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[儿童肝门脉硬化合并门静脉血栓形成:3例报告]

[Hepatoportal sclerosis associated with portal vein thrombosis in children: report of 3 cases].

作者信息

Hessel G, Escanhoela C A, de Oliveira A D, De-Maria H K, Onishi E, Yamada R M, Ferreira A G

机构信息

Departamento de Pediatria, Faculdade de Ciências Médicas da Universidade Estadual de Campinas-FCM/UNICAMP, SP.

出版信息

Arq Gastroenterol. 1997 Apr-Jun;34(2):121-5.

PMID:9496428
Abstract

Hepatoportal sclerosis is the term used to name a clinicopathological condition responsible for non-chirrotic portal hypertension. A three cases report of children with hepatoportal sclerosis is presented associated with portal vein thrombosis. The first two patients presented as main complaint upper digestive hemorrhage and the third one was admitted for investigation of hepatosplenomegaly. The ultrasonographic exam revealed alterations indicative of extrahepatic portal vein thrombosis in the three cases. The patients underwent liver biopsy for they had presented altered liver enzymes. The main histological findings were: subintimal sclerosis, portal fibrosis and telangiectases of the intrahepatic venous branches, consistent with the diagnosis of hepatoportal sclerosis. The three patients showed good evolution, being the hemorrhage controlled in the first two cases through esclerotherapy of esophageal varices.

摘要

肝门静脉硬化是用于命名一种导致非肝硬化性门静脉高压的临床病理状况的术语。本文报告了3例与门静脉血栓形成相关的儿童肝门静脉硬化病例。前两名患者以消化上消化道出血为主诉就诊,第三名患者因肝脾肿大接受检查。超声检查显示3例均有提示肝外门静脉血栓形成的改变。由于患者出现肝酶改变,均接受了肝活检。主要组织学表现为:内膜下硬化、门静脉纤维化和肝内静脉分支扩张,符合肝门静脉硬化的诊断。3例患者病情进展良好,前两例通过食管静脉曲张硬化治疗控制了出血。

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