Characteristics of patients
| Variable | All patients n=78 | Men n=46 | Women n=32 |
|---|---|---|---|
| Age (years): median (25–75) | 34.8 (28.6–47.1) | 37.5 (20–61) | 41.5 (19–56.5) |
| Follow-up time (years): median (25–75) | 8.1 (3.7−12.6) | 7.8 (3.7–12.2) | 9.0 (3.6–13.3) |
| Age at diagnosis (years): median (25–75) | 10 (4−23.8) | 10 (4−24.5) | 11.5 (2.2−23.5) |
| No situs inversus | 16 (8.3−27.8) | 14 (9−27) | 18 (7.8−30) |
| Situs inversus | 6 (0.1−17.5) | 6 (0.4−14.8) | 7 (0.04−19.8) |
| Ethnic group | |||
| West Indies/sub-Saharan Africa, n | 3 | 2 | 1 |
| Turkey, n | 3 | 2 | 1 |
| Asia, n | 3 | 1 | 2 |
| Maghreb/Egypt, n | 19 | 13 | 6 |
| Europe, n | 48 | 28 | 20 |
| South America, n | 2 | 0 | 2 |
| Consanguinity, n | 17 | 8 | 9 |
| Body mass index (kg/m2), median (25−75) | 22.9 (20.8−26.1) | 23.4 (21.7−26.1) | 22.6 (20.1−26.4) |
| Respiratory symptoms | |||
| Neonatal respiratory distress, n | 18 | 11 | 7 |
| Age at onset of respiratory symptoms (months), median (25−75) | 4 (0−36) | 6 (0−18) | 3 (0−78) |
| <3 years, n | 54 | 33 | 21 |
| ≥3 years, n | 24 | 13 | 11 |
| Chronic cough, n | 74 | 42 | 32 |
| Dyspnoea (mMRC scale), n | |||
| mMRC 0 | 33 | 27 | 6 |
| mMRC 1 | 28 | 11 | 17 |
| mMRC ≥2 | 17 | 8 | 9 |
| Respiratory complications | |||
| Massive haemoptysis, n | 2 | 0 | 2 |
| Allergic bronchopulmonary aspergillosis, n | 2 | 1 | 1 |
| Extrapulmonary comorbidities | |||
| Situs inversus, n (%) | 37 (47.4) | 21 (45.6) | 16 (50) |
| Polycystic kidney disease, n | 2 | 1 | 1 |
| Retinitis pigmentosa, n | 3 | 3 | 0 |
| Psychomotor impairment, n | 3 | 2 | 1 |
| Polydactylia, n | 1 | 1 | 0 |
| Smokers, n | 17 | 10 | 7 |
mMRC scale, modified Medical Research Council scale.