Rouillard, Kaitlyn R Hill, David B Schoenfisch, Mark H
Published in
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
The combination of antibacterial and mucolytic actions makes nitric oxide (NO) an attractive dual-action cystic fibrosis (CF) therapeutic. The delivery of any therapeutic agent through pathological mucus is difficult, and the use of inhaled NO gas is inherently limited by toxicity concerns. Herein, we directly compare the ability of NO to eradicate...
Vermeulen, Francois Proesmans, Marijke Vermaelen, Mathias Boon, Mieke De Boeck, Kris
Published in
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Increased abundance of Enterobacteriaceae(EB) in the respiratory microbiome of young CF patients was reported to precede Pseudomonas aeruginosa(PA) colonisation. We explored whether impending PA colonisation can be predicted by growth of EB in routine airway cultures and whether EB contribute to CF lung disease severity. We retrospectively studied ...
Derella, Cassandra C Lee, Nichole Crandall, Reva Blackburn, Marsha Looney, Jacob Mangieri, Amie Rodriguez-Miguelez, Paula Tucker, Matthew A Harris, Ryan A
Published in
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
New treatments have improved life-expectancy in patients with cystic fibrosis (CF); however, cardiovascular health remains an area of concern in this population. Flow-mediated dilation (FMD), a non-invasive assessment of vascular endothelial function that predicts future cardiovascular disease and events, is attenuated in patients with CF compared ...
Padoan, Rita Cirilli, Natalia Falchetti, Diego Cesana, Bruno Mario
Published in
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Meconium ileus (MI) is a risk factor for poor outcomes in cystic fibrosis (CF) patients. The aim of this study was to identify the risk factors for poor 12-month clinical outcomes in MI-CF newborns. This retrospective, multicentre, observational study of MI-CF infants born 2009-2015 recorded their pre- and neonatal histories, intestinal occlusion t...
Amaral, Margarida D Beekman, Jeffrey M
Published in
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
The European Cystic Fibrosis Society (ECFS) Basic Science Working Group (BSWG) organized a session on the topic "Activating Alternative Chloride Channels to Treat CF: Friends or Foes?", within the 16th ECFS Basic Science Conference gathering ∼200 researchers from CF Basic Science. The session was organized into two round tables, each chaired by Mar...
Scaravilli, Vittorio Morlacchi, Letizia Corinna Merrino, Alessandra Piacentino, Edoardo Marasco, Davide Zanella, Alberto Nosotti, Mario Rosso, Lorenzo Polli, Federico Blasi, Francesco
...
Published in
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Predictors and outcomes of intraoperative extracorporeal membrane oxygenation (ECMO) during lung transplantation (LUTX) for cystic fibrosis (CF) are unknown. We retrospectively collected the clinical data at enlistment of the CF patients who underwent double LUTX from January 2013 to December 2018 at an Italian tertiary referral center. We compared...
Loukou, Ioanna Moustaki, Maria Plyta, Marina Douros, Konstantinos
Published in
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
The combination of lumacaftor and ivacaftor (LUM/IVA) is a recently approved CFTR modulator treatment for homozygous F508del CF patients. Our study aimed at evaluating the change in the rate of lung function decline after one-year treatment with LUM/IVA. The study evaluated patients homozygous for F508del, 12 to 23 years old. All had been treated f...
van Straten, Marcel Brody, Alan S Ernst, Caroline Guillerman, R Paul Tiddens, Harm A W M Nagle, Scott K
Published in
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Numerous issues must be addressed when developing standard operating procedures for clinical research studies involving chest computed tomography of lung disease in patients with cystic fibrosis (CF). Study success depends on the provision of adequate funding and the identification of personnel with the necessary expertise to conduct the study, alo...
Aksit, M A Bowling, A D Evans, T A Joynt, A T Osorio, D Patel, S West, N Merlo, C Sosnay, P R Cutting, G R
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Published in
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Cell-based studies have shown that W1282X generates a truncated protein that can be functionally augmented by modulators. However, modulator treatment of primary cells from individuals who carry two copies of W1282X generates no functional CFTR. To understand the lack of response to modulators, we investigated the effect of W1282X on CFTR RNA trans...
Kazmerski, Traci M Nelson, Eliza B Newman, Lori R Haviland, Miriam J Luff, Donna Leichtner, Alan M Hayes, Margaret M Miller, Elizabeth Emans, S Jean Sawicki, Gregory S
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Published in
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Sexual and reproductive health (SRH) is an important disease-specific concern for adolescent and young adult (AYA) women that is not consistently addressed in cystic fibrosis (CF) centers. This study identifies educational needs and preferences of interprofessional CF providers regarding SRH in AYA women with CF. Interprofessional CF providers part...
