Managing Budd-Chiari Syndrome with Ayurvedic Treatment: A Case Report.

Ayurvedic therapy has been shown to be effective in treating various liver disorders. Budd-Chiari syndrome (BCS) is a rare but serious disorder characterized by obstruction of the hepatic venous outflow. The prognosis of patients is usually poor. We hereby present the case of a 42-year-old, obese female patient with BCS who was treated exclusively with herbo-mineral Ayurvedic medicines. This patient had inferior vena cava, portal vein, and hepatic vein thromboses with moderate liver fibrosis. The main line of treatment was the use of herbo-mineral compounds to remove the blood clots present in the above-mentioned veins. Ayurvedic treatment resulted in the restoration of health with normalization of liver function and regression of thromboses. This case study provides primary evidence of the probable potential of Ayurveda in improving therapeutic outcomes inpatients with BCS.

Direct intrahepatic portocaval shunt for treatment of portal thrombosis and Budd-Chiari syndrome.

Budd-Chiari syndrome (BCS) refers to hepatic venous outflow obstruction that in severe cases can lead to acute liver failure prompting consideration of revascularization or transplantation. Here, a 22 year old female with angiographically proven BCS secondary to JAK2/V617F positive Polycythemia vera on therapeutic warfarin presented with acute liver failure (ALF). Imaging revealed a new, near complete thrombotic occlusion of the main portal vein with extension into the superior mesenteric vein. An emergent direct intrahepatic portocaval shunt (DIPS) was created and liver function promptly normalized. She has been maintained on rivaroxaban since that time. Serial assessment over 1 year demonstrated continued shunt patency and improved flow in the mesenteric vasculature on ultrasound as well as normal liver function. DIPS is a viable alternative in the treatment of ALF from BCS when standard recanalization is not feasible. Improved blood flow may also improve portal/mesenteric clot burden. While further investigation is needed, new targeted anticoagulants may be viable as a long term anticoagulation strategy.

Clinical and prothrombotic profile of hepatic vein outflow tract obstruction.

OBJECTIVE: To study the clinical as well as prothrombotic profile and outcome of hepatic venous outflow tract obstruction in children. METHODS: This is a prospective study of a cohort of hepatic venous outflow tract obstruction (HVOTO) pediatric cases. All children and adolescents presenting with acute or chronic liver disease were screened for HVOTO with ultrasound and Doppler imaging and confirmed by multidetector computerised tomography (MDCT) with contrast enhancement. RESULTS: Of the 162 cases of chronic liver disease, 13 (7.4 %) were diagnosed to have HVOTO. Ascites and edema over the feet were the most prominent features. Anabolic steroids and herbal drugs were being taken by one case each. Six cases were diagnosed on Doppler and for rest 7 cases conclusive diagnosis was made on multidetector computerised tomography. Five out of 13 cases were heterogenous (CT) for mutation of the gene encoding methylene tetrahydrofolate reductase (MTHFR) and one case of these was also heterogenous for Factor Leiden V. One case was known celiac and developed HVOTO and was also found to be having hepatocellular carcinoma. Other causes were drug induced, pressure on inferior vena cava (IVC) and inferior vena cava (IVC) web. Thus the authors could find a prothrombotic cause for 10 out of 13 (76.9 %) cases. Three cases did not need any intervention. In one patient with infective thrombus of the IVC intervention was not planned. Six underwent angioplasty and 3 underwent transjugular intrahepatic portosystemic shunt. All were asymptomatic with improving growth parameters at follow up. CONCLUSIONS: Ascites, pedal edema, prominent abdominal veins and hepatomegaly should raise the suspicion of HVOTO in childhood liver disease. Majority of the cases would be harbouring a prothrombotic cause. MTHFR mutation was the commonest cause of HVOTO in the present study. Angioplasty and/or transjugular intrahepatic portosystemic shunt (TIPSS) can successfully treat HVOTO.

Budd-Chiari syndrome secondary to toxic pyrrolizidine alkaloid exposure.

In this report, we describe a case of pyrrolizidine alkaloid-related Budd-Chiari syndrome in Hong Kong. A 10-month-old boy presented with ascites, right pleural effusion, and hepatomegaly after consumption of herbal drinks for 3 months. His clinical (including imaging) features were compatible with Budd-Chiari syndrome. Budd-Chiari syndrome is a rare disease entity in paediatric patients. In our case, extensive workup performed to look for the underlying cause of Budd-Chiari syndrome was unrevealing, except for toxic pyrrolizidine alkaloid exposure in his herbal drinks.

Pivotal role of PAI-1 in a murine model of hepatic vein thrombosis.

Hepatic veno-occlusive disease (VOD) is a common complication of high-dose chemotherapy associated with bone marrow transplantation. While the pathogenesis of VOD is uncertain, plasminogen activator inhibitor-1 (PAI-1) has emerged as a diagnostic marker and predictor of VOD in humans. In this study, we investigated the role of PAI-1 in a murine model of VOD produced by long-term nitric oxide synthase inhibition using L-NAME. After 6 weeks, wild-type (WT) mice developed extensive fibrinoid hepatic venous thrombi and biochemical evidence of hepatic injury and dysfunction. In contrast, PAI-1-deficient mice were largely protected from the development of hepatic vein thrombosis. Furthermore, WT mice that received tiplaxtinin, an antagonist of PAI-1, were effectively protected from L-NAME-induced thrombosis. Taken together, these data indicate that NO and PAI-1 play pivotal and antagonistic roles in hepatic vein thrombosis and that PAI-1 is a potential target in the prevention and treatment of VOD in humans.

Effective hepatic artery chemoembolization for advanced hepatocellular carcinoma with extensive tumour thrombus through the hepatic vein.

BACKGROUND AND AIMS: Advanced hepatocellular carcinoma (HCC) with extensive tumour growth through the hepatic vein still has an extremely poor prognosis, even after cancer chemotherapy and/or transarterial embolization. Although aggressive surgical treatments using extracorporeal circulation and liver transplantation have been performed by some authors, the reported results were still unsatisfactory. In this study, we report the favourable result of hepatic artery chemoembolization and subsequent surgical resection in three patients with advanced HCC with extensive tumour thrombus through the hepatic vein. METHODS AND RESULTS: Three irresectable patients with HCC with extensive tumour thrombus through the hepatic vein underwent hepatic artery chemoembolization with aclarubicin, mitomycin C, lipiodol and/or Gelfoam. After the reduction of tumour extent with hepatic artery chemoembolization, two of the three patients underwent surgical resection. These two patients are still alive at 59 and 21 postoperative months, respectively. In the other case, the extent of the tumour and functional reserve of the liver prevented us from performing surgical resection, but the patient is doing well 62 months after the initial treatment. CONCLUSIONS: Hepatic artery chemoembolization with aclarubicin, mitomycin C, lipiodol and/or Gelfoam might be an effective treatment for irresectable advanced HCC with extensive tumour thrombus into the inferior vena cava or the right atrium through the hepatic vein. Radical surgical resection might be applicable for selected patients without high surgical risk after reducing tumour extent by hepatic artery chemoembolization.

Enfermedad hepática veno-oclusiva asociada a la ingestión de humanrripa (senecio tephrosioides) / Hepatic veno-oclusive disease associated to ingetion of huamanrripa (senecio tephrosioides)

Se reporta un caso de enfermedad veno-oclusiva del hígado (EHVO) en una mujer de 38 años de edad que había consumido ocasionalmente "huamanrripa" (Senecio tephrosioides) durante algunos años como antitusígeno. Fue hospitalizada por presentar dolor abdominal difuso, ictericia y anasarca durante 10 semanas. El estudio histológico de la biopsia hepática mostró marcada congestión a predominio centrolobulillar, focos de necrosis y en algunas áreas, inversión de la arquitectura del lobulillo hepático. En los siguientes 13 meses ha sido hospitalizada en 4 ocasiones por complicaciones de hipertensión portal. Este es el primer caso reportado de enfermedad hepática veno-oclusiva asociada a la ingesta de S. tephrosioides. Es muy probable que esta planta usada en la medicina tradicional peruana contenga alcaloides pirrolizidina, sustancias hepato- y nefrotóxicas que estan presentes en otras especies del género Senecio. Debido a la popularidad actual de las medicinas alternativas se espera un aumento en la incidencia de EHVO

Fatal veno-occlusive disease of the liver associated with herbal tea consumption and radiation.

A case is presented of fatal veno-occlusive disease of the liver in a patient undergoing treatment by whole abdomen irradiation for carcinoma of the ovary. She had also been exposed to herbal tea and chemotherapy, each of which may cause veno-occlusive disease in its own right. Interaction of these agents is postulated and should be considered before submitting patients to combination treatment.

[Veno-occlusive disease of the liver. Treatment by portocaval shunt]. / Maladie veino-occlusive du foie. Traitement par anastomose porto-cave.

A case of hepatic veno-occlusive disease revealed by abdominal pains and the rapid constitution of ascites in a 33-year old French female is reported. She had taken medicinal plants in order to loose weight. Liver biopsy showed typical histological changes consisting of centrizonal hemorrhagic necrosis and centrolobular vein obstruction with endophlebitis. Owing to an increase of ascites, a side-to-side portacaval shunt was performed. Eight months postoperatively, ascites had completely disappeared and the liver biopsy showed only a moderate centrolobular vein fibrosis. Five years later, the clinical state is normal.

A case of veno-occlusive disease of the liver in Britain associated with herbal tea consumption.

Veno-occlusive disease of the liver with clinical and pathological features similar to those of the condition occurring in the Caribbean is described in a young woman resident in Britain. The diagnosis was made from liver biopsies and hepatic venography and was confirmed at necropsy. Small amounts of pyrroliziding alkaloids were recovered from a sample of mate (Paraguay) tea, owned by the patient, to which she was addicted. It seems probable that the consumption of large amounts of this tea over a period of years was the cause of the hepatic disease.