...Stefanie Vincken; Sylvia Verbanck; Sue Braun; Nathalie Buyck; Cindy Zienebergh; Christiane Knoop; Eef Vanderhelst Introduction: Elexacaftor/tezacaftor/ivacaftor (ETI) has shown significant improvements in pulmonary and nutritional status in persons with cystic fibrosis (pwCF). Less is known about...

...Haley Belt; Evbu O. Enakpene; Judy L. Jensen; Alice P. Moe; Holly Carveth; Barbara C. Cahill; Nathan Hatton; Theodore G. Liou; Anwar Tandar Introduction: Patent foramen ovale (PFO) affects about 25% of the population. We studied outcomes in cystic fibrosis (CF). Methods: We conducted a case-control...

... increase the requirement of aminoglycoside (AG) antibiotics in people with cystic fibrosis (pwCF). Several studies have shown that AGs have a cumulative effect on ototoxicity. We aimed to investigate the relationship between AG exposure and ototoxicity by using 3 different methods in patients with CF...

...A. Susanne Dittrich; Markus Dumke; Ferdinand Kapl; Philipp Schneider; Sabine Wege; Simon Gräber; Mirjam Stahl; Felix J. Herth; Lutz Naehrlich; Marcus A. Mall; Olaf Sommerburg; German CF Registry Background: Pulmonary disease is the major cause for morbidity and mortality in cystic fibrosis (CF...

... with cystic fibrosis (pwCF). Objectives: We studied the effect of CFTR triple combination therapy elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) on sleep in pwCF. Method: This was a prospective, observational sleep study in clinically stable adult pwCF. All participants underwent overnight polysomnography...

...Elisabeth Kieninger; Corin Willers; Katrin Röthlisberger; Sophie Yammine; Orso Pusterla; Grzegorz Bauman; Enno Stranzinger; Oliver Bieri; Philipp Latzin; Carmen Casaulta Background: Inhalation therapy is one of the cornerstones of the daily treatment regimen in patients with cystic fibrosis (CF...

...Andrea Radine; Claudius Werner; Johanna Raidt; Gerard W. Dougherty; Laura Kerschke; Heymut Omran; Joerg Grosse-Onnebrink Background: Cough is a key symptom in patients with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD). Objective: The study objectives were to test whether cough...

..., restriction decreases the functional residual capacity (FRC) and forced vital capacity (FVC), while ventilation inhomogeneity (lung clearance index [LCI]) increases. Pulmonary function response to restriction is unknown in individuals with cystic fibrosis (CF). Objectives: To examine short-term effects...

...Tamara del Corral; Maria Àngels Cebrià i Iranzo; Ibai López-de-Uralde-Villanueva; Roberto Martínez-Alejos; Isabel Blanco; Jordi Vilaró Background: Exercise-based rehabilitation is already a part of cystic fibrosis (CF) treatment; however, patient adherence is low. Objectives: To assess...

...Friederike Sophie Magnet; Jens Callegari; Doris Dieninghoff; Marc Spielmanns; Jan Hendrik Storre; Claudia Schmoor; Wolfram Windisch Background: Pseudomonas aeruginosa infection impairs respiratory muscle function in adolescents with cystic fibrosis, but its impact on adult patients has not been...

...Alice Panchaud; Ermindo R. Di Paolo; Angela Koutsokera; Ursula Winterfeld; Etienne Weisskopf; David Baud; Alain Sauty; Chantal Csajka Health management of cystic fibrosis (CF) patients should be maximized during pregnancy and breastfeeding because of its significant impact on the maternal...

...Lilian Masoud-Landgraf; Sophia Johler; Alexandra Badura; Gebhard Feierl; Josefa Luxner; Ute Wagner-Eibel; Ernst Eber; Gernot Zarfel; Andrea J. Grisold Background: Cystic fibrosis (CF) is the most common life-limiting inherited disease in Caucasian populations. While pathological changes can be seen...

...Chris Burtin; Helge Hebestreit A relevant proportion of children and adults with cystic fibrosis (CF) have a marked decrease in exercise tolerance, which can be partly related to impaired muscle function and decreased physical activity levels in daily life, in addition to lung disease. Preliminary...

...Katharine Hurt; Diana Bilton Treatment of cystic fibrosis lung disease has developed from an understanding of the abnormal airway surface liquid resulting from a lack of function of the cystic fibrosis transmembrane regulator protein. Mucus plugging resulting in infection and inflammation leads...

...Bruce K. Rubin; Ronald W. Williams Patients with non-cystic fibrosis bronchiectasis (NCFB) share many of the respiratory symptoms and the disease progression of cystic fibrosis (CF). As there are no approved therapies for the management of NCFB, an approach has been to use therapies similar...

... and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements. Bronchiectasis Chest Children Computed tomography Cystic fibrosis Ivacaftor Pediatrics The recent...

...Argyris Tzouvelekis; Paschalis Ntolios; Demosthenes Bouros Chronic lung diseases such as idiopathic pulmonary fibrosis and cystic fibrosis or chronic obstructive pulmonary disease and asthma are leading causes of morbidity and mortality worldwide with a considerable human, societal and financial...

...Rubin I. Cohen; Nicole Ginsberg; Donna Tsang; Li Chen Wann; Xiaobing Ye; Shu Fang Liu Background: The mechanisms of fat mass (FM) loss in cystic fibrosis (CF) are poorly understood but could represent complex pathways involving dysregulation of appetite-modulating peptides and an amplified...

... perfusion, bullae, and emphysema in patients with cystic fibrosis. Objectives: The purpose of this study was to compare both modalities, CT and MRI, using the Helbich-Bhalla score with a special focus on reliable detection of a mosaic pattern. Methods: Out of 51 patients examined by MRI on a 1.5-Tesla...

... of pre-existing diabetes after lung transplantation (LT) in cystic fibrosis (CF) has never been described. Objectives: We aimed to assess the outcome of CF-related diabetes (CFRD) after LT, with the hypothesis that suppressing chronic inflammatory foci may improve glucose tolerance in some patients...