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Newborn screening alone insufficient to improve pulmonary outcomes for cystic fibrosis.

仅新生儿筛查不足以改善囊性纤维化的肺部结局。

  • 影响因子:2.88
  • DOI:10.1016/j.jcf.2020.06.002
  • 作者列表:"Barreda CB","Farrell PM","Laxova A","Eickhoff JC","Braun AT","Coller RJ","Rock MJ
  • 发表时间:2020-06-13
Abstract

BACKGROUND:The Wisconsin Cystic Fibrosis Neonatal Screening Project was a randomized clinical trial (RCT) revealing that children receiving an early diagnosis of CF via newborn screening (NBS) had improved nutritional outcomes but similar lung disease severity compared to those who presented clinically. Because the evaluations of these subjects by protocol ended in 2012, our objective was to assess long-term pulmonary and mortality outcomes. METHODS:Retrospective analysis of the RCT cohort utilized longitudinal outcome measures obtained from the Cystic Fibrosis Foundation Patient Registry (CFFPR). Data included screening assignment, clinical characteristics, percent predicted forced expiratory volume in 1 s (ppFEV1) and mortality. A random intercept model was used to compare the ppFEV1 decline of subjects between the two groups up to age 26 years. Mortality was analyzed using the Kaplan-Meier method. RESULTS:Of the 145 subjects who consented to the original study, 104 subjects met inclusion criteria and had adequate data in the CFFPR. Of 57 subjects in the screened group and 47 in the control group, the rates of ppFEV1 decline were 1.76%/year (95% CI 1.62 to 1.91%) and 1.43%/year (95% CI 1.26 to 1.60%), respectively (p<0.0002). Pseudomonas aeruginosa acquired before 2 years was partially responsible. There was no difference in mortality between the two groups. CONCLUSIONS:NBS alone does not improve pulmonary outcomes in CF, particularly when other risk factors supervene. In an era prior to strict infection control and current therapies, NBS for CF may be associated with worse pulmonary outcomes.

摘要

背景: 威斯康星囊性纤维化新生儿筛查项目是一项随机临床试验 (RCT),揭示了通过新生儿筛查 (NBS) 接受 CF 早期诊断的儿童与临床表现相比,营养结局有所改善,但肺部疾病严重程度相似。由于方案对这些受试者的评价于 2012 年结束,我们的目标是评估长期肺部和死亡率结局。 方法: RCT 队列的回顾性分析采用囊性纤维化基金会患者登记 (CFFPR) 获得的纵向结局指标。数据包括筛查分配、临床特征、预测 1 s 用力呼气容积百分比 (ppFEV1) 和死亡率。采用随机截距模型比较两组受试者截至 26 岁的 ppFEV1 下降情况。使用 Kaplan-Meier 方法分析死亡率。 结果: 在同意原始研究的 145 例受试者中,104 例受试者符合纳入标准,在 CFFPR 中有足够的数据。在筛选组的 57 名受试者和对照组的 47 名受试者中,ppFEV1 下降率为 1.76%/年 (95% CI 1.62 ~ 1.91%) 和 1.43%/年 (95% CI 1.26 至 1.60%),分别 (p<0.0002)。2 年前获得的铜绿假单胞菌是部分原因。两组死亡率无差异。 结论: NBS 单用并不能改善 CF 的肺部结局,尤其是当其他危险因素超出时。在严格控制感染和目前治疗之前的时代,CF 的 NBS 可能与较差的肺部结局相关。

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影响因子:4.40
发表时间:2020-01-01
DOI:10.1007/s00262-019-02431-8
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