21例肝肺综合征临床分析

肝肺综合征（hepatopulmonary syndrome，HPS）指慢性肝功能不全患者因肺内血管扩张而出现严重低氧血症，是各种慢性肝脏疾病终末期的一种严重并发症，预后差。HPS近年来引起广泛重视，其三联征为肝功能不全、肺血管扩张和低氧血型引。我院2003年5月至2008年5月对收治61例肝硬化患者进行血气分析等检测发现21例HPS,     

肝硬化和肝外门静脉阻塞病人肝肺综合征流行率

肝肺综合征 (ＨＰＳ)是发生于慢性肝病病人由肺外血管异常扩张所致的动脉低氧血症为表现的症候群 ,其基本特征为 :①有肝功能不全依据 ;②休息状态时有低氧血症 ;③肺内血管扩张(ＩＰＶＤｓ)。有关ＨＰＳ的研究多为西方肝硬化病人 ,本研究的目的则是评估一组印度肝硬化和肝外门静脉阻塞 (ＥＨＰＶＯ)病人的ＨＰＳ流行率、临床特点和风险因素。方法　 54例确诊为肝硬化 ,男 42、女 1 2例 ,平均年龄 ( 44 2± 1 3 )岁 ;50例确诊为ＥＨＰＶＯ ,男 3 1、女 1 9例 ,平均年龄 ( 2 3 3± 7 8)岁。作为本研究对象。凡有胸腔积液、影响呼吸的张力性…     

肝肺综合征

1概念肝肺综合征(HPS)是在排除原发性心肺疾病,慢性肝病基础上出现的一种严重并发症,主要表现3个方面:基础肝脏病;肺血管扩张;低氧血症。HPS实际上是基础肝脏病、肺血管扩张、低氧血症构成的三联体[1]。临床上以进行性呼吸困难、胸闷、憋气为突出表现的一系列病变,严重缺氧还会导致气促、胸痛、紫绀、杵状指(趾)[2],检查胸部无明显阳性体征,为慢性肝病的严重并发症。2病因及发病机制2.1病因HPS最常见于各种病因所致的肝硬化,也可发生于非硬化的门脉高压症[3],甚至是无肝硬化,无门脉高压的急、慢性病毒性肝炎[4,5]。2.2发病机制HPS的发…     

肝肺综合征和门静脉肺动脉高压

肝脏在维护人体健康和维持机体内环境方面起着重要的作用,患病肝脏将对人体多器官产生有害的作用.包括呼吸系统.终末期肝硬化患者或非肝硬化门脉高压患者会出现呼吸系统功能障碍,包括低氧血症、肺膨胀不全以及肝性胸腔积液等,并通过不同的致病机制影响肺血管系统,即可并发肝肺综合征和(或)门静脉肺动脉高压.肝肺综合征以肺血管扩张为特征,而门静脉肺动脉高压则是以肺内血管阻力增加为特征.肝病晚期患者体内血管活性物质平衡紊乱可能是两者发病机制之一,而决定两者发展的共同因素是门静脉高压,两者是本质不同的并发症,临床表现完全不同,现就两者的各自特点及相互关系作一综述.     

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肺功能不良是肝病的一个常见征象,常与肺实质损害、胸膜病变及肺循环异常有关.肺血管处于肝脏的"下游",肝硬化时对来自肠道的血管活性物质代谢异常或门脉高压均可导致肺循环的改变和损伤.肝肺综合征(hepatopulmonary syndrome,HPS)的发生主要与肺循环异常有关,定义为在肝病和(或)门静脉高压症进展的基础上出现肺血管扩张、低氧血症,临床上以进行性呼吸困难和低氧血症为主要表现.简言之可概括为3大主征:肝病、肺血管扩张、低氧血症.     

肝肺综合征的回顾与进展

肝肺综合征(hepatopulmonary syndrome,HPS)是指肝功能不全引起肺血管扩张、肺气体交换障碍导致的低氧血症及其一系列的病理生理变化和临床表现,多见于肝硬化患者。     

肝肺综合征

肝肺综合征 (Ｈｅｐａｔｏｐｕｌｍｏｎａｒｙｓｙｎｄｒｏｍｅ ,ＨＰＳ)是指在肝病基础上发生的肺血管异常 ,动脉氧合功能降低 ,临床出现低氧血症的功能性病变。肝病、肺泡 动脉血氧分压差 (ＡａＤＯ2 )上升和肺内血管扩张为该综合征的三大主征。随着原位肝移植(ＯＬＴ)的成功 ,晚期肝病患者的肺血管异常再次受到关注。ＨＰＳ是终末期肝脏病的一种严重肺部并发症 ,因此 ,加强对ＨＰＳ的临床研究 ,寻找有效的预防和治疗方法 ,对降低肝硬化的病死率有重要意义。一、概况ＨＰＳ早在 10 0多年前就已被注意到。 1884年Ｆｌｕｃｋｉｇｅｒ曾报道…     

肝肺综合征22例临床分析

肝肺综合征(HPS)是指肝功能不全引起肺血管扩张、肺气体交换障碍导致的低氧血症，多见于肝硬化者。1999～2002年，我院诊治HPS22例。现报告如下。     

肝硬化患者血氧水平的变化和意义

目的　探讨肝硬化合并低氧血症的发病机制、发生率及临床治疗效果。方法　用自动血气分析仪检测 2 0例正常者、5 0例肝硬化患者动脉血氧水平。结果　肝硬化组肺泡 动脉氧分压差 (AaDpO2 )显著高于对照组 ,动脉血氧分压 (PaO2 )明显低于对照组。肝性低氧血症的发生率是 5 4% ( 2 7/ 5 0 ) ,在肝功能ChildC级患者最为明显 ,肝性低氧血症与门脉高压、蜘蛛痣、杵状指 (趾 )密切相关。肝硬化ChildA级、B级患者常规治疗前后动脉血氧水平有显著差异。结论　肝硬化合并低氧血症与门脉高压和肺内血管扩张有关 ,检测动脉血氧水平有助于肝硬化预后判断和肝肺综合征(HPS)的诊断。相当一部分HPS患者的临床治疗效果欠佳。     

对肝肺综合征诊治的探讨

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。     

Nitric oxide metabolites,nitrative stress,and paraoxonase activity in hepatopulmonary syndrome

Aim. To investigate possible abnormalities of vasoactive compounds, nitrative stress, and antioxidant activity of paraoxonase (PONa) in human hepatopulmonary syndrome (HPS), we determined endothelin-1 (ET), nitric oxide (NOx) metabolites, PONa alongside crude plasma nitrotyrosine (NT) as surrogate marker of nitrative stress. Material and methods. Liver cirrhosis (LC) patients with HPS (n = 12) were matched by age, sex, and Child–Pugh score to LC patients without HPS (n = 15) and to healthy controls (CTR) (n = 15); plasma NO₂^? (nitrite) (vascular metabolite), NO₃^? (nitrate) (inflammatory metabolite), and PONa were determined by a colorimetric assay, ET, and NT by immunoassays. Results. HPS patients showed higher level of ET (p = 0.0002), NO₂^? (p = 0.002), NO₃^? (p = 0.0001), NT (p < 0.0001), and lower PONa (p = 0.0004) than CTR; post-hoc analysis revealed greater ET (p < 0.05) and NO₃^? (p < 0.005) in LC patients with HPS than in LC patients without HPS. NT correlated to Child–Pugh score within HPS (p = 0.04) and LC (p = 0.02). Conclusion. Our HPS patients are characterized by elevated plasma levels of ET and NOx metabolites and lower PONa. Reduced PONa alongside elevated NO₃^? and NT suggests that defective antioxidation may favor nitrative stress and both may be implicated in the pathogenesis of HPS.     

^（99）Tc^m-MAA全身显像在肝肺综合征诊断中的作用

目的 研究99Tcm-聚合白蛋白(99Tcm-MAA)全身显像在判断肝硬化患者肝肺综合征(HPS)中的作用.方法 74例受试者分为肝硬化组和正常对照组;肝硬化组分为HPS组和非HPS.全部受试者均行99Tcm-MAA全身显像,然后计算分流率.结果 ①肝硬化患者的分流率[(4.6±0.7)%]高于正常对照组的分流率[(2.7±0.8)%],差别有统计学意义.②HPS组中呼吸困难和杵状指的发生率高于非HPS组;HPS组与非HPS组相比,PaO2、血氧饱和度(SaO2)降低,PO2(A-a)增高.HPS组分流率[(7.1±0.9)%]与非HPS组分流率[(3.0±1.2)%]相比明显增高.③不同Child-pugh分级之间PaO2、SaO2、PO2(A-a)的差异有统计学意义;而不同Child-pugh分级之间分流率差异无统计学意义.结论 99Tcm-MAA全身显像是诊断HPS一种无创、准确检查方法.     

23例肝肺综合征临床分析

探讨肝肺综合征(HPS)的临床特点和预后。67例肝硬化患者均应用血气分析仪测定其动脉血氧分压(PaO2),其中Ch ild-Pugh分级A级30例,B级22例,C级15例。67例肝硬化患者中23例合并HPS,发病率为34.3%。其发病率与Ch ild-Pugh分级有关,与A级比较C级HPS,发病率显著增高(P<0.05);门静脉增宽与不宽者、胃底食管静脉曲张与未发现曲张者、有蜘蛛痣与无蜘蛛痣者分别合并有HPS,两者比较均差异有显著性(P<0.05)。23例HPS患者中7例死亡,HPS均不是直接死亡原因。有门脉高压、蜘蛛痣时提示HPS的存在。早期诊断和治疗有助于缓解低氧血症,改善预后。     

Hepatopulmonary syndrome and right ventricular diastolic functions: an echocardiographic examination

Aim: Liver functions are affected in the course of cardiac diseases, and similarly, liver diseases affect cardiac functions. Many studies in the literature have shown that left ventricular systolic and/or diastolic dysfunction may develop during chronic liver disease. However, there are limited studies investigating right ventricular functions during chronic liver diseases. Methods: A total of 84 patients who had no systolic and/or diastolic dysfunction in the left ventricle (LV) were evaluated; 46 patients with liver cirrhosis; 10 (21.74%) cirrhotic patients with hepatopulmonary syndrome (HPS) (group 1), 36 (78.26) cirrhotic patients without HPS (group 2), and 38 healthy individuals were treated as control. Results: Right ventricular diastolic dysfunction was determined in all patients of group 1 (100%), 26 of group 2 (72.22 %), and 4 of the controls (10.52%) (P < 0.05). Tricuspid deceleration time (dt) was significantly different between the groups (P < 0.05). In addition, right atrium (RA) diameters, right ventricle (RV) diameters, and RV wall thickness were significantly different between the groups (P < 0.05). Pulmonary artery pressure (P < 0.05) and pulmonary vascular resistance (P < 0.05) were also seen to be higher in group 1 than those in group 2 and control group. Conclusions: Right ventricular diastolic dysfunction rate is high in chronic liver diseases. In the presence of HPS, right ventricular diastolic dysfunction is more remarkable in patients than those without HPS. Right ventricular diastolic dysfunction may result in dilatation and hypertrophy in the right heart.     

Hepatopulmonary syndrome and portopulmonary hypertension: recent knowledge in pathogenesis and overview of clinical assessment

Hepatopulmonary syndrome and portopulmonary hypertension are cardiopulmonary complications, which are not infrequently seen in patients with liver disease and/or portal hypertension. These entities are both clinically and pathophysiologically different: the hepatopulmonary syndrome is characterized by abnormal pulmonary vasodilation and right‐to‐left shunting resulting in gas exchange abnormalities, whereas portopulmonary hypertension is caused by pulmonary artery vasoconstriction leading to hemodynamic failure. As both hepatopulmonary syndrome and portopulmonary hypertension are associated with significantly increased morbidity and mortality, and as these patients are commonly asymptomatic, all liver transplantation candidates should be actively screened for the presence of these two complications. The aim of is this review is to provide an overview on the hepatopulmonary syndrome and portopulmonary hypertension with primary focus on diagnosis and recent knowledge regarding pathogenesis and therapeutic targets.     

《2016年国际肝移植学会实践指南:肝肺综合征与门脉性肺动脉高压的诊断与管理》摘译

肝肺综合征(hepatopulmonary syndrome,HPS)和门脉性肺动脉高压(portopulmonary hypertension,POPH)是由肝实质或血管异常导致的两种不同的肺血管疾病.HPS及POPH对肝移植有重要的临床意义.2002年,欧洲呼吸学会肝肺疾病学组发布了此2种疾病的诊疗指南.国际肝移植学会基于上述指南制订了此实践指南.通过检索Medline数据库(1985-2015年)回顾了1000篇以上关于HPS及POPH的相关文献,基于个案报道、小宗病例报道、登记机构、数据库及专家意见形成了本临床指南.以上2种疾病均缺乏随机、对照的临床研究.本指南包括5个部分:定义及诊断标准、HPS、POPH、对肝移植的影响、对未来临床研究的建议.     

Echocardiographic detection of intrapulmonary shunting in a patient with hepatopulmonary syndrome: case report and review of the literature

Transthoracic echocardiography is a useful tool in the evaluation of patients with intrapulmonary and intracardiac shunts. We describe a case of a 49-year-old female with severe hypoxemia in the setting of aortic stenosis and cirrhosis of the liver. The use of agitated saline contrast during an echocardiography study helped to establish the diagnosis of intrapulmonary arteriovenous shunting consistent with the hepatopulmonary syndrome, thereby confirming the etiology of her symptoms and laboratory findings. This case report highlights the utility of echocardiography in diagnosing intrapulmonary shunts and assists in the understanding of the pathophysiology of hypoxemia in such patients.     

胆红素吸附联合血浆置换治疗肝肺综合征呼吸衰竭的临床观察

目的观察胆红素吸附联合血浆置换治疗肝肺综合征呼吸衰竭效果,探讨作用机制。方法 134例各种原因肝病合并肝肺综合征患者随机分为血液净化组和对照组,测定纤支镜肺灌洗液中胆红素、IL-6浓度,监测两组机械通气(MV)参数变化,血气分析监测氧代谢情况,评估血液净化对肝肺综合征合并呼吸功能衰竭的治疗效果。结果与对照组相比,血液净化组纤支镜肺灌洗胆红素浓度、IL-6浓度(P0.05),均明显下降,机械通气脱机时间早,时间短,呼吸机支持力度小(P0.05),氧分压升高而肺泡-动脉氧分压差下降(P0.05),肺功能明显改善。结论血液净化可有效治疗肝肺综合征改善肺功能,纠正呼吸衰竭,原因与清除肺组织局部胆红素沉积、炎症介质等因索有关。     

Two cases of hepatopulmonary syndrome with improved liver function following long-term oxygen therapy

Hepatopulmonary syndrome (HPS) is a complication of liver disease that is characterized by hypoxemia and intrapulmonary vascular dilatations. The only established therapy for this disorder is liver transplantation. Here, we report two patients (a 63-year-old woman and a 72-year-old man) with HPS associated with hepatitis C virus-related cirrhosis. We gave the patients low-dose oxygen supplementation to improve their respiratory symptoms. Surprisingly, their liver function improved from Child Pugh class C to class A, and ascites disappeared after a year of oxygen supplementation. We believe that long-term oxygen therapy contributed to the improvement of liver function in these two cases. Long-term oxygen therapy might offer a new therapeutic approach to improve liver function in patients with cirrhosis with hypoxemia.