Obesity in adults with cystic fibrosis following prescription of elexacaftor/tezacaftor/ivacaftor [0.03%]
用于囊性纤维化成人患者的elexacaftor/tezacaftor/ivacaftor处方后的肥胖问题
Joshua S Ostrenga,Christina M Mingora,Sylvia Szentpetery et al.
Joshua S Ostrenga et al.
We conducted a descriptive analysis of people with CF 20 years of age and older using CF Foundation Patient Registry data to provide (1) current estimate of the overweight and obese population and (2) the effect of elexacaftor-tezacaftor-iv...
Safety and efficacy of elexacaftor/tezacaftor/ivacaftor in children ≥2 years with cystic fibrosis: 96-week interim results from a phase 3 open-label extension study [0.03%]
一项针对≥2岁囊性纤维化患者接受elexacaftor/tezacaftor/ivacaftor治疗的安全性和有效性的三期开放标签延长研究的96周中期结果
Jennifer L Goralski,Cori Daines,Jessica E Pittman et al.
Jennifer L Goralski et al.
Background: Elexacaftor/tezacaftor/ivacaftor(ELX/TEZ/IVA) was safe and efficacious in children with CF 2-5y with at least one F508del allele in 24-week phase 3 Trial 445-111. Children who completed 445-111 were eligible f...
A functional comparison of vanzacaftor/tezacaftor/deutivacaftor and elexacaftor/tezacaftor/ivacaftor in patient-derived intestinal organoids with rare CFTR variants [0.03%]
携带罕见CFTR变异的肠道类器官对凡卡法托/泰扎卡法托/氘代伊伐库法托与艾拉卡法托/泰扎卡法托/伊伐库法托药效比较研究
Suzanne Kroes,Lara Zaidi,Heleen N Sonneveld-van Kooten et al.
Suzanne Kroes et al.
Background: Cystic fibrosis (CF) results from pathogenic CFTR variants that impair epithelial chloride and bicarbonate transport. CFTR modulators such as elexacaftor/tezacaftor/ivacaftor (ETI) have transformed CF care, an...
A multi-center study of non-invasive colorectal cancer evaluation in cystic fibrosis (NICE-CF) [0.03%]
囊性纤维化患者的非侵入性结直肠癌评估的多中心研究(NICE-CF)
Christine Hachem,William Tressel,Karen D Hinckley Stukovsky et al.
Christine Hachem et al.
Background: People with Cystic Fibrosis (PwCF) have a higher risk of digestive cancers compared to non-CF age-matched cohorts. Colonoscopy is the standard for colorectal cancer (CRC) and precancerous lesion screening. In ...
Genotoxic pks + E. coli is strongly associated with ileocolonic neoplasia in adults with Cystic Fibrosis [0.03%]
细胞毒pks＋大肠杆菌与囊性纤维化成人回盲部肿瘤强相关
Josie van Dorst,Nicole Taylor,B L D Uthpala Pushpakumara et al.
Josie van Dorst et al.
Background: Polyketide synthase island-positive (pks+) Escherichia coli is a genotoxic gut bacterium linked to colorectal cancer (CRC) tumorigenesis via the genotoxin colibactin. In adults with Cystic Fibrosis (CF), there...
Is the gut a reservoir of non-tuberculous mycobacteria in people with cystic fibrosis? [0.03%]
囊性纤维化患者肠道是否是非结核分枝杆菌的储存库？
Jennifer Hudson,Nicole Taylor,Michael J Coffey et al.
Jennifer Hudson et al.
Non-tuberculous mycobacteria (NTM) represent a significant disease burden in the cystic fibrosis (CF) population. Whilst the acquisition of NTM to the respiratory tract is predominantly attributed to environmental exposures, the contributio...
Vanzacaftor-Tezacaftor as an alternative therapeutic resource for the ETI-Resistant L467F-F508del Allele: Ex vivo prediction and exploratory clinical assessment [0.03%]
药物 vanzacoftor-tezacaftor 可能是对 ETI 耐药的L467F-F508del基因型进行 ex vivo 预测和临床探索性评估的一种新的治疗选择
Valeria Capurro,Emanuela Pesce,Federico Cresta et al.
Valeria Capurro et al.
Background: The triple combination of Elexacaftor/Tezacaftor/Ivacaftor (ETI) has revolutionized cystic fibrosis (CF) treatment; however, a subset of patients with eligible genotypes remains unresponsive. We previously ide...
Cystic fibrosis care utilization from 2019-2023: a descriptive analysis of adult and pediatric CF care utilization in the post-ETI and post-pandemic Era [0.03%]
囊性纤维化护理利用情况（2019-2023）：后抗菌酶治疗和疫情时代成人及儿童CF护理利用描述性分析
Samar E Atteih,Rachel S Mbassa,Joseph M Collaco et al.
Samar E Atteih et al.
Background: Cystic fibrosis (CF) care utilization decreased during the COVID-19 pandemic. This study aims to describe the utilization of recommended components of CF care over the past five years to determine the extent t...
Ensuring equity in reproductive carrier screening of CFTR with increasing population diversity [0.03%]
确保囊性纤维化跨膜传导调节蛋白生殖系携带者筛查的公平性以应对人口多样性增加带来的挑战
Mia J Gruzin,Jaysen Knezovich,Sarah Poll et al.
Mia J Gruzin et al.
Background: Carrier screening for cystic fibrosis (CF; CFTR gene) identifies individuals at risk of having affected children. Screening was originally developed for populations of European ancestry, in whom disease incide...
Maldigestion, dysbiosis, inflammation and dysmotility: a toxic tetrad in the CF gut [0.03%]
消化不良、菌群失调、炎症和运动功能障碍：囊性纤维化肠道的毒性四联症
Rosara Bass,Alan R Smyth
Rosara Bass
Gastrointestinal symptoms are common in CF, present in at least 65%, and possibly as high as 98% of people with CF (PwCF) and up to 20% reporting these symptoms to be moderate to severe. Chronic GI symptoms including flatulence, bloating, n...