Meijer Berrie, Simsek Melek, Blokzijl Hans, de Man Robert A, Coenraad Minneke J, Dijkstra Gerard, van Nieuwkerk Carin Mj, Mulder Chris Jj, de Boer Nanne Kh
Department of Gastroenterology and Hepatology, VU University Medical Center Amsterdam, The Netherlands.
Department of Gastroenterology and Hepatology, University Medical Center Groningen, Groningen, The Netherlands.
United European Gastroenterol J. 2017 Aug;5(5):658-667. doi: 10.1177/2050640616680550. Epub 2016 Nov 16.
Nodular regenerative hyperplasia is an uncommon liver condition associated with several autoimmune disorders and drugs. The clinical symptoms of nodular regenerative hyperplasia vary from asymptomatic to severe complications of portal hypertension (nodular regenerative hyperplasia-syndrome).
The purpose of this study was to identify the prognosis and optimal management, as well as the role of liver transplantation, in nodular regenerative hyperplasia.
The pathology databases of all three Dutch liver transplant units were retrospectively scrutinised for explanted livers diagnosed with nodular regenerative hyperplasia or without clear diagnosis. Pre- and post-transplantation clinical, biochemical, radiological and histological information was obtained from electronic and paper records.
In total, 1886 patients received a liver transplant. In 255 patients, nodular regenerative hyperplasia could not be excluded. After detailed chart review, the native livers of 11 patients (0.6%) (82% male, median age: 44 years) displayed nodular regenerative hyperplasia. Seven patients (64%) had underlying disorders or drug exposure which possibly caused nodular regenerative hyperplasia. Laboratory and imaging abnormalities were present in all patients but did not contribute to the diagnosis of nodular regenerative hyperplasia. Five-year survival was 73% (median follow-up: four years, range: 2-248 months).
Nodular regenerative hyperplasia is a rare finding in patients, predominantly young males, transplanted for end-stage liver disease with unknown aetiology. Nonetheless, liver transplantation may have an important role in end-stage nodular regenerative hyperplasia-syndrome.
结节性再生性增生是一种罕见的肝脏疾病，与多种自身免疫性疾病和药物有关。结节性再生性增生的临床症状从无症状到门静脉高压的严重并发症（结节性再生性增生综合征）不等。
本研究的目的是确定结节性再生性增生的预后和最佳治疗方法，以及肝移植的作用。
对荷兰所有三个肝移植单位的病理数据库进行回顾性审查，以查找诊断为结节性再生性增生或诊断不明确的移植肝脏。从电子和纸质记录中获取移植前后的临床、生化、放射学和组织学信息。
共有1886例患者接受了肝移植。255例患者不能排除结节性再生性增生。经过详细的病历审查，11例患者（0.6%）（82%为男性，中位年龄：44岁）的原生肝脏表现为结节性再生性增生。7例患者（64%）有潜在疾病或药物接触史，可能导致结节性再生性增生。所有患者均有实验室和影像学异常，但这些异常对结节性再生性增生的诊断无帮助。五年生存率为73%（中位随访时间：四年，范围：2-248个月）。
结节性再生性增生在因病因不明的终末期肝病接受移植的患者中是一种罕见的发现，主要为年轻男性。尽管如此，肝移植在终末期结节性再生性增生综合征中可能具有重要作用。
