Skip to main content

14.02.2019 | AIRWAY BIOLOGY

Proof of Concept: Very Rapid Tidal Breathing Nasal Nitric Oxide Sampling Discriminates Primary Ciliary Dyskinesia from Healthy Subjects

verfasst von: Mathias G. Holgersen, June K. Marthin, Kim G. Nielsen

Erschienen in: Lung | Ausgabe 2/2019

Einloggen, um Zugang zu erhalten

Abstract

Introduction

Nasal nitric oxide (nNO) is extremely low in individuals with primary ciliary dyskinesia (PCD) and is recommended as part of early workup. We investigated whether tidal breathing sampling for a few seconds was as discriminative between PCD and healthy controls (HC) as conventional tidal breathing sampling (cTB-nNO) for 20–30 s.

Methods

We performed very rapid sampling of tidal breathing (vrTB-nNO) for 2, 4 and 6 s, respectively. Vacuum sampling with applied negative pressure (vrTB-nNOvac; negative pressure was applied by pinching the sampling tube) for < 2 s resulted in enhanced suction of nasal air during measurement. Feasibility, success rate, discriminatory capacity, repeatability and agreement were assessed for all four sampling modalities.

Results

We included 13 patients with PCD, median (IQR) age of 21.8 (12.2–27.7) years and 17 HC, 25.3 (14.5–33.4) years. Measurements were highly feasible (96.7% success rate). Measured NO values with vrTB-nNO modalities differed significantly from TB-nNO measurements (HC: p < 0.001, PCD: p < 0.05). All modalities showed excellent discrimination. The vacuum method gave remarkably high values of nNO in both groups (1865 vs. 86 ppb), but retained excellent discrimination. vrTB-nNO4sec, vrTB-nNO6sec and vrTB-nNOvac showed identical specificity to cTB-nNO (all: 1.0, 95% CI 0.77–1.0).

Conclusion

vrTB-nNO sampling requires only a few seconds of probe-in-nose time, is feasible, and provides excellent discrimination between PCD and HC. Rapid TB-nNO sampling needs standardisation and further investigations in infants, young children and patients referred for PCD workup.
Anhänge
Nur mit Berechtigung zugänglich
Literatur
2.
Zurück zum Zitat Brown D, Pittman J, Leigh M, Fordham L, Davis S (2008) Early lung disease in young children with primary ciliary dyskinesia. Pediatr Pulmonol 43(5):514–516CrossRefPubMed Brown D, Pittman J, Leigh M, Fordham L, Davis S (2008) Early lung disease in young children with primary ciliary dyskinesia. Pediatr Pulmonol 43(5):514–516CrossRefPubMed
3.
Zurück zum Zitat Marthin J, Petersen N, Skovgaard L, Nielsen K (2010) Lung function in patients with primary ciliary dyskinesia: a cross-sectional and 3-decade longitudinal study. Am J Respir Crit Care Med 181(11):1262–1268CrossRefPubMed Marthin J, Petersen N, Skovgaard L, Nielsen K (2010) Lung function in patients with primary ciliary dyskinesia: a cross-sectional and 3-decade longitudinal study. Am J Respir Crit Care Med 181(11):1262–1268CrossRefPubMed
4.
Zurück zum Zitat Goutaki M, Meier A, Halbeisen F, Lucas J, Dell S, Maurer E et al (2016) Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis. Eur Respir J 48(4):1081–1095CrossRefPubMed Goutaki M, Meier A, Halbeisen F, Lucas J, Dell S, Maurer E et al (2016) Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis. Eur Respir J 48(4):1081–1095CrossRefPubMed
5.
Zurück zum Zitat Behan L, Dimitrov BD, Kuehni CE, Hogg C, Carroll M, Evans HJ et al (2016) PICADAR: a diagnostic predictive tool for primary ciliary dyskinesia. Eur Respir J 47(4):1103–1112CrossRefPubMedPubMedCentral Behan L, Dimitrov BD, Kuehni CE, Hogg C, Carroll M, Evans HJ et al (2016) PICADAR: a diagnostic predictive tool for primary ciliary dyskinesia. Eur Respir J 47(4):1103–1112CrossRefPubMedPubMedCentral
6.
Zurück zum Zitat Kuehni C, Frischer T, Strippoli M, Maurer E, Bush A, Nielsen K et al (2010) Factors influencing age at diagnosis of primary ciliary dyskinesia in European children. Eur Respir J 36(6):1248–1258CrossRefPubMed Kuehni C, Frischer T, Strippoli M, Maurer E, Bush A, Nielsen K et al (2010) Factors influencing age at diagnosis of primary ciliary dyskinesia in European children. Eur Respir J 36(6):1248–1258CrossRefPubMed
7.
Zurück zum Zitat Behan L, Dunn Galvin A, Rubbo B, Masefield S, Copeland F, Manion M et al (2016) Diagnosing primary ciliary dyskinesia: an international patient perspective. Eur Respir J 48(4):1096–1107CrossRefPubMedPubMedCentral Behan L, Dunn Galvin A, Rubbo B, Masefield S, Copeland F, Manion M et al (2016) Diagnosing primary ciliary dyskinesia: an international patient perspective. Eur Respir J 48(4):1096–1107CrossRefPubMedPubMedCentral
8.
Zurück zum Zitat Shapiro A, Davis S, Polineni D, Manion M, Rosenfeld M, Dell S et al (2018) Diagnosis of primary ciliary dyskinesia. An official American Thoracic Society Clinical Practice guideline. Am J Respir Crit Care Med 197(12):1535–4970CrossRef Shapiro A, Davis S, Polineni D, Manion M, Rosenfeld M, Dell S et al (2018) Diagnosis of primary ciliary dyskinesia. An official American Thoracic Society Clinical Practice guideline. Am J Respir Crit Care Med 197(12):1535–4970CrossRef
10.
Zurück zum Zitat Beydon N, Chambellan A, Alberti C, de Blic J, Clement A, Escudier E et al (2015) Technical and practical issues for tidal breathing measurements of nasal nitric oxide in children. Pediatr Pulmonol 50(12):1374–1382CrossRefPubMed Beydon N, Chambellan A, Alberti C, de Blic J, Clement A, Escudier E et al (2015) Technical and practical issues for tidal breathing measurements of nasal nitric oxide in children. Pediatr Pulmonol 50(12):1374–1382CrossRefPubMed
11.
Zurück zum Zitat Marthin J, Nielsen K (2011) Choice of nasal nitric oxide technique as first-line test for primary ciliary dyskinesia. Eur Respir J 37(3):559–565CrossRefPubMed Marthin J, Nielsen K (2011) Choice of nasal nitric oxide technique as first-line test for primary ciliary dyskinesia. Eur Respir J 37(3):559–565CrossRefPubMed
12.
Zurück zum Zitat American Thoracic Society, European Respiratory Society (2005) ATS/ERS recommendations for standardized procedures for the online and offline measurement of exhaled lower respiratory nitric oxide and nasal nitric oxide, 2005. Am J Respir Crit Care Med 171(8):912–930CrossRef American Thoracic Society, European Respiratory Society (2005) ATS/ERS recommendations for standardized procedures for the online and offline measurement of exhaled lower respiratory nitric oxide and nasal nitric oxide, 2005. Am J Respir Crit Care Med 171(8):912–930CrossRef
13.
Zurück zum Zitat Leigh M, Hazucha M, Chawla K, Baker B, Shapiro A, Brown D et al (2013) Standardizing nasal nitric oxide measurement as a test for primary ciliary dyskinesia. Ann Am Thorac Soc 10(6):574–581CrossRefPubMedPubMedCentral Leigh M, Hazucha M, Chawla K, Baker B, Shapiro A, Brown D et al (2013) Standardizing nasal nitric oxide measurement as a test for primary ciliary dyskinesia. Ann Am Thorac Soc 10(6):574–581CrossRefPubMedPubMedCentral
14.
Zurück zum Zitat Shapiro A, Josephson M, Rosenfeld M, Yilmaz O, Davis S, Polineni D et al (2017) Accuracy of nasal nitric oxide measurement as a diagnostic test for primary ciliary dyskinesia. A systematic review and meta-analysis. Ann Am Thorac Soc 14(7):1184–1196PubMedPubMedCentral Shapiro A, Josephson M, Rosenfeld M, Yilmaz O, Davis S, Polineni D et al (2017) Accuracy of nasal nitric oxide measurement as a diagnostic test for primary ciliary dyskinesia. A systematic review and meta-analysis. Ann Am Thorac Soc 14(7):1184–1196PubMedPubMedCentral
16.
Zurück zum Zitat Mateos-Corral D, Coombs R, Grasemann H, Ratjen F, Dell SD (2011) Diagnostic value of nasal nitric oxide measured with non-velum closure techniques for children with primary ciliary dyskinesia. J Pediatr 159(3):420–424CrossRefPubMed Mateos-Corral D, Coombs R, Grasemann H, Ratjen F, Dell SD (2011) Diagnostic value of nasal nitric oxide measured with non-velum closure techniques for children with primary ciliary dyskinesia. J Pediatr 159(3):420–424CrossRefPubMed
17.
Zurück zum Zitat Knowles M, Ostrowski L, Leigh M, Sears PR, Davis S, Wolf W et al (2014) Mutations in RSPH1 cause primary ciliary dyskinesia with a unique clinical and ciliary phenotype. Am J Respir Crit Care Med 189(6):707–717CrossRefPubMedPubMedCentral Knowles M, Ostrowski L, Leigh M, Sears PR, Davis S, Wolf W et al (2014) Mutations in RSPH1 cause primary ciliary dyskinesia with a unique clinical and ciliary phenotype. Am J Respir Crit Care Med 189(6):707–717CrossRefPubMedPubMedCentral
18.
Zurück zum Zitat Frommer A, Hjeij R, Loges N, Edelbusch C, Jahnke C, Raidt J et al (2015) Immunofluorescence analysis and diagnosis of primary ciliary dyskinesia with radial spoke defects. Am J Respir Cell Mol Biol 53(4):563–573CrossRefPubMedPubMedCentral Frommer A, Hjeij R, Loges N, Edelbusch C, Jahnke C, Raidt J et al (2015) Immunofluorescence analysis and diagnosis of primary ciliary dyskinesia with radial spoke defects. Am J Respir Cell Mol Biol 53(4):563–573CrossRefPubMedPubMedCentral
20.
Zurück zum Zitat Krouwer J (2008) Why Bland-Altman plots should use X, not (Y + X)/2 when X is a reference method. Stat Med 27(5):778–780CrossRefPubMed Krouwer J (2008) Why Bland-Altman plots should use X, not (Y + X)/2 when X is a reference method. Stat Med 27(5):778–780CrossRefPubMed
21.
Zurück zum Zitat Marthin J, Nielsen K (2013) Hand-held tidal breathing nasal nitric oxide measurement a promising targeted case-finding tool for the diagnosis of primary ciliary dyskinesia. PLoS ONE 8(2):e57262CrossRefPubMedPubMedCentral Marthin J, Nielsen K (2013) Hand-held tidal breathing nasal nitric oxide measurement a promising targeted case-finding tool for the diagnosis of primary ciliary dyskinesia. PLoS ONE 8(2):e57262CrossRefPubMedPubMedCentral
Metadaten
Titel
Proof of Concept: Very Rapid Tidal Breathing Nasal Nitric Oxide Sampling Discriminates Primary Ciliary Dyskinesia from Healthy Subjects
verfasst von
Mathias G. Holgersen
June K. Marthin
Kim G. Nielsen
Publikationsdatum
14.02.2019
Verlag
Springer US
Erschienen in
Lung / Ausgabe 2/2019
Print ISSN: 0341-2040
Elektronische ISSN: 1432-1750
DOI
https://doi.org/10.1007/s00408-019-00202-x

Leitlinien kompakt für die Innere Medizin

Mit medbee Pocketcards sicher entscheiden.

Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag

Update Innere Medizin

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.