Arvanitaki M, Adler M
Service de Hepatogastroenterologie, Universite Libre de Bruxelles, Hôpital Erasme, Brussels, Belgium.
Hepatogastroenterology. 2001 Sep-Oct;48(41):1425-9.
BACKGROUND/AIMS: Nodular regenerative hyperplasia of the liver, is a noncirrhotic liver disease, characterized by nodules in the hepatic parenchyma, which clinically presents primarily with manifestations of portal hypertension. The aims of this study are i) to review the clinical, histological and diagnostic aspects of 14 documented cases of NRHL, and ii) to assess the evolution and management of this condition in the cases reviewed.
The diagnosis of nodular regenerative hyperplasia of the liver was based on liver biopsy in all cases. Imaging studies (ultrasonography, computed tomography scan and magnetic resonance imaging scan) were performed as part of the diagnostic evaluation. Clinical manifestations and biochemical tests were recorded at the time of diagnosis. Management and prognosis were also reviewed.
The most common clinical manifestations were those of portal hypertension, namely splenomegaly, esophageal varices and variceal bleeding. The histological findings were nodules in the hepatic parenchyma, the typical histologic feature of nodular regenerative hyperplasia of the liver, with mild periportal fibrosis and intraportal lymphocytic infiltration. Biochemical tests showed normal synthetic liver function, as evidenced by normal serum albumin, bilirubin and prothrombin time. Elevation of gamma-glutamyl transpeptidase and alkaline phosphatase due to cholestasis was noted. Management was directed to portal hypertension and variceal bleeding, with beta-blockers, sclerotherapy, mesenteric-caval shunt and transjugular intrahepatic portosystemic shunt with satisfactory results.
Nodular regenerative hyperplasia of the liver is an uncommon condition but it should be considered in patients with unexplained portal hypertension and distinguished from liver cirrhosis, in view of the differences in the natural history and prognosis. Liver biopsy confirms the diagnosis. Management is directed primarily to portal hypertension and variceal bleeding, which is the main source of mortality. Liver failure is uncommon due to satisfactory preservation of liver function.
背景/目的：肝结节状再生性增生是一种非肝硬化性肝病，其特征为肝实质内出现结节，临床上主要表现为门静脉高压症状。本研究的目的是：i）回顾14例有记录的肝结节状再生性增生病例的临床、组织学及诊断方面情况；ii）评估所回顾病例中该疾病的病情发展及治疗情况。
所有病例均通过肝活检诊断肝结节状再生性增生。作为诊断评估的一部分，进行了影像学检查（超声、计算机断层扫描和磁共振成像扫描）。记录诊断时的临床表现及生化检查结果。还回顾了治疗及预后情况。
最常见的临床表现为门静脉高压症状，即脾肿大、食管静脉曲张及曲张静脉出血。组织学检查发现肝实质内有结节，这是肝结节状再生性增生的典型组织学特征，伴有轻度门周纤维化及门脉内淋巴细胞浸润。生化检查显示肝功能合成正常，血清白蛋白、胆红素及凝血酶原时间正常即为证据。因胆汁淤积导致γ-谷氨酰转肽酶和碱性磷酸酶升高。治疗针对门静脉高压及曲张静脉出血，采用β受体阻滞剂、硬化疗法、肠系膜-腔静脉分流术及经颈静脉肝内门体分流术，效果满意。
肝结节状再生性增生是一种罕见疾病，但对于不明原因门静脉高压患者应考虑此病，并鉴于其自然史及预后的差异与肝硬化相鉴别。肝活检可确诊。治疗主要针对门静脉高压及曲张静脉出血，这是主要的死亡原因。由于肝功能保存良好，肝衰竭并不常见。
