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Biliary disease and cholecystectomy after initiation of elexacaftor/ivacaftor/tezacaftor in adults with cystic fibrosis
Journal of Cystic Fibrosis ( IF 5.2 ) Pub Date : 2020-07-01 , DOI: 10.1016/j.jcf.2020.07.014
Julie Safirstein 1 , Jonathan J Grant 2 , Emily Clausen 3 , Deepika Savant 4 , Rebecca Dezube 5 , Gina Hong 3
Affiliation  

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/tezacaftor/ivacaftor (ELX/TEZ/IVA), increasing numbers of CF patients have been initiated on highly effective cystic fibrosis transmembrane regulator (CFTR) modulator therapy. While elevations in hepatic panel are known potential side effects of CFTR modulators, there have been no published cases of biliary disease or acute cholecystitis attributed to these medications. In this case series, we describe seven patients at two adult CF centers with biliary colic shortly after initiation with ELX/TEZ/IVA, six of whom required cholecystectomy.

中文翻译:

在患有囊性纤维化的成人中开始使用 elexacaftor/ivacaftor/tezacaftor 后的胆道疾病和胆囊切除术

患有囊性纤维化 (CF) 的个体患胆囊异常和胆道疾病的风险增加,但文献中报道的这些 CF 表现的发生率差异很大。随着 elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) 的批准,越来越多的 CF 患者开始接受高效的囊性纤维化跨膜调节剂 (CFTR) 调节剂治疗。虽然肝组升高是已知的 CFTR 调节剂的潜在副作用,但尚未公布归因于这些药物的胆道疾病或急性胆囊炎病例。在这个案例系列中,我们在两个成人 CF 中心描述了在 ELX/TEZ/IVA 开始后不久患有胆绞痛的 7 名患者,其中 6 人需要进行胆囊切除术。
更新日期:2020-07-01
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