Pediatric Gastroenterology, Hepatology & Nutrition

  • 제27권1호
  • /
  • Pages.71-76
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  • 2024
  • /
  • 2234-8646(pISSN)
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  • 2234-8840(eISSN)
대한소아소화기영양학회 (The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition)
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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

  • Yu Gyoung Bak (Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center Children's Hospital) ;
  • Ho Jung Choi (Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center Children's Hospital) ;
  • Yeong Eun Kim (Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center Children's Hospital) ;
  • Seak Hee Oh (Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center Children's Hospital) ;
  • Kyung Mo Kim (Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center Children's Hospital)
  • 투고 : 2023.08.02
  • 심사 : 2023.09.20
  • 발행 : 2024.01.15
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초록

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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참고문헌

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