Acceleration of lung disease in children with cystic fibrosis afterPseudomonas aeruginosaacquisition

• DOI: 10.1002/ppul.2009
• Publication Date: 2023-11-19T13:10:49Z
• Authors: Michael R. Kosorok; Lan Zeng; Susan E.H. West; Michael J. Rock; Mark L. Splaingard; Anita Laxova; Christopher G. Green; Jannette Collins; Philip M. Farrell
• Keywords: Vital capacity

Abstract As part of the ongoing Wisconsin Cystic Fibrosis (CF) Neonatal Screening Project, we had unique opportunity to study longitudinal relationship between Pseudomonas aeruginosa ( Pa ) acquisition and infection developing lung disease in children with CF. The primary objective was determine whether associated a measurable change progression disease. Two outcome measures were used 56 patients who diagnosed through newborn screening: 1) additive chest radiograph score (WCXR), based on average scores from pulmonologist radiologist, 2) highest forced expired volume 1 sec (FEV )/forced vital capacity (FVC) ratio. We two acquisition: time first positive protocol‐determined oropharyngeal (with cough) culture, magnitude antibody titer detected by ELISA assays, using as antigen crude cell lysate, purified exotoxin A, or an elastase toxoid prepared three strains. Other predictor variables included age, pancreatic status, height‐for weight‐for‐age‐percentiles. best regression model for predicting changes WCXR culture elastase. Prior acquisition, worsened 0.45 points/year P > 0.25); after rate worsening increased significantly < 0.001) 1.40 points/year. Each level (log base 0.48 points 0.001). FEV /FVC only culture. ratio declined 1.29%/year; infection, decrease accelerated 1.81%/year = Our data show that is declining pulmonary status CF, this effect probably gradual rather than precipitous. Because these treated aggressively, our estimates effects may be conservative. also conclude appears more sensitive detecting early Pediatr Pulmonol. 2001; 32:277–287. © 2001 Wiley‐Liss, Inc.