1,721,089 research outputs found

    The role of thrombophilia in splanchnic vein thrombosis

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    In the last few years, the mechanistic role of thrombophilia due to hypercoagulability and of clonal disorders of hemopoiesis such as chromosome Philadelphia-negative chronic myeloproliferative disorders has been increasingly recognized in primary splanchnic vein thrombosis. As in deep venous thrombosis of the lower limbs, the frequent finding of several prothrombotic disorders in the same individual has led to the concept of primary splanchnic vein thrombosis as a multifactorial disease. Significant progress has been made in determining the molecular bases of inherited thrombophilia, and particularly in the identification of molecular markers of clonal disease in the so-called occult or latent myeloproliferative disorders. In this review article, the authors discuss the current knowledge on the role of thrombophilia in extrahepatic portal vein obstruction and in the Budd-Chiari syndrome, two of the most clinically relevant splanchnic vein thromboses. Copyrigh

    Therapeutic and clinical aspects of portal vein thrombosis in patients with cirrhosis

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    Portal vein thrombosis (PVT) is a frequent complication in cirrhosis, particularly in advanced stages of the disease. As for general venous thromboembolism, risk factors for PVT are slow blood flow, vessel wall damage and hypercoagulability, all features of advanced cirrhosis. Actually, the old dogma of a hemorrhagic tendency in cirrhosis has been challenged by new laboratory tools and the clinical evidence that venous thrombosis also occurs in cirrhosis. The impaired hepatic synthesis of both pro- and anticoagulants leads to a rebalanced hemostasis, more liable to be tipped towards thrombosis or even bleeding. Conventional anticoagulant drugs (low molecular weight heparin or vitamin K antagonists) may be used in cirrhosis patients with PVT, particularly in those eligible for liver transplantation, to prevent thrombosis progression thus permitting/facilitating liver transplant. However, several doubts exist on the level of anticoagulation achieved as estimated by coagulation tests, on the efficacy of treatment monitoring and on the correct timing for discontinuation in non-transplant candidates, while in transplant candidates there is expert consensus on continuing anticoagulation until transplantation. The recent introduction of direct acting oral anticoagulant drugs (DOACs) in other clinical settings generates much interest on their possible application in patients with cirrhosis and PVT. However, DOACs were not evaluated yet in patients with liver disease and cannot be recommended for the present time

    Diagnosis and monitoring of portal hypertension

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    Currently, oesophago-gastroduodenoscopy is the standard method to diagnose the presence of oesophago-gastric varices and to estimate the risk of bleeding. It is recommended that all patients undergo endoscopic screening for varices at the time when cirrhosis is diagnosed. After screening endoscopy, patients with medium or large varices should be treated to prevent bleeding, while all other patients should undergo periodic surveillance endoscopy. However, at a given point in time a variable proportion of patients will not have varices, since the prevalence of varices is variable. Thus, screening all cirrhotic patients with endoscopy to detect the presence of varices implies a number of unnecessary endoscopies. In recent years a wealth of new methods have been proposed as alternatives to conventional oesophago-gastroduodenoscopy for the non-invasive or minimally invasive diagnosis of oesophageal varices. Three of these methods (the platelet count/spleen diameter ratio, Fibrotest and Fibroscan) are truly non-invasive. Of these, the former is promising and needs a proper validation, Fibrotest appears to be insufficiently precise, while Fibroscan needs further evaluation. Multidetector CT oesophagography and capsule endoscopy are not entirely "non-invasive", since the first requires air insufflation into the oesophagus via an orally passed tube, and the latter requires swallowing the capsule. Multidetector CT oesophagography is promising, but needs further evaluation; capsule endoscopy is safe and reliable and might be proposed as an alternative to oesophago-gastroduodenoscopy in patients unable or unwilling to undergo oesophago-gastroduodenoscopy

    Abnormalities of hemostasis and bleeding in chronic liver disease : the paradigm is challenged

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    Chronic liver disease is characterized by a global hemostatic defect including platelet-vessel wall interaction (primary hemostasis), coagulation and fibrinolysis that may cause abnormalities of the relevant laboratory tests. The causal relationship between abnormal tests and bleeding has been widely accepted, despite the fact that abnormal tests are poorly associated with the timing and incidence of actual bleeding. In this article, we review recent evidence from the literature that disputes the above paradigm, and opens new venues for laboratory/clinical research and patient management in this field

    Cirrhosis and portal hypertension: the importance of risk stratification, the role of hepatic venous pressure gradient measurement

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    Portal hypertension is the main prognostic factor in cirrhosis. The recent emergence of potent antiviral drugs and new algorithm of treatment for the management of complications due to portal hypertension have sensibly changed our perception of cirrhosis that can be now considered as a multistage liver disease whose mortality risk can be reduced by a tailored approach for any stage of risk. Experts recommend to move toward a pathophysiological classification of cirrhosis that considers both structural and functional changes. The hepatic venous pressure gradient HVPG, is the reference gold standard to estimate the severity of portal hypertension in cirrhosis. It correlates with both structural and functional changes that occur in cirrhosis and carries valuable prognostic information to stratify the mortality risk. This article provides a general overview of the pathophysiology and natural course of cirrhosis and portal hypertension. We propose a simplified classification of cirrhosis based on low, intermediate and high mortality stage. The prognostic information provided by HVPG is presented according to each stage. A comparison with prognostic models based on clinical and endoscopic variables is discussed in order to evidence the additional contribute given by HVPG on top of other clinical and instrumental variables widely used in clinical practice
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