对肝肺综合征诊治的探讨

A study on the diagnosis and treatment of hepatopulmonary syndrome

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（１ｋＰａ＝７．５ｍｍＨｇ），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。

Objective To enhance the understanding of hepatopulmonary syndrome (HPS). Methods 6 cases of HPS confirmed by liver function tests, blood gas analyses and image examinations were reviewed. Results The liver disease associated with HPS was cirrhosis in all the 6 cases. The clinical manifestations were progressive dyspnea, cyanosis (6/6), clubbing finger (5/6) and hypoxemia associated with liver disease (mean PaO 2 6 87 kPa, SaO 2 85.2%) in addition to orthodeoxia and platypnea. Intrapulmonary vascular dilation was demonstrated with dynamic lung perfusion scan (6 cases) or contrast enhanced echocardiography (1 case). Conclusion The constellation of clinical findings of portal hypertension, cutaneous spider nevi and clubbing finger is strongly suggestive of HPS. In treating HPS vascular embolization and liver transplantation may be initiated in addition to a variety of therapeutic agents.