Nasal nitric oxide and nitric oxide synthase expression in primary ciliary dyskinesia

Pifferi, Massimo; Bush, Andrew; Maggi, Fabrizio; Michelucci, Angela; Ricci, Veronica; Conidi, Me; Cangiotti, Angela Maria; Bodini, Alessandro; Simi, Paolo; Macchia, Pierantonio; Boner, Al

doi:10.1183/09031936.00044310

Cited by 50 publications

(38 citation statements)

References 30 publications

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“…Recent reports reinforce the suggestion that low levels of nNO can be used as a screening tool for the early identification of PCD patients [20,26] and that they are indicative of disease also in uncooperative children when they remain persistently low and when other diseases have been excluded [27]. Our present data confirms that nNO levels in PCD patients are very low, which is in agreement with previous studies [2,4,7].…”

Section: Discussionsupporting

confidence: 83%

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Different Levels of Exhaled Nasal Nitric Oxide in Patients Diagnosed with Primary Dyskinesia

Bodini¹,

Peroni²,

Tenero³

et al. 2015

Arch Pulmonol Respir Care

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Background: Primary ciliary dyskinesia (PCD) is a genetic disease characterized by abnormally beating cilia. In these patients levels of nasal nitric oxide (nNO) are lower than those observed in healthy subjects. Objectives:We recorded the nNO levels in PCD patients in order to use those nNO measurements in the screening and identification of patients with symptoms suggestive of disease PCD disease. Methods:We measured nasal NO in 36 PCD patients (3 uncooperative younger children and 33 cooperative adult patients) and did a nNO re-evaluation after 12 months in patients with higher levels of nNO.Results: Twenty-seven PCD patients showed very low nNO levels (29.1 ppb) and nine cooperative patients had high nNO levels (583.3 ppb, p<0.001) (T0); the PCD patients with high nNO levels were re-evaluated after 12 months (T1).The median T0 and T1 nNO values of the seven PCD patients were 360 ppb and 324 ppb (p=0.0180), respectively; in 6 patients with high levels of nNO the diagnosis of PCD was confirmed by electron microscopy, and in one subject the diagnosis was confirmed for secondary ciliary dyskinesia.Conclusions: Low levels of nNO remain indicative of PCD disease; high levels of nNO are supportive of PCD, but cannot be used to exclude diagnosis.These results suggest that repeated measures are warranted when nNO is occasionally high in patients with symptoms suggestive of PCD disease, and at present electron microscopy is still the only valid evaluation tool in unclear cases of PCD.

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Section: Discussionsupporting

confidence: 83%

“…In a recent study of ours we confirmed that NO gas is implicated in the modulation of ciliary function in PCD patients [26]. This study could reflect the correlation between NO and ciliary function, which may explain some of the nNO levels and clinical variability of the disease.…”

Section: Discussionsupporting

confidence: 65%

Different Levels of Exhaled Nasal Nitric Oxide in Patients Diagnosed with Primary Dyskinesia

Bodini¹,

Peroni²,

Tenero³

et al. 2015

Arch Pulmonol Respir Care

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“…Four studies reported nNO levels in consecutive referrals to a PCD diagnostic service; two were excluded (lack of sampling details and unclear PCD diagnosis) [19,33]. CORBELLI et al [10] Seven studies compared nNO levels in PCD patients with those in non-CF bronchiectasis (table 4).…”

Section: Study Selection and Risk Of Biasmentioning

confidence: 99%

Nasal nitric oxide screening for primary ciliary dyskinesia: systematic review and meta-analysis

et al. 2014

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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 (n5478) and 265.0¡118.9 nL?min -1 in healthy controls (n5338). Weighted mean difference for PCD versus healthy controls was 231.1 nL?min -1 (95% CI 193.3-268.9; n5338) and 114.1 nL?min -1 (95% CI 101.5-126.8; n5415) 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. @ERSpublications Nasal nitric oxide is a useful and increasingly versatile screening tool in primary ciliary dyskinesia at all ages http://ow.ly/AR5mqThis article has supplementary material available from erj.ersjournals.com

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“…In healthy individuals, nNO is produced by the paranasal sinuses via the action of the enzyme nitric oxide synthase (NOS). 13 In the respiratory epithelium, the action of…”

Section: Introductionmentioning

confidence: 99%

Decreased Levels of Nasal Nitric Oxide in Children With Midline Neuroanatomical Anomalies: A Possible Connection Between Ciliary Dysfunction and Isolated Nervous System Defects

Goez

Scott

Al-Jabri

et al. 2015

Pediatric Neurology

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Nasal nitric oxide and nitric oxide synthase expression in primary ciliary dyskinesia

Cited by 50 publications

References 30 publications

Different Levels of Exhaled Nasal Nitric Oxide in Patients Diagnosed with Primary Dyskinesia

Different Levels of Exhaled Nasal Nitric Oxide in Patients Diagnosed with Primary Dyskinesia

Nasal nitric oxide screening for primary ciliary dyskinesia: systematic review and meta-analysis

Decreased Levels of Nasal Nitric Oxide in Children With Midline Neuroanatomical Anomalies: A Possible Connection Between Ciliary Dysfunction and Isolated Nervous System Defects

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