Results 61 to 70 of about 7,376 (199)

Transcriptomic Responses to Ivacaftor and Prediction of Ivacaftor Clinical Responsiveness

American Journal of Respiratory Cell and Molecular Biology, 2019
Abstract Ivacaftor is a drug that was recently approved by the U.S. Food and Drug Administration for the treatment of patients with cystic fibrosis (CF) and at least one copy of the G511D mutation in the CFTR (CF transmembrane conductance regulator) gene. The transcriptomic effect of ivacaftor in patients with CF remains unclear. Here,
Tao, Sun, Zhe, Sun, Yale, Jiang, Annabel A, Ferguson, Joseph M, Pilewski, Jay K, Kolls, Wei, Chen, Kong, Chen   +7 more
openaire   +3 more sources

Physiologically‐based pharmacokinetic modeling of the drug–drug interaction between ivacaftor and lefamulin in cystic fibrosis patients

CPT: Pharmacometrics & Systems Pharmacology
Lefamulin is being evaluated as a treatment for bacterial exacerbations in cystic fibrosis (CF). Ivacaftor is approved for the treatment of patients with CF.
Magali Amiel, Alice Ke, Steven P. Gelone, Hannah M. Jones, Wolfgang Wicha   +4 more
doaj   +1 more source

The effect of CFTR modulators on a cystic fibrosis patient presenting with recurrent pancreatitis in the absence of respiratory symptoms: a case report

BMC Gastroenterology, 2019
Background Cystic fibrosis (CF) is a genetic disorder of the epithelial CFTR apical chloride channel resulting in multi-organ manifestations, including pancreatic exocrine secretion.
J. Dixon Johns, Steven M. Rowe
doaj   +1 more source

Ivacaftor: Five‐year outcomes in the West of Scotland cystic fibrosis population

The Clinical Respiratory Journal, 2023
Introduction Ivacaftor has shown to be effective in patients with cystic fibrosis (CF) with a G551D mutation. Objectives This work aims to evaluate ivacaftor's effectiveness and safety in the real world, over 5 years, in the West of Scotland CF ...
Yasmin Al‐Din, Carol Dryden, Gordon MacGregor, David Young, Cristina Coelho   +4 more
doaj   +1 more source

Alteration of Membrane Cholesterol Content Plays a Key Role in Regulation of Cystic Fibrosis Transmembrane Conductance Regulator Channel Activity

Frontiers in Physiology, 2021
Altered cholesterol homeostasis in cystic fibrosis patients has been reported, although controversy remains. As a major membrane lipid component, cholesterol modulates the function of multiple ion channels by complicated mechanisms.
Guiying Cui, Kirsten A. Cottrill, Kerry M. Strickland, Sarah A. Mashburn, Michael Koval, Nael A. McCarty   +5 more
doaj   +1 more source

Continuous glucose monitoring detects spontaneous reactive and fasting hypoglycaemia in cystic fibrosis adults without diabetes

Internal Medicine Journal, EarlyView.
Abstract People with cystic fibrosis (pwCF) experience a spectrum of glucose disorders. Spontaneous hypoglycaemia is a unique entity affecting pwCF and can be an incidental finding on biochemistry. Our prospective, single‐arm pilot study of eight pwCF without confirmed diabetes and incidental biochemical hypoglycaemia found 62.5% demonstrated fasting ...
Shanal Kumar, Karen Hay, Felicity Loel, Angela Matson   +3 more
wiley   +1 more source

Repurposing Ivacaftor for treatment of Staphylococcus aureus infections

, 2017
Drug repurposing of non-antimicrobials is a novel method to augment a seriously depleted drug pipeline for targeting drug-resistant pathogens. This article highlights the potent antimicrobial activity of Ivacaftor against Staphylococcus aureus, including
Sidharth Chopra, Arunava Dasgupta, Thakare, Ritesh, Swetarka Das, Gorakhnath R. Jachak, D. Srinivasa Reddy, Ritesh Thakare, N. Vasudevan, Singh, Alok Kumar, Chopra, Sidharth, Alok Kumar Singh, Vasudevan, N., Reddy, D. Srinivasa, Das, Swetarka, Jachak, Gorakhnath R., Dasgupta, Arunava   +15 more
core   +1 more source

Ion Channel Dysfunction and Therapeutic Targeting in Salivary Gland Disorders

Oral Diseases, EarlyView.
ABSTRACT Objective Salivary gland hypofunction and xerostomia represent major clinical complications of radiation therapy, autoimmune disorders such as Sjögren's disease, and inherited epithelial ion transport defects. This review integrates current knowledge on ion channel dysfunction as a central mechanistic driver of salivary gland pathology and ...
Tarek Mohamed Abd El‐Aziz, Shankara N. Varadarajan, Viktor Drel, Prakash Arullampalam, Mahmoud I. Shoulkamy, Ahmed Al‐Sabi, Brij B. Singh   +6 more
wiley   +1 more source

Ivacaftor for cystic fibrosis

BMJ, 2018
### What you need to know A 33 year old man with cystic fibrosis has come to discuss a new therapy for which he is eligible because of his genotype Phe508del/Gly551Asp. He has moderate lung function impairment with a forced expiratory volume in one second (FEV1) of 67% of predicted, which has declined from 85% over the preceding five years.
Barry, Peter J., Donaldson, Anna L., Jones, Andrew M.   +2 more
openaire   +3 more sources

Effects of ivacaftor on severely ill patients with cystic fibrosis carrying a G551D mutation [PDF]

, 2013
BackgroundRecently, ivacaftor, a CFTR-potentiator, has been shown to be effective and safe in patients with cystic fibrosis carrying a G551D mutation and moderately impaired lung function.
Helge Hebestreit, Hebestreit, Helge, Käding, Manfred, Sauer-Heilborn, Annette, Mainz, Jochen G., Manfred Käding, Annette Sauer-Heilborn, Jochen G. Mainz, Rainald Fischer, Fischer, Rainald   +9 more
core   +1 more source