肝肺综合征研究现状与展望

肝肺综合征(HPS)是指在慢性肝病门脉高压的基础上肺内血管异常扩张诱导产生的动脉血氧合缺陷,气体交换障碍、低血氧血症等一系列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分流形成有关.本文对血液分流:肺内分流、门肺分流和胸膜分流;通气/血流比例失调;弥散障碍及血管活性物质:肺内一氧化氮、内皮素-1、肿瘤坏死因子-α和乙酮可可碱改变等因素在HPS中的作用机制进行了系统的综述,旨在为探讨HPS的发病机制、临床特征和治疗提供理论依据.     

肝肺综合征的诊治

肝肺综合征(HPS)是指慢性肝病或者急性肝衰竭患者因肺内血管扩张,阻力降低,使肺泡-小动脉氧梯度增大,气体交换障碍,导致通气和灌注不相适应所致的低氧血症,是各种慢性肝脏疾病终末期一种严重的并发症。HPS诊断的成立还应该具备:患者既往可能无肺部或心脏的基础疾病;无论是否存在低氧血症,氧分压降低都是HPS的可靠证据,且肺泡-小动脉内氧梯度增大(≥15mm Hg),年龄≥64岁者≥20mm Hg。当从卧位到直立体位变化时,氧值明显下降;肺内血管扩张和(或)动静脉分流。该病发病机制复杂,起病隐匿,症状缺乏特异性,临床上往往重视不够,早期诊断及治疗均较为困难。目前尚缺乏有效的治疗,远期预后不良,肝移植是目前惟一的根本性治疗方法,但也存在着免疫排斥及复发的可能。     

肝肺综合征诊治的研究进展

肝肺综合征(hepatopulmonary syndrome,HPS)是指以肝脏疾病为基础病因,以肺内血管扩张和肺内动静脉分流导致呼吸困难和低氧血症的疾病。近年来,尽管对HPS的病理生理和发病机制进行了大量研究,但目前仍无特效药物治疗,而肝移植仍然是唯一有效的治疗方法。本文重点介绍肝肺综合征的发病机理、临床表现、诊断、治疗以及最新的研究进展。     

肝肺综合征肝移植术后早期并发症的护理

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低氧血症及其一系列病理生理变化和临床表现。多见于肝硬化病人。HPS在肝硬化中的发病率为5％～29％，如不进行肝移植，病死率高达41％。有研究表明，HPS病人肺内血管扩张，肺内广泛右向左分流在移植术后数周内可持续存在，     

肝肺综合征的研究现状

肝肺综合征（hepatopulmonmy syndrome,HPS）是在慢性肝病和（或）门静脉高压基础上出现肺血管扩张、动脉血氧合功能异常,临床上以肝功能不全、肺血管扩张、进行性呼吸困难、低氧血症为主要表现,并具有慢性肝病、肺泡动脉血氧分压差[P（A-a）2]增大或动脉血氧分压（PaO2）降低和肺内血管扩张的临床三联症[1].HPS起病隐匿,临床症状不典型,且发病机制复杂,检查诊断困难,无有效治疗手段,导致其临床预后不良,本文就HPS的病因、发病机制、临床表现及诊断与治疗的研究进展作一综述,供临床诊疗参考.     

肝肺综合征肝移植术后早期并发症的护理

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低氧血症及其一系列病理生理变化和临床表现,多见于肝硬化病人.     

肝肺综合征的诊断和治疗

肝肺综合征 (hepatopulm onary syndrome,HPS)表现为慢性肝病 ,低氧血症和肺内血管扩张三联征。首先是由Kennedy等 [1 ]于 1977年正式提出的。是慢性肝病终末期的一种并发症 ,可见于各种原发和继发性肝损害 ,但也可见于非肝硬变性门脉高压。一般认为 ,在上述疾病基础上 ,血管活性介质 (如一氧化氮和内皮素等 )的改变引起肺内血管扩张 ,导致肺的低通气 /灌住 (灌注量多于通气量 )和解剖性分流 (动静脉交通 ) ,最终表现为低氧血症 [2 ]。虽然其病理生理学尚不明确 ,也缺乏有效的药物治疗 ,但其诊断方法的进一步完善和肝移植治疗的良好前景引…     

肝肺综合征

近年人们逐渐认识了一种肝病的严重并发症——肝肺综合征 (hepatopulm onary syndrome,HPS)。此征发病率高 ,治疗相当困难 ,远期预后不良。因此 ,对肝肺综合征的早期诊断和及时治疗将有利于改善患者的预后 ,本文对本征做一综述。1　概况HPS是指肝病时发生肺血管异常 ,动脉氧合功能降低 ,出现低氧血症的功能性病变。1988年 Eriksson等首先将肝硬变患者伴有肺内分流和肺内灌注障碍引起的低氧血症 ,命名为功能性肝肺综合征。 1989年 Sherlock将肝脏疾病所引起的各种肺部变化 ,统称为肝肺综合征。这些肺部病变包括低氧血症、肺内动静脉分流…     

肝肺综合征

肝肺综合征(Hepato Pulmonary Syndrome,HPS)是近年来才被逐渐认识的一组临床综合征。系指继发于慢性肝脏疾病(肝功能不全)基础上的肺血管扩张,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。HPS早期容易被忽视,常可加重肝病患者的病情,使预后变差。     

肝肺综合征63例临床回顾性分析

肝肺综合征 (Hepatopulmonarysyndrome ,HPS)是指肝病终末期发生的肺血管异常引起的血氧合功能障碍 ,为肝功能不全、肺血管扩张和低氧血症综合征[1 ] 。本文根据我院近几年来收集的 63例肝肺综合征的临床资料进行回顾性分析 ,以探讨肝肺综合征的临床特点和预后 ,为临床诊治提供     

Hepatopulmonary syndrome.

The hepatopulmonary syndrome (HPS) is a rare complication of liver cirrhosis and is characterised by the typical triad of liver cirrhosis, arterial hypoxemia, and intrapulmonary vascular dilatation. Except for pleural effusions associated with liver cirrhosis no other disease of the lungs or the heart is detectable. The structural hallmark of HPS is dilatation of pulmonary precapillary vessels which impairs diffusion-perfusion and causes unequal ventilation-perfusion. The diagnosis of HPS is based on PaO2 measurements when breathing room air and 100% oxygen. The increased intrapulmonary vascular diameter allows microbubbles to traverse the lung capillaries when agitated saline is administered intravenously. Only on rare occasions is a patient limited by his pulmonary impairment, the leading morbidity is that of liver disease and its classical complications. Drug therapy is of no proven benefit, oxygen supplementation might improve dyspnea. Vascular embolisation of discrete arteriovenous shunts, if present, or liver transplantation may dramatically improve pulmonary function in selected patients.     

对比增强超声心动图对拟肝移植患者肺内分流的评估价值

背景：晚期肝病患者的肺内血管异常可以导致肺内右向左分流及严重低氧血症，目前国内对筛查肺内血管扩张尚缺乏一种简便、敏感、有效的方法。 目的：评估对比增强超声心动图对晚期肝病肺内分流的临床诊断价值。 设计、时间及地点：前瞻性病例对比观察，于2004-02／2006-02在解放军第四五八医院肝病中心完成。 对象：解放军第四五八医院肝病中心收治的男性拟行肝移植的晚期肝病患者24例。 方法：在无任何血管扩张药治疗情况下例行常规检查。采用对比增强超声心动图筛查晚期肝病患者肺内右向左分流的发生率，并根据左心室微泡的显示程度半定量分析记录为1^＋～3^＋。将患者分为经超声心动图证实有肺内分流组和无肺内分流组。 主要观察指标：拟行肝移植的晚期肝病患者肺内右向左分流的发生率及临床特点。结果：①24例患者中10例（41．7％）经对比增强超声心动图证实肺内右向左分流，左心室显影异常程度1^＋～2^＋，其中6例1^＋，4例2^＋，出现在右心室显影后6～10余个心动周期。②两组患者的年龄、性别、动脉血气分析指标、肝功能指标差异无显著性意义（P〉0．05）。③经超声心动图证实肺内分流组患者的上消化道出血发生率、门脉高压指征脾脏厚度及右心功能参数肺动脉收缩压、Tei指数均高于无肺内分流组（P〈0．05～0．01）。 结论：晚期肝病合并肺内分流而无低氧血症时肺血管扩张比较常见，对比增强超声心动图为诊断肺内血管扩张提供了一种敏感、非创伤的早期检查手段。门脉高压症是发生肺内血管扩张的主要因素，右心室Tei指数可作为评估肺内血管扩张患者右心功能的重要参数。     

Successful treatment of adult-onset still disease caused by pulmonary infection-associated hemophagocytic lymphohistiocytosis: A case report

BACKGROUND Adult-onset still disease(AOSD) and hemophagocytic syndrome(HPS) are two inflammatory diseases with very similar clinical manifestations. HPS is one of the most serious complications of AOSD and its risk of death is very high. It is difficult to identify HPS early in patients with AOSD, but early identification and proper treatment directly affects the prognosis.CASE SUMMARY A 39-year-old male showed a high spiking fever and myalgia. Laboratory data revealed elevated white blood cell, serum ferritin, and neutrophil percentage.However, his fever failed to relieve after a clear diagnosis of AOSD caused by pulmonary infection and treatment by antibiotics and corticosteroids;further laboratory data showed elevated serum ferritin, C-reactive protein, erythrocyte sedimentation rate and triglyceride, as well as liver abnormalities. Bone marrow smear showed hemophagocytosis. Secondary HPS was definitely diagnosed. The high fever disappeared and the laboratory findings returned to normal values after treatment by high-dose intravenous methylprednisolone and methotrexate.CONCLUSION For AOSD patients with high suspicion of HPS, active examination needs to be considered for early diagnosis, and timely using of adequate amount of corticosteroids is the key to reducing risk of HPS death.     

Severe hypoxemia associated with liver disease: Mayo Clinic experience and the experimental use of almitrine bismesylate

Severe hypoxemia associated with chronic liver disease is an uncommon disorder most likely due to an intrapulmonary vascular abnormality that has characteristics of both ventilation-perfusion mismatching and diffusion limitation. Anatomically, the intrapulmonary vascular abnormalities can occasionally be detected by angiography. Physiologically, the gas exchange abnormalities can be substantiated by contrast-enhanced two-dimensional echocardiography. Although orthodeoxia and platypnea have frequently been found in these patients, echocardiographic data suggest that vascular abnormalities can exist in the absence of orthodeoxia. We describe 11 patients who had severe hypoxemia and chronic liver disease and review their pulmonary angiographic, contrast echocardiographic, and arterial blood gas findings. Among five of these patients who were given almitrine bismesylate, an experimental medication thought to alter ventilation-perfusion relationships in patients with chronic obstructive pulmonary disease, one had improved oxygenation. We recommend that patients with hypoxemia associated with chronic liver disease have detailed studies to rule out reversible forms of hypoxemia and that those with severe hypoxemia undergo testing to determine the existence of intrapulmonary vascular abnormalities, especially if liver transplantation is considered.     

Pulmonary aspects of chronic liver disease and liver transplantation

A vast spectrum of pulmonary pathologic conditions occurs in association with chronic liver diseases, and clinically important manifestations, such as arterial hypoxemia, can result. Both pulmonary vascular and parenchymal abnormalities can contribute to the dysfunction, as evidenced by results of pulmonary function tests and gas exchange studies. The clinical implications of identifying such pulmonary problems range from alleviation of symptoms, especially dyspnea, to comprehensive assessment of patients before and after liver transplantation. Physicians should be aware of these potential pulmonary disorders that can complicate liver disease and liver transplantation so that management of affected patients can be improved.     

Essential echocardiographic evaluation in patients with suspected pulmonary hypertension: an overview for the practicing physician

Prompt and accurate diagnosis of patients presenting with symptoms suggestive of pulmonary arterial hypertension (PAH) is of outmost importance as delays in identifying this clinical entity have detrimental effects on both morbidity and mortality. Initial noninvasive assessment of these patients has traditionally included a number of routine tests of which transthoracic echocardiography (TTE) has been shown to either confirm the presence of structural anomalies of the right ventricle (RV) indicative of PAH or exclude other potential causes of pulmonary hypertension (PH). Consequently, TTE has become a well-validated and readily available imaging tool not only used for this initial screening but also for routine follow-up of PH patients. Since chronic PH is known to unbalance the normal hemodynamic and mechanical homeostatic interaction between the RV and pulmonary circulation; the resulting response is that of an abnormal RV remodeling, clinically translated into progressive RV hypertrophy and dilatation. An enlarged and hypertrophied RV not only would eventually lose effective contractility but also this gradual decline in RV systolic function is the main abnormality in determining adverse clinical outcomes. Therefore, it is of outmost importance that TTE examination be comprehensive but most importantly accurate and reproducible. This review aims to highlight the most important objective measures that can be routinely employed, without added complexity, that will certainly enhance the interpretation and advance our understanding of the hemodynamic and mechanical abnormalities that PH exerts on the RV.     

前列地尔注射液治疗小儿高原性心脏病的疗效观察

目的：探讨前列地尔注射液微泵给药治疗小儿高原性心脏病（ high altitude heart disease ,HAHD）的效果和安全性。方法60例HAHD患儿按照完全随机设计法分为观察组和对照组各30例,两组均给予常规治疗,观察组在此基础上以微泵静脉持续给予前列地尔注射液[5 ng/（kg＆#183; h）],连续应用3天,每天12小时。比较两组治疗后右室射血分数（RVEF）、肺动脉收缩压（ SPAP）、动脉血氧分压（ PaO2）、动脉血二氧化碳分压（ PaCO2）等血气指标变化和临床疗效总有效率。结果观察组治疗后RVEF、SPAP、PaO2、PaCO2指标改善均明显优于对照组,临床总有效率高于对照组,差异有统计学意义（ P ＜0.05）。结论常规治疗基础上加用前列地尔注射液治疗小儿HAHD可明显增强疗效,改善心功能,降低肺动脉压力,纠正缺氧状态。     

慢性高原病主肺动脉扩张的病理生理及影像诊断

慢性高原病(chronic mountain sickness,CMS)患者主肺动脉(main pulmonary artery,MPA)扩张发生发展的核心和关键是慢性高原低压性缺氧,其次是寒冷。且两者的复合作用可增强其对心血管系统的影响。影像学检查不仅可以有效地诊断MPA扩张,还能评价其与肺动脉高压的关系以及评估其对心脏的影响。在诸多的检查中,MRI占有不可替代的位置。作者主要针对CMS患者MPA扩张的发病机理、临床意义及影像诊断进行综述。     

鼻NK/T细胞淋巴瘤合并噬血细胞综合征临床分析

目的探讨鼻NK/T细胞淋巴瘤合并噬血细胞综合征(HPS)的临床特征、治疗方法及预后。方法对4例鼻NK/T细胞淋巴瘤合并HPS患者的临床资料进行回顾性分析。结果 4例鼻NK/T细胞淋巴瘤患者符合HPS诊断标准,初诊时具有多项淋巴瘤相关不良预后因素1,例以HPS为首发症状,3例发生HPS时处在疾病进展期,3例骨髓检查均发现淋巴瘤细胞浸润。合并HPS后病情进展迅速,最明显症状是发热、血象进行性下降、纤维蛋白原降低、血清铁蛋白升高、骨髓中出现噬血现象。给予HLH-2004为基础的方案结合化疗后,HPS均有不同程度改善,但由于原发病无法控制,HPS很快复发,患者合并肝功能异常、凝血异常或弥散性血管内凝血,最终死亡。结论鼻NK/T细胞淋巴瘤合并HPS时预后差,常发生在淋巴瘤复发进展期或终末阶段。HLH-2004为基础的方案联合化疗有希望逆转病情,延缓疾病进展,为原发病治疗创造机会。