Results 71 to 80 of about 3,864 (165)

Beyond the Surface: Novel Therapy Approach for Pancreatitis in the Setting of CFTR Dysfunction

Case Reports in Gastrointestinal Medicine, Volume 2026, Issue 1, 2026.
Objectives Despite cystic fibrosis transmembrane conductance regulator (CFTR) proteins being present throughout the entire body and organ systems, typical presentation of cystic fibrosis (CF) involves lung disease. We report a series of individuals that were referred to our CF clinic for evaluation following a diagnosis of chronic pancreatitis or acute
Brittany A. Wright, Ellie Hodapp, Douglas B. Hornick, Tahuanty A. Peña, Poulami Jha   +4 more
wiley   +1 more source

EPS6.03 Pulmonary pathogen prevalence 12 months after elexacaftor/tezacaftor/ivacaftor introduction: results from the Danish National Cystic Fibrosis Cohort [PDF]

, 2023
M. Jeppesen, S. Jensen-Fangel, Camilla Bjørn Jensen, Terese L. Katzenstein, M. Wang, M. Frahm Olesen, T. Pressler, Marianne Skov, Hanne Vebert Olesen, Tavs Qvist   +9 more
openalex   +1 more source

Towards personalized medicine in cystic fibrosis:Focusing on clinical pharmacology of CFTR modulators [PDF]

This thesis aimed to improve understanding of the clinical pharmacology of CFTR modulators in children with cystic fibrosis (CF) using real-world data, thereby supporting progress towards personalized medicine.To enable pharmacokinetic analyses, two ...
Vonk, S.E.M.
core   +2 more sources

Immunoreactive Trypsinogen in Infants Born to Women with Cystic Fibrosis Taking Elexacaftor–Tezacaftor–Ivacaftor

International Journal of Neonatal Screening, 2023
Most people with cystic fibrosis (CF) are diagnosed following abnormal newborn screening (NBS), which begins with measurement of immunoreactive trypsinogen (IRT) values. A case report found low concentrations of IRT in an infant with CF exposed to the CF
Payal Patel, Jana Yeley, Cynthia Brown, Melissa Wesson, Barbara G. Lesko, James E. Slaven, James F. Chmiel, Raksha Jain, Don B. Sanders   +8 more
doaj   +1 more source

From Pathophysiology to Treatment: Contemporary Approaches to CFRD in the Pediatric and Adolescent Population

Pediatric Diabetes, Volume 2026, Issue 1, 2026.
Cystic fibrosis‐related diabetes (CFRD) is the most prevalent nonrespiratory complication of cystic fibrosis (CF), with its prominence growing as survival rates improve due to advances in CFTR modulator therapies. Its prevalence increases with age, affecting nearly 50% of patients with CF (PwCF) over 30 years old.
Dogus Vuralli, Andrea Scaramuzza
wiley   +1 more source

Cystic fibrosis-related diabetes in the era of modern treatment using CFTR modulators in pediatric patients—a systematic review

Frontiers in Pediatrics
BackgroundCystic fibrosis-related diabetes (CFRD) is a common comorbidity in cystic fibrosis (CF), particularly in the pediatric population. As cystic fibrosis transmembrane conductance regulator modulators (CFTRm) become widely used, there is growing ...
Anna Pietrzykowska, Jerzy Darmoń, Katarzyna Szymocha, Wiktoria Donocik, Mateusz Tarasiewicz, Julia Stadnik, Przemysława Jarosz-Chobot   +6 more
doaj   +1 more source

In silico, in vitro and ex vivo characterization of cystic fibrosis transmembrane conductance regulator pathogenic variants localized in the fourth intracellular loop and their rescue by modulators

British Journal of Pharmacology, Volume 182, Issue 24, Page 6063-6080, December 2025.
Background and Purpose Cystic fibrosis (CF) is due to loss‐of‐function variants of the CF transmembrane conductance regulator (CFTR) channel. The most effective treatment for people with CF carrying the F508del mutation is the triple combination of elexacaftor–tezacaftor–ivacaftor (ETI).
Emanuela Pesce, Valeria Tomati, Valeria Capurro, Mariateresa Lena, Cristina Pastorino, Miro Astore, Serdar Kuyucak, Benoit Chevalier, Elvira Sondo, Federico Cresta, Alice Dighero, Vito Terlizzi, Cristina Fevola, Stefano Costa, Maria Cristina Lucanto, Valeria Daccò, Laura Claut, Francesca Ficili, Benedetta Fabrizzi, Renata Bocciardi, Federico Zara, Carlo Castellani, Luis J. V. Galietta, Shafagh A. Waters, Alexandre Hinzpeter, Nicoletta Pedemonte   +25 more
wiley   +1 more source

Respiratory infections after elexacaftor/tezacaftor/ivacaftor treatment in people with cystic fibrosis: analysis of the European Cystic Fibrosis Society Patient Registry [PDF]

Background: Elexacaftor/tezacaftor/ivacaftor (ETI) has improved outcomes for people with cystic fibrosis (pwCF). This study evaluated changes in airway microbiological infection status after initiating ETI.
De Rose, Virginia, Gambazza, Simone, Kerem, Eitan, Mei Zahav, Meir, null, null, Orenti, Annalisa, Pollak, Mordechai, Prais, Dario   +7 more
core   +1 more source

Lung function improvement on triple modulators: high-resolution, nationwide data from the Danish Cystic Fibrosis Cohort

ERJ Open Research
Background People living with cystic fibrosis in Denmark had early, universal access to triple modulator treatment with elexacaftor/tezacaftor/ivacaftor.
Christian Leo-Hansen, Daniel Faurholt-Jepsen, Tavs Qvist, Christine Højte, Bibi U. Nielsen, Thomas Bryrup, Esben H. Henriksen, Terese Katzenstein, Marianne Skov, Inger H.M. Mathiesen, Majbritt Jeppesen, Søren Jensen-Fangel, Hanne V. Olesen, Frederik Fouirnaies Buchvald, Kim Gjerum Nielsen, Espen Jimenez-Solem, Christian Ritz, Tacjana Pressler, Mette F. Olsen, the TransformCF Study Group   +19 more
doaj   +1 more source

The Concise Guide to PHARMACOLOGY 2025/26: Ion channels

British Journal of Pharmacology, Volume 182, Issue S1, Page S152-S241, December 2025.
The Concise Guide to Pharmacology 2025/26 marks the seventh edition in this series of biennial publications in the British Journal of Pharmacology. Presented in landscape format, the guide provides a comparative overview of the pharmacology of drug target families. The concise nature of the Concise Guide refers to the style of presentation, being clear,
Stephen P. H. Alexander, Jörg Striessnig, Alasdair J. Gibb, Alistair A. Mathie, Emma L. Veale, Eamonn Kelly, Chloe J. Peach, Jane F. Armstrong, Elena Faccenda, Simon D. Harding, Christopher Southan, Jamie A. Davies, Richard W. Aldrich, Bernard Attali, Austin M. Baggetta, Elvir Becirovic, David J. Beech, Martin Biel, Roslyn M. Bill, Ana I. Caceres, William A. Catterall, Han Chow Chua, Alex C. Conner, Henry Danahay, Paul Davies, Katrien De Clerq, Markus Delling, Francesco Di Virgilio, Simonetta Falzoni, Wei Fang, Stefanie Fenske, Anna Fortuny‐Gomez, Samuel Fountain, George K. Chandy, Mohammad‐Reza Ghovanloo, Steve A. N. Goldstein, Martin Gosling, Christian Grimm, Stephan Grissmer, Kotdaji Ha, Tim G. Hales, Verena Hammelmann, Israel Hanukoglu, Meiqin Hu, Ad P. Ijzerman, Sairam V. Jabba, Mike Jarvis, Anders A. Jensen, Sven E. Jordt, Leonard K. Kaczmarek, Stephan Kellenberger, Charles Kennedy, Brian King, Philip Kitchen, Qiang Liu, Joseph W. Lynch, Rian W. Manville, Jessica Meades, Verena Mehlfeld, Annette Nicke, Stefan Offermanns, Edward Pérez‐Reyes, Leigh D. Plant, Stephan A. Pless, Lachlan D. Rash, Dejian Ren, Hussein N. Rubaiy, Mootaz M. Salman, Werner Sieghart, Lucia G. Sivilotti, Trevor G. Smart, Terrance P. Snutch, Paolo Tammaro, Jinbin Tian, James S. Trimmer, Charlotte Van den Eynde, Paola Vergani, Joris Vriens, Stephen G. Waxman, Aguan D. Wei, Brenda T. Winn, Heike Wulff, Haoxing Xu, Fan Yang, Lixia Yue, Xiaoli Zhang, Michael Zhu   +86 more
wiley   +1 more source