Barrier to using APRI and GPR as identifiers of cystic fibrosis liver disease [0.03%]
使用APRI和GPR作为囊性纤维化肝病标志物的障碍
Zachary M Sellers
Zachary M Sellers
Cystic fibrosis-associated liver disease (CFLD) is the third most common cause of death in cystic fibrosis (CF). Poor ability to identify early, non-cirrhotic liver disease hampers interventions to mitigate complications associated with CFL...
Clinical characteristics and outcomes associated with Inquilinus infection in cystic fibrosis [0.03%]
囊性纤维化中因侵入菌感染而导致的临床特点和结果分析
Patricia M Lenhart-Pendergrass,Lindsay J Caverly,Brandie D Wagner et al.
Patricia M Lenhart-Pendergrass et al.
Background: Molecular diagnostics have led to the identification of a broad range of bacterial species in cystic fibrosis (CF) including Inquilinus. The clinical significance of Inquilinus in CF has not been thoroughly ch...
Emerging preclinical modulators developed for F508del-CFTR have the potential to be effective for ORKAMBI resistant processing mutants [0.03%]
针对F508del-CFTR开发的新型临床前调节剂有可能成为ORKAMBI耐受处理突变体的有效药物
Onofrio Laselva,Claire Bartlett,Alec Popa et al.
Onofrio Laselva et al.
Background: F508del is prototypical of Class 2 CFTR mutations associated with protein misprocessing and reduced function. Corrector compounds like lumacaftor partially rescue the processing defect of F508del-CFTR whereas ...
Sarah Shrager Lusman,Drucy Borowitz,Bruce C Marshall et al.
Sarah Shrager Lusman et al.
Individuals with cystic fibrosis (CF) now have an increased life expectancy, due to advances in care provided by a multidisciplinary team. The care model has expanded over time to include multiple subspecialties. The Cystic Fibrosis Foundat...
Biliary disease and cholecystectomy after initiation of elexacaftor/ivacaftor/tezacaftor in adults with cystic fibrosis [0.03%]
使用elexacaftor/ivacaftor/tezacaftor治疗囊性纤维化成人患者的胆道疾病和胆囊切除术
Julie Safirstein,Jonathan J Grant,Emily Clausen et al.
Julie Safirstein et al.
Individuals with cystic fibrosis (CF) have an increased risk for gallbladder abnormalities and biliary tract disease, but the reported incidence of these manifestations of CF varies widely in the literature. With the approval of elexacaftor...
Citrullination of extracellular histone H3.1 reduces antibacterial activity and exacerbates its proteolytic degradation [0.03%]
组蛋白H3.1的瓜氨酸化会降低其抗细菌活性并加剧其蛋白质水解降解
Lloyd Tanner,Ravi K V Bhongir,Christofer A Q Karlsson et al.
Lloyd Tanner et al.
Background: Cystic fibrosis (CF), involves excessive airway accumulation of neutrophils, often in parallel with severe infection caused by Pseudomonas aeruginosa. Free histones are known to possess bactericidal properties...
Prospective cohort study of ototoxicity in persons with cystic fibrosis following a single course of intravenous tobramycin [0.03%]
Tobramycin导致囊性纤维化患者耳毒性的前瞻性队列研究
Angela Garinis,Malcolm Gleser,Alexis Johns et al.
Angela Garinis et al.
Introduction: Aminoglycoside (AG) antibiotics, such as tobramycin, are known to be ototoxic but important clinically due to their bactericidal efficacy. Persons with cystic fibrosis (CF) are at risk for AG-induced ototoxi...
Cystic fibrosis related diabetes: Revisiting the OGTT and alternate screening tests [0.03%]
囊性纤维化相关糖尿病：重新审视口服葡萄糖耐量试验及替代筛查方法
Christine L Chan
Christine L Chan
Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis [0.03%]
高效调节剂治疗对囊性纤维化患者鼻窦症状的影响
Emily DiMango,Jonathan Overdevest,Claire Keating et al.
Emily DiMango et al.
Background: Elexacaftor-tezacaftor-ivacaftor is a highly effective modulator for cystic fibrosis (CF) patients homozygous or heterozygous for F508del. Effects of the drug on sinonasal symptoms have not been studied. ...
Ivacaftor or lumacaftor/ivacaftor treatment does not alter the core CF airway epithelial gene response to rhinovirus [0.03%]
依伐卡特或卢安卡夫/依伐卡特治疗不会改变人鼻病毒对CF气道上皮基因的反应
Emma De Jong,Luke W Garratt,Kevin Looi et al.
Emma De Jong et al.
Background: Aberrant responses by the cystic fibrosis airway epithelium during viral infection may underly the clinical observations. Whether CFTR modulators affect antiviral responses by CF epithelia is presently unknown...