Results 11 to 20 of about 89 (70)

Respiratory Oscillometry and Multimodal Lung Function Assessment of Elexacaftor/Tezacaftor/Ivacaftor Response in Children and Young Adults With Cystic Fibrosis

Pediatric Pulmonology, Volume 61, Issue 2, February 2026.
ABSTRACT Background To date, there is no data on forced oscillation technique (FOT) as a tool to evaluate elexacaftor/tezacaftor/ivacaftor (ETI) modulator in young patients with cystic fibrosis (pwCF). Objective Our study aimed to assess the effect of a 6‐month ETI treatment on FOT parameters and compare it to the effect on spirometry and nitrogen ...
Christos Kogias, Athina Sopiadou, Sotirios Fouzas, Petrina Vantsi, Elissavet‐Anna Chrysochoou, Evangelia Desli, Maria Galogavrou, Nikolaos Gasparis, Elpis Hatziagorou   +8 more
wiley   +1 more source

Effects of cAMP and CFTR modulation on apical fluid pH in human airway Calu‐3 cells

Physiological Reports, Volume 14, Issue 3, February 2026.
Abstract The airway epithelium serves as the first line of defense against inhaled insults present in the external environment by acting as a physical barrier and through host defense mechanisms. Proper maintenance of these host defense mechanisms relies on the regulation of airway surface liquid (ASL) composition and properties, a process that is ...
Jenny P. Nguyen, Nadia Milad, Jeremy A. Hirota   +2 more
wiley   +1 more source

Light‐Enabled [2σ + 2π] Cycloadditions of Bicyclo[1.1.0]butanes and Alkenes by Ti‐Salen Photocatalysis

ChemistryEurope, Volume 4, Issue 1, January 2026.
This study reports an unprecedented Ti(Salen)‐catalyzed [2σ+2π] photocycloaddition of bicyclo[1.1.0]butanes and alkenes, featuring broad substrate scope, excellent functional group tolerance, scalability, and potential pharmaceutical applications. Notably, this work represents the first application of a Ti(Salen) complex as an efficient photoredox ...
Jinping Xing, Dexia Li, Tianxiang Li, Yang Xu, Haosong Ren, Jun Zheng   +5 more
wiley   +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, Volume 82, Issue 1, Page 24-32, January 2026.
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

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

Acne in Cystic Fibrosis: Tips for Prescribing Isotretinoin in the Genomodulatory Era

Case Reports in Dermatological Medicine, Volume 2026, Issue 1, 2026.
A 16‐year‐old boy with cystic fibrosis (CF) on fat‐soluble vitamin (including vitamin A) supplementation develops severe acne. He is commenced on isotretinoin, a vitamin A derivative. The package insert says that vitamin A supplementation should be stopped, presenting a therapeutic dilemma.
Julia O’Mahony, David Mullane, Cathal O’Connor, Harleen Khatra   +3 more
wiley   +1 more source

Greenness, Whiteness, and Blueness Assessment With a Novel and Fully Validated HPLC Method for Simultaneous Analysis of Lumacaftor and Ivacaftor in Sweat

International Journal of Analytical Chemistry, Volume 2026, Issue 1, 2026.
Sweat is an alternative biological fluid to plasma, urine, hair, and saliva, and it is promising for various pharmaceutical research types. Excessive sweating is one of the symptoms of cystic fibrosis, a hereditary disease. In this study, an easy, simple, applicable, and economical HPLC method was proposed for sweat analysis of the lumacaftor/ivacaftor
Serkan Levent, Abeer Elriş, Saniye Özcan, Nafiz Öncü Can, Deepali   +4 more
wiley   +1 more source

Predictive factors for lumacaftor/ivacaftor clinical response [PDF]

Journal of Cystic Fibrosis, 2019
Ivacaftor-lumacaftor combination therapy corrects the F508 del-CFTR mutated protein which causes Cystic Fibrosis. The clinical response of the patients treated with the combination therapy is highly variable. This study aimed to determine factors involved in the individual's response to lumacaftor-ivacaftor therapy.Sweat test was assessed at baseline ...
Masson, Alexandra, Schneider-Futschik, Elena K., Baatallah, Nesrine, Nguyen-Khoa, Thao, Girodon, Emmanuelle, Hatton, Aurélie, Flament, Thomas, Le Bourgeois, Muriel, Chedevergne, Frederique, Bailly, Céline, Kyrilli, Sylvia, Achimastos, Diane, Hinzpeter, Alexandre, Edelman, Aleksander, Sermet-Gaudelus, Isabelle   +14 more
openaire   +2 more sources

Integrating Multiple Omics and AI Screening Reveals That the Srp14/Flt1 Axis Exacerbates Neuroinflammation in Neuropathic Pain Through the PI3K Akt Signaling Pathway

International Journal of Clinical Practice, Volume 2026, Issue 1, 2026.
Neuropathic pain (NP) is a chronic pain condition caused by somatosensory nervous system damage or disease, often imposing a significant economic burden. Current treatments are often ineffective and may cause side effects or dependency, highlighting the need for an improved understanding of NP pathology.
Jianhua Lu, Fangran Yan, Zhihong Lai, Yumeng Lu, Zenghua Zhou, Zhiwen Luo   +5 more
wiley   +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