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KMID : 1038820240270010071
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Pediatric Gastroenterology, Hepatology & Nutrition
2024 Volume.27 No. 1 p.71 ~ p.76
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Successful Use of Bortezomib for Recurrent Progressive Familial Intrahepatic Cholestasis Type II After Liver Transplantation: A Pediatric Case with a 9-Year Follow-Up
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Bak Yu-Gyoung
Choi Ho-Jung
Kim Yeong-Eun
Oh Seak-Hee
Kim Kyung-Mo
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Abstract
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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.
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KEYWORD
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Liver transplantation, Bortezomib, PFIC type II, Bile salt export pump deficiency
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Listed journal information
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