Current concepts in the management of non-cirrhotic non-malignant portal vein thrombosis

Non-cirrhotic non-malignant portal vein thrombosis(NCPVT)is an uncommon condition characterised by thrombosis of the portal vein,with or without extension into other mesenteric veins,in the absence of cirrhosis or intra-abdominal malignancy.Complications can include intestinal infarction,variceal bleeding and portal biliopathy.In this article,we address current concepts in the management of NCPVT including identification of risk factors,classification and treatment,and review the latest evidence on medical and interventional management options.

Transjugular intrahepatic portosystemic shunt and splenectomy are more effective than endoscopic therapy for recurrent variceal bleeding in patients with idiopathic noncirrhotic portal hypertension

BACKGROUND Transjugular intrahepatic portosystemic shunt(TIPS),splenectomy plus esophagogastric devascularization(SED)and endoscopic therapy+non-selectiveβ-blockers(ET+NSBB)are widely applied in secondary prevention of recurrent gastroesophageal variceal bleeding in patients with liver cirrhosis.These different treatments,however,have not been compared in patients with idiopathic noncirrhotic portal hypertension(INCPH).AIM To compare the outcomes of TIPS,SED and ET+NSBB in the control of variceal rebleeding in patients with INCPH.METHODS This retrospective study recruited patients from six centers across China.Demographic characteristics,baseline profiles and follow-up clinical outcomes were collected.Post-procedural clinical outcomes,including incidence of rebleeding,hepatic encephalopathy(HE),portal vein thrombosis(PVT)and mortality rates,were compared in the different groups.RESULTS In total,81 patients were recruited,with 28 receiving TIPS,26 SED,and 27 ET+NSBB.No significant differences in demographic and baseline characteristics were found among these three groups before the procedures.After treatment,blood ammonia was significantly higher in the TIPS group;hemoglobin level and platelet count were significantly higher in the SED group(P<0.01).Rebleeding rate was significantly higher in the ET+NSBB group(P<0.01).Mortality was 3.6%,3.8%and 14.8%in the TIPS,SED and ET+NSBB groups,respectively,with no significant differences(P=0.082).Logistic regression analysis showed that mortality was significantly correlated with rebleeding,HE,portal thrombosis and superior mesenteric vein thrombosis(P<0.05).CONCLUSION In patients with INCPH,TIPS and SED were more effective in controlling rebleeding than ET+NSBB,but survival rates were not significantly different among the three groups.Mortality was significantly correlated with rebleeding,HE and PVT.

Outcomes of partial splenic embolization in patients with massive splenomegaly due to idiopathic portal hypertension

AIM To determine the outcomes of partial splenic em-bolization(PSE) for massive splenomegaly due to idiopathic portal hypertension(IPH).METHODS In this prospective study, we evaluated the charac-teristics and prognosis of consecutive patients with IPH who underwent PSE for all indications at a single medical center between June 2009 and January 2015. The inclusion criteria were: presence of hypersplenism, massive splenomegaly, and resultant pancytopenia. The exclusion criteria were: presence of other diseases causing portal hypertension. During the post-PSE period, the patients were hospitalized. All patients underwent abdominal computed tomography imaging 4 wk post-PSE to determine total splenic and non-infarcted splenic volumes.RESULTS A total of 11 patients, with median age of 33.27 ± 4.8 years, were included in the study. Mean spleen size was 22.9 cm(21-28 cm), and severe hypersplenismwas diagnosed in all patients before PSE. Post-PSE, leukocyte and platelet counts increased significantly, reaching peak levels in the second week with gradual decreases thereafter. Liver function tests did not exhibit significant changes during post-intervention follow-up. All patients developed post-embolization syndrome, and one patient experienced serious complications; all complications were successfully treated with conservative therapy and no death occurred. CONCLUSION Our findings showed that PSE has a lower complication rate than previously-reported surgical complication rates, which supports this intervention as a viable alternative for high-risk operable patients with severe hypersplenism.

Clinical characteristics of idiopathic portal hypertension

Idiopathic portal hypertension is one of the interesting causes of portal hypertension. Even in very developed medical centers, this disorder is still one of the most important misdiagnoses of clinical practice. To inexperienced physicians, presenting esophageal varices and upper gastrointestinal bleeding usually prompt an unfortunate diagnosis of cirrhosis. A heterogenous clinical presentation and progression of this disorder should be recognized by physicians, and management should be directed towards some specific problems confined to this disorder. Although a genetic basis and other factors are implicated in its pathogenesis, exact underlying mechanism(s) is (are) unknown. In this review, we discuss the heterogeneity of idiopathic portal hypertension, its etiopathogenesis, clinical presentation and management issues. With the expectation of an excellent prognosis, a practicing gastroenterologist should be aware that "not all varices mean cirrhosis".

Diagnostic challenges in non-cirrhotic portal hypertension-porto sinusoidal vascular disease

Non-cirrhotic portal hypertension consists of a group of diseases characterized by signs and complications of portal hypertension,which differ from cirrhosis through histological alterations,hemodynamic characterization and,clinical outcome.Because of the similarities in clinical presentation and imaging signs,frequently these patients,and particularly those with porto-sinusoidal vascular disease(PSVD),are misdiagnosed as having liver cirrhosis and thus raising difficulties in their diagnosis.The most challenging differentiation to be considered is between PSVD and cirrhosis and,although not pathognomonic,liver biopsy is still the standard of diagnosis.Although they still require extended validation before being broadly used,new non-invasive methods for the diagnosis of porto-sinusoidal vascular disease,like transient elastography,contrast-enhanced ultrasound or metabolomic profiling,have shown promising results.Another issue is the differentiation between PSVD and chronic extrahepatic portal vein obstruction,especially now when it is known that 40%of patients suffering from PSVD develop portal vein thrombosis.In this particular case,once the portal vein thrombosis occurred,the diagnosis of PSVD is impossible according to the current guidelines.Moreover,so far,the differentiation between PSVD and sinusoidal obstruction syndrome has not been clear so far in particular circumstances.In this review we highlighted the diagnostic challenges regarding the PSVD,as well as the current techniques used in the evaluation of these patients.

Duodenal variceal bleeding secondary to idiopathic portal hypertension treated with transjugular intra-hepatic portosystemic shunt plus embolization: A case report

BACKGROUND Duodenal varices are a lesser-known complication with non-cirrhotic portal hypertension. We report a circuitous route from missed diagnosis of duodenal varices to correction. An extremely rare case of duodenal variceal bleeding secondary to idiopathic portal hypertension(IPH) is expounded in this study, which was controlled by transjugular intra-hepatic porto-systemic shunt(TIPS) plus embolization. CASE SUMMARY A 46-year-old woman with anemia for two years was frequently admitted to the local hospital. Upon examination, anemia was attributed to gastrointestinal tract bleeding, which resulted from duodenal variceal bleeding detected by repeated esophagogastroduodenoscopy. At the end of a complete workup, IPH leadingto duodenal varices was diagnosed. Portal venography revealed that the remarked duodenal varices originated from the proximal superior mesenteric vein. TIPS plus embolization with coils and Histoacryl was performed to obliterate the rupture of duodenal varices. The anemia resolved, and the duodenal varices completely vanished by 2 mo after the initial operation. CONCLUSION TIPS plus embolization may be more appropriate to treat the bleeding of large duodenal varices.

Pathological abnormalities in splenic vasculature in non-cirrhotic portal hypertension:Its relevance in the management of portal hypertension

BACKGROUND Portal hypertension(PH)is associated with changes in vascular structure and function of the portosplenomesenteric system(PSMS).This is referred to as portal hypertensive vasculopathy.Pathological abnormalities of PSMS has been described in the literature for cirrhotic patients.Raised portal pressure and hyperdynamic circulation are thought to be the underlying cause of this vasculopathy.In view of this,it is expected that pathological changes in splenic and portal vein similar to those reported in cirrhotic patients with PH may also be present in patients with non-cirrhotic PH(NCPH).AIM To investigate pathological abnormalities of splenic vein in patients with NCPH,and suggest its possible implications in the management of PH.METHODS A prospective observational study was performed on 116 patients with NCPH[Extrahepatic portal vein obstruction(EHPVO):53 and non-cirrhotic portal fibrosis(NCPF):63]who underwent proximal splenorenal shunt(PSRS),interposition shunt or splenectomy with devascularization in JIPMER,Pondicherry,India,a tertiary level referral center,between 2011-2016.All patients were evaluated by Doppler study of PSMS,computed tomography portovenogram and upper gastrointestinal endoscopy.An acoustic resonance forced impulse(ARFI)scan and abdomen ultrasound were done for all cases to exclude cirrhosis.Intraoperative and histopathological assessment of the harvested splenic vein was performed in all.The study group was divided into delayed and early presentation based on the median duration of symptoms(i.e.108 mo).RESULTS The study group comprising of 116 patients[77(66%)females and 39(34%)males]with NCPH had a median age of 22 years.Median duration of symptoms was 108 mo.The most common presentation in both EHPVO and NCPF patients was upper gastrointestinal bleeding(hematemesis and melena).The ARFI scan revealed a median score of 1.2(1.0-1.8)m/s for EHPVO and 1.5(0.9-2.8)m/s for NCPF.PSRS was performed in 84 patients(two of whom underwent interposition PSRS using a 10 mm Dacron graft);splenoadrenal shunt in 9;interposition mesocaval shunt in 5;interposition 1st jejunal to caval shunt in 1 patient and devascularization with splenectomy in 17 patients.Median presplenectomy portal pressure was 25(range:15-51)mm Hg.In 77%cases,the splenic vein was abnormal upon intraoperative assessment.Under macroscopic examination,wall thickening was observed in 108(93%),venous thrombosis in 32(28%)and vein wall calcification in 27(23%)cases.Upon examination under a surgical magnification loupe,21(18%)patients had intimal defects in the splenic vein.Histopathological examination of veins was abnormal in all cases.Medial hypertrophy was noted in nearly all patients(107/116),while intimal fibrosis was seen in 30%.Ninety one percent of patients with intimal fibrosis also had venous thrombosis.Vein wall calcification was found in 22%,all of whom had intimal fibrosis and venous thrombosis.The proportion of patients with pathological abnormalities in the splenic vein were significantly greater in the delayed presentation group as compared to the early presentation group.CONCLUSION Pathological changes in the splenic vein similar to those in cirrhotic patients with PH are noted in NCPH.We recommend that PH in NCPH be treated as systemic and pulmonary hypertension equivalent in the gastrointestinal tract,and that early aggressive therapy be initiated to reduce portal pressure and hemodynamic stress to avoid potential lethal effects.

Non-cirrhotic portal hypertension with large regenerative nodules: A diagnostic challenge

Non-cirrhotic portal hypertension is a poorly understood condition characterized by portal hypertension in the absence of conventional hepatic cirrhosis and described in association with blood coagulation disorders, myeloproliferative and immunological diseases and with exposure to toxic drugs. Very recently, precise classification criteria have been proposed in order to define four distinct subcategories. The present case highlights how the clinical presentation, the confounding results from imaging studies, and the difficulties in the histological evaluation often render cases of non-cirrhotic portal hypertension a real diagnostic challenge. It also underscores the classification problems which can be faced once this diagnosis is performed. Indeed, the different subcategories proposed result from the prevalent subtypes in a spectrum of hepatic regenerative responses to a variety of injuries determining microcirculatory dis-turbances. More flexibility in classification should derive from this etiopathogenic background.

Isolated gastric variceal bleeding related to non-cirrhotic portal hypertension following oxaliplatin-based chemotherapy:A case report

BACKGROUND Sinusoidal obstruction syndrome has been reported after oxaliplatin-based chemotherapy,but liver fibrosis and non-cirrhotic portal hypertension(NCPH)are rarely reported.CASE SUMMARY Here,we describe the case of a 64-year-old woman who developed isolated gastric variceal bleeding 16 mo after completing eight cycles of oxaliplatin combined with capecitabine chemotherapy after colon cancer resection.Surprisingly,splenomegaly and thrombocytopenia were not accompanied by variceal bleeding,which has been reported to have predictive value for gastric variceal formation.However,a liver biopsy showed fibrosis in the portal area,suggesting NCPH.The patient underwent endoscopic treatment and experienced no further symptoms.CONCLUSION It is necessary to guard against long-term complications after oxaliplatin-based chemotherapy.Sometimes splenic size and platelet level may not always accurately predict the occurrence of portal hypertension.

Peliosis hepatis as an early histological finding in idiopathic portal hypertension: A case report

Peliosis hepatis is a rare condition characterized by dilatation of hepatic sinusoids and blood-filled spaces in the liver mainly observed in subjects exposed to toxic substances or estrogens, which is frequently asymptomatic. Non-cirrhotic idiopathic portal hypertension （NCIPH） is also a vascular disease of the liver rarely observed in European countries, which is usually diagnosed only when the hemorrhagic complications of portal hypertension occur. We report a case of NCIPH in a young Caucasian male who was diagnosed with liver peliosis, showing ultrasonographic and endoscopic signs of portal hypertension four years after. A second biopsy was diagnostic for NCIPH. Even if the pathogenesis remains obscure, peliosis hepatis can be considered as an early sign of vascular disease of the liver, which may progress to more definite conditions.

Systemic mastocytosis: A rare cause of non-cirrhotic portal hypertension

Mastocytosis is a clonal neoplastic disorder of the mast cells(MC) that can be limited to the skin(cutaneous mastocytosis) or involve one or more extracutaneous organs(systemic mastocytosis). The clinical manifestations of mastocytosis are heterogeneous ranging from indolent disease with a long-term survival to a highly aggressive neoplasm with survival of about 6 mo. Although liver involvement in aggressive systemic mastocytosis(ASM) is relatively common, the development of portal hypertension with or without cirrhosis is rare. We report a case of ASM without skin involvement in a 72-year-old caucasian male who presented with non-cirrhotic portal hypertension based on clinical, analytical, imagiological and endoscopic findings. Given the hematological picture, the correct diagnosis was established based on ancillary tests for MC using bone marrow aspirates and biopsy. Extensive involvement of the liver and gastrointestinal tract was histologically documented. The disease progressed rapidly and severe pancytopenia and recurrent upper gastrointestinal bleeding became the dominant problem. This case illustrates the challenge in establishing a diagnosis of ASM especially when the clinical picture is atypical and without skin involvement. Gastroenterologists should consider infiltrative disease, particularly systemic mastocytosis, as a differential diagnosis in a clinical case of portal hypertension of unknown etiology.

A rabbit model of non- cirrhotic portal hypertension by repeated injections of E. coli through indwelling cannulation of the gastrosplenic vein

BACKGROUND: Non-cirrhotic portal hypertension is acommon cause of portal hypertension in developing coun-tries. To understand its etiopathogenesis we developed ananimal model by repeated portal endotoxemia inducedthrough the gastrosplenic vein.METHODS: Twenty-nine rabbits (1.5-2.0 kg) were divid-ed into control (group n = 13) and experimental ( groupn = 16) groups. Heat killed E. coli were injected throughan indwelling cannula into the gastrosplenic vein in pre-sensitized animals. The animals were sacriflced at 1, 3 and6 months.RESULTS: The mean portal pressure in group animalswas significantly (P < 0. 05) higher than in group at 1(17.5 ±3.4 vs 10.4±2.2 mmHg), 3 (17.8±1.3 vs7.2 +3.6mmHg), and 6 (19.8±3.1 vs 10.3±4.8 mmHg) months.Similarly, the mean splenic weight in group was signifi-cantly greater than in group (P <0.05). Histopathologi-cally, the spleen showed medullary congestion, hemosid-rin-laden macrophages and mild fibrosis. Histologically,the liver had normal parenchyma with mild portal lympho-cytic infiltrates and kupffer cell hyperplasia. No significantanomalies were detected by liver function tests.CONCLUSIONS: The rabbit model showed significantsplenomegaly with a persistent increase in portal pressureand mild fibrosis without hepatic parenchymal injury, quiteakin to non-cirrhotic portal fibrosis as seen in humans. Re-current intra-abdominal infection may play an importantrole in the pathogenesis of non-cirrhotic portal fibrosis.

Successful treatment of noncirrhotic portal hypertension with eculizumab in paroxysmal nocturnal hemoglobinuria: A case report

BACKGROUND Idiopathic non-cirrhotic portal hypertension(INCPH)is mainly associated with thrombophilia in Western countries.Paroxysmal nocturnal hemoglobinuria(PNH)is a rare hematologic disease that manifests with hemolytic anemia,thrombosis,and peripheral blood cytopenias.Portal and hepatic venous thrombosis were reported in PNH.A rare case of INCPH complicating PNH is described.CASE SUMMARY A 63-year old woman with a 2-year past medical history of PNH without treatment was admitted because of jaundice and refractory ascites requiring large volume paracentesis.Liver histology revealed portal venopathy with portal fibrosis and sclerosis,nodular regenerative hyperplasia,parenchymal ischemic changes,and focal sinusoidal and perivenular fibrosis without bridging fibrosis or cirrhosis,all indicative of INCPH.The flow cytometry confirmed PNH diagnosis and eculizumab treatment was initiated.Her condition was improved gradually,bilirubin was normalized 6 months following initiation of eculizumab,and 1 year later diuretics were stopped.CONCLUSION Eculizumab improved intravascular hemolysis and reversed clinical manifestations of INCPH in a patient with paroxysmal nocturnal hemoglobinuria.

Application of ultrasonography-elastography score to suspect porto-sinusoidal vascular disease in patients with portal vein thrombosis

Background:Porto-sinusoidal vascular disease(PSVD)and portal vein thrombosis(PVT)are causes of portal hypertension characterized respectively by an intrahepatic and a pre-hepatic obstacle to the flow in the portal system.As PVT may be a consequence of PSVD,in PVT patients at presentation,a pre-existing PSVD should be suspected.In these patients the identification of an underlying PSVD would have relevant implication regarding follow-up and therapeutic management,but it could be challenging.In this setting ultrasonography may be valuable in differential diagnosis.The aim of the study was to use ultrasonography to identify parameters to discriminate between PSVD and“pure”PVT and then to suspect PVT secondary to a pre-existing PSVD.Methods:Fifty-three patients with histologically proven PSVD and forty-eight patients affected by chronic PVT were enrolled and submitted to abdominal ultrasonography with elastography by acoustic radiation force impulse(ARFI).Results:ARFI was higher and superior mesenteric vein(SMV)diameter was wider in PSVD patients than in PVT patients.Thus,a prognostic score was obtained as linear combinations of the two parameters with a good discrimination capacity between PSVD and PVT(the area under the curve=0.780;95%confidence interval:0.690-0.869).Conclusions:A score based on ARFI and SMV diameter may be useful to suspect an underlying PSVD in patients with PVT and to identify a subgroup of patients to be submitted to liver biopsy.

Coeliac disease as a potential cause of idiopathic portal hypertension:a case report

Idiopathic portal hypertension is a disorder that has various clinical features.It is mostly characterized by bleeding oesophageal varices,obvious splenomegaly,anaemia and,occasionally,jaundice and ascites.Here we described an interesting case of idiopathic portal hypertension caused by coeliac disease in a 38-year-old woman.By putting this patient on a gluten-free diet,liver function tests became normal and portal vein diameter returned to normal range.This report indicates that,in coeliac disease,repetitive stimulation by antigens along the portal vein—and immune responses to them—can result in the development of idiopathic portal hypertension.

特发性非肝硬化门静脉高压症的关键基因通路筛选与潜在中药预测

目的采用生物信息学方法对特发性非肝硬化门静脉高压症(idiopathic non-cirrhotic portal hypertension,INCPH)的基因芯片数据进行分析,获取疾病发生发展的关键基因和信号通路,预测治疗INCPH的潜在中药。方法从基因表达综合数据库(gene expression omnibus,GEO)数据库下载关于INCPH的基因芯片数据集GSE77627,利用R语言对数据进行标准化并筛选INCPH的差异基因(differential genes,DEGs),并利用Metascape数据库对所有DEGs进行基因本体(gene ontology,GO)和京都基因与基因组百科全书(Kyoto encyclopedia of genes and genomes,KEGG)富集分析,并通过STRING数据库构建蛋白-蛋白互作网络;同时,利用CytoHubba插件筛选Degree值排名前15的DEGs作为关键基因。随后将关键基因与医学本体信息检索平台互相映射,以P<0.05为标准筛选治疗INCPH的潜在中药,并从TCMSP数据库中筛选潜在中药有效成分,导入Cytoscape软件构建中药相关网络图,并预测关键作用靶点。结果共获得1880个DEGs,其中表达上调的基因有1061个,表达下调的基因有819个。利用STRING数据库以及Cytoscape环境下的cytoHubba插件分析DEGs,筛选Dgree值排名15位的基因作为关键基因,分别是RPS27A、CDC42、EIF4E、MAPK1、PIK3R1、RPS6、RPS9、RPS8、RPL15、RPL27A、RPL24、RPL27、RPL26、RPL12和MAPK14。GO、KEGG分析显示DEGs主要参与配子生成、糖尿病并发症中的AGE-RAGE信号通路等信号通路。结论筛选得到干预INCPH的潜在中药为人参、丹参、黄芪等,可能成为INCPH治疗的潜在分子药物来源。

Portal ductopathy:Clinical importance and nomenclature

Non-cirrhotic portal hypertension(PHT)accounts for about 20%of all PHT cases,portal vein thrombosis(PVT) resulting in cavernous transformation being the most common cause.All known complications of PHT may be encountered in patients with chronic PVT.However,the effect of this entity on the biliary tree and pancreatic duct has not yet been fully established.Additionally,a dispute remains regarding the nomenclature of common bile duct abnormalities which occur as a result of chronic PVT.Although many clinical reports have focused on biliary abnormalities,only a few have evaluated both the biliary and pancreatic ductal systems.In this review the relevant literature evaluating the effect of PVT on both ductal systems is discussed,and findings are considered with reference to results of a prominent center in Turkey,from which the term"portal ductopathy"has been put forth to replace"portal biliopathy".

Transhepatic catheter-directed thrombolysis for portal vein thrombosis after partial splenic embolization in combination with balloon-occluded retrograde transvenous obliteration of splenorenal shunt

A 66-year-old woman underwent partial splenic embolization （PSE） for hypersplenisrn with idiopathic portal hypertension （IPH）. One week later, contrast-enhanced CT revealed extensive portal vein thrombosis （PVT） and dilated portosystemic shunts. The PVT was not dissolved by the intravenous administration of urokinase. The right portal vein was canulated via the percutaneous transhepatic route under ultrasonic guidance and a 4 Fr. straight catheter was advanced into the portal vein through the thrombus. Transhepatic catheter-directed thrombolysis was performed to dissolve the PVT and a splenorenal shunt was concurrently occluded to increase portal blood flow, using balloon-occluded retrograde transvenous obliteration （BRTO） technique. Subsequent contrast-enhanced CT showed good patency of the portal vein and thrombosed splenorenal shunt. Transhepatic catheter-directed thrombolysis combined with BRTO is feasible and effective for PVT with portosystemic shunts.

74例特发性非硬化性门静脉高压症患者的临床特征分析

目的 特发性非硬化性门静脉高压(INCPH)是一种少见的引起门静脉高压的病因,对INCPH患者的临床特点进行分析以辅助诊断及鉴别诊断。方法 选取北京佑安医院2019年1月-2022年7月经肝穿刺病理明确诊断为INCPH的住院患者74例,并以同期住院的332例肝硬化患者作为对照组,记录其人口学指标、实验室指标、胃镜、肝弹性检查、病理检查及并发症等资料,进行组间差异比较,并根据受试者工作特征曲线(ROC曲线)评价LSM、APRI、FIB-4对INCPH的鉴别诊断能力,采用DeLong检验方法进行ROC曲线下面积(AUC)比较。符合正态分布的计量资料两组间比较采用成组t检验,非正态分布的计量资料两组间比较采用Mann-Whitney U检验。计数资料两组间比较采用χ^(2)检验。结果 INCPH患者中46.55%的患者起病时无明显症状,43.24%患者曾被误诊为肝硬化。INCPH患者合并消化道出血比例明显多于肝硬化患者(62.16%vs 41.27%,χ^(2)=10.67,P<0.01),但合并中-重度腹水患者比例明显少于肝硬化患者(16.21%vs 29.82%,χ^(2)=34.98,P<0.01),无肝性脑病发生。病理方面,89.19%(66/74)患者病理表现为典型的闭塞性门静脉病。INCPH患者肝功能指标、MELD评分、Child-Pugh评分等指标明显优于肝硬化患者,肝硬度值[9.05(7.18~12.33) vs 25.32(16.21~47.23),Z=-8.41,P<0.01]、APRI评分[0.70(0.41~1.28) vs 1.35 (0.80~2.39),Z=-6.21,P<0.01]、FIB-4指数[2.99(1.62~4.81) vs 6.68(4.06~10.42),Z=-8.39,P<0.01]均较肝硬化低。LSM、FIB-4、APRI对INCPH与肝硬化患者具有很好的鉴别诊断能力,尤其是LSM的AUC达到0.92(0.87~0.96),敏感度和特异度分别为92.68%和81.60%。结论 INCPH患者起病较隐匿,门静脉高压相关并发症发生率较高,而肝功能相对较好,尤其是LSM<14.5 kPa的患者,临床遇到此类患者需警惕INCPH可能。

特发性门脉高压肝移植术后随访第3年再发1例报告及文献复习

目的复习1例特发性门脉高压(IPH)患者接受肝移植(LT)后第3年出现病情"再发",并进行了相关文献复习,以提高对该病的认识.方法报道1例我们诊治的IPH患者的病例资料,并检索MEDLINE、EMBASE、万方等数据库经LT治疗的IPH患者的研究报道,分析其治疗和转归.结果本文报道的病例为57岁女性,因消化道出血、腹水行LT术,组织病理学检查诊断为IPH;术后随访第3年病情复发,行经皮肝穿刺活检术,病理学检查提示结节性再生性增生(NRH)、轻度汇管区炎症及纤维化,提示IPH再发;文献检索到81例LT治疗的IPH患者,其中42例在LT前诊断为肝硬化;LT后最长随访时间为248个月,8例死亡,其中5例分别在首次LT后3.5月~14年进行肝活检,组织病理学检查提示NRH,3例分别于LT后第7月、第3年和第14年出现具有门脉高压表现的NRH.结论具有严重的门脉高压或肝功能衰竭的IPH患者需要LT治疗.少数患者在LT后可能出现IPH"再发".