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Nasal nitric oxide (nNO) concentrations are low in patients with primary ciliary dyskinesia (PCD) providing a noninvasive screening test.We conducted a systematic review of the literature to examine the utility of nNO in screening for PCD, in particular 1) different respiratory manoeuvres during sampling (velum closure, tidal breathing, etc.), 2) accuracy in screening young/uncooperative children, 3) stationary versus portable analysers, and 4) nNO in “atypical” PCD.96 papers were assessed according to modified PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) criteria and 22 were included in this review.Meta-analysis of 11 studies comparing nNO during a velum closure breath hold gave a mean±sd nNO of 19.4±18.6 nL·min-1 in PCD (n = 478) and 265.0±118.9 nL·min-1 in healthy controls (n = 338). Weighted mean difference for PCD versus healthy controls was 231.1 nL·min-1 (95% CI 193.3–268.9; n = 338) and 114.1 nL·min-1 (95% CI 101.5–126.8; n = 415) for PCD versus cystic fibrosis. Five studies of nNO measurement during tidal breathing demonstrated that this is an acceptable manoeuvre in young children where velum closure is not possible, but the discriminatory value was reduced. Four small studies of portable NO analysers suggest these are reliable tools for screening for PCD. However, nNO must be interpreted alongside clinical suspicion. Future studies should focus on standardising sampling techniques and reporting.

Chronic Obstructive Pulmonary Disease (COPD) has a high mortality. Many indices have been developed to classify disease severity including the 2007 and 2011 GOLD classification and BODE index. However, no study has compared the prediction of mortality by those indices. This is an observational study of 96 patients with COPD and followed prospectively for a median of 60 months. We performed at baseline pulmonary function tests, 6-minute walking test, dyspnea scale mMRC, anthropometric and questionnaire measures of exacerbations. BODE index and GOLD 2007 and GOLD 2011 classification was calculated. The capacity of all indices to predict mortality was compared using Cox proportional hazard analysis and Kaplan Meier survival cruves. A total of 55 men and 41 women were studied, of whom 50% died during a follow-up time mean of 69 +/- 37 months. The mean FEV1 was 27% +/- 5%. Statistically significant associations were observed between mortality and BODE index (p = 0.024) and male sex (p = 0.021). Cox regression analysisshowed that the only statistically significant association was with BODE (p=0,037; IC: 95% 1,024-2,17). The Kaplan-Meier curves for BODE, 2007 GOLD and GOLD ABCD were calculated and are The BODE index predicts all cause mortality better than the old GOLD classification based on FEV1 and the new ABCD grading . We also observed that patients classified as GOLD B has higher mortality than those classified as GOLD C.

The file attached to this record is the author's final peer reviewed version. The Publisher's final version can be found by following the DOI link. Non-typeable Haemophilus influenzae (NTHi) is the most common pathogen in primary ciliary dyskinesia (PCD) patients. We hypothesised that abnormal ciliary motility and low airway nitric oxide (NO) levels on airway epithelial cells from PCD patients might be permissive for NTHi colonisation and biofilm development. We used a primary epithelial cell co-culture model to investigate NTHi infection. Primary airway epithelial cells from PCD and non-PCD patients were differentiated to ciliation using an air–liquid interface culture and then co-cultured with NTHi. NTHi adherence was greater on PCD epithelial cells compared to non-PCD cells ( p