Results 81 to 90 of about 3,564 (173)

Utility of Fecal Elastase‐1 in Estimating Exocrine Pancreatic Function in Cystic Fibrosis: A Scoping Review

open access: yesPediatric Pulmonology, Volume 61, Issue 5, May 2026.
ABSTRACT Background In people with cystic fibrosis (pwCF), identification of exocrine pancreatic insufficiency (EPI) is essential to prevent steatorrhea and, if not managed actively, can lead to catastrophic consequences. Fecal elastase‐1 (FE‐1) is a widely used test to screen for EPI in cystic fibrosis (CF).
Senthilkumar Sankararaman   +3 more
wiley   +1 more source

Function and Structure Relationships With Inflammation Differ in Two Chronic Suppurative Lung Diseases

open access: yesPediatric Pulmonology, Volume 61, Issue 5, May 2026.
ABSTRACT Rationale Cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) are characterized by neutrophilic airway inflammation but differ in clinical features. Objectives We investigated relationships of pulmonary and systemic inflammatory markers with functional and structural lung disease.
Teresa Fuchs   +9 more
wiley   +1 more source

Tezacaftor/Ivacaftor in Subjects with Cystic Fibrosis and F508del/F508del-CFTR or F508del/G551D-CFTR.

open access: yes, 2017
RATIONALE: Tezacaftor (formerly VX-661) is an investigational small molecule that improves processing and trafficking of the cystic fibrosis transmembrane conductance regulator (CFTR) in vitro, and improves CFTR function alone and in combination with ...
Tullis, E   +9 more
core   +2 more sources

The Impact of Cystic Fibrosis‐Related Diabetes on Barriers to Self‐Management

open access: yesPediatric Pulmonology, Volume 61, Issue 5, May 2026.
ABSTRACT Background Cystic fibrosis (CF) is a complex disease requiring adherence to an intensive medical regimen to maintain health. For those who develop CF‐related diabetes (CFRD, 20% adolescents, 30%–50% adults), daily self‐management is additionally complex and burdensome.
Rebecca J. Vitale   +7 more
wiley   +1 more source

Lung function improvement on triple modulators: high-resolution, nationwide data from the Danish Cystic Fibrosis Cohort

open access: yesERJ Open Research
Background People living with cystic fibrosis in Denmark had early, universal access to triple modulator treatment with elexacaftor/tezacaftor/ivacaftor.
Christian Leo-Hansen   +19 more
doaj   +1 more source

Integrating Human Intestinal Organoids into FDA's New Approach Methodologies for Drug Discovery

open access: yesAdvanced Science, Volume 13, Issue 20, 9 April 2026.
Illustration summarizes how human intestinal organoids (HIOs) are becoming transformative in preclinical research. Preclinical drug discovery pipelines often rely on animal models for ADMET studies, even though interspecies ADME gaps, poor external validity, and high attrition rates are common.
Debarun Patra   +6 more
wiley   +1 more source

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

open access: yesAnales 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   +14 more
doaj   +1 more source

3D Automated Segmentation of Bronchial Abnormalities on Ultrashort Echo Time MRI: A Quantitative MR Outcome in Cystic Fibrosis

open access: yesJournal of Magnetic Resonance Imaging, Volume 63, Issue 4, Page 1190-1200, April 2026.
ABSTRACT Background Cystic fibrosis (CF) monitoring relies on computed tomography (CT), but ultra‐short echo time MRI (UTE‐MRI) offers a radiation‐free alternative. However, its clinical adoption is hindered by the laborious and subjective manual analysis, which prevents standardized quantification of bronchial abnormalities.
Amel Imene Hadj Bouzid   +11 more
wiley   +1 more source

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

open access: yesAnales 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   +20 more
doaj   +1 more source

Patient‐Derived Intestinal Organoids in the Global Cystic Fibrosis Landscape

open access: yesPediatric Pulmonology, Volume 61, Issue 4, April 2026.
ABSTRACT Cystic fibrosis (CF) care has advanced rapidly, yet diagnosis and inclusion in patient registries remain severely limited in low‐ and middle‐income countries (LMICs). Barriers include restricted newborn screening, limited availability of sweat chloride testing, and underrepresentation of non‐European CFTR variants in standard panels.
Suzanne Kroes   +7 more
wiley   +1 more source

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