Acneiform Eruption Following Elexacaftor-Tezacaftor-Ivacaftor Treatment in Patients With Cystic Fibrosis.
Sign Up to like & getrecommendations! Published in 2022 at "JAMA dermatology"
DOI: 10.1001/jamadermatol.2022.5208
Abstract: Importance A new treatment for cystic fibrosis combining 3 CFTR modulators-elexacaftor (ELX), tezacaftor (TEZ), and ivacaftor (IVA)-has recently been approved for cystic fibrosis treatment. The cutaneous adverse effects following treatment with this combination are poorly… read more here.
Keywords: cystic fibrosis; patients cystic; iva; treatment ... See more keywords
Association of Body Mass Index With Clinical Outcomes in Patients With Cystic Fibrosis
Sign Up to like & getrecommendations! Published in 2022 at "JAMA Network Open"
DOI: 10.1001/jamanetworkopen.2022.0740
Abstract: Key Points Question Is higher-than-normal body mass index (BMI) associated with better clinical outcomes in patients with cystic fibrosis? Findings In this systematic review and meta-analysis of studies including 9114 patients with cystic fibrosis, BMI… read more here.
Keywords: clinical outcomes; patients cystic; body mass; mass index ... See more keywords
Body Mass Index and Clinical Outcomes in Persons Living With Cystic Fibrosis-Is Bigger Always Better?
Sign Up to like & getrecommendations! Published in 2022 at "JAMA network open"
DOI: 10.1001/jamanetworkopen.2022.0749
Abstract: Cystic fibrosis (CF) is the most common lethal genetic disorder in the White population, affecting nearly 100 000 individuals worldwide.1 Variations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene leads to abnormal chloride and… read more here.
Keywords: clinical outcomes; status; mass; cystic fibrosis ... See more keywords
Real-world Associations of US Cystic Fibrosis Newborn Screening Programs With Nutritional and Pulmonary Outcomes.
Sign Up to like & getrecommendations! Published in 2022 at "JAMA pediatrics"
DOI: 10.1001/jamapediatrics.2022.2674
Abstract: Importance Newborn screening (NBS) for cystic fibrosis (CF) has been universal in the US since 2010, but its association with clinical outcomes is unclear. Objective To describe the real-world effectiveness of NBS programs for CF… read more here.
Keywords: age; cohort; age years; newborn screening ... See more keywords
Head-to-head trials of antibiotics for non-cystic fibrosis bronchiectasis
Sign Up to like & getrecommendations! Published in 2017 at "Cochrane Database of Systematic Reviews"
DOI: 10.1002/14651858.cd012590
Abstract: This is a protocol for a Cochrane Review (Intervention). The objectives are as follows: To evaluate the comparative effects of different antibiotics in the treatment of adults and children with non-cystic fibrosis bronchiectasis. read more here.
Keywords: head; cystic fibrosis; fibrosis bronchiectasis; non cystic ... See more keywords
Structural Consequences of the 1,2,3‐Triazole as an Amide Bioisostere in Analogues of the Cystic Fibrosis Drugs VX‐809 and VX‐770
Sign Up to like & getrecommendations! Published in 2020 at "ChemMedChem"
DOI: 10.1002/cmdc.202000220
Abstract: Although the 1,2,3‐triazole is a commonly used amide bioisostere in medicinal chemistry, the structural implications of this replacement have not been fully studied. Employing X‐ray crystallography and computational studies, we report the spatial and electronic… read more here.
Keywords: cystic fibrosis; fibrosis drugs; analogues cystic; amide bioisostere ... See more keywords
Towards Innovative Antibacterial Correctors for Cystic Fibrosis Targeting the Lung Microbiome with a Multifunctional Effect
Sign Up to like & getrecommendations! Published in 2022 at "ChemMedChem"
DOI: 10.1002/cmdc.202200277
Abstract: Cystic fibrosis (CF) is a genetic disease caused by loss‐of‐function mutations in the CFTR gene, which codes for a defective ion channel. This causes an electrolyte imbalance and results in a spiral of negative effects… read more here.
Keywords: effect; towards innovative; lung; innovative antibacterial ... See more keywords
Src kinase inhibition reduces inflammatory and cytoskeletal changes in ΔF508 human cholangiocytes and improves cystic fibrosis transmembrane conductance regulator correctors efficacy
Sign Up to like & getrecommendations! Published in 2018 at "Hepatology"
DOI: 10.1002/hep.29400
Abstract: Cystic fibrosis transmembrane conductance regulator (CFTR), the channel mutated in cystic fibrosis (CF), is expressed by the biliary epithelium (i.e., cholangiocytes) of the liver. Progressive clinical liver disease (CF‐associated liver disease; CFLD) occurs in around… read more here.
Keywords: cystic fibrosis; kinase; src kinase; fibrosis transmembrane ... See more keywords
Cystic fibrosis F508del carriers and cancer risk: Results from the UK Biobank
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DOI: 10.1002/ijc.33431
Abstract: Cystic fibrosis (CF) carriers carrying one defective copy of a CFTR germline mutation are common in the general population. A recent study reported associations of CF carriers with risk for cancers of digestive organs and… read more here.
Keywords: cystic fibrosis; risk; study; f508del carriers ... See more keywords
Exploring parental cystic fibrosis disclosure to well children
Sign Up to like & getrecommendations! Published in 2022 at "Journal of Genetic Counseling"
DOI: 10.1002/jgc4.1658
Abstract: Cystic fibrosis (CF), a genetic disease and chronic illness, affects multiple organ systems and requires exceptional medical care and treatment. Few studies have assessed the diagnosis disclosure process to well children when their sibling(s) have… read more here.
Keywords: well children; disclosure process; exploring parental; disclosure ... See more keywords
Neutrophil‐derived extracellular vesicles promote feed‐forward inflammasome signaling in cystic fibrosis airways
Sign Up to like & getrecommendations! Published in 2022 at "Journal of Leukocyte Biology"
DOI: 10.1002/jlb.3ab0321-149r
Abstract: Cystic fibrosis (CF) airways feature high extracellular levels of the IL‐1 family of proinflammatory mediators. These mediators are cleavage products of caspase‐1, the final protease in the inflammasome cascade. Due to the proven chronic presence… read more here.
Keywords: extracellular vesicles; neutrophil derived; inflammasome signaling; feed forward ... See more keywords