肝窦阻塞综合征八例临床分析

目的探讨肝窦阻塞综合征（SOS）的临床诊治方法。方法回顾性分析8例SOS的临床资料,并进行随访。结果8例SOS主要临床表现为腹胀（8例）、肝区疼痛（7例）、腹水征（8例）及肝肿大（7例）等。8例的肝功能损害程度大多较轻,血清-腹水白蛋白梯度均大于11．1g／L,血清与腹水CA125均显著升高。超声检查均见肝脏肿大、胆囊壁水肿或增厚、门静脉增宽且流速缓慢、肝静脉变细以及腹腔积液等;MRI表现为门脉期及延迟期肝实质不均匀片状强化,肝叶、段静脉腔内造影剂充盈不良。经皮肝穿刺活检均见肝窦扩张、淤血及肝细胞变性、坏死,3例发现小静脉管腔狭窄、管壁增厚伴纤维组织增生。8例中1例行肝移植术后痊愈,4例经内科治疗后逐渐康复,3例死亡。结论SOS的临床表现以突出的门脉高压症为特点,CA125常显著升高,超声及MRI对本病的诊断与鉴别诊断有重大价值,而经皮肝穿刺活检的价值有限,联合应用影像学方法与病理活检可提高诊断正确率。早期应用抗凝药物是治疗本病的关键,严重病例可行肝移植术。     

吡咯烷生物碱导致的肝窦阻塞综合征的MRI特点

[目的]探讨吡咯烷生物碱导致的肝窦阻塞综合征的MRI特点。[方法]回顾性分析2010-09——2017-12期间共43例吡咯烷生物碱导致的肝窦阻塞综合征患者的基本信息、临床资料及MRI资料;MRI资料由2位经验丰富的影像科医师采用独立盲法阅片获得。[结果]吡咯烷生物碱导致的肝窦阻塞综合征患者的MRI特点为:平扫期腹水43例,肝肿大28例,胆囊壁水肿39例,门静脉区水肿41例,胸腔积液24例,脾大10例;门静脉期43例患者均可见肝脏不均一低信号病变,其中32例表现为沿肝静脉分布的"爪形"强化;平衡期27例肝右静脉狭窄,16例肝右静脉显影不清,42例下腔静脉狭窄。[结论]吡咯烷生物碱导致的肝窦阻塞综合征具有特征性的MRI表现,因此MRI可以为吡咯烷生物碱导致的肝窦阻塞综合征提供一种有效的诊断方法。     

肝窦阻塞综合征1例

正患者男,65岁,因"腹胀、右上腹痛1个月",于2016年10月1日入潍坊市阳光融和医院。曾在当地医院就诊,生化检查(2016年9月3日):总胆红素55.6μmol/L,直接胆红素15.7μmol/L,丙氨酸氨基转移酶1134 U/L,天门冬氨酸氨基转移酶826 U/L,血清白蛋白31.5 g/L。凝血五项:凝血酶原时间14.2 s,国际标准化比值1.22,活化部分凝血活     

土三七导致肝窦阻塞综合征20例

目的分析土三七导致肝窦阻塞综合征(SOS)患者的临床特点及诊治方法。方法回顾性分析2011年11月至2015年12月收治的20例服用土三七所致肝窦阻塞综合征患者的临床诊治资料。结果 20例患者中以老年人居多,大多数在服药的4个月内发病,均以腹胀为首发表现,所有患者肝脏CT或MRI检查均有"地图样"改变,肝静脉变细或不显示,1例患者出现肝肿大且无腹水,余19例均有腹水,有饮酒史和无饮酒史患者的肝损伤未见显著差异。20例患者中,1例治愈,7例好转,其中有2例是行TIPS治疗后好转。结论土三七可导致SOS的发生,肝脏CT或MRI检查有特征性的表现,早期抗凝、改善微循环治疗有一定疗效,应重视土三七的肝毒性。     

16例吡咯烷生物碱相关肝窦阻塞综合征的临床病理分析

目的观察吡咯烷生物碱(PA)植物致肝窦阻塞综合征(HSOS)患者的肝穿刺活体组织检查(简称活检)病理学表现。方法选取2012年至2017年诊断为PA-HSOS患者,收集患者一般情况、肝功能指标、服药史、肝穿刺活检时间、肝穿刺病理组织切片、起病6个月后的临床预后等资料。用临床资料进行临床分期;观察不同临床分期患者的病理组织学表现。对数据采用Wilcoxin符号秩和检验、非配对t检验、一元线性回归分析。结果收集到16例患者,起病、随访6个月后丙氨酸转氨酶水平分别为59.25 U/L和25.50 U/L、天冬氨酸转氨酶分别为108 U/L和45 U/L,差异均有统计学意义。而总胆汁酸分别为35μmol/L和36.15μmol/L,白蛋白分别为32.45 g/L和31 g/L,差异无统计学意义。将PA-HSOS病理发展过程分为早期、中期、晚期。早期小叶中心带窦内皮完整性受损,红细胞进入窦内皮与肝细胞之间的窦周间隙。中期出血带内红细胞溶解,网状纤维塌陷密集,胶原纤维沉积,尚有血流的腔隙充血扩张,其腔内覆有窦内皮细胞;出血带周围肝板出现再生现象,部分肝窦代偿性扩张。晚期出血带内胶原沉积形成大面积纤维瘢痕,其内多数有血流的扩张腔隙内覆血管内皮;边缘带肝细胞呈双排再生,插入纤维间隔。肝穿刺组织内重度出血损伤的肝小叶比例和患者预后无关。结论PA-HSOS早期小叶中心带红细胞通过受损的窦内皮进入窦周间隙,表现为肝板出血性坏死。至中晚期出现肝板再生和血管改造,故大部分患者临床病程呈自限性。病理分期和肝穿刺活检时间具有明显相关性,但无法根据标本出血损伤程度判断患者预后。     

老年肝窦阻塞综合征研究进展

<正>肝窦阻塞综合征（hepatic sinusoidal obstruction syndrome,HSOS）临床比较少见,常见于骨髓造血干细胞移植（hematopoietic stem cell transplantation,HSCT）后或是急性髓系白血病化疗后,也可由食用含吡咯生物碱（pyrrolidine alkaloids,PAs）的植物如土三七而引起。在中国,HSOS病因主要与食用土三七等含吡咯生物碱的中草药或中成药制剂有关。吡咯生物碱（PAs）是一种天然的植物毒素,食用含有PAs的中草药或制剂后发生的HSOS,称为吡咯生物碱相关肝窦阻塞综合征（pyrrolidine alkaloid-related HSOS,PA-HSOS）。     

吡咯生物碱相关肝窦阻塞综合征研究进展

肝窦阻塞综合征( hepatic sinusoidal obstruction syndrome,HSOS)是一种以肝血窦、肝小静脉和小叶间静脉内皮细胞为主要靶点的血管性药物性肝损伤( drug-induced liver injury,DILI).在大多数情况下,急性门静脉高压是其典型的临床特征[1].     

土三七外敷导致肝窦阻塞综合征1例

患者:男性,50岁,因乏力伴左下肢胫前溃疡1年于2016年10月入住我院。患者于1年前无明显诱因出现乏力,伴左下肢胫前溃疡、皮肤色素沉着、水肿、疼痛、瘙痒,2个月后出现面部瘙痒、水肿,于当地医院就诊,予以腹部CT及其他检查,诊断为"肝硬化,左下肢溃疡",具体原因不详,予相关药物治疗。此后上述症状反复出现,3个月前患者再次出现左下肢胫前皮肤瘙痒、疼痛,于南昌大学第一附属     

千里光致肝窦阻塞综合征伴停经1例

肝窦阻塞综合征(hepatic sinusoidal obstruction syndrome,SOS)是一种少见的肝脏血管性疾病,含吡咯烷生物碱的植物为其常见致病因素,严重者可出现致命性多器官功能衰竭。由于SOS早期症状不典型,发病机制复杂,缺乏特效的治疗手段,目前误诊率高、预后较差。本文对1例千里光所致SOS伴停经病例进行报道,并回顾相关文献,为临床防治药物性肝血管损伤提供参考。     

Liver stiffness and perfusion changes for hepatic sinusoidal obstruction syndrome in rabbit model

BACKGROUND Hepatic sinusoidal obstruction syndrome(SOS)is caused by damage to hepatic sinusoidal endothelial cells that results in fibrous obliteration of intrahepatic venules and necrosis of hepatocytes.Currently the diagnosis is primarily based on nonspecific clinical features and invasive liver biopsy.Therefore,noninvasive imaging methods are required for the early diagnosis and severity assessment of hepatic SOS.AIM To determine the effectiveness of supersonic shear wave imaging(SSI)and dual energy computed tomography(DECT)for diagnosing hepatic SOS using a rabbit model.METHODS Among nine New Zealand white rabbits(3-4 kg,male),three in control group ingested normal saline for 20 d and six in the SOS group ingested 6-thioguanine(5 mg/kg/d)for 20 d.Liver stiffness was measured using SSI on days 0,3,10,and 20.On the same days,liver perfusion was evaluated from virtual monochromatic images of 55 keV and iodine map using DECT.Morphologic changes in the liver were assessed using CT.Final pathology scores were compared between the two groups.Liver stiffness and perfusion parameters were compared according to the groups,days,and pathology scores.RESULTS Final pathology scores were significantly higher in the SOS than the control group(median 22 vs 2,P=0.024).No gross morphologic changes were seen in livers.Liver stiffness,Hounsfield Unit values,and iodine concentrations were higher in the SOS compared to the control group on days 10 and 20(all,P≤0.007).Compared to day 0,liver stiffness and perfusion parameters were higher on day 20 in the SOS group(all,P≤0.001).Correlation coefficients for liver stiffness(r=0.635),Hounsfield Unit values(r=0.587),and iodine concentration(r=0.611)with final pathology scores were positive without significance(all,P>0.05).CONCLUSION Liver stiffness and perfusion parameters were significantly increased in the livers of a rabbit SOS model.SSI and DECT might aid in early diagnosis of hepatic SOS.     

Tusanqi and hepatic sinusoidal obstruction syndrome

Hepatic sinusoidal obstruction syndrome (HSOS), characterized by hepatomegaly, ascites and hyperbilirubinemia, is caused by toxic injury to hepatic sinusoidal endothelial cells. One major etiology of HSOS in China is the intake of products containing pyrrolizidine alkaloids (PA) such as Tusanqi. The manifestations of patients with HSOS are usually non‐specific, presenting with abnormal liver function and portal hypertension. Diagnosis of the disease depends mostly on liver histopathology when clinical and imaging data are not sufficient. A history of Tusanqi intake is mostly important for the diagnosis. Due to a lack of effective, evidence‐based treatments for HSOS, avoiding the mistaken use of PA‐containing products including Tusanqi is important for the prevention of HSOS.     

布-加综合征、肝血窦阻塞综合征与肝硬化的鉴别

布-加综合征是由肝静脉或其开口以上的下腔静脉阻塞性病变引起的以下腔静脉阻塞、门静脉高压为特点的综合征,诊断依靠影像学检查,治疗的关键是解除梗阻。肝血窦阻塞综合征是指肝窦内皮完整性破坏和肝窦内充血阻塞而产生的肝内窦后性门静脉高压症,临床表现类似布-加综合征,诊断依靠肝组织活检。这两种疾病的治疗和预后均与肝实质病变导致的肝硬化不同,所以临床上需要注意这三种疾病的鉴别。     

Noninvasive imaging of hepatic dysfunction: A state-of-the-art review

Hepatic dysfunction represents a wide spectrum of pathological changes, which can be frequently found in hepatitis, cholestasis, metabolic diseases, and focal liver lesions. As hepatic dysfunction is often clinically silent until advanced stages, there remains an unmet need to identify affected patients at early stages to enable individualized intervention which can improve prognosis. Passive liver function tests include biochemical parameters and clinical grading systems (e.g., the Child-Pugh score and Model for End-Stage Liver Disease score). Despite widely used and readily available, these approaches provide indirect and limited information regarding hepatic function. Dynamic quantitative tests of liver function are based on clearance capacity tests such as the indocyanine green (ICG) clearance test. However, controversial results have been reported for the ICG clearance test in relation with clinical outcome and the accuracy is easily affected by various factors. Imaging techniques, including ultrasound, computed tomography, and magnetic resonance imaging, allow morphological and functional assessment of the entire hepatobiliary system, hence demonstrating great potential in evaluating hepatic dysfunction noninvasively. In this article, we provide a state-of-the-art summary of noninvasive imaging modalities for hepatic dysfunction assessment along the pathophysiological track, with special emphasis on the imaging modality comparison and selection for each clinical scenario.     

Pyrrolizidine alkaloids-induced hepatic sinusoidal obstruction syndrome: Pathogenesis,clinical manifestations,diagnosis,treatment,and outcomes

Hepatic sinusoidal obstruction syndrome (HSOS) can be caused by the intake of pyrrolizidine alkaloids (PAs). To date, PAs-induced HSOS has not been extensively studied. In view of the difference in etiology of HSOS between the West and China, clinical profiles, imaging findings, treatment, and outcomes of HSOS associated with hematopoietic stem cell transplantation or oxaliplatin might be hardly extrapolated to PAs-induced HSOS. Reactive metabolites derived from PAs form pyrrole-protein adducts that result in toxic destruction of hepatic sinusoidal endothelial cells. PAs-induced HSOS typically manifests as painful hepatomegaly, ascites, and jaundice. Laboratory tests revealed abnormal liver function tests were observed in most of the patients with PAs-induced HSOS. In addition, contrast computed tomography and magnetic resonance imaging scan show that patients with PAs-induced HSOS have distinct imaging features, which reveal that radiological imaging provides an effective noninvasive method for the diagnosis of PAs-induced HSOS. Liver biopsy and histological examination showed that PAs-induced HSOS displayed distinct features in acute and chronic stages. Therapeutic strategies for PAs-induced HSOS include rigorous fluid management, anticoagulant therapy, glucocorticoids, transjugular intrahepatic portosystemic shunt, liver transplantation, etc. The aim of this review is to describe the pathogenesis, clinical profiles, diagnostic criteria, treatment, and outcomes of PAs-induced HSOS.