Results 81 to 90 of about 975,042 (316)

VX‐659–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles

New England Journal of Medicine, 2018
BACKGROUND The next‐generation cystic fibrosis transmembrane conductance regulator (CFTR) corrector VX‐659, in triple combination with tezacaftor and ivacaftor (VX‐659–tezacaftor–ivacaftor), was developed to restore the function of Phe508del CFTR protein
J. Davies, S. Moskowitz, C. Brown, A. Horsley, M. Mall, E. McKone, B. Plant, Dario Prais, B. Ramsey, J. Taylor-Cousar, E. Tullis, A. Uluer, C. Mckee, S. Robertson, R. Shilling, C. Simard, F. van Goor, D. Waltz, F. Xuan, T. Young, S. Rowe   +20 more
semanticscholar   +1 more source

Mechanisms of Mitochondrial Toxicity and Cytotoxicity Caused by Pseudomonas aeruginosa Pyocyanin in Human Nasal Epithelial Cells

International Forum of Allergy &Rhinology, EarlyView.
ABSTRACT Background Pseudomonas aeruginosa is an opportunistic pathogen in cystic fibrosis‐related chronic rhinosinusitis (CF‐CRS) that produces phenazine metabolites pyocyanin and 1‐hydroxyphenazine (1‐HP), which may have detrimental effects on mitochondria, reactive oxygen species (ROS), Ca2+ signaling, and apoptosis.
Joel C. Thompson, April Park, Yobouet Ines Kouakou, Zoey A. Miller, Nabil F. Darwich, Nithin D. Adappa, James N. Palmer, Ryan M. Carey, Robert J. Lee   +8 more
wiley   +1 more source

The complexities of elexacaftor/tezacaftor/ivacaftor therapeutic drug monitoring in a person with cystic fibrosis and Mycobacterium abscessus pulmonary disease

European Clinical Respiratory Journal
Therapeutic drug monitoring (TDM) of elexacaftor/tezacaftor/ivacaftor (ETI) remains challenging due to a lack of clarity around the parameters that govern ETI plasma concentrations, whilst the use of concomitant CYP3A inducers rifabutin and rifampicin is
Claire Y Mou, Daniel J Henderson, Angela G Matson, Karen M Herd, David W Reid, Timothy Riddles, Ellie Johnson, Brett McWhinney, Rebecca Swenson, Andrew Burke, Ieuan E S Evans   +10 more
doaj   +1 more source

Inflammation in cystic fibrosis: An update

Pediatric Pulmonology, 2018
Inflammation plays a critical role in cystic fibrosis (CF) lung pathology and disease progression making it an active area of research and important therapeutic target.
E. Roesch, D. Nichols, J. Chmiel
semanticscholar   +1 more source

Animal models of benign airway stenosis: Advances in construction techniques, evaluation systems, and perspectives

Animal Models and Experimental Medicine, EarlyView.
Currently, the animals commonly used to establish animal models of benign airway stenosis (BAS) include mice, rats, pigs, dogs, rabbits, and ferrets. The establishment methods involve one or a combination of two methods such as mechanical injury, tracheal cautery, cuff overpressure intubation, laser injury, and endoscopic silver nitrate cauterization ...
Wusheng Zhang, Yilin Chen, Chengcheng Yang, Yuchao Dong, Haidong Huang, Hui Shi, Chong Bai   +6 more
wiley   +1 more source

Pseudomonas aeruginosa Acquisition in Cystic Fibrosis Patients in Context of Otorhinolaryngological Surgery or Dentist Attendance: Case Series and Discussion of Preventive Concepts

Case Reports in Infectious Diseases, 2015
Introduction. P. aeruginosa is the primary cause for pulmonary destruction and premature death in cystic fibrosis (CF). Therefore, prevention of airway colonization with the pathogen, ubiquitously present in water, is essential.
Jochen G. Mainz, Andrea Gerber, Michael Lorenz, Ruth Michl, Julia Hentschel, Anika Nader, James F. Beck, Mathias W. Pletz, Andreas H. Mueller   +8 more
doaj   +1 more source

Challenges of modeling pristane‐induced lupus in non‐human primates

Animal Models and Experimental Medicine, EarlyView.
Systemic lupus erythematosus (SLE) in cynomolgus monkeys was modeled by two intraperitoneal injections of pristane at a dose of 3.5 mL/kg, administered at weeks 1 and 23. Serum samples from all monkeys that received pristane tested positive for antinuclear antibodies.
Elena Vladimirovna Vinogradova, Maria Alexandrovna Varavko, Yakov Yuryevich Ustyugov, Alexey Alexandrovich Aleksandrov, Dmitry Alexandrovich Khochenkov, Yulia Nikolaevna Linkova   +5 more
wiley   +1 more source

Activity of the new cephalosporin CXA-101 (FR264205) against Pseudomonas aeruginosa isolates from chronically infected cystic fibrosis patients [PDF]

, 2009
Laura Zamorano, Carlos Juan, Ana Fernández-Olmos, Yigong Ge, Rafael Cantà n, Antonio Oliver   +5 more
openalex   +1 more source

Resilience to Endoplasmic Reticulum Stress Mitigates Membrane Hyperexcitability Underlying Late Disease Onset in a Murine Model of SCA6

Annals of Neurology, EarlyView.
Objective An enduring puzzle in many inherited neurological disorders is the late onset of symptoms despite expression of function‐impairing mutant protein early in life. We examined the basis for onset of impairment in spinocerebellar ataxia type 6 (SCA6), a canonical late‐onset neurodegenerative ataxia which results from a polyglutamine expansion in ...
Haoran Huang, Taylor L. Charron, Min Fu, Miranda Dunn, Deborah M. Jones, Praveen Kumar, Satoshi Ishishita, Allan‐Hermann Pool, Ashwinikumar Kulkarni, Genevieve Konopka, Vikram G. Shakkottai   +10 more
wiley   +1 more source

Advances and Challenges in Micro/Nanocarrier‐Based Dermal Drug Delivery Systems for Acne Treatment

Advanced NanoBiomed Research, EarlyView.
Topical acne treatment faces challenges from skin barriers and complex causes. Micro/nanocarriers improve drug delivery by enhancing bioavailability, targeting, and duration. Combining carriers creates synergistic effects for systematic intervention.
Jintao Yang, Jie Chen, Yunmiao Ma, Qingwei Zhou, Yu Kong, Yingxiang Chen, Junjie Chi, Haihua Hu   +7 more
wiley   +1 more source