关键词: ascites biopsy encephalopathy nodular regenerative hyperplasia portal hypertension varices

来  源:   DOI:10.3389/frtra.2023.1221765   PDF(Pubmed)

Abstract:
Nodular regenerative hyperplasia (NRH) is a primary disease of the liver that may cause noncirrhotic portal hypertension. Common causes include autoimmune, hematologic, immune deficiency, and myeloproliferative disorders. Given the limited data regarding the development of NRH in contemporary immunosuppressive protocols and the occurrence of NRH post-liver transplantation, we systematically reviewed NRH as it pertains to liver transplantation. We performed a comprehensive search for NRH and transplantation. Nineteen studies were identified with relevant data for NRH as an indication for a liver transplant. Thirteen studies were identified with relevant data pertaining to NRH development after liver transplant. Pooled analysis revealed 0.9% of liver transplant recipients had NRH. A total of 113 patients identified with NRH underwent liver transplantation. Most series report transplants done after the failure of endoscopic banding and TIPS management of portal hypertension. Reported 5-year graft and patient survival ranged from 73%-78% and 73%-90%. The pooled incidence of NRH after liver transplant for all indications was 2.9% and caused complications of portal hypertension. Complications related to portal hypertension secondary to NRH are a rare indication for a liver transplant. NRH can develop at any time after liver transplantation often without an identifiable cause, which may lead to portal hypertension requiring treatment or even re-transplantation.
摘要:
结节性再生增生(NRH)是肝脏的原发性疾病,可能导致非肝硬化门脉高压。常见的原因包括自身免疫性,血液学,免疫缺陷,和骨髓增生性疾病。鉴于关于NRH在当代免疫抑制方案中的发展和肝移植后NRH的发生的有限数据,我们系统回顾了NRH与肝移植的关系。我们对NRH和移植进行了全面的搜索。19项研究确定了NRH作为肝移植指征的相关数据。确定了13项研究,其中涉及肝移植后NRH发育的相关数据。汇总分析显示,有0.9%的肝移植受者患有NRH。共有113例NRH患者接受了肝移植。大多数系列报道了在门脉高压的内镜绑扎和TIPS管理失败后进行的移植。报告的5年移植物和患者生存率为73%-78%和73%-90%。所有适应症的肝移植后NRH的合并发生率为2.9%,并引起门静脉高压症的并发症。与NRH继发的门静脉高压相关的并发症是肝移植的罕见指征。NRH可以在肝移植后的任何时间发展,通常没有可识别的原因,这可能导致门静脉高压症需要治疗甚至重新移植。
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