肝肺综合征的临床特点及诊断

Hepatopulmonary Syndrome:Clinical Features and Diagnosis

目的:探讨肝肺综合征(HPS)的临床特点及诊断方法,提高对其认识。方法:回顾性分析我院1986年1月-2003年12月确诊的13例患者的临床资料。结果:除1例为慢性酒精性肝损害基础上的急性药物性肝炎外,12例均为肝硬化患者,据Child-Pugh分级标准,肝功能B级和C级者11例(84.60％);平均动脉血氧分压(PaO2)为6.94±1.5 kPa,10例中9例直立位缺氧大于10％。临床主要表现为呼吸困难(100％)、紫绀(100％)、杵状指(84.6％)、肝掌(84.6%)、面部毛细血管扩张(84.6％)、蜘蛛痣(69.2％)。9例行肺功能检查患者中8例为弥散功能障碍。所有患者均经99mrc标记的人血白蛋白聚合颗粒(99mTc-MAA)首次通过肺灌注显像确诊,测定肺内分流率为22％-57％。11例同时计算吸入100％氧气时动静脉分流占总血流量百分比(Qs/Qt)的结果为10.9％-27.4％,均小于前者所得。结论:肝肺综合征的发生与肝功能不全和门脉高压症都有关系,同时有门静脉高压、紫绀、杵状指、蜘蛛痣或面部毛细血管扩张时应考虑该诊断。自立位缺氧(>10％)为典型表现,99mTc-MAA首次通过肺灌注显像足有效的诊断方法,所测定的肺内分流率大于Qs/Qt。

Objective:To study the clinical features and diagnostic methods of hepatopulmonary syndrome (HPS) .Methods: 13 cases of HPS from Jan, 1986 to Dec,2003 in PUMC Hospital were investigated retrospectively. Results: 12 patients had hepatic cirrhosis while the other one had drug induced acute hepatitis on chronic alcoholic liver injury ,11 of 13 cases (84.6 % ) had the liver function of Child-Pugh class B and class C. The mean PaO2 was 6.9 ± 1.5 kPa and 9 of 10 cases had orthodeoxia(a fall in PaO2 of > 10% from the supine to the standing position) .The main clinical manifestations were dyspnea (100% ),cyanosis( 100%) .elubbing finger (84.6%) .liver palms(84.6% ), telangiectasis on face(84.6%) and spider angiomata(69.2% ) .8 of 9 patients showed the diffusing disorders on pulmonary function test. All the cases were diagnosed by radionuclide lung perfusion scanning using technetium-labled macroaggregeted albumin particles (99mTc-MAA) with the shtunt ratio of 22% - 57% , while the results of Qs/Qt (the shunt ratio with 100% oxygen inspired) were 10.9% - 27.4% . Conclusion: Both hepatic disfunction and portal hypertension account for HPS. The presence of portal hypertension, cyanosis, clubbing Finger, telangiectasis on face or cutaneous spider angiomata strongly suggest the diagnosis of HPS while orthodeoxia is very common. The 99mTc-MAA lung perfusion scanning is able to determine the presence of and to quantify intrapulmonary shunting. The shunt tatio is more prominent compared with Qs/Qt in HPS.