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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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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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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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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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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Association between Polyunsaturated Fatty Acid Profile and Bronchial Inflammation in Bronchiolitis Obliterans by Silvija P. Jerkic, Laura Bächle, Ruth Pia Duecker, Lucia Gronau, Andreas G. Chiocchetti, Stefan Zielen, Ralf Schubert

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Pyoverdine, the Major Siderophore in Pseudomonas aeruginosa, Evades NGAL Recognition by Mary E. Peek, Abhinav Bhatnagar, Nael A. McCarty, Susu M. Zughaier

“…Pseudomonas aeruginosa is the most common pathogen that persists in the cystic fibrosis lungs. Bacteria such as P. aeruginosa secrete siderophores (iron-chelating molecules) and the host limits bacterial growth by producing neutrophil-gelatinase-associated lipocalin (NGAL) that specifically scavenges bacterial siderophores, therefore preventing bacteria from establishing infection. …”
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The Contribution of the Airway Epithelial Cell to Host Defense by Frauke Stanke

“…In the context of cystic fibrosis, the epithelial cell has been characterized in terms of its ion transport capabilities. …”
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Inflammation and ER Stress Downregulate BDH2 Expression and Dysregulate Intracellular Iron in Macrophages by Susu M. Zughaier, Brandon B. Stauffer, Nael A. McCarty

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Impact of elexacaftor/tezacaftor/ivacaftor (ETI) on the prescription refill rate (PRR) for inhaled medications in people with CF: an Italian multicenter analysis by Andrea Gramegna, Giuseppe Cimino, Marco Cipolli, Giovanna Gandini, Maria Adelaide Calderazzo, Angelica Profiti, Federica Piedepalumbo, Francesco Blasi

“…Abstract This retrospective population-based study investigated the impact of elexacaftor/tezacaftor/ivacaftor (ETI) therapy on inhaled medication adherence in people with cystic fibrosis (pwCF). Prescription refill rate (PRR) for several inhaled medications were compared before and after ETI introduction in three major Italian CF centers. …”
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Case Study: Analyzing CFTR Mutations and SNPs in Pulmonary Fibrosis Patients with Unclear Symptoms by Sahar Yousaf, null Sumaira, Iqbal Bano, Atia Rehman, Samra Kousar, Muhammad Usman Ghani, Mariam Shahid

“…Cystic fibrosis (CF) is a genetic monogenic disorder inherited in an autosomal recessive manner, marked by persistent airway infections in the endobronchial region. …”
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Motion-Genetic Testing is Useful in the Diagnosis of Nonhereditary Pancreatic Conditions: Arguments Against the Motion by Jonathan A Cohn

“…Mutations of two genes, the cystic fibrosis transmembrane conductance regulator gene (CFTR) and the pancreatic secretory trypsin inhibitor gene (PSTI), are associated with an increase in the risk of idiopathic chronic pancreatitis. …”
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Energy Expenditure in Infants in Health and Disease by Ross Shepherd

“…In cystic fibrosis, both TEE and REE are 25% higher than values in healthy infants. …”
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