A Síndrome de Budd-Chiari (SBC) acontece quando o fluxo de saída de sangue das veias do fígado é bloqueado, seja nas próprias veias do fígado ou na parte final da veia cava inferior.
Introdução
O que você precisa saber de cara
A Síndrome de Budd-Chiari (SBC) acontece quando o fluxo de saída de sangue das veias do fígado é bloqueado, seja nas próprias veias do fígado ou na parte final da veia cava inferior.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 12 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 27 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
3 genes identificados com associação a esta condição. Padrão de herança: Multigenic/multifactorial.
Non-receptor tyrosine kinase involved in various processes such as cell growth, development, differentiation or histone modifications. Mediates essential signaling events in both innate and adaptive immunity. In the cytoplasm, plays a pivotal role in signal transduction via its association with type I receptors such as growth hormone (GHR), prolactin (PRLR), leptin (LEPR), erythropoietin (EPOR), thrombopoietin receptor (MPL/TPOR); or type II receptors including IFN-alpha, IFN-beta, IFN-gamma and
Endomembrane systemCytoplasmNucleus
Calcium-binding chaperone that promotes folding, oligomeric assembly and quality control in the endoplasmic reticulum (ER) via the calreticulin/calnexin cycle. This lectin interacts transiently with almost all of the monoglucosylated glycoproteins that are synthesized in the ER (PubMed:7876246). Interacts with the DNA-binding domain of NR3C1 and mediates its nuclear export (PubMed:11149926). Involved in maternal gene expression regulation. May participate in oocyte maturation via the regulation
Endoplasmic reticulum lumenCytoplasm, cytosolSecreted, extracellular space, extracellular matrixCell surfaceSarcoplasmic reticulum lumenCytoplasmic vesicle, secretory vesicle, Cortical granuleCytolytic granule
Central regulator of hemostasis. It serves as a critical cofactor for the prothrombinase activity of factor Xa that results in the activation of prothrombin to thrombin
Secreted
Factor V deficiency
A blood coagulation disorder leading to a hemorrhagic diathesis known as parahemophilia.
Variantes genéticas (ClinVar)
410 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 227 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
44 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Síndrome Budd-Chiari
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Outros ensaios clínicos
9 ensaios clínicos encontrados, 3 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 1.088
Successful anesthetic management of >10-liter blood loss in a Budd-Chiari syndrome patient undergoing living donor liver transplantation: A case report and review of literature.
Budd-Chiari syndrome is an uncommon condition induced by thrombotic or nonthrombotic obstruction of the hepatic venous outflow and is characterized by hepatomegaly, ascites, abdominal pain. The successful anaesthetic and transfusion treatment of a patient with BCS who had a hepatic hydatid cyst following LDLT is described in this report. Venous obstruction and parasitic cysts together pose a significant perioperative risk, especially in cases of severe intraoperative bleeding. A 30 years old woman was admitted with weight loss, abdominal distension, recurrent haematemesis, and progressive jaundice. She had undergone multiple endoscopic variceal band ligations and treatment for pulmonary tuberculosis. Imaging revealed hepatic vein thrombosis, caudate lobe hypertrophy, and a hydatid cyst in segment VII that measured 5.8 × 4 cm. Anaemia (haemoglobin 7.6 g/dL) with preserved renal and coagulation function was found in the laboratory (MELD 14, Child-Turcotte-Pugh A6). LDLT was scheduled for her following multidisciplinary optimization. General anesthesia was achieved and then transesophageal echocardiography, arterial, central venous and PiCCO lines, were placed for invasive haemodynamic monitoring. The more than 10 liters of intraoperative blood loss were controlled by an organized massive transfusion protocol that used packed red blood cells, plasma, cryoprecipitate, and 2.9 litres of autologous blood through cell salvage. In order to preserve haemodynamic stability and keep mean arterial pressure above 70 mmHg, norepinephrine and vasopressin infusion were used. The patient showed stable graft function and recovered without any problems. Even in severe hemorrhagic episodes during LDLT for BCS, successful outcomes can be ensured by multidisciplinary coordination, advanced monitoring, and adherence to transfusion protocols.
Hepatic Angiosarcoma Associated with Chronic Schistosomiasis in a Patient with Decompensated Cirrhosis: A Rare Case Report.
Hepatic angiosarcoma (HA) is a rare endothelial malignancy with poor prognosis. Chronic Schistosoma infection causes periportal fibrosis and portal hypertension, but has not been established as a risk factor for hepatic vascular tumors. We report a case of high-grade HA coexisting with chronic schistosomiasis in a patient presenting with acute decompensated cirrhosis. A 64-year-old Filipino woman with no known chemical exposures presented with progressive abdominal pain, jaundice, ascites, and hepatic encephalopathy. Doppler ultrasound initially suggested Budd-Chiari syndrome, later excluded on advanced imaging. MRI showed multifocal hepatic lesions, and CT demonstrated multiple intra-abdominal hematomas without active bleeding. Liver biopsy confirmed poorly differentiated HA (CD31+/CD34+) and revealed calcified Schistosoma eggs. Her course was complicated by hemorrhagic shock after biopsy and paracentesis, spontaneous bacterial peritonitis, acute kidney injury, and worsening hepatic encephalopathy. She died despite aggressive supportive care. This case illustrates the diagnostic challenges and rapid progression of HA, especially in coagulopathic patients where biopsy carries significant risk. The coexistence of chronic schistosomiasis raises the possibility of parasitic inflammation contributing to hepatic vascular tumorigenesis. HA should be considered among unexplained hepatic masses with atypical imaging and clinical features.
Hepatic vein angioplasty and stenting in a paediatric patient with Budd-Chiari syndrome secondary to CHAPLE syndrome: is endovascular therapy the answer?
CHAPLE syndrome is a recently described genetic disorder characterised by dysregulation of the complement pathway, with the acronym standing for 'CD55 deficiency with complement hyperactivation, angiopathic thrombosis and protein-losing enteropathy'. Some patients develop thrombotic complications such as Budd-Chiari syndrome (BCS). Due to its rarity, management guidelines are lacking. We attempted endovascular angioplasty and stenting of the thrombosed right hepatic vein in a male child with BCS secondary to CHAPLE, diagnosed by symptoms and genetic testing. This case is potentially the first documented instance of CHAPLE syndrome associated with BCS in which endovascular therapy was used. The child was on enoxaparin post procedure, and although ascites and effusions initially improved, the stent occluded, necessitating repeat angioplasty and stenting 5 days later. Re-thrombosis occurred despite good intraoperative flow, leading to supportive care to manage symptoms. Our case shows limited benefits of endovascular therapy in such cases.
EASL Clinical Practice Guidelines on vascular diseases of the liver.
Vascular diseases of the liver include portal vein thrombosis (with or without cirrhosis), portosinusoidal vascular disorder, Budd-Chiari syndrome, sinusoidal obstruction syndrome, non-obstructive sinusoidal dilatation and peliosis, splanchnic artery aneurysms, and hepatic arteriovenous fistulas. Except for portal vein thrombosis in cirrhosis, these are all rare conditions. Since the last Clinical Practice Guidelines were issued by the European Association for the Study of the Liver in 2016, much data has been published on the diagnosis and management - medical and interventional - of patients with vascular liver diseases. Based on a thorough review of the relevant literature, recommendations are provided to address key clinical dilemmas. The document emphasises personalised care, considering individual risk factors and clinical presentation. Multidisciplinary management involving hepatologists, haematologists, pathologists, interventional radiologists and surgeons is essential in this area. Our aim is to provide guidance on the management of patients with vascular liver diseases based on the best available evidence.
Radiological Interventions in Pediatric Budd-Chiari Syndrome: Current Trends and Review of Literature.
Pediatric Budd-Chiari syndrome (BCS) is a rare but serious vascular disorder of the liver characterized by obstruction of the hepatic venous outflow leading to portal hypertension and liver dysfunction. Radiological endovascular interventions have revolutionized its management by providing minimally invasive options to restore venous patency and improve clinical outcomes. Early interventions are critical to prevent irreversible hepatic damage. Comparative studies highlight that endovascular therapies have high technical and clinical success with low complication rates. This review consolidates current evidence on the role of hepatic vein and inferior vena cava angioplasty, stenting, mechanical thromboaspiration, transjugular intrahepatic portosystemic shunt (TIPS), and direct intrahepatic portosystemic shunt (DIPS) in children with BCS. Doppler ultrasonography (US) remains the primary diagnostic modality, accurately localizing venous obstructions and guiding interventions. Post-procedural anticoagulation and surveillance with Doppler US are essential for long-term optimization. Novel techniques like 2D shear wave elastography enable non-invasive assessment of liver and splenic stiffness, reflecting fibrosis regression and hemodynamic improvement over time, and are being increasingly used for response assessment. This review underscores the evolving role of radiological endovascular techniques as first-line management for pediatric BCS, drawing upon established techniques and recent advancements to optimize patient outcomes.
Publicações recentes
Reno-hemiazygos venous connection identified on contrast-enhanced abdominal CT: a developmental variant.
Long-Term Efficacy and Outcomes of Endovascular Interventions in Budd-Chiari Syndrome-A Real World Analysis.
Impact of the COVID-19 Pandemic on Splanchnic Thrombosis Mortality: A United States National Analysis (2018-2023).
The application of Doppler ultrasound in evaluating angioplasty outcomes and predicting recurrence in Budd-Chiari syndrome.
[Diagnosis and treatment of Budd-Chiari syndrome (literature review)].
📚 EuropePMC2.558 artigos no totalmostrando 195
The application of Doppler ultrasound in evaluating angioplasty outcomes and predicting recurrence in Budd-Chiari syndrome.
European radiology[Diagnosis and treatment of Budd-Chiari syndrome (literature review)].
Angiologiia i sosudistaia khirurgiia = Angiology and vascular surgeryPrognostic scores for early mortality following transjugular intrahepatic portosystemic shunt insertion: external validation in a contemporary Australian cohort.
Internal medicine journalObliterative Hepatocavopathy: The "Acrobatic Dolphin" Mimic as Endovascular Procedure Guide.
JNMA; journal of the Nepal Medical AssociationSuccessful anesthetic management of >10-liter blood loss in a Budd-Chiari syndrome patient undergoing living donor liver transplantation: A case report and review of literature.
Pakistan journal of medical sciencesHepatocellular Carcinoma Associated with Hepatic Sarcoidosis and Budd-Chiari Syndrome: A Case Report and Review of the Literature.
Internal medicine (Tokyo, Japan)Ultrasound Characteristics of Budd-Chiari Syndrome - A Multi-Centre Analysis.
Digestive diseases (Basel, Switzerland)Transvenous Extrahepatic Portosystemic Shunts (TEPS): Intravascular Ultrasound-Guided Creation of Portocaval, Mesocaval, and Splenorenal Shunts.
Journal of vascular and interventional radiology : JVIRManagement of Splanchnic Venous Thrombosis: An Update.
HamostaseologieLiver Transplantation From a Recently Transplanted Multiorgan Patient: a Case Report.
Transplantation proceedingsBudd-Chiari syndrome in a young adult: Failed hepatic venoplasty and refusal of liver transplantation.
Radiology case reportsIatrogenic Hepatocellular Adenoma in Relation to an Unusual Complication of Cardiac Surgery.
JACC. Case reportsBudd-Chiari Syndrome Secondary to Essential Thrombocythaemia Complicated by Acquired Von Willebrand Disease and Mimicking Hepatic Malignancy: A Case Report.
Cureus[TIPS: current and innovative concepts].
Zeitschrift fur GastroenterologieHepatic Angiosarcoma Associated with Chronic Schistosomiasis in a Patient with Decompensated Cirrhosis: A Rare Case Report.
Case reports in gastroenterologyElevated serum erythropoietin level in JAK2+ polycythemia vera with small vessel Budd-Chiari syndrome.
BloodAcute Variceal Bleeding 11 Years After a Successful Mesoatrial Shunt for Budd-Chiari Syndrome: A Case Report.
CureusInferior Vena Cava-Atrial Anastomosis in Liver Transplant Recipient with Inferior Vena Cava Occlusion: A Case Report and Literature Review.
Journal of clinical medicineHepatic vein angioplasty and stenting in a paediatric patient with Budd-Chiari syndrome secondary to CHAPLE syndrome: is endovascular therapy the answer?
BMJ case reportsNon-Promyelocytic Acute Myeloid Leukaemia With Budd-Chiari Syndrome: A Rare Concurrence in a Child.
Pediatric blood & cancer[When should vascular liver disease be considered?].
La Revue du praticien[Venous diseases of the liver: what are we talking about?].
La Revue du praticien[Special features of anticoagulant treatment in vascular liver diseases].
La Revue du praticienEndovascular Treatment of a Giant Splenic Artery Aneurysm Associated with Budd-Chiari Syndrome.
European journal of vascular and endovascular surgery : the official journal of the European Society for Vascular SurgeryParoxysmal Nocturnal Hemoglobinuria-Driven Hepatic Vein Thrombosis: A Case of Budd-Chiari Syndrome in Disguise.
CureusEstablishment and Significance of a Nomogram Prediction Model for the Risk of Hepatocellular Carcinoma in Budd-Chiari Syndrome.
Journal of hepatocellular carcinomaMultidisciplinary collaboration successfully treated Budd Chiari syndrome complicated with hepatocellular carcinoma rupture and bleeding: A case report.
MedicineAmebic Liver Abscess: An Unusual Cause of Budd-Chiari Syndrome.
Case reports in infectious diseasesEarly Endovascular Intervention in Combination with Medical Therapy versus Medical Therapy Alone in the Initial Management of Patients with Primary Budd-Chiari Syndrome: A Randomized Controlled Trial.
Journal of vascular and interventional radiology : JVIRStepping Up Interventional Radiology Evidence in Budd-Chiari Syndrome.
Journal of vascular and interventional radiology : JVIRCongenital Afibrinogenemia With Coexisting Factor V Leiden Mutation Complicated by Budd-Chiari Syndrome: A Case Report.
CureusDelayed Massive Haemoptysis Following EBUS-TBNA Revealing a Pseudoaneurysm of an Ectopic Bronchial Artery: A Rare Complication at the Crossroads of Interventional Pulmonology and Radiology.
Respirology case reportsAblation of Atrial Tachycardia in a Budd-Chiari Syndrome Patient via Inferior Vena Cava-Azygos-Superior Vena Cava Approach.
Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, IncUnusual Etiology of Budd-Chiari Syndrome in an Adolescent: A Case of Combined Thrombophilic Disorder.
Clinical case reportsPathophysiology of Primary Budd-Chiari Syndrome: A Narrative Review.
CureusChronic abdominal pain: Budd-Chiari syndrome with converging pathologies.
Journal of vascular nursing : official publication of the Society for Peripheral Vascular NursingBudd-Chiari Syndrome: Presentation, Management, and Prognosis.
The American journal of gastroenterologyMalignant Right Atrial Mass With Inferior Vena Cava Extension Presenting as Budd-Chiari Syndrome: A Multimodality Imaging Case Report.
CureusUnveiling the Spectrum of Liver Endurance in Systemic Lupus Erythematosus: A Single-Center Experience From Kashmir, India.
CureusBeyond the Obstruction: Recognising Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Plasma Cell Disorder, and Skin Changes (POEMS) Syndrome in a Patient With Budd-Chiari Syndrome.
CureusCorrigendum to "Letter to the editor: "Budd-Chiari syndrome from pleomorphic sarcoma compressing the inferior vena cava: A case report" [Radiol Case Rep 21, (2026) 85-86].
Radiology case reportsAnalysis of risk factors for recurrence of Budd-Chiari syndrome: A retrospective study with zero-inflated model.
MedicineA Comparative Study of Ultrasonography and CT Venography in the Diagnosis of Budd-Chiari Syndrome.
Journal of clinical ultrasound : JCULiver failure due to metastatic melanoma: A case report.
World journal of clinical casesBudd-Chiari Syndrome: Update on Classification and Intravascular US.
Radiographics : a review publication of the Radiological Society of North America, IncInterventional Radiology Management of Budd-Chiari Syndrome: Current Concepts.
Techniques in vascular and interventional radiologyEASL Clinical Practice Guidelines on vascular diseases of the liver.
Journal of hepatologyCommon features of Budd Chiari syndrome in Sudanese population: a computed tomography-based review and descriptive analysis.
Frontiers in medicineRetrieval of ruptured balloon during hepatic venous balloon angioplasty using Dormia basket.
BMJ case reportsHyperhomocysteinemia: An Unusual Cause of Budd-Chiari Syndrome.
CureusBudd-Chiari syndrome: Prognostic scores, special populations, and management challenges.
World journal of gastroenterologyRecent advances of circular RNAs in gastrointestinal cancer.
World journal of clinical oncologyBudd-Chiari Syndrome in the Background of Chronic Kidney Disease: A Hypercoagulability-Driven Association or Coincidence? A Case Report.
Clinical case reportsPrimary Budd-Chiari syndrome due to lupus anticoagulant positive antiphospholipid syndrome.
BMJ case reportsRadiological Interventions in Pediatric Budd-Chiari Syndrome: Current Trends and Review of Literature.
Journal of clinical and experimental hepatologyEfficacy and Safety of Direct Oral Anticoagulants in Budd-Chiari Syndrome After Percutaneous Recanalization.
Hepatology research : the official journal of the Japan Society of HepatologyAyurvedic management of Budd Chiari Syndrome - A case report.
Journal of Ayurveda and integrative medicinePediatric Portal Hypertension Interventions: Ectopic Varices, Portal Vein Recanalization, Portosystemic Shunt Creation, and Maintenance of Surgical Shunts.
Seminars in interventional radiologyDiaphragmatic Ectopic Variceal Bleeding Secondary to Chronic Primary Idiopathic Budd-Chiari Syndrome.
Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological AssociationParoxysmal nocturnal hemoglobinuria revealed by atypical thromboses: A case of cerebral, hepatic, and portal vein involvement leading to cirrhosis.
Radiology case reportsModified Sharp Recanalization for Inferior Vena Cava Occlusion in Patient with Budd-Chiari Syndrome.
Interventional radiology (Higashimatsuyama-shi (Japan)The Role of Interventional Radiology for the Treatment of Liver CE and AE Lesions: Current Concepts.
Visceral medicineThe protective mechanism of taurine deoxycholic acid (TCDCA) through LPAR1 regulation in Buga syndrome.
European journal of medical researchEffect of Direct Oral Anticoagulants Versus Traditional Anticoagulation in Budd-Chiari Syndrome.
Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/HemostasisComparative efficacy and safety of endovascular intervention versus conservative therapy in Budd-Chiari syndrome: a retrospective cohort study.
BMC gastroenterologySelf-Expanding metal stent placement for hepatic venous outflow obstruction following ex vivo liver resection and autotransplantation.
Langenbeck's archives of surgeryLetter to Editor re:Comparison of DIPSS and other interventions in Budd-Chiari syndrome.
Indian journal of gastroenterology : official journal of the Indian Society of GastroenterologyBudd-Chiari Syndrome and Pregnancy-A Review.
Journal of clinical and experimental hepatology[Expert consensus on perioperative management of Budd-Chiari syndrome(2025 edition)].
Zhonghua wai ke za zhi [Chinese journal of surgery]Blocked Veins, Damaged Gut: Unraveling the Link Between Celiac Disease and Budd-Chiari Syndrome.
Digestive diseases and sciencesTriple Threat: SLE, APS, and Clotting Factor Abnormalities Leading to Subacute Budd-Chiari Syndrome. A Case Report and Review of the Literature.
Clinical medicine insights. Case reportsAccidental Fall Revealing an Atypical Presentation of a Rare Disease: A Case of Budd-Chiari Syndrome.
CureusMultisystemic Tuberculosis Masquerading as Aggressive Cardiac Tumor Causing Budd-Chiari Syndrome Disseminated to the Brain Resulting in Death of a Six-Year-Old Boy.
Pathogens (Basel, Switzerland)Clinical Characteristics and Outcomes of Adults with Budd-Chiari Syndrome: A Single-Center Experience in Colombia.
Digestive diseases (Basel, Switzerland)Liver Transplantation in Childhood: A 2-Year Single Center Experience.
Transplantation proceedingsRecognition of hemodynamic change at a brief glance in Budd-Chiari syndrome by 4D flow MRI.
Radiology case reportsAccessory hepatic vein recanalization: a viable approach in hepatic vein outflow tract obstruction.
The British journal of radiologyAggressive management and liver transplantation in Budd-Chiari syndrome secondary to Behçet's disease.
Oxford medical case reportsTransfemoral Hepatic Vein Puncture as a Nonpercutaneous Alternative Facilitating Anatomical Recanalization in Budd-Chiari Syndrome.
Journal of clinical and experimental hepatologyBudd-Chiari syndrome from pleomorphic sarcoma compressing the inferior vena cava: A case report.
Radiology case reportsA Novel Score for Predicting Long-Term Outcomes in Recanalisation-Treated Patients With Budd-Chiari Syndrome: A Multicentre Study.
Liver international : official journal of the International Association for the Study of the LiverCardiovascular risk assessment and predictors of cardiac decompensation after transjugular intrahepatic portosystemic shunt in patients with cirrhosis.
World journal of gastroenterologyAnticoagulant therapy for cancer-associated splanchnic vein thrombosis: Outcomes during a one-year follow-up period.
Thrombosis researchLong-term outcomes of transjugular intrahepatic portosystemic shunt in Budd-Chiari syndrome: A multicenter study.
Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the LiverBudd-chiari syndrome associated with acute promyelocytic leukemia: a case and literature review.
Hematology (Amsterdam, Netherlands)How I Do It: A Two-Stage Nephrectomy and Caval Thrombectomy for Renal Cell Carcinoma With Level IV Thrombus Complicated by Budd-Chiari Syndrome.
Journal of surgical oncologyHemodynamics and restenosis risk assessment in Budd-Chiari syndrome: A CFD study.
Technology and health care : official journal of the European Society for Engineering and MedicineVascular Liver Disease: Emerging Concepts in the Sinusoidal Space.
Clinics in liver diseaseInterventional Radiological Management for Budd-Chiari Syndrome: A 10-Year Retrospective, Multicenter Survey on 997 Patients in China.
Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological AssociationRole of Vascular Liver Diseases in Hepatocellular Carcinoma Development.
CancersPost-surgical Hepatic Herniation: An Exceptionally Rare Occurrence.
CureusHepatic sarcoidosis presenting as Budd Chiari syndrome leading to cirrhosis.
Radiology case reportsThoracoscopic Transthoracic Hepatectomy for Hepatocellular Carcinoma in Budd-Chiari Syndrome.
Asian journal of endoscopic surgeryInfantile Onset Budd Chiari Syndrome: Challenges and Outcome of Liver Transplantation After Radiological Interventions.
Journal of clinical and experimental hepatologyDevelopment and validation of a machine learning model based on multiple kernel for predicting the recurrence risk of Budd-Chiari syndrome.
Frontiers in physiologyThe Significance of Sound Touch Elastography of the Liver and Spleen in Assessing Treatment Efficacy in Budd-Chiari Syndrome.
Annali italiani di chirurgiaYttrium-90 radioembolization for advanced hepatocellular carcinoma with Budd-Chiari syndrome: A case report.
World journal of clinical oncologyDevelopment and validation of a radiomics-based prediction model for variceal bleeding in patients with Budd-Chiari syndrome-related gastroesophageal varices.
World journal of gastroenterologyA case report on the treatment of diaphragmatic eventration-induced Budd-Chiari syndrome: the first innovative application of diaphragm plication.
Frontiers in medicineNavigating the crossroads: exploring the intersection of celiac disease and Budd-Chiari syndrome - insights, challenges, and management strategies.
Annals of medicine and surgery (2012)Budd-Chiari Syndrome Treated With IVC Stenting With Subsequent Right Hepatic Vein to IVC Retrograde Sharp Recanalization.
Case reports in hepatologyBudd-Chiari Syndrome Secondary to Myelofibrosis in a Patient With Polycythemia Vera: A 16-Year Disease Progression Case Highlighting JAK2 Mutation Pathogenesis.
CureusSuccessful Deceased Donor Liver Transplantation with Median Sternotomy for Budd-Chiari Syndrome: A Case Report and Review of the Literature.
Surgical case reportsA Rare Case of Budd-Chiari Syndrome in a Young Female.
Clinical case reportscirc_0046599 Promotes HCC Progression by Competing with miR-1322 to Enhance NT5DC2 Expression.
Journal of CancerBudd-Chiari syndrome in a 33-year-old woman with hypercoagulable state: A case report.
Radiology case reportsObstruction of the hepatic veins-a rare case of Budd-Chiari syndrome: A case report.
SAGE open medical case reportsHepatic Congestive Perfusion Abnormality Due to Budd-Chiari Syndrome with Mid-Hepatic Vein Stenosis.
Journal of vascular and interventional radiology : JVIRSplanchnic vein thrombosis (2003-2022): a Swiss nationwide epidemiological study.
Thrombosis researchA case of Budd-Chiari syndrome with acute venous thrombosis and liver failure: highlighting the importance of early intervention and anticoagulation therapy.
Clinical journal of gastroenterologyBudd-Chiari Syndrome as the Presenting Feature of Systemic Immunoglobulin Light Chain Amyloidosis.
European journal of case reports in internal medicineA case report of hemothorax due to intrathoracic variceal rupture linked to Budd-Chiari syndrome.
BMC pulmonary medicineSevere abdominal wall varices and acute-on-chronic liver failure caused by the familial clustering of Budd-Chiari syndrome.
Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the LiverBudd-Chiari syndrome in a patient with antiphospholipid syndrome: hiding in plain sight.
BMJ case reportsBudd-Chiari Syndrome as an Initial Presentation of Essential Thrombocythemia.
CureusOutcomes of Hepatic Venoplasty in Budd-Chiari Syndrome.
Journal of the College of Physicians and Surgeons--Pakistan : JCPSPVascular liver disorders in patients with antiphospholipid syndrome: a national retrospective multicentre study.
Rheumatology (Oxford, England)Portal sinusoidal vascular diseases: Assessment and therapy.
Hepatology (Baltimore, Md.)The etiology of chronic splanchnic vein thrombosis in adults: a two-center analysis.
American journal of blood researchClinical characteristics and risk factors of hepatocellular carcinoma development in Budd-Chiari syndrome patients after endovascular treatment.
Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the LiverThe pringle maneuver for Budd-Chiari syndrome-related orthotopic liver transplantation: an experience from a large referral center.
BMC surgeryBudd Chiari syndrome associated with antiphospholipid syndrome: Clinical characteristics and prognosis of 17 patients from single center.
LupusA Rare Case of Piggyback Syndrome Following Orthotopic Liver Transplant.
Experimental and clinical transplantation : official journal of the Middle East Society for Organ TransplantationBudd-Chiari Syndrome after Living Liver Donor Hepatectomy.
Internal medicine (Tokyo, Japan)Comparison of direct intrahepatic portosystemic shunt and other major radiological interventions in patients with Budd-Chiari syndrome.
Indian journal of gastroenterology : official journal of the Indian Society of GastroenterologyPortal vein Recanalization via Paraumbilical vein with Direct Intrahepatic Portosystemic Shunt (PVR-DIPS) in Chronic Budd-Chiari Syndrome with Extrahepatic Portal Venous Obstruction.
Cardiovascular and interventional radiologyNext-Generation Sequencing Identifies Novel Germline Mutations in Patients with Budd-Chiari Syndrome-Associated Hepatocellular Carcinoma.
Digestive diseases and sciences[Differential diagnosis of hepatic lesions associated to alterations of liver vascularization : regenerative nodules, adenomas or hepatocellular carcinoma ?].
Revue medicale de Liege[The professor A. S. Brumberg, the founder of pathologoanatomy service of the Kursk Oblast].
Problemy sotsial'noi gigieny, zdravookhraneniia i istorii meditsinyCorrelation of shear wave elastography with liver biopsy in children with Chronic Liver disease.
Pakistan journal of medical sciencesHow to do: En bloc nephro-adrenalectomy with right hepatectomy and caval thrombectomy for adrenocortical carcinoma causing budd chiari syndrome.
ANZ journal of surgeryIntravenous leiomyomatosis presenting as Budd-Chiari syndrome: a case report and literature review.
Orphanet journal of rare diseasesLiver Transplantation for Children With Budd-Chiari Syndrome: A Case Report From Vietnam and Literature Review.
Pediatric transplantationHepatic Amyloidosis Manifesting as Budd-Chiari Syndrome: An Unusual Presentation.
CureusBudd-Chiari syndrome secondary to diaphragmatic hernia.
Anales de pediatriaA rare pediatric case: Budd-Chiari Syndrome and upper gastrointestinal bleeding in a 5-year-old boy.
International journal of surgery case reportsCombining Low-energy Images in Dual-energy Spectral CT With Deep Learning Image Reconstruction Algorithm to Improve Inferior Vena Cava Image Quality.
Journal of computer assisted tomographyThe Change in Liver Volume After Inferior Vena Cava and/or Hepatic Vein Venoplasty in Patients With Budd Chiari Syndrome With at Least One Patent Hepatic Vein Presenting With Ascites.
Journal of clinical and experimental hepatologyElectroconvulsive therapy in the treatment of catatonia in a patient with Budd Chiari syndrome: a case report.
NeurocaseBudd-Chiari Syndrome as an Initial Manifestation of Incomplete Systemic Lupus Erythematosus.
European journal of case reports in internal medicineCongestive myelopathy secondary to epidural venous engorgement and venous congestion in a patient with Budd-Chiari syndrome and its management through inferior vena cava stenting.
BMJ case reportsAspiration thrombectomy of the hepatic veins in Budd Chiari Syndrome.
Acta gastro-enterologica BelgicaBehçet's disease and factor V Leiden: A thrombogenic synergy causing budd-chiari syndrome.
Radiology case reportsDirect Oral Anticoagulants in Budd-Chiari Syndrome.
European journal of haematologyIatrogenic Budd-chiari Syndrome from Misplacement of Right Internal Jugular Central Vein Catheter: A Case Report.
Current medical imagingVideo-assisted repair of traumatic Budd-Chiari syndrome performed by mini-thoracotomy: A case report.
JTCVS techniquesAcute Onset Budd-Chiari Syndrome in the Postpartum Period: A Case of Missed Diagnosis Leading to Rapid Deterioration.
CureusSternotomy and extracorporal circulation for fulminant Budd-Chiari syndrome due to leiomyosarcoma of the inferior vena cava.
Cardio-oncology (London, England)A rare case of Behçet's disease complicated by Budd-Chiari syndrome and perforated duodenal ulcers in a young male.
Oxford medical case reportsThe histological grading of fibrosis in Budd-Chiari syndrome: A chronic liver disease, different from others.
Indian journal of gastroenterology : official journal of the Indian Society of GastroenterologyComplex Antiphospholipid Syndrome Successfully Controlled for 17 Years with Personalized Enoxaparin Therapy.
European journal of case reports in internal medicineSurgical Management of Infective Endocarditis Complicated by Budd-Chiari Syndrome.
CureusChronic Budd-Chiari syndrome as an initial manifestation of primary antiphospholipid syndrome in a young patient.
Polish archives of internal medicineEarly relaparotomy in recipients after living donor liver transplantation: causes, risk factors, and consequences.
Turkish journal of medical sciencesSystematic review of treatments for the gastrointestinal manifestations of systemic lupus erythematosus.
Seminars in arthritis and rheumatismRevealing an Exceptional Case of Budd-Chiari Syndrome.
CureusVeins Tell the Tale: Visible Clues of Budd-Chiari Syndrome.
The American journal of medicineNovel procedure for hepatic venous outflow block after liver resection: A case report.
World journal of clinical casesTransjugular Intrahepatic Portosystemic Shunt for Budd-Chiari Syndrome: A Single-Centre Experience.
Journal of clinical medicineA machine learning algorithm for the detection of paroxysmal nocturnal haemoglobinuria (PNH) in UK primary care electronic health records.
Orphanet journal of rare diseasesSystemic Amyloidosis Presenting as Budd-Chiari Syndrome: A Case Report.
Middle East journal of digestive diseasesGiant pedunculated liver hydatid cyst causing inferior vena cava syndrome: a case report.
Journal of medical case reportsGuidelines for the diagnosis and treatment of idiopathic portal hypertension, extrahepatic portal obstruction, and Budd-Chiari syndrome in Japan.
Hepatology research : the official journal of the Japan Society of HepatologyHepatocellular Carcinoma Associated with Secondary Budd-Chiari Syndrome.
Internal medicine (Tokyo, Japan)Application of liver biopsy in etiological diagnosis of unexplained portal hypertension: Porto-sinusoidal vascular disease should not be ignored.
MedicineUnusual case of liver cirrhosis presenting as a mass compressing the inferior vena cava: A case report.
Radiology case reportsInterventions in Budd-Chiari syndrome: an updated review.
Abdominal radiology (New York)Point-of-care ultrasound in the diagnosis and treatment of Budd-Chiari syndrome: A rare case report and literature review.
HeliyonBehçet's Syndrome With Multiple Bilateral Pulmonary Aneurysms Associated With Endomyocardial Fibrosis Presented With Pulmonary Emboli: A Case Report.
CureusLiver Transplantation for Budd-Chiari Syndrome From Myeloproliferative Neoplasms - Management and Long-Term Results.
Transplantation proceedings[Budd-Chiari syndrome and JAK2 gene mutation].
[Rinsho ketsueki] The Japanese journal of clinical hematologyHepatocellular carcinoma as a complication of chronic Budd-Chiari syndrome (BCS) in a child.
BMJ case reportsMembranous Caval Obliteration and "Showerhead"-like Venous Obstruction in Budd-Chiari Syndrome.
Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological AssociationCardiovascular Manifestations in Behçet's Disease.
Yonsei medical journalImaging findings of Budd-Chiari syndrome caused by intravenous leiomyomatosis: a case report.
Frontiers in medicineEffect of TIPS insertion on waitlist mortality and access to liver transplantation in Budd-Chiari syndrome.
Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation SocietyBudd-Chiari Syndrome Associated With Autoimmune Hemolytic Anemia, Immune Thrombocytopenic Purpura, and Lupus Nephritis: A Case Report.
CureusGut dysbiosis contributes to the development of Budd-Chiari syndrome through immune imbalance.
mSystemsA 27-Year-Old Female With JAK2 Mutation: A Case of Budd-Chiari Syndrome Secondary to Prolonged Oral Contraceptive Pill Use.
CureusStudy on the Analysis of Gender Trends Among the First Authors of Publications on Budd-Chiari Syndrome.
CureusBudd Chiari Syndrome: Radiological Aspects of the Disease Pertaining to Infertility and Role of Interventional Radiology in Successful Conception.
Journal of clinical and experimental hepatologyBudd-Chiari syndrome after BNT162b2 mRNA vaccination: two case reports.
European journal of hospital pharmacy : science and practiceA rare case of Gynura-segetum-related hepatic sinus obstruction syndrome complicated with alcoholic liver disease.
JPMA. The Journal of the Pakistan Medical AssociationFeasibility and long-term outcomes of hepatic vein recanalization in Budd-Chiari syndrome through intrahepatic collateral pathways.
European journal of gastroenterology & hepatologyBudd-Chiari syndrome following abdominal trauma.
Global cardiology science & practiceBudd-Chiari syndrome associated with congenital afibrinogenaemia reversed after orthotopic liver transplant.
BMJ case reportsA Clinical Case of Timely Diagnosis and Successful Treatment of Budd-Chiari Syndrome With Fulminant Cytolysis in the Setting of a First-time Diagnosed Myeloproliferative Disease.
Military medicineLiver Transplantation After Previous Vena Cava Resection: Case Report.
Transplantation proceedingsTEN-YEAR OUTCOMES OF TIPS FOR BUDD-CHIARI SYNDROME: SYSTEMATIC REVIEW AND META-ANALYSIS.
Arquivos de gastroenterologiaExperience of Performing Hepatic Interventional Radiological Procedures in a Tertiary Care Hospital in Odisha: A Case Series.
Journal of clinical and experimental hepatologyHepatic, gastric and bone marrow AL amyloidosis that began with Budd-Chiari syndrome: a case report.
Annals of hematologyMetabolic dysfunction-associated steatotic liver disease related cirrhosis and incidence of portal vein thrombosis.
European journal of gastroenterology & hepatologyManagement of non-cirrhotic variceal bleeding in pregnant women with Budd-Chiari syndrome in the early second trimester: A case report.
Narra JPercutaneous hepatic vein recanalization in pediatric Budd-Chiari syndrome: Comment.
Annals of pediatric cardiologySuccessful surgical management of cardiac tamponade following direct intrahepatic portosystemic shunt in a child with Budd-Chiari syndrome: A potentially life-threatening situation.
Annals of pediatric cardiologyAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Successful anesthetic management of >10-liter blood loss in a Budd-Chiari syndrome patient undergoing living donor liver transplantation: A case report and review of literature.
- Hepatic Angiosarcoma Associated with Chronic Schistosomiasis in a Patient with Decompensated Cirrhosis: A Rare Case Report.
- Hepatic vein angioplasty and stenting in a paediatric patient with Budd-Chiari syndrome secondary to CHAPLE syndrome: is endovascular therapy the answer?
- EASL Clinical Practice Guidelines on vascular diseases of the liver.
- Radiological Interventions in Pediatric Budd-Chiari Syndrome: Current Trends and Review of Literature.
- Reno-hemiazygos venous connection identified on contrast-enhanced abdominal CT: a developmental variant.
- Long-Term Efficacy and Outcomes of Endovascular Interventions in Budd-Chiari Syndrome-A Real World Analysis.
- Impact of the COVID-19 Pandemic on Splanchnic Thrombosis Mortality: A United States National Analysis (2018-2023).
- The application of Doppler ultrasound in evaluating angioplasty outcomes and predicting recurrence in Budd-Chiari syndrome.
- [Diagnosis and treatment of Budd-Chiari syndrome (literature review)].
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:131(Orphanet)
- OMIM OMIM:600880(OMIM)
- MONDO:0010947(MONDO)
- GARD:5968(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q183098(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
