Results 141 to 150 of about 7,856 (243)

Unveiling the Power of Deuterium in Drug Discovery: A Comprehensive Overview

MedComm, Volume 7, Issue 6, June 2026.
The role of deuterium replacement in drug discovery, its progress, opportunities, and challenges. ABSTRACT Deuterium, the heavy isotope of hydrogen, has unfolded as a cornerstone in modern drug discovery due to its potential to influence metabolic stability and pharmacokinetic behavior.
Mukta Lele, Ajit Manchare, Swapnali Parit, Amol D. Gholap, Krishna Jadhav, Navnath Hatvate, Keshav Raj Paudel, Satish Rojekar   +7 more
wiley   +1 more source

Advances in the treatment of cystic fibrosis: CFTR modulators

Anales de Pediatría (English Edition)
Cystic fibrosis is a severe genetic disease caused by variants in the CFTR gene. Although it is a multisystem disease, respiratory involvement is the main cause of morbidity and mortality. Cystic fibrosis transmembrane conductance regulator modulator (CFTRm) therapies have advanced the treatment of this disease by improving function of this protein ...
Maria Dolores Pastor-Vivero, Jordi Costa i Colomer, Carlos Martín de Vicente, Saioa Vicente-Santamaria, Ruth Garcia Romero, David González Jiménez, Carmen Luna Paredes   +6 more
openaire   +3 more sources

Interrelationships among handgrip strength, body composition, physical activity, and quality of life in adults with cystic fibrosis: A cross‐sectional study

Nutrition in Clinical Practice, Volume 41, Issue 3, Page 849-858, June 2026.
Abstract Background In individuals with cystic fibrosis (CF), lean mass and muscle strength are important predictors of clinical outcomes. This study evaluated associations among body composition, handgrip strength, muscle quality, physical activity, and health‐related quality of life in CF. Methods This observational, cross‐sectional study included 27
Benjamin H. Crain, Carly Harris, Moriah P. Bellissimo, Lucia A. Gonzalez Ramirez, Elizabeth A. Ivie, William R. Hunt, Vin Tangpricha, Thomas R. Ziegler, Ryan A. Harris, Jessica A. Alvarez   +9 more
wiley   +1 more source

Clinical Use of Home Spirometry in Children With Cystic Fibrosis

Pediatric Pulmonology, Volume 61, Issue 6, June 2026.
ABSTRACT Background The use of home spirometry (HSPIR) has increased in pediatric cystic fibrosis (CF) care, but how it has been used clinically and its impact on clinical care have not been described. The purpose of this study was to address this knowledge gap through a secondary analysis of data from a HSPIR quality improvement project to ...
Lucy Tan, Andrew Borowiec, James A. Reed, Mark Rath, Reid Masi, Laura Bennett, Don B. Sanders, Daniel J. Weiner, Clement L. Ren   +8 more
wiley   +1 more source

Performance Evaluation of a Novel Cystic Fibrosis Caregiver Burden Measure Demonstrates Significant Challenges for Parents of Children With CF During the Early Years: The Irish Comparative Outcomes Study (ICOS)

Pediatric Pulmonology, Volume 61, Issue 6, June 2026.
ABSTRACT Background As a part of the Irish Comparative Outcome national cohort study of childhood CF, this cross‐sectional study investigated challenges faced by parents of children with CF (CWCF) in the Republic of Ireland using a newly validated modified tool. Methods Parents completed the modified “Challenge of Living with Cystic Fibrosis‐Short Form”
Rini Bhatnagar, Nancy Bhardwaj, Gwinyai Masukume, Ricardo Segurado, Barry Linnane, Mary Herzig, Muireann Ni Chronin, David Mullane, Des W. Cox, Paul McNally, Sheila Javadpour, Ruth O'Reilly, Basil Elnazir, Peter Greally, Fiona Healy, Michael Williamson, Dubhfeasa Slattery, Gareth McCray, Gillian Lancaster, Claire Glasscoe, Kevin W. Southern, Laura Kirwan, Patricia Fitzpatrick   +22 more
wiley   +1 more source

Physiologically Based Pharmacokinetic Modeling of Elexacaftor/Tezacaftor/Ivacaftor in Infants With Cystic Fibrosis

CPT: Pharmacometrics &Systems Pharmacology, Volume 15, Issue 6, June 2026.
ABSTRACT Ivacaftor is the only cystic fibrosis transmembrane conductance regulator modulator approved for infants ≥ 1 month. The elexacaftor/tezacaftor/ivacaftor combination, approved for children aged ≥ 2 years, has been shown to significantly slow CF progression.
Ngoc Hoa Truong, Sihem Benaboud, Naïm Bouazza, Eric Deneuville, Anne Mornand, Philippe Reix, Mélanie Ribault, Pierre‐Régis Burgel, Léo Froelicher‐Bournaud, Steeve Rouillon, Mohammed Rohi Sanoufi, Gabrielle Lui, Saïk Urien, Jean‐Marc Tréluyer, Isabelle Sermet‐Gaudelus, Frantz Foissac, Modul‐CF study group, Melissa Baravalle, Mahassen Barboura, Lilia Benhalla, Khadidja Bennour, Katia Bessaci, Antoine Bessou, Thiphaine Bihouée, Anne‐Sophie Bonnel, Nesrine Bouleghem, Stéphanie Bui, Frédérique Chedevergne, Harriet Corvol, Laure Cosson, Laure Couderc, Marie‐Laure Dalphin, Paola De Carli, Eric Deneuville, Pierre Foucaud, Asma Gabsi, Elsa Gachelin, Fatiha Hassani, Véronique Houdouin, Frédéric Huet, Marie Jamin, Kadiatou Kaba, Guillaume Labbé, Jane Languepin, Muriel Laurans, Cathy Léréna, Alexia Letierce, Clotilde Livrozet, Christophe Marguet, Laurent Mély, Rania Messaoudi, Marie Mittaine, Camille Ohlmann, Caroline Périsson, Thomas Perrin, Carole Piccini‐Bailly, Cinthia Rames, Philippe Reix, Natascha Rémus, Anna Ronayette, Djouher Sahki, Manuela Scalbert, Isabelle Sermet, Floriane Socchi, Nathalie Stremler, Aurélie Tatopoulos, Guillaume Thouvenin, Tom Toin, Françoise Troussier, Laurence Weiss, Marie‐Christine Werck‐Galois, Nathalie Wizla   +71 more
wiley   +1 more source

Inflammatory response in CF airway epithelial cells: a comparative study of modulators and wild-type CFTR rescue

Frontiers in Pharmacology
The combination of pharmacological modulators such as lumacaftor, tezacaftor, and elexacaftor restore CFTR activity at the plasma membrane and improve lung function in patients carrying CFTR mutations such as F508del, their effects on inflammation are ...
Amal Kouadri, Amal Kouadri, Amal Kouadri, Camille Lyko, Camille Lyko, Camille Lyko, Carine El Hajjar, Carine El Hajjar, Carine El Hajjar, Johanna Cormenier, Johanna Cormenier, Johanna Cormenier, Kevin Kevin Gemy, Kevin Kevin Gemy, Kevin Kevin Gemy, Nadia Alfaidy, Nadia Alfaidy, Nadia Alfaidy, Mohamed Benharouga, Mohamed Benharouga, Mohamed Benharouga   +20 more
doaj   +1 more source

Novel Therapeutics for Chronic Obstructive Pulmonary Disease: From Empirical Bronchodilation to Precision Medicine

Respirology, Volume 31, Issue 6, Page 561-577, June 2026.
ABSTRACT Chronic obstructive pulmonary disease (COPD) remains the third leading cause of death worldwide, and conventional bronchodilator‐based therapies have limited efficacy in preventing exacerbations and disease progression. The 2024–2026 period represents a historic inflection point: three mechanistically distinct agents received Food and Drug ...
Naoya Fujino, Hisatoshi Sugiura
wiley   +1 more source

Pregnancies in women with rare diseases: Selected maternal and perinatal outcomes

Acta Obstetricia et Gynecologica Scandinavica, Volume 105, Issue 6, Page 1088-1095, June 2026.
Pregnancies in women with rare diseases carry substantial disease‐related (23.2%) and pregnancy‐specific (25.1%) risks. Complication rates drop markedly when conditions are stable before conception. Abstract Introduction Rare diseases (RD) are characterized by chronicity and may be associated with reduced life expectancy and quality of life.
Philipp Kosian, Kristin Niederhöfer, Elena Jost, Ulrich Gembruch, Tim Bender, Lorenz Grigull, Waltraut Maria Merz   +6 more
wiley   +1 more source

In utero and postnatal ivacaftor/lumacaftor therapy rescues multiorgan disease in CFTR-F508del ferrets

JCI Insight
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, with F508del being the most prevalent mutation.
Idil Apak Evans, Xingshen Sun, Bo Liang, Amber R. Vegter, Lydia Guo, Thomas J. Lynch, Yan Zhang, Yulong Zhang, Yaling Yi, Yu Yang, Zehua Feng, Soo Yeun Park, Amanita Shonka, Hannah McCumber, Lisi Qi, Peipei Wu, Guangming Liu, Allison Lacina, Kai Wang, Katherine N. Gibson-Corley, David K. Meyerholz, Dominique H. Limoli, Bradley H. Rosen, Ziying Yan, Douglas J. Bartels, John F. Engelhardt   +25 more
doaj   +1 more source