Table 1.
Demographic and clinical variables of the study population, 2005–2020.
| Survived, N (%) | Died, N (%) | Total, N (%) | p | |
|---|---|---|---|---|
| 4,085 (88.8%) | 516 (11.2%) | 4,601 (100%) | ||
| Sex | 0.8401 | |||
| Female | 1,943 (47.6%) | 243 (47.1%) | 2,186 (47.5%) | |
| Male | 2,142 (52.4%) | 273 (52.9%) | 2,415 (52.5%) | |
| Diagnosis category | < 0.0011 | |||
| Diagnosed with meconium ileus | 480 (11.8%) | 48 (9.3%) | 528 (11.5%) | |
| Age < 6 weeks or through NBS/Prenatal screening | 2,747 (67.2%) | 296 (57.4%) | 3,043 (66.1%) | |
| Age 6 weeks–< 2 years | 376 (9.2%) | 97 (18.8%) | 473 (10.3%) | |
| Age 2 – 17 years | 325 (8.0%) | 46 (8.9%) | 371 (8.1%) | |
| Age ≥ 18 years | 157 (3.8%) | 29 (5.6%) | 186 (4.0%) | |
| F508del homozygous | 0.8641 | |||
| No | 2,223 (54.4%) | 275 (54.1%) | 2,498 (54.4%) | |
| Yes | 1860 (45.6%) | 233 (45.9%) | 2,093 (45.6%) | |
| Lung transplant | < 0.0012 | |||
| No | 3,885 (95.1%) | 321 (62.2%) | 4,206 (91.4%) | |
| Yes | 200 (4.9%) | 195 (37.8%) | 395 (8.6%) | |
| Pancreatic exocrine status | < 0.4361 | |||
| Insufficient | 2,836 (69.4%) | 368 (71.3%) | 3,204 (69.6%) | |
| Sufficient | 972 (23.8%) | 110 (21.3%) | 1,082 (23.5%) | |
| Missing | 277 (6.8%) | 38 (7.4%) | 315 (6.8%) | |
| Mean FEV1pp across the lifetime | < 0.0012 | |||
| Not assessed, age < 6 years3 | 516 (12.6%) | 3 (0.6%) | 519 (11.3%) | |
| Mean (SD) | 83.1 (21.0) | 55.7 (19.7) | 79.7 (22.7) | |
| Range | 17.0–130.0 | 10.0–111.6 | 10.0–130.0 | |
| Missing | 138 (3.9%) | 27 (0.6%) | 165 (3.6%) | |
| Last recorded BMI4 | < 0.0011 | |||
| Underweight | 182 (4.5%) | 134 (26.0%) | 316 (6.9%) | |
| Adequate weight status | 2,670 (65.4%) | 312 (60.5%) | 2,982 (64.8%) | |
| Overweight | 1,076 (26.3%) | 47 (9.1%) | 1,123 (24.4%) | |
| Missing | 157 (3.8%) | 23 (4.5%) | 180 (3.9%) | |
| Mean last recorded BMI | ||||
| Adults (N) | 2,359 | 462 | 2,821 | < 0.0012 |
| BMI, Mean (SD) | 23.5 (4.9) | 20.6 (3.5) | 23.0 (4.8) | |
| Range | 13.9–167.5 | 12.5–35.8 | 12.5–157.5 | |
| Missing | 89 | 21 | 110 | |
| Children (N) | 1,726 | 54 | 1,780 | < 0.0012 |
| BMI percentile, Mean (SD) | 0.6 (0.3) | 0.4 (0.3) | 0.6 (0.3) | |
| Range | 0.0–1.0 | 0.0–1.0 | 0.0–1.0 | |
| Missing | 68 | 2 | 70 |
BMI body mass index, FEV1pp percent predicted forced expiratory volume in one second.
1 Pearson’s Chi-squared test, 2 Linear Model ANOVA, 3 Lung function not captured for those < 6 years of age, 4 Adult individuals (≥ 18 years) were classified into BMI categories based on World Health Organization guidelines as underweight (< 18.5 kg/m2), adequate weight (18.5–24.9 kg/m2), or overweight (≥ 25.0 kg/m2). Children were classified as underweight (< 10th percentile), adequate weight (10–85th percentile), or overweight (> 85th percentile).