Results 101 to 110 of about 9,635 (207)

Ivacaftor pharmacokinetics and lymphatic transport after enteral administration in rats

Frontiers in Pharmacology
Background: Ivacaftor is a modern drug used in the treatment of cystic fibrosis. It is highly lipophilic and exhibits a strong positive food effect.
Jiří Pozniak, Pavel Ryšánek, David Smrčka, Petr Kozlík, Tomáš Křížek, Jaroslava Šmardová, Anežka Nováková, Debanjan Das, Daniel Bobek, Mahak Arora, Jiří Hofmann, Tereza Doušová, Martin Šíma, Ondřej Slanař   +13 more
doaj   +1 more source

Molecular basis of cystic fibrosis and modern therapeutic approaches [PDF]

, 2019
Identifikacija molekularne povezanosti između genske nefunkcionalnosti CFTR gena te patofiziološke i kliničke pozadine cistične fibroze omogućila je razvoj suvremenih terapijskih modela koji su primarno utemeljeni na individualiziranom pristupu ...
Dragičević, Dorian
core   +2 more sources

The potentiator ivacaftor is essential for pharmacological restoration of F508del-CFTR function and mucociliary clearance in cystic fibrosis

JCI Insight
Pharmacological rescue of F508del-CFTR by the triple combination CFTR modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI) leads to unprecedented clinical benefits in patients with cystic fibrosis (CF).
Anita Balázs, Tihomir Rubil, Christine K. Wong, Jasmin Berger, Marika Drescher, Kathrin Seidel, Mirjam Stahl, Simon Y. Graeber, Marcus A. Mall   +8 more
doaj   +1 more source

Lumacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease homozygous for F508del-CFTR [PDF]

, 2018
Atkinson, Jeffrey, Barto, Tara Lynn, Haddad, Tarik, Jain, Manu, Marigowda, Gautham, Pilewski, Joseph, Tang, Rui, Taylor-Cousar, Jennifer L, Tian, Simon, VH14-809-106 Investigator Group,, Waltz, David   +10 more
core   +2 more sources

CFTR and ENaC functions in cystic fibrosis [PDF]

, 2014
La fibrosis quística se debe a la ausencia o defecto del canal transmembrana regulador de la fibrosis quística (CFTR), un canal de cloruro codificado en el gen cftr que juega un papel clave en la homeostasis del agua e iones.
Kotsias, Basilio Aristides, Marino, Gabriela Inés, Palma, Alejandra Graciela   +2 more
core  

CARATTERIZZAZIONE DELLA FUNZIONE DI PI3K-GAMMA COME NUOVO BERSAGLIO TERAPEUTICO NELLA FIBROSI CISTICA [PDF]

, 2017
La fibrosi cistica (FC) è una malattia genetica a trasmissione autosomica recessiva (AR) causata dalla mutazione del gene CFTR (regolatore della conduttanza transmembrana della fibrosi cistica) che codifica per un canale del Cloro AMP ciclico dipendente.
Pirozzi, Flora
core  

Lumacaftor-ivacaftor in the treatment of cystic fibrosis: design, development and place in therapy

Drug Design, Development and Therapy, 2019
GJ ConnettNational Institute for Health Research, Southampton Respiratory Biomedical Research Centre, University Hospital Southampton NHS Foundation Trust, Southampton, SO16 6YD, UKAbstract: Lumacaftor-ivacaftor is a combination of two small molecule ...
Connett GJ
doaj  

Impact of lumacaftor/ivacaftor on the bacterial and fungal respiratory pathogens in cystic fibrosis: a prospective multicenter cohort study in Sweden

Therapeutic Advances in Respiratory Disease
Background: A significant decline in pulmonary exacerbation rates has been reported in CF patients homozygous for F508del treated with lumacaftor/ivacaftor. However, it is still unclear whether this reduction reflects a diminished microbiological burden.
Mahasin Al Shakirchi, Kimmo Sorjonen, Lena Hjelte, Lena Klingspor, Peter Bergman, Petrea Ericson, Marcus Svedberg, Ulrika Lindberg, Christine Hansen, Isabelle de Monestrol   +9 more
doaj   +1 more source

Efficacy and safety of lumacaftor/ivacaftor combination therapy in patients with cystic fibrosis homozygous for Phe508del CFTR by pulmonary function subgroup [PDF]

, 2016

core   +1 more source

Cystic Fibrosis: Breakthrough Drugs at Break-the-Bank Prices. [PDF]

, 2015
O'Sullivan, Brian P, Orenstein, David M, Quinton, Paul M   +2 more
core   +1 more source