Published online Feb 26, 2023. doi: 10.12998/wjcc.v11.i6.1393
Peer-review started: November 8, 2022
First decision: November 24, 2022
Revised: December 7, 2022
Accepted: February 2, 2023
Article in press: February 2, 2023
Published online: February 26, 2023
Processing time: 108 Days and 5.9 Hours
Core Tip: We presented a case of multiple intrahepatic bile duct stones, cholestasis, and progressive jaundice after surgical treatment, which was diagnosed upon the finding of adenosine triphosphate-binding cassette subfamily B member 11 (ABCB11) and uridine 5’-diphospho-glucuronosyltransferase 1A1 (UGT1A1) gene mutation from the genetic study. Since the patient refused to undergo liver transplantation, his postoperative aggravating jaundice was medically managed, but he soon died due to liver failure. The UGT1A1 gene is related to the glucuronidation of indirect bilirubin. Its mutation leads to increased indirect bilirubin, while the ABCB11 gene is involved in bile transport, and its mutation may lead to disturbance of bile acid transport, changes in bile composition, cholestasis, and the formation of intrahepatic bile duct stones. Surgical treatment in such patients may induce exacerbation of cholestasis, and liver transplantation should be the preferred treatment if medical management fails.