Researchers discovered that the modified aspartate aminotransferase-to-platelet ratio index score (mAPRI) is a highly specific and noninvasive tool for the diagnosis of cystic fibrosis-related liver disease (CFRLD), as published in Clinical Transplantation. Despite the fact that liver disease is a significant cause of morbidity and mortality in patients with cystic fibrosis (CF), there is still no…
A new lipid nanoparticle delivery platform is able to deliver functional CFTR mRNA as an aerosol to the intended cells in the airway epithelium, according to new preclinical data presented at the American Thoracic Society 2022 International Conference that took place in San Francisco, California between May 13, 2022, and May 18, 2022. The delivery…
Positive expiratory pressure (PEP) is more effective than usual care or no intervention in patients with cystic fibrosis who are aged 16 or more years, according to a new study published in the journal Therapeutic Advances in Respiratory Disease. It is safe and has no adverse effects, researchers reported. However, there is not enough evidence…
Patients with cystic fibrosis (CF) show elevated levels of oxidative stress in their serum, according to a study published in Antioxidants. These levels tended to increase with patient age and the severity of the disease. Elevated levels of both protein and lipid oxidation products were detected in patients with CF, compared to healthy controls, during…
Individualized high-quality carbohydrate diets, supplemented by pancreatic enzyme replacement therapy and enteric tube feeding if needed, can significantly improve the nutritional status of patients with cystic fibrosis (CF), according to findings published in Nutrition. The researchers conducted a systematic review of the literature from June 1, 2016, to March 14, 2021, to determine the most…
Double lung transplantation is a feasible treatment approach for end-stage lung disease due to cystic fibrosis (CF), according to a new study published in Transplantation Proceedings. Although this approach is technically challenging, the researchers found significantly better survival among transplant recipients with CF than among those with other underlying conditions. “The main goal is to…
Lung function deteriorates after 2 years in children with cystic fibrosis (CF), according to a new study published in the Journal of Cystic Fibrosis. The study also showed that the isolation of Pseudomonas aeruginosa before 6 months of age and minor abnormalities in infant lung function tests are associated with a higher preschool Lung Clearance…
Researchers presented a case of an infant born with both alpha-1 antitrypsin deficiency (AATD) and cystic fibrosis (CF) and published their findings in BMC Pediatrics. The study is thought to be the only known case of a child born with both diseases. “We report on a boy with early CF diagnosis presenting with meconium ilius…
Positive expiratory pressure (PEP) devices more effectively clear mucus from the lungs of patients with cystic fibrosis (CF) compared to physical exercise, other respiratory techniques, or no intervention, according to results of a new study published in Therapeutic Advances in Respiratory Diseases. “PEP devices generate a pressure contrary to that produced by the airways during…
Researchers reported differences in the microbiology and metabolites of the sputum from patients with cystic fibrosis (CF) and nontuberculous mycobacterial (NTM) lung infections vs those with only CF, as published in mSphere. They suggested that these changes may be potential risk factors for developing such infections. The experimental study conducted by Breen and colleagues analyzed…
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