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成人肝脏部分结节样转化合并静脉导管持续存在。基于发病机制假说的综述

Partial nodular transformation of liver in an adult with persistent ductus venosus. Review with hypothesis on pathogenesis.

作者信息

Wanless I R, Lentz J S, Roberts E A

出版信息

Arch Pathol Lab Med. 1985 May;109(5):427-32.

PMID:3838656
Abstract

Partial nodular transformation of the liver (PNT) is a rare condition of unknown pathogenesis in which nodules composed of hepatocytes replace portions of the parenchyma. There is usually evidence of portal hypertension and portal vein thrombosis. We present a case of PNT in a man with persistent ductus venosus and hypoplasia of the major intrahepatic portal veins but without evidence of portal hypertension or portal vein thrombosis. Portal venules were largely absent between nodules, as documented by morphometry. We suggest the pathogenesis of PNT is similar to that previously proposed for nodular regenerative hyperplasia, that is, atrophy occurs in parenchyma with insufficient blood supply and nodules arise by hyperplasia in areas with adequate supply. Partial nodular transformation and nodular regenerative hyperplasia differ mainly in the cause and distribution of the portal vein obliteration.

摘要

肝脏部分结节样转化(PNT)是一种发病机制不明的罕见病症,其中由肝细胞构成的结节取代了部分实质。通常存在门静脉高压和门静脉血栓形成的证据。我们报告一例患有持续性静脉导管和肝内主要门静脉发育不全的男性PNT病例,但无门静脉高压或门静脉血栓形成的证据。形态计量学记录显示,结节之间基本没有门静脉小分支。我们认为PNT的发病机制与先前提出的结节性再生性增生相似,即实质中血液供应不足会发生萎缩,而供应充足区域则通过增生形成结节。部分结节样转化和结节性再生性增生的主要区别在于门静脉闭塞的原因和分布。

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