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

Nicole Beydon^{1

2

3}, Panayiotis Kouis⁴, June K Marthin⁵, Philipp Latzin⁶, Murielle Colas⁷, Stephanie D Davis⁸, Eric Haarman⁹, Amanda Lea Harris^{10

11}, Claire Hogg¹², Emma Kilbride¹³, Claudia E Kuehni^{6

14}, Diana Marangu¹⁵, Kim G Nielsen^{5

16}, Catherine Pendergrast^{17

18}, Phil Robinson^{19

20

21}, Nisreen Rumman^{22

23}, Matthew Rutter²⁴, Woolf T Walker^{10

11}, Thomas Ferkol⁸, Jane S Lucas^{25

11

3}

Affiliations

¹ AP-HP, Sorbonne Université, Unité Fonctionnelle de Physiologie - Explorations Fonctionnelles Respiratoires et du Sommeil, Hôpital Armand Trousseau, Paris, France.
² Sorbonne Université, INSERM U938, Centre de Recherche Saint-Antoine, Hôpital Saint-Antoine, Paris, France.
³ N. Beydon and J.S. Lucas co-chaired the Task Force and contributed equally.
⁴ Respiratory Physiology Laboratory, Medical School, University of Cyprus, Nicosia, Cyprus.
⁵ Danish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital Rigshospitalet, Copenhagen, Denmark.
⁶ Division of Pediatric Respiratory Medicine and Allergology, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
⁷ Parent Representative, Paris, France.
⁸ Department of Pediatrics, University of North Carolina at Chapel Hill School of Medicine, UNC Children's, Chapel Hill, NC, USA.
⁹ Department of Pediatric Pulmonology, Emma Children's Hospital, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.
¹⁰ Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK.
¹¹ Primary Ciliary Dyskinesia Centre, NIHR Southampton Respiratory Biomedical Research Unit, University of Southampton, Southampton, UK.
¹² Department of Paediatric Respiratory Medicine and Primary Ciliary Dyskinesia Centre, Royal Brompton Hospital, Imperial College London, London, UK.
¹³ Paediatric Respiratory Laboratory, Children's Health Ireland, Dublin, Ireland.
¹⁴ Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
¹⁵ Department of Paediatrics and Child Health, University of Nairobi, Nairobi, Kenya.
¹⁶ Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark.
¹⁷ Lung Function Laboratory, Department of Respiratory and Sleep Medicine, Women's and Children's Hospital, Adelaide, Australia.
¹⁸ Innovation, Implementation and Clinical Translation in Health (IIMPACT) Research Concentration, University of South Australia, Adelaide, Australia.
¹⁹ Department of Respiratory and Sleep Medicine, Royal Children's Hospital, Parkville, Australia.
²⁰ Department of Paediatrics, University of Melbourne, Parkville, Australia.
²¹ Murdoch Children's Research Institute, Parkville, Australia.
²² Department of Pediatrics, Makassed Hospital, Al-Quds University, East Jerusalem, Palestine.
²³ Caritas Baby Hospital, Bethlehem, Palestine.
²⁴ Lung Function Department, Cambridge University Hospitals NHS Foundation Trust, Addenbrooke's Hospital, Cambridge, UK.
²⁵ Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK jlucas1@soton.ac.uk.

PMID: 36822632
DOI: 10.1183/13993003.02031-2022

Free article

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

Nicole Beydon et al. Eur Respir J. 2023.

Free article

. 2023 Apr 20;61(4):2202031.

doi: 10.1183/13993003.02031-2022. Print 2023 Apr.

Authors

Affiliations

¹ AP-HP, Sorbonne Université, Unité Fonctionnelle de Physiologie - Explorations Fonctionnelles Respiratoires et du Sommeil, Hôpital Armand Trousseau, Paris, France.
² Sorbonne Université, INSERM U938, Centre de Recherche Saint-Antoine, Hôpital Saint-Antoine, Paris, France.
³ N. Beydon and J.S. Lucas co-chaired the Task Force and contributed equally.
⁴ Respiratory Physiology Laboratory, Medical School, University of Cyprus, Nicosia, Cyprus.
⁵ Danish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital Rigshospitalet, Copenhagen, Denmark.
⁶ Division of Pediatric Respiratory Medicine and Allergology, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
⁷ Parent Representative, Paris, France.
⁸ Department of Pediatrics, University of North Carolina at Chapel Hill School of Medicine, UNC Children's, Chapel Hill, NC, USA.
⁹ Department of Pediatric Pulmonology, Emma Children's Hospital, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.
¹⁰ Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK.
¹¹ Primary Ciliary Dyskinesia Centre, NIHR Southampton Respiratory Biomedical Research Unit, University of Southampton, Southampton, UK.
¹² Department of Paediatric Respiratory Medicine and Primary Ciliary Dyskinesia Centre, Royal Brompton Hospital, Imperial College London, London, UK.
¹³ Paediatric Respiratory Laboratory, Children's Health Ireland, Dublin, Ireland.
¹⁴ Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
¹⁵ Department of Paediatrics and Child Health, University of Nairobi, Nairobi, Kenya.
¹⁶ Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark.
¹⁷ Lung Function Laboratory, Department of Respiratory and Sleep Medicine, Women's and Children's Hospital, Adelaide, Australia.
¹⁸ Innovation, Implementation and Clinical Translation in Health (IIMPACT) Research Concentration, University of South Australia, Adelaide, Australia.
¹⁹ Department of Respiratory and Sleep Medicine, Royal Children's Hospital, Parkville, Australia.
²⁰ Department of Paediatrics, University of Melbourne, Parkville, Australia.
²¹ Murdoch Children's Research Institute, Parkville, Australia.
²² Department of Pediatrics, Makassed Hospital, Al-Quds University, East Jerusalem, Palestine.
²³ Caritas Baby Hospital, Bethlehem, Palestine.
²⁴ Lung Function Department, Cambridge University Hospitals NHS Foundation Trust, Addenbrooke's Hospital, Cambridge, UK.
²⁵ Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK jlucas1@soton.ac.uk.

PMID: 36822632
DOI: 10.1183/13993003.02031-2022

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 pre-school-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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Conflict of interest statement

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.

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

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

Authors

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Abstract

Conflict of interest statement

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