TABLE 1

Characteristics of the patient cohort#

Subjects n69
Age years51±18
Sex 
 Male24 (35) (carriers=3)
 Female45 (65) (carriers=3)
FEV1 % predicted47±27
Chronic airways colonisation with Pseudomonas aeruginosa34 (49) (carriers=3)
Associated conditions 
 Idiopathic33 (48) (carriers=2)
 COPD/emphysema8 (12)
 Asthma/ABPA6 (9) (carriers=1)
 Post infectious5 (7)
 Primary ciliary dyskinesia5 (7)
 Immunodeficiency5 (7) (carriers=2)
 CFTR-related disorder3 (4) (carriers=1)
 Other4 (6)
  • Data are presented as mean±sd or n (%), unless otherwise stated. FEV1: forced expiratory volume in 1 s; COPD: chronic obstructive pulmonary disease; ABPA: allergic bronchopulmonary aspergillosis; CFTR: cystic fibrosis transmembrane conductance regulator. #: the number of p.W493R-SCNN1A carriers is listed in brackets; : indicates the diagnosis at the beginning of the study.