Effectiveness of Palivizumab in Preventing RSV Hospitalization in High Risk Children: A Real-World Perspective by Nusrat Homaira, William Rawlinson, Thomas L. Snelling, Adam Jaffe

“…Data are limited to allow commenting on the protective effect of palivizumab among other high risk children, including those with Down syndrome, cystic fibrosis, and haematological malignancy, indicating further research is warranted in these groups.…”
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Vest Chest Physiotherapy Airway Clearance is Associated with Nitric Oxide Metabolism by Joseph H. Sisson, Todd A. Wyatt, Jacqueline A. Pavlik, Pawanjit S. Sarna, Peter J. Murphy

“…Vest chest physiotherapy (VCPT) enhances airway clearance in cystic fibrosis (CF) by an unknown mechanism. Because cilia are sensitive to nitric oxide (NO), we hypothesized that VCPT enhances clearance by changing NO metabolism. …”
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Protocol for functional screening of CFTR-targeted genetic therapies in patient-derived organoids using DETECTOR deep-learning-based analysis by Mattijs Bulcaen, Ronald B. Liu, Kasper Gryspeert, Sam Thierie, Anabela S. Ramalho, François Vermeulen, Xavier Casadevall I Solvas, Marianne S. Carlon

“…Summary: Here, we present a protocol for the rapid functional screening of gene editing and addition strategies in patient-derived organoids using the deep-learning-based tool DETECTOR (detection of targeted editing of cystic fibrosis transmembrane conductance regulator [CFTR] in organoids). …”
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Diabetic Muscle Infarction: A Rare Cause of Acute Limb Pain in Dialysis Patients by G. De Vlieger, B. Bammens, F. Claus, R. Vos, K. Claes

“…In the current paper, we present two diabetic patients with cystic fibrosis, who are treated with automated peritoneal dialysis and suffered from episodic lower limb infarction. …”
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Insights into the diverse roles of the terminal oxidases in Burkholderia cenocepacia H111 by Sarah Paszti, Olivier Biner, Yilei Liu, Kim Bolli, Sarah Dorothy Jeggli, Gabriella Pessi, Leo Eberl

“…Abstract Burkholderia cenocepacia H111 is an obligate aerobic bacterium which has been isolated from a cystic fibrosis (CF) patient. In CF lungs the environment is considered micro-oxic or even oxygen-depleted due to bacterial activities and limited oxygen diffusion in the mucus layer. …”
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The CFTR Met 470 allele is associated with lower birth rates in fertile men from a population isolate. by Gülüm Kosova, Joseph K Pickrell, Joanna L Kelley, Patrick F McArdle, Alan R Shuldiner, Mark Abney, Carole Ober

“…Although little is known about the role of the cystic fibrosis transmembrane regulator (CFTR) gene in reproductive physiology, numerous variants in this gene have been implicated in etiology of male infertility due to congenital bilateral absence of the vas deferens (CBAVD). …”
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Qualitative study exploring the views and perceptions of parents/carers of young children with CF regarding the introduction of CFTR modulator therapy (The REVEAL study; PaRents pE... by Kevin W Southern, Sioned Haf Davies, Faye Wade, Heather Fogg, Adam Walsh

“…Background Cystic fibrosis (CF) is associated with a historically high treatment burden which causes anxiety and exhaustion for parents of children with CF, especially in the early years of a child’s life. …”
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Inhibition of CFTR-mediated intestinal chloride secretion by nornidulin: Cellular mechanisms and anti-secretory efficacy in human intestinal epithelial cells and human colonoids. by Chamnan Yibcharoenporn, Thidarat Kongkaew, Nichakorn Worakajit, Rungtiwa Khumjiang, Praphatsorn Saetang, Saravut Satitsri, Vatcharin Rukachaisirikul, Chatchai Muanprasat

“…Secretory diarrhea, a major global health concern, particularly among young children, is often characterized by excessive chloride secretion through the cystic fibrosis transmembrane conductance regulator (CFTR) channel. …”
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Awareness and attitudes of pregnant women concerning genetic disorders and pregnancy termination in northeastern Iran by Nafiseh Todarbary, Abouzar Irandegani, Mojtaba Meshkat, Aida Gholoobi, Tayebeh Hamzehloei

“…Education level was associated with abortion agreement for various conditions, including cleft lip or palate, Alzheimer’s disease, Turner syndrome, cystic fibrosis, phenylketonuria, Huntington’s disease, autism, fragile X syndrome, and epilepsy. …”
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Airway basal stem cell therapy for lung diseases: an emerging regenerative medicine strategy by Xingren Liu, Xin Wang, Xue Wu, Shuhua Zhan, Yan Yang, Caiyu Jiang

“…Airway BSCs, which are strongly capable of self-renewal and multi-lineage differentiation, can effectively attenuate airway epithelial injury caused by environmental factors or genetic disorders, such as cystic fibrosis. This review comprehensively explores the efficacy and action mechanisms of airway BSCs across various lung disease models and describes potential strategies for inducing pluripotent stem cells to differentiate into pulmonary epithelial lineages on the basis of the original research findings. …”
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Hypermutability bypasses genetic constraints in SCV phenotypic switching in Pseudomonas aeruginosa biofilms by Romina A. Tobares, Román A. Martino, Claudia A. Colque, Gaston L. Castillo Moro, Alejandro J. Moyano, Andrea G. Albarracín Orio, Andrea M. Smania

“…Abstract Biofilms are critical in the persistence of Pseudomonas aeruginosa infections, particularly in cystic fibrosis patients. This study explores the adaptive mechanisms behind the phenotypic switching between Small Colony Variants (SCVs) and revertant states in P. aeruginosa biofilms, emphasizing hypermutability due to Mismatch Repair System (MRS) deficiencies. …”
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Neonatal meconium aspiration syndrome associated with ABCA3 gene mutation and mycoplasma infection: a case report by Oliver Stelzig, Beatrix Mühlegger, Anna Zschocke, Ursula Kiechl-Kohlendorfer, Elke Griesmaier

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Role of the two-component system AmgRS in early resistance of Pseudomonas aeruginosa to cinnamaldehyde by Eline Dubois, Vladimir Spasovski, Patrick Plésiat, Catherine Llanes

“…This discovery highlights the pivotal role of AmgRS in mediating defense against aldehyde biocides, emphasizing its significance in the persistence of P. aeruginosa, a pathogen associated with hospital-acquired infections and cystic fibrosis, and underscores the potential impact on clinical treatment strategies.…”
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Factors Associated With Multi-Drug Resistant Organisms Among Bronchiectasis Patients: A Retrospective Study of Bronchiectasis Patients in Jordan by Al Oweidat K, Toubasi AA, Khraisat FA, Al-Sayegh TN, Al-Harasis LM, Albtoosh AS

“…The diagnosis of bronchiectasis was established through lung Computerized Tomography (CT) scans and clinical symptom assessment.Results: The study encompassed 235 patients, revealing a notably higher occurrence of MDROs among non-cystic fibrosis patients compared to their counterparts (P-value=0.001). …”
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Implications of Diminishing Lifespan Marginal Utility for Valuing Equity in Cost-Effectiveness Analysis by R. Scott Braithwaite

“…The example compares alternative valuations of approaches to reduce inequality in cystic fibrosis outcomes between US centers serving mostly White patients and centers serving mostly non-Black Hispanic patients, with versus without DMLU. …”
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Development of ICF-based patient-reported outcome and experience measures to study social participation among people with chronic diseases: a mixed-methods protocol by Christophe Vanroelen, Maxim Van den broecke, Sarah de Jong, Régine Kiasuwa Mbengi

“…This protocol presents the phases for the disease-specific development of patient-reported outcome and experience measures to assess SP and its potential determinants among people living in Belgium with cancer, cystic fibrosis, diabetes, HIV or a neuromuscular disease.Methods and analysis This protocol applies the PROMIS Instrument Development and Validation Scientific Standards and COnsensus-based Standards for the selection of health Measurement INstruments to develop PRMs in a disease-specific manner to quantify the components of the International Classification of Functioning, Disability and Health (ICF). …”
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DJK-5, an anti-biofilm peptide, increases Staphylococcus aureus sensitivity to colistin killing in co-biofilms with Pseudomonas aeruginosa by Samuel J. T. Wardell, Deborah B. Y. Yung, Anupriya Gupta, Mihnea Bostina, Joerg Overhage, Robert E. W. Hancock, Daniel Pletzer

“…In particular, pathogens such as Pseudomonas aeruginosa and Staphylococcus aureus are frequently co-isolated from the sputum of patients with cystic fibrosis and are commonly found in chronic wound infections. …”
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Fetoplacental Discrepancy with Normal Karyotype in Amniotic Fluid and Two Different Cell Lines in Placenta by Veronica Ortega, Christina Mendiola, Eric Williamson, Kenneth Higby, Gopalrao V. N. Velagaleti

“…Mother was a carrier for the Cystic Fibrosis (delta F508), Factor V Leiden mutations, HbD-Los Angeles and HbQ-India variants. …”
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Essential oils modulate virulence phenotypes in a multidrug-resistant pyomelanogenic Pseudomonas aeruginosa clinical isolate by Jayalekshmi Haripriyan, Christy Rose Binu, Nitasha D Menon, Muralidharan Vanuopadath, Malavika B. Hari, N Namitha, Kesiya Binoy, Anil Kumar, Bipin G. Nair, Victor Nizet, Geetha B. Kumar

“…Abstract Pyomelanogenic P. aeruginosa, frequently isolated from patients with urinary tract infections and cystic fibrosis, possesses the ability to withstand oxidative stress, contributing to virulence and resulting in persistent infections. …”
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Dual RNA sequencing of a co-culture model of Pseudomonas aeruginosa and human 2D upper airway organoids by Cayetano Pleguezuelos-Manzano, Wouter A. G. Beenker, Gijs J. F. van Son, Harry Begthel, Gimano D. Amatngalim, Jeffrey M. Beekman, Hans Clevers, Jeroen den Hertog

“…Abstract Pseudomonas aeruginosa is a Gram-negative bacterium that is notorious for airway infections in cystic fibrosis (CF) subjects. Bacterial quorum sensing (QS) coordinates virulence factor expression and biofilm formation at population level. …”
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