肝肺综合征23例临床分析

目的提高对肝肺综合征(HPS)的临床认识,减少漏诊、误诊。方法回顾我院确诊的23例肝肺综合征病例,分析肝肺综合征的临床特点。结果肝肺综合征男、女发病比例为1.3∶1,平均年龄(42±21)岁,除1例急性重症肝炎外,其余22例均为肝硬化,门静脉高压发生率为82.6%。根据Child-Pugh分级标准,肝功能B、C级为78.2%。主要临床表现:呼吸困难发生率为91.3%,发绀为91.3%,肝掌为69.5%,杵状指(趾)为65.1%,面部血管扩张为56.5%,蜘蛛痣为56.5%。动脉血氧分压(PaO2)平均值为(50.8±14.1)mmHg(1mmHg=0.133kPa),直立性低氧血症发生率为85.7%(12/14),肺一氧化碳弥散量百分比(DLCO%)平均值为43.1%。39.1%(9/23)患者胸部X线检查表现为HPS而出现双下肺斑点状、小结节影或网状结节影。23例HPS均进行了99m锝-大颗粒聚合白蛋白(99mTc-MAA)核素首次通过肺灌注显像,平均分流率为36.3%。结论HPS主要发生于肝硬化Child-Pugh分级较重和(或)门静脉高压的患者。临床上对不能解释的低氧血症,同时有门静脉高压、蜘蛛痣(面部血管扩张)和(或)杵状指(趾)表现者应高度怀疑肝肺综合征。直立性低氧血症是HPS较特异性表现。HPS可有肺弥散功能降低、胸部影像学异常改变,但不具有特异性。99mTc-MAA核素首次通过肺灌注显像有助于确定肺血管扩张,明确HPS的诊断。

Clinical analysis of 23 cases of hepatopulmonary syndrome

OBJECTIVE: To describe the clinical features and to highlight the main criteria for diagnosis of hepatopulmonary syndrome (HPS). METHODS: Twenty-three patients with HPS were retrospectively investigated. RESULTS: Twenty-two cases had cirrhosis while one had acute hepatitis. The male to female ratio was 1.3:1, with an mean age of (42 +/- 21) years. 82.6% (19/23) of the patients were complicated with portal hypertension. The liver function of 78.2% of the cases was Child-Pugh grade B and C. The main clinical manifestations were dyspnea (91.3%), cyanosis (91.3%), liver palms (69.5%), clubbing fingers (65.1%), telangiectasis on face (56.5%), and spider angiomata (56.5%). The mean PaO(2) was (50.8 +/- 14.1) mm Hg (1 mm Hg = 0.133 kPa). Incidence of orthodeoxia was 85.7% (12/14). Mean diffusing capacity of lung function test was 43.1%. Chest X-ray was abnormal in 9 of the 39.1 (9/23) patients. Faint "mottled" shadows, nodular or reticulonodular opacities on bilateral lower zones were shown. All the 23 cases were diagnosed by radionuclide lung perfusion scanning technetium-labled macroaggregated albumin particles ((99m)Tc-MAA) and the mean shunt ratio was 36.3%. CONCLUSION: HPS is often associated with advanced Child-Pugh grade or portal hypertension. When "unexplained" hypoxaemia, portal hypertension, spider angiomata (or telangiectasis on face), or clubbing finger are present in these patients, HPS should be highly suspected. The presence of orthodeoxia is suggestive of HPS. (99m)Tc-MAA lung perfusion scanning is able to determine the presence of intrapulmonary shunting and the diagnosis of HPS.