Demographic data for the cohort
| | |
|---|---|
| Pediatric cohort | |
| Cohort size | 23 (82% response rate) |
| Age (years) | 7.81 (3.90, 12.49) |
| Age at diagnosis (years) (n = 20) | 1.19 (0.80, 2.93) |
| Screened due to family history | 6 (21%) |
| Home IgRT | 16 (70%) |
| Home SCIg | 15 (94%) |
| Bronchiectasis | 1 (4%) |
| Conjunctivitis | 2 (9%) |
| Post-infection deafness | 1 (4%) |
| Infective gastrointestinal complications | 1 (4%) |
| Noninfectious gastrointestinal complications | 1 (4%) |
| Mental health disorder | 1 (4%) |
| Median annual bacterial infection incidence (n = 20) | 1.20 (0.80, 1.88) |
| Last recorded FEV1 Z-score (n = 10) | 0.21 (−1.02, 0.60) |
| Adult cohort | |
| Cohort size | 44 (92% response rate) |
| Age (years) | 36.22 (29.15, 48.76) |
| Age at diagnosis (years) (n = 39) | 3.37 (1.16, 10.79) |
| Screened due to family history | 12 (25%) |
| Home IgRT | 31 (74%) |
| Home SCIg | 14 (45%) |
| Bronchiectasis | 28 (64%) |
| Conjunctivitis | 7 (16%) |
| Post-infection deafness | 3 (7%) |
| Infective gastrointestinal complications | 1 (2%) |
| Noninfectious gastrointestinal complications | 4 (9%) |
| Mental health disorder | 9 (21%) |
| Median annual bacterial infection incidence | 0.91 (0.68, 1.50) |
| Last recorded FEV1 Z-score | −1.23 (−3.00, −0.65) |
| | |
|---|---|
| Cohort size | 23 (82% response rate) |
| Age (years) | 7.81 (3.90, 12.49) |
| Age at diagnosis (years) ( | 1.19 (0.80, 2.93) |
| Screened due to family history | 6 (21%) |
| Home IgRT | 16 (70%) |
| 15 (94%) | |
| Bronchiectasis | 1 (4%) |
| Conjunctivitis | 2 (9%) |
| Post-infection deafness | 1 (4%) |
| Infective gastrointestinal complications | 1 (4%) |
| Noninfectious gastrointestinal complications | 1 (4%) |
| Mental health disorder | 1 (4%) |
| Median annual bacterial infection incidence ( | 1.20 (0.80, 1.88) |
| Last recorded FEV1 Z-score ( | 0.21 (−1.02, 0.60) |
| Cohort size | 44 (92% response rate) |
| Age (years) | 36.22 (29.15, 48.76) |
| Age at diagnosis (years) ( | 3.37 (1.16, 10.79) |
| Screened due to family history | 12 (25%) |
| Home IgRT | 31 (74%) |
| 14 (45%) | |
| Bronchiectasis | 28 (64%) |
| Conjunctivitis | 7 (16%) |
| Post-infection deafness | 3 (7%) |
| Infective gastrointestinal complications | 1 (2%) |
| Noninfectious gastrointestinal complications | 4 (9%) |
| Mental health disorder | 9 (21%) |
| Median annual bacterial infection incidence | 0.91 (0.68, 1.50) |
| Last recorded FEV1 Z-score | −1.23 (−3.00, −0.65) |
Data are shown as median (IQR). The denominator is shown where not all data are available. SCIg, SC immunoglobulin.
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