门静脉高压相关性肺动脉高压1例

一、病例资料 患者,女性,47岁,于1个月前出现胸闷、气短、呼吸困难,活动后加重,伴头昏,休息后稍好转.4年前发现乙型肝炎表面抗原阳性,平时无纳差、厌油腻等症状,未予治疗.有月经过多引起的贫血史10年,2007年和2009年分别输血1次.入院查体:血压114/91 mm Hg(1 mm Hg=0.133 kPa),端坐呼吸.贫血貌,皮肤巩膜轻度黄染.双肺呼吸音清,无干湿啰音.心界稍扩大,心率80次/min,肺动脉区第二心音亢进,心脏各瓣膜区未闻及杂音.腹软,肝肋下未触及,脾肋下5cm,质中,移动性浊音阴性.双下肢轻度凹陷性水肿.     

门脉高压相关性肺动脉高压临床分析

目的探讨门脉高压相关性肺动脉高压(PoPH)的临床特点和经胸超声心动测量右心房线性指数同心功能或肝功能的关系。 方法选择首都医科大学附属北京安贞医院呼吸与危重症医学科2012年1月至2018年12月收治的门脉高压相关性肺动脉高压患者11例,回顾性分析11例患者的临床症状和体征、影像检查资料、实验室检查结果、右心导管检查测算数据、心肺功能测定结果、治疗和转归等。所有患者进行NHYA分级与mMRC量表评分。实验室检查结果包括血常规、生化、凝血、B型脑钠肽、动脉血气分析等,用以计算Child-Pugh及MELD等评分。 结果11例中男性5例,女性6例,肝硬化门脉高压者9例,非肝硬化门脉高压者2例,追溯病史发现肺动脉高压距离门脉高压诊断时间为0.5至19年,中位时间5年。NHYA分级与改良MRC呼吸困难(mMRC)评分存在正相关关系[r＝0.9608,95%CI(0.8460,0.9896), P<0.0001],与Child-Pugh评分无相关关系。右心房线性指数与BNP存在相关关系[r＝0.7930,95%CI(0.3684,0.9439),P＝0.0036],与终末期肝脏疾病评分(MELD)无相关关系。所有患者均存在不同程度的低氧血症,肺泡-动脉血氧分压差值较预计值升高(t＝3.669,P＝0.0018),具有显著性差异。就诊时已出现右心功能严重下降的患者预后差。 结论PoPH患者多以运动性呼吸困难为首发症状,与心功能严重程度相关,与肝功能无显著线性关系,临床随访不能只评价肝功能。随访中超声心动图中右心房径线的测量有助于心功能评估。尽早干预肺动脉压力的升高,有助于右心功能的保护,改善预后。     

特发性门静脉高压症1例报告并文献复习

特发性门静脉高压症（Idiopathic portal hypertension,IPH）是一种病因未明,以窦前性门脉压增高为主要特征,主要临床表现为门脉高压,显著脾脏肿大伴脾功能亢进,而肝功能基本正常的综合征。IPH临床少见,现将本科近期肝脏活检确诊的1例IPH,结合文献复习报道如下。     

系统性红斑狼疮并发肺动脉高压1例并文献复习

目的提高对系统性红斑狼疮（SLE）并发肺动脉高压（PHT）的发病机制、临床表现、治疗及预后的认识。方法对1例并发PHT的SLE病例进行分析和讨论,并结合相关文献复习。结果SLE并发PHT的机制尚不明确。其临床表现无特异性,早期症状多为呼吸困难。SLE患者出现雷诺征时,应高度怀疑PHT的存在。大剂量糖皮质激素联合免疫抑制剂可显著降低PHT。结论SLE并发PHT常提示预后不良,应尽早诊断及治疗。针对原发病的强化治疗可有效降低PHT。     

非肝硬化性门静脉高压2例报道并文献复习

门脉高压常见病因为各种原因引起的肝硬化,占门静脉高压的80％以上,非肝硬化性门脉高压仅占5％～10％[1],但目前随着诊断技术的提高,临床上非肝硬化性门脉高压日益受到重视,部分患者通过适当的治疗,预后较好.现将我科收治的2例非肝硬化性门脉高压临床及病理特点分析如下.     

特发性非肝硬化性门脉高压1例并文献复习

<正>特发性非肝硬化性门脉高压(idiopathic non-cirrhotic portal hypertension,INCPH)是一种血管性疾病,临床表现为门脉高压的一系列症候,但缺乏肝硬化的病理学表现,约占门脉高压症患者的10%。因缺乏相对特异的诊断标准,易误诊。现报道1例我科诊治的以脾功能亢进症为首发症状的、经13年就医路终确诊的INCPH患者,以期提高临床医师对该病的诊治水平。1病例摘要患者女,68岁。主因“发现脾肿大13年,间断腹泻8月”于2020年9月9日入院。患者于13年前因“急性胆囊炎、胆囊结石”于我院普外科行手术治疗,     

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.     

门脉高压性肺动脉高压研究进展

门脉高压性肺动脉高压是一种特殊类型的肺动脉高压,现从发病率、发病机制、临床特点、诊断及治疗等方面阐述门脉高压性肺动脉高压的研究进展。     

肺动脉高压靶向药物联合治疗门脉性肺高血压1例并文献综述

患者女,31 岁,因发现HBsAg阳性9年于2016年9月入院.患者2007年体检发现HBsAg阳性且ALT升高,当地医院给予普通干扰素联合恩替卡韦治疗1 年后患者自行停药.后间断复查肝功能示ALT异常,患者自行于外院服用中药治疗.本次入院前门诊复查ALT 118 U/L,自诉无腹胀、胸闷、憋气等不适,为进一步检查...     

Biliary stricture caused by portal biliopathy: case report and literature review

Portal biliopathy is a rare condition that is usually not diagnosed and only in few cases causes symptoms. Those symptoms are caused by vascular obstruction of the biliary tree in patients with portal hypertension. We report a case of a 29 years man who presented with history of intermittent jaundice, persistent elevation of hepatic function test and hematemesis as a manifestation of portal hypertension without liver damage. We present the clinical, radiological and pathological characteristics and literature review of the cases that had been reported, their diagnoses, treatment and clinical implication.     

Noncirrhotic portal hypertension associated with didanosine: a case report and literature review

Noncirrhotic portal hypertension (NCPH) has recently been reported as a liver complication in human immunodeficiency virus (HIV)-infected patients and has been found to be associated with exposure to didanosine. Here, we describe the case of an HIV-infected patient with portal hypertension who initially presented with massive ascites and portal vein thrombosis. The patient's HIV-1 infection was well-controlled with highly active antiretroviral therapy (lamivudine/didanosine plus nevirapine) for 3 years since its diagnosis in 2007. He had no history of alcoholism, drug abuse, or liver diseases. An extensive work-up for other possible causes of liver disease was performed, but the results were inconclusive. In addition to reporting this case, we have reviewed the literature on didanosine-related NCPH and analyzed the findings of 61 similar previously reported cases.     

A case of systemic sclerosis complicated by idiopathic portal hypertension: case report and literature review

We encountered a 62-year-old woman who had systemic sclerosis (SSc) complicated by idiopathic portal hypertension (IPH). She had a 10-year history of scleroderma and Raynaud's phenomenon. She also had pancytopenia, splenomegaly, and esophageal varices. Treatment with prednisolone and endoscopic variceal ligation resulted in improvement of her symptoms. According to our literature review, the prognosis of patients with SSc complicated by IPH is relatively poor. However, the factors that predict outcome of these patients have not been elucidated.     

由肾球旁细胞瘤导致的继发性高血压低血钾1例病例报道及文献复习

肾球旁细胞瘤(juxtaglomerular cell tumor,JGCT)是一种罕见的肾脏内分泌肿瘤,可引起高血压。肾球旁细胞功能障碍导致肾素过度表达,进而导致高血压、高肾素、继发性醛固酮增高、低血钾等临床症状[1],也有无症状的病例报告[2]。     

Thrombolytic therapy in patients with portal vein thrombosis: case report and review of the literature

A 29-year-old male patient with Crohn's disease of the terminal ileum and previous abdominal surgery was admitted because of severe abdominal pain and signs of bacterial sepsis. The diagnosis of portal vein thrombosis and multiple liver abscesses due to Streptococcus intermedius septicaemia was made and antibiotic therapy was instituted immediately. As high-dose heparin therapy was ineffective, urokinase was administered intravenously over a total of 7 days. Within 2 days, the patient's symptoms completely subsided. Colour duplex ultrasonography revealed complete recanalization of the main stem of the portal vein; the right branch of the portal vein, however, remained occluded. Other case reports on thrombolytic therapy in patients with portal vein thrombosis are reviewed.     

Meningitis caused by Abiotrophia defectiva: case report and literature review

The genus Abiotrophia comprises fastidious Gram-positive bacteria previously classified as nutritionally variant streptococci (NVS). The isolation of NVS from the central nervous system (CNS) is very rare. We describe a case of meningitis due to Abiotrophia defectiva in a patient who underwent a total hip arthroplasty 4 days previously. It is possible that the organism could be introduced through the spinal anesthesia. We also review all cases of CNS infections caused by NVS.