A doença veno-oclusiva hepática (DVO hepática) é uma condição resultante de lesão tóxica nos capilares sinusoidais hepáticos que leva à obstrução das pequenas veias hepáticas.
Introdução
O que você precisa saber de cara
A doença veno-oclusiva hepática (DVO hepática) é uma condição resultante de lesão tóxica nos capilares sinusoidais hepáticos que leva à obstrução das pequenas veias hepáticas.
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1/20kRara
1/10kPouco freq.
1/5kIncomum
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 16 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 51 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
1 gene identificado com associação a esta condição. Padrão de herança: Not applicable.
Transcription factor. May be a nuclear hormone receptor coactivator. Enhances transcription of genes with retinoic acid response elements (RARE)
Nucleus
Hepatic venoocclusive disease with immunodeficiency
Autosomal recessive primary immunodeficiency associated with hepatic vascular occlusion and fibrosis. The immunodeficiency is characterized by severe hypogammaglobulinemia, combined T and B-cell immunodeficiency, absent lymph node germinal centers, and absent tissue plasma cells.
Medicamentos e terapias
Mecanismo: Plasminogen activator
Mecanismo: Antithrombin-III activator
Variantes genéticas (ClinVar)
97 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 509 variantes classificadas pelo ClinVar.
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 — Doença veno-oclusiva hepática
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25 ensaios clínicos encontrados, 3 ativos.
Publicações mais relevantes
Transjugular intrahepatic portosystemic shunt improves survival in anticoagulation-resistant hepatic sinusoidal obstructive syndrome patients: A multicenter retrospective study.
Anticoagulation therapy is recommended during the acute or subacute stage for patients with pyrrolizidine alkaloid-hepatic sinusoidal obstruction syndrome (PA-HSOS). Transjugular intrahepatic portosystemic shunts (TIPS) is suggested as a step-up treatment when patients do not respond to anticoagulants. However, more evidence of the efficacy of TIPS is needed. To evaluate the effect of TIPS in these patients. Between January 2013 and September 2020, we retrospectively enrolled patients with PA-HSOS who did not respond to short-term anticoagulation therapy at four hospitals. The patients were divided into a TIPS treatment group and an anticoagulation therapy group. Baseline information and clinical characteristics were collected and recorded. Survival in both groups was the primary study endpoint and the risk factors for patient death were further analyzed. A total of 99 patients were enrolled according to the inclusion and exclusion criteria (63 in the TIPS group and 36 in the anticoagulation therapy group). There were 17 deaths during the median follow-up time of 32.5 months. Treatment, age, aspartate aminotransferase, and serum total bilirubin were independent risk factors for predicting death. The survival of patients in the TIPS group was significantly greater than that of patients in the continuing anticoagulation therapy group (P = 0.028). When stratified by the Drum-Tower Severity Scoring, in the TIPS group, mild and moderate patients had better outcomes than severe patients. TIPS can improve the transplant-free survival rate in patients with PA-HSOS who do not respond to short-term anticoagulation therapy, and patients with mild and moderate Drum-Tower Severity Scoring grade can benefit from TIPS.
Epidemiology and Outcomes of Hepatic Veno-Occlusive Disease after Allogeneic Hematopoietic Stem Cell Transplantation: A Descriptive Analysis of the U.S. National Inpatient Sample.
Hepatic veno-occlusive disease (HVOD), or sinusoidal obstruction syndrome (SOS), is a potentially liefe-threatening complication following allogeneic stem cell transplantation (allo-SCT). Historically, HVOD has affected up to 60% of allo-SCT recipients, with reported mortality rates exceeding 75% in severe cases. The approval of defibrotide in 2016 represented a major therapeutic milestone, significantly improving outcomes in high-risk patients. Despite this advancement, large-scale data on HVOD epidemiology & outcomes in allo-SCT recipients remain limited. This study aims to address these gaps & offer updated insights into its clinical impact. We utilized National Inpatient Sample (2016-2020) to examine factors associated with HVOD in allo-SCT recipients. Admissions for allo-SCT & HVOD were identified using International Classification of Diseases, Tenth Revision. Baseline characteristics, including patient demographics, hospital characteristics, comorbidities, complications & outcomes were compared between allo-SCT admissions with & without HVOD. Categorical variables were analyzed by χ² test (%), & continuous variables by adjusted Wald's test (mean±SD). During the study period, 200 allo-SCT admissions with HVOD were identified. HVOD admissions were younger (45.05 vs. 52.42 years; P=0.0045) & were more likely to be female (43.72% vs. 25%; P=0.0191). They had higher rates of acute graft-versus-host disease (GVHD) (17.5% vs. 6.47%; P=0.0043), acute respiratory failure (32.5% vs. 8.37%; P<0.001), & acute kidney injury (50% vs. 20.66%; P<0.001). Hepatic complications, including portal hypertension (7.5% vs. 0.52%; P<0.001), hepatic encephalopathy (12.5% vs. 0.28%; P<0.001), cirrhosis (7.5% vs. 0.07%; P<0.001), ascites (37.5% vs. 1.97%; P<0.001) & spontaneous bacterial peritonitis (2.5% vs. 0.06%; P<0.001) were also more common in this cohort. HVOD was associated with worse outcomes, including longer hospital stays (45.5 vs. 29.51 days; P<0.001) & over 6 times higher in-hospital mortality (30% vs. 4.72%; P<0.001). HVOD also imposed a greater economic burden, with more than double mean total hospitalization charges ($1,321,650 vs. $541,391.1; P<0.001) & nearly triple hospitalization costs ($358,463.2 vs. $141,464.1; P<0.001). This study describes HVOD-specific characteristics in allo-SCT admissions. Patients with HVOD were younger, had higher comorbidity burden, & showed increased rates of acute GVHD, multiorgan dysfunction, & hepatic complications. They also faced high in-hospital mortality, longer hospital stays, & greater healthcare costs.
Two Cases of Hepatic Epithelioid Hemangioendothelioma Misdiagnosed With Hepatic Veno-Occlusive Disease.
Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor of vascular origin with an incidence of < 0.1/100,000. The disease is easily misdiagnosed. The aim of this article is to increase public awareness and vigilance of HEHE. We report two cases presenting with fever, abdominal discomfort, abnormal liver function, and jaundice. Computed tomography (CT) and magnetic resonance imaging (MRI) of the abdomen suggested hepatic stasis, venous compression, and ascites, and liver biopsy was initially misdiagnosed as hepatic veno-occlusive disease (VOD), which did not improve with treatment. In the first case, a male patient, after liver transplantation, pathological and immunohistochemical (IHC) analyses revealed hyperplasia of blood vessels in the liver tissue with dilated lumen and heterogeneous cells. Immunohistochemistry was performed and showed CD34 positivity, confirming the diagnosis of HEHE. The second female patient had liver bruising and ascites on imaging, and the first hepatic puncture was reported to be VOD, which did not improve with treatment. Repeat hepatic puncture was performed, and the diagnosis of HEHE was confirmed after a second repathology with additional immunohistochemistry for HEHE. These misdiagnosis cases highlight the challenge of diagnosing HEHE. This is the first report of misdiagnosis of HEHE as VOD. This article analyzes the underlying causes of misdiagnosis of HEHE and emphasizes the causes of imaging misdiagnosis and the importance of repeated hepatic puncture biopsy and immunohistochemistry in the diagnosis of HEHE.
Real-World Outcome of Defibrotide Treatment for Severe Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome after Hematopoietic Cell Transplantation.
Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a serious complication following hematopoietic cell transplantation (HCT), with a markedly poor prognosis in patients with severe or very severe presentations. Defibrotide (DF) is the sole approved treatment for severe to very severe VOD/SOS, but real-world evidence remains limited, particularly in East Asian populations. We retrospectively analyzed 73 adult patients with severe or very severe VOD/SOS treated with DF between 2016 and 2023 at a single tertiary center in Korea. Diagnosis and grading were based on the revised European Society for Blood and Marrow Transplantation criteria. During the study period, national reimbursement guidelines for DF evolved from requiring ≥4 of 6 severity criteria to ≥2 of 5 severity criteria, allowing earlier initiation of treatment. The median time from HCT to diagnosis of VOD/SOS was 24 days (range, 1 to 843 days), and DF was administered within a median of 1 day after diagnosis. At DF initiation, 40 patients were classified as severe and 33 were classified as very severe; disease progression resulted in 53 patients reaching the very severe category. The overall response rate was 46.5%, and complete resolution was achieved in 39.7% of cases. The 100-day overall survival was 34.2%, significantly higher in the patients receiving DF within 2 days of diagnosis (36.0% versus 17.4%; P = .049). High total bilirubin levels at diagnosis and at the worst disease period were strongly associated with poor long-term survival regardless of severity criteria. These data provide real-world support for the early use of DF and suggest the prognostic utility of total bilirubin level in guiding treatment for VOD/SOS following HCT.
Impact of Oral Eicosapentaenoic Acid on Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome Onset Prevention: Single-Center Retrospective Analysis.
Veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a serious complication associated with allogeneic hematopoietic stem cell transplantation (allo-HSCT). Although ursodeoxycholic acid and defibrotide can reduce the risk of VOD/SOS, preventive efforts remain insufficient. In 2009, we began prescribing oral eicosapentaenoic acid (EPA) to mitigate post-transplant complications. Notably, no patients receiving EPA developed VOD/SOS. In this retrospective study, we analyzed 138 transplants from 123 patients who underwent allo-HSCT at Ehime University Hospital between April 2008 and June 2024. Ninety-one patients received EPA after allo-HSCT (EPA group), while 47 patients did not (no-EPA group). EPA was administered at a dose of 1800 mg/d, divided into 2 or 3 doses. All patients were taking ursodeoxycholic acid. VOD/SOS occurred in 3 patients in the no-EPA group (6.5%) and in none of the EPA group (P = 0.014). Early post-transplant mortality within 100 d did not differ significantly between groups. Although causal inference is limited by the retrospective design of the study, our findings suggest that EPA use may be associated with a lower incidence of VOD/SOS. These findings warrant prospective, multicenter trials to determine whether EPA can improve transplant outcomes by reducing the incidence of VOD/SOS.
Publicações recentes
[Clinical Analysis of Allogeneic Hematopoietic Stem Cell Transplantation for Very Early-Onset Inflammatory Bowel Disease Caused by IL-10RA Mutation].
Transjugular intrahepatic portosystemic shunt improves survival in anticoagulation-resistant hepatic sinusoidal obstructive syndrome patients: A multicenter retrospective study.
🥇 Meta-análiseEpidemiology and Outcomes of Hepatic Veno-Occlusive Disease after Allogeneic Hematopoietic Stem Cell Transplantation: A Descriptive Analysis of the U.S. National Inpatient Sample.
Two Cases of Hepatic Epithelioid Hemangioendothelioma Misdiagnosed With Hepatic Veno-Occlusive Disease.
Proteomic Signatures and Early Biomarkers Predicting Veno-Occlusive Disease in Pediatric Patients Undergoing Haploidentical Hematopoietic Cell Transplantation with Busulfan-Based Conditioning and Post-Transplant Cyclophosphamide.
📚 EuropePMC361 artigos no totalmostrando 198
[Clinical Analysis of Allogeneic Hematopoietic Stem Cell Transplantation for Very Early-Onset Inflammatory Bowel Disease Caused by IL-10RA Mutation].
Zhongguo shi yan xue ye xue za zhiTransjugular intrahepatic portosystemic shunt improves survival in anticoagulation-resistant hepatic sinusoidal obstructive syndrome patients: A multicenter retrospective study.
World journal of hepatologyHepatic Sinusoidal Obstruction Syndrome Induced by Pyrrolizidine Alkaloids from Gynura segetum: Mechanisms and Therapeutic Advances.
Molecules (Basel, Switzerland)Gancao decoction ameliorated senecionine-induced Hepatic Sinusoidal Obstruction Syndrome in mice by inhibiting NET formation and senecionine bioactivation in liver.
Journal of ethnopharmacologyEpidemiology and Outcomes of Hepatic Veno-Occlusive Disease after Allogeneic Hematopoietic Stem Cell Transplantation: A Descriptive Analysis of the U.S. National Inpatient Sample.
Hematology/oncology and stem cell therapyTwo Cases of Hepatic Epithelioid Hemangioendothelioma Misdiagnosed With Hepatic Veno-Occlusive Disease.
Case reports in hepatologyChronic portal hypertension following inotuzumab ozogamicin in a low-risk patient: a case of overriding baseline risk and long-term management.
Annals of hematology[Research progress on animal models relevant to porto-sinusoidal vascular disease].
Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatologyProteomic Signatures and Early Biomarkers Predicting Veno-Occlusive Disease in Pediatric Patients Undergoing Haploidentical Hematopoietic Cell Transplantation with Busulfan-Based Conditioning and Post-Transplant Cyclophosphamide.
Transplantation and cellular therapyReal-world treatment outcome and toxicity analysis of inotuzumab ozogamicin in adult patients with relapsed or refractory B-cell precursor acute lymphoblastic leukemia: multicenter prospective observational study.
Therapeutic advances in hematologyEffects of glutathione- S -transferase polymorphisms on intravenous busulfan in hematopoietic stem cell transplant patients: a meta-analysis.
Pharmacogenetics and genomicsTransjugular intrahepatic portosystemic shunt for hepatic sinusoidal obstruction syndrome with primary biliary cholangitis and alcoholic liver disease: a case report.
Frontiers in medicine[Inotuzumab ozogamicin-associated sinusoidal obstruction syndrome/veno-occlusive disease diagnosed by transjugular liver biopsy].
[Rinsho ketsueki] The Japanese journal of clinical hematologyModeling antithymocyte globulin-induced microvasculopathy using human iPSC-derived vascularized liver organoids.
Cell reports. MedicineNon-caucasian ethnicity as a risk factor for sinusoidal obstruction syndrome/hepatic veno-occlusive disease following haematopoietic stem cell transplantation.
Anales de pediatriaCommunity perceptions and practices on hepatic veno-occlusive disease in Tigray, Ethiopia: An explorative study challenging the attribution to Ageratum conyzoides.
PLoS neglected tropical diseasesReal-World Outcome of Defibrotide Treatment for Severe Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome after Hematopoietic Cell Transplantation.
Transplantation and cellular therapyImpact of Oral Eicosapentaenoic Acid on Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome Onset Prevention: Single-Center Retrospective Analysis.
Transplantation and cellular therapyEfficacy of ibrutinib and diagnostic liver biopsy for idiopathic refractory ascites after allogeneic stem cell transplantation.
International journal of hematologyPrognostic Impact of the Level of Minimal Residual Disease Prior to Allogeneic Hematopoietic Stem Cell Transplantation on Pediatric Patients With Acute Lymphoblastic Leukemia: A 10-Year Single-Center Study.
Clinical lymphoma, myeloma & leukemiaVeno-Occlusive Disease in Very Young Children: A Profile of High Morbidity With Low Mortality.
Pediatric blood & cancerMultiple Hematopoietic Stem Cell Transplantations in Pediatric Acute Myeloid Leukemia: Prognostic Significance of Remission and Severe Sinusoidal Obstruction Syndrome.
Pediatric blood & cancerTransient and Shear-Wave Elastography in the Detection of Hepatic Sinusoidal Obstruction Syndrome in Patients Undergoing Hematopoietic Stem Cell Transplantation: A Systematic Review and Meta-Analysis.
Transplantation and cellular therapySerum ascites albumin gradient in predicting the severity of hepatic sinusoidal obstruction syndrome induced by pyrrolizidine alkaloids.
Clinical and experimental hepatologyEfficacy and safety of transjugular intrahepatic portosystemic shunt in hepatic sinusoidal obstruction syndrome: systematic review and meta-analysis.
Frontiers in medicineSyndecan-1 is Associated With Sinusoidal Obstruction Syndrome and Severe Acute Graft-Versus-Host Disease After Pediatric Hematopoietic Stem Cell Transplantation.
Pediatric blood & cancerAlbumin-Bilirubin and Severity as Key Factors of Recovery in Patients With PA-HSOS Undergoing Transjugular Intrahepatic Portosystemic Shunt.
Annali italiani di chirurgiaAntibody-mediated rejection without elevated anti-blood type antibody after ABO-incompatible living donor liver transplantation: a case report.
Clinical journal of gastroenterologyGenetic Susceptibility in Sinusoidal Obstruction Syndrome/Veno-Occlusive Disease: A Case-Control Study.
International journal of molecular sciencesDefibrotide for Protecting Against and Managing Endothelial Injury in Hematologic Malignancies and COVID-19.
BiomoleculesDrug-induced sinusoidal obstruction syndrome: a real-world pharmacovigilance study based on the FDA Adverse Event Reporting System (FEARS).
BMC pharmacology & toxicologyVascular Liver Disease: Emerging Concepts in the Sinusoidal Space.
Clinics in liver diseaseHepatotoxicity of Chemotherapy and Immune Checkpoint Inhibitors.
Clinics in liver diseaseThe Liver in Hematologic Malignancies: Lymphoma, Leukemia, and Myeloma.
Clinics in liver diseaseResults of a Phase II Trial for Intermediate-Risk Rhabdomyosarcoma Treatment Protocol JRS-I: A Report From the Japan Rhabdomyosarcoma Study Group.
Pediatric blood & cancerUnveiling the clinical spectrum of Porto-Sinusoidal Vascular Disorder (PSVD) with chronic HBV infection: insights from a retrospective Chinese cohort.
Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the LiverPU.1 aggravates hepatic sinusoidal obstruction syndrome by upregulating PTBP1 and activating the Wnt/β-catenin pathway.
Histology and histopathologyUtility of tolvaptan sodium phosphate for refractory fluid retention in post-transplant sinusoidal obstruction syndrome.
International journal of hematologyPlatelet aggregation elicits FasL expression and hepatocyte apoptosis in sinusoidal obstruction syndrome.
Scientific reportsSinusoidal Obstruction Syndrome After Allogeneic Hematopoietic Cell Transplantation With Post-Transplant Cyclophosphamide.
Transplantation and cellular therapyClassical and Late-Onset SOS/VOD After Allogeneic HSCT: A Japanese Transplant Registry Analysis.
American journal of hematologyValidation of Drum Tower Severity Scoring (DTSS) System for Pyrrolizidine Alkaloid-Induced Hepatic Sinusoidal Obstruction Syndrome.
Journal of digestive diseasesHepatic sinusoidal congestion associated with inotuzumab therapy in patients with B-cell acute lymphoblastic leukemia, a proposal for a new clinical entity: Calicheamicin syndrome.
Journal of oncology pharmacy practice : official publication of the International Society of Oncology Pharmacy PractitionersUltrasonographic scoring system for SOS/VOD in pediatric hematopoietic stem cell transplant recipients.
International journal of hematologyManagement of liver sinusoidal obstruction syndrome/veno-occlusive disease in adults: a 2025 perspective from an international expert group.
Bone marrow transplantationAtypical features of hepatic veno-occlusive disease/sinusoidal obstruction syndrome after inotuzumab ozogamicin in adult patients with acute lymphoblastic leukemia.
Blood researchDiagnostic accuracy of liver stiffness measurement for the diagnosis of veno-occlusive disease/sinusoidal obstruction syndrome after hematopoietic stem cell transplantation (HSCT), the ELASTOVOD STUDY: an investigator-initiated, prospective, multicentre diagnostic clinical trial.
Bone marrow transplantationS-Adenosylmethionine Inhibits Plasminogen-Activating Inhibitor-1 and Protects Male Mice From FOLFOX-Induced Liver Injury.
Cellular and molecular gastroenterology and hepatologyOperational procedure sharing pathway in veno-occlusive disease: a Delphi consensus-based recommendations.
Frontiers in oncologySoluble Thrombomodulin Is Associated With Endothelial Dysfunction Syndromes After Pediatric Hematopoietic Stem Cell Transplantation.
Pediatric blood & cancerCIBMTR Registry Data on Inotuzumab Ozogamicin Treatment in Patients with ALL Who Proceeded to Hematopoietic Stem Cell Transplant-A Podcast.
Targeted oncologyInhibition of MALT1 Protease Attenuates Hepatic Sinusoidal Obstruction Syndrome by Modulating NRF2/HO1 and NF-κB Pathway.
Liver international : official journal of the International Association for the Study of the LiverComplement activation and vascular complications after pediatric allogeneic hematopoietic stem cell transplantation.
Scientific reports[A case report of gastric cancer combined with hepatic sinusoidal obstruction syndrome].
Zhonghua wei chang wai ke za zhi = Chinese journal of gastrointestinal surgeryEfficacy and safety of currently approved and lower starting doses of inotuzumab ozogamicin in adult patients with relapsed or refractory acute lymphoblastic leukemia: a phase IV study.
HaematologicaPretransplant Minimal Pleural and Peritoneal Effusion Is a Potential Poor Prognostic Indicator in Allogeneic Hematopoietic Stem Cell Transplantation.
Clinical transplantationA case of late-onset sinusoidal obstruction syndrome diagnosed histologically before meeting diagnostic criteria.
International journal of hematologyDrug-induced hepatic sinusoidal obstruction syndrome: current advances and future perspectives.
Archives of toxicologyA Two-Center Study of a Prognostic Model Related to Acute Kidney Injury After Allogeneic Hematopoietic Stem Cell Transplantation in Children: Development of a New Predictive Dynamic Nomogram.
Pediatric blood & cancerPerioperative outcomes of sinusoidal obstruction syndrome in patients undergoing liver resection for colorectal metastases after neoadjuvant chemotherapy: a retrospective cohort research.
Annals of surgical treatment and researchThe EASIX score as a predictor of sinusoidal obstruction syndrome and nonrelapse mortality in paediatric patients receiving allogeneic haematopoietic stem cell transplantation.
Bone marrow transplantationContinuous Kidney Replacement Therapy in Children With Sinusoidal Obstruction Syndrome After Hematopoietic Cell Transplant: Outcome and Liberation.
Pediatric blood & cancerBurden of illness of non-hematopoietic stem cell transplant-related hepatic sinusoidal obstruction syndrome: A systematic review.
HeliyonPrognostic Factors Associated With Increased Mortality in Pediatric Veno-Occlusive Disease Following Hematopoietic Cell Transplantation.
Clinical transplantationPotential therapeutic uses of L-citrulline beyond genetic urea cycle disorders.
Journal of inherited metabolic diseasePorto-sinusoidal vascular disorder with known etiologies had more severe portal hypertension and poorer outcomes.
Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver[Rifaximin curative effect and mechanism on monocrotaline-induced hepatic sinusoidal obstruction syndrome in mice].
Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatologyThe benefits of prophylactic defibrotide: Are the tides turning?
Pediatric blood & cancer[Clinical analysis of allogeneic hematopoietic cell transplantation in 9 patients with hematological malignancies complicated by Gilbert's syndrome].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhiAn international survey to better understand the current incidence, severity, and management of VOD/SOS.
Bone marrow transplantation[Sinusoidal obstruction syndrome/veno-occlusive disease: a complication to overcome].
[Rinsho ketsueki] The Japanese journal of clinical hematologyHepatic sinusoidal obstruction syndrome after immune-checkpoint-inhibitor therapy: when the liver sends SOS.
BMJ case reportsEarly defibrotide therapy and risk factors for post-transplant veno-occlusive disease/sinusoidal obstruction syndrome in childhood.
Pediatric blood & cancerRisk Factors for Sinusoidal Obstruction Syndrome After Hematopoietic Stem Cell Transplantation in Children and Young Adults: A Systematic Review and Meta-Analysis.
Clinical transplantation[Application Values of New Ultrasound Technologies in the Diagnosis of Hepatic Sinusoidal Obstruction Syndrome].
Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae SinicaeBiomarker-derived fast-and-frugal decision tree for preemption of veno-occlusive disease/sinusoidal obstructive syndrome.
Blood advancesHepatic veno-occlusive disease with asparaginase products: a review of cases reported to the FDA adverse event reporting system and published in the literature.
Pediatric hematology and oncologyRefractory Thrombocytopenia is the Earliest Diagnostic Criterion for Sinusoidal Obstruction Syndrome in Children.
Journal of pediatric hematology/oncologyIntrinsic Causes of Nonfibrotic Portal Hypertension-A Clinicopathologic Review of 56 Patients.
International journal of surgical pathologyHepatic Sinusoidal Disorders.
Radiographics : a review publication of the Radiological Society of North America, IncLiver elastography for risk-assessment of liver toxicity and risk factors for Sinusoidal obstruction syndrome in patients with acute lymphoblastic leukemia receiving inotuzumab ozogamicin.
Blood cancer journalInotuzumab ozogamicin in adult acute lymphoblastic leukemia: Development, current status, and future directions.
CancerANZTCT practice statement: sinusoidal obstruction syndrome/veno-occlusive disease diagnosis and management.
Internal medicine journalHepatic Veno-Occlusive Disease and Colorectal Cancer: Expect the Unexpected.
Life (Basel, Switzerland)A rare case of Gynura-segetum-related hepatic sinus obstruction syndrome complicated with alcoholic liver disease.
JPMA. The Journal of the Pakistan Medical Association[Porto sinusoidal vascular disease: an unusual cause of digestive bleeding].
Revista de gastroenterologia del Peru : organo oficial de la Sociedad de Gastroenterologia del PeruGut microbiota promotes macrophage M1 polarization in hepatic sinusoidal obstruction syndrome via regulating intestinal barrier function mediated by butyrate.
Gut microbesHemostasis and complement in allogeneic hematopoietic stem cell transplantation: clinical significance of two interactive systems.
Bone marrow transplantationVerification of the Prediction Accuracy of a Biomarker-Based Prognostic for Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome (VOD/SOS) After Hematopoietic Cell Transplantation (HCT).
Transplantation and cellular therapyIncidence, Etiology and Prognosis of Initial Liver Injury After Allogeneic Hematopoietic Stem Cell Transplantation: A Multi-Center Retrospective Study.
Transplantation proceedingsPorto-sinusoidal Vascular Disease and Portal Hypertension.
Clinics in liver diseaseTacrolimus-Induced Hepatic Vein Occlusive Disease After Deceased Donor Liver Transplantation: A Case Report.
Transplantation proceedingsSinusoidal Obstruction Syndrome/Veno-Occlusive Disease after Unmanipulated Haploidentical Hematopoietic Stem Cell Transplantation with Post-Transplantation Cyclophosphamide: A Study on Behalf of the Spanish Hematopoietic Stem Cell Transplantation and Cellular Therapy Group (GETH).
Transplantation and cellular therapyReview of imaging findings in hepatic veno-occlusive disease.
European journal of radiologyVeno-Occlusive Disease: A Life-saving Novel Approach With Plasma Exchange, IVIG, and Steroid, Without Defibrotide.
Journal of pediatric hematology/oncologySepsis and pneumonia caused by Enterococcus faecium following liver transplantation treated with contezolid as the first-line therapy.
Journal of global antimicrobial resistance6-Mercaptopurine-associated Sinusoidal Obstructive Syndrome During Interim Maintenance I: A Case Report.
Journal of pediatric hematology/oncologyShear wave elastography and dispersion imaging for hepatic veno-occlusive disease prediction after pediatric hematopoietic stem cell transplantation: a feasibility study.
Pediatric radiologyFunctional metabolomics characterizes the contribution of farnesoid X receptor in pyrrolizidine alkaloid-induced hepatic sinusoidal obstruction syndrome.
Archives of toxicologyLiver stiffness measurements predict Sinusoidal Obstructive Syndrome after hematopoietic stem cell transplantation.
Bone marrow transplantationDiagnosing and Grading of Sinusoidal Obstructive Syndrome after Hematopoietic Stem Cell Transplant of Children, Adolescent and Young Adults treated in a Pediatric Institution with Pediatric Protocols.
Transplantation and cellular therapy[Role of liver sinusoidal endothelial cell damage in the developmental process of hepatic sinusoidal obstruction syndrome: a focus on the research progress of immune inflammatory mechanisms].
Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatologyEvaluation of Circulating Endothelial Cells as Direct Marker of Endothelial Damage in Allo-Transplant Recipients at High Risk of Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome.
Transplantation and cellular therapyHepatic veno-occlusive disease complicated with extreme hyperammonaemia (920 µmol/L) treated with defibrotide, lactulose, rifampin and haemodialysis.
BMJ case reportsAutotaxin is a potential predictive marker for the development of veno-occlusive disease/sinusoidal obstruction syndrome after allogeneic hematopoietic cell transplantation.
Annals of hematologyGynura segetum induces hepatic sinusoidal obstruction syndrome in a child: A case report.
MedicineClinical features and risk factors of hepatic sinusoidal obstruction syndrome in children after hematopoietic stem cell transplantation: A single-center experience.
Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for HaemapheresisBile cast nephropathy after sinusoidal obstruction syndrome.
Kidney internationalIncidence, management and outcome of Hepatic Veno-Occlusive disease /Sinusoidal Obstruction Syndrome after hematopoietic stem cell transplant in Thalassemia major patients: A prospective study of Pakistani BMT.
Pakistan journal of medical sciencesCilostazol improves the prognosis after hepatectomy in rats with sinusoidal obstruction syndrome.
Journal of gastroenterology and hepatologyAnticoagulant therapy likely increases risk of bleeding in Gynura segetum-induced hepatic sinus obstruction syndrome.
MedicineEvaluation of Busulfan as a Third-Party Immunoassay on a Clinical Chemistry Analyzer.
The journal of applied laboratory medicineMultiplex Proteomics in the Identification of Potential Biomarkers of Very Severe Sinusoidal Obstruction Syndrome/Veno-Occlusive Disease in Allogeneic Hematopoietic Cell Transplant Patients Treated with Defibrotide.
Acta haematologicaUtility of the refined EBMT diagnostic and severity criteria 2023 for sinusoidal obstruction syndrome/veno-occlusive disease.
Bone marrow transplantationIncidence and risk factors of early onset VOD/SOS differ in younger vs older adults after stem cell transplantation.
Blood advancesAzathioprine-induced Veno-occlusive Hepatotoxicity in a Patient with Myasthenia Gravis.
Current drug safetyGemtuzumab Ozogamicin in Acute Myeloid Leukemia: Efficacy, Toxicity, and Resistance Mechanisms-A Systematic Review.
BiomedicinesDissociation in hepatic vein pressure gradient, liver stiffness measurement and complications in histological subtypes of porto-sinusoidal vascular disease.
Journal of clinical pathologyAnalysis of laboratory parameters before the occurrence of hepatic sinusoidal obstruction syndrome in children, adolescents, and young adults after hematopoietic stem cell transplantation.
Journal of cancer research and clinical oncology[Treatment response of a two-dose regimen of dose-adjusted inotuzumab ozogamicin in relapsed/refractory B-cell acute lymphoblastic leukemia].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhiHistological evaluation of pyrrolizidine alkaloid-induced hepatic sinusoidal obstruction syndrome: Correlation with Drum Tower Severity Scoring.
Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the LiverUtility of liver stiffness measurement in the diagnosis of sinusoidal obstruction syndrome/veno-occlusive disease after hematopoietic stem cell transplantation.
Journal of medical ultrasonics (2001)Clinical outcomes of early defibrotide discontinuation: a single-center case series.
Bone marrow transplantationImpact of inotuzumab ozogamicin on outcome in relapsed or refractory acute B-cell lymphoblastic leukemia patients prior to allogeneic hematopoietic stem cell transplantation and risk of sinusoidal obstruction syndrome/venous occlusive disease.
HaematologicaEarly-onset hepatic veno-occlusive disease after liver transplantation: an institutional experience and analysis of a literature-based cohort.
Surgery todayPrimary Budd-Chiari syndrome versus sinusoidal obstruction syndrome: a review.
Current medical research and opinionAssaying ADAMTS13 Activity as a Potential Prognostic Biomarker for Sinusoidal Obstruction Syndrome in Mice.
International journal of molecular sciencesProspective Assessment of Treatment-Induced Liver Injury as a Cause of Diffuse Pathologic Hepatic Enhancement in Contrast-Enhanced Ultrasound.
Ultrasound in medicine & biologyRifaximin Prevents Intestinal Barrier Dysfunction and Alleviates Liver Injury in MCT-induced HSOS Mice.
Current medical sciencePhase 2 study of inotuzumab ozogamicin for measurable residual disease in acute lymphoblastic leukemia in remission.
BloodConstructing training set using distance between learnt graphical models of time series data on patient physiology, to predict disease scores.
PloS onePost-allogeneic Hematopoietic Stem Cell Transplantation Portal Hypertension Not Associated with Liver Cirrhosis, Veno-occlusive Disease, or Graft-versus-host Disease.
Internal medicine (Tokyo, Japan)HokUS-10 scoring system predicts the treatment outcome for sinusoidal obstruction syndrome after allogeneic hematopoietic stem cell transplantation.
Scientific reportsAndrographolide attenuated MCT-induced HSOS via regulating NRF2-initiated mitochondrial biogenesis and antioxidant response.
Cell biology and toxicologyRare variants in complement system genes associate with endothelial damage after pediatric allogeneic hematopoietic stem cell transplantation.
Frontiers in immunologyDiagnosis of sinusoidal obstruction syndrome: can biopsy be the key?
Annals of hematology[Evaluation of the efficacy of TIPS in 27 patients with hepatic sinus obstruction syndrome in the near and medium term].
Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatologyTransjugular Intrahepatic Portosystemic Shunt is effective to treat portal hypertension due to sinusoidal obstructive syndrome.
Clinics and research in hepatology and gastroenterologyBusulfan-induced hepatic sinusoidal endothelial cell injury: Modulatory role of pirfenidone for therapeutic purposes.
Toxicology in vitro : an international journal published in association with BIBRACurrent incidence, severity, and management of veno-occlusive disease/sinusoidal obstruction syndrome in adult allogeneic HSCT recipients: an EBMT Transplant Complications Working Party study.
Bone marrow transplantationPrognostic risk factors for patients with hepatic sinusoidal obstruction syndrome caused by pyrrolizidine alkaloids.
MedicineRetrospective study of risk factors for pericardial effusion after haematopoietic stem cell transplantation in children.
Hematology (Amsterdam, Netherlands)Liver Pathology After Hematopoietic Stem Cell Transplantation.
Surgical pathology clinicsConcordance between HokUS-10 Scoring and Transjugular Liver Biopsy for the Diagnosis of Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome after Allogeneic Stem Cell Transplantation.
Transplantation and cellular therapy[Posttransplant complications: GVHD and SOS/VOD].
[Rinsho ketsueki] The Japanese journal of clinical hematologyDefibrotide prophylaxis for prevention of sinusoidal obstruction syndrome - Authors' reply.
The Lancet. HaematologyDefibrotide prophylaxis for prevention of sinusoidal obstruction syndrome.
The Lancet. HaematologyAssessment of hepatic veno-occlusive disease/sinusoidal obstruction syndrome using different scanning approaches for the ultrasonographic evaluation of portal vein blood flow and hepatic artery resistive index in hematopoietic stem cell transplant recipients.
Journal of medical ultrasonics (2001)A phase 2 trial of defibrotide for the prevention of chimeric antigen receptor T-cell-associated neurotoxicity syndrome.
Blood advancesTransjugular intrahepatic portosystemic shunt for pyrrolidine alkaloids-induced hepatic sinusoidal obstruction syndrome: a retrospective cohort study.
European journal of gastroenterology & hepatologyPoint shear-wave elastography for the diagnosis of veno-occlusive disease in children and young adults.
Pediatric radiologyPeritoneal and Pleural Drains in Pediatric Hematopoietic Cell Transplant Recipients with Veno-Occlusive Disease are Safe and Do Not Adversely Impact Clinical Outcomes.
Hematology/oncology and stem cell therapyMelatonin attenuates monocrotaline-induced hepatic sinusoidal obstruction syndrome in rats via activation of Sirtuin-3.
Journal of biochemical and molecular toxicologyPrevention of veno-occlusive disease/sinusoidal obstruction syndrome: a never-ending story and no easy answer.
Bone marrow transplantationTransjugular Intrahepatic Portosystemic Shunt Benefits for Hepatic Sinusoidal Obstruction Syndrome Associated with Consumption of Gynura Segetum: a Propensity Score-Matched Analysis.
Cardiovascular and interventional radiologySinusoidal obstruction syndrome associated with disseminated toxoplasmosis involving the liver after allogeneic hematopoietic stem cell transplantation: A case report.
Journal of infection and chemotherapy : official journal of the Japan Society of ChemotherapyThymosin β4 Exerts a Cytoprotective Function and Attenuates Liver Injury in Murine Hepatic Sinusoidal Obstruction Syndrome after Hematopoietic Stem Cell Transplantation.
Transplantation and cellular therapyMini-hyper-CVD plus inotuzumab ozogamicin, with or without blinatumomab, in the subgroup of older patients with newly diagnosed Philadelphia chromosome-negative B-cell acute lymphocytic leukaemia: long-term results of an open-label phase 2 trial.
The Lancet. HaematologySinusoidal Obstruction Syndrome (SOS) and Defibrotide: We Are Not There Yet.
Transplantation and cellular therapyThe Role of the Transjugular Intrahepatic Porto-Systemic Shunt in an Emergency Setting.
Life (Basel, Switzerland)Low Plasma Levels of Hyaluronic Acid Might Rule Out Sinusoidal Obstruction Syndrome after Hematopoietic Stem Cell Transplantation.
Disease markersDiagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in adult patients: a refined classification from the European society for blood and marrow transplantation (EBMT).
Bone marrow transplantationHypofibrinolysis in pediatric patients with veno-occlusive disease in hematopoietic stem cell transplantation.
Journal of cancer research and clinical oncologyProspective assessment of risk biomarkers of sinusoidal obstruction syndrome after hematopoietic cell transplantation.
JCI insightA novel integrative multi-omics approach to unravel the genetic determinants of rare diseases with application in sinusoidal obstruction syndrome.
PloS oneCan defibrotide prophylaxis prevent sinusoidal obstruction syndrome following haematopoietic stem-cell transplantation?
The Lancet. HaematologyDefibrotide plus best standard of care compared with best standard of care alone for the prevention of sinusoidal obstruction syndrome (HARMONY): a randomised, multicentre, phase 3 trial.
The Lancet. HaematologyMolecular Advances in Sinusoidal Obstruction Syndrome/Veno-Occlusive Disease.
International journal of molecular sciencesUsefulness of two-dimensional shear wave elastography in diagnosing hepatic veno-occlusive disease in pediatric patients undergoing hematopoietic stem cell transplantation.
Ultrasonography (Seoul, Korea)Incidence of Sinusoidal Obstruction Syndrome/Veno-Occlusive Disease and Treatment with Defibrotide in Allogeneic Transplantation: A Multicenter Australasian Registry Study.
Transplantation and cellular therapy[Research progress of hepatic sinusoidal obstruction syndrome].
Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatologyUpdate on the role of gemtuzumab-ozogamicin in the treatment of acute myeloid leukemia.
Therapeutic advances in hematologyBiomarkers for early complications post hematopoietic cell transplantation: Insights and challenges.
Frontiers in immunologyHepatic Veno-occlusive disease (VOD) in multiple myeloma patient receiving Belantamab Mafodotin.
Leukemia & lymphomaRisk assessment of short-term intake of pyrrolizidine alkaloids in food: derivation of an acute reference dose.
Food additives & contaminants. Part A, Chemistry, analysis, control, exposure & risk assessment[A Case of Sinusoidal Obstruction Syndrome and Nodular Regenerative Hyperplasia after Using Oxaliplatin for Rectal Cancer].
Gan to kagaku ryoho. Cancer & chemotherapyMid- to long-term outcomes of initial transjugular intrahepatic portosystemic shunt versus anticoagulation for pyrrolizidine alkaloid-induced hepatic sinusoidal obstruction syndrome.
European journal of gastroenterology & hepatologyPost-Transplantation Sinusoidal Obstruction Syndrome in Adult Patients with B Cell Acute Lymphoblastic Leukemia Treated with Pretransplantation Inotuzumab.
Transplantation and cellular therapyHepatobiliary disease after bone marrow transplant.
Expert review of gastroenterology & hepatologyDifferential Diagnosis of Hyperferritinemia in Critically Ill Patients.
Journal of clinical medicineInterprofessional Team-based Care of the Hematopoietic Cell Transplantation Patient With Hepatic Veno-occlusive Disease/Sinusoidal Obstruction Syndrome.
Journal of pediatric hematology/oncologyTransjugular Intrahepatic Portosystemic Shunt in Liver Transplant Recipients: Outcomes in Six Adult Patients.
Vascular and endovascular surgeryPredictive factors of collateral vessel development induced by oxaliplatin-based chemotherapy.
International journal of clinical oncologyPrognostic Biomarkers for Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome in Myeloablative Allogeneic Hematopoietic Cell Transplantation: Results from the Blood and Marrow Transplant Clinical Trials Network 1202 Study.
Transplantation and cellular therapyReal-world use of defibrotide for veno-occlusive disease/sinusoidal obstruction syndrome: the DEFIFrance Registry Study.
Bone marrow transplantationDefibrotide improved the outcome of monocrotaline induced rat hepatic sinusoidal obstruction syndrome.
BMC gastroenterologyEffects of bicyclol on hepatic sinusoidal obstruction syndrome induced by Gynura segetum.
Journal of clinical laboratory analysisPlugged percutaneous liver biopsy using Tru-cut needle and coils: A retrospective study.
Indian journal of gastroenterology : official journal of the Indian Society of GastroenterologyHepatic veno-occlusive disease accompanied by thrombotic microangiopathy developed during treatment of juvenile dermatomyositis and macrophage activation syndrome: A case report.
Modern rheumatology case reportsEnoxaparin attenuates pyrrolizidine alkaloids-induced hepatic sinusoidal obstruction syndrome by inhibiting oncostatin M expression.
Liver international : official journal of the International Association for the Study of the LiverSystematic screening and focused evaluation for veno-occlusive disease/sinusoidal obstructive syndrome (VOD/SOS) following allogeneic stem cell transplant is associated with earlier diagnosis and prompt institution of defibrotide treatment.
Frontiers in transplantationUltrasonographic monitoring of sinusoidal obstruction syndrome in patients treated with inotuzumab ozogamicin.
International journal of hematology[Risk factors for acute kidney injury after hematopoietic stem cell transplantation in children: a retrospective study].
Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatricsModel establishment and microarray analysis of mice with oxaliplatin‑induced hepatic sinusoidal obstruction syndrome.
Molecular medicine reportsA Retrospective Cohort Study of the Efficacy, Safety, and Clinical Value of 6-TG versus 6-MP Maintenance Therapy in Children with Acute Lymphoblastic Leukemia.
BioMed research internationalUsefulness of ultrasonography and elastography in diagnosing oxaliplatin-induced sinusoidal obstruction syndrome.
International journal of clinical oncologySinusoidal Obstruction Syndrome of the Liver Associated With 6-Mercaptopurine During Maintenance in a Child With T-cell Acute Lymphoblastic Leukemia.
Journal of pediatric hematology/oncologyMini-hyper CVD + CRIB (condensed rituximab, inotuzumab ozogamicin, and blinatumomab) for refractory pediatric B-acute lymphoblastic leukemia.
Pediatric blood & cancerAssociações
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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.
- Transjugular intrahepatic portosystemic shunt improves survival in anticoagulation-resistant hepatic sinusoidal obstructive syndrome patients: A multicenter retrospective study.
- Epidemiology and Outcomes of Hepatic Veno-Occlusive Disease after Allogeneic Hematopoietic Stem Cell Transplantation: A Descriptive Analysis of the U.S. National Inpatient Sample.
- Two Cases of Hepatic Epithelioid Hemangioendothelioma Misdiagnosed With Hepatic Veno-Occlusive Disease.
- Real-World Outcome of Defibrotide Treatment for Severe Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome after Hematopoietic Cell Transplantation.
- Impact of Oral Eicosapentaenoic Acid on Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome Onset Prevention: Single-Center Retrospective Analysis.
- [Clinical Analysis of Allogeneic Hematopoietic Stem Cell Transplantation for Very Early-Onset Inflammatory Bowel Disease Caused by IL-10RA Mutation].
- Proteomic Signatures and Early Biomarkers Predicting Veno-Occlusive Disease in Pediatric Patients Undergoing Haploidentical Hematopoietic Cell Transplantation with Busulfan-Based Conditioning and Post-Transplant Cyclophosphamide.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:890(Orphanet)
- MONDO:0019514(MONDO)
- GARD:13004(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q5731687(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
