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1.  Risk Factors for the Progression of Cystic Fibrosis Lung Disease throughout Childhood 
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.
doi:10.1513/AnnalsATS.201309-303OC
PMCID: PMC3972988  PMID: 24261460
pulmonary function test; chest X-ray; newborn screening; nutrition; sex
2.  Regional Differences in the Evolution of Lung Disease in Children with Cystic Fibrosis 
Pediatric Pulmonology  2011;47(7):635-640.
SUMMARY
Progression of lung disease is a major event in children with cystic fibrosis (CF), but regional differences in its evolution are unclear. We hypothesized that regional differences occur beginning in early childhood. We examined this issue by evaluating 132 patients followed in the Wisconsin Neonatal Screening Project between 1985 and 2010. We scored chest x-rays obtained every 1–2 years with the Wisconsin chest x-ray system, in which we divided the lungs into quadrants, and gave special attention to ratings for bronchiectasis (BX) and nodular/branching opacities. We compared the upper and lower quadrant scores, and upper right and left quadrant scores, as patients aged using a multivariable generalized estimation equation (GEE) model. We did a confirmatory analysis for a subset of 81 patients with chest computerized tomography (CT) images obtained in 2000 and scored using the Brody scoring system. The chest x-ray analysis shows that the upper quadrants have higher BX (p<0.001) and nodular/branching opacities (p<0.001) scores than the lower quadrants. CT analysis likewise reveals that the upper quadrants have more BX (p=0.02). Patients positive for mucoid PA showed significantly higher BX scores than patients with nonmucoid PA (p= 0.001). Chest x-ray scoring also revealed that the upper right quadrant has more BX (p< 0.001) than the upper left quadrant, and CT analysis was again confirmatory (p< 0.001). We conclude that pediatric patients with CF develop more severe lung disease in the upper lobes than the lower lobes in association with mucoid PA infections and also have more severe lung disease on the right side than on the left side in the upper quadrants. A variety of potential explanations such as aspiration episodes may be clinically relevant and provide insights regarding therapies.
doi:10.1002/ppul.21604
PMCID: PMC3310260  PMID: 22162514
cystic fibrosis; lung disease; bronchiectasis; upper lobes; mucoid Pseudomonas aeruginosa
3.  The Sensitivity of Lung Disease Surrogates in Detecting Chest CT Abnormalities in Children with Cystic Fibrosis 
Pediatric Pulmonology  2011;47(6):567-573.
Rationale
Chest CT scans detect structural abnormalities in children with cystic fibrosis (CF), even when pulmonary function tests (PFTs) are normal. The use of chest CT is limited in clinical practice, because of concerns over expense, increased resource utilization, and radiation exposure. Quantitative chest radiography scores are useful in detecting mild lung disease, but whether they are sensitive to the presence of CT scan abnormalities has not been evaluated.
Objective
To determine in a cross-sectional study if quantitative chest radiography is a more sensitive marker of chest CT abnormalities than other lung disease surrogates.
Methods
Brody chest CT scores were calculated for 81 children enrolled in the Wisconsin CF Neonatal Screening Project. We determined the sensitivity for Wisconsin (WCXR) and Brasfield (BCXR) chest radiography scores, PFTs, positive cultures for P. aeruginosa (PA), and parental report of symptoms to detect a Brody score worse than the median score for study participants.
Measurements and Main Results
The mean FEV1 for the study population was 91% predicted. Abnormal WCXR and BCXR scores had the highest sensitivity to detect a chest CT score worse than the median; abnormal PFTs, parental report of symptoms, and the presence of PA had much lower sensitivity (p<0.001).
Conclusions
In this cross sectional study, quantitative chest radiography has excellent sensitivity to detect an abnormal chest CT and may have a role in monitoring lung disease progression in children with CF.
doi:10.1002/ppul.21621
PMCID: PMC3309112  PMID: 22170734
Chest x-ray; quantitative radiography; pulmonary function
4.  COMPARING AGE OF CYSTIC FIBROSIS DIAGNOSIS AND TREATMENT INITIATION AFTER NEWBORN SCREENING WITH TWO COMMON STRATEGIES 
Journal of Cystic Fibrosis  2011;11(2):150-153.
Background
Newborn screening (NBS) for CF has become widespread, although there are multiple strategies. Little is known about outcomes such as age of diagnosis after different NBS methods.
Methods
We used the U.S. Cystic Fibrosis Foundation Patient Registry to identify infants with CF born between 2001 and 2008 in states that utilized NBS. We compared ages at diagnosis, genotyping, sweat test, and first visit to a CF Centre between states that used serial immunoreactive trypsinogen (IRT/IRT) levels and states that used IRT and DNA analysis (IRT/DNA).
Results
We identified 1,288 infants with CF. Compared to infants born in IRT/IRT states, infants born in IRT/DNA states were younger at the time of diagnosis (median 2.3 weeks versus 4.0 weeks in IRT/IRT states, p<0.001), genotyping (0.7 weeks versus 5.3 weeks, p<0.001), and initial CF Centre visit (5.9 weeks versus 7.7 weeks, p=0.008).
Conclusions
Although there is room to improve outcomes with both strategies, infants born in IRT/DNA states have treatment initiated at a younger age than infants born in IRT/IRT states.
doi:10.1016/j.jcf.2011.10.002
PMCID: PMC3302923  PMID: 22104951
5.  Chest Computed Tomography Scores of Severity Are Associated with Future Lung Disease Progression in Children with Cystic Fibrosis 
Rationale: Most children with cystic fibrosis (CF) experience a slow decline in spirometry, although some children continue to be at risk for more significant lung disease progression. Chest computed tomography (CT) scans have been shown to be more sensitive to changes in lung disease than spirometry and may provide a means for predicting future lung disease progression.
Objectives: We hypothesized that Brody chest CT scan scores obtained in 2000 in a prospectively monitored cohort of children with CF would be associated with the most recent measures of lung disease severity.
Methods: Brody chest CT scan scores were calculated for 81 children enrolled in the Wisconsin CF Neonatal Screening Project. Multivariable linear regression was used to determine associations between Brody scores and the most recent (age 21 yr or June 30, 2010, whichever was later) measures of CF lung disease.
Measurements and Main Results: The mean observation time after the chest CT scan was 7.5 years. Brody chest CT scan scores were significantly associated with the most recent measures of spirometry (P < 0.001) and Wisconsin and Brasfield chest radiograph scores (P < 0.001). The strength of this association was much stronger than spirometry obtained near the time of the chest CT scan (P < 0.01) but not chest radiograph scores.
Conclusions: Chest CT scan scores are associated with future lung disease severity, and quantitative chest imaging (chest CT scan and chest radiograph scores) is more strongly associated with future lung disease severity than measures of spirometry. These findings may help clinicians identify patients at risk of future lung disease progression.
doi:10.1164/rccm.201105-0816OC
PMCID: PMC3208650  PMID: 21737586
chest x-ray; pulmonary function tests; FEV1; quantitative chest radiography

Results 1-5 (5)