Results 31 to 40 of about 2,795 (144)

Impact of cystic fibrosis transmembrane conductance regulator modulator therapies on liver stiffness and liver enzymes: An observational perspective single‐center cohort study

JPGN Reports, EarlyView.
Abstract Objectives The efficacy of cystic fibrosis transmembrane conductance regulator (CFTR)‐modulator therapies in preventing or ameliorating cystic fibrosis liver disease (CFLD) by correcting CFTR in cholangiocytes is not well‐documented. This study aimed to assess liver function during CFTR‐modulators.
Laura Giugliano, Elisabetta Augustoni, Antonio Pizzol, Anna Opramolla, Cristina Chiadò, Anna Zagaria, Irene Esposito, Giulia Roberto, Alice Ponte, Andrea Evangelista, Paola Quarello, Michele Pinon, Pier Luigi Calvo   +12 more
wiley   +1 more source

Impact of elexacaftor-tezacaftor-ivacaftor in lung transplantation for cystic fibrosis in the United States

JHLT Open
Background: Cystic fibrosis (CF) is an autosomal recessive condition leading to progressive lung disease and often necessitating lung transplantation. Historically, CF has been one of the leading indications for lung transplants in the United States. The
Tahuanty A. Pena, MD, MS, Brittany Wright, PharmD, Kalpaj R. Parekh, MBBS, Julia Kleney-Tait, MD, PhD   +3 more
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

Aplasia Cutis Congenita in the Setting of Maternal Cystic Fibrosis

Annals of Internal Medicine: Clinical Cases, 2022
Aplasia cutis congenita is a rare scalp defect. An affected neonate was delivered after in utero exposure beginning in the midtrimester to elexacaftor/tezacaftor/ivacaftor for maternal cystic fibrosis management. Although aplasia cutis congenita has many
Govind Kallumkal, Robert Egerman
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

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

Novel reaction to new cystic fibrosis medication Trikafta

Clinical Case Reports, 2021
We present a novel case of an urticaria multiforme‐type drug reaction to the new cystic fibrosis medication Trikafta (elexacaftor + tezacaftor + ivacaftor).
Julian Stashower, Patrick Carr, Virginia Miller, Barrett Zlotoff   +3 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