Institution
Cystic Fibrosis Trust
Nonprofit•London, United Kingdom•
About: Cystic Fibrosis Trust is a nonprofit organization based out in London, United Kingdom. It is known for research contribution in the topics: Population & Cystic fibrosis. The organization has 71 authors who have published 56 publications receiving 1433 citations.
Topics: Population, Cystic fibrosis, Ivacaftor, Cystic fibrosis transmembrane conductance regulator, Medicine
Papers
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QIMR Berghofer Medical Research Institute1, Charité2, University of Alabama at Birmingham3, Charles University in Prague4, National Institutes of Health5, Paris Descartes University6, St. Michael's Hospital7, Istituto Giannina Gaslini8, University of Auckland9, Nanyang Technological University10, Cystic Fibrosis Trust11, European Medicines Agency12, University of Washington13, University of Wisconsin-Madison14, University of British Columbia15, Katholieke Universiteit Leuven16, Seattle Children's Research Institute17, Post Graduate Institute of Medical Education and Research18, University College Dublin19, University of Giessen20, University of Michigan21, Queen's University Belfast22, Johns Hopkins University23, University of Liverpool24, Woolcock Institute of Medical Research25, University of Toronto26, University of Cape Town27
TL;DR: Advances in clinical care have been multifaceted and include earlier diagnosis through the implementation of newborn screening programmes, formalised airway clearance therapy, and reduced malnutrition through the use of effective pancreatic enzyme replacement and a high-energy, high-protein diet.
427 citations
TL;DR: There have been significant advances in the understanding of T NF signalling pathways in the last decade, and this short review aims to elucidate some of the most recent advances involving TNF signalling in health and disease.
Abstract: The master pro-inflammatory cytokine, tumour necrosis factor (TNF), has been shown to modulate multiple signalling pathways, with wide-ranging downstream effects. TNF plays a vital role in the typical immune response through the regulation of a number of pathways encompassing an immediate inflammatory reaction with significant innate immune involvement as well as cellular activation with subsequent proliferation and programmed cell death or necrosis. As might be expected with such a broad spectrum of cellular effects and complex signalling pathways, TNF has also been implicated in a number of disease states, such as rheumatoid arthritis, ankylosing spondylitis, and Crohn’s disease. Since the time of its discovery over 40 years ago, TNF ligand and its receptors, TNF receptor (TNFR) 1 and 2, have been categorised into two complementary superfamilies, namely TNF (TNFSF) and TNFR (TNFRSF), and 19 ligands and 29 receptors have been identified to date. There have been significant advances in our understanding of TNF signalling pathways in the last decade, and this short review aims to elucidate some of the most recent advances involving TNF signalling in health and disease.
190 citations
TL;DR: A consensus is achieved on 10 research priorities which will be attractive to funders which will help to clarify treatment uncertainties in cystic fibrosis.
Abstract: There remain many treatment uncertainties in cystic fibrosis (CF). With limited resources, research should focus on questions which are most important to the CF community. We conducted a James Lind Alliance Priority Setting Partnership in CF. Research questions were elicited and then prioritised in successive surveys. A workshop agreed the final top 10. Online methods avoided cross infection and widened participation. The elicitation survey had 482 respondents (1080 questions) and prioritisation survey 677 respondents. Participants were drawn equally from the patient and clinical communities globally. We have achieved a consensus on 10 research priorities which will be attractive to funders.
175 citations
TL;DR: The findings support disease modification by CFTR modulation with ivacaftor and are directionally similar to and consistent with US findings.
170 citations
Authors
Showing all 71 results
| Name | H-index | Papers | Citations |
|---|---|---|---|
| Anne E Holland | 56 | 395 | 13943 |
| Michael F. McDermott | 55 | 148 | 14562 |
| Bruce C. Marshall | 40 | 88 | 8229 |
| Peter G. Middleton | 39 | 136 | 5577 |
| Sinisa Savic | 36 | 155 | 4139 |
| Fabio Martinon | 36 | 71 | 10177 |
| Harriet Corvol | 35 | 137 | 3354 |
| Daniel Peckham | 33 | 188 | 4401 |
| Susan C. Charman | 32 | 100 | 3347 |
| Keith G. Brownlee | 29 | 82 | 3256 |
| Albert Faro | 26 | 79 | 1791 |
| Siobhán B. Carr | 25 | 91 | 3074 |
| Narelle S Cox | 18 | 75 | 1154 |
| Luiz Vicente Ribeiro Ferreira da Silva Filho | 16 | 40 | 921 |
| C. Wong | 13 | 19 | 787 |