Elafin expression is regulated by CFTR-mutation and TGFβ1 in human bronchial epithelial cells [0.03%]
CFTR基因突变和TGFβ1调控人支气管上皮细胞中ELAFIN的表达
Jan Christoph Thomassen,Christina Vohlen,Ernst Rietschel et al.
Jan Christoph Thomassen et al.
Background: Cystic Fibrosis (CF) lung disease is characterized by inflammation and progressive matrix remodeling. These processes are influenced by genetic modifiers such as Transforming Growth Factor β1, (TGFβ1) which ...
The novel CFAbd-Score.kidⒸ reveals a significant decline of abdominal symptoms in children with Cystic fibrosis aged 6 through 11 years on Elexacaftor/Tezacaftor/Ivacaftor - first results [0.03%]
新型CFAbd-Score.kidⒸ揭示了6至11岁囊性纤维化儿童在使用Elexacaftor/Tezacaftor/Ivacaftor治疗后的腹部症状显著减轻——初步结果
Jochen G Mainz,Pauline Sadrieh,Lilith Bechinger et al.
Jochen G Mainz et al.
Background: Recently, elexacaftor/tezacaftor/ivacaftor (ETI), the turning point in the course of Cystic fibrosis (CF), was also approved for children with CF (cwCF) aged 6-11 years carrying at least one F508del mutation. ...
Prevalence and production of superantigen exotoxins by cystic fibrosis Staphylococcus aureus isolates [0.03%]
胞囊纤维化表皮葡萄球菌中超抗原外毒素的产生及分布频率
Ying Sun,Buqu Hu,Sahil Chhabra et al.
Ying Sun et al.
Staphylococcus aureus (SA) is the most common cystic fibrosis (CF) lung pathogen that is uniquely capable of producing superantigen (SAg) exotoxins, which are recognized as the most potent activators of the immune system and inducers of inf...
Factors associated with sustained Pseudomonas aeruginosa infection following elexacaftor/tezacaftor/ivacaftor treatment: Real-world data from the European cystic fibrosis society patient registry [0.03%]
elexacaftor/tezacaftor/ivacaftor治疗后绿脓杆菌持续感染的影响因素：来自欧洲囊性纤维化学会患者登记处的现实世界数据分析
Mordechai Pollak,Simone Gambazza,Annalisa Orenti et al.
Mordechai Pollak et al.
Background: Elexacaftor/tezacaftor/ivacaftor (ETI) therapy reduces airway infection rates with Pseudomonas aeruginosa (PsA). This study assessed factors associated with sustained chronic PsA infection and evaluated clinic...
Routine cell-free DNA prenatal screening identifies pregnancies at high risk for cystic fibrosis that may benefit from fetal therapy [0.03%]
常规的无细胞DNA产前筛查可识别囊性纤维化高风险妊娠以进行胎儿治疗受益
J Wynn,S Rego,D Chandler-Brown et al.
J Wynn et al.
Recent improvements in cell-free DNA technology have enabled non-invasive prenatal testing (NIPT) to screen for fetal single-gene autosomal recessive conditions from maternal blood as early as the first trimester. This technique can determi...
Developing a collaborative network for cystic fibrosis in Africa: A call to action [0.03%]
呼吁在非洲建立一个囊性纤维化协作网络
Leah Ratner,Hugues Abriel,Muriel Helmers et al.
Leah Ratner et al.
Background: Cystic fibrosis (CF) is a genetic disorder that remains underrecognized across Africa, where limited diagnostic capacity, low awareness, and competing health priorities contribute to delayed or missed diagnose...
COVID-19 in people with Cystic Fibrosis beyond the pre-omicron era: a prospective study with a specific focus on long COVID [0.03%]
超越奥密克戎时代的囊性纤维化患者的COVID-19：一项关于长期新冠的前瞻性研究
Carla Colombo,Paola Medino,Marco Cipolli et al.
Carla Colombo et al.
Background: The long-term clinical consequences of COVID-19 in cystic fibrosis (CF) remain largely unexplored. This study aimed to assess the incidence of long COVID in a large population of people with CF. ...
Identifying cystic fibrosis programs in the United States with disproportionately disadvantaged patient populations: Methodology and implications for care [0.03%]
美国囊性纤维化患者群体中识别少数族裔比例较高的医疗中心：对医疗照护的启示及方法学分析
Gabriela R Oates,Elizabeth H Baker,Lucia D Juarez et al.
Gabriela R Oates et al.
Introduction: Providing care to under-resourced patients places additional burdens on CF care teams, therefore it is important to identify programs that serve disproportionately disadvantaged populations. ...
Biomarkers associated with clinical response to cystic fibrosis pulmonary exacerbation treatment and adjuvant prednisone therapy [0.03%]
与囊性纤维化肺部急性加重治疗和辅予泼尼松治疗相关的临床反应的生物标志物
Bradley S Quon,Jasleen K Matta,Michelle Shaw et al.
Bradley S Quon et al.
Background: Cystic fibrosis (CF) pulmonary exacerbations are characterized by increased inflammation. The objectives of this study were to identify inflammatory biomarkers associated with 1) early clinical response to IV ...
An alternative view of changes in lung function among people with cystic fibrosis [0.03%]
囊性纤维化患者肺功能变化的另一种观点
Oliver J McElvaney,Christopher H Goss
Oliver J McElvaney