Results 31 to 40 of about 7,354 (172)

The impact of ivacaftor on sinonasal pathology in S1251N-mediated cystic fibrosis patients.

PLoS ONE, 2020
ImportanceSinonasal symptoms in patients suffering from cystic fibrosis can negatively influence the quality of life and sinuses can be a niche for pathogens causing infection and inflammation leading to a decrease of lung function.
Romee Gostelie, Inge Stegeman, Gitte Berkers, Joost Bittermann, Ivonne Ligtenberg-van der Drift, Peter-Jan van Kipshagen, Karin de Winter-de Groot, Lucienne Speleman   +7 more
doaj   +1 more source

Predicting Maternal and Fetal Exposures of Elexacaftor-Tezacaftor-Ivacaftor during Pregnancy through Physiologically Based Pharmacokinetic Models. [PDF]

Clin Pharmacol Ther
The use of elexacaftor/tezacaftor/ivacaftor (ETI) has been associated with increased fertility in women with cystic fibrosis (CF) and is increasingly used during pregnancy to support both maternal and fetal health. However, little is known about the pharmacokinetics (PK) of ETI during pregnancy, which is crucial for optimizing its efficacy and safety ...
Hong E, Hebert MF, Fay E, Turner G, Eshaghian P, Trimble A, Chung PS, Rao AP, Beringer PM.   +8 more
europepmc   +2 more sources

Pharmacogenomics in clinical practice: Biomarker information in Brazilian drug labels

British Journal of Clinical Pharmacology, EarlyView.
This review examines the PGx annotations in package inserts (bulas in Brazilian Portuguese) approved by ANVISA, the Brazilian Health Regulatory Agency, for 19 gene–drug pairs with strong or moderate recommendations for initial dosing alteration in the CPIC (Clinical Pharmacogenetic Implementation Consortion) guidelines and PGx testing required or ...
Guilherme Suarez‐Kurtz
wiley   +1 more source

Structure of ABCB1/P-Glycoprotein in the Presence of the CFTR Potentiator Ivacaftor

Membranes, 2021
ABCB1/P-glycoprotein is an ATP binding cassette transporter that is involved in the clearance of xenobiotics, and it affects the disposition of many drugs in the body.
Alessandro Barbieri, Nopnithi Thonghin, Talha Shafi, Stephen M. Prince, Richard F. Collins, Robert C. Ford   +5 more
doaj   +1 more source

Quantitative Systems Toxicology Predicts Ivacaftor‐Induced Oxidative Stress Contributes to CFTR Modulator Hepatotoxicity

Clinical Pharmacology &Therapeutics, EarlyView.
Cystic fibrosis (CF) is a chronic hereditary disease that affects tens of thousands of people worldwide. The introduction of CFTR modulator therapies such as elexacaftor/tezacaftor/ivacaftor (ETI) has significantly improved the quality of life of people with CF.
Alan Shi, Cole Cornwell, Kyunghee Yang, Paul M. Beringer   +3 more
wiley   +1 more source

Real-world effectiveness of ivacaftor in children with cystic fibrosis and the G551D mutation

Anales de Pediatría (English Edition), 2019
Introduction: Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator that has been shown to improve the nutritional status and lung function of cystic fibrosis patients with the G551D mutation in clinical trials.
David Gomez-Pastrana, Chinedu Nwokoro, Mike McLean, Sarah Brown, Nanna Christiansen, Caroline S. Pao   +5 more
doaj   +1 more source

The validated French CFAbd‐Score reveals a lower burden of gastrointestinal symptoms in patients on Elexacaftor/Tezacaftor/Ivacaftor

Journal of Pediatric Gastroenterology and Nutrition, EarlyView.
Abstract Objectives Multiorgan abdominal involvement is a hallmark of Cystic fibrosis (CF). The CFAbd‐Score© is the first CF‐specific gastrointestinal patient reported outcome‐measure (PROM) developed following FDA‐guidelines. The PROM has proved to sensitively differentiate people with CF (pwCF) from healthy controls (HC).
Isabelle Sermet‐Gaudelus, Pauline Sadrieh, Anne Sophie Bonnel, Raphael Enaud, Nathalie Wizla, Jeanne Languepin, Ambre Nangui, Audrey Deelawon, Johanna Rave, Samira Dabelow, Anton Barucha, Carlos Zagoya, Franziska Duckstein, Jochen Georg Mainz   +13 more
wiley   +1 more source

Ivacaftor for the treatment of patients with cystic fibrosis and the G551D mutation: a systematic review and cost-effectiveness analysis

Health Technology Assessment, 2014
Background: Ivacaftor (Kalydeco®, Vertex Pharmaceuticals) is the first of a new class of drugs that target the underlying protein defect in cystic fibrosis (CF).
Penny Whiting, Maiwenn Al, Laura Burgers, Marie Westwood, Steve Ryder, Martine Hoogendoorn, Nigel Armstrong, Alex Allen, Hans Severens, Jos Kleijnen   +9 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

Obstetric and Neonatal Outcomes in Pregnancies From a Dedicated Cystic Fibrosis‐Maternal Health Service: A Retrospective Study

BJOG: An International Journal of Obstetrics &Gynaecology, EarlyView.
ABSTRACT Objective A comprehensive review of maternal, obstetric and neonatal outcomes in pregnancies in females with cystic fibrosis (fwCF) following the introduction of Elexacaftor/Tezacaftor/Ivacaftor (ETI) therapy in a novel, dedicated CF‐Maternal Health service.
Rebecca Scott, Amy Downes, Ladina Weitnauer, Rebecca Dobra, Natasha Singh, Rachel Robinson, Emily Diable, Saraha Collins, Elaine Bowman, Thomas Tobin, Andrew Jones, Nicholas Simmonds, Imogen Felton   +12 more
wiley   +1 more source