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原发性肺动脉高压:一例与肝外门静脉高压相关的罕见病例。

Primary pulmonary hypertension: an unusual case associated with extrahepatic portal hypertension.

作者信息

Cohen M D, Rubin L J, Taylor W E, Cuthbert J A

出版信息

Hepatology. 1983 Jul-Aug;3(4):588-92. doi: 10.1002/hep.1840030419.

DOI:10.1002/hep.1840030419
PMID:6862372
Abstract

A patient with both extrahepatic portal hypertension and primary pulmonary hypertension is reported. The pulmonary hypertension developed without a surgical portal-systemic shunt, and at autopsy there was no evidence of a large spontaneous shunt. This association of pulmonary arterial hypertension and portal venous hypertension without either intrinsic liver disease or a large portal-systemic anastomosis has not been reported previously. Our case supports the concept that portal hypertension with its attendant portal-systemic collateral circulation may be the major predisposing cause of pulmonary hypertension both in intrinsic liver disease and extrahepatic portal vein obstruction.

摘要

本文报告了一例同时患有肝外门静脉高压症和原发性肺动脉高压症的患者。该患者的肺动脉高压在未进行外科门体分流手术的情况下发生,尸检时未发现存在大的自发性分流的证据。此前尚未有肺动脉高压与门静脉高压并存且既无内在肝脏疾病又无大的门体吻合的相关报道。我们的病例支持这样一种观点,即伴有随之而来的门体侧支循环的门静脉高压可能是内在肝脏疾病和肝外门静脉阻塞中肺动脉高压的主要诱发原因。

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