Recent developments in the field of vascular liver diseases.

Knowledge in the field of vascular liver disease is continuously expanding. The present update will discuss recent data on i) the Abernethy malformation in adults; ii) portal vein thrombosis in cirrhosis; iii) advancing expertise in recanalization of the portal vein and iv) experience in using direct oral anticoagulants in the field of vascular liver disease.

Understanding the Similarities and Differences between Hepatic and Pulmonary Veno-Occlusive Disease.

Hepatic veno-occlusive disease (HVOD), alias sinusoidal obstruction syndrome, may develop as a complication of chemotherapy in the setting of hematopoietic stem cell transplantation. HVOD is less frequently described after exposure to chemotherapy in the nontransplant setting and can also be a complication after ingestion of toxins, such as pyrrolizidine alkaloids. Veno-occlusive disease may also affect the lungs, and it is therefore termed pulmonary veno-occlusive disease (PVOD).

Safety of supramesocolic surgery in patients with portal cavernoma without portal vein decompression. Large single centre experience.

Background: Supra-mesocolic surgery (SMS) is complicated in patients with portal vein cavernoma (PC) and portal decompression is recommended. The aim of this study was to report a large single centre of SMS in patients with PC without portal decompression.

Methods: Between 2006 and 2013, all patients who met inclusion criteria were analyzed retrospectively.

Interplay of Inflammation and Endothelial Dysfunction in Bone Marrow Transplantation: Focus on Hepatic Veno-Occlusive Disease.

Endothelial cells are unique multifunctional cells with basal and inducible metabolic and synthetic functions. Various stimuli can induce physiological or pathological changes in endothelial cell biology. Hematopoietic stem cell transplantation (HSCT) requires high-dose irradiation and/or chemotherapy and is associated with increased risk of bacterial infections and immune reactions.

Portal cavernoma cholangiopathy: consensus statement of a working party of the Indian national association for study of the liver.

Portal cavernoma cholangiopathy (PCC) is defined as abnormalities in the extrahepatic biliary system including the cystic duct and gallbladder with or without abnormalities in the 1st and 2nd generation biliary ducts in a patient with portal cavernoma. Presence of a portal cavernoma, typical cholangiographic changes on endoscopic or magnetic resonance cholangiography and the absence of other causes of these biliary changes like bile duct injury, primary sclerosing cholangitis, cholangiocarcinoma etc are mandatory to arrive a diagnosis. Compression by porto-portal collateral veins involving the paracholedochal and epicholedochal venous plexuses and cholecystic veins and ischemic insult due to deficient portal blood supply or prolonged compression by collaterals bring about biliary changes.

The coagulation system in patients with end-stage liver disease.

In patients with cirrhosis, routine laboratory tests for primary hemostasis and coagulation usually show anomalies that are associated with excess bleeding in other settings, in particular low platelet counts and prolonged prothrombin time. However, under conditions similar to those in vivo, primary hemostasis and thrombin production do not appear to be decreased in patients with cirrhosis, particularly when the platelet count is above 75,000/μl. Furthermore, there is laboratory and epidemiological evidence of a mild procoagulant and prothrombotic state in patients with cirrhosis.

Small hepatic veins Budd-Chiari syndrome.

Budd-Chiari syndrome is a rare disorder characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atrio-caval junction, in the absence of heart failure or constrictive pericarditis. Various imaging modalities are available for investigating the gross hepatic vascular anatomy but there are rare forms of this disease where the obstruction is limited to the small intrahepatic veins, with normal appearance of the large hepatic veins at imaging. In this cases only a liver biopsy can demonstrate the presence of a small vessels outflow block.

Good long-term outcome of Budd-Chiari syndrome with a step-wise management.

Unlabelled: Budd-Chiari syndrome (BCS) is a rare, life-threatening disease caused by obstruction of hepatic venous outflow. The aim of the study was to assess long-term outcome and identify prognostic factors in BCS patients managed by a step-wise approach using anticoagulation, angioplasty/thrombolysis, transjugular intrahepatic portosystemic shunting (TIPS), and orthotopic liver transplantation (OLT). We reviewed long-term data on 157 patients previously included by the European Network for Vascular Disorders of the Liver, a multicenter prospective study of newly diagnosed BCS patients in nine European countries.

Myeloproliferative neoplasms in Budd-Chiari syndrome and portal vein thrombosis: a meta-analysis.

Myeloproliferative neoplasms (MPNs) are the most common cause of Budd-Chiari syndrome (BCS) and nonmalignant, noncirrhotic portal vein thrombosis (PVT). In this meta-analysis, we determined the prevalence of MPNs and their subtypes as well as JAK2V617F and its diagnostic role in these uncommon disorders. MEDLINE and EMBASE databases were searched.

Standardized care management ensures similar survival rates in HIV-positive and HIV-negative patients with hepatocellular carcinoma.

Objective: It has been suggested that HIV infection has a detrimental impact on patients with hepatocellular carcinoma (HCC). The present study sought to test this hypothesis, while controlling for tumor extension and liver disease.

Design And Setting: A case control and a cohort approach were performed in patients with HCC managed prospectively via dedicated multidisciplinary team meeting in a single tertiary institution between 2004 and 2009.

Obliterative portal venopathy: findings at CT imaging.

Purpose: To retrospectively analyze the computed tomographic (CT) findings in a single-center series of adult patients with biopsy-proved obliterative portal venopathy (OPV) and to compare them with those observed in patients with cirrhosis.

Materials And Methods: The requirement for informed consent was waived. This institutional review board-approved study included 42 consecutive patients with a histologically proved diagnosis of OPV who underwent CT at diagnosis.

High-b-value diffusion-weighted MR imaging of benign hepatocellular lesions: quantitative and qualitative analysis.

Purpose: To analyze the signal intensity (SI) of benign hepatocellular lesions in high-b-value diffusion-weighted (DW) magnetic resonance (MR) images and to compare the apparent diffusion coefficient (ADC) values of focal nodular hyperplasias (FNHs) with those of hepatocellular adenomas (HCAs).

Materials And Methods: This retrospective study was approved by institutional review board, with waiver of informed consent. Inclusion criteria were consecutive patients with diagnosed FNH or HCA who underwent MR imaging with a DW sequence of the liver at three b values, 0, 150, and 600 sec/mm2.

Idiopathic noncirrhotic portal hypertension.

Idiopathic noncirrhotic portal hypertension (INCPH) is characterized by an increased portal venous pressure gradient in the absence of a known cause of liver disease and portal vein thrombosis. In contrast to the high prevalence of this disorder in India, INCPH is a rare disease in the Western world. The etiology of INCPH can be divided in five categories: chronic infections, exposure to medication or toxins, thrombophilia, immunological disorders, and genetic disorders.

Suspected interaction between sorafenib and HAART in an HIV-1 infected patient: a case report.

Sorafenib prolongs survival of patients with unresectablehepatocellular carcinoma (HCC). It is likely to be used in human immunodeficiency virus (HIV) infected patients. Interactions between sorafenib and highly active antiretroviral therapy (HAART) have not been studied yet.

The JAK2 46/1 haplotype in Budd-Chiari syndrome and portal vein thrombosis.

The germline JAK2 46/1 haplotype has been associated with the development of JAK2(V617F)-positive as well as JAK2(V617F)-negative myeloproliferative neoplasms (MPNs). In this study we examined the role of the 46/1 haplotype in the etiology and clinical presentation of patients with splanchnic vein thrombosis (SVT), in which MPNs are the most prominent underlying etiological factor. The single-nucleotide polymorphism rs12343867, which tags 46/1, was genotyped in 199 SVT patients.

Hepatic veins as a site of clot formation following liver resection.

Pulmonary embolism occurs more frequently after hepatectomy than previously thought but is infrequently associated with peripheral deep vein thrombosis. In this paper, we report 2 cases of postoperative hepatic vein thrombosis after liver resection. Both patients had undergone major hepatectomy of a non-cirrhotic liver largely exposing the middle hepatic vein.

Proteomic analysis reveals that apolipoprotein A1 levels are decreased in patients with Budd-Chiari syndrome.

Background & Aims: Budd-Chiari syndrome (BCS) is a rare vascular liver disorder caused by thrombosis of the hepatic veins. In some patients, no known thrombophilic factor can be identified. This study aimed to identify novel factors that might play a role in thrombosis in BCS-patients by using a proteomic approach.

Obliterative portal venopathy: portal hypertension is not always present at diagnosis.

Background & Aims: Previous studies on obliterative portal venopathy (OPV) have been biased due to the selection of patients with non-cirrhotic portal hypertension. The aim of this study was to clarify the characteristics of OVP diagnosed by liver biopsy.

Methods: Fifty-nine consecutive patients with OPV were retrospectively selected on strict histological criteria.

Incidental focal solid liver lesions: diagnostic performance of contrast-enhanced ultrasound and MR imaging.

Objective: To prospectively assess the diagnostic performance of contrast-enhanced ultrasound (CEUS) and MR imaging in incidental solid focal liver lesions not characterised on ultrasound.

Materials And Methods: Forty-seven patients with 50 lesions underwent MR imaging and CEUS: 24 focal nodular hyperplasias (FNH), 11 adenomas, 10 haemangiomas, 1 focal fatty change and 4 malignant lesions were identified. Two experienced radiologists randomly reviewed contrast-enhanced MR imaging and CEUS data, and provided the most likely diagnosis.

Impaired fibrinolysis as a risk factor for Budd-Chiari syndrome.

In Budd-Chiari syndrome (BCS), thrombosis develops in the hepatic veins or inferior vena cava. To study the relationship between hypofibrinolysis and BCS, we measured plasma levels of fibrinolysis proteins in 101 BCS patients and 101 healthy controls and performed a plasma-based clot lysis assay. In BCS patients, plasminogen activator inhibitor 1 (PAI-1) levels were significantly higher than in controls (median, 6.