Results 181 to 190 of about 1,231,622 (330)

Clinical pharmacology of CFTR modulators

With the development of cystic fibrosis transmembrane receptor (CFTR) modulating drugs, the landscape in cystic fibrosis (CF) care has changed dramatically. These drugs enable the treatment of the underlying cause of the disease. Although CFTR modulators show an impressive clincal effect at group level in people with CF (pwCF) with specific mutations ...
openaire   +2 more sources

Interrater Reliability of a Modified Bronchoscopy Scoring Tool in Children With Cystic Fibrosis

Pediatric Pulmonology, Volume 61, Issue 1, January 2026.
ABSTRACT Background Flexible bronchoscopy (FB) is widely used in the management of children with Cystic Fibrosis (CF) to visualize airway abnormalities, assess inflammation and detect infection. While previous scoring systems have been proposed to quantify visual airway findings in general pediatric populations, no standardized tool exists for ...
Alexandra Bosetti, Srdjan Micic, Andreas Hector, Christian Bieli, Elias Seidl, Alexander Moeller   +5 more
wiley   +1 more source

P347 Mental health after initiating triple CFTR modulators in a Polish paediatric cystic fibrosis centre - a preliminary report [PDF]

, 2023
U. Borawska-Kowalczyk, K. Walicka-Serzysko, Magdalena Postek, J. Milczewska, Dorota Sands   +4 more
openalex   +1 more source

Impact of Elexacaftor‐Tezacaftor‐Ivacaftor on Quality of Life in Children With Cystic Fibrosis

Pediatric Pulmonology, Volume 61, Issue 1, January 2026.
ABSTRACT Objectives CFTR modulators have revolutionized cystic fibrosis (CF) management by targeting the defective protein rather than its consequences. Their impact on quality of life (QoL) have been studied in numerous trials, but few data are available on QoL in patients receiving Elexacaftor‐Tezacaftor‐Ivacaftor (ETI), notably in children given its
Sara Kümmerli, Clara Fernandez Elviro, Nicolas Regamey, Anne Mornand, Mohamed Faouzi, Isabelle Rochat, Sylvain Blanchon   +6 more
wiley   +1 more source

Persistence and evolution of Pseudomonas aeruginosa following initiation of highly effective modulator therapy in cystic fibrosis

mBio
Today, more than 90% of people with cystic fibrosis (pwCF) are eligible for the highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy called elexacaftor/tezacaftor/ivacaftor (ETI) and its use is widespread.
Catherine R. Armbruster, Yasmin K. Hilliam, Anna C. Zemke, Samar Atteih, Christopher W. Marshall, John Moore, Junu Koirala, Leah Krainz, Jordan R. Gaston, Stella E. Lee, Vaughn S. Cooper, Jennifer M. Bomberger   +11 more
doaj   +1 more source

P031 Perspectives for gene therapy with the use of CFTR modulators in patients with cystic fibrosis [PDF]

, 2023
Luana da Silva Baptista Arpini, Fernanda Mayrink Gonçalves Liberato, R.d.C.N.C. Melotti, Sabrina da Silva Santos, Gina Torres Rego Monteiro   +4 more
openalex   +1 more source

Exploring Exercise as Airway Clearance in Cystic Fibrosis: A Qualitative Study From the ExACT‐CF Feasibility Trial

Pediatric Pulmonology, Volume 61, Issue 1, January 2026.
ABSTRACT Question Cystic fibrosis (CF) affects > 11,300 people in the UK and is characterized by thick, sticky mucus in the lungs, leading to recurrent infections, inflammation, and progressive respiratory decline. Chest physiotherapy remains a cornerstone of airway clearance; however, many people with CF (pwCF) find it burdensome and time‐consuming ...
Emily Taylor, Dia Soilemezi, Don S. Urquhart, Steve Cunningham, Steff Lewis, Aileen Rae Neilson, Hannah Ensor, Ioannis Vogiatizis, Lorna Allen, Zoe L. Saynor, the ExACT‐CF study group, Don S. Urquhart, Emily Taylor, Steve Cunningham, Steff Lewis, Aileen Rae Neilson, Dia Soilemezi, Hannah Ensor, Ioannis Vogiatizis, Lorna Allen, Debbie Miller, Donna Bowen, Ellyse Kilarski, Ellen Lacey, Gary Connett, Julian Legg, Hazel Evans, Thomas Daniels, Mark Allenby, Mary Carroll, Robert Gray, Carlos Echevarria, Lisa Morrison, Gordon McGregor, Mary Abkir, Clare Saunders, Zoe L. Saynor   +36 more
wiley   +1 more source

A Case of Cystic Fibrosis in a Japanese Man With Congenital Bilateral Absence of the Vas Deferens and Recurrent Pancreatitis Caused by a Homozygous c.1210‐11 T > G Variant of the Cystic Fibrosis Transmembrane Conductance Regulator Gene

Reproductive Medicine and Biology, Volume 25, Issue 1, January/December 2026.
ABSTRACT Case We herein report a rare case of a Japanese man diagnosed with cystic fibrosis (CF) following a workup for male infertility and recurrent pancreatitis and discuss the clinical and diagnostic implications in a population wherein CF is exceptionally rare. A 27‐year‐old Japanese man who presented with azoospermia underwent clinical evaluation,
Tomoki Saito, Shinnosuke Kuroda, Teppei Takeshima, Takashi Kawahara, Junichi Teranishi, Jurii Karibe, Fuka Anzai, Miki Tanoshima, Kazuhide Makiyama, Yasushi Yumura   +9 more
wiley   +1 more source

Effect of elexacaftor-tezacaftor-ivacaftor on nasal potential difference and lung function in Phe508del rats

Frontiers in Pharmacology
Introduction:Phe508del is the most common cystic fibrosis transmembrane conductance regulator (CFTR) gene variant that results in the recessive genetic disorder cystic fibrosis (CF). The recent development of highly effective CFTR modulator therapies has
Nicole Reyne, Nicole Reyne, Nicole Reyne, Patricia Cmielewski, Patricia Cmielewski, Patricia Cmielewski, Alexandra McCarron, Alexandra McCarron, Alexandra McCarron, Ronan Smith, Ronan Smith, Ronan Smith, Elena Schneider-Futschik, Nina Eikelis, Piraveen Pirakalathanan, David Parsons, David Parsons, David Parsons, Martin Donnelley, Martin Donnelley, Martin Donnelley   +20 more
doaj   +1 more source

Clinically approved CFTR modulators rescue Nrf2 dysfunction in cystic fibrosis airway epithelia [PDF]

, 2019
Dana C. Borcherding, Matthew Siefert, Songbai Lin, John J. Brewington, Hesham A. Sadek, John P. Clancy, Scott M. Plafker, Assem Ziady   +7 more
openalex   +1 more source