Search Results - "JOHNSON, Simon R"

Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: a prospective national cohort study by Bee, Janet, Fuller, Sharon, Miller, Suzanne, Johnson, Simon R

“…Mechanistic target of rapamycin inhibitors reduce loss of lung function in lymphangioleiomyomatosis (LAM), although their benefit varies between individuals…”
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Extracellular Matrix Cross-Linking Enhances Fibroblast Growth and Protects against Matrix Proteolysis in Lung Fibrosis by Philp, Christopher J, Siebeke, Ivonne, Clements, Debbie, Miller, Suzanne, Habgood, Anthony, John, Alison E, Navaratnam, Vidya, Hubbard, Richard B, Jenkins, Gisli, Johnson, Simon R

“…Idiopathic pulmonary fibrosis (IPF) is characterized by accumulation of extracellular matrix (ECM) proteins and fibroblast proliferation. ECM cross-linking…”
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Lymphangioleiomyomatosis in patients with tuberous sclerosis: a national centre audit by Johnson, Jan, Somerfield, Wendy, Johnson, Simon R

“…Lymphangioleiomyomatosis (LAM) is common in tuberous sclerosis complex (TSC) yet under recognised with management mostly based upon evidence obtained from…”
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Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline by Gupta, Nishant, Finlay, Geraldine A, Kotloff, Robert M, Strange, Charlie, Wilson, Kevin C, Young, Lisa R, Taveira-DaSilva, Angelo M, Johnson, Simon R, Cottin, Vincent, Sahn, Steven A, Ryu, Jay H, Seyama, Kuniaki, Inoue, Yoshikazu, Downey, Gregory P, Han, MeiLan K, Colby, Thomas V, Wikenheiser-Brokamp, Kathryn A, Meyer, Cristopher A, Smith, Karen, Moss, Joel, McCormack, Francis X

“…Recommendations regarding key aspects related to the diagnosis and pharmacological treatment of lymphangioleiomyomatosis (LAM) were recently published. We now…”
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Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management by McCormack, Francis X, Gupta, Nishant, Finlay, Geraldine R, Young, Lisa R, Taveira-DaSilva, Angelo M, Glasgow, Connie G, Steagall, Wendy K, Johnson, Simon R, Sahn, Steven A, Ryu, Jay H, Strange, Charlie, Seyama, Kuniaki, Sullivan, Eugene J, Kotloff, Robert M, Downey, Gregory P, Chapman, Jeffrey T, Han, MeiLan K, D'Armiento, Jeanine M, Inoue, Yoshikazu, Henske, Elizabeth P, Bissler, John J, Colby, Thomas V, Kinder, Brent W, Wikenheiser-Brokamp, Kathryn A, Brown, Kevin K, Cordier, Jean F, Meyer, Cristopher, Cottin, Vincent, Brozek, Jan L, Smith, Karen, Wilson, Kevin C, Moss, Joel

“…Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that primarily affects women. The purpose of these guidelines is to provide recommendations for…”
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Lymphangioleiomyomatosis by Johnson, Simon R., BSc, MBBS, DM, FRCP, Taveira-DaSilva, Angelo M., MD, PhD, Moss, Joel, MD, PhD

“…Lymphangioleiomyomatosis is a rare multisystem disease predominantly affecting women that can occur sporadically or in association with tuberous sclerosis…”
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Sirolimus Therapy for Angiomyolipoma in Tuberous Sclerosis and Sporadic Lymphangioleiomyomatosis: A Phase 2 Trial by DAVIES, D. Mark, DE VRIES, Petrus J, CROSS, Justin J, TATTERSFIELD, Anne E, KINGSWOOD, J. Chris, SAMPSON, Julian R, JOHNSON, Simon R, MCCARTNEY, Deborah L, COX, Jane A, SERRA, Andreas L, WATSON, Peter C, HOWE, Christopher J, DOYLE, Tim, POINTON, Kate

“…Renal angiomyolipomas are a frequent manifestation of tuberous sclerosis and sporadic lymphangioleiomyomatosis (LAM). These disorders are associated with…”
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High-capacity hydrogen storage in lithium and sodium amidoboranes by Chen, Ping, Xiong, Zhitao, Yong, Chaw Keong, Wu, Guotao, Shaw, Wendy, Karkamkar, Abhi, Autrey, Thomas, Jones, Martin Owen, Johnson, Simon R, Edwards, Peter P, David, William I. F

“…The safe and efficient storage of hydrogen is widely recognized as one of the key technological challenges in the transition towards a hydrogen-based energy…”
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Tuning the Decomposition Temperature in Complex Hydrides: Synthesis of a Mixed Alkali Metal Borohydride by Nickels, E. Anne, Jones, Martin Owen, David, William I.F, Johnson, Simon R, Lowton, Rebecca L, Sommariva, Marco, Edwards, Peter P

“…Variegated decomposers: The synthesis of the first mixed alkali metal borohydride, which contains lithium and potassium, is presented. LiK(BH4)2 (see picture;…”
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A lung targeted miR-29 mimic as a therapy for pulmonary fibrosis by Chioccioli, Maurizio, Roy, Subhadeep, Newell, Rachel, Pestano, Linda, Dickinson, Brent, Rigby, Kevin, Herazo-Maya, Jose, Jenkins, Gisli, Ian, Steward, Saini, Gauri, Johnson, Simon R., Braybrooke, Rebecca, Yu, Guying, Sauler, Maor, Ahangari, Farida, Ding, Shuizi, DeIuliis, Joseph, Aurelien, Nachelle, Montgomery, Rusty L., Kaminski, Naftali

“…MicroRNAs are non-coding RNAs that negatively regulate gene networks. Previously, we reported that systemically delivered miR-29 mimic MRG-201 reduced fibrosis…”
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Accelerated extracellular matrix turnover during exacerbations of COPD by Sand, Jannie M B, Knox, Alan J, Lange, Peter, Sun, Shu, Kristensen, Jacob H, Leeming, Diana J, Karsdal, Morten A, Bolton, Charlotte E, Johnson, Simon R

“…Exacerbations of chronic obstructive pulmonary disease (COPD) contribute significantly to disease progression. However, the effect on tissue structure and…”
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Extra-cellular matrix proteins induce matrix metalloproteinase-1 (MMP-1) activity and increase airway smooth muscle contraction in asthma by Rogers, Natasha K, Clements, Debbie, Dongre, Arundhati, Harrison, Tim W, Shaw, Dominic, Johnson, Simon R

“…Airway remodelling describes the histopathological changes leading to fixed airway obstruction in patients with asthma and includes extra-cellular matrix (ECM)…”
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Air travel and incidence of pneumothorax in lymphangioleiomyomatosis by Gonano, Cynthia, Pasquier, Jérôme, Daccord, Cécile, Johnson, Simon R, Harari, Sergio, Leclerc, Violette, Falconer, Lucy, Miano, Eleonora, Cordier, Jean-François, Cottin, Vincent, Lazor, Romain

“…Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of women characterized by multiple lung cysts leading to respiratory insufficiency and frequent…”
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Wild type mesenchymal cells contribute to the lung pathology of lymphangioleiomyomatosis by Clements, Debbie, Dongre, Arundhati, Krymskaya, Vera P, Johnson, Simon R

“…Lymphangioleiomyomatosis (LAM) is a rare disease leading to lungs cysts and progressive respiratory failure. Cells of unknown origin accumulate in the lungs…”
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The clinical profile of tuberous sclerosis complex (TSC) in the United Kingdom: A retrospective cohort study in the Clinical Practice Research Datalink (CPRD) by Kingswood, Christopher, Bolton, Patrick, Crawford, Pamela, Harland, Christopher, Johnson, Simon R, Sampson, Julian R, Shepherd, Charles, Spink, Jayne, Demuth, Dirk, Lucchese, Lara, Nasuti, Paola, Gray, Elizabeth, Pinnegar, Alun, Magestro, Matthew

“…Abstract Background Tuberous Sclerosis Complex (TSC) is a multi-system genetic disorder characterised by the development of benign growths and diverse clinical…”
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A Mechanism for Non-stoichiometry in the Lithium Amide/Lithium Imide Hydrogen Storage Reaction by David, William I. F, Jones, Martin O, Gregory, Duncan H, Jewell, Catherine M, Johnson, Simon R, Walton, Allan, Edwards, Peter P

“…We demonstrate, through structural refinement from synchrotron X-ray diffraction data, that the mechanism of the transformation between lithium amide and…”
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Sirolimus Therapy in Tuberous Sclerosis or Sporadic Lymphangioleiomyomatosis by S, Tim Doyle, L.R.C.P. &, Elmslie, Frances, Johnson, Simon R, Sci, Julian R. Sampson, D.M., F. Med, Kingswood, J. Chris, McCartney, Deborah L, de Vries, Petrus J, Davies, D. Mark, Cox, Jane A, Saggar, Anand, Tattersfield, Anne E

“…To the Editor: Tuberous sclerosis is an autosomal dominant disorder characterized by hamartomatous growths in many organs and caused by inherited mutations of…”
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Elevated PLAUR is observed in the airway epithelium of asthma patients and blocking improves barrier integrity by Portelli, Michael A, Bhaker, Sangita, Pang, Vincent, Bates, David O, Johnson, Simon R, Mazar, Andrew P, Shaw, Dominick, Brightling, Christopher, Sayers, Ian

“…Expression of the urokinase plasminogen activator receptor (uPAR) is elevated in the airway epithelium in asthma; however, the contribution of uPAR to asthma…”
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Clinical utility of diagnostic guidelines and putative biomarkers in lymphangioleiomyomatosis by Chang, William Y C, Cane, Jennifer L, Blakey, John D, Kumaran, Maruti, Pointon, Kate S, Johnson, Simon R

“…Lymphangioleiomyomatosis is a rare disease occurring almost exclusively in women. Diagnosis often requires surgical biopsy and the clinical course varies…”
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The Monoammoniate of Lithium Borohydride, Li(NH3)BH4: An Effective Ammonia Storage Compound by Johnson, Simon R., David, William I. F., Royse, David M., Sommariva, Marco, Tang, Christina Y., Fabbiani, Francesca P. A., Jones, Martin O., Edwards, Peter P.

“…Store it up: The ammoniate, Li(NH3)BH4, has been prepared and fully characterized by Raman and NMR spectroscopy and X‐ray powder diffraction. The potential for…”
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