Inflammation in Primary Ciliary Dyskinesia: An exploratory study
Research type
Research Study
Full title
Inflammation in Primary Ciliary Dyskinesia: An exploratory study
IRAS ID
217025
Contact name
Christopher O'Callaghan
Contact email
Sponsor organisation
Division of Research and Innovation
Duration of Study in the UK
4 years, 11 months, 31 days
Research summary
Children and adults with chronic respiratory disease often develop permanent lung damage called bronchiectasis. Worryingly an underlying cause is only identified in approximately 50% of those affected.
This project will focus on a condition called Primary Ciliary Dyskinesia that is a major cause of bronchiectasis. It is an inherited disease of the structure and function of the millions of motile cilia that line the respiratory tract. These microscopic hair like projections beat over a million times a day clearing mucus, inhaled particles and pathogens. In PCD the dyskinetically beating cilia cannot clear mucus. This leads to chronic infection and bronchiectasis. In the upper airway, patients suffer chronic nasal and sinus problems, recurrent middle ear infections and hearing loss.
Extremely little is known about the disease processes in PCD and there is no evidence on which to base treatment. Current management is largely extrapolated from Cystic Fibrosis care which may not be ideal as the pathophysiology of these diseases are very different. An extensive exploration of the pathophysiology of PCD is required.
We aim to gain an understanding of inflammation of the respiratory tract in PCD and to document the clinical features associated with this, in particular, clinical signs and cough.
We will recruit children managed by Leeds, Leicester and Birmingham Paediatric National PCD Services, adults who attend Leeds and Leicester PCD service, or patients diagnosed by the nationally funded PCD diagnostic service which includes children attending Great Ormond Street Hospital. We will recruit a healthy volunteer control group and a Cystic Fibrosis comparator group.
This project is designed to run over a five-year period to ensure adequate time to complete and reduce burden on participants.
REC name
London - Bloomsbury Research Ethics Committee
REC reference
18/LO/1227
Date of REC Opinion
8 Aug 2018
REC opinion
Unfavourable Opinion