Acceleration of lung disease in children with cystic fibrosis afterPseudomonas aeruginosaacquisition

Authors:
  • MKMichael R. Kosorok
    Michael R. Kosorok
  • LZLan Zeng
    Lan Zeng
  • SWSusan E.H. West
    Susan E.H. West
  • MRMichael J. Rock
    Michael J. Rock
  • MSMark L. Splaingard
    Mark L. Splaingard
  • ALAnita Laxova
    Anita Laxova
  • CGChristopher G. Green
    Christopher G. Green
  • JCJannette Collins
    Jannette Collins
  • PFPhilip M. Farrell
    Philip M. Farrell
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Abstract
As part of the ongoing Wisconsin Cystic Fibrosis (CF) Neonatal Screening Project, we had the unique opportunity to study the longitudinal relationship betweenPseudomonas aeruginosa(Pa) acquisition and infection and developing lung disease in children with CF. The primary objective was to determine whether acquisition ofPawas associated with a measurable change in the progression of lung disease. Two outcome measures were used to study 56 patients who were diagnosed through newborn screening: 1) Wisconsin additive chest radiograph score (WCXR), based on the average of scores from a pulmonologist and a radiologist, and 2) the highest forced expired volume in 1 sec (FEV1)/forced vital capacity (FVC) ratio. We used two measures ofPaacquisition: 1) time of first positive protocol‐determined oropharyngeal (with cough) culture, and 2) the magnitude of antibody titer detected by ELISA assays, using as antigen a crude cell lysate, purified exotoxin A, or an elastase toxoid prepared from threePastrains. Other predictor variables included age, pancreatic status, height‐for age, and weight‐for‐age‐percentiles.The best regression model for predicting changes in the WCXR included time to first positive culture and antibody titer forPaelastase. Prior toPaacquisition, WCXR worsened by 0.45 points/year (P > 0.25); afterPaacquisition, the rate of worsening increased significantly (P < 0.001) to 1.40 points/year. Each antibody titer level (log base 2) increased the score by 0.48 points (P < 0.001). The best regression model for predicting change in the FEV1/FVC included only time to first positive culture. Prior toPaacquisition, the FEV1/FVC ratio declined by 1.29%/year; afterPainfection, the rate of decrease significantly accelerated to 1.81%/year (P = 0.001).Our data show thatPaacquisition is associated with declining pulmonary status in children with CF, and that this effect is probably gradual rather than precipitous. Because these patients were diagnosed and treated aggressively, our estimates of the effects ofPaacquisition may be conservative. We also conclude that the WCXR appears to be more sensitive than FEV1/FVC in detecting early changes in lung disease associated with CF. Pediatr Pulmonol. 2001; 32:277–287.

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