Articles tagged with Cystic Fibrosis
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Cystic fibrosis patients may benefit from a new lipid-based nanoparticle therapy that enables cells to produce the correct protein, allowing for improved respiratory function. The therapy has shown promise in treating all cystic fibrosis patients, regardless of their genetic mutation.
Researchers at the University of Iowa have discovered glandular myoepithelial cells that can regenerate airways after severe injury. These reserve stem cells can develop into new replacement cells in both submucosal glands and the lining of the airway, offering a potential therapeutic target for lung diseases.
Researchers found that E. coli from cystic fibrosis patients grew faster on glycerol compared to healthy controls. The study suggests a link between increased intestinal fat in CF patients and the selection of specific microorganisms.
The structure of chloride channel TMEM16A has been determined, revealing a unique activation mechanism that could restore hydration of the mucus layer in cystic fibrosis patients. Researchers believe that activating this channel could compensate for the defect in chloride ion secretion, paving the way for novel therapies.
A new study from Columbia University Irving Medical Center reveals that cancer gene PTEN works with CFTR to keep lung tissue free of potentially dangerous infections. The findings may explain why cystic fibrosis patients have a higher risk of GI cancer.
A team of researchers at the University of Montreal Hospital Research Centre has discovered a promising solution to improving treatments for cystic fibrosis. By adding quorum-sensing inhibitors to current drugs, they were able to restore treatment efficacy in cells of cystic fibrosis patients.
Researchers developed a simple, minimally-invasive test to predict which CFTR drug works best for each patient. Nasospheroids, grown from nasal samples, are exposed to drugs and their reactions measured, offering a quick alternative to rectal biopsy.
Research shows cystic fibrosis patients in the US have better nutritional status and faster lung function improvements compared to those in Canada. The study suggests early implementation of newborn screening and quality improvement initiatives may contribute to these positive trends.
A new lab model was developed to test new therapeutics for cystic fibrosis. The model uses primary bronchial epithelial cells from CF patients and infectious/inflammatory factors normally found in the CF airways.
A recent study found that genetic variants in epithelial sodium channels may help rehydrate the airways of cystic fibrosis patients, reducing bacterial build-up and promoting stable lung function. This discovery brings ENaCs into the spotlight as a potential new therapeutic target.
Researchers discovered two predator bacteria, Vampirovibrio and Bdellovibrio, in cystic fibrosis patients' lungs, which may prevent colonization of disease-associated bacteria. The findings indicate a more complex lung microbiota than previously thought.
Researchers developed a new type of sweat test that can overcome the challenge of ambiguous results in current tests. The test identifies alternative molecules found in sweat associated with CF, providing staging and prognostic information.
As more adults are diagnosed with cystic fibrosis, radiologists must monitor the disease's complex and heterogeneous spectrum. The condition affects approximately 70,000 people worldwide, with up to 7% of cases diagnosed in adulthood, often with subtle manifestations.
A computerized decision-making model has been developed to promote shared decision-making with cystic fibrosis patients, taking into account their preferences for measures of lung function and health. The tool helps prioritize home treatments by integrating patients' personal goals, values, and treatment preferences.
Research from the University of Iowa finds that cystic fibrosis changes the structure of mucus in airways, making it harder for lungs to clear particles. This abnormal mucus production contributes to lung infections and inflammation, a major cause of life-threatening illness in CF patients.
Researchers from Queensland University of Technology found that airborne pseudomonas aeruginosa bacteria can remain viable in the air for up to 45 minutes after being expelled by human coughs and sneezes. This could have significant implications for infection control in hospitals, particularly for patients with cystic fibrosis.
A phase 3 trial found that a lumacaftor and ivacaftor combination improves lung function in children with cystic fibrosis, reducing air clearance time by 15 days. The treatment also slows chloride levels in sweat, suggesting it corrects gene mutations.
Researchers discover a silent change in the CFTR gene that alters protein production and transport in cells. This finding reveals new insights into cystic fibrosis genetic complexity.
Researchers at Boston University's Center for Regenerative Medicine have developed a way to grow and purify the earliest lung progenitors from human stem cells, creating tiny 'bronchospheres' that model cystic fibrosis. The breakthrough could lead to new personalized medicine approaches for treating lung disease.
Researchers found that a harmful bacterium in the lungs of cystic fibrosis patients produces substances that enhance the growth of other bacteria, known as streptococci, which can inhibit the harmful bacterium's biofilm. Streptococci adhesins play a crucial role in this process.
A Stanford-led study has developed a wearable sweat sensor that can diagnose cystic fibrosis and monitor diabetes, offering a non-invasive and real-time solution. The device collects sweat, measures its molecular constituents, and transmits the results electronically for analysis and diagnostics.
Researchers developed an imaging technique using hyperpolarized helium MRI to measure the effectiveness of ivacaftor for cystic fibrosis patients. The study found a dramatic increase in lung improvement with long-term treatment.
Researchers from George Washington University have discovered a potential new drug to treat and stop the progression of cystic fibrosis. Thymosin α1 (Tα1) corrects genetic and tissue defects, reduces inflammation, and increases CFTR maturation, stability, and activity.
Researchers at Boston University School of Medicine have developed a novel approach to generate airway cells from stem cells using the Wnt signaling pathway. These cells can be grown into three-dimensional spheres and used to study lung diseases such as cystic fibrosis.
Researchers found that mucin proteins fail to unfold normally in CF airways due to dehydration, leading to thick and sticky mucus. This understanding could lead to therapies targeting the abnormal compact form of mucin proteins, such as rehydrating the airway lining with hypertonic saline.
Researchers have determined the three-dimensional structure of the human protein responsible for cystic fibrosis. The study reveals similarities with its zebrafish counterpart, allowing for a deeper understanding of how the protein functions normally and leads to the disease.
The study found extensive variation among Burkholderia cenocepacia isolates from cystic fibrosis patients, with changes in clinically relevant bacterial phenotypes. Genetic determinants of motility and biofilm formation may be promising targets for anti-virulence drugs.
A study published in Annals of Internal Medicine found that Canadians with cystic fibrosis have a significant survival advantage over American patients, with a median age of survival of 50.9 years compared to 40.6 years in the US. The difference is attributed to better healthcare coverage and nutrition in Canada.
A recent study published in the Annals of Internal Medicine reveals that people with cystic fibrosis are living longer in Canada than in the United States, with a median age of survival being 50.9 years in Canada compared to 40.6 years in the US. The study found that after accounting for factors such as age and disease severity, the ri...
A new study measures cumulative dosage over a patient's lifetime and finds that high exposure increases the risk of permanent hearing loss in cystic fibrosis patients. Researchers suggest alternative treatment strategies to mitigate this risk.
Researchers at UNC School of Medicine have isolated a protein called SPLUNC1 that plays a vital role in regulating proper airway function. When missing or depleted, it can cause airway constriction and difficulty breathing in people with asthma.
The updated guidelines provide better direction for clinicians to make accurate diagnoses and recommend personalized treatment. The new criteria lower the threshold for possible cystic fibrosis, reconsidering patients with chloride levels between 30-40 millimoles per liter.
Scientists have linked a new class of bacterial enzymes to the chronic lung inflammation hallmark of cystic fibrosis. The discovery provides two potential strategies for interrupting or correcting this interference by opportunistic bacteria, such as Pseudomonas aeruginosa.
Researchers created the first three-dimensional map of the cystic fibrosis protein, revealing a vulnerable spot in the protein responsible for many cases of the disease. The map shows that one half of the channel bears more disease-causing mutations, including one responsible for 70% of disease cases.
Researchers analyzed over 1,000 M. abscessus isolates from 500 global CF center patients, finding near-identical clones in different geographies and suggesting widespread transmission within the CF community. The study also highlights airborne transmission via contaminated surfaces as a potential mode of infection.
A new study reveals that a global, transmissible, and increasingly virulent M. abscessus infection is spreading rapidly among cystic fibrosis patients, requiring combination treatment with multiple antibiotics for extended periods.
A new pilot study using Bluetooth technology has shown remarkable improvements in cystic fibrosis adherence, with 80% of patients adhering to their treatment. The study also found significant reductions in measures of airflow limitation and disease flare-ups.
A new, inexpensive method for detecting chloride concentrations in sweat has been developed by Penn State biomaterials scientists. The fluorescent sensor is highly sensitive and selective for chloride, the key diagnostic marker in cystic fibrosis.
Scientists have discovered how ataluren works to treat genetic disorders by allowing the cell's machinery to 'run' over premature stop signs. The drug inserts amino acids similar to those in non-mutated genes, enabling proteins to function normally.
Researchers developed efficient lentiviral and adeno-associated virus-based approaches to deliver CFTR genes to the airways of pig models with cystic fibrosis. These methods resulted in functional CFTR expression, improving early cystic fibrosis pig phenotypes.
A DNA-modulating drug, BET inhibitor, attenuates CF lung inflammation by suppressing Th17 cell response and inflammatory factor release. In a mouse model, BET inhibitor treatment decreases lung inflammation without promoting infection, suggesting potential therapeutic use for CF patients.
Researchers developed mini-gut organoids from cystic fibrosis patient cells to screen drugs and personalize treatment. The mini-guts responded to CFTR-targeting therapies in vitro, correlating with clinical trial data and paving the way for tailored treatments.
Researchers developed synthetic transporters that can carry chloride through lipid-bilayer membranes, potentially replacing faulty channels in cystic fibrosis. The new molecules show high selectivity for chloride over protons and hydroxide, addressing a potential toxic effect.
Researchers have found that tromethamine, a drug approved to treat metabolic acidosis, can raise the pH of the airway surface liquid (ASL) and enhance bacterial killing activity in cystic fibrosis patients. This suggests that tromethamine may be beneficial in treating CF airway disease.
Researchers have developed a novel therapeutic strategy for treating cystic fibrosis, restoring lung cell function to 50% of healthy levels. By using a yeast genetic model, they identified key targets that can rescue the misfolding of the deltaF508-CFTR protein, which affects nearly 90% of patients with cystic fibrosis.
Scientists at Queen's University Belfast have discovered a new molecule that can potentially prolong the life of individuals with cystic fibrosis by improving airway hydration and mucous clearance. The treatment targets the epithelial sodium channel (ENaC), preventing chronic infections and inflammation that cause progressive lung damage.
A potential drug therapy developed at the University of Pittsburgh has proven effective against bacterial biofilms and a deadly respiratory virus simultaneously. The eCAP was 50 times more effective at fighting the biofilm than traditional treatment, and reduced viable virus particles by over 150-fold in RSV-infected airway cells.
A new study from McGill University Health Centre finds that newborn screening for cystic fibrosis results in better nutrition, fewer hospitalizations, and lower rates of infection. Children diagnosed through newborn screening are also diagnosed at an earlier age and benefit more from new treatments.
Researchers have discovered that ivacaftor restores CFTR function, improves airflow, and increases the capacity and flexibility of small airways in cystic fibrosis patients. The study suggests that loss of CFTR in airway smooth muscle cells is responsible for some CF-associated symptoms.
Researchers have developed a low-cost prototype using Xbox Kinect sensors to assess respiratory function, providing more accurate measurements than traditional spirometry methods. The system enables physicians to measure and assess how a chest wall moves, which could help identify numerous respiratory problems.
A new strategic research centre will aid health professionals design bespoke physical activity and exercise plans for young people with cystic fibrosis. The centre aims to develop guidelines and recommendations for tailored activity and exercise regimes, addressing the current lack of definitive guidelines.
Recent research on cystic fibrosis lung infections highlights the ability of Pseudomonas aeruginosa to adapt and evolve in chronically infected patients. The review suggests that understanding this evolution is crucial for developing alternative treatment strategies to minimize symptoms and improve patient outcomes.
Scientists at Eindhoven University of Technology and McGill University have successfully transported the defective CFTR protein to cell walls, a crucial step towards developing a cystic fibrosis drug. The researchers discovered a naturally occurring substance called fusicoccin-A that enables this transport process.
Scientists discovered that a proton pump called ATP12A causes problems in CF lungs by increasing airway acidity. This discovery may lead to new therapies targeting this protein to halt lung disease progression. The finding builds upon earlier work using CF pigs, which showed an abnormally acidic airway liquid.
Cystic fibrosis drug ivacaftor has been shown to improve disease markers and pancreatic function in young children with a specific mutation. The study's findings suggest a potential window of opportunity for early intervention and mitigation of organ damage.
Researchers have identified alveolar macrophages as key contributors to cystic fibrosis airway inflammation, with the overabundance of XBP-1 protein mediating their inflammatory effect. This discovery bolsters the case for targeting this pathway in therapy.
A leading researcher at the Case Western Reserve University School of Nursing is developing new treatments for cystic fibrosis, a life-threatening genetic disease. The scientist will lead two studies to explore gene-modifying treatments that can minimize CF symptoms, including medications and genetic correction.
Researchers at TSRI identified a mutant protein's incorrect interactions with other cellular neighbors, which disrupted its normal function. By removing these interactions, they partially restored the protein's normal function, suggesting new therapeutic targets to treat cystic fibrosis.
Researchers at U of T discovered genetic mutations in Candida albicans that allow it to defend itself against neighboring bacteria. The fungus has evolved to ignore bacterial signals, potentially becoming a more significant threat to patients with weakened immune systems.
Researchers have made encouraging results using gene therapy to treat cystic fibrosis by inserting a healthy copy of the CFTR gene into affected cells. The approach restored chloride and fluid transport in both mouse models and human-derived cell cultures, suggesting a potential cure for the genetic disorder.