TY - JOUR T1 - Differences in symptoms between patients with and without PCD: Data from a PCD diagnostic referral centre JF - European Respiratory Journal JO - Eur Respir J VL - 44 IS - Suppl 58 SP - P1245 AU - Laura Behan AU - Samantha Packham AU - Amanda Harris AU - Sin Tung Mak AU - Hazel J. Evans AU - Karen Lock AU - Woolf T. Walker AU - Jane S. Lucas Y1 - 2014/09/01 UR - http://erj.ersjournals.com/content/44/Suppl_58/P1245.abstract N2 - Background: Primary Ciliary Dyskinesia (PCD) is an inherited condition characterised by pulmonary infections, rhinosinusitis and situs abnormalities (∼50%). Symptoms are mostly non-specific thus guidance on who needs testing is lacking.Method: From 2006 to 2013, data was prospectively collected using a proforma from 947 consecutive patients referred for PCD testing. Information included neonatal symptoms, respiratory, ENT and cardiac history, symptoms at time of assessment, situs abnormalities and family history. Patients were deemed PCD positive following a portfolio of tests including nasal nitric oxide, ciliary beat analysis, electron microscopy +/- culture at air liquid interface.Results: 775 patients had sufficient data for inclusion, 47% male and 53% female. 90 (11%) were PCD positive. The median age at assessment was 6 years (range: 0-80 years). The rates of neonatal symptoms (72% vs. 18%; p<0.001) neonatal respiratory support (36% vs. 6%; p<0.001) history of pneumonia (39% vs. 29%; p<0.001), situs abnormality (41% vs. 4%; p<0.001), rhinitis (80% vs. 51%; p<0.001) wet cough (79% vs. 64% p=0.008) hearing loss (45% vs. 22%; p<0.001) and cardiac anomaly (22% vs. 3.5% p<0.001) were significantly higher in the PCD positive group. No significant difference was found between the two groups in presence of cough, sinusitis, history of bronchiectasis and hydrocephalus.Conclusion: Significant differences exist in the clinical history, current condition and symptoms of patients with a positive diagnosis of PCD compared to those found to be negative. Symptoms starting in early life were particularly suggestive of PCD. This data may improve predication of who to test for PCD. ER -