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Cystic fibrosis (CF) is one of the most challenging pediatric illnesses for families to manage. There is, however, limited research that considers the associations between family functioning and treatment adherence in children and adolescents with CF. Nineteen children with CF (mean age=12.42 years, mean forced expiratory volume in one second (FEV)=90.9% predicted) and their families participated in the study. Caregiver and child participants completed interview-based assessments and were then videotaped during a family mealtime. Mean scores on several domains of family functioning fell in the "unhealthy" range. Better family functioning was found among older children. Better family functioning was also associated with better adherence to antibiotic treatment and worse adherence to enzymes. Findings suggest that family functioning may be an important correlate of treatment adherence in children and adolescents with CF. Future research should replicate these findings in larger samples of children and adolescents with CF.
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http://dx.doi.org/10.1089/ped.2014.0327 | DOI Listing |
Acta Neuropathol Commun
September 2025
Department of Biomedical and Clinical Sciences and Department of Clinical Pathology, Linköping University, 58185, Linköping, Sweden.
Disruptions in synaptic transmission and plasticity are early hallmarks of Alzheimer's disease (AD). Endosomal trafficking, mediated by the retromer complex, is essential for intracellular protein sorting, including the regulation of amyloid precursor protein (APP) processing. The VPS35 subunit, a key cargo-recognition component of the retromer, has been implicated in neurodegenerative diseases, with mutations such as L625P linked to early-onset AD.
View Article and Find Full Text PDFBMC Nurs
September 2025
Department of Nursing Administration, Faculty of Nursing, Alexandria University, Alexandria, Egypt.
Background: Organizational virtuousness and just culture, which both foster justice, honesty, and trust, have a major impact on positive work environments in the healthcare industry. Strengthening nurses' emotional engagement and vocational commitment requires these components. With an emphasis on the mediating function of just culture, this study attempts to investigate the relationship between organizational virtuousness and nurses' vocational commitment.
View Article and Find Full Text PDFNature
September 2025
Liangzhu Laboratory, Zhejiang University, Hangzhou, China.
Monogenic lupus offers valuable insights into the underlying mechanisms and therapeutic approaches for systemic lupus erythematosus (SLE). Here we report on five patients with SLE carrying recessive mutations in phospholipase D family member 4 (PLD4). Deleterious variants in PLD4 resulted in impaired single-stranded nucleic acid exonuclease activity in in vitro and ex vivo assays.
View Article and Find Full Text PDFNat Commun
September 2025
Department of Biochemistry, University of Illinois, Urbana-Champaign, IL, USA.
Individuals with progressive liver failure risk dying without liver transplantation. However, our understanding of why regenerative responses are disrupted in failing livers is limited. Here, we perform multiomic profiling of healthy and diseased human livers using bulk and single-nucleus RNA- and ATAC-seq.
View Article and Find Full Text PDFSignal Transduct Target Ther
September 2025
Institute of Neuroscience, Center for Excellence in Brain Science and Intelligence Technology, Chinese Academy of Sciences, Shanghai, China.
Essential tremor (ET) is a common neurological disease that is characterized by 4-12 Hz kinetic tremors of the upper limbs and high genetic heterogeneity. Although numerous candidate genes and loci have been reported, the etiology of ET remains unclear. A novel ET-related gene was initially identified in a five-generation family via whole-exome sequencing, and other variants were identified in 772 familial ET probands and 640 sporadic individuals via whole-genome sequencing.
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