Patient 1 2 3 4* 5*
Clinical details
Gender M F F F M
Age 23yrs 18yrs 39yrs 5yrs 9yrs
Sitis inversus No Yes No Yes Yes
ENT symptoms Yes Yes Yes Yes Yes
Respiratory distress at birth No No No No No
Productive/ wet cough Yes Yes Yes Yes Yes
Bronchiectasis Yes Yes Yes No No
Recurrent chest infections Yes Yes Yes Yes Yes
Results
Nasal nitric oxide (ppb) 25 293 3 463 394
CBF (Hz) 12.6 8.5 0 11.5 8.9
Cilia beat pattern Static or stiff, rapid incomplete beat slow Mainly static, some slow dyskinetic  movement Dyskineitic and rapid incomplete beat Static, dyskinetic   and co-ordinated beat
%IDA absent at TEM 42% 36% 78% 21% 33%
Immunofluorescence to DNALI1 Yes Yes Yes Yes Yes
*Patients’s 4 and 5 were siblings
Table 3: Diagnostic test results for patients with consistent loss of IDA at review.