Evidence for the Involvement of Lipid Rafts and Plasma Membrane Sphingolipid Hydrolases in Pseudomonas aeruginosa Infection of Cystic Fibrosis Bronchial Epithelial Cells by Domitilla Schiumarini, Nicoletta Loberto, Giulia Mancini, Rosaria Bassi, Paola Giussani, Elena Chiricozzi, Maura Samarani, Silvia Munari, Anna Tamanini, Giulio Cabrini, Giuseppe Lippi, Maria Cristina Dechecchi, Sandro Sonnino, Massimo Aureli

“…Cystic fibrosis (CF) is the most common autosomal genetic recessive disease caused by mutations of gene encoding for the cystic fibrosis transmembrane conductance regulator. …”
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Targeted Antibiotic Prophylaxis for Lung Transplantation in Cystic Fibrosis Patients Colonised with Pseudomonas aeruginosa Using Multiple Combination Bactericidal Testing by Helmy Haja Mydin, Paul A. Corris, Audrey Nicholson, John D. Perry, Gerard Meachery, Emma C. L. Marrs, Steven Peart, Christine Fagan, James L. Lordan, Andrew J. Fisher, Frances K. Gould

“…Early infection is a recognised complication after lung transplantation in patients with cystic fibrosis (CF). Our centre uses multiple combination bactericidal testing (MCBT) when determining appropriate peritransplant prophylactic regimens. …”
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A Pilot Study to Compare Tobramycin 80 mg Injectable Preparation wth 300 mg Solution for Inhalation in Cystic Fibrosis Patients by Wilfried H Nikolaizik, Delia Vietzke, Felix Ratjen

“…BACKGROUND: Inhaled tobramycin has been shown to improve lung function in cystic fibrosis (CF) patients chronically infected with Pseudomonas aeruginosa. …”
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Using Community Engagement to Create a Telecoaching Intervention to Improve Self-Management in Adolescents and Young Adults With Cystic Fibrosis: Qualitative Study by Christina L Duncan, Emily F Muther, Jennifer J Lindwall, Kristine Durkin, Elizabeth Ruvalcaba, Eliza Williamson, Corrine Ahrabi-Nejad, Evelyn Bord, Angela Green, Megan L Harrison, Deepika Polineni

“… BackgroundAdolescents and young adults (AYA) with cystic fibrosis (CF) are at risk for deviating from their daily treatment regimen due to significant time burden, complicated daily therapies, and life stressors. …”
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Enfrentamento e Maternidade no contexto da Fibrose Cística: estudos de caso // Coping and Maternity in the context of Cystic Fibrosis: case studies by Juliana Laux Soares Schenkel, Tagma Marina Schneider Donelli, Tonantzin Gonçalves

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Body composition changes and clinical outcomes in pediatric cystic fibrosis during 24 months of lumacaftor ivacaftor therapy based on real-world data by Marcell Imrei, Adrienn F. Kéri, Éva Gács, Ildikó Gönczi, Melinda Meláth, Éva Kosaras, Botond Demeter, Csaba Péterfia, Klára Vass, Gyöngyi Székely, Klementina Ocskay, Andrea Párniczky

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BPI-ANCA Provides Additional Clinical Information to Anti-Pseudomonas Serology: Results from a Cohort of 117 Swedish Cystic Fibrosis Patients by Ulrika Lindberg, Malin Carlsson, Thomas Hellmark, Mårten Segelmark

“…Patients with cystic fibrosis (CF) colonized with Pseudomonas aeruginosa (P. aeruginosa) have worse prognosis compared with patients who are not. …”
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Differential Effects of Angelicin Analogues on NF-κB Activity and IL-8 Gene Expression in Cystic Fibrosis IB3-1 Cells by Ilaria Lampronti, Maria Giulia Manzione, Gianni Sacchetti, Davide Ferrari, Susanna Spisani, Valentino Bezzerri, Alessia Finotti, Monica Borgatti, Maria Cristina Dechecchi, Giorgia Miolo, Giovanni Marzaro, Giulio Cabrini, Roberto Gambari, Adriana Chilin

“…Other analogues displayed higher activities; in particular, the most interesting compounds showing relevant anti-inflammatory effects were found to cause 56–83% reduction of IL-8 mRNA expression at low concentrations (1–10 μM), without changes in cell proliferation pattern, demonstrating their potential interest for a possible development of anti-inflammatory therapy of cystic fibrosis.…”
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Gap Junctions Are Involved in the Rescue of CFTR-Dependent Chloride Efflux by Amniotic Mesenchymal Stem Cells in Coculture with Cystic Fibrosis CFBE41o- Cells by Annalucia Carbone, Roberto Zefferino, Elisa Beccia, Valeria Casavola, Stefano Castellani, Sante Di Gioia, Valentina Giannone, Manuela Seia, Antonella Angiolillo, Carla Colombo, Maria Favia, Massimo Conese

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The effectiveness of hyaluronic acid in reducing adverse effects associated with inhaled hypertonic saline therapy in patients with cystic fibrosis: A systematic review and meta-analysis by Sana Arshid, Saad Ehsan Ullah, Junaid Imran, Meeran Asher Syed, Aakash Choradia, Nicole Gousy, Sukhman Boparai, Mudassir Shoaib, Bidhan Bikram Shah, Aadarsh Netha

“…# Background Inhaled hypertonic saline (HS) is an effective mucolytic agent in patients with cystic fibrosis (CF). However, adverse events can impair the clinical utility of hypertonic saline (HS) in this patient population. …”
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Genetic Deletion and Pharmacological Inhibition of PI3Kγ Reduces Neutrophilic Airway Inflammation and Lung Damage in Mice with Cystic Fibrosis-Like Lung Disease by Maria Galluzzo, Elisa Ciraolo, Monica Lucattelli, Eriola Hoxha, Martina Ulrich, Carlo Cosimo Campa, Giuseppe Lungarella, Gerd Doring, Zhe Zhou-Suckow, Marcus Mall, Emilio Hirsch, Virginia De Rose

“…Neutrophil-dominated airway inflammation is a key feature of progressive lung damage in cystic fibrosis (CF). Thus, reducing airway inflammation is a major goal to prevent lung damage in CF. …”
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Daily versus three-times-weekly azithromycin in Chinese patients with non-cystic fibrosis bronchiectasis: protocol for a prospective, open-label and randomised controlled trial by Jing Li, Fei Chen, Wei Xiao, Feng Li, Lan Chen, Ting He, Yanxiong Mao, Aiping Zou, Bo Fan, Weihao Ni, Huimin You, Wenjiang Fu

“…Introduction Non-cystic fibrosis bronchiectasis (NCFB) brought a heavy healthcare burden worldwide. …”
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Human-based complex in vitro models: their promise and potential for rare disease therapeutics by Surat Parvatam, Francesca Pistollato, Lindsay J. Marshall, Fabia Furtmann, Devashree Jahagirdar, Mohua Chakraborty Choudhury, Sujata Mohanty, Harshita Mittal, Saveetha Meganathan, Rakesh Mishra

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Aspartic acid unveils as antibiofilm agent and tobramycin adjuvant against mucoid and small colony variants of Pseudomonas aeruginosa isolates in vitro within cystic fibrosis airway mucus by Rosana Monteiro, Ana Margarida Sousa, Maria Olívia Pereira

“…Antibiotics are central to managing airway infections in cystic fibrosis (CF), yet current treatments often fail due to the presence of Pseudomonas aeruginosa biofilms, settling down the need for seeking therapies targeting biofilms. …”
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PEG-PLGA nanoparticles deposited in Pseudomonas aeruginosa and Burkholderia cenocepacia by Tinatini Tchatchiashvili, Helena Duering, Lisa Mueller-Boetticher, Christian Grune, Dagmar Fischer, Mathias W. Pletz, Oliwia Makarewicz

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Aqueous extracts of Moringa oleifera and Cinnamomum cassia as promising sources of antibiofilm compounds against mucoid and small colony variants of Pseudomonas aeruginosa and Stap... by Eduarda Silva, Pedro Ferreira-Santos, José A. Teixeira, Maria Olivia Pereira, Cristina M.R. Rocha, Ana Margarida Sousa

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Rescue of Mutant CFTR Channel Activity by Investigational Co-Potentiator Therapy by Mafalda Bacalhau, Filipa C. Ferreira, Marcelo Folhadella M. F. Azevedo, Talita P. Rosa, Camilla D. Buarque, Miquéias Lopes-Pacheco

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Prevalence and characteristics of gastrointestinal disorders, medication use, and diagnostic interventions in pediatric patients with cystic fibrosis: a nested case–control analysis from the TriNetX EMR-derived global research network real-world dataset by Carolena Trocchia, Carolena Trocchia, Maua Mosha, Bailey Hamner, Neil Goldenberg, Racha Khalaf

Subjects: “…cystic fibrosis…”
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Aquagenic wrinkling of the palms: A report of two cases from a family medicine setting in Eastern Saudi Arabia and literature review by Maryam K. Alsoweilem, Rasha H. Alsheikh

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