Stephen C Bourke

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BACKGROUND Few patients with amyotrophic lateral sclerosis currently receive non-invasive ventilation (NIV), reflecting clinical uncertainty about the role of this intervention. We aimed to assess the effect of NIV on quality of life and survival in amyotrophic lateral sclerosis in a randomised controlled trial. METHODS 92 of 102 eligible patients(More)
BACKGROUND Noninvasive ventilation (NIV) probably improves survival in ALS, but the magnitude and duration of any improvement in quality of life (QoL) and the optimal criteria for initiating treatment are unclear. METHODS QoL (Short Form-36 [SF-36], Chronic Respiratory Disease Questionnaire, Sleep Apnea Quality of Life Index) and respiratory function were(More)
BACKGROUND Despite exacerbations of chronic obstructive pulmonary disease (COPD) being both common and often fatal, accurate prognostication of patients hospitalised with an exacerbation is difficult. For exacerbations complicated by pneumonia, the CURB-65 prognostic tool is frequently used but its use in this population is suboptimal. METHODS Consecutive(More)
UNLABELLED We describe our institutional efficacy experience of azithromycin 250 mg thrice weekly in adult non-cystic fibrosis bronchiectasis. METHODS Eligibility criteria for prophylactic azithromycin included 3 exacerbations requiring rescue antibiotics over the previous 6 months. The clinical records of 56 bronchiectasis patients on azithromycin were(More)
Erdheim-Chester disease is a rare multisystem histiocytic disorder in which death most commonly results from pulmonary or cardiac involvement. We report the case history of a 55 year old man with this condition with extensive and progressive pulmonary disease. He had no response to prednisolone alone, but treatment with prednisolone plus cyclophosphamide(More)
BACKGROUND Most patients with ALS have evidence of respiratory muscle weakness at diagnosis, and death is usually due to respiratory failure. Sleep disruption, possibly due to apneas, hypopneas, orthopnea, or REM-related desaturation, is common. The relative impact of these factors on quality of life has not been established. METHODS The authors recruited(More)
Respiratory muscle weakness in neuromuscular disease causes significant morbidity and mortality. The published data on respiratory muscle activity and breathing during sleep in normal subjects, the impact of respiratory muscle weakness on sleep and breathing and the relations to daytime respiratory function in neuromuscular disease are reviewed here. In(More)
BACKGROUND Bronchiectasis is the outcome of a number of different airway insults. Very few studies have characterised the aetiology and utility of a dedicated screening proforma in adult patients attending a general bronchiectasis clinic. METHODS A prospective observational study of 189 bronchiectasis patients attending two centres in the North East of(More)
Cryptogenic fibrosing alveolitis (CFA), known as idiopathic pulmonary fibrosis in the USA, is characterised by inflammation and fibrosis of the alveoli and interstitium of the lungs, favouring the subpleural and basal regions. By definition the diagnosis demands that all known causes of pulmonary fibrosis have been excluded. It is now clear that the CFA(More)