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Mutational spectrum of CFTR in cystic fibrosis patients with gastrointestinal and hepatobiliary manifestations
CONCLUSION: The study reports novel and reported variants in the CFTR gene in CF patients in Pakistani population having distinct phenotypes. It also emphasizes screening suspected Pakistani CF patients for the p. (Met1Thr) variant because of its increased observance and prevalence in the study. Moreover, the findings also signify searching for additional pathogenic variants in the genome of CF patients, which may modify the phenotypes. The findings contribute valuable information for the diagnosis, genetic counseling, and potential therapeutic strategies for CF patients in Pakistan.PMID:38662334 | DOI:10.1007/s11033-024-0...
Source: Molecular Biology Reports - April 25, 2024 Category: Molecular Biology Authors: Nadia Waheed Rehmana Waris Maryam Naseer Ayesha Razzaq Nighat Haider Abid Ali Shah Asmat Ullah Source Type: research

The Impact of Transmissible Microbes: How the Cystic Fibrosis Community Mobilized Against Cepacia
Perspect Biol Med. 2023;66(1):89-106. doi: 10.1353/pbm.2023.0005.ABSTRACTLong before COVID-19 made social distancing familiar, people with cystic fibrosis (CF) already practiced such behaviors. CF is held up as a classic example of genetic disease, yet people with CF are also susceptible to bacteria from the environment and from other CF patients. Starting in the 1980s, a bacterial epidemic in the CF population highlighted clashing priorities of connection, physical safety, and environmental protection. Policymakers ultimately called for the physical separation of people with CF from one another via recommendations that re...
Source: Perspectives in Biology and Medicine - April 25, 2024 Category: Medical Ethics Authors: Rebecca Mueller Source Type: research

In vitro platform to model the function of ionocytes in the human airway epithelium
CONCLUSION: Our results suggest that ionocytes could have role beyond transepithelial ion transport by regulating epithelial properties and homeostasis in the airway epithelium.PMID:38664797 | DOI:10.1186/s12931-024-02800-7 (Source: Cell Research)
Source: Cell Research - April 25, 2024 Category: Cytology Authors: Marta Vil à-González Laetitia Pinte Ricardo Fradique Erika Causa Heleen Kool Mayuree Rodrat Carola Maria Morell Maha Al-Thani Linsey Porter Wenrui Guo Ruhina Maeshima Stephen L Hart Frank McCaughan Alessandra Granata David N Sheppard R Andres Floto Emma Source Type: research

In vitro platform to model the function of ionocytes in the human airway epithelium
Pulmonary ionocytes have been identified in the airway epithelium as a small population of ion transporting cells expressing high levels of CFTR (cystic fibrosis transmembrane conductance regulator), the gene mut... (Source: Respiratory Research)
Source: Respiratory Research - April 25, 2024 Category: Respiratory Medicine Authors: Marta Vil à-González, Laetitia Pinte, Ricardo Fradique, Erika Causa, Heleen Kool, Mayuree Rodrat, Carola Maria Morell, Maha Al-Thani, Linsey Porter, Wenrui Guo, Ruhina Maeshima, Stephen L. Hart, Frank McCaughan, Alessandra Granata, David N. Sheppard, R. Tags: Research Source Type: research

 Pseudomonas aeruginosa Lipid A Structural Variants Induce Altered Immune Responses
Am J Respir Cell Mol Biol. 2024 Apr 24. doi: 10.1165/rcmb.2024-0059OC. Online ahead of print.ABSTRACTPseudomonas aeruginosa causes chronic lung infection in cystic fibrosis (CF), resulting in structural lung damage and progressive pulmonary decline. P. aeruginosa in the CF lung undergoes numerous changes, adapting to host-specific airway pressures while establishing chronic infection. P. aeruginosa undergoes lipid A structural modification during CF chronic infection, not seen in any other disease state. Lipid A, the membrane anchor of lipopolysaccharide (i.e., endotoxin), comprises the majority of the outer membrane of Gr...
Source: American Journal of Respiratory Cell and Molecular Biology - April 24, 2024 Category: Molecular Biology Authors: Casey E Hofstaedter Ian P O'Keefe Charles M Met Ling Wu Jelly Vanderwoude Sunny Shin Stephen P Diggle Sebastian A Riquelme David A Rasko Yohei Doi Janette M Harro Benjamin T Kopp Robert K Ernst Source Type: research

 Pseudomonas aeruginosa Lipid A Structural Variants Induce Altered Immune Responses
Am J Respir Cell Mol Biol. 2024 Apr 24. doi: 10.1165/rcmb.2024-0059OC. Online ahead of print.ABSTRACTPseudomonas aeruginosa causes chronic lung infection in cystic fibrosis (CF), resulting in structural lung damage and progressive pulmonary decline. P. aeruginosa in the CF lung undergoes numerous changes, adapting to host-specific airway pressures while establishing chronic infection. P. aeruginosa undergoes lipid A structural modification during CF chronic infection, not seen in any other disease state. Lipid A, the membrane anchor of lipopolysaccharide (i.e., endotoxin), comprises the majority of the outer membrane of Gr...
Source: Am J Respir Cell Mol... - April 24, 2024 Category: Respiratory Medicine Authors: Casey E Hofstaedter Ian P O'Keefe Charles M Met Ling Wu Jelly Vanderwoude Sunny Shin Stephen P Diggle Sebastian A Riquelme David A Rasko Yohei Doi Janette M Harro Benjamin T Kopp Robert K Ernst Source Type: research

 Pseudomonas aeruginosa Lipid A Structural Variants Induce Altered Immune Responses
Am J Respir Cell Mol Biol. 2024 Apr 24. doi: 10.1165/rcmb.2024-0059OC. Online ahead of print.ABSTRACTPseudomonas aeruginosa causes chronic lung infection in cystic fibrosis (CF), resulting in structural lung damage and progressive pulmonary decline. P. aeruginosa in the CF lung undergoes numerous changes, adapting to host-specific airway pressures while establishing chronic infection. P. aeruginosa undergoes lipid A structural modification during CF chronic infection, not seen in any other disease state. Lipid A, the membrane anchor of lipopolysaccharide (i.e., endotoxin), comprises the majority of the outer membrane of Gr...
Source: Mol Biol Cell - April 24, 2024 Category: Molecular Biology Authors: Casey E Hofstaedter Ian P O'Keefe Charles M Met Ling Wu Jelly Vanderwoude Sunny Shin Stephen P Diggle Sebastian A Riquelme David A Rasko Yohei Doi Janette M Harro Benjamin T Kopp Robert K Ernst Source Type: research

Altered serine metabolism promotes drug tolerance in Mycobacterium abscessus via a WhiB7-mediated adaptive stress response
Antimicrob Agents Chemother. 2024 Apr 23:e0145623. doi: 10.1128/aac.01456-23. Online ahead of print.ABSTRACTMycobacterium abscessus is an emerging opportunistic pathogen responsible for chronic lung diseases, especially in patients with cystic fibrosis. Treatment failure of M. abscessus infections is primarily associated with intrinsic or acquired antibiotic resistance. However, there is growing evidence that antibiotic tolerance, i.e., the ability of bacteria to transiently survive exposure to bactericidal antibiotics through physiological adaptations, contributes to the relapse of chronic infections and the emergence of ...
Source: Antimicrobial Agents and Chemotherapy - April 23, 2024 Category: Microbiology Authors: C élia Bernard Yi Liu G érald Larrouy-Maumus Christophe Guilhot Kaymeuang Cam Christian Chalut Source Type: research

Lytic bacteriophages induce the secretion of antiviral and proinflammatory cytokines from human respiratory epithelial cells
by Paula F. Zamora, Thomas G. Reidy, Catherine R. Armbruster, Ming Sun, Daria Van Tyne, Paul E. Turner, Jonathan L. Koff, Jennifer M. Bomberger Phage therapy is a therapeutic approach to treat multidrug-resistant (MDR) infections that employs lytic bacteriophages (phages) to eliminate bacteria. Despite the abundant evidence for its success as an antimicrobial in Eastern Europe, there is scarce data regarding its effects on the human host. Here, we aimed to understand how lytic phages interact with cells of the airway epithelium, the tissue site that is colonized by bacterial biofilms in numerous chronic respiratory disord...
Source: PLoS Biology: Archived Table of Contents - April 23, 2024 Category: Biology Authors: Paula F. Zamora Source Type: research

Changes in urinary glutathione sulfonamide (GSA) levels between admission and discharge of patients with cystic fibrosis
Data from the Australian Early Respiratory Surveillance Team for Cystic Fibrosis (AREST CF) program suggests that pulmonary inflammation, infection, and structural disease can be present not only in the absence of clinical symptoms, but in patients as young as 3 months of age [1,2]. Furthermore, acute pulmonary exacerbations, particularly those requiring hospitalisation, are significantly correlated with loss of lung function and adverse lung health outcomes [3,4]. Published data suggest that exaggerated neutrophilic inflammation is a primary driver of early CF lung disease progression [1,2,5 –7]. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - April 23, 2024 Category: Respiratory Medicine Authors: Tamara L Blake, Peter D Sly, Isabella Andersen, Claire E Wainwright, David W Reid, Scott C Bell, Briana R Smith, Anthony J Kettle, Nina Dickerhof Tags: Short Communication Source Type: research

Cystic fibrosis newborn screening in Switzerland – evaluation and scenarios for improvement after 11 years of follow-up
Newborn bloodspot screening (NBS) for cystic fibrosis (CF) has become an essential component of early CF diagnosis in many countries [1,2]. The main goal of NBS programmes is to detect infants with a treatable disease early, to initiate treatment, prevent symptoms, and decrease mortality [3]. While CF-NBS programmes have benefits for children with CF, it is important to be aware of possible harmful effects. Every follow-up test, such as a second IRT measurement or sweat test, due to a false-positive CF-NBS result causes distress for the family and burdens the health-care system [4 –6]. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - April 23, 2024 Category: Respiratory Medicine Authors: ESL Pedersen, CM de Jong Carmen, M Jurca, DO Berger, J Sanz, SHM Sluka, M Poms, MR Baumgartner, N Regamey, CE Kuehni, J Barben, CS Rueegg, Swiss Cystic Fibrosis Newborn Screening Group Tags: Original Article Source Type: research

Impact of pseudomonas aeruginosa on resource utilization and costs in patients with exacerbated non-cystic fibrosis bronchiectasis
Conclusion Exacerbated NCFB with PA+ was associated with increased antibiotic usage, greater resource utilization, and increased costs. The major contributor to the cost differences was the use of inpatient services. Treatment strategies aimed at reducing the need for inpatient treatment could lessen the disparities observed in patients with NCFB.PMID:38646702 | DOI:10.1080/13696998.2024.2340382 (Source: Journal of Medical Economics)
Source: Journal of Medical Economics - April 22, 2024 Category: Health Management Authors: Meg Franklin Michael E Minshall Federica Pontenani Sunjay Devarajan Source Type: research

Concentrations of dehydroepiandrosterone-sulphate (DHEA-S) in people with cystic fibrosis on and off elexacaftor-tezacaftor-ivacaftor
Cystic fibrosis (CF) is a multi-system disorder caused by defects in the CF trans-membrane conductance regulator. As people with CF (pwCF) lead longer and healthier lives, the impact of steroid hormones on their health has become increasingly important. DHEA-S and its potential implications on the health of adults with CF has not been well-explored. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - April 22, 2024 Category: Respiratory Medicine Authors: Shadaan Abid, Lauren Shaffer, Andrew Bayat, Minjae Lee, Ashley Keller, Raksha Jain Tags: Short Communication Source Type: research

The anoctamins: Structure and function
Cell Calcium. 2024 Apr 15;120:102885. doi: 10.1016/j.ceca.2024.102885. Online ahead of print.ABSTRACTWhen activated by increase in intracellular Ca2+, anoctamins (TMEM16 proteins) operate as phospholipid scramblases and as ion channels. Anoctamin 1 (ANO1) is the Ca2+-activated epithelial anion-selective channel that is coexpressed together with the abundant scramblase ANO6 and additional intracellular anoctamins. In salivary and pancreatic glands, ANO1 is tightly packed in the apical membrane and secretes Cl-. Epithelia of airways and gut use cystic fibrosis transmembrane conductance regulator (CFTR) as an apical Cl- exit ...
Source: Cell Calcium - April 20, 2024 Category: Cytology Authors: Rainer Schreiber Jiraporn Ousingsawat Karl Kunzelmann Source Type: research