非肝硬化性门脉纤维化伴肺动静脉交通及肺动脉高压。

Non-cirrhotic portal fibrosis associated with pulmonary arteriovenous communication and pulmonary arterial hypertension.

作者信息

Chongsrisawat V, Vivatvakin B, Suwangool P, Vajragupta L, Poovorawan Y

机构信息

Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.

出版信息

Southeast Asian J Trop Med Public Health. 1998 Mar;29(1):76-9.

PMID:9740273

Abstract

A case of non-cirrhotic portal fibrosis associated with pulmonary arteriovenous communication and pulmonary arterial hypertension is reported. The patient was a 7-year old boy who presented with hematemesis, cyanosis, hypoxemia and orthodeoxia. His liver pathology was compatible with non-cirrhotic portal fibrosis. His pulmonary angiography showed arteriovenous shunting and pulmonary arterial hypertension (mean pulmonary artery pressure 34 mmHg). His sister also had non-cirrhotic portal fibrosis with neither hypoxemia nor orthodeoxia. This report raises the possibility of non-cirrhotic portal fibrosis having a genetic etiology.

摘要

报告了一例与肺动静脉交通和肺动脉高压相关的非肝硬化性门脉纤维化病例。患者为一名7岁男孩，出现呕血、发绀、低氧血症和直立性低氧血症。他的肝脏病理符合非肝硬化性门脉纤维化。他的肺血管造影显示动静脉分流和肺动脉高压（平均肺动脉压34 mmHg）。他的妹妹也患有非肝硬化性门脉纤维化，但既无低氧血症也无直立性低氧血症。本报告提出了非肝硬化性门脉纤维化具有遗传病因的可能性。