Successful Use of Bortezomib for Recurrent Progressive Familial Intrahepatic Cholestasis Type II After Liver Transplantation: A Pediatric Case with a 9-Year Follow-Up

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...

Full description

Saved in:
Bibliographic Details
Published inPediatric gastroenterology, hepatology & nutrition Vol. 27; no. 1; pp. 71 - 76
Main Authors Yu Gyoung Bak, Ho Jung Choi, Yeong Eun Kim, Seak Hee Oh, Kyung Mo Kim
Format Journal Article
LanguageKorean
Published 2024
Subjects
Bortezomib
PFIC type II
Bile salt export pump deficiency
Liver transplantation
Online AccessGet full text

Cover

More Information
Summary: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.
Bibliography:KISTI1.1003/JNL.JAKO202408043284635
ISSN:2234-8646
2234-8840