Nakanuma Y, Hoso M, Sasaki M, Terada T, Katayanagi K, Nonomura A, Kurumaya H, Harada A, Obata H
Department of Pathology (II), Kanazawa University School of Medicine, Japan.
Histopathology. 1996 Mar;28(3):195-204. doi: 10.1046/j.1365-2559.1996.d01-412.x.
Non-cirrhotic, long-standing portal hypertension of unknown aetiology is being re-evaluated histopathologically and clinically. In this study, we examined 107 livers with this condition (92 wedge biopsy and 15 autopsy specimens) from five institutions in Japan. These cases were histologically categorized into four groups: idiopathic portal hypertension (66 cases), nodular regenerative hyperplasia (14 cases), partial nodular transformation (two cases), and incomplete septal cirrhosis (25 cases). These four groups shared several histological features: dense portal fibrosis with portal venous obliteration and intralobular slender fibrosis. In addition, the histopathological features characteristic of one group were also found to a mild degree in other groups. The histopathological lesions preceding portal venous obliteration remain speculative. However, the portal venous obliteration may be responsible for the occurrence of sustained portal hypertension and several of the pathological changes in these livers. It seems likely that idiopathic portal hypertension, nodular regenerative hyperplasia, partial nodular transformation and incomplete septal cirrhosis comprise a family of non-cirrhotic, long-standing portal hypertension in Japan, and the histological differences between them may reflect chronological progression of a single disease.
病因不明的非肝硬化性、长期门静脉高压正从组织病理学和临床方面进行重新评估。在本研究中，我们检查了来自日本五个机构的107例患有这种疾病的肝脏（92例楔形活检和15例尸检标本）。这些病例在组织学上分为四组：特发性门静脉高压（66例）、结节性再生性增生（14例）、部分结节样转化（2例）和不完全性间隔性肝硬化（25例）。这四组具有一些共同的组织学特征：伴有门静脉闭塞的致密门静脉纤维化和小叶内纤细纤维化。此外，一组特有的组织病理学特征在其他组中也有轻度发现。门静脉闭塞之前的组织病理学病变仍存在推测。然而，门静脉闭塞可能是这些肝脏中持续性门静脉高压发生及一些病理变化的原因。特发性门静脉高压、结节性再生性增生、部分结节样转化和不完全性间隔性肝硬化似乎构成了日本非肝硬化性、长期门静脉高压的一个家族，它们之间的组织学差异可能反映了单一疾病的时间进程。
