无门静脉高压的原发性胆汁性肝硬化相关肺动脉高压：病例报告
Pulmonary hypertension associated with primary biliary cirrhosis in the absence of portal hypertension: a case report.
作者信息
Yoshida E M, Erb S R, Ostrow D N, Ricci D R, Scudamore C H, Fradet G
机构信息
Department of Medicine, University of British Columbia, Vancouver, Canada.
出版信息
Gut. 1994 Feb;35(2):280-2. doi: 10.1136/gut.35.2.280.
Abstract
Pulmonary hypertension is well described in association with portal hypertension of any cause including end stage primary biliary cirrhosis (PBC). The essential feature of this association is the presence of portosystemic shunting, including surgically created shunts. A patient with primary pulmonary hypertension and PBC without portal hypertension is reported. This suggests that primary pulmonary hypertension may be associated with PBC in the absence of portal hypertension. Decisions regarding appropriate organ transplantation may depend on whether pulmonary hypertension is primary or secondary to portal hypertension.
摘要
肺动脉高压与任何病因所致的门静脉高压相关，包括终末期原发性胆汁性肝硬化（PBC），这一点已有充分描述。这种关联的基本特征是存在门体分流，包括外科手术造成的分流。本文报道了一名患有原发性肺动脉高压且无门静脉高压的PBC患者。这表明原发性肺动脉高压可能在无门静脉高压的情况下与PBC相关。关于合适器官移植的决策可能取决于肺动脉高压是原发性的还是继发于门静脉高压。
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