Risk Factors for the Progression of Cystic Fibrosis Lung Disease throughout Childhood.

Sanders DB, Li Z, Laxova A, Rock MJ, Levy H, Collins J, Ferec C, Farrell PM.

Source

University of Wisconsin School of Medicine and Public Health, Pediatrics, American Family Children's Hospital, 600 Highland Avenue, K4/920, Madison, Wisconsin, United States, 53792-9988, 608-265-3425, 608-263-0510 ; dbsanders@pediatrics.wisc.edu.

Abstract

Rationale: Previous studies of risk factors for progression of lung disease in cystic fibrosis (CF) have suffered from limitations that preclude a comprehensive understanding of the determinants of CF lung disease throughout childhood. The epidemiologic component of the 27-year Wisconsin Randomized Clinical Trial of CF Neonatal Screening Project (WI RCT) afforded us a unique opportunity to evaluate the significance of potential intrinsic and extrinsic risk factors for lung disease in children with CF. Objectives: Describe the most important intrinsic and extrinsic risk factors for progression of lung disease in children with CF. Methods: Variables hypothesized at the onset of the WI RCT study to be determinants of the progression of lung disease and potential risk factors previously identified in the WI RCT study were assessed with multivariable GEE models for repeated measures of chest radiograph scores and pulmonary function tests (PFTs) in the WI RCT cohort. Measurements and Main Results: Combining all patients in the WI RCT, 132 subjects were observed for a mean of 16 years and contributed 1,579 chest radiographs, and 1,792 PFTs. The significant determinants of lung disease include genotype, poor growth, hospitalizations, meconium ileus, and infection with mucoid Pseudomonas aeruginosa. The previously described negative effect of female gender was not seen. Conclusions: Modifiable extrinsic risk factors are the major determinants of progression of lung disease in children with CF. Better interventions to prevent or treat these risk factors may lead to improvements in lung health for children with CF.