Robson, Evelyn Alexandra;
(2024)
An exploratory study to investigate Inflammation and Cough in paediatric and adult patients with Primary Ciliary Dyskinesia, a comparison with Cystic Fibrosis and Healthy individuals.
Doctoral thesis (M.D(Res)), UCL (University College London).
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Abstract
Background: Primary Ciliary Dyskinesia (PCD) is a rare autosomal recessive disease characterised by abnormal ciliary function, leading to recurrent respiratory tract infections and bronchiectasis. Current management is largely based on that for CF although the pathophysiology of these conditions is very different. Two possible objective measures of interest to our group were nasal inflammatory mediators and cough monitoring. Night time cough frequency and nasal inflammatory mediators were explored in adults and children attending Leeds teaching hospitals for management of PCD (n=23), compared with Cystic fibrosis (CF, n=27)) and healthy controls (HC, n=21) during respiratory exacerbations and at baseline health. Methods: A prospective exploratory study was completed using validated techniques including Leicester cough monitor for cough frequency (measured as coughs per hour (c/hr)) and Mesoscale discovery (MSD) nasosorption kit to assess a pro-inflammatory panel of cytokines from the nose, including: IFN-gamma, IL1-beta, IL2, IL4, IL6, IL8, IL10, IL12p70, IL13, TNF-alpha. Results: The North of England PCD cohort included 145 children (<18 years) and 100 adults (>18 years). Adults with CF had higher baseline cough frequency (3.4c/hr) than adults with PCD (0.69c/hr). Children with PCD had higher baseline cough frequency (0.82c/hr) than children with CF (0.14c/hr) and HC (0.16c/hr). All measured pro-inflammatory cytokines were higher in PCD, compared to CF and HC at baseline health. A decrease in cough frequency from the start of a significant exacerbation to 4 weeks after was noted for both PCD and CF (4.3c/hr to 1.05c/hr for PCD and 3.98c/hr to 1.43c/hr for CF). A clear reduction in levels of inflammatory mediators were reported in PCD, with an increase back to baseline after 4 weeks. Conclusion: These findings highlight clinically significant differences between PCD and CF, emphasising the importance of not extrapolating evidence for CF management into PCD. Both cough frequency monitoring and nasal sampling for upper airway inflammation could be used as objective markers of disease, with further exploration in larger national work.
| Type: | Thesis (Doctoral) |
|---|---|
| Qualification: | M.D(Res) |
| Title: | An exploratory study to investigate Inflammation and Cough in paediatric and adult patients with Primary Ciliary Dyskinesia, a comparison with Cystic Fibrosis and Healthy individuals |
| Open access status: | An open access version is available from UCL Discovery |
| Language: | English |
| Additional information: | Copyright © The Author 2022. Original content in this thesis is licensed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International (CC BY-NC 4.0) Licence (https://creativecommons.org/licenses/by-nc/4.0/). Any third-party copyright material present remains the property of its respective owner(s) and is licensed under its existing terms. Access may initially be restricted at the author’s request. |
| UCL classification: | UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health UCL |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10193555 |
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