Budd Chiari Syndrome: an unnoticed diagnosis

Falcão, Camila Kruschewsky; Fagundes, Gustavo F C; Lamos, Gustavo Checolli; Felipe-Silva, Aloísio; Lovisolo, Silvana Maria; Martines, João Augusto dos Santos; Campos, Fernando Peixoto Ferraz de

doi:10.4322/acr.2015.009

Cited by 5 publications

(3 citation statements)

References 18 publications

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“…In extreme cases, it can be life-threatening, especially when complications of advanced LC emerge. [ 24 ] Therapeutic strategies of LC caused by BCS are not the same as that caused by other common etiologies. A stepwise treatment has been recommended currently, from anticoagulation, thrombolysis to endovascular intervention, portosystemic shunt, and even liver transplantation.…”

Section: Discussionmentioning

confidence: 99%

Liver cirrhosis caused by chronic Budd–Chiari syndrome

Lin

Zhang²,

Wang³

et al. 2017

Medicine

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Chronic Budd–Chiari syndrome (BCS) is a rare cause of liver cirrhosis (LC) and tends to be misdiagnosed in clinical practice. In order to characterize LC caused by chronic BCS, we conducted this retrospective observational study. Medical records of all patients who were initially diagnosed as chronic BCS with LC when discharged from our department from January, 2011 to October, 2016 were reviewed. Cirrhotic patients with known causes and cases lacked key data were excluded. Data of remaining patients was collected and analyzed. A total of 15 cases were included in this study. Patients with LC caused by chronic BCS were characterized by preserved liver function and prominent portal hypertension (PH). Abdominal distention and edema of lower extremities were most common initial manifestations. Intra- or extrahepatic collaterals on imaging studies were of great importance for differential diagnosis. Most of these patients received interventional angioplasty followed by anticoagulation with warfarin and survived without obvious complications of PH. Chronic BCS was a rare but important cause of LC and should always be considered in patients with chronic liver disease and so-called cryptogenic LC. Early diagnosis and timely treatment may improve outcome. Correct interpretation of imaging examinations was fundamental to avoiding misdiagnosis.

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Section: Discussionmentioning

confidence: 99%

Liver cirrhosis caused by chronic Budd–Chiari syndrome

Lin

Zhang²,

Wang³

et al. 2017

Medicine

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“…The clinical presentation of BCS varies widely depending upon the extent as well as the site and rapidity of hepatic venous outflow obstruction. This causes varied degrees of liver involvement, resulting in about 20% of the patients having little to no symptoms at all[ 49 , 50 ]. Owing to the development of intrahepatic, extrahepatic, or portosystemic collaterals, these patients do not show any discernible signs of venous obstruction.…”

Section: Clinical Presentation Of Budd-chiari Syndromementioning

confidence: 99%

Budd-Chiari syndrome in myeloproliferative neoplasms: A review of literature

Găman¹,

Cozma²,

Manan³

et al. 2023

World J Clin Oncol

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Myeloproliferative neoplasms (MPNs) are defined as clonal disorders of the hematopoietic stem cell in which an exaggerated production of terminally differentiated myeloid cells occurs. Classical, Philadelphia-negative MPNs, i.e. , polycythemia vera, essential thrombocythemia and primary myelofibrosis, exhibit a propensity towards the development of thrombotic complications that can occur in unusual sites, e.g. , portal, splanchnic or hepatic veins, the placenta or cerebral sinuses. The pathogenesis of thrombotic events in MPNs is complex and requires an intricate mechanism involving endothelial injury, stasis, elevated leukocyte adhesion, integrins, neutrophil extracellular traps, somatic mutations ( e.g. , the V617F point mutation in the JAK2 gene), microparticles, circulating endothelial cells, and other factors, to name a few. Herein, we review the available data on Budd-Chiari syndrome in Philadelphia-negative MPNs, with a particular focus on its epidemiology, pathogenesis, histopathology, risk factors, classification, clinical presentation, diagnosis, and management.

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“…Surgical treatment has limited indications, but important, especially in the cases with obstruction at the level of IVC, which do not apply for endovascular treatment; nonetheless, the prognostic is often sombre. Orthotopic hepatic transplant represents the ideal therapy [7,8].…”

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confidence: 99%

Budd-Chiari Syndrome:Rare Cause, But Important of Portal Hypertension

G¹,

Pelin²,

Macovei³

et al. 2017

Rev. Chim.

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Budd-Chiari syndrome (BCS) (artery-occlusive hepatic disease) is a rare disease characterized by the obstruction of the blood flow at the level of the suprahepatic veins till their flow in the inferior vena cava (IVC) or at the level of IVC on the segment between the suprahepatic veins and the right atrium. The most frequent symptoms are abdominal pains, hepatomegaly and ascites. The imagistic investigations have an essential role in the early establishment of the diagnostic, evaluating the extension of disease and the management of BCS. Treatment depends on the presence or absence of symptoms and how acute the disease is.We present the case of a 43-years old woman, who had had for two month dyspeptic symptoms, increase of volume of the abdomen and oedema in the lower limbs. The biological investigations indicated hepatic dysfunction and thrombocytosis. The abdominal ultrasound showed modifications of chronic hepatopathy with signs of portal hypertension. The abdominal computer-tomography emphasized hepatomegaly with multiple nodules of regeneration, signs of portal hypertension (splenomegaly, moderate ascites), caudate lobe hypertrophy and thrombosis IVC. The patient was diagnosed with BCS and essential thrombocythemia. She started the medical treatment and was listed for liver transplantation. Budd-Chiari syndrome has to be taken into account every time we investigate the etiology of an acute or chronic hepatopathies, because the early diagnose can improve the patient�s prognostic.

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Budd Chiari Syndrome: an unnoticed diagnosis

Cited by 5 publications

References 18 publications

Liver cirrhosis caused by chronic Budd–Chiari syndrome

Liver cirrhosis caused by chronic Budd–Chiari syndrome

Budd-Chiari syndrome in myeloproliferative neoplasms: A review of literature

Budd-Chiari Syndrome:Rare Cause, But Important of Portal Hypertension

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