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Vanzacaftor-tezacaftor-deutivacaftor for children aged 6-11 years with cystic fibrosis (RIDGELINE Trial VX21-121-105): an analysis from a single-arm, phase 3 trial.
Lancet Respir Med
December 2024
Population Policy and Practice Department, UCL Great Ormond Street Institute of Child Health, University College London, London, UK; Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. Electronic address:
Background: In phase 2 trials in people with cystic fibrosis aged 18 years and older, vanzacaftor-tezacaftor-deutivacaftor has been shown to be a safe and effective, once-daily cystic fibrosis transmembrane conductance regulator (CFTR) modulator. Restoring normal CFTR function early in life has the potential to prevent manifestations of cystic fibrosis. We aimed to evaluate the safety, tolerability, efficacy, and pharmacokinetics of vanzacaftor-tezacaftor-deutivacaftor in children with cystic fibrosis aged 6-11 years.
View Article and Find Full Text PDFQuantitative Electroencephalography Alpha:Delta Ratio and Suppression Ratio Monitoring During Infant Aortic Arch Reconstruction.
Pediatr Neurol
December 2024
Department of Surgery, University of Rochester Medical Center, Rochester, New York. Electronic address:
Background: During infant aortic arch reconstruction, traditional electroencephalography (EEG) provides only qualitative data limiting neuromonitoring efficacy. Interhemispheric differences in the alpha:delta ratio (ADR) and suppression ratio (SR) measured using quantitative EEG generate numerical trends that may suggest cerebral ischemia. We hypothesized that the ADR and SR during cardiopulmonary bypass (CPB) would correlate with hemodynamics, and that ADR and SR interhemispheric differences would precede neurological injury from infants requiring aortic arch reconstruction.
View Article and Find Full Text PDFCare trajectories and adherence to respiratory management recommendations in persons living with amyotrophic lateral sclerosis: a ten-year cohort study in a French tertiary university centre.
Amyotroph Lateral Scler Frontotemporal Degener
January 2025
University Grenoble Alpes, Grenoble, France.
: This study determined real-life care trajectories before and after initiation of noninvasive ventilation (NIV) in patients with amyotrophic lateral sclerosis (ALS). Caregiver adherence to respiratory management recommendations and the associated survival rate of people with ALS were also assessed. : Data were obtained from a tertiary center prospective ALS database that included 10 years of follow-up data for people with ALS.
View Article and Find Full Text PDFIntroduction: Cerebral oximetry measurement using near-infrared spectroscopy (NIRS) has been highlighted as a technology that can provide noninvasive information on regional cerebral oxygen saturation (rSO2) during CPR even though its effectiveness has not been fully confirmed. The research focuses on the use of NIRS to predict the return of spontaneous circulation (ROSC) and neurological outcomes.
Objectives: The purpose of the study is to evaluate the validity of using regional cerebral oxygen saturation (rSO2) measurement compared to ETCO2 during CPR to and its association with ROSC, as well as to evaluate the neuroprognostic value of NIRS.
Prevalence of Inpatient Pulse Oximetry in Operative and Nonoperative Settings: A Multicenter Study.
CHEST Crit Care
December 2024
Department of Medicine, Boston University Chobanian & Avedisian School of Medicine, Pulmonary and Critical Care Medicine, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD; Department of Medicine, and the Department of Health Policy and Management, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD.
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