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。     

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。     

肝肺综合征

肝肺综合征是指肝功能不全患者肺血管异常、动脉氧合功能降低及低氧血症等所致的一组临床症状。本文对肝肺综合征的病因、发病机理、临床表现、诊断和治疗进行综述。     

肝肺综合征19例临床分析

肝肺综合征(hepatopulmonary syndrone，HPS)是指由肝脏疾病导致肺血管异常，动脉氧合功能降低和低氧血症等一系列临床表现的综合征。本文通过19例肝硬化患者临床分析，探讨其发生机制、诊断和治疗。     

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…     

对肝肺综合征诊治的76例临床分析

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76例HPS患者的临床资料,并与无HPS的133例肝硬化患者在肺功能、血气分析等方面进行比较。结果:在HPS组中除4例为慢性酒精性肝损害基础上的急性药物性肝炎外,72例均为肝硬化患者。临床表现为呼吸困难、紫绀、杵状指及蜘蛛痣。据Child-Pugh分级标准,肝功能B级和C级者59例(77.6%),平均PaO2为(79.93±11.29)mm Hg(1 mm Hg=0.133 kPa),对照组为(92.48±13.53)mm Hg,2组比较有统计学意义(P0.01)。HPS组中76例中有48例直立性缺氧10%(63.1%)。不同门静脉内径、有无蜘蛛痣、不同肝功能分级的PaO2和SaO2值比较,均有统计学意义(P0.01)。影像学检查结果示肺内血管扩张。结论:肝肺综合征的发生与肝功能不全有明显的关系,该病患者常有呼吸困难、紫绀、杵状指、蜘蛛痣及直立性缺氧等典型表现,患者低氧血症发生率高,而肺功能存在弥散功能障碍,肺血管有明显的扩张。     

肝肺综合征的诊断及治疗研究进展

肝肺综合征是一种与肝病有关的肺血管扩张所致的临床综合征,以动脉氧合障碍为主要表现,是肝脏疾病的肺部严重并发症.其发病机制复杂,发病隐匿,晚期肝病发病率高,目前临床重视不足,远期预后较差,故临床医生需加强对其认识,早期发现,早期诊治,以便改善预后.此文主要对近年来肝肺综合征的诊断及治疗的最新进展进行综述.     

肝肺综合征的诊断和治疗

肝肺综合征是指继发于肝脏疾病而发生的肺血管扩张、肺部气体交换障碍、动脉血氧分压下降并出现有关临床表现的综合征。早期发现并及时处理有利于改善预后。除临床症状和体征外，血气分析可见直立性动脉血氧分压下降；超声波、放射性核素及肺血管造影等检查有助于发现肺内扩张的血管。氧疗简便而有效；药物治疗尚无确实效果；肺血管栓塞疗法可能有效；肝移植后大部分病人动脉血氧分压可明显提高，故肝肺综合征有严重低氧血症不再是肝     

肝肺综合征发病机制研究进展

肝肺综合征 (HPS)是指肝功能不全引起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 ,目前认为HPS主要与肺内毛细血管扩张和形成分流有关。本文对一氧化氮、内皮素 1、血红素氧化酶和一氧化碳等因素在肝肺综合征血管病变中的作用机制进行了系统地综述 ,旨在为探讨HPS的发病机制和临床治疗提供理论依据。     

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.     

Clinical and haemodynamic aspects of hepatopulmonary syndrome in Indian patients with cirrhosis

BACKGROUND: Hepatopulmonary syndrome consists of the triad of hepatic dysfunction and/or portal hypertension, intrapulmonary vascular dilatation and hypoxaemia, in the absence of detectable primary cardiopulmonary diseases. In the present study, we examined the frequency of hepatopulmonary syndrome among Indian patients with cirrhosis, and studied clinical predictors and pulmonary haemodynamic alterations. METHODS: Forty-five patients with cirrhosis and no cardiopulmonary diseases were investigated by air-contrast echocardiography. Where patients were positive, arterial blood gas analysis was carried out. Positive contrast echocardiography with PO2 < 70 mmHg confirmed the diagnosis of hepatopulmonary syndrome. Three cases with the syndrome and 24 without were assessed for haemodynamic status by hepatic and pulmonary catheterization. RESULTS: Four of 45 cases of cirrhosis (8.9%) had positive contrast echocardiographies, including three (6.7%) with hepatopulmonary syndrome and one 'subclinical' case (positive contrast echocardiography without hypoxaemia). Under haemodynamic study, the mean pulmonary arterial and pulmonary capillary wedge pressures appeared to be lower among those patients with hepatopulmonary syndrome. CONCLUSIONS: In this small study, the frequency of hepatopulmonary syndrome was relatively low (6.7%). Cyanosis was the only reliable clinical indicator, and there was no clear relationship with the severity of cirrhosis by Child's grading.     

The hepatopulmonary syndrome

The hepatopulmonary syndrome is a triad of liver disease, increased alveolar-arterial oxygen gradient and intrapulmonary vascular dilatations. Manifestations include orthodeoxia, platypnoea and hyperdynamic circulation. Intrapulmonary vascular abnormalities, perhaps mediated by nitric oxide, cause hypoxaemia by shunting, a perfusion-diffusion defect, and ventilation-perfusion mismatching. Contrast-enhanced echocardiography is the method of choice for demonstrating pulmonary vascular abnormalities, although perfusion lung scanning is a more specific and sensitive test. Angiography is best reserved for patients with poor response to 100% oxygen and defines whether vascular dilatations are of the diffuse 'spongy' type or, less commonly, discrete arteriovenous communications amenable to embolization. About 80% of patients with the hepatopulmonary syndrome eventually have improved oxygenation after liver transplantation, thereby making worsening hypoxaemia the primary indication for transplantation in many instances. Nevertheless, severe hypoxaemia carries a peri-operative mortality of 30% and reliable predictors of successful outcome after transplantation remain to be determined.     

Prognostic significance of the hepatopulmonary syndrome in patients with cirrhosis

BACKGROUND AND AIMS: The hepatopulmonary syndrome (HPS) has been defined by chronic liver disease, arterial deoxygenation, and widespread intrapulmonary vasodilation. Mortality of patients with HPS is considered to be high, but the effect of HPS on survival in patients with cirrhosis remains unclear. METHODS: A total of 111 patients with cirrhosis were studied prospectively by using transthoracic contrast echocardiography for detection of pulmonary vasodilation, blood gas analysis, and pulmonary function test. Twenty different clinical characteristics and survival times were noted. RESULTS: Twenty-seven patients (24%) had HPS. Their mortality was significantly higher (median survival, 10.6 months) compared with patients without HPS (40.8 mo, P < 0.05), even after adjusting for liver disease severity (2.9 vs. 14.7 months in Child-Pugh class C with [n = 15] and without HPS [n = 35, P < 0.05]; 35.3 vs. 44.5 months in Child-Pugh class B with [n = 7] and without HPS [n = 23, P = NS]), and exclusion of patients who underwent liver transplantation during follow-up (median survival 4.8 vs. 35.2 months, P = 0.005). Causes of death were mainly nonpulmonary and liver-related in the 19 patients with and the 35 patients without HPS who died. In multivariate analysis, HPS was an independent predictor of survival besides age, Child-Pugh class, and blood urea nitrogen. Mortality correlates with severity of HPS. CONCLUSIONS: The presence of HPS independently worsens prognosis of patients with cirrhosis. This should influence patient management and scoring systems and accelerate the evaluation process for liver transplantation.     

Progress in investigating the pathogenesis of hepatopulmonary syndrome

BACKGROUND: The pathogenesis of hepatopulmonary syndrome is complicated and remains unknown. This review aims to provide an updated knowledge about the pathogenesis of the syndrome. DATA SOURCES: Five medical databases, MEDLINE, Science-Direct, OVID, Springer Link, and Wiley InterScience were searched for articles on hepatopulmonary syndrome, cirrhosis, angiogenesis, intestinal endotoxemia, nitric oxide, carbon monoxide, and other related subjects. RESULTS: Currently, imbalance between vasodilation and vaso...     

Successful resolution of very severe hepatopulmonary syndrome following adult‐to‐adult living donor liver transplantation: Report of two cases

Hepatopulmonary syndrome (HPS) is a severe complication in patients with chronic liver disease with poor prognosis. Liver transplantation (LT) is a promising treatment for HPS; however, very severe HPS, which is defined by an arterial oxygen pressure (PaO₂) of less than 50 mmHg and a right–left intrapulmonary shunt rate of more than 20%, may be a contraindication to LT, including living donor LT (LDLT). Here, we report two cases of decompensated liver cirrhosis with very severe HPS which were resolved after adult‐to‐adult LDLT including ABO‐incompatible LDLT. Both patients required oxygen supportive therapy in combination with specialized respiratory care postoperatively, followed by improvement of oxygenation and substantial decreases of intrapulmonary shunt rate. These findings suggest very severe HPS can be resolved by LDLT, including ABO‐incompatible LDLT, and reduced graft volume did not impede the reversal of intrapulmonary shunting. Our current report may indicate that adult‐to‐adult LDLT, including ABO‐incompatible LDLT, is becoming an effective therapeutic method and prompt a review of previous reports as well as our own files with particular regard to the indication of LDLT for decompensated liver cirrhosis with very severe HPS.     

Cirrhotic rats with bacterial translocation have higher incidence and severity of hepatopulmonary syndrome

BACKGROUND: Bacterial translocation, that is, extra-intestinal dissemination of gut bacteria, occurs in approximately 50% of humans and rats with cirrhosis and plays a significant role in enhanced tumor necrosis factor-alpha (TNF-alpha) production. The authors' previous studies have indicated that prevention of bacterial translocation with norfloxacine or inhibition of TNF-alpha with pentoxifylline treatment decreased both the incidence and severity of hepatopulmonary syndrome by attenuating the induction of pulmonary intravascular macrophages in cirrhotic rats. In the present study the hypothesis was tested that the cirrhotic rats with bacterial translocation had higher TNF-alpha production, higher level of sequestration of macrophages in pulmonary vessels, and increased incidence and severity of hepatopulmonary syndrome. METHODS: Rats were studied 5 weeks after common bile duct ligation or sham operation. Bacterial translocation was defined by positive mesenteric lymph node cultures. Hepatopulmonary syndrome was assessed by measurements of alveoloarterial oxygen difference (AaPO(2)) and intrapulmonary shunt. The TNF-alpha concentration in plasma was measured by ELISA. Pulmonary intravascular macrophage sequestration was assessed by lung morphometric analysis. RESULTS: Bacterial translocation occurred in 48% of cirrhotic rats. Plasma concentrations of TNF-alpha and the percentage of vessels with pulmonary intravascular macrophages were higher in the cirrhotic rats with bacterial translocation. Rats with bacterial translocation also had a higher incidence (9% vs 63%, P < 0.01) and severity of hepatopulmonary syndrome, as indicated by higher levels of both AaPO(2) and intrapulmonary shunt. CONCLUSIONS: These results suggest that bacterial translocation may play a role in the pathogenesis of hepatopulmonary syndrome by inducing pulmonary intravascular macrophages through TNF-alpha upregulation.     

Hepatopulmonary syndrome in noncirrhotic portal hypertensive patients

Hepatopulmonary syndrome has yet not been sufficiently assessed in noncirrhotic portal hypertension. The prevalence of hepatopulmonary syndrome was determined in 31 consecutive patients with noncirrhotic portal hypertension (19 idiopathic portal hypertension, 7 portal vein thrombosis, 5 congenital hepatic fibrosis) and 46 patients with liver cirrhosis. Contrast echocardiography was carried out in all patients. Macroaggregated albumin lung perfusion scans were performed in patients with positive contrast echocardiogram. Hepatopulmonary syndrome was detected in 5 (10.8%) cirrhotic and 3 (9.7%) noncirrhotic portal hypertensive patients (2 idiopathic portal hypertension, 1 portal vein thrombosis). All patients with hepatopulmonary syndrome had an increased shunt fraction (13–62%) and a decreased diffusion capacity of carbon monoxide (40–79%), and 7 of them were hypoxemic (P_aO₂, 31.6–69.8 mm Hg). These findings show that hepatopulmonary syndrome may occur in both liver cirrhosis and noncirrhotic portal hypertension and that portal hypertension is the predominant etiopathogenic factor related to hepatopulmonary syndrome.