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What Is Most Suitable for Children With Cystic Fibrosis—The Relationship Between Spirometry, Oscillometry, and Multiple Breath Nitrogen Washout

Frontiers in Pediatrics, 2022
IntroductionIn cystic fibrosis (CF), pathological lung changes begin early in life. The technological progress currently gives many diagnostic possibilities. However, pulmonary function testing in children remains problematic.ObjectivesOur study aimed to
Magdalena Postek, Magdalena Postek, Katarzyna Walicka-Serzysko, Katarzyna Walicka-Serzysko, Justyna Milczewska, Justyna Milczewska, Dorota Sands, Dorota Sands   +7 more
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Scedosporium species and Lomentospora prolificans in Italian cystic fibrosis patients: prevalence and distribution in seven centers using a selective medium

Microbiologia Medica, 2021
Background and aims. Scedosporium species and Lomentospora prolificans are the filamentous fungi isolated more frequently from the cystic fibrosis (CF) lower airways, after Aspergillus fumigatus. Previous studies showed that, in CF patients, Scedosporium
Lisa Cariani, Arianna Biffi, Davide Guarneri, Antonio Teri, Daniela Girelli, Chiara Vignati, Patrizia Morelli, Silvia Campana, Daniela Dolce, Vita Dora Iula, Maria Rosaria Catania, Natalia Cirilli, Sara Rapagnani, Martina Rossitto, Ersilia Vita Fiscarelli   +14 more
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Establishing CREATE: lessons learned in setting up a training environment for early-career researchers in respiratory medicine

BMC Medical Education, 2022
Background The purpose of the National Health and Medical Research Council Centre of Research Excellence in Pulmonary Fibrosis (CRE-PF) is to improve and extend the lives of patients living with pulmonary fibrosis through the development of a ...
Katherine Christian, Alison Hey-Cunningham, Tamera Corte, Nicole Goh, Jade Jaffar, Paul Reynolds, Alan Teoh, Lauren Troy   +7 more
doaj   +1 more source

The Diagnosis of Cystic Fibrosis in Adult Age. Data from the Italian Registry

Diagnostics, 2021
Cystic Fibrosis (CF) registries are an essential resource of epidemiological and clinical data. Although the median age at diagnosis is usually reported in the first months of life, a minority of individuals is diagnosed during adulthood. The aim of this
Rita Padoan, Serena Quattrucci, Annalisa Amato, Vincenzo Carnovale, Donatello Salvatore, Marco Salvatore, Giuseppe Campagna   +6 more
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Association between Polyunsaturated Fatty Acid Profile and Bronchial Inflammation in Bronchiolitis Obliterans

Mediators of Inflammation, 2023
Introduction. Bronchiolitis obliterans (BO) is a chronic lung disease, which occurs after an insult to the lower airways, in particular after airway infections or after stem cell transplantation, and which results in persistent inflammation.
Silvija P. Jerkic, Laura Bächle, Ruth Pia Duecker, Lucia Gronau, Andreas G. Chiocchetti, Stefan Zielen, Ralf Schubert   +6 more
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A small molecule high throughput screening platform to profile conformational properties of nascent, ribosome-bound proteins

Scientific Reports, 2022
Genetic mutations cause a wide spectrum of human disease by disrupting protein folding, both during and after synthesis. Transient de-novo folding intermediates therefore represent potential drug targets for pharmacological correction of protein folding ...
Hideki Shishido, Jae Seok Yoon, William R. Skach   +2 more
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Cystic fibrosis in Tuscany: evolution of newborn screening strategies over time to the present

Italian Journal of Pediatrics, 2021
Background Cystic fibrosis (CF) is a life-threatening disease affecting about 1:3000 newborns in Caucasian populations. The introduction of newborn screening for cystic fibrosis (CF NBS) has improved the clinical outcomes of individuals with CF through ...
Matteo Botti, Vito Terlizzi, Michela Francalanci, Daniela Dolce, Maria Chiara Cavicchi, Anna Silvia Neri, Valeria Galici, Gianfranco Mergni, Lucia Zavataro, Claudia Centrone, Filippo Festini, Giovanni Taccetti   +11 more
doaj   +1 more source

Elexacaftor/tezacaftor/ivacaftor as rescue therapy in a patient with the cystic fibrosis genotype F508DEL/G1244E

Clinical Case Reports, 2021
Elexacaftor/tezacaftor/ivacaftor (ETI) is a cystic fibrosis (CF) transmembrane regulator (CFTR) modulator. It is known to be efficacious in stable patients with severe pneumopathy, but there are few data concerning its effectiveness during acute ...
Donatello Salvatore, Carmela Colangelo, Michele D’Andria, Giovanni Marsicovetere, Domenica Passarella   +4 more
doaj   +1 more source

Efficacy of the Simeox^® Airway Clearance Technology in the Homecare Treatment of Children with Clinically Stable Cystic Fibrosis: A Randomized Controlled Trial

Children, 2023
Background: Cystic fibrosis (CF) patients require regular airway clearance therapy (ACT). The aim of this study was to evaluate homecare therapeutic effects of a new ACT (Simeox®) added to the optimal standard of care, including home chest physiotherapy,
Dorota Sands, Katarzyna Walicka-Serzysko, Justyna Milczewska, Magdalena Postek, Natalia Jeneralska, Aleksandra Cichocka, Ewa Siedlecka, Urszula Borawska-Kowalczyk, Laurent Morin   +8 more
doaj   +1 more source

Paraquat ingestion in an adult with cystic fibrosis (CF): Diagnostic and management dilemmas

Respirology Case Reports, 2023
N,N'‐dimethyl‐4,4'bipyridinium dichloride (Paraquat) is a potent herbicide used widely in agriculture. We report the effects of an ingestion of paraquat by a 28 year old male with cystic fibrosis and the diagnostic and management challenges this posed in
Ieuan E. S. Evans, Shanal Kumar, Megan France, Daniel Smith, Philip Masel, George Tay, Daniel Henderson, Scott C. Bell, David Reid   +8 more
doaj   +1 more source