Results 31 to 40 of about 2,435 (141)

USO DE EXACAFTOR/ TEZACAFTOR /IVACAFTOR EN PACIENTES CON FIBROSIS QUÍSTICA POST-TRASPLANTE PULMONAR

Neumología Pediátrica, 2023
El uso de moduladores de CFTR en pacientes con fibrosis quística post trasplante pulmonar es un tema todavía controversial. Varias publicaciones reportan los beneficios del modulador elexacaftor/tezacaftor/ivacaftor en los síntomas extrapulmonares de la ...
Pablo Jorquera Pinto
doaj   +1 more source

Adapted Linear Binning Method to Assess Pulmonary Ventilation and Perfusion in Children Using Lung Matrix Pencil Decomposition (MP)‐MRI

Magnetic Resonance in Medicine, EarlyView.
ABSTRACT Purpose To adapt and validate a linear binning technique, developed for hyper‐polarized 129Xe MRI, for functional lung MRI with matrix‐pencil decomposition (MP)‐MRI. Methods First, a dedicated normalization was applied to the perfusion‐weighted and the ventilation‐weighted map histograms.
Marion Curdy, Carmen Streibel, Oliver Bieri, Elisabeth Kieninger, Florian Wyler, Lukas Ebner, Grzegorz Bauman, Philipp Latzin   +7 more
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

Updated EAACI Statement on Drug Hypersensitivity Skin Testing: Methodology and Non‐Irritative Concentrations

Allergy, EarlyView.
ABSTRACT These updated EAACI guidelines aim to standardize skin testing methodologies for both immediate and non‐immediate drug hypersensitivity reactions. For immediate reactions, the optimal testing window is 4–6 weeks post‐reaction; whereas beyond 6 months, false‐negative results increase.
Annick Barbaud, Lene Heise Garvey, Elizabeth Phillips, Michael Ardern‐Jones, Sevim Bavbek, Patrizia Bonadonna, Jean‐Christoph Caubet, Ana‐Maria Copaescu, Valentina Gueli, Jose Julio Laguna, Ricardo Madrigal‐Burgaleta, Francesca Mori, Vito Sabato, Maria J Torres, Knut Brockow   +14 more
wiley   +1 more source

Successful pregnancy in a cystic fibrosis patient with a severe impairment of lung function receiving Elexacaftor-Tezacaftor-Ivacaftor

Respiratory Medicine Case Reports, 2022
Before the arrival of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulators women with CF and impaired lung function were experiencing a high risk of complications and mortality during and the years after pregnancy.
Zisis Balmpouzis, Annabelle Faure van Rossum, David Baud, Alice Panchaud, Georgia Mitropoulou, Jesica Mazza Stalder, Angela Koutsokera   +6 more
doaj   +1 more source

Cystic Fibrosis and Colorectal Cancer Risk: Reprogramming of the Intestinal Epithelial Niche and Cell‐State Plasticity in the CFTR Modulator Era

Cell Proliferation, EarlyView.
In cystic fibrosis disorder, CFTR dysfunction drives multilayered intestinal niche disruption, including dysbiosis, inflammation, hypoxia, metabolic stress and genotoxic injury, promoting cell‐state plasticity and a pre‐neoplastic ecosystem. CFTR modulators and environmental factors may partially reshape these processes, highlighting opportunities for ...
Bala Umashankar, Ines Pankonien, Margarida Amaral, Chee Y. Ooi, Thierry Jardé, Shafagh A. Waters   +5 more
wiley   +1 more source

Longitudinal profiling of the intestinal microbiome in children with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor

mBio
The intestinal microbiome influences growth and disease progression in children with cystic fibrosis (CF). Elexacaftor-tezacaftor-ivacaftor (ELX/TEZ/IVA), the newest pharmaceutical modulator for CF, restores the function of the pathogenic mutated CF ...
Seth A. Reasoner, Rachel Bernard, Adam Waalkes, Kelsi Penewit, Janessa Lewis, Andrew G. Sokolow, Rebekah F. Brown, Kathryn M. Edwards, Stephen J. Salipante, Maria Hadjifrangiskou, Maribeth R. Nicholson   +10 more
doaj   +1 more source

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

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

Efficacy of Trikafta (ELX/TEZ/IVA) & Symdeko (TEZ/IVA) in Treating Cystic Fibrosis with F508del Allele: A Systematic Review and Meta-analysis

Thoracic Research and Practice
The objective of the study was to assess and compare the efficacy of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) treatment with TEZ/IVA treatment in individuals diagnosed with cystic fibrosis (CF) and carrying the F508del allele.
Kainat Hussain, Sonali Karhana, Aakriti Garg, Mohd Ashif Khan   +3 more
doaj   +1 more source