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Respiratory Medicine

Lead contact: Dr Paul Aurora

Introduction 

Respiratory illness is the single most common cause of paediatric admission to NHS hospitals, accounting for 25% of neonatal deaths and 10% of all infant deaths.  One child in ten is affected by asthma, while cystic fibrosis (CF) is the most common lethal inherited condition in the United Kingdom.  The paediatric respiratory service at GOSH encounters a large number of rare clinical conditions and research projects have been established to look in detail at the pathophysiology, clinical course, effective therapy and functional impairments in some of these conditions.  In addition, the Portex Unit has an outstanding record for the development and validation of lung function tests for use in infants and young children, and the application of these measurements in epidemiological and clinical research studies, and the respiratory medicine service collaborates closely with this work. Finally, the hospital is home to many world class paediatric speciality services, and the respiratory unit collaborates closely with numerous research projects in other patient groups. In particular, collaborative research with the paediatric cardiothoracic transplant service is currently very active. Dr P Aurora is the academic lead for the department, but all senior staff are actively involved in the department’s research programme.  

Aims 

• To increase the understanding of the pathophysiology of lung disease in children
• To develop new therapeutic strategies for lung disease in children

Current and Recent Projects

• Longitudinal monitoring of very young children diagnosed with CF, with the aim of developing new outcome measures for the detection of early lung disease, and then utilising these outcome measures in therapeutic trials. Much of this ground-breaking research is part of a pan-London collaboration, and is co-ordinated through the Portex Unit.
• Longitudinal study of young children with asthma, and correlation of subtle physiological change with evidence of inflammation and structural change. The aim of this work is to develop a better understanding of the pathophysiology of early childhood asthma, with the aim of developing and refining new treatments. As with the CF work, this research is part of a developing multicentre collaboration, and co-ordinated through the Portex Unit.
• Establishment of a national registry for children with rare 'Orphan' lung disease (see www.bpold.co.uk)
• A longitudinal study of the impact of viral infections following lung transplantation. This multicentre international study is funded by the National Institutes of Health (NIH) in the US
• A multicentre international study of the impact of paediatric lung transplantation on health related quality of life in CF. This study is also funded by the NIH.
• Comparison of urokinase versus primary video-assisted thoroscopic surgery for the treatment of empyema in children. (3 yr study).  This is the only prospective evidence based study to guide treatment in this disease.  By recommending urokinase treatment in children with these conditions there is the potential saving to the NHS of £1.2 million.
• The treatment of upper airway obstruction secondary to Pierre-Robin sequence with nasopharygeal airways
• Ciliary function in children following lung transplantation (joint work with the University of Leicester)
• Signalling pathways of macrolide antibiotics
• Assessing the impact of hypoxia in children with cystic fibrosis
• The impact of gastro-oesophageal reflux upon outcomes following lung transplantation, and how to best measure and treat this complication
• Lung transplantation in high risk children with severe pulmonary hypertension and children with CF with unusual infections
• Measurement of non-invasive markers of inflammation on the intensive care unit
• Physiological and structural lung disease in children with connective tissue disease
• Physiological and structural lung disease in children following stem cell transplantation
• 8-isoprostane and exhaled nitric oxide in children with atopic dermatitis
• Stem cell engraftment in the human and mouse lung
• Assessment of body composition in cystic fibrosis
• Assessing the role of vitamins D and K on bone disease in cystic fibrosis
• The use of inhaled dry powdered mannitol in cystic fibrosis
• The effect of hypoxia in sickle cell disease     

Current and Recent Higher Degree Submissions 

The unit always has a number of students undertaking research degrees. These are usually medical trainees, but also occasionally trainees from other disciplines. Recent submissions & awards include

• Dr Samatha Sonnappa (PhD thesis awarded 2009) for her work on lung function abnormalities in preschool children with asthma

Involvement in National and International Scientific and Professional Organisations 

Members of the department are active in a number of scientific and professional organisations, where they have responsibility for stimulating collaborative research, and developing agreed standards for both research and clinical practice. These organisations include the British Paediatric Respiratory Society (Dr C Wallis), the Association of Respiratory Technicians and Physiologists (Mrs E Fettes), the European Respiratory Society (Dr P Aurora), and The International Society of Heart and Lung Transplantation (Dr P Aurora).