陆慰萱 王莞尔 罗慰慈 李方 朱元珏 马毅 100730 中国医学科学院、中国协和医科大学 北京协和医院呼吸科(陆慰萱、王莞尔、罗慰慈、朱元珏、马毅)，核医学科(李方) 中华内科杂志 1998 0 0 12


    关键词：低氧；肝功能衰竭；血管舒张；肝肺综合征 期刊 zhnkzz 0 论 著 fur -->


    

【摘要 】 目的 提高对肝肺综合征(HPS)的认识。方法 回顾性分析了6例HPS的临床资料，所有患者均经动态肺灌注显像检查。结果 6例HPS的病因均为肝炎后肝硬化。除有肝病的临床表现外，还有进行性呼吸困难、紫绀(6/6)、杵状指(5/6)和低氧血症，平均动脉血氧分压为6.87kPa(1 kPa =7.5 mmHg)，动脉血氧饱和度为85.2%。此外，还有立位性缺氧或平卧呼吸。6例患者经动态肺灌注显像均异常，有肺内血管扩张。结论 有门脉高压、皮下蜘蛛痣和杵状指体征时，提示HPS的存在。早期诊断和治疗有助于缓解低氧血症，改善患者的预后。

A study on the diagnosis andtreatment of hepatopulmonary syndrome Lu Weixuan, Wang Waner, Luo Weici, et al. Department of Respiratory Diseases, Peking UnionMedical College Hospital, Peking Union Medical College, Chinese Academy of MedicalSciences, Beijing 100730

【Abstract 】 Objective Toenhance the understanding of hepatopulmonary syndrome (HPS). Methods 6 cases of HPS confirmed by liver function tests, blood gasanalyses and image examinations were reviewed. Results The liver disease associated with HPS was cirrhosis in all the 6cases. The clinical manifestations were progressive dyspnea, cyanosis (6/6), clubbingfinger (5/6) and hypoxemia associated with liver disease (mean PaO₂ 6.87 kPa,SaO₂ 85.2%) in addition to orthodeoxia and platypnea. Intrapulmonary vasculardilation was demonstrated with dynamic lung perfusion scan (6 cases) or contrast-enhancedechocardiography (1 case). Conclusion The constellation of clinical findings of portal hypertension,cutaneous spider nevi and clubbing finger is strongly suggestive of HPS. In treating HPSvascular embolization and liver transplantation may be initiated in addition to a varietyof therapeutic agents.

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