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Nasal nitric oxide measurement in children for the diagnosis of primary ciliary dyskinesia: European Respiratory Society technical standard

Nasal nitric oxide measurement in children for the diagnosis of primary ciliary dyskinesia: European Respiratory Society technical standard
Nasal nitric oxide measurement in children for the diagnosis of primary ciliary dyskinesia: European Respiratory Society technical standard

Nasal nitric oxide (nNO) is extremely low in most people with primary ciliary dyskinesia (PCD) and its measurement is an important contributor to making the diagnosis. Existing guidelines and technical standards focus on nNO measurements in older, cooperative children using chemiluminescence analysers. However, measurements of nNO in pre-school-age children (age 2-5 years) may facilitate early diagnosis and electrochemical rather than chemiluminescence analysers are widely used. Pre-schoolers often need different methods to be employed when measuring nNO. Hence, a European Respiratory Society Task Force has developed this technical standard as the first step towards standardising sampling, analysis and reporting of nNO measured as part of the diagnostic testing for PCD in all age groups, including preschool-age children. Furthermore, we considered both chemiluminescence and electrochemical analysers that are in use worldwide. There was a paucity of quality evidence for electrochemical analysers and sampling methods used in young children, and the Task Force proposes future research priorities to allow updates of this technical standard.

0903-1936
Beydon, Nicole
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Kouis, Panayiotis
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Marthin, June K.
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Latzin, Philipp
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Colas, Murielle
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Davis, Stephanie D.
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Haarman, Eric G.
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Harris, Amanda
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Hogg, Claire
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Kilbride, Emma
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Kuehni, Claudia E.
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Marangu, Diana
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Nielsen, Kim G.
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Prendergrast, Catherine
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Robinson, Phil
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Rumman, Nisreen
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Rutter, Matthew
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Walker, Woolf
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Ferkol, Thomas
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Lucas, Jane
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Beydon, Nicole
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Kouis, Panayiotis
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Marthin, June K.
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Latzin, Philipp
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Colas, Murielle
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Davis, Stephanie D.
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Haarman, Eric G.
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Harris, Amanda
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Hogg, Claire
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Kilbride, Emma
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Kuehni, Claudia E.
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Marangu, Diana
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Nielsen, Kim G.
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Prendergrast, Catherine
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Robinson, Phil
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Rumman, Nisreen
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Rutter, Matthew
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Walker, Woolf
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Ferkol, Thomas
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Lucas, Jane
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Beydon, Nicole, Kouis, Panayiotis, Marthin, June K., Latzin, Philipp, Colas, Murielle, Davis, Stephanie D., Haarman, Eric G., Harris, Amanda, Hogg, Claire, Kilbride, Emma, Kuehni, Claudia E., Marangu, Diana, Nielsen, Kim G., Prendergrast, Catherine, Robinson, Phil, Rumman, Nisreen, Rutter, Matthew, Walker, Woolf, Ferkol, Thomas and Lucas, Jane (2023) Nasal nitric oxide measurement in children for the diagnosis of primary ciliary dyskinesia: European Respiratory Society technical standard. European Respiratory Journal, 61 (4), [2202031]. (doi:10.1183/13993003.02031-2022).

Record type: Article

Abstract

Nasal nitric oxide (nNO) is extremely low in most people with primary ciliary dyskinesia (PCD) and its measurement is an important contributor to making the diagnosis. Existing guidelines and technical standards focus on nNO measurements in older, cooperative children using chemiluminescence analysers. However, measurements of nNO in pre-school-age children (age 2-5 years) may facilitate early diagnosis and electrochemical rather than chemiluminescence analysers are widely used. Pre-schoolers often need different methods to be employed when measuring nNO. Hence, a European Respiratory Society Task Force has developed this technical standard as the first step towards standardising sampling, analysis and reporting of nNO measured as part of the diagnostic testing for PCD in all age groups, including preschool-age children. Furthermore, we considered both chemiluminescence and electrochemical analysers that are in use worldwide. There was a paucity of quality evidence for electrochemical analysers and sampling methods used in young children, and the Task Force proposes future research priorities to allow updates of this technical standard.

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Accepted/In Press date: 10 January 2023
e-pub ahead of print date: 31 January 2023
Published date: 1 April 2023
Additional Information: Funding Information: We are grateful to the ERS Guidelines Working Group for their advice and support. Thank you to Lynn Reeves (University of Southampton, Southampton, UK) for the administrative support for the Task Force. Members of the Task Force are members of BEAT-PCD, an ERS Clinical Research Collaboration, and the European Reference Network for Rare Diseases (ERN-Lung). Funding Information: Support statement: This work was supported by the European Respiratory Society information for this article has been deposited with the Crossref Funder Registry. Funding Information: Conflict of interest: N. Beydon reports educational writing payments as a member of the HERMES examination committee for paediatric respiratory medicine of the European Respiratory Society, and a leadership role as Chair of Group 07.01 of the European Respiratory Society (Paediatric respiratory physiology and sleep), outside the submitted work. P. Latzin reports grants from Vertex and OM Pharma, lecture honoraria from Vertex, Vifor and OM Pharma, and advisory board participation from Polyphor, Santhera (DMC), Vertex, Vifor, OM Pharma and Sanofi Aventis, outside the submitted work. S.D. Davis reports grants from the National Institutes of Health/National Heart, Lung, and Blood Institute, to support the present manuscript, and consulting fees from Parion Sciences and ReCode Therapeutics, and advisory board participation for the PCD Foundation, outside the submitted work. T. Ferkol reports grants from the National Institutes of Health (HL096458) to support the present manuscript, and grants from the National Institutes of Health (HL116211, HL125241, AI146999), National Health and Medical Research Council (NHMRC1043768) and Parion Sciences, advisory board participation for the PCD Foundation, Parion Sciences and Translate Bio, and is past president and executive board member of the American Thoracic Society, outside the submitted work. J.S. Lucas reports grants from AAIR Charity and NIHR Research for patient benefit, and consulting fees from Translate Bio, outside the submitted work. All other authors have nothing to disclose. Publisher Copyright: Copyright ©The authors 2023.

Identifiers

Local EPrints ID: 475043
URI: http://eprints.soton.ac.uk/id/eprint/475043
ISSN: 0903-1936
PURE UUID: b3894e4e-99a3-489e-a38c-011b6aef6153
ORCID for Jane Lucas: ORCID iD orcid.org/0000-0001-8701-9975

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Date deposited: 09 Mar 2023 18:46
Last modified: 17 Mar 2024 07:40

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Contributors

Author: Nicole Beydon
Author: Panayiotis Kouis
Author: June K. Marthin
Author: Philipp Latzin
Author: Murielle Colas
Author: Stephanie D. Davis
Author: Eric G. Haarman
Author: Amanda Harris
Author: Claire Hogg
Author: Emma Kilbride
Author: Claudia E. Kuehni
Author: Diana Marangu
Author: Kim G. Nielsen
Author: Catherine Prendergrast
Author: Phil Robinson
Author: Nisreen Rumman
Author: Matthew Rutter
Author: Woolf Walker
Author: Thomas Ferkol
Author: Jane Lucas ORCID iD

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