International consensus statement on routine blood testing in primary ciliary dyskinesia
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064203%3A_____%2F25%3A10499327" target="_blank" >RIV/00064203:_____/25:10499327 - isvavai.cz</a>
Alternative codes found
RIV/00216208:11130/25:10499327
Result on the web
<a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=8RIIa5RHyu" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=8RIIa5RHyu</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1183/23120541.01071-2024" target="_blank" >10.1183/23120541.01071-2024</a>
Alternative languages
Result language
angličtina
Original language name
International consensus statement on routine blood testing in primary ciliary dyskinesia
Original language description
BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterised by dysfunction of motile cilia. Symptoms include recurrent and chronic airway infections which can lead to deteriorating lung function and inflammatory destructive lung disease in the form of persistent atelectasis and bronchiectasis. Routine blood testing may be used as a tool for disease monitoring and management. However, currently there are no consensus-based guidelines within the field of PCD. BEAT-PCD together with the ERN-LUNG PCD-Clinical Trial Network aimed to develop an international expert consensus statement on which routine blood tests should be conducted in patients with PCD. METHODS: 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, which was defined as >=80% agreement for each statement. Two patient representatives were included in the consensus process. RESULTS: The expert panel reached consensus on 51 out of 101 statements (50%) on routine blood testing in children and adults with PCD to be performed at diagnosis, annually and on exacerbation. The statements include biomarkers for inflammation, haemoglobin, iron status, vitamin D, immune function, inhalant allergies, liver and kidney function, and allergic bronchopulmonary aspergillosis. CONCLUSIONS: This is the first international consensus on routine blood testing in PCD. It highlights blood tests that may be relevant to perform at diagnosis, annually and on exacerbation in people with PCD. Further research on the clinical usefulness of routine blood testing in PCD is needed.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30209 - Paediatrics
Result continuities
Project
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Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
Others
Publication year
2025
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
ERJ Open Research
ISSN
2312-0541
e-ISSN
2312-0541
Volume of the periodical
11
Issue of the periodical within the volume
3
Country of publishing house
GB - UNITED KINGDOM
Number of pages
11
Pages from-to
01071-2024
UT code for WoS article
001523572100001
EID of the result in the Scopus database
2-s2.0-105009080106