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Newborn screening alone insufficient to improve pulmonary outcomes for cystic fibrosis

Journal of Cystic Fibrosis Jun 17, 2020

Barreda CB, Farrell PM, Laxova A, et al. - The Wisconsin Cystic Fibrosis Neonatal Screening Project demonstrated improved nutritional outcomes but similar lung disease severity among children receiving an early diagnosis of cystic fibrosis (CF) via newborn screening (NBS) vs those who presented clinically. As evaluations of these individuals by protocol ended in 2012, researchers here examined long-term pulmonary and mortality outcomes. Longitudinal outcome measures retrieved from the Cystic Fibrosis Foundation Patient Registry (CFFPR) were used in the retrospective analysis of the RCT cohort. Among the 145 individuals who consented to the original study, 104 individuals met inclusion criteria and had adequate data in the CFFPR. Per findings, they suggest decline of percent predicted forced expiratory volume in 1 s /year as a valuable index in longitudinal studies of lung disease. They suggest no longer value of survival data in cystic fibrosis cohort comparison studies. Although better nutrition was achieved with NBS, there was no lessening in lung disease severity. This suggests the necessity of performing NBS but indicates it as insufficient in reducing lung disease severity.

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