Clinical variables | Clinical outcomes |

Age at diagnosis | Median 10.0 years; range 3 months–40.0 years |

Female | 27 (39.7%) |

Situs inversus | 29 (42.6%) |

Consanguinity (n=60) | 65 (92.6%) |

Congenital heart disease^{#} (n=67) | 8 (11.9%) |

Neonatal respiratory symptoms (n=67) | 57 (85.1%) |

Persistent wet cough | 57 (83.8%) |

Persistent rhinosinusitis | 64 (94.1%) |

Conductive hearing loss and/or otitis media with effusion (glue ear) | 50 (73.5%) |

Finger clubbing (n=67) | 13 (19.4%) |

nNO (nL·min^{−1}) (n=36) | Median 14.5; range 5–62.5 |

FEV_{1} z-score (n=28) | Median −1.90 (−5.0–1.32) |

FVC z-score (n=28) | Median −1.72 (−5.64–0.57) |

BMI z-score n=28 | Median −0.36 (−3.03–2.57) |

n=68 unless otherwise stated. BMI: body mass index; FEV_{1}: forced expiratory volume in 1 s; FVC: forced vital capacity; nNO: nasal nitric oxide.

^{#}Atrial septal defect n=2; right isomerism with complex cardiac defects n=1; situs ambiguous with complex cardiac defects n=1; subaortic membrane n=1; ventricular septal defect n=3.