Publications by authors named "Simon Range"

Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterised by impaired mucociliary clearance leading to irreversible lung damage. In contrast to other rare lung diseases like cystic fibrosis (CF), there are only few clinical trials and limited evidence-based treatments. Management is mainly based on expert opinions and treatment is challenging due to a wide range of clinical manifestations and disease severity.

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Article Synopsis
  • A study checked how much inhaled medicine people with cystic fibrosis (CF) received compared to how much they actually used.
  • It found that many people had more medicine than they needed, leading to a waste of money—about £1,124 per person on average.
  • The results show that people who didn't use their medicine as much wasted the most, suggesting that it's important to help people use their medicines properly.
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Background: Lung clearance index (LCI) is a measure of abnormal ventilation distribution derived from the multiple breath inert gas washout (MBW) technique. We aimed to determine the clinical utility of LCI in non-CF bronchiectasis, and to assess two novel MBW parameters that distinguish between increases in LCI due to specific ventilation inequality (LCIvent) and increased respiratory dead space (LCIds).

Methods: Forty-three patients with non-CF bronchiectasis and 18 healthy control subjects underwent MBW using the sulphur hexafluoride wash-in technique, and data from 40 adults with CF were re-analysed.

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Macrophages in the airways form an important element of immune defense and inflammation. We analyzed induced sputum from airways of patients with CF for the types of macrophages present, their receptor expression, and phagocytic function. In samples from patients and age-matched controls, macrophages were analyzed by multicolor flow cytometry, scavenger receptor expression was studied at the protein and mRNA level, and receptor function was investigated using fluorescent particles.

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Interleukin (IL)-8, the C-X-C chemokine, is a potent neutrophil chemoattractant that has been implicated in a number of inflammatory airway diseases such as cystic fibrosis. Here we tested the hypothesis that bradykinin, an inflammatory mediator and chloride secretagogue, would increase IL-8 generation in airway epithelial cells through autocrine generation of endogenous prostanoids. Bradykinin increased IL-8 generation in both a non-cystic fibrosis (A549) and cystic fibrosis epithelial cell line (CFTE29) that was inhibited by the nonselective cyclooxygenase (COX) inhibitor indomethacin and the COX-2 selective inhibitor NS-398.

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