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  1. 7 de may. de 2024 · To analyze the evolution of clinical and anthropometric characteristics of children and adolescents with cystic fibrosis (CF) over 24 months, including the period of the COVID-19 pandemic. Methods A longitudinal study with data collection from May 2018 to November 2020 in physical and electronic records from a pediatric reference center, including individuals with CF aged up to 18 years.

  2. 27 de abr. de 2024 · Cystic fibrosis. Pulmonary exacerbations. Diagnosis. Treatment. Antibiotics. 1. Introduction. Although clinical outcomes for people with CF (PwCF) have dramatically improved over the last several decades, pulmonary exacerbations (PEx) remain common.

  3. 10 de may. de 2024 · Maternal and fetal outcomes in multiparous women with Cystic Fibrosis. Highlights. •. Over a quarter of wwCF in the cohort are multiparous, i.e. have had ≥3 pregnancies. •. A greater decline in FEV1 is seen in multiparous wwCF with 2 severe mutations. •. Multigravid pregnancies show no more exacerbations or disease CF complications. •.

  4. 10 de may. de 2024 · Cystic fibrosis (CF) is caused by deleterious variants in each CFTR gene. We investigated the utility of whole-gene CFTR sequencing when fewer than two pathogenic or likely pathogenic (P/LP) variants were detected by conventional testing (sequencing of exons and flanking introns) of CFTR.

  5. Hace 1 día · Cystic fibrosis (CF) is a genetic disorder resulting in the secretion of highly viscous mucus in the airways, leading to lung infection, respiratory failure, morbidity and mortality. CF is attributed to mutation in the CF Transmembrane Conductance Regulator (CFTR) gene that codes for a chloride transporting channel at the cell plasma membrane. More than 2,000 mutations in the CF gene have been ...

  6. Hace 6 días · Jennifer Kelleher, Kristine Durkin, David A Fedele, Kathryn Moffett, Stephanie S Filigno, Courtney Lynn, Robin S Everhart, Lori J Stark, Christina L Duncan, Giving adolescents with cystic fibrosis a voice: Predicting cystic fibrosis nutritional adherence from their decision-making involvement, Journal of Pediatric Psychology, 2024;, jsae034 ...

  7. 7 de may. de 2024 · Cystic Fibrosis (CF) is the most common life-threatening monogenetic disease affecting an estimated 100,000 people worldwide [ 1. ]. The disease affects multiple organs but most significantly it leads to chronic, progressive lung disease and malnutrition from pancreatic insufficiency.