Abstract
Primary ciliary dyskinesia (PCD) is a genetic disease characterized by abnormalities in ciliary structure and leads to bronchiectasis. The disease is probably underrecognized in the cohort of bronchiectasis due to extensive diagnostic testing and low recognition of PCD.
To find a reliable screening method for PCD in the cohort of non-cystic fibrosis bronchiectasis.
All patients who presented to our outpatient clinic were analysed. Nasal NO and a symptom score of key clinical features of PCD (neonatal respiratory symptoms, chronic cough since childhood, otitis media, rhinosinusitis, recurrent pneumonia) were measured post-hoc and compared in the two groups of PCD-bronchiectasis and non-PCD-bronchiectasis.
118 patients (42 male, 76 females) had a sufficient nasal NO and were eligible for analysis. The median (IQR) in nNO levels in parts per billion (ppb) in the PCD and non- PCD groups were 35 (IQR 22-70) and 525 (IQR 367-690), respectively. The median nasal NO levels were significantly lower in the PCD- group than in the other group (p<0,001). The median (IQR) of symptom score in the PCD- group and in the non-PCD group were 4 (IQR 4-5) and 1 (IQR 0-2), respectively (p<0.001). Using ROC curve analysis, a symptom score of 2.5 had the best discriminative value. The combination of nasal NO and the symptom score leads to a positive predictive value (PPV) for PCD of 94% and negative predictive value (NPV) of 100%.
Low nasal NO and a clinical scoring of typical PCD signs and symptoms is a suitable and cheap screening test for PCD in the cohort of non-cystic fibrosis bronchiectasis.
- Copyright ©the authors 2016
