2005 | 2006 | 2007 | 2008 | 2009 | 2010 | 2011 | 2012 | |
---|---|---|---|---|---|---|---|---|
Number of patients | 129 | 126 | 144 | 148 | 161 | 166 | 177 | 175 |
Age, mean (SD) | 9.7 (5.9) | 10.0 (5.4) | 10.0 (5.6) | 10.5 (5.6) | 10.4 (6.0) | 10.0 (5.9) | 10.1 (6.0) | 10.6 (5.7) |
% male/%female | 47/53 | 45/55 | 45/55 | 45/55 | 45/55 | 46/54 | 50/50 | 49/51 |
Ethnicity (%) | ||||||||
Caucasian | 94.6 | 94.4 | 94.4 | 94.6 | 95.0 | 94.0 | 94.4 | 94.9 |
African American/Black | 4.7 | 4.0 | 4.2 | 4.1 | 3.7 | 4.2 | 4.0 | 3.4 |
Asian | 1.6 | 1.6 | 1.4 | 1.4 | 1.2 | 1.2 | 1.1 | 1.1 |
Other | 0 | 0 | 0 | 0 | 0 | 0.6 | 0.6 | 1.2 |
Genotype (%) | ||||||||
Delta F508 homozygous | 47.3 | 47.6 | 46.5 | 46.6 | 47.2 | 45.2 | 41.8 | 40.0 |
Delta F508 heterozygote | 35.7 | 34.9 | 37.5 | 37.8 | 35.4 | 36.7 | 38.4 | 41.1 |
Other mutations (no delta F508) | 15.5 | 16.7 | 16.0 | 15.5 | 17.4 | 18.1 | 19.2 | 18.9 |
No genotype data | 1.6 | 0.8 | 0 | 0 | 0 | 0 | 0.6 | 0 |
Median BMI percentile 2–20 years | 53 | 55.5 | 53.1 | 50.5 | 55.7 | 58.8 | 64.5 | 60.4 |
Percent on pancreatic enzymes (%) | 89.1 | 87.3 | 88.2 | 88.5 | 87.6 | 84.3 | 81.9 | 80.0 |
FEV1 (% predicted) 6–17 years | 88 | 91 | 92 | 92 | 92 | 95 | 93 | 93 |
Inhaled Tobramycin use (>6 years old with Pseudomonas) | 89.9 | 74.2 | 76.4 | 73.7 | 77.5 | 76.3 | 73.8 | 71.7 |
1+ pulmonary exacerbations per year (%) <18 years | 31.9 | 25.6 | 29.2 | 23.7 | 24.8 | 23.7 | 16.4 | 25.5 |