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. 2014 Jan;11(1):63-72.
doi: 10.1513/AnnalsATS.201309-303OC.

Risk factors for the progression of cystic fibrosis lung disease throughout childhood

Don B Sanders  1 , Zhanhai Li, Anita Laxova, Michael J Rock, Hara Levy, Jannette Collins, Claude Ferec, Philip M Farrell
Affiliations

Risk factors for the progression of cystic fibrosis lung disease throughout childhood

Don B Sanders et al. Ann Am Thorac Soc. 2014 Jan.

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 generalized estimating equation models for repeated measures of chest radiograph scores and pulmonary function tests 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 pulmonary function tests. 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 sex 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.

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Figures

Figure 1.
Figure 1.
Study cohort flowchart. *Sweat test result at least 60 mmol/L and signs/symptoms of cystic fibrosis (CF). PFT = pulmonary function test; WCXR = Wisconsin chest X-ray.
Figure 2.
Figure 2.
(A) Wisconsin chest X-ray (WCXR) scores and (B) FEV1 % predicted for patients with cystic fibrosis transmembrane conductance regulator (CFTR) genotype F508del/F508del (group A), F508del/any class I–III mutation (group B), and any class IV–V mutation (group C). The range of WCXR scores was from 0 to 100, with 0 the best and 100 the worst. Shaded areas indicate 95% confidence intervals.
Figure 3.
Figure 3.
(A) Wisconsin chest X-ray (WCXR) scores and (B) FEV1 % predicted for patients who ever or never had a positive respiratory culture for mucoid Pseudomonas aeruginosa. Shaded areas indicate 95% confidence intervals.
Figure 4.
Figure 4.
(A) Wisconsin chest X-ray (WCXR) scores and (B) FEV1 % predicted for male and female patients. Shaded areas indicate 95% confidence intervals.
Figure 5.
Figure 5.
(A) Wisconsin chest X-ray (WCXR) scores and (B) FEV1 % predicted for patients with body mass index (BMI) less than the 10th percentile for age and patients with BMI equal to or exceeding the 10th percentile for age. Shaded areas indicate 95% confidence intervals.
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References

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