Abstract
BackgroundNasal nitric oxide (nNO) measurement is an established first line test in the work-up for primary ciliary dyskinesia (PCD). Tidal breathing nNO (TB-nNO) measurements require minimal cooperation and are potentially useful even in young children. Hand-held NO devices are becoming increasingly widespread for asthma management. Therefore, we chose to assess whether hand-held TB-nNO measurements reliably discriminate between PCD, and Healthy Subjects (HS) and included Cystic Fibrosis (CF) patients as a disease control group known to have intermediate nNO levels.MethodsIn this cross sectional, single centre, single occasion, proof-of-concept study in children and adults with PCD and CF, and in HS we compared feasibility, success rates, discriminatory capacity, repeatability and agreement between a hand-held electrochemical device equipped with a nNO software application sampling at flow rates 2 ml/s or 5 ml/s, and two stationary chemiluminescence devices, applying both tidal breathing and velum closure techniques.ResultsMeasurements were done in 16 PCD patients, 21 patients with CF and 20 HS aged between 3.8 and 60.9 years. Hand-held TB-nNO showed high success rate (96.5–100%) vs. velum closure nNO techniques (70.2–89.5%). Hand-held TB-nNO sampling at flow rate 5 ml/s showed equally high discriminative power (PCD vs. HS [p<0.0001] and PCD vs. CF [p<0.0001]) and reaching close to 100% sensitivity and specificity, superior repeatability (CV% = 10%) and equal limits of agreement compared to TB-nNO by stationary devices and even compared to velum closure sampling.ConclusionHand-held TB-nNO discriminates significantly between PCD, CF and HS and shows promising potential as a widespread targeted case-finding tool for PCD, although further studies are warranted before implementation.
Highlights
Diagnosis of Primary Ciliary Dyskinesia (PCD) is important to obtain best possible general care and improve or stabilise lung function after diagnosis and initiation of treatment[1]
The current recommended method since 2005 is aspiration at constant flow rate from one nostril with gas entrained via the other nostril during velum closure (VC)[9], which may not be compatible with measurements in very young children[7]
We hypothesised that TB-Nasal nitric oxide (nNO) measurement would demonstrate higher feasibility compared to VC-nNO measurement, and further that hand-held Tidal breathing nNO (TB-nNO) values would exhibit high discrimination between primary ciliary dyskinesia (PCD) and non-PCDs compared to VC-nNO and to TB-nNO measured by stationary devices. In this proof-of-concept study we aimed to investigate the discriminative power of hand-held TB-nNO measurements to separate between PCD, Cystic Fibrosis (CF) and healthy and to assess the agreement between hand-held TB-nNO and stationary TBnNO, and VC-nNO values in children and adults
Summary
Diagnosis of Primary Ciliary Dyskinesia (PCD) is important to obtain best possible general care and improve or stabilise lung function after diagnosis and initiation of treatment[1]. Nasal Nitric Oxide (nNO) concentration discriminates significantly between PCD and non-PCD[2,3,4,5,6], is quick, noninvasive, and since 2009 recommended in Europe as the preferred first-line test for PCD in advance of confirmatory diagnostic tests[7],[8]. We recently reported significant discrimination between PCD and non-PCD (including CF patients) and acceptability of 95% in subjects ,6 years of age, with a minimum age for compliance of 14 days of age by TB-nNO technique, using a stationary chemiluminescence device[5]. Nasal nitric oxide (nNO) measurement is an established first line test in the work-up for primary ciliary dyskinesia (PCD). We chose to assess whether hand-held TB-nNO measurements reliably discriminate between PCD, and Healthy Subjects (HS) and included Cystic Fibrosis (CF) patients as a disease control group known to have intermediate nNO levels
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