Articles tagged with Cystic Fibrosis
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Researchers at the University of Arizona are working on a novel, non-invasive lung test to measure airway fluid composition in cystic fibrosis patients. The study aims to compare the accuracy of this test with the invasive bronchoalveolar lavage procedure.
Researchers at UNC Chapel Hill have discovered a genetic risk factor for severe liver disease in people with cystic fibrosis, specifically the SERPINA1 gene variant. Those carrying this variant are five times more likely to develop cirrhosis and other liver complications than those with the normal version of the gene.
Researchers created airway spheres using animal and human cells, providing a new model to study dynamic processes in lung diseases. The 3-D spheres lined with ciliary and secretory cells can be used to investigate mechanisms underlying cancer and chronic asthma.
Researchers at UNC School of Medicine have found an efficient way to deliver a corrected gene to lung cells, restoring normal function in tissue models. This breakthrough could lead to improved treatment options for cystic fibrosis patients.
Researchers at University of North Carolina have discovered a new approach to delivering a corrected CF gene to lung cells using parainfluenza virus, which could potentially cure cystic fibrosis lung disease. The method has shown promise in laboratory models and may pave the way for future clinical trials.
Research suggests that breaking down lung mucus with cystic fibrosis medicines can reduce the severity of bacterial infections. The study's findings have important implications for managing long-term infections in patients with cystic fibrosis.
A new embryo test can detect both genetic and chromosomal abnormalities, offering a faster and cheaper alternative to current fertility treatments. The test, known as genome-wide karyomapping, can identify diseases caused by specific gene mutations and aneuploidy at the same time.
A study of 15 women with cystic fibrosis found that assisted reproductive technology (ART) was a safe and successful option for them to become pregnant. The researchers achieved 17 pregnancies in 13 of the 15 women, resulting in 12 live births.
Researchers at Duke University Medical Center followed genetically labeled cells in mouse lungs for over a year to understand natural renewal and healing processes. They found that lung stem cells play a crucial role in maintaining lung function and identified the secretory cell's ability to give rise to ciliated cells.
A new therapy that increases airway hydration may prevent mucous buildup in cystic fibrosis patients. GS-9411, an aerosol-based agent, prevents sodium absorption and allows the surface to remain moist.
Genetic screening for cystic fibrosis carrier mutations is universally recommended for the reproductive-age population. New reference materials have been developed to ensure accuracy in genetic testing, but some mutations may lead to false results due to large deletions or interference with laboratory methods.
Current treatments for cystic fibrosis, including hypertonic saline, macrolide antibiotics, and ibuprofen, are crucial for improving life expectancy. Patients with CF can benefit from airway clearance techniques, nutrition supplements, and medications to prevent bacterial infections.
Combining a mainstay antibiotic with drugs to deprive bacteria of iron boosts infection killing, according to Dartmouth researchers. Their study found that using two FDA-approved iron chelators and tobramycin eradicated biofilms by 90 percent.
A study found that consuming fructose-sweetened beverages increases visceral adiposity and lipids while decreasing insulin sensitivity in overweight/obese humans. This increase in heart attack susceptibility remains unknown due to long-term effects of fructose over-consumption.
Researchers discovered that neutrophils respond to conflicting signals from cystic fibrosis patients' lungs, leading to severe lung damage. The study's findings suggest that targeting the molecular pathway responsible for this response could lead to new disease therapies.
Researchers found a correlation between small genetic differences in the IFRD1 gene and lung disease severity in cystic fibrosis patients. The study also discovered that the IFRD1 protein regulates neutrophil function, which causes inflammatory damage to airways.
Researchers identified IFRD1 as a gene that modifies cystic fibrosis lung disease severity by regulating neutrophil function. Deleting IFRD1 in mice reduced inflammation and disease, suggesting potential new targets for treatment.
Researchers have successfully prevented cystic fibrosis lung disease in a mouse model by administering amiloride, a drug that inhibits hyperactive sodium channels. Early inhalation treatment prevents chronic lung damage and airway inflammation, providing a new therapeutic approach for the widespread genetic disease.
Researchers have developed a nanoemulsion that effectively kills a wide range of bacteria resistant to antibiotics, including those causing cystic fibrosis. The treatment uses ultra-fine oil-and-water emulsions that disrupt bacterial outer membranes, making resistance unlikely and offering a potential alternative to antibiotics.
Researchers found that firefly luciferase tests can produce false positive results, which may impact the screening process. The study's findings suggest more work is needed to ensure the accuracy of the screening process.
Lung airway epithelial cells initiate an immune response to inhaled bacteria by signaling white blood cells to move into the lungs. Researchers have identified a way to inhibit proteases and restrict junctions between cells, reducing inflammation and allowing optimal white blood cell response.
Adult cystic fibrosis patients can accurately predict their mortality based on their own reported quality of life. Patient-reported pain was found to be a critical factor in determining survival rates, highlighting the importance of using patient-reported measures in clinical trials.
Researchers have identified genetic clues to Pseudomonas aeruginosa's success that will aid in the design of novel therapeutic strategies for cystic fibrosis patients. The study found that prophage genes contribute to the strain's ability to adapt to specific environments.
A study found that nearly half of children with cystic fibrosis were vitamin D deficient, despite receiving recommended doses. Increasing vitamin D intake is crucial to address deficiencies and prevent bone loss in this population.
Researchers at the University of Missouri have successfully produced pigs born with cystic fibrosis, mirroring the exact symptoms of humans with the disease. The pigs, carrying only one copy of the mutated gene, are being studied to potentially develop treatments for the fatal lung disease.
Researchers found that targeting Streptococcus milleri group (SMG) bacteria can disrupt Pseudomonas aeruginosa bacterial communities, leading to clinical benefits for patients. The approach has shown positive results in treating severe lung infections and may also be a treatment option for individuals with chronic lung infections.
Scientists have identified a direct role for the missing protein CFTR in cystic fibrosis, allowing it to recognize and clear lung-damaging bacteria. This breakthrough could lead to new treatments and prolong the lives of patients, who currently face a high risk of death before their 35th birthday due to chronic lung infections.
A new drug therapy called VX-770 has shown promising results in improving the quality of life for cystic fibrosis patients. Early trials have demonstrated a 50% decrease in sweat salt concentration and a 10% increase in lung function, offering new hope for this devastating disease.
Scientists discovered that biofilm bacteria produce harmful chemicals, including a protein similar to rattlesnake venom, which can cause disease progression and resistance to antibiotics. This research has significant implications for the treatment of hospital superbugs, cystic fibrosis, and cancer.
Researchers discovered a way to restore function of CF airway cells by manipulating cellular quality control machinery. This technique could significantly reduce sticky mucus that clogs lungs and leads to life-threatening infections.
Researchers developed PTC124 to bypass premature 'stop' signals in CFTR genes, allowing the protein-making machinery to function normally. The phase II trial showed improvements in chloride ion transport and lung respiratory function, offering a potential treatment for cystic fibrosis.
A Phase 2 trial of PTC124 demonstrated statistically significant improvements in CFTR-mediated chloride transport and lung function in patients with nonsense-mutation CF. The study showed that PTC124 induced chloride transport responses across various patient genotypes, offering a potential new treatment paradigm for this genetic disease.
Researchers have discovered a gene that helps clear mucous in the lungs and digestive system of people with cystic fibrosis. By correcting abnormal levels of this gene, they were able to prevent blockages and improve survival rates in mice with CF.
A study finds that insufficient hydration of the airway surfaces leads to pathologies typical of chronic obstructive lung diseases in humans. This could lead to a new therapeutic strategy for treating asthma and COPD by improving mucus clearance through sodium channel blockers.
Researchers at University of Toronto have identified compounds that block the activity of a key protein (ExoS) in cystic fibrosis patients. These compounds may also serve as a model for future therapies against the HIV virus, according to a new study published in PLoS Genetics.
Scientists have created a genetically modified pig model that carries the mutated gene responsible for cystic fibrosis, mimicking lung disease in humans. This breakthrough could lead to better understanding of the disease and development of new treatments.
Computer simulations revealed that a defective gene causes cystic fibrosis by misfolding the CFTR protein. The study identifies critical amino acid pairs essential for proper protein folding, providing guidance for designing small molecule drugs.
A study found that genetic variations modifying MBL2 expression are associated with more severe CF symptoms, including faster lung decline in patients deficient in the protein. This may lead to new approaches for treating those at risk of increased disease severity.
Researchers have discovered a peptide in scorpion venom that controls chloride channel movement, potentially treating cystic fibrosis. The novel GaTx1 peptide could inhibit CFTR channels, increasing water production and thinning mucus in airway cells.
A recent MUHC study reveals that cystic fibrosis-related bone problems are an additional pathology stemming from the Cftr gene mutation, not a side effect of treatment. The study found that mice with the mutation have lower bone mineral density and mass than control mice, without pancreatic insufficiency or steroid treatment.
Researchers at the University of Alabama at Birmingham found that PTC124 restored normal function in up to 29 percent of abnormal cystic-fibrosis protein cases. The drug works by rescuing faulty proteins that lead to illnesses, including cystic fibrosis and over 2,400 genetic diseases.
A study found that secondhand smoke exposure is associated with decreased lung function in individuals with cystic fibrosis. Certain mutations in the CFTR gene determine the magnitude of this effect, suggesting a potential role for gene-environment interactions in worsening lung outcomes.
Researchers at Johns Hopkins Medicine discovered that secondhand smoke exposure can weaken lung function in patients with cystic fibrosis. The study found that even a small genetic change can double the negative effects of secondhand smoke on lung function, accelerating reduced lung function and lifespan.
Researchers at UAB have provided physical proof that two proteins involved in Cystic Fibrosis (CF) interact closely enough to cause a biochemical imbalance. This discovery may aid in understanding the biological underpinnings of CF and speeding up the development of new drugs to treat or cure the disease.
A CU-Boulder technology has been used to identify unexpected bacteria strains in the lung fluid of Denver children suffering from cystic fibrosis. The method uses nucleic acid gene sequencing to rapidly detect and classify pathogens found in the lungs, identifying over 60 species of bacteria in samples from 28 patients.
A new study found that treatment with ibuprofen is associated with a significantly slower rate of decline in lung function in children and adolescents with cystic fibrosis. The study showed a 29 percent reduction in loss of lung function compared to those who did not use the anti-inflammatory drug.
A study found that a specific polymorphism in the glucocorticoid receptor gene is associated with lung disease progression in cystic fibrosis. Patients with the BclI GG genotype showed more pronounced deterioration in lung function, suggesting they may benefit from inhaled steroids.
Cystic fibrosis patients may benefit from a new therapeutic strategy that targets deadly bacterial infections by manipulating the binding of antimicrobials in mucus. By reducing the charge on lysozyme, researchers found it could float independently in the mucus and kill bacteria effectively.
Researchers have identified potential targets for ALS treatment by studying SOD1 gene mutations. Additionally, a new study on Candida parapsilosis has revealed that lipase is an essential virulence factor. Further research suggests that the timing of treatment with immune-modifying drugs can significantly impact viral infection outcomes.
Lucas R. Hoffman receives the ICAAC Young Investigator Award from the American Society for Microbiology for his groundbreaking research on biofilm formation and antibiotic interactions. His work has led to significant advancements in the care of patients with cystic fibrosis.
A Canadian study found that high-dose ibuprofen can slow the decline of lung function in children with mild cystic fibrosis. The treatment reduced hospital stays and improved quality of life for patients.
Researchers have discovered a weakness in the armour that protects B. cenocepacia from antibiotics, identifying a key sugar required for its protection. This finding may lead to novel molecules to disrupt its synthesis and develop new treatments.
A newly discovered bacterial enzyme may play a critical role in the pathogenesis of lung injury in cystic fibrosis patients. The study suggests that targeting this enzyme could be a viable near-term approach to improving the length and quality of life for many CF patients.
A study by UNC researchers found that normal breathing's rhythmic motion regulates mucus clearance, promoting lung health in CF patients. This rhythm also re-hydrates airways, accelerating mucus removal.
A new study in The Lancet suggests that universal newborn screening for cystic fibrosis is associated with lower treatment costs and reduced hospital admissions. This could lead to cost savings that offset the costs of the screening program, making it a more viable option globally.
A small percentage of CF patients with a rare genetic stop mutation responded positively to gentamicin treatment, suggesting potential targeted treatments for the disease. Gentamicin reversed stop codons and restored the CFTR protein, improving respiration in affected patients.
Primary ciliary dyskinesia is a rare genetic disease that causes chronic airway disease and affects about 1 in 15,000 children. Newborns with respiratory distress are at risk of having this condition, which can lead to persistent wheezing, cough, and recurrent infections.
Researchers discovered a new immune defense mechanism that produces a highly effective antibacterial compound called hypothiocyanite. However, this process is defective in airway tissue and cells containing the CF gene mutation, suggesting that thiocyanate levels may be low in CF patients.
Researchers at Children's Hospital of Pittsburgh have identified interleukin-23 (IL-23) as the key molecule causing destructive lung inflammation in cystic fibrosis patients. This discovery provides a specific target for developing new therapies to improve quality of life for these patients.
Researchers have identified unique expression domains for two proteins, SPLUNC1 and SPLUNC2, in the lungs of people with cystic fibrosis. The discovery could help understand the immune system's role in the disease and potentially prevent lung damage.