percentage predicted forced expiratory volume in 1 second using longitudinal semiparametric modeling with natural cubic splines for age (knots at quantiles) and with subject-specific random effects, adjusted for sex and CFTR (cystic fibrosis transmembrane conductance regulator) genotype, race, and ethnicity and included time-varying covariates for gaps in care, insurance type, underweight body mass index, CF-related diabetes status, and chronic infections. Results: A total of 24,328 individuals with 1,082,899 encounters in the CFFPR met inclusion criteria. In the cohort, 8,413 (35%) individuals had at least a single ⩾12-month episode of discontinuity, whereas 15,915 (65%) had continuous care. Of the encounters preceded by a 12-month gap, 75.8% occurred in patients 18 years and older. Compared with those with continuous care, those with a discontinuous care episode had a lower follow-up percentage predicted forced expiratory volume in 1 second at the index visit (-0.81%; 95% confidence interval, -1.00, -0.61) after adjustment for other variables. The magnitude of this difference was much greater (-2.1%; 95% confidence interval, -1.5, -2.7) in young adult F508del homozygotes. Conclusions: There was a high rate of ⩾12-month gap in care, especially in adults, documented in the CFFPR. Discontinuous care identified in the CFFPR was strongly associated with decreased lung function, especially in adolescents and young adults homozygous for the F508del CFTR mutation. This may have implications for identifying and treating people with lengthy gaps in care and may have implications for CFF care recommendations.

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来源期刊
Annals of the American Thoracic Society
Annals of the American Thoracic Society Medicine-Pulmonary and Respiratory Medicine
CiteScore
9.30
自引率
3.60%
发文量
0
期刊介绍: The Annals of the American Thoracic Society (AnnalsATS) is the official international online journal of the American Thoracic Society. Formerly known as PATS, it provides comprehensive and authoritative coverage of a wide range of topics in adult and pediatric pulmonary medicine, respiratory sleep medicine, and adult medical critical care. As a leading journal in its field, AnnalsATS offers up-to-date and reliable information that is directly applicable to clinical practice. It serves as a valuable resource for clinical specialists, supporting their formative and continuing education. Additionally, the journal is committed to promoting public health by publishing research and articles that contribute to the advancement of knowledge in these fields.
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