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Introduction of Ivacaftor/Lumacaftor in Children With Cystic Fibrosis Homozygous for F508del in the Netherlands: A Nationwide Real-Life Study.

Pediatr Pulmonol

January 2025

Beatrix Children's Hospital Department of Pediatric Pulmonology and Pediatric Allergy, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.

Introduction: Lumacaftor/ivacaftor (lum/iva) was introduced in the Netherlands in 2017. We investigated 1-year efficacy of lum/iva on lung function and small airway and structural lung disease evaluated by multiple breath nitrogen washout and CT scan. Additionally, we investigated effects of lum/iva on exacerbations, anthropometry, sweat chloride and safety in children with CF in the Netherlands.

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Public Health.

Alzheimers Dement

December 2024

National Center for Neurological Disorders, Shanghai, China.

Background: Whether lung function prospectively affects cognitive brain health independent of their overlapping factors remains largely unknown. This study aimed to investigate the longitudinal association between decreased lung function and cognitive brain health and to explore underlying biological and brain structural mechanisms.

Method: This population-based cohort included 43,1834 non-demented participants with spirometry from the UK Biobank.

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Background And Objectives: Patients with primary ciliary dyskinesia (PCD) have a reduction in exercise capacity from the early stages. Although there are studies investigating these patients' lower extremity exercise capacity using various methodologies, there needs to be more research on the functional exercise capacity of the upper extremities. This study aimed to compare pulmonary function, upper extremity functional exercise capacity, muscle oxygenation, muscle strength, and physical activity level in children with PCD and controls.

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Background: Pulmonary ischemia-reperfusion injury (PIRI) is a major cause of fatality post-lung transplantation. Though some long non-coding RNAs (lncRNAs) have been studied in acute lung injury (ALI), their effects on PIRI remain undefined. The present study aims to explore the underlying mechanism of small nucleolar RNA host gene 16 (SNHG16) in PIRI.

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Public Health.

Alzheimers Dement

December 2024

Centre for Brain Research, Indian Institute of Science, Bangalore, Karnataka, India.

Background: Worsening pulmonary health may be independently associated with declining cognitive function. However, the association between specific types of pulmonary impairment with cognitive function is not well understood. The present study aims to determine the differential impact of Chronic Obstructive Pulmonary Disease (COPD) and Preserved Ratio Impaired Spirometry (PRISm) in a rural Indian aging cohort METHOD: Data from participants (n = 1223) of the Srinivaspura Aging, Neuro Senescence and COGnition (SANSCOG) cohort were analyzed.

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