Cystic fibrosis (CF) is due to a mutation in the cystic fibrosis transmembrane conductance regulator gene (CFTR), which leads to unusual water and chloride secretion across epithelial surfaces. The lungs are responsible for most morbidity, though other organs are frequently affected. Sleep abnormalities have long been recognized in CF. Abnormal ventilation and oxygenation, sinus disease, deconditioning due to muscle weakness and recurrent infections, and inflammation have been thought to play a role in sleep disorders in CF. However, there is evidence that CFTR gene dysregulation can affect circadian rhythms in CF. Early recognition and treatment of circadian rhythms may improve outcomes in CF.
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http://dx.doi.org/10.1007/s41030-022-00184-x | DOI Listing |
Tuberk Toraks
December 2024
Division of Pediatric Pulmonology, Department of Pediatrics, Ankara University Faculty of Medicine, Ankara, Türkiye.
Allergic bronchopulmonary aspergillosis (ABPA) is a complex hypersensitivity reaction observed in asthma as well as cystic fibrosis (CF) patients due to the colonization of the airways by Aspergillus fumigatus. While ABPA is most commonly observed in CF patients (2-9%), it is seen at a rate of 1-2% in patients diagnosed with asthma. ABPA is mostly seen in steroid dependent adult asthma patients and has rarely been reported in pediatric asthma patients.
View Article and Find Full Text PDFDrug Dev Ind Pharm
January 2025
Charles Institute of Dermatology, School of Medicine, University College Dublin, Dublin 4, Ireland.
Objective: Highly branched poly(β-amino ester) (HPAEs)-based gene therapy holds promise for treating lung cystic fibrosis (CF). However, the translation of HPAEs/DNA nanoparticles into clinical applications poses a significant challenge due to the requirement for high concentrations of the formulation.
Methods: In this work, a straightforward and scalable concentration method was developed for concentrating HPAEs/DNA polyplexes.
J Patient Exp
December 2024
University of Alabama at Birmingham, Birmingham, AL, USA.
People with cystic fibrosis (PwCF), families, and clinicians, partner to co-produce care, navigate access barriers, address mental health and social factors, follow specific infection prevention and control practices, and share decision-making regarding treatments and daily care. Standard patient satisfaction and experience of care surveys are not tailored to return relevant, actionable data for specific populations. To improve the care experience, the U.
View Article and Find Full Text PDFInfect Drug Resist
December 2024
Department of Medical Laboratory, The Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai, Shandong, People's Republic of China.
Purpose: is a conditioned pathogen in the medical setting and mainly affects patients with cystic fibrosis. We found co-infection with complex (Bcc) in many patients with respiratory tract infections, including H7N9 and COVID-19. However, previous studies have not focused on co-infections with BCC and respiratory viruses.
View Article and Find Full Text PDFBMJ Sex Reprod Health
December 2024
Department of Obstetrics and Gynecology, Oregon Health & Science University, Portland, Oregon, USA.
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