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.
Research type
Research Study
Full 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.
IRAS ID
253667
Contact name
Christopher O'Callaghan
Contact email
Sponsor organisation
UCL Institute of Child Health
Duration of Study in the UK
1 years, 6 months, 1 days
Research summary
The airways we breathe through and our nasal passages and sinuses are lined by cells. Growing out of these cells are millions and millions of microscopic hairs called cilia. The cilia are motile, each beating over a million times a day clearing mucus and particles and pathogens we inhale. Primary ciliary dyskinesia (PCD) is a rare inherited disease that stops cilia beating properly leading to recurrent infection and lung damage called bronchiectasis and nasal and sinus disease. PCD is very poorly understood and there is virtually no research on which to base clinical care. Our current management is based on that for Cystic fibrosis which is likely to be inappropriate as the underlying defects causing these diseases are so different.
This study aims to improve our understanding of PCD and how it differs from Cystic fibrosis. Cough is essential in PCD to clear mucus from the lungs and is present every day. Using a cough monitor we will record overnight cough in PCD and Cystic fibrosis patients and in healthy controls to see if this helps us to determine if cough may predict an exacerbation of lung disease. We will also determine if the amount of coughing is different between the groups when they are well. By collecting secretions from the nose using simple filter paper and sputum that is coughed up we will measure levels of inflammation and determine if there is infection as infection and inflammation may predispose to cough. To help subjects cough up we ask them to inhale some salty water. This is routinely done in our clinics.
The results from this study will help guide further research on the possible use of cough monitors to recognise the earliest stage of a respiratory exacerbation that may allow early assessment and treatment. It will also allow us to determine if the airways of PCD patients are inflamed in the absence of infection which may alter the way we treat them.REC name
London - Bloomsbury Research Ethics Committee
REC reference
18/LO/2085
Date of REC Opinion
13 Dec 2018
REC opinion
Favourable Opinion