Dynamic Perfluorinated Gas MRI Shows Improved Lung Ventilation in People with Cystic Fibrosis after Elexacaftor/Tezacaftor/Ivacaftor: An Observational Study

Article Critical Care Medicine

Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two F508del Alleles

Simon Y. Graeber et al.

Summary: ELX/TEZ/IVA improves CFTR function in patients with cystic fibrosis, leading to improved lung ventilation and morphology.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2022)

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Article Respiratory System

Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaf tor/tezacaf tor/ivacaf tor therapy in adults with cystic fibrosis

Lena Wucherpfennig et al.

Summary: This study found that therapy with Elexacaftor/Tezacaftor/Ivacaftor can improve chest and sinus abnormalities in adult cystic fibrosis patients, indicating the reversibility of structural lung and sinus abnormalities in patients with established disease.

JOURNAL OF CYSTIC FIBROSIS (2022)

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Article Radiology, Nuclear Medicine & Medical Imaging

Three-dimensional Ultrashort Echotime Magnetic Resonance Imaging for Combined Morphologic and Ventilation Imaging in Pediatric Patients With Pulmonary Disease

Simon Veldhoen et al.

Summary: The study demonstrates the initial results of spiral 3D UTE-MRI for combined morphologic and functional imaging in pediatric patients, showing short scanning time and potential monitoring applicability for various pulmonary diseases.

JOURNAL OF THORACIC IMAGING (2021)

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Article Radiology, Nuclear Medicine & Medical Imaging

Comparison of single breath hyperpolarized129Xe MRI with dynamic19F MRI in cystic fibrosis lung disease

Andrew McCallister et al.

Summary: In CF subjects, ventilation abnormalities can be identified by both (19)F and (HPXe)-Xe-129 imaging, but these abnormalities are not entirely congruent. (19)F and (HPXe)-Xe-129 imaging provide complementary information that allows for differentiation of normally ventilated, slowly ventilated, and non-ventilated regions in the lungs.

MAGNETIC RESONANCE IN MEDICINE (2021)

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Review Pediatrics

Entering the era of highly effective modulator therapies

Kavita Dave et al.

Summary: CFTR modulators are oral drugs that improve the function of the CFTR protein to treat cystic fibrosis, with ivacaftor as a highly effective potentiator that is available clinically worldwide. However, for common CFTR mutations like F508del, combination therapy of correctors and potentiators is required for clinical efficacy.

PEDIATRIC PULMONOLOGY (2021)

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Article Critical Care Medicine

A Phase 3 Open-Label Study of Elexacaftor/Tezacaftor/lvacaftor in Children 6 through 11 Years of Age with Cystic Fibrosis and at Least One F508del Allele

Edith T. Zemanick et al.

Summary: Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) has been shown to be safe and efficacious in children aged 6 through 11 with at least one F508del-CFTR allele, supporting its use in this patient population. The treatment improved lung function, respiratory scores, lung clearance, sweat chloride levels, and body mass index in these children over the 24-week period. The safety and pharmacokinetic profiles were consistent with those observed in older patients.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2021)

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Review Respiratory System