Barge Sandrine, Grando Véronique, Nault Jean-Charles, Broudin Chloé, Beaugrand Michel, Ganne-Carrié Nathalie, Roulot Dominique, Ziol Marianne
Service d'Hépato-Gastro-entérologie, Groupe hospitalier Paris-Seine-Saint Denis, Hôpital Jean Verdier, France et Université Paris 13, Bobigny, France.
Faculté de Médecine, INSERM UMR-1162, Génomique fonctionnelle des Tumeurs solides, IUH, France Université Paris Descartes, Paris, France.
Liver Int. 2016 Jul;36(7):1059-66. doi: 10.1111/liv.12974. Epub 2015 Oct 19.
Nodular regenerative hyperplasia (NRH) is a rare histological disorder associated with a wide variety of systemic diseases.
We aimed (i) to report the prevalence of NRH in a database of liver biopsies (LBs) and the frequency of portal hypertension (PHT) at diagnosis, and (ii) to investigate whether associated diseases and/or specific histological lesions, including abnormalities of the microvasculature, were related to PHT.
Patients with a histological diagnosis of NRH, referred by seven clinical departments, were retrospectively selected. Clinical, biological, radiological, haemodynamic and endoscopic data at diagnosis were recorded. LBs were reassessed for microvascular abnormalities.
NRH was diagnosed in 4.4% of LBs (n = 159, male: 52%, mean age: 54). Among patients referred for unexplained liver enzyme abnormalities, 15% had NRH. PHT was present at diagnosis in 45 patients (38%), including 13 with portal thrombosis; 65% of patients had an associated disorder. Obliteration of portal vein branches, observed in the LBs of 17 patients (11%), was significantly associated with PHT (P = 0.02). Periportal angiomatosis, observed in 101 patients (63%), was associated with the absence of PHT (P < 10(-4) ).
We suggest that NRH is a frequent histological lesion in the setting of unexplained liver enzyme abnormalities. PHT is present at the time of diagnosis in 1/3 of patients regardless of the presence of associated disease. The frequency of periportal angiomatosis in NRH without obliteration of portal vein branches, and its association with the absence of PHT suggest that obstructive portal venopathy would not represent the most frequent mechanism involved in NRH.
结节性再生性增生（NRH）是一种罕见的组织学疾病，与多种全身性疾病相关。
我们旨在（i）报告肝活检（LB）数据库中NRH的患病率以及诊断时门静脉高压（PHT）的发生率，（ii）研究相关疾病和/或特定组织学病变，包括微血管异常，是否与PHT相关。
回顾性选择由七个临床科室转诊的组织学诊断为NRH的患者。记录诊断时的临床、生物学、放射学、血流动力学和内镜数据。对肝活检标本重新评估微血管异常情况。
在4.4%的肝活检标本中诊断出NRH（n = 159，男性：52%，平均年龄：54岁）。在因不明原因肝酶异常转诊的患者中，15%患有NRH。45例患者（38%）诊断时存在PHT，其中13例有门静脉血栓形成；65%的患者有相关疾病。在17例患者（11%）的肝活检标本中观察到门静脉分支闭塞，与PHT显著相关（P = 0.02）。101例患者（63%）观察到门静脉周围血管瘤病，与无PHT相关（P < 10⁻⁴）。
我们认为NRH在不明原因肝酶异常的情况下是一种常见的组织学病变。无论是否存在相关疾病，1/3的患者在诊断时存在PHT。在无门静脉分支闭塞的NRH中门静脉周围血管瘤病的发生率及其与无PHT的相关性表明，阻塞性门静脉病并非NRH中最常见的机制。
