硼替佐米成功治疗肝移植后复发的进行性家族性肝内胆汁淤积症II型：一个随访 9 年的儿科病例。

IF 1.3 Q3 PEDIATRICS Pediatric Gastroenterology, Hepatology & Nutrition Pub Date : 2024-01-01 Epub Date: 2024-01-09 DOI:10.5223/pghn.2024.27.1.71

Yu Gyoung Bak, Ho Jung Choi, Yeong Eun Kim, Seak Hee Oh, Kyung Mo Kim

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引用次数: 0

摘要

进行性家族性肝内胆汁淤积症（PFIC）II型的复发给肝移植术后护理带来了挑战。PFIC二型患者在移植后可能会出现胆汁淤积，但γ-谷氨酰转移酶活性正常，这模拟了最初的胆盐输出泵（BSEP）蛋白缺乏症，并与一种免疫球蛋白G抗体（抗BSEP）介导的排斥反应有关。硼替佐米能有效诱导活跃的抗体产生浆细胞凋亡，这些浆细胞可能在抗体介导的排斥反应中发挥作用。在本病例中，我们使用硼替佐米治疗一名儿童肝移植后的PFIC II型复发。

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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.

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 antibody-mediated rejection. In this case, we used bortezomib to treat PFIC type II recurrence after liver transplantation in a child.

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来源期刊

Pediatric Gastroenterology, Hepatology & Nutrition PEDIATRICS-

CiteScore

3.90

自引率

0.00%

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期刊介绍： Pediatric Gastroenterology, Hepatology and Nutrition (Pediatr Gastroenterol Hepatol Nutr), an official journal of The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition, is issued bimonthly and published in English. The aim of Pediatr Gastroenterol Hepatol Nutr is to advance scientific knowledge and promote child healthcare by publishing high-quality empirical and theoretical studies and providing a recently updated knowledge to those practitioners and scholars in the field of pediatric gastroenterology, hepatology and nutrition. Pediatr Gastroenterol Hepatol Nutr publishes review articles, original articles, and case reports. All of the submitted papers are peer-reviewed. The journal covers basic and clinical researches on molecular and cellular biology, pathophysiology, epidemiology, diagnosis, and treatment of all aspects of pediatric gastrointestinal diseases and nutritional health problems.