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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 investigate the clinical characteristics and diagnosis of hepatopulmonary syndrome (HPS) and to improve its understanding. Methods: The clinical data of 13 patients diagnosed in our hospital from January 1986 to December 2003 were analyzed retrospectively. Results: All the 12 cases were patients with cirrhosis, except for acute drug - induced hepatitis based on chronic alcoholic liver injury. According to Child - Pugh grading criteria, 11 cases (84.60%) had liver function grade B and C grade. The mean arterial oxygen pressure (PaO2) was 6.94 ± 1.5 kPa, and in 9 of 10 patients, the hypoxia was greater than 10%. Clinical manifestations were dyspnea (100%), cyanosis (100%), clubbing (84.6%), liver palpation (84.6%), facial telangiectasia (84.6%) and spider nevus (69.2%). Among the 9 routine pulmonary function tests, 8 were diffuse dysfunction. All patients were diagnosed by 99mrc labeled human albumin aggregates (99mTc-MAA) for the first time by pulmonary perfusion imaging. The intrapulmonary shunt rate was 22% -57%. In 11 cases, the percentage of arterial-venous shunt to total blood flow (Qs / Qt) was 10.9% -27.4% at the same time when 100% oxygen was inhaled, all less than the former. CONCLUSIONS: The incidence of hepatopulmonary syndrome is associated with hepatic insufficiency and portal hypertension, with diagnosis of portal hypertension, cyanosis, clubbing, spider nevus or facial telangiectasia. Self-standing hypoxia (> 10%) is a typical manifestation. 99mTc-MAA is the first effective method for lung perfusion imaging. The measured intrapulmonary shunt rate is greater than Qs / Qt.