Search results for "Budd–Chiari syndrome"

PAI-1 4G-4G, MTHFR 677TT, V Leiden 506Q, and Prothrombin 20210A in Splanchnic Vein Thrombosis: Analysis of Individual Patient Data From Three Prospec…

2015

There are no univocal opinions on the role of genetic thrombophilia on splanchnic vein thrombosis (SVT). We defined genetic thrombophilia the presence of one of these thrombophilic genetic factors (THRGFs): PAI-1 4G-4G, MTHFR 677TT, V Leiden 506Q, and prothrombin 20210A.To evaluate the frequencies of these THRGFs in SVT patients, we analyzed individual data of 482 Caucasian patients, recruited from 2000 to 2014 in three prospective studies. SVT was defined as the presence of thrombosis of portal (PVT), mesenteric (MVT), splenic (SPVT), cava (CT), and hepatic vein (Budd Chiari syndrome, BCS). Pre-hepatic SVT (pre-HSVT) was defined as PVT with or without MVT/SPVT, without BCS. Post-hepatic SV…

Budd-Chiari syndrome with portal, splenic, and superior mesenteric vein thrombosis treated with TIPS: who dares wins.

2003

A 20 year old man presented with severe ascites and malnutrition due to Budd-Chiari syndrome (BCS) with portal vein (PV), and splenic and mesenteric vein (SMV) thrombosis in the proximal 3 cm. He had received regular and more frequent paracenteses of up to 17 litres each for eight months. He had a poor BCS prognostic index of 8.41 (table 1). After referral, despite full anticoagulation and diuretic drugs, he deteriorated over three weeks. No thrombophilic disorder was found. View this table: Table 1 Comparison of laboratory and clinical data before and two months after transjugular intrahepatic portosystemic stent shunt (TIPS) He underwent transjugular intrahepatic portosystemic stent shunt…

TIPS for acute and chronic Budd-Chiari syndrome: a single-centre experience.

2003

Abstract Background/Aims : Transjugular intrahepatic portosystemic shunt (TIPS) is a technically challenging but feasible treatment for Budd–Chiari syndrome (BCS). However, information about the outcome, particularly in patients with liver failure, is scarce. We report our experience of TIPS for BCS. Methods : Fifteen patients with BCS underwent TIPS. Eight had hepatic failure and seven underwent TIPS for BCS uncontrolled by medical treatment. Results : Fourteen out of 15 had successful TIPS placement. Out of the eight hepatic failure patients, four died soon after TIPS: one liver rupture, one portal vein rupture, one liver failure and one pulmonary oedema. Another patient had a significant…

Portal vein thrombosis and Budd-Chiari syndrome as onset of polycythemia vera

2013

Budd-Chiari syndrome may be defined as a heterogeneous group of vascular disorders characterized by obstruction of hepatic venous return to the level of hepatic venules, supra-hepatic veins, inferior vena cava or right atrium. The main cause of this syndrome is represented by myeloproliferative diseases and, in particular, by polycythemia vera. The latter may cause multiple splanchnic thrombosis, including portal vein thrombosis, particularly important for its clinical outcomes (ascites, collateral vessels genesis, etc.). We report 2 cases of a Budd-Chiari syndrome induced by polycythemia vera characterized by an abnormal clinical onset, both as regards subjects’ age (29 and 39 years old, r…

AISF position paper on liver disease and pregnancy.

2016

Abstract The relationship between liver disease and pregnancy is of great clinical impact. Severe liver disease in pregnancy is rare; however, pregnancy-related liver disease is the most frequent cause of liver dysfunction during pregnancy and represents a severe threat to foetal and maternal survival. A rapid differential diagnosis between liver disease related or unrelated to pregnancy is required in women who present with liver dysfunction during pregnancy. This report summarizes the recommendation of an expert panel established by the Italian Association for the Study of the Liver (AISF) on the management of liver disease during pregnancy. The article provides an overview of liver disea…

Budd-Chiari Syndrome: Spectrum of Imaging Findings

2007

OBJECTIVE: The objective of our study was to illustrate the imaging findings of Budd-Chiari syndrome, including CT, MRI, sonographic, and angiographic findings. CONCLUSION: The key imaging findings in Budd-Chiari syndrome are occlusion of the hepatic veins, inferior vena cava, or both; caudate lobe enlargement; inhomogeneous liver enhancement; and the presence of intrahepatic collateral vessels and hypervascular nodules. Awareness of these findings is important for early diagnosis and appropriate treatment.

Budd-Chiari Syndrom – eine seltene Manifestation der hereditären Thrombophilie

2000

Das Budd-Chiari Syndrom ist eine seltene Manifestationsform der hereditaren oder erworbenen Thrombophilie. Bei einer 30 jahrigen Patientin konnte, nach anfanglichen diagnostischen Schwierigkeiten, ein BCS diagnostiziert und erfolgreich, durch portocavalen Seit-zu-Seit Shunt, behandelt werden. Bei der Chiari-Trias aus abdominellen Schmerzen, Hepatomegalie und Ascites mus das BCS in die differentialdiagnostischen Uberlegungen mit einbezogen und gegebenenfalls durch invasive Diagnostik ausgeschlossen werden. Therapiemoglichkeiten sind die Anticoagulation und die Anlage eines portosystemischen Shunts (TIPS oder chirurgischer Shunt). Eine Lebertransplantation kommt als ultima ratio bei Leberinsu…

Alloimmune hemolytic anemia after liver transplantation from an ABO-identical and Rh-nonidentical donor in a patient with postpartum Budd-Chiari synd…

2006

Cirrhosis: CT and MR imaging evaluation

2006

In this article, we present the CT and MR imaging characteristics of the cirrhotic liver. We describe the altered liver morphology in different forms of viral, alcoholic and autoimmune end-stage liver disease. We present the spectrum of imaging findings in portal hypertension, such as splenomegaly, ascites and varices. We describe the patchy and lacelike patterns of fibrosis, along with the focal confluent form. The process of hepatocarcinogenesis is detailed, from regenerative to dysplastic nodules to overt hepatocellular carcinoma. Different types of non-neoplastic focal liver lesions occurring in the cirrhotic liver are discussed, including arterially enhancing nodules, hemangiomas and p…

Thrombophilic Genetic Factors PAI-1, MTHFRC677T, V Leiden 506Q, and Prothrombin 20210A in Noncirrhotic Portal Vein Thrombosis and Budd-Chiari Syndrom…

2013

Thrombophilic genetic factors PAI-1, MTHFRC677T, V Leiden 506Q, and Prothrombin 20210A were studied as risk factors in 235 Caucasian subjects: 85 patients with abdominal thrombosis (54 with portal vein thrombosis (PVT) and 31 with Budd-Chiari syndrome (BCS) without liver cirrhosis or hepatocellular carcinoma) and 150 blood bank donors. Seventy-five patients with PVT/BCS showed associated disease or particular clinical status (46 PVT/29 BCS): 37 myeloproliferative neoplasm (20 PVT/17 BCS), 12 abdominal surgery (10 PVT/2 BCS), 10 contraception or pregnancy (6 PVT/4 BCS), 7 abdominal acute disease (6 PVT/1 BCS), and 9 chronic disease (4 PVT/5 BCS); ten patients did not present any association …