Table 1.
Characteristics of the Participants at Enrollment
| N | n (%) or Mean (SD) | |
|---|---|---|
|
| ||
| Male | 100 | 55 (55) |
|
| ||
| Age, mo | 100 | 14 (6.2) |
|
| ||
| CFTR Functional Class1 | 100 | |
| Minimal | 78 (78) | |
| Residual | 6 (6) | |
| Unclassified | 16 (16) | |
|
| ||
| Pancreatic insufficient2 | 100 | 93 (93) |
|
| ||
| Diagnosis by newborn screening3 | 100 | 21 (21) |
|
| ||
| Height percentile | 100 | 32.7 (26.4) |
|
| ||
| Weight percentile | 100 | 27.7 (26.3) |
|
| ||
| P. aeruginosa positive4 | 99 | 12 (12) |
|
| ||
| S. aureus positive4 | 99 | 42 (42) |
|
| ||
| Parent-reported cough5 | 99 | |
| None | 47 (48) | |
| Rare | 45 (45) | |
| In morning | 6 (6) | |
| Productive | 1 (1) | |
|
| ||
| Cigarette smoke exposure6 | 100 | 11 (11) |
|
| ||
| FEF75 | 79 | |
| ml/s | 321 (124) | |
| z score | -0.47 (1.5) | |
|
| ||
| FEV0.5 | 79 | |
| ml | 330 (92) | |
| Z score | 0.10 (1.3) | |
|
| ||
| FRC | 93 | |
| ml | 258 (84) | |
| Z score | 1.77 (2.5) | |
|
| ||
| Brasfield CXR Score7 | 96 | 22 (1.5) |
|
| ||
| Wisconsin CXR Score8 | 96 | 3.5 (2.7) |
1
Minimal, both CFTR mutations in functional class 1, 2 or 3. Residual, one or both mutations in functional class 4 or 517, 18
2
Reported use of pancreatic enzyme replacement therapy
3
Newborn screening for CF was being performed at only two of the participating centers at the time of study enrollment, the University of Colorado and Children’s Hospital Boston
4
Based on enrollment oropharyngeal culture
5
As per reference 20
6
Mother smoked during pregnancy or child exposed to smoke after birth, by parent report
7
Scores can range from 0 to 25. A higher score indicates milder disease.
8
Scores can range from 0 to 100. A higher score indicates more severe disease.