Table 2.
Baseline Characteristics of US CFFPR Cohorts, 1993–2016.
| Original 1993–1997 Cohort, n = 11,63030 | New 1993–1997 Cohort, n = 9,941 | New 5-Year Cohorts* | |||||
|---|---|---|---|---|---|---|---|
| 1993–1998, n = 9,757 | 1999–2004, n = 13,073 | 2005–2010, n = 15,043 | 2011–2016, n = 17,635 | ||||
| Deaths within 5 years: % | 12.7 | 12.2 † | 13.2 † | 10.0 ‡ | 7.5 ‡ | 7.3 ‡ | |
| Age: Median (Range) | 15.42 (5.50–71.05) | 15.37 (6.00–71.05) † | 15.44 (6.00–66.47) † | 15.29 (6.01–72.15) † | 16.25 (6.00–74.31) § | 17.93 (6.00–81.14) § | |
| Sex: % Female | 46.6 | 46.8 † | 46.8 † | 47.0 † | 47.6 † | 48.5 ǁ | |
| FEV1%: Median (Range) | 67.94 (5.11–191.46) | 70.92 (6.02–184.13) ** | 74.52 (6.02–184.13) ** | 85.79 (9.52–174.36) ** | 88.49 (14.57–178.99) ** | 91.43 (11.00–196.24) ** | |
| Weight-for-age z-score: Mean (SD) | −0.85 (1.06) | −0.83 (1.05) † | −0.76 (1.05) § | −0.46 (1.00) § | −0.25 (1.01) § | −0.08 (1.00) § | |
| Pancreatic Sufficiency: % Affected | 5.3 | 6.3 †† | 4.2 ‡ | 4.5 †† | 6.1 †† | 9.7 ‡ | |
| Diabetes: % Affected | 6.2 | 6.3 † | 7.0 §§ | 9.5 ‡ | 14.6 ‡ | 21.1 ‡ | |
| Methicillin Sensitive S aureus: % Infected | 30.8 | 30.9 † | 36.3 ‡ | 48.6 ‡ | 61.1 ‡ | 61.3 ‡ | |
| B cepacia complex: % Infected | 3.6 | 3.7 † | 4.0 † | 3.9 † | 3.7 † | 3.9 † | |
| Prior Year Pulmonary Exacerbations: Median (Range) | 0 (0–5) | 0 (0–5) ‡ | 0 (0–5) ‡ | 0 (0–5) ‡ | 0 (0–5) ‡ | 0 (0–5) ‡ | |
| % with Number of Pulmonary Exacerbations‖‖ | 0 | 51.7 | 59.6 | 59.3 | 66.5 | 59.5 | 59 |
| 1 | 19.5 | 17.3 | 17.5 | 11.7 | 23.2 | 22.3 | |
| 2 | 12.8 | 10.8 | 10.8 | 10.2 | 9.2 | 10.4 | |
| 3 | 6.8 | 5.5 | 5.5 | 4 | 4 | 4.3 | |
| 4 | 3.9 | 3.3 | 3.3 | 3 | 2.2 | 1.9 | |
| 5+ | 5.3 | 3.6 | 3.6 | 4.6 | 2 | 2.2 | |
| Prognostic risk score: Mean (SD)*** | −3.10 (1.94) | −3.30 (1.83) | −3.43 (1.84) | −3.98 (1.81) | −4.18 (1.69) | −4.26 (1.73) | |
| Prognostic risk score: Median (Range)*** | −3.29 (−8.89–2.71) | −3.51 (−8.83–2.37) | −3.65 (−8.83–2.07) | −4.28 (−9.57–2.12) | −4.43 (−10.71–1.64) | −4.50 (−10.16–1.79) | |
| 100 × Predicted Probability of Death within 5 Years Using the Original Model: Median (Range) | 3.597 (0.014–93.76) | 2.902 (0.015–91.45) | 2.535 (0.015–88.77) | 1.359 (0.007–89.27) | 1.177 (0.002–83.75) | 1.098 (0.004–85.68) | |
*Comparisons used corrected data for FEV1, height and weight and weight-for -age z-score, prognostic risk score and predicted probabilities of death calculated using those corrected values.
Compared to original 1993–1997 cohort †P-value not significant; ‡P < 0.001 by χ2; §P < 0.001 by t-test; ‖P = 0.001 by χ2; **P < 0.001 by Mann-Whitney-Wilcoxon test; ††P < 0.01 by χ2; §§P < 0.05 by χ2.
‖‖Statistical comparisons were not made for percentages of patients with each Number of Pulmonary Exacerbations. See main text paragraph in Results concerning prognostic risk scores.
***Prognostic risk score is the equivalent to the log-odds of death within 5-years.