Chest
Clinical InvestigationsNITRIC OXIDENitric Oxide Metabolites Are Not Reduced in Exhaled Breath Condensate of Patients With Primary Ciliary Dyskinesia*
Section snippets
Patients
Patients were recruited from the pediatric PCD Clinic at the Royal Brompton Hospital. PCD was diagnosed by nasal brushing with an estimation of CBF and the determination of ultrastructural defects using electron microscopy. We studied 15 patients with PCD (seven boys; mean [± SEM] age, 10.3 ± 0.7 years; age range, 7 to 14 years) who had a mean FEV1 of 73 ± 2.1% of predicted. Eight patients were receiving therapy with inhaled corticosteroids. The age-matched control group consisted of 14 healthy
Lower and Upper Airway NO
The mean exhaled NO levels were significantly decreased in PCD patients compared to those in healthy subjects (3.2 ± 0.2 vs 8.4 ± 0.9 parts per billion [ppb], respectively; p < 0.0001) [Fig 1]. There was no significant difference between the steroid-naive and steroid-treated groups (3.4 ± 0.3 vs 3.0 ± 0.3 ppb, respectively). There was no correlation between exhaled NO and FEV1, NO2−, NO2−/NO3− and S-nitrosothiol levels in exhaled breath condensate. Upper airway NO levels also were found to be
Discussion
This study was designed to investigate whether the levels of NO metabolites, such as NO2−, NO2−/NO3−, and S-nitrosothiol, in exhaled breath condensate were reduced in PCD patients, as might be expected from the lower levels of exhaled and nasal NO that have been described previously.12 Surprisingly, no differences were found in the levels of exhaled NO2−, NO2−/NO3−, or exhaled S-nitrosothiol between patients with PCD and healthy subjects. There was a trend toward decreased exhaled NO2− levels
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Cited by (45)
Non-cystic fibrosis bronchiectasis in children and adolescents: Neglected and emerging issues
2020, Pediatrics and NeonatologyCitation Excerpt :Interestingly, the application of metabolomics to EBC also showed how nuclear magnetic resonance spectroscopy discriminates healthy subjects from CF and PCD patients.11 In EBC from PCD children, 8-isoprostane, a marker of oxidative stress, is increased,12 and nitric oxide (NO) metabolites are similar to controls, despite reduced nasal NO (nNO).13 It is hoped that further studies will clarify the role of EBC metabolic profiling in the characterization of bronchiectasis with different etiology.
The effect of L-Arginine on Ciliary Beat Frequency in PCD patients, non-PCD respiratory patients and healthy controls
2018, Pulmonary Pharmacology and TherapeuticsCitation Excerpt :The reduced expression of the iNOS isoform in nasal epithelial cells has been proposed as the likely reason for the reduced nNO levels observed in PCD [11]. However, other studies reported similar total NOS activity as well as localization and abundance of iNOS in PCD and non-PCD subjects [12,13]. An increase in l-Arginine availability could have a significant effect on ciliary activity through the increase of endogenous NO. A limited number of animal and human studies have provided evidence regarding the stimulating effect of increasing l-Arginine levels and the subsequent activation of cyclic guanosine monophosphate pathway on Ciliary Beat Frequency (CBF) [14].
Exhaled breath condensate: Determination of non-volatile compounds and their potential for clinical diagnosis and monitoring. A review
2013, Analytica Chimica ActaCitation Excerpt :In EBC of healthy controls, the measured range of nitrosothiols was between 0.05 and 0.8 μM [174,175]. Increased levels of RS–NO were detected in patients with severe asthma [141,174], in severe cases of CF in adults [175] and COPD [141] and were decreased after glucocorticoid treatment [176]. On contrary, no increase was observed in mild asthma [141] and reduced values were found in children with CF [177] and in children with acute asthmatic respiratory failure [178].
Expression of nitric oxide synthases in primary ciliary dyskinesia
2011, Human PathologyCitation Excerpt :Because we did not find any difference in cell fluorescence between patients with PCD and control patients without PCD, we conclude that inactivation of NO by superoxide is unlikely to be a significant cause for the major drop of nasal NO concentration in patients with PCD. Our results are in line with the results of Csoma et al [24], who found that despite lower levels of exhaled NO in patients with PCD, no differences were found in exhaled NO metabolites between patients with PCD and healthy subjects, suggesting that NOS activity may not be decreased as much as might be expected on the basis of low exhaled and nasal NO levels in patients with PCD. One possible explanation for the discrepancy between a preservation of NOS activity and a drop of nasal NO might be an abnormal subcellular localization of NOS2, which were not examined by our microscopical analysis.
Respiratory Ciliary Dysfunction
2008, Pediatric Respiratory MedicineExhaled breath condensate in children: Present knowledge and future prospects
2008, Journal of Breath Research
This study was supported by the European and Hungarian Respiratory Society, the Hungarian Immunology and Allergology Society (Hungary), and the National Heart and Lung Institute (UK).