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.

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.

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.

乳糜泻是特发性门静脉高压症的潜在原因:一例报告

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.