Results 41 to 50 of about 2,379 (179)

Mejoría de la inflamación intestinal tras tratamiento con moduladores de la proteína CFTR en pacientes con fibrosis quística

Anales de Pediatría
Resumen: Introducción: Los tratamientos con moduladores de la proteína CFTR han mejorado la salud respiratoria y digestiva de los pacientes con fibrosis quística.
Ruth García Romero, Concepción López Cárdenes, Elena Crehuá Gaudiza, Marina Álvarez Beltrán, Mercedes Murray Hurtado, Carlos Tutau Gómez, Inés Loverdos Eseverri, Encarni Torcuato Rubio, Camila García Volpe, Enrique Salcedo Lobato, María Medina Martínez, Carmen Martin Fernández, Ana Moreno Álvarez, Ana Reyes Domínguez, David González Jiménez   +14 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

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

Improvement of intestinal inflammation after treatment with CFTR modulators in cystic fibrosis patients

Anales de Pediatría (English Edition)
Introduction: Treatments with CFTR protein modulators have improved respiratory and digestive health in patients with cystic fibrosis. Objective: To assess changes in intestinal inflammation through the analysis of fecal calprotectin in patients with ...
Ruth García Romero, Concepción López Cárdenes, Elena Crehuá Gaudiza, Marina Álvarez Beltrán, Mercedes Murray Hurtado, Carlos Tutau Gómez, Inés Loverdos Eseverri, Encarni Torcuato Rubio, Camila García Volpe, Enrique Salcedo Lobato, María Medina Martínez, Carmen Martin Fernández, Ana Moreno Álvarez, Ana Reyes Domínguez, David González Jiménez, Saioa Vicente Santamaría, Ana Tabares González, Jose Ramon Gutierrez Martinez, Sara Sierra San Nicolás, Pilar Ortiz Pérez, Luis Peña Quintana   +20 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

Divergent neurobehavioral effects of CFTR modulators elexacaftor and ivacaftor in mice

Acta Psychologica
Recent advances in cystic fibrosis transmembrane conductance regulator (CFTR) modulator combination therapies have markedly improved survival and quality of life for people with cystic fibrosis (CF).
Qian Ge, Amy Fagan, Hui Lu, Jianyang Du, Leah R. Reznikov   +4 more
doaj   +1 more source

Cystic fibrosis modulator therapy can reverse cystic bronchiectasis

Respirology Case Reports, 2023
Bronchiectasis is often considered progressive and irreversible, so cases of regression or reversal are an important step in understanding the underlying pathophysiological mechanisms.
Peter G. Middleton, Nicholas J. Simmonds
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

Organic Synthesis and Current Understanding of the Mechanisms of CFTR Modulator Drugs Ivacaftor, Tezacaftor, and Elexacaftor

Molecules
The monogenic rare disease Cystic Fibrosis (CF) is caused by mutations in the gene encoding the CF transmembrane conductance (CFTR) protein, an anion channel expressed at the apical plasma membrane of epithelial cells.
Filipa C. Ferreira, Camilla D. Buarque, Miquéias Lopes-Pacheco   +2 more
doaj   +1 more source

CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells

Frontiers in Physiology, 2021
A deficiency in cystic fibrosis transmembrane conductance regulator (CFTR) function in CF leads to chronic lung disease. CF is associated with abnormalities in fatty acids, ceramides, and cholesterol, their relationship with CF lung pathology is not ...
Mieke Veltman, Mieke Veltman, Juan B. De Sanctis, Marta Stolarczyk, Nikolai Klymiuk, Nikolai Klymiuk, Andrea Bähr, Andrea Bähr, Rutger W. Brouwer, Rutger W. Brouwer, Edwin Oole, Edwin Oole, Juhi Shah, Tomas Ozdian, Jie Liao, Carolina Martini, Danuta Radzioch, John W. Hanrahan, John W. Hanrahan, Bob J. Scholte, Bob J. Scholte   +20 more
doaj   +1 more source