Results 61 to 70 of about 9,288 (216)

Aligning clinical care with consumer priorities: an adult cystic fibrosis centre experience of co‐designing a model of care

Internal Medicine Journal, EarlyView.
Abstract Cystic fibrosis (CF) is a multi‐system genetic condition, and CF modulator therapies have transformed health outcomes promising improved longevity. Our aim was to co‐design and implement a nested endocrine and metabolic model of care called CF Endocrine for adults with CF that aligned with consumer and healthcare provider priorities and ...
Shanal Kumar, Vanessa Moore, Daniel Smith, Angela Matson   +3 more
wiley   +1 more source

Efectividad de ivacaftor en vida real en niños con fibrosis quística y mutación G551D

Anales de Pediatría, 2019
Resumen: Introducción: Ivacaftor es un potenciador de la proteína reguladora de la conductancia transmembrana de la fibrosis quística (CFTR) que ha demostrado en ensayos clínicos mejoría del estado nutricional y la función pulmonar de pacientes con ...
David Gomez-Pastrana, Chinedu Nwokoro, Mike McLean, Sarah Brown, Nanna Christiansen, Caroline S. Pao   +5 more
doaj   +1 more source

Ivacaftor: A Novel Mutation Modulating Drug [PDF]

Journal of Clinical and Diagnostic Research, 2014
Cystic fibrosis (CF) is multisystemic disorder presenting in newborn period to adulthood, predominantly affecting respiratory system. It is caused by mutation in CF transmembrane conductance regulator gene.
Harit Kapoor, Astha Koolwal, Ankur Singh
doaj   +1 more source

Comparison of pharmacogenomic guidance in Australian prescribing resources and international pharmacogenomic guidelines

Internal Medicine Journal, EarlyView.
Abstract Background Pharmacogenomic‐guided medication management optimises drug therapy to enhance patient outcomes. Despite clinical utility, implementation in Australia remains limited, partly due to the lack of clear and consistent guidance. Aim This study evaluated the presence and consistency of pharmacogenomic testing indication categories and ...
Ruby Soueid, Jackson L. A. Thomas, Luke B. Hesson, Stephen Hughes, Sophie L. Stocker   +4 more
wiley   +1 more source

Ivacaftor for cystic fibrosis

BMJ, 2018
### What you need to know A 33 year old man with cystic fibrosis has come to discuss a new therapy for which he is eligible because of his genotype Phe508del/Gly551Asp. He has moderate lung function impairment with a forced expiratory volume in one second (FEV1) of 67% of predicted, which has declined from 85% over the preceding five years.
Barry, Peter J., Donaldson, Anna L., Jones, Andrew M.   +2 more
openaire   +3 more sources

Ion Channel Dysfunction and Therapeutic Targeting in Salivary Gland Disorders

Oral Diseases, EarlyView.
ABSTRACT Objective Salivary gland hypofunction and xerostomia represent major clinical complications of radiation therapy, autoimmune disorders such as Sjögren's disease, and inherited epithelial ion transport defects. This review integrates current knowledge on ion channel dysfunction as a central mechanistic driver of salivary gland pathology and ...
Tarek Mohamed Abd El‐Aziz, Shankara N. Varadarajan, Viktor Drel, Prakash Arullampalam, Mahmoud I. Shoulkamy, Ahmed Al‐Sabi, Brij B. Singh   +6 more
wiley   +1 more source

Ivacaftor: Five‐year outcomes in the West of Scotland cystic fibrosis population

The Clinical Respiratory Journal, 2023
Introduction Ivacaftor has shown to be effective in patients with cystic fibrosis (CF) with a G551D mutation. Objectives This work aims to evaluate ivacaftor's effectiveness and safety in the real world, over 5 years, in the West of Scotland CF ...
Yasmin Al‐Din, Carol Dryden, Gordon MacGregor, David Young, Cristina Coelho   +4 more
doaj   +1 more source

Uncovering Cystic Fibrosis Carrier: Insights From a Heterozygous CFTR‐F508del Rabbit Model

International Forum of Allergy &Rhinology, Volume 16, Issue 8, Page 788-796, August 2026.
ABSTRACT Background Chronic rhinosinusitis (CRS) is a heterogeneous inflammatory disorder frequently associated with impaired mucociliary clearance and bacterial infection. Individuals carrying a single cystic fibrosis transmembrane conductance regulator (CFTR) mutation exhibit partial CFTR dysfunction and are increasingly recognized as being at risk ...
Do‐Yeon Cho, Alexis E. McFeely, Daniel Skinner, Shaoyan Zhang, Justin Hedvat, Weslee Ping‐ay, Daniel M. Beswick, Justin H. Turner, Bradford A. Woodworth, Jie Xu   +9 more
wiley   +1 more source

Successful Rapid Desensitization to Ceftazidime/Avibactam in a Patient With Cystic Fibrosis and Multidrug‐Resistant Pseudomonas aeruginosa Pneumonia: A Case Report

Clinical Case Reports, Volume 14, Issue 8, August 2026.
ABSTRACT Ceftazidime/avibactam (CAZ‐AVI) is an important therapeutic option for multidrug‐resistant (MDR) Pseudomonas aeruginosa infections; however, hypersensitivity reactions may preclude its use and create significant therapeutic challenges. We report a 21‐year‐old woman with cystic fibrosis and recurrent MDR P.
Zuoren Zhou, Jinqi Zhu, Ge Wu, Xi Kang, Qing Wang, Jie Liu, Yan Jiang   +6 more
wiley   +1 more source

CFTR Modulator Therapy Enhances Peripheral Blood Monocyte Contributions to Immune Responses in People With Cystic Fibrosis

Frontiers in Pharmacology, 2020
BackgroundCFTR modulators decrease some etiologies of CF airway inflammation; however, data indicate that non-resolving airway infection and inflammation persist in individuals with CF and chronic bacterial infections.
Katherine B. Hisert, Katherine B. Hisert, Timothy P. Birkland, Kelly Q. Schoenfelt, Matthew E. Long, Brenda Grogan, Suzanne Carter, W. Conrad Liles, Edward F. McKone, Lev Becker, Anne M. Manicone, Sina A. Gharib   +11 more
doaj   +1 more source