TY - JOUR
T1 - Biliary disease and cholecystectomy after initiation of elexacaftor/ivacaftor/tezacaftor in adults with cystic fibrosis
AU - Safirstein, Julie
AU - Grant, Jonathan J.
AU - Clausen, Emily
AU - Savant, Deepika
AU - Dezube, Rebecca
AU - Hong, Gina
N1 - Funding Information:
We would like to acknowledge Dr. Kristoffel Dumon and Dr. Emma Furth for their clinical contributions to this work. Also, the authors would like to thank the patients described in the case series for their participation.
Publisher Copyright:
© 2020
PY - 2021/5
Y1 - 2021/5
N2 - 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.
AB - 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.
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U2 - 10.1016/j.jcf.2020.07.014
DO - 10.1016/j.jcf.2020.07.014
M3 - Article
C2 - 32736949
AN - SCOPUS:85088801362
SN - 1569-1993
VL - 20
SP - 506
EP - 510
JO - Journal of Cystic Fibrosis
JF - Journal of Cystic Fibrosis
IS - 3
ER -