A BEAT-PCD ERS CRC consensus statement for routine blood testing in primary ciliary dyskinesia in collaboration with PCD-Clinical Trial Network

<bold>Background:</bold> Primary Ciliary Dyskinesia (PCD) is a rare genetic disorder characterized by dysfunction of motile cilia. Common symptoms include recurrent and chronic airway infection which can result in deteriorating obstructive lung function, destroyed and chronically collapsed lobar segments, and bronchiectasis. Routine blood testing may be used as a tool for disease monitoring and management, however currently there are no guidelines or consensus within the field of PCD. We aimed to develop an international expert consensus statement on which routine blood tests should be conducted in patients with PCD. <bold>Methods:</bold> An international panel of 33 PCD experts from 17 countries was established to generate consensus on routine blood testing in PCD. A modified Delphi technique with three e-survey rounds was used to reach consensus. Consensus was reached on statements if there was at least 80% agreement. Patient representatives were included in the Delphi process. <bold>Results:</bold> The expert panel reached consensus on 50 statements on routine blood testing in adults and children with PCD at diagnosis, annually and at exacerbation. Agreement on statements ranged from 80% to 100%. The statements include biomarkers for inflammation, haemoglobin, iron status, vitamin D, immune function, inhalant allergies, liver and kidney function, and allergic bronchopulmonary aspergillosis (ABPA). <bold>Conclusion:</bold> This is the first international consensus on routine blood testing in PCD. It highlights which blood tests may be relevant to conduct at diagnosis, annually and at exacerbation in PCD patients. Further research on the clinical usefulness of routine blood testing in PCD is needed.

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Published
2024-09-14
DOI
https://doi.org/10.1183/13993003.congress-2024.pa4834
Primary Topic
Cystic Fibrosis Research Advances
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article
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article

A BEAT-PCD ERS CRC consensus statement for routine blood testing in primary ciliary dyskinesia in collaboration with PCD-Clinical Trial Network

Pinelopi Anagnostopoulou, Heymut Omran, Francesca Santamaria, Michael R. Loebinger et al.
Cystic Fibrosis Research Advances
article

A BEAT-PCD ERS CRC consensus statement for routine blood testing in primary ciliary dyskinesia in collaboration with PCD-Clinical Trial Network

Pinelopi Anagnostopoulou, Heymut Omran, Francesca Santamaria, Michael R. Loebinger, Mieke Boon, Eleonora Dehlink, Philippe Reix, Nicola Ullmann, Panayiotis K. Yiallouros, D. Peckham, Philip Robinson, Silvia Castillo, Kim G. Nielsen, Felix C. Ringshausen, S.B. Carr, Anne Schlegtendal, Lucy Morgan, Bernard Maître, J Nielsen, Petr Pohunek, Christina Kavouridou, Guillaume Thouvenin, Bulent Taner Karadag, Henryk Mazurek, Tavs Qvist, W. Thomson Walker, J. Duckers, Sarah Altaraihi, Eric G. Haarman, Damien M.S. Destouches, Johanna Raidt, Evie Robson, Massimo Pifferi, Uğur Özçelik, Suzanne Crowley, Jobst Röhmel
article en

Abstract

<bold>Background:</bold> Primary Ciliary Dyskinesia (PCD) is a rare genetic disorder characterized by dysfunction of motile cilia. Common symptoms include recurrent and chronic airway infection which can result in deteriorating obstructive lung function, destroyed and chronically collapsed lobar segments, and bronchiectasis. Routine blood testing may be used as a tool for disease monitoring and management, however currently there are no guidelines or consensus within the field of PCD. We aimed to develop an international expert consensus statement on which routine blood tests should be conducted in patients with PCD. <bold>Methods:</bold> An international panel of 33 PCD experts from 17 countries was established to generate consensus on routine blood testing in PCD. A modified Delphi technique with three e-survey rounds was used to reach consensus. Consensus was reached on statements if there was at least 80% agreement. Patient representatives were included in the Delphi process. <bold>Results:</bold> The expert panel reached consensus on 50 statements on routine blood testing in adults and children with PCD at diagnosis, annually and at exacerbation. Agreement on statements ranged from 80% to 100%. The statements include biomarkers for inflammation, haemoglobin, iron status, vitamin D, immune function, inhalant allergies, liver and kidney function, and allergic bronchopulmonary aspergillosis (ABPA). <bold>Conclusion:</bold> This is the first international consensus on routine blood testing in PCD. It highlights which blood tests may be relevant to conduct at diagnosis, annually and at exacerbation in PCD patients. Further research on the clinical usefulness of routine blood testing in PCD is needed.

University of Pisa (IT), Oslo University Hospital (NO), Cardiff and Vale University Health Board (GB), University of Leeds (GB), Royal Children's Hospital (AU), Inserm (FR), Leeds Teaching Hospitals NHS Trust (GB), University Hospitals of the Ruhr-University of Bochum (DE), Sahlgrenska University Hospital (SE), Copenhagen University Hospital (DK), Rigshospitalet (DK), University Hospital Southampton NHS Foundation Trust (GB), University of Cyprus (CY), Medizinische Hochschule Hannover (DE), Humboldt-Universität zu Berlin (DE), Sorbonne Université (FR), Hôpital Armand-Trousseau (FR), Hospital Clínico Universitario de Valencia (ES), Royal Brompton Hospital (GB), Assistance Publique – Hôpitaux de Paris (FR), University Hospital in Motol (CZ), Hospices Civils de Lyon (FR), Lung Institute (US), University Hospital Münster (DE), Federico II University Hospital (IT), National Center for Tuberculosis and Lung Disease (GE), Bambino Gesù Children's Hospital (IT), Centre de Recherche Saint-Antoine (FR), Bochum University of Applied Sciences (DE), Archbishop Makarios III Hospital (CY), Amsterdam University Medical Centers (NL), Hôpital Intercommunal de Créteil (FR), Public Risk Management Association (US), Azienda Ospedaliera Universitaria Pisana (IT), Hacettepe University (TR), Marmara University (TR), Medical University of Vienna (AT), University of Amsterdam (NL), Macquarie University (AU), KU Leuven (BE)
Openalex Percentile: Top 63%
Cystic Fibrosis Research Advances
DOISourceOpenAlex
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