Background
Budd–Chiari syndrome (BCS) is a rare disease characterized by hepatic venous outflow tract obstruction (HVOTO).
Methods
Recent literature has been analyzed for this narrative review.
...Results
Primary BCS/HVOTO is a result of thrombosis. The same patient often has multiple risk factors for venous thrombosis and most have at least one. Presentation and etiology may differ between Western and certain Eastern countries. Myeloproliferative neoplasms are present in 40% of patients and are usually associated with the V617F-JAK2 mutation in myeloid cells, in particular peripheral blood granulocytes. Presentation and symptoms vary, thus this diagnosis must be considered in any patient with acute or chronic liver disease. Doppler ultrasound, computed tomography, or magnetic resonance imaging of the hepatic veins and inferior vena cava usually successfully provide noninvasive identification of the obstruction or its consequences in the collaterals of hepatic veins or the inferior vena cava. The reported life expectancy in these patients is 3 years after the first symptoms. The therapeutic strategy includes first, anticoagulation, correction of risk factors, diuretics, and prophylaxis for portal hypertension, then angioplasty for short-length venous stenosis followed by transjugular intrahepatic portosystemic shunt (TIPS) and finally liver transplantation. The progression of treatment is based on the response to therapy at each step. This strategy results in a 5-year survival rate of nearly 85%. The medium-term prognosis depends upon the severity of liver disease, and the long-term outcome can be jeopardized by transformation of underlying conditions and hepatocellular carcinoma.
Conclusion
BCS/HVOTO hepatic manifestations of BCS/HVOTO can be controlled in most patients with medical or radiological interventions. Underlying disease has become the major determinant of patient outcome.
Primary Budd-Chiari syndrome is characterized by a blocked hepatic venous outflow tract at various levels from small hepatic veins to inferior vena cava, resulting from thrombosis or its fibrous ...sequellae. This rare disease affects mainly young adults. Multiple risk factors have been identified and are often combined in the same patient. Myeloproliferative diseases of atypical presentation account for nearly 50% of patients; their diagnosis can be made by showing the V617F mutation in Janus tyrosine kinase-2 gene of peripheral blood granulocytes and, should this mutation be absent, by showing clusters of dystrophic megacaryocytes at bone marrow biopsy. Presentation and manifestations are extremely varied, so that the diagnosis must be considered in any patient with acute or chronic liver disease. Doppler-ultrasound, computed tomography or magnetic resonance imaging of hepatic veins and inferior vena cava are usually successful in demonstrating non-invasively the obstacle or its consequences, the collaterals to hepatic veins or inferior vena cava. The disease is considered to be spontaneously lethal within 3 years of first symptoms. A therapeutic strategy has been proposed where anticoagulation, correction of risk factors, diuretics and prophylaxis for portal hypertension are used first; then angioplasty for short-length venous stenoses; then TIPS; and ultimately liver transplantation. Treatment progression is dictated by the response to previous therapy. This strategy has achieved 5-year survival rates approaching 90%. Medium-term prognosis depends on the severity of liver disease. Long-term outcome might be jeopardized by transformation of underlying conditions and hepatocellular carcinoma.
Primary Budd-Chiari Syndrome Garcia-Pagán, Juan Carlos; Valla, Dominique-Charles
The New England journal of medicine,
04/2023, Letnik:
388, Številka:
14
Journal Article
Summary To survey the burden of liver disease in Europe and its causes 260 epidemiological studies published in the last five years were reviewed. The incidence and prevalence of cirrhosis and ...primary liver cancer are key to understand the burden of liver disease. They represent the end-stage of liver pathology and thus are indicative of the associated mortality. About 0.1% of Hungarian males will die of cirrhosis every year compared with 0.001% of Greek females. WHO estimate that liver cancer is responsible for around 47,000 deaths per year in the EU. Harmful alcohol consumption, viral hepatitis B and C and metabolic syndromes related to overweight and obesity are the leading causes of cirrhosis and primary liver cancer in Europe. Chronic hepatitis B affects 0.5–0.7% of the European population. In the last decade the prevalence of chronic hepatitis C was 0.13–3.26%. It is of great concern that about 90% of people in Europe infected by viral hepatitis are unaware of their status. Available data suggest the prevalence rate of NAFLD is 2–44% in the general European population (including obese children) and 42.6–69.5% in people with type 2 diabetes. Each of these four major causes of liver disease is amenable to prevention and treatment, reducing the burden of liver disease in Europe and saving lives. Further surveys are urgently needed to implement cost-effective prevention programmes and novel treatments to tackle this problem.
In cirrhosis, portal vein thrombosis (PVT) could be a cause or a consequence of the progression of liver disease. We analyzed data from a prospective trial of ultrasound screening for hepatocellular ...carcinoma in order to identify risk factors for and the impact of PVT in patients with cirrhosis. In all, 1,243 adults with cirrhosis without PVT were enrolled from 43 liver units in France and Belgium between June 2000 and March 2006. The mean follow‐up was 47 months. Doppler ultrasonography was used to check the portal vein. Progression of liver disease was defined by the development of: ascites, hepatic encephalopathy, variceal bleeding, prothrombin <45%, serum bilirubin >45 μmol/L, albumin <28 g/L, and/or creatinine >115 μmol/L. G20210A prothrombin and factor V gene mutations were assessed in sera stored at three large centers. The 5‐year cumulative incidence of PVT was 10.7%. PVT was mostly partial and varied over time. The development of PVT was independently associated with baseline esophageal varices (P = 0.01) and prothrombin time (P = 0.002), but not with disease progression before PVT, or prothrombotic mutations. Disease progression was independently associated with baseline age (hazard ratio HR 1.55; 95% confidence interval CI: 1.11‐2.17), body mass index (HR 1.40; 95% CI: 1.01‐1.95), prothrombin time (HR 0.79; 95% CI: 0.70‐0.90), serum albumin (HR 0.97; 95% CI: 0.94‐0.99), and esophageal varices (HR 1.70; 95% CI: 1.21‐2.38) but not with the prior development of PVT (HR 1.32; 95% CI: 0.68‐2.65). Conclusion: In patients with cirrhosis, the development of PVT is associated with the severity of liver disease at baseline, but does not follow a recent progression of liver disease. There is no evidence that the development of PVT is responsible for further progression of liver disease. (Hepatology 2015;61:660‐667)
Sinusoidal obstruction syndrome Valla, Dominique-Charles; Cazals-Hatem, Dominique
Clinics and research in hepatology and gastroenterology,
09/2016, Letnik:
40, Številka:
4
Journal Article
Recenzirano
Odprti dostop
Summary Sinusoidal obstruction syndrome (SOS) is characterized by damage to small hepatic vessels affecting particularly sinusoidal endothelium. Damaged sinusoids can be associated with a partial or ...complete occlusion of small hepatic veins, hence the previous denomination of hepatic veno-occlusive disease (VOD). Exposure to certain exogenous toxins appears to be specific to this condition and is frequently included in its definition. Typical histopathological features of SOS in a liver biopsy specimen are presented in the text. The purpose of this article is to provide an overview on the different entities corresponding to this general definition. Such entities include: (i) liver disease related to pyrrolizidine alcaloids; (ii) liver injury related to conditioning for hematopoietic stem cell transplantation; (iii) vascular liver disease occurring in patients treated with chemotherapy for liver metastasis of colorectal cancer; and (iv) other liver diseases related to toxic agents.
Sinusoidal dilatation found in the absence of an impaired sinusoidal blood outflow has been so far of unclear significance. Sinusoidal dilatation may actually be a nonspecific feature of impaired ...portal venous blood inflow, whatever the cause, or a feature of severe systemic inflammatory reaction syndrome, whatever the cause. Sinusoidal dilatation is mainly located in the centrilobular area even in the absence of an outflow block. A predominantly periportal location is specifically found in oral contraceptive users, associated with an inflammatory condition. There is strong evidence for the association of sinusoidal dilatation and oxaliplatin‐based chemotherapy but not for estroprogestative steroids or thiopurine derivatives. Exposure to anabolic androgen steroids appears to cause sinusoidal changes different from a mere sinusoidal dilatation. Conclusion: There is evidence of activation of the interleukin‐6 and vascular endothelial growth factor pathways in sinusoidal dilatation, but the mechanisms linking the activation of these pathways with the microvascular changes must be identified. (Hepatology 2015;62:956–963)
The components of the hepatic vascular system (hepatic arteries, portal and hepatic veins, sinusoids, and lymphatics) can be damaged by various types of injury. Each of the resulting conditions is ...rare, which has limited knowledge and awareness. In the last two decades, international collaborations have allowed to reach critical masses of data, which has driven significant progresses in understanding and management of vascular disorders of the liver. The present paper discusses definitions, denominations, and diagnosis of such vascular disorders with the exception of those affecting hepatic arteries. Evolving pathogenic or pathophysiologic views relevant to the clinical aspects are also overviewed.
Hepatocellular adenomas (HCAs) are divided into genotype/phenotype subgroups associated with different evolutive profiles. Therefore, recognition of subtype is of clinical importance in patient ...management. Magnetic resonance imaging (MRI) is considered the most informative imaging modality and liver biopsy a key diagnostic tool whose role in HCA subtyping has never been extensively studied. The purpose of our study was to evaluate the diagnostic performance of MRI and liver biopsy with and without immunohistochemistry and to assess the interobserver agreement for MR classification in a consecutive series of resected HCAs. Forty‐seven HCAs with preoperative MRI and biopsy were retrospectively included. MRI data were reviewed independently by two abdominal radiologists blind to the pathological results and classification. Subtyping of HCAs on liver biopsy was made blindly to clinical, biological, and imaging data and to final classification. Routine histological analysis was based on morphological criteria and immunohistochemistry was systematically performed when enough tissue was available. Final subtyping of HCA was based on the examination of the surgical specimen. Radiologists correctly classified HCAs in 85%. The interobserver kappa correlation coefficient was 0.86. Routine histological analysis led to 76.6% of correct classification and 81.6% when immunophenotypical characteristics were available. The additional value of immunophenotypical markers is best in HCAs containing steatosis. Agreement between MRI findings and routine histological analysis was observed in 74.5%, leading to a likelihood ratio of subtype diagnosis higher than 20.Conclusion: MRI and biopsy analysis are two efficient methods in subtyping HCAs and their association increases the diagnosis confidence. Interobserver variability in MRI criteria is very low. (HEPATOLOGY 2011;)