National Heart & Lung Institute (NHLI)

Respiratory Paediatrics

Professor Andrew Bush, Head of Group

The Paediatric respiratory group is focused on physiological work within the department and a wide range of collaborative programs within NHLI, Imperial College and beyond.

Our physiological interests include the use of respiratory mass spectrometry to measure lung growth non-invasively. Collaborative programs include the measurement of inflammation in airways disease, and clinical trial end points and pathophysiology of cystic fibrosis.

Our focus is translational research, using our clinical base to generate ideas, linking with basic science to develop them, and bringing the results back to the clinic. An obvious exemplar of this was our trial of Azithromycin in children with cystic fibrosis, the first paediatric trial in the world to establish the value of azithromycin in this age group.

Current projects include:

Current projects

Early airway pathology in infant wheeze.

We are using endobronchial biopsy and lavage studies to determine the earliest pathological changes in preschool wheeze, in order to determine the time window between onset of symptoms and the development of structural airway wall changes. We are studying two groups of children – very early wheeze (one year of age) studied in collaboration with Dr Haahtela in Finland; and a second group (median age four years) with severe and established wheeze referred to the Royal Brompton Hospital. This work also involves determining the normal evolution of airway wall structural wall development, in collaboration with Dr Alison Hislop at the Institute of Child Health. Funding is from Asthma UK.

The use of inflammatory markers to guide therapy in asthma.

We have previously shown that sputum eosinophils and exhaled nitric oxide refine the clinical decision making as to whether to reduce inhaled corticosteroids in aymptomatic children with asthma. We are progressing to a study in severe asthma in which we will randomise 80 children with severe asthma into a group treated by standard means, and a group whose therapy is determined using inflammatory markers (induced sputum, exhaled breath condensate), to see if therapy can be reduced without loss of asthma control. Funding is from the British Lung Foundation.

Early airway changes in CF.

We are using bronchoscopy, bronchial biopsy and airway brushing to determine the very earliest changes in the CF airway, jointly with Gene Therapy. Children undergo bronchoscopy as part of their routine diagnostic work up, and this includes an endobronchial biopsy. We are also involved in studies of airway inflammation and novel mediators, jointly with Leukocyte Biology, and the role of different strains of Pseudomonas aeruginosa, jointly with Dr Ty Pitt, PHLS, Colindale. Funding is from the CF Trust.

Evolution of pulmonary function changes over time in infants with Cystic Fibrosis.

This is a long running, pan-London collaboration, based at the Institute of Child Health (Prof. Janet Stocks) in which cohorts of children with cystic fibrosis are being studied from infancy to mid-childhood  using the raised volume squeeze technique, lung clearance index (infants) and lung clearance index, plethysmography and spirometry in the preschool child. Funding is from the CF Trust.

The use of respiratory mass spectrometry in the assessment of alveolar growth.

We have studied the effects of congenital heart disease and preterm birth on alveolar growth using CO18. We are now studying the effects of sickle cell disease on alveolar growth and development using the same techniques. We are further developing the techniques to study peripheral muscle metabolism during exercise.

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