Articles tagged with Cystic Fibrosis
Researchers have developed a novel system of gene therapy that targets lung stem cells to treat cystic fibrosis. By using a natural compound found in the lungs, the therapy enables airway cells to take up the therapeutic gene and shows substantial recovery from the disease defect.
A study of 74 patients with non-classic cystic fibrosis revealed that nearly a third had no detectable changes in their CFTR genes, sparking debate about the role of epigenetics and alternative causes. The findings may lead to improved diagnosis and treatment options for these patients.
A recent UCSF-led study found that fluoroquinolones were widely prescribed to patients discharged from the hospital despite narrow-spectrum antibiotics being appropriate. The study highlights the serious erosion of effectiveness due to overuse and increasing bacterial resistance, particularly in cystic fibrosis patients.
A study found that azithromycin improved forced expiratory volume in one second's exhalation (FEV1) by around 5% in some children with cystic fibrosis. The drug was well-tolerated and showed no significant difference in quality-of-life assessments compared to placebo.
Two new studies found a relationship between high cat allergen concentrations in the home and increased asthma symptoms among sensitized women. Researchers also discovered common delays in TB diagnosis and treatment, as well as abnormal pulmonary function linked to bacterial infection in cystic fibrosis patients.
A novel gene therapy approach has been successfully tested on a 33-year-old cystic fibrosis patient, using a patented DNA compaction technology. The treatment aims to deliver healthy genes into cells to produce normal proteins needed by patients with the disease.
Researchers found Pseudomonas aeruginosa thrives in low-oxygen environments of the airways, adapting to remove oxygen and producing protective sugar coatings. This discovery suggests a new strategy to target specific enzymes critical for bacterial survival, offering hope for improved treatment options.
Researchers have identified aquaporin-1 protein's role in regulating water movement into and out of cells, a fundamental requirement for life. In a study comparing normal lungs to those without the protein, scientists found increased airway wall thickness and fluid leakage in those with aquaporin-1.
The UNC-Chapel Hill Gene Therapy Center will use the funds to translate basic research knowledge into safe human clinical trials, with a focus on cystic fibrosis and hemophilia. The center aims to provide new treatments for diseases caused by single-gene defects.
Researchers found that genetic mutations in people with chronic pancreas inflammation are associated with an increased risk of pancreatitis. The study identified specific mutations in the CF and PSTI genes, which can lead to a 900-fold increase in pancreatitis risk for those with two mutations.
Researchers investigate phenotypic variability in cystic fibrosis, revealing its impact on disease progression and treatment outcomes. The study highlights the importance of personalized medicine approaches to better manage CF patients.
Scientists at UNC Chapel Hill have discovered a highly ordered array of signaling molecules controlling the passage of chloride and other ions in healthy people. This breakthrough may lead to new treatments for cystic fibrosis by restoring CFTR function through genetic engineering or drug therapy.
Researchers found that inhaling tobramycin twice daily for 12 months effectively eradicated Pseudomonas aeruginosa from the airways of 14 cystic fibrosis patients, with nine remaining free from infection at two-year follow-up. The treatment regimen cures chronic lung infections in patients started soon after colonisation.
Adults with cystic fibrosis (CF) are susceptible to cross-infection, including superinfection, from Pseudomonas aeruginosa. Chronic colonization occurs in up to 80% of CF patients, leading to increased illness and death. Genotypic changes in strains suggest social contact is a common mode of transmission.
Researchers at University of North Carolina at Chapel Hill School of Medicine believe they have solved a key part of the cystic fibrosis puzzle by finding that there's too little salt in patients' lungs. Long-lasting treatments may be needed to reverse the disease and improve symptoms.
Dr. Boucher is a leading researcher on cystic fibrosis, publishing over 300 articles and developing novel treatments. The University of North Carolina's genomics initiative receives $245 million in funding to advance research and therapies.
A clearer picture has been obtained on why the protein doesn't function properly in the milder form of cystic fibrosis, attributed to segments sticking together and interfering with chloride flow. This discovery opens doors for new avenues of research in rational drug design.
Jayraman et al. used fluorescent indicators to measure ASL salinity in normal human and CFTR-/- mice, finding no significant difference between the two groups. This noninvasive approach may provide new insights into lung diseases with poorly understood ASL properties.
Researchers at Johns Hopkins have identified a previously unknown system that helps control the protein product made by the cystic fibrosis gene, called CFTR. The discovery could lead to improved drug testing and screening for cystic fibrosis therapies.
A team of researchers has completed the genome sequence of Pseudomonas aeruginosa, the largest bacterium sequenced so far. The findings may lead to innovative therapies for patients with cystic fibrosis and those who develop severe infections.
Researchers at UNC-CH have successfully repaired a genetic problem causing cystic fibrosis, offering new hope for patients. The technique involves correcting a mutation involving unnecessary information inside a gene, resulting in the production of normal messenger RNA.
Researchers have discovered a reversible lipid imbalance that may be responsible for the common symptoms of cystic fibrosis. Administration of DHA corrected the imbalance and reversed signs of cystic fibrosis in mice with the genetic defect.
A University of Iowa study reveals that high salt concentrations in the airways of people with cystic fibrosis prevent antimicrobial peptides from working effectively. This finding provides new ideas about potential treatments for the disease.
Researchers have discovered that movement of salt and liquid in and out of lung surface cells plays a central role in regulating the lungs' internal liquid coating. This finding confirms a theory of how lungs protect themselves and may suggest better ways of treating cystic fibrosis.
Scientists have extended their gene repair technology to correct point and frameshift genetic mutations, with potential applications for treating cystic fibrosis and other diseases caused by DNA deletions.
A study found that a significant number of infertile men have cystic fibrosis gene mutations undetected by routine screening. This raises concerns about the risk of passing on defective genes to future generations. The researchers suggest improving screening methods for men and testing women for gene mutations to mitigate this risk.
Cystic fibrosis damages young lungs due to chronic bacterial infections and a lack of liquid layer, which hinders the body's natural cleansing process. Researchers have discovered that the film of liquid coating airways is depleted in CF patients, preventing cilia from functioning properly.
Researchers at UNC-CH found that Pamidronate is highly effective in partially reversing bone loss caused by immuno-suppressant drugs after lung transplants. Patients treated with the drug experienced a 10% reversal of bone loss, and showed significant increases in femur and spine mineral densities.
Researchers found a strong association between the cystic fibrosis gene and chronic pancreatitis, revealing genetics plays a significant role in its development. The study's findings have significant implications for treating patients with this condition, including those who are not heavy drinkers.
A shellfish toxin study has discovered a mechanism that may help treat cystic fibrosis by stimulating fluid flow through protein phosphatase inhibition. This could lead to loosening of accumulated mucus in the lungs and gut, reducing bacterial infections and early death.
A bacterial pesticide, Burkholderia cepacia, has raised concerns over its potential to cause deadly lung infections in people with cystic fibrosis. Experts are calling for a ban on its use in pest control until it is proven safe.
A new study reveals that many laboratories offering genetic tests for cystic fibrosis don't test for enough mutations, which can lead to unreliable results. The recommended widespread screening is premature due to the lack of standardization in testing labs.
A study led by Johns Hopkins Researchers found that a drug, sodium 4-phenylbutyrate (4PBA), may help cystic fibrosis patients with the deltaF508 mutation by allowing more CFTR proteins to reach cell surfaces. This phenomenon occurs at concentrations normally seen in patients taking the drug for urea cycle disorders.
Researchers at UNC-CH School of Medicine found a genetic mutation, 5T allele, associated with milder forms of cystic fibrosis and other chronic lung and sinus infections. The study suggests that the 5T allele may be linked to infertility and urological abnormalities in infertile men.