Results 61 to 70 of about 1,132,527 (387)

Elexacaftor/Tezacaftor/Ivacaftor Efficacy in a Cohort of Italian Patients with CFTR Rare Mutations

International Journal of Translational Medicine
Background: Cystic Fibrosis is an inherited disorder caused by mutations in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene, encoding a chloride and bicarbonate channel widely expressed in epithelia.
Francesca Lucca, Sonia Volpi, Mirco Ros, Benedetta Fabrizzi, Ilaria Meneghelli, Marica Bordicchia, Francesca Buniotto, Alessia Lancini, Cecilia Brignole, Francesca Pauro, Valentino Bezzerri, Marco Cipolli   +11 more
doaj   +1 more source

Peroxisome Proliferator-Activated Receptor alpha (PPAR alpha) down-regulation in cystic fibrosis lymphocytes [PDF]

, 2006
Background: PPARs exhibit anti-inflammatory capacities and are potential modulators of the inflammatory response. We hypothesized that their expression and/or function may be altered in cystic fibrosis (CF), a disorder characterized by an excessive host ...
A Augarten, A Linden, A Madej, A Trifilieff, AA Ionescu, AE Lovett-Racke, AP Sampson, B Staels, BM Forman, C Andersson, C Hubeau, C Hubeau, DC Jones, E Dagli, F Akbiyik, F McAllister, G Chinetti, H Pall, I Inoue, J Carlstedt-Duke, JD Dignam, JD Tugwood, JH Parmentier, JL Su, JM Shipley, JP Despres, JT Zakrzewski, K Balough, K Beier, KA Kreuzer, LS Nixon, M Ollero, M Roulet, MA Birrell, ME Poynter, N Marx, N Marx, P Delerive, P Greally, P Reineck, PM Farrell, PR Devchand, RB Moss, RB Moss, RB Moss, RU Sorensen, SD Freedman, SM Loitsch, SW Chung, TA Waldmann, TP Dean, TZ Khan, W Wahli, Z Israelian-Konaraki   +53 more
core   +4 more sources

Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis

Science Translational Medicine, 2019
Increased airway mucus, occurs before infection or structural lung disease in early cystic fibrosis and promotes inflammation. Early changes in cystic fibrosis Cystic fibrosis (CF) is a genetic disorder caused by mutations in the CFTR gene.
C. Esther, M. Muhlebach, C. Ehre, D. Hill, M. Wolfgang, M. Kesimer, K. Ramsey, K. Ramsey, M. Markovetz, Ian C. Garbarine, M. Forest, Ian Seim, Bryan T Zorn, Cameron B. Morrison, M. Delion, William R. Thelin, D. Villalon, J. Sabater, L. Turkovic, S. Ranganathan, S. Stick, S. Stick, R. Boucher   +22 more
semanticscholar   +1 more source

Biomaterial Strategies for Targeted Intracellular Delivery to Phagocytes

Advanced Functional Materials, EarlyView.
Phagocytes are essential to a functional immune system, and their behavior defines disease outcomes. Engineered particles offer a strategic opportunity to target phagocytes, harnessing inflammatory modulation in disease. By tuning features like size, shape, and surface, these systems can modulate immune responses and improve targeted treatment for a ...
Kaitlyn E. Woodworth, Neal I. Callaghan, Locke Davenport Huyer   +2 more
wiley   +1 more source

Sensing of Chloride Ions in Sweat by Means of Printed Extended‐Gate Organic Electrochemical Transistors

Advanced Functional Materials, EarlyView.
In this work, a platform based on an Extended‐Gate OECT is successfully developed and tailored for the detection of physiologically relevant chloride ion concentrations in artificial eccrine sweat. Leveraging the extended‐gate architecture, a high‐performance OECT based on inkjet‐printed pg2T‐TT is coupled with an inkjet‐printed Ag/AgCl electrode ...
Cristiano Bortolotti, Andrea Ottomaniello, Paola Parlanti, Virgilio Mattoli, Dario Natali, Andrea Aliverti, Adrica Kyndiah, Mario Caironi   +7 more
wiley   +1 more source

A Human Kidney Tubuloid Model of Repeated Cisplatin‐Induced Cellular Senescence and Fibrosis for Drug Screening

Advanced Healthcare Materials, EarlyView.
Replicating aging and senescence‐related pathophysiological responses in kidney organoids remains a significant challenge. Human adult renal tubular organoid, tubuloids, are successfully developed recapitulating cellular senescence that is the central pathophysiological mechanism of chronic kidney disease (CKD).
Yuki Nakao, Makiko Mori, Yuta Sekiguchi, Iori Morita, Ryota Shindoh, Shintaro Mandai, Tamami Fujiki, Hiroaki Kikuchi, Fumiaki Ando, Koichiro Susa, Takayasu Mori, Ayumi Suzuki, Yuji Nashimoto, Hirokazu Kaji, Yuma Waseda, Soichiro Yoshida, Yasuhisa Fujii, Eisei Sohara, Shinichi Uchida, Kensuke Miyake, Yutaro Mori   +20 more
wiley   +1 more source

National survey on pediatric respiratory physiotherapy units: primary ciliary dyskinesia and non-CF bronchiectasis

Italian Journal of Pediatrics
Background Currently, there is a lack of data concerning the organization and characteristics of Italian pediatric physiotherapy units for the treatment of patients with chronic lung diseases, especially those with rare conditions such as Primary Ciliary
Beatrice Tani, Nicola Ullmann, Paola Leone, Alessandra Boni, Eugenio Barbieri, Matteo D’Angelo, Sara De Dominicis, Beniamino Giacomodonato, Stefania Monduzzi, Irene Piermarini, Chiara Pizziconi, Beatrice Ferrari, Renato Cutrera   +12 more
doaj   +1 more source

A new targeted CFTR mutation panel based on next-generation sequencing technology [PDF]

, 2017
Searching for mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR) is a key step in the diagnosis of and neonatal and carrier screening for cystic fibrosis (CF), and it has implications for prognosis and personalized therapy ...
Alberti, Luisella, Biffignandi, Alice, Bruno, Sabina Maria, Colombo, Carla, Corbetta, Carlo, Costantino, Lucy, Giannone, Valentina, Lucarelli, Marco, Porcaro, Luigi, Seia, Manuela, Torresani, Erminio   +10 more
core   +2 more sources

Microbiology of Cystic Fibrosis Airway Disease

Seminars in Respiratory and Critical Care Medicine, 2019
Although survival of individuals with cystic fibrosis (CF) has been continuously improving for the past 40 years, respiratory failure secondary to recurrent pulmonary infections remains the leading cause of mortality in this patient population.
A. Blanchard, V. Waters
semanticscholar   +1 more source

Body Biofluids for Minimally‐Invasive Diagnostics: Insights, Challenges, Emerging Technologies, and Clinical Potential

Advanced Healthcare Materials, EarlyView.
Recent advances in diagnostics have accelerated the development of miniaturized wearable technologies for the continuous monitoring of diseases. This paradigm is shifting healthcare away from invasive, centralized blood tests toward decentralized monitoring, using alternative body biofluids.
Lanka Tata Rao, Chandan Kumar Mandal, Fernando Patolsky   +2 more
wiley   +1 more source