Radzhabova N I, Mansurov Kh Kh, Mirodzhov G K
Klin Med (Mosk). 2003;81(1):67-9.
The article deals with differential-diagnostic criteria of inborn liver fibrosis and hepatoportal sclerosis. Congenital liver fibrosis is inherited and manifests at young age. It is characterized by marked hepatomegaly with stony dense liver, moderate splenomegaly with hypersplenism and portal hypertension in high activity of alkaline phosphatase and 5-HT, renal affection. Fibrosis locates around the biliary ducts. Hepatoportal sclerosis manifests more frequently in the adults with moderate hepatomegaly, marked splenomegaly with hypersplenism and predominance of anemia, high portal hypertension. Fibrosis is moderate and locates around portal vein branches in which thrombi are found.
本文探讨先天性肝纤维化和肝门脉硬化的鉴别诊断标准。先天性肝纤维化是遗传性的,在年轻时发病。其特征为肝脏明显肿大且质地坚硬如石,脾脏中度肿大伴脾功能亢进以及门静脉高压,碱性磷酸酶和5 - HT活性高,伴有肾脏病变。纤维化位于胆管周围。肝门脉硬化在成年人中更常见,肝脏中度肿大,脾脏明显肿大伴脾功能亢进且以贫血为主,门静脉高压明显。纤维化程度中等,位于发现血栓的门静脉分支周围。