Articles tagged with Cystic Fibrosis
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Children's Hospital of Pittsburgh researchers aim to develop more effective treatments for patients with chronic lung diseases, including cystic fibrosis and idiopathic pulmonary fibrosis. The $12.8 million NIH grant will support the development of new diagnostic methods and therapies.
Researchers at UNC Health Care identified the cellular system that degrades faulty CFTR protein in cystic fibrosis, allowing some proteins to regain their proper shape. This understanding provides insight into potential therapeutics aimed at curing the disease.
Researchers found that cystic fibrosis patients develop unique diabetes due to differences in insulin-producing cell function, rather than pancreas destruction. This discovery may help improve understanding of other forms of diabetes and work towards a cure.
Researchers found that stopping inhaled corticosteroids in cystic fibrosis patients had no significant increase in lung-related adverse effects. The study showed that replacing the medication with a placebo was safe for most patients, suggesting that it may be possible to lower drug burden and reduce side effects.
A $4.5 million partnership aims to improve lung health by studying immune responses to infections and allergens in Canadians affected by asthma, cystic fibrosis, and other respiratory diseases. Experts available for comment on the initiative and its implications.
Researchers found that cystic fibrosis airway glands have defective gland secretion due to a lack of fluid secretion from glands. The study suggests that loss of CFTR-mediated fluid secretion is the main cause of mucus buildup in cystic fibrosis.
A study published in the Journal of Clinical Investigation found a connection between cataract development and cholesterol levels. Researchers discovered that rats with specific gene mutations had lower cholesterol levels in their eyes, suggesting an abnormality in epithelial cell maturation contributing to lens opacity. This link has ...
Researchers discovered that a genetic mutation in Pseudomonas aeruginosa makes it susceptible to destruction by slightly acidified sodium nitrite, a common food preservative. This finding offers new hope for treating cystic fibrosis airway disease.
A saltwater aerosol solution has been found to restore the thin lubricant layer on airway surfaces, promoting mucus clearance and reducing lung damage in cystic fibrosis patients. The therapy is inexpensive and shows promise for slowing disease progression and improving patient outcomes.
Researchers found that hypertonic saline treatment increases mucus volume, helping to clear bacteria and debris from airways, potentially limiting lung infections. The study's results could lead to improved outcomes for CF patients, especially if benefits are seen in infants and young children.
A study of 13,041 cystic fibrosis patients found that those enrolled in clinical trials showed a slower decline in lung function compared to non-participants. The authors suggest that access to better healthcare through more office visits may be the key reason for this difference.
Researchers developed a breath test that measures sulfur compounds in exhaled breath to detect bacterial growth in cystic fibrosis patients. The test found significantly higher levels of sulfides in cystic fibrosis patients, suggesting an increased amount of bacterial growth in the lungs.
Researchers found that specific versions of the TGFb1 gene are linked to worse lung disease in cystic fibrosis patients. The study's large size and robust design enabled scientists to identify a potential target for improving CF therapy.
A Canadian paper comments on a decline in cystic fibrosis birth rates since the onset of genetic testing. The study found that newborns with cystic fibrosis had lower birth weights and were at higher risk of prematurity compared to non-affected children.
K-State researchers are working on a new drug to treat cystic fibrosis by designing chloride-selective pores that can help ions travel across cell membranes. Their goal is to create a medication that works efficiently and effectively at low doses, improving the lives of those affected by the disease.
Researchers have discovered a highly specific pathway that causes inflammation in lung tissue, which may contribute to lung damage in adult patients with cystic fibrosis. The study identifies interleukin-23 and interleukin-17 as promising targets for neutralization.
Scientists have successfully delivered genes to the lungs of CF mice using DNA nanoparticles, enabling real-time imaging and assessment of gene expression. This breakthrough technology holds promise for treating serious lung diseases like cystic fibrosis with novel nucleic acid-based therapies.
A new study found that a once-daily treatment of the antibiotic tobramycin is effective in treating cystic fibrosis patients, particularly in reducing kidney-related side effects. The trial recruited 219 patients and showed similar results in both children and adults.
Researchers find that low-dose hydrocortisone infusion speeds up pneumonia resolution and prevents sepsis-related complications. Sildenafil protects against altitude-induced pulmonary problems, improves gas exchange, and limits hypoxemia. Patient segregation reduces Pseudomonas aeruginosa transmission in cystic fibrosis patients
Researchers will investigate the spread of 'superbugs' and develop more effective antimicrobial agents and vaccine strategies to treat lung infections. The goal is to determine when segregation of CF patients is necessary, reducing social strain on families and individuals.
Researchers discovered two compounds that block red tide toxin effects on respiratory systems, showing promise in treating cystic fibrosis. The compounds improved mucus clearance and may work at concentrations without side effects.
Early diagnosis of cystic fibrosis through newborn screening can lead to improved height and weight measurements, better pulmonary status, and cognitive benefits. Implementing CF newborn screening in all states aims to give people with this disease the best odds at living longer, healthier lives.
Research at the University of Pittsburgh School of Medicine found that COPD patients with the most advanced disease are more likely to harbor Pneumocystis jiroveci in their lungs. This colonization is strongly associated with increased airway obstruction and worse lung function, even after adjusting for smoking history.
A team of researchers has discovered that people with cystic fibrosis (CF) actually have very little mucus in their airways, contradicting long-held assumptions. The study suggests that the substance clogging CF patients' lungs is pus and that mucus may protect the airway from infection.
Researchers used a custom-built small animal imaging system to demonstrate gene expression in mice with cystic fibrosis. The results show promise for novel gene delivery methods and potential treatments for the debilitating disease.
A new study found that lung transplants significantly increase the survival rate of cystic fibrosis patients by almost 4.5 years. The study followed 247 patients with CF who were on the waiting list for lung transplantation and found that the risks associated with the surgery decreased over time.
Researchers at Yale University have discovered that curcumin, a compound found in turmeric, can correct the defective chloride channel responsible for cystic fibrosis. In tissue culture and mouse model systems, curcumin restored normal function to the nasal and rectal epithelia of CFTR mice.
A team of researchers has successfully corrected a genetic defect responsible for cystic fibrosis in mice using turmeric extract. Curcumin, a compound found in turmeric, was able to release the mutant CFTR protein from an inappropriate compartment inside the cell and allow it to function normally.
Patients with chronic obstructive pulmonary disease (COPD) are at increased risk of nonvertebral fractures from high-dose inhaled corticosteroid treatment. Researchers also found greater acetaminophen use linked to newly diagnosed asthma in women. Air pollution exposure worsens cystic fibrosis patients' pulmonary exacerbations.
A Cincinnati Children's Hospital study found a deficiency in lipoxins, key regulators of inflammation, in children with cystic fibrosis. This discovery provides impetus for developing novel therapeutics to decrease lung inflammation.
A new study published in the American Thoracic Society's Journal found that preterm infants exposed to cigarette smoke in utero experience increased obstructive apneas and decreased respiratory arousal index. Additionally, researchers discovered that rhDNase treatment significantly reduces airway inflammation in cystic fibrosis patient...
Researchers have discovered a link between cystic fibrosis and fatty acid defects, with abnormally high levels of arachidonic acid and low levels of docosahexaenoic acid found in patients. The study suggests that correcting this imbalance may lead to new treatment options for the disease.
Researchers at Johns Hopkins Medicine have identified a genetic pattern in the CFTR gene that can predict disease severity in individuals with the 5T mutation. The study found that combinations of thymine and guanine repeats in the CFTR gene affect disease status, with certain patterns being more common in people with lung disease.
Cystic fibrosis patients with inhaled antibiotics show improved lung function and reduced bacterial growth. Effective antibiotic therapy is essential for managing CF lung disease.
A new bead-array test can identify the 25 most common cystic fibrosis mutations in just 15 seconds, enabling potential parents to make informed decisions about conception and treatment options. The test requires only genomic DNA from a patient and uses a single reaction to screen for all 31 CF mutations.
Researchers found that glutamate regulates the movement of bicarbonate and chloride into epithelial cells, controlling mucus transport and water flow. This discovery contributes to understanding how cystic fibrosis is controlled by gland cell membranes and may lead to future therapies for severe forms of the disease.
Researchers from National Jewish Health will present data on the prevalence of obstructive sleep apnea in difficult-to-control asthma patients. Additionally, they will discuss risk factors for 'hot tub lung' caused by nontubercular mycobacteria and a genetic mutation linked to mycobacterial infections.
Scientists create hybrid virus that can attach to lung cells' top surface, allowing healthy genes to enter and correct genetic defects associated with cystic fibrosis. The new approach increases production of viral particles, a crucial step towards preclinical studies.
A recent Phase I study of compacted DNA gene therapy for cystic fibrosis demonstrated safety and tolerability. The treatment resulted in a meaningful increase in chloride ion transport in the nose, suggesting successful gene transfer and potential therapeutic benefits.
Researchers at the University of Washington used quantitative proteomic analysis to study Pseudomonas aeruginosa bacteria linked to cystic fibrosis. The study found that quorum sensing may help the bacteria adapt in the airway, leading to inflammation and fatal outcomes.
Airway epithelial cells use a repair mechanism that is triggered when the cell barrier is breached, allowing messenger molecules to communicate with receptors and initiate rapid repair. This study suggests that certain diseases, such as asthma and cystic fibrosis, may impair this mechanism, leading to abnormal cellular changes.
Researchers tested a combination of a vibrating vest and a mechanical cough-assist device to improve respiratory function in stroke patients. The treatment resulted in significant increases in forced vital capacity and minute ventilation, and improved oxygen saturation levels.
Researchers found that adult CF patients can achieve educational and work accomplishments despite the progressive nature of the disease. Pulmonary function and exercise capacity testing are limited in predicting their ability to work or attend school.
Researchers identified a new compound, thiazolidinone, that effectively inhibits CFTR-mediated chloride secretion, reducing excess intestinal fluid secretion in mice with secretory diarrhea. The study's findings suggest that this compound may be useful in treating secretory diarrhea linked to the cystic fibrosis gene.
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.