Successful Use of Bortezomib for Recurrent Progressive Familial Intrahepatic Cholestasis Type II After Liver Transplantation: A Pediatric Case with a 9-Year Follow-Up
10.5223/pghn.2024.27.1.71
- Author:
Yu Gyoung BAK
1
;
Ho Jung CHOI
;
Yeong Eun KIM
;
Seak Hee OH
;
Kyung Mo KIM
Author Information
1. Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center Children’s Hospital, Seoul, Korea
- Publication Type:Short Communication
- From:Pediatric Gastroenterology, Hepatology & Nutrition
2024;27(1):71-76
- CountryRepublic of Korea
- Language:English
-
Abstract:
Recurrence of progressive familial intrahepatic cholestasis (PFIC) type II poses challenges during postoperative liver transplant care. Posttransplant patients with PFIC type II risk developing recurrent cholestasis with normal gamma-glutamyl transferase activity, which mimics the original bile salt export pump (BSEP) protein deficiency and is related to a form of immunoglobulin G antibody (anti-BSEP)-mediated rejection. Bortezomib effectively induces apoptosis of actively antibody-producing plasma cells that may have a role in antibodymediated rejection. In this case, we used bortezomib to treat PFIC type II recurrence after liver transplantation in a child.
