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MCID: HPT046
MIFTS: 54

Hepatic Veno-Occlusive Disease

Categories: Cardiovascular diseases, Gastrointestinal diseases, Liver diseases, Rare diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Hepatic Veno-Occlusive Disease

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MalaCards integrated aliases for Hepatic Veno-Occlusive Disease:

Name: Hepatic Veno-Occlusive Disease 12 73 20 58 54 15 70 32
Sinusoidal Obstruction Syndrome 20 58
Veno-Occlusive Disease 12 54
Hepatic Venoocclusive Disease 73
Hepatic Vein Thrombosis 70
Hepatic Vein Occlusion 70
Venoocclusive Disease 70
Budd-Chiari Syndrome 70

Characteristics:

Orphanet epidemiological data:

58
hepatic veno-occlusive disease
Inheritance: Not applicable; Prevalence: 1-5/10000 (Europe); Age of onset: All ages; Age of death: any age;

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Gastrointestinal diseases Liver diseases Cardiovascular diseases
See all MalaCards categories (disease lists)
ICD10: 32 33
Orphanet: 58  
Rare hepatic diseases


External Ids:

Disease Ontology 12 DOID:0080177
ICD10 32 K76.5
MESH via Orphanet 45 D006504
ICD10 via Orphanet 33 K76.5
UMLS via Orphanet 71 C0019156
Orphanet 58 ORPHA890
UMLS 70 C0019154 C0019156 C0856761 more
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Summaries for Hepatic Veno-Occlusive Disease

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GARD : 20 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs. Orpha Number: 890 Definition Hepatic veno-occlusive disease (hepatic VOD) is a condition resulting from toxic injury to the hepatic sinusoidal capillaries that leads to obstruction of the small hepatic veins. Epidemiology Prevalence is unknown but hepatic VOD is a rare disease. Endemic zones have been identified in which the disease is associated with the consumption of herbal tea containing pyrrolizidine alkaloids. Epidemics affecting several hundred individuals may occur after contamination of the harvest with these plants. Clinical description It affects children and adults. The clinical picture is characterized by painful hepatomegaly, jaundice, and fluid retention that manifests by weight gain, edemas, and ascites. Hepatic insufficiency manifesting as coagulopathy and hepatic encephalopathy may occur. Functional renal insufficiency is common. In severe cases, multiple organ failure or severe bacterial infections may occur.In developed countries, hepatic veno-occlusive disease is mainly associated with the conditioning regimen for hematopoietic stem cell transplantation, with 10 to 60% of transplant patients (depending on the conditioning regimen protocol used) developing hepatic VOD. Hepatic VOD may also occur after chemotherapy or radiation therapy. Etiology Hepatic sinusoidal endothelial cell lesions appear to be the primary cause of the disease, leading to non-thrombotic occlusion of hepatic veins with concentric subendothelial thickening associated with edema and eventually fibrosis. Diagnostic methods Diagnosis is based on recognition of the clinical manifestations (hepatomegaly, jaundice, and weight gain) and their association with a potential cause of hepatic VOD (hematopoietic stem cell transplantation or chemotherapy, exposure to pyrrolizidine alkaloids), together with exclusion of other causes of liver disease. Liver biopsy is often indicated. Imaging studies, particularly hepatic Doppler- ultrasonography, may help to confirm the diagnosis. Differential diagnosis Differential diagnoses include other causes of acute or chronic liver disease, and, in the case of hematopoietic stem cell transplantation, graft-versus-host disease and severe infections. Management and treatment No specific treatment is available. Defibrotide is frequently used, mainly on the basis of limited studies (historic comparisons) and because side effects are uncommon. Preventive treatment with heparin is often indicated, but its efficacy has not been proven. In case of hematopoietic stem cell transplantation, the main prophylactic treatment is the use of less hepatotoxic conditioning regimens. Prognosis Severity and course of the disease vary between patients. In severe cases, the prognosis is poor with a high mortality rate (up to 90%) due to multiple organ failure.

MalaCards based summary : Hepatic Veno-Occlusive Disease, also known as sinusoidal obstruction syndrome, is related to hepatic venoocclusive disease with immunodeficiency and pulmonary edema. An important gene associated with Hepatic Veno-Occlusive Disease is SERPINC1 (Serpin Family C Member 1), and among its related pathways/superpathways are Response to elevated platelet cytosolic Ca2+ and Formation of Fibrin Clot (Clotting Cascade). The drugs inotuzumab ozogamicin and Immunoglobulins have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, liver and myeloid, and related phenotypes are hepatomegaly and renal insufficiency

Disease Ontology : 12 A hepatic vascular disease that is characterized by obstruction of some of the small veins of the liver.

Wikipedia : 73 Hepatic veno-occlusive disease or veno-occlusive disease with immunodeficiency is a potentially... more...

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Related Diseases for Hepatic Veno-Occlusive Disease

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Diseases related to Hepatic Veno-Occlusive Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 466)
# Related Disease Score Top Affiliating Genes
1 hepatic venoocclusive disease with immunodeficiency 33.6 SP140 SP110
2 pulmonary edema 31.1 SERPINC1 CXCL8 CSF3
3 portal hypertension 31.0 VEGFA THPO F3 CXCL8
4 portal vein thrombosis 30.8 SERPINE1 SERPINC1 PLAT
5 esophageal varix 30.7 THPO SERPINC1 F3
6 thrombotic microangiopathy 30.5 VEGFA THBD F3
7 toxic shock syndrome 30.4 SERPINC1 F3 CXCL8
8 thrombotic thrombocytopenic purpura 30.4 THBD SERPINC1 PLAT F3
9 protein c deficiency 30.4 THBD SERPINE1 SERPINC1
10 ischemic colitis 30.4 VEGFA SERPINE1 SERPINC1
11 nonbacterial thrombotic endocarditis 30.4 SERPINC1 PLG F3
12 mitral valve stenosis 30.4 SERPINC1 PLG F3
13 purpura 30.3 THPO THBD SERPINC1 F3
14 mucositis 30.3 VEGFA CSF3 ABCB1
15 respiratory failure 30.2 VEGFA THBD F3 CXCL8
16 hemolytic-uremic syndrome 30.2 THBD PLG F3 CXCL8
17 bilirubin metabolic disorder 30.2 VEGFA SERPINC1 F3 CSF3 CD33 ABCB1
18 aspergillosis 30.2 PLG CXCL8 CSF3
19 endocarditis 30.2 SERPINE1 SERPINC1 PLAT CXCL8
20 disseminated intravascular coagulation 30.2 THBD SERPINE1 SERPINC1 PLG PLAT F3
21 engraftment syndrome 30.1 CXCL8 CSF3 ABCB1
22 cardiac tamponade 30.1 PLG PLAT F8 F3
23 ischemia 30.1 VEGFA PLG PLAT FGF2
24 non-alcoholic steatohepatitis 30.0 SERPINE1 HFE CXCL8
25 compartment syndrome 30.0 SERPINC1 PLG F8 F3
26 poems syndrome 30.0 VEGFA FGF2 CSF3
27 exanthem 30.0 VEGFA F3 CXCL8 CSF3
28 liver cirrhosis 30.0 VEGFA THPO SERPINC1 HFE F3 CXCL8
29 nodular regenerative hyperplasia 30.0 TPMT F3
30 soft tissue sarcoma 30.0 VEGFA CSF3 ABCB1
31 varicose veins 29.9 VEGFA THBD SERPINC1 PLAT HFE CXCL8
32 thrombosis 29.9 THBD SERPINE1 SERPINC1 PLG PLAT F8
33 adult respiratory distress syndrome 29.9 THBD SERPINE1 PLG CXCL8 CSF3
34 thrombophlebitis 29.8 SERPINE1 SERPINC1 PLG PLAT F8 F3
35 deficiency anemia 29.6 THPO SERPINC1 PLG HFE CSF3
36 leukemia, acute myeloid 29.6 VEGFA THPO FGF2 CXCL8 CSF3 CD33
37 pancytopenia 29.6 TPMT THPO CSF3 CD33
38 antiphospholipid syndrome 29.6 THBD SULT1A3 SERPINE1 SERPINC1 PLAT F3
39 peripheral nervous system disease 29.5 VEGFA HFE FGF2 CXCL8 CSF3
40 childhood acute lymphocytic leukemia 29.5 VEGFA TPMT HFE ABCB1
41 neutropenia 29.4 TPMT THPO CXCL8 CSF3 ABCB1
42 myeloma, multiple 29.4 VEGFA FGF2 CXCL8 CSF3 CD33 ABCB1
43 leukemia, acute lymphoblastic 29.4 TPMT THPO SERPINC1 CSF3 CD33 ABCB1
44 pulmonary hypertension 29.2 VEGFA THBD SERPINE1 SERPINC1 PLG PLAT
45 vascular disease 29.1 VEGFA THBD SERPINE1 SERPINC1 PLG PLAT
46 acute leukemia 29.0 TPMT THPO CSF3 CD33 ABCB1
47 myelodysplastic syndrome 29.0 VEGFA THPO HFE FGF2 CXCL8 CSF3
48 hepatic vascular disease 28.9 VEGFA THPO THBD SERPINE1 SERPINC1 PLG
49 aplastic anemia 28.8 VEGFA TPMT THPO THBD SERPINE1 SERPINC1
50 systemic lupus erythematosus 28.8 VEGFA THBD SULT1A3 SERPINE1 F3 CXCL8

Comorbidity relations with Hepatic Veno-Occlusive Disease via Phenotypic Disease Network (PDN):


Heart Disease

Graphical network of the top 20 diseases related to Hepatic Veno-Occlusive Disease:



Diseases related to Hepatic Veno-Occlusive Disease
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Symptoms & Phenotypes for Hepatic Veno-Occlusive Disease

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Human phenotypes related to Hepatic Veno-Occlusive Disease:

58 31 (show all 12)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hepatomegaly 58 31 hallmark (90%) Very frequent (99-80%) HP:0002240
2 renal insufficiency 58 31 hallmark (90%) Very frequent (99-80%) HP:0000083
3 ascites 58 31 hallmark (90%) Very frequent (99-80%) HP:0001541
4 elevated hepatic transaminase 58 31 hallmark (90%) Very frequent (99-80%) HP:0002910
5 jaundice 58 31 hallmark (90%) Very frequent (99-80%) HP:0000952
6 abdominal pain 58 31 hallmark (90%) Very frequent (99-80%) HP:0002027
7 respiratory failure 58 31 hallmark (90%) Very frequent (99-80%) HP:0002878
8 increased total bilirubin 58 31 hallmark (90%) Very frequent (99-80%) HP:0003573
9 increased body weight 58 31 hallmark (90%) Very frequent (99-80%) HP:0004324
10 prolonged partial thromboplastin time 58 31 frequent (33%) Frequent (79-30%) HP:0003645
11 hepatic encephalopathy 58 31 very rare (1%) Very rare (<4-1%) HP:0002480
12 abnormality of coagulation 58 Frequent (79-30%)

GenomeRNAi Phenotypes related to Hepatic Veno-Occlusive Disease according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00106-A-0 9.6 SP140
2 Decreased viability GR00249-S 9.6 ABCB1 FGF2 SERPINE1 SP140 TPMT VEGFA
3 Decreased viability GR00381-A-1 9.6 PLAT
4 Decreased viability GR00386-A-1 9.6 CSF3 F8 FGF2 SULT1A3 THPO
5 Decreased viability GR00402-S-2 9.6 ABCB1 AZU1 CD33 CXCL8 SP140 THPO

MGI Mouse Phenotypes related to Hepatic Veno-Occlusive Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.8 ABCB1 CSF3 F3 F8 FGF2 HFE
2 immune system MP:0005387 9.4 ABCB1 CSF3 F3 F8 HFE PLAT
Sources

Drugs & Therapeutics for Hepatic Veno-Occlusive Disease

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Drugs for Hepatic Veno-Occlusive Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 53)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
inotuzumab ozogamicin Approved, Investigational Phase 4 635715-01-4
2 Immunoglobulins Phase 4
3 Antibodies Phase 4
4
Dalteparin Approved Phase 3 9005-49-6
5
Heparin Approved, Investigational Phase 3 9005-49-6 772 9812414
6
Tinzaparin Approved Phase 3 9041-08-1, 9005-49-6 25244225
7
Alprostadil Approved, Investigational Phase 2, Phase 3 745-65-3 149351 5280723
8 Cola Phase 3
9 Heparin, Low-Molecular-Weight Phase 3
10 calcium heparin Phase 3
11 Anti-Inflammatory Agents Phase 3
12 Coagulants Phase 3
13
Bilirubin Phase 2, Phase 3 635-65-4 5280352
14 Orange Approved Phase 1, Phase 2
15
Bevacizumab Approved, Investigational Phase 1, Phase 2 216974-75-3
16
Sargramostim Approved, Investigational Phase 2 123774-72-1, 83869-56-1
17
Cytarabine Approved, Investigational Phase 2 147-94-4 6253
18
Lenograstim Approved, Investigational Phase 2 135968-09-1
19
Cladribine Approved, Investigational Phase 2 4291-63-8 20279
20
Acetylcysteine Approved, Investigational Phase 2 616-91-1 12035
21
Molgramostim Investigational Phase 2 99283-10-0
22 Imatinib Mesylate Phase 1, Phase 2 220127-57-1 123596
23 Angiogenesis Inhibitors Phase 1, Phase 2
24 Anti-Bacterial Agents Phase 1, Phase 2
25 Protein Kinase Inhibitors Phase 1, Phase 2
26 Antineoplastic Agents, Immunological Phase 2
27 Adjuvants, Immunologic Phase 2
28 Immunosuppressive Agents Phase 2
29 Immunologic Factors Phase 2
30 Gemtuzumab Phase 2
31 Antimetabolites Phase 2
32 Anticoagulants Phase 2
33 Serine Proteinase Inhibitors Phase 2
34 Antithrombin III Phase 2
35 Antithrombins Phase 2
36 HIV Protease Inhibitors Phase 2
37
protease inhibitors Phase 2
38 Respiratory System Agents Phase 2
39 Antidotes Phase 2
40 Pharmaceutical Solutions Phase 2
41 Antioxidants Phase 2
42 Protective Agents Phase 2
43 Expectorants Phase 2
44 Anti-Infective Agents Phase 2
45 Antiviral Agents Phase 2
46 N-monoacetylcystine Phase 2
47
Serine Investigational, Nutraceutical Phase 2 56-45-1 5951
48
Oxaliplatin Approved, Investigational 61825-94-3 5310940 9887054 6857599 43805
49
Defibrotide Approved, Investigational 83712-60-1
50 glutamine

Interventional clinical trials:

(show all 33)
# Name Status NCT ID Phase Drugs
1 A PHASE 4, OPEN-LABEL, RANDOMIZED STUDY OF TWO INOTUZUMAB OZOGAMICIN DOSE LEVELS IN ADULT PATIENTS WITH RELAPSED OR REFRACTORY B-CELL ACUTE LYMPHOBLASTIC LEUKEMIA ELIGIBLE FOR HEMATOPOIETIC STEM CELL TRANSPLANTATION AND WHO HAVE RISK FACTOR(S) FOR VENO-OCCLUSIVE DISEASE Recruiting NCT03677596 Phase 4 inotuzumab ozogamicin-dose level 2;Inotuzumab ozogamicin-dose level 1
2 Using Ultrasound Elastography to Predict Development of Sinusoidal Obstruction Syndrome Active, not recruiting NCT03858530 Phase 4
3 Validating Ultrasound Biomarkers for Hepatic Sinusoidal Obstruction Syndrome in Pediatric Hematopoietic Cell Transplant Patients Withdrawn NCT03963999 Phase 4 Contrast Enhanced Ultrasound (CEUS)
4 Phase III Double-Blind Trial Comparing Low-Molecular Weight Heparin (LMWH) Versus Placebo in Patients With Advanced Cancer Completed NCT00003674 Phase 3 dalteparin;standard therapy
5 A Phase III Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Effects of Fragmin (5,000 IU Subcutaneously) in Preventing Catheter-Related Complications When Given Daily to Cancer Patients With Central Venous Catheters Completed NCT00006083 Phase 3 Fragmin
6 Defibrotide for Patients With Hepatic Veno-occlusive Disease: A Treatment IND Study Completed NCT00628498 Phase 3 Defibrotide;Defibrotide
7 Defibrotide for the Treatment of Severe Hepatic Veno-Occlusive Disease in Hematopoetic Stem Cell Transplant Patients: A Historically-Controlled, Multi-Center Phase 3 Study to Determine Safety & Efficacy Completed NCT00358501 Phase 3 Defibrotide
8 Prospective Randomized Study of the Incidence and Outcome of Veno-Occlusive Disease (VOD) With the Prophylactic Use of Defibrotide (DF) in Pediatric Stem Cell Transplantation Completed NCT00272948 Phase 2, Phase 3 Defibrotide;Defibrotide;Defibrotide
9 A Phase 3, Randomized, Adaptive Study Comparing the Efficacy and Safety of Defibrotide vs Best Supportive Care in the Prevention of Hepatic Veno-Occlusive Disease in Adult and Pediatric Patients Undergoing Hematopoietic Stem Cell Transplant Completed NCT02851407 Phase 3 Defibrotide
10 Pharmacokinetic Study of Lipo-PGE1 for Prevention of VOD After HSCT Active, not recruiting NCT02338440 Phase 2, Phase 3 lipoprostaglandin E1
11 Defibrotide for Hematopoietic Stem Cell Transplant Patients With Severe Hepatic Venocclusive Disease: A Phase I/II Study to Determine the Minimal Effective Dose Completed NCT00003966 Phase 2 defibrotide
12 A Pilot Trial of Using Pre-Transplant Risk Stratification and Prophylactic Defibrotide to Prevent Serious Thrombotic Microangiopathy in High-Risk Hematopoietic Stem Cell Transplant Patients Completed NCT03384693 Phase 2 Defibrotide
13 Adjunct Targeted Biologic Inhibition in Children With Multivessel Intraluminal Pulmonary Vein Stenosis Completed NCT00891527 Phase 1, Phase 2 Bevacizumab (Avastin) and Imatinib Mesylate (Gleevec)
14 Phase II Open Label Study to Assess Efficacy of 5-day Defibrotide Treatment for Hepatic SOS/VOD Recruiting NCT04313036 Phase 2 Defibrotide
15 A Phase II Study of Cladribine, Cytarabine, and Granulocyte-Colony Stimulating Factor With Fractionated Gemtuzumab Ozogamicin (CLAG-GO) for the Treatment of Patients With Persistent, Relapsed or Refractory Acute Myeloid Leukemia Recruiting NCT04050280 Phase 2 Cladribine, Cytarabine, and Granulocyte-Colony Stimulating Factor with Fractionated Gemtuzumab Ozogamicin (CLAG-GO)
16 Antithrombin-III for Patients With Hepatic Veno-occlusive Diseases Following Hematopoietic Stem Cell Transplantation Active, not recruiting NCT01886248 Phase 2 Freeze-dried Concentrated Human Antithrombin â…¢ 500 IU
17 Prospective, Randomized Study Using N-Acetylcysteine as Prophylaxis of Sinusoidal Obstruction Syndrome in Patients Undergoing Hematopoietic Stem Cell Transplantation Not yet recruiting NCT04712435 Phase 2 N-acetylcysteine;Placebo
18 Hepatic Artery Infusion of CD34+ Cells, Isolated by the Isolex 300i Device, in Stem Cell Transplant Recipients With Hepatic Failure Due to Veno-occlusive Disease Withdrawn NCT00062543 Phase 1
19 A Randomized Control Studies on the Effects of Glutamine on the Clinical Outcome of Bone Marrow Transplant Recipients With Special Reference to Veno-occlusive Disease and Mucositis. Unknown status NCT00563498 Glutamine
20 Prevention of the Hepatic Sinusoidal Obstruction Syndrome Secondary to Oxaliplatin-based Neoadjuvant Chemotherapy for Colorectal Liver Metastases by Means of Anticoagulants Completed NCT00885950
21 Using Ultrasound Elastography to Predict Development of Sinusoidal Obstruction Syndrome Completed NCT02483481
22 A Retrospective Study About Detection of Sinusoidal Obstruction Syndrome With Ultrasound After Allogeneic Hematopoietic Stem Cell Transplantation Completed NCT04141735
23 A Multi-centre, Multinational, Prospective Observational Registry to Collect Safety and Outcome Data in Patients Diagnosed With Severe Hepatic Veno-occlusive Disease (VOD) Following Hematopoietic Stem Cell Transplantation (HSCT) and Treated With Defitelio® Completed NCT03032016
24 Balloon Angioplasty Alone Versus in Combination With Stent Placement for the Treatment of Budd-Chiari Syndrome in China: An Randomized Controlled Trial Completed NCT02201485
25 Using Ultrasound Elastography to Predict Development of Hepatic Sinusoidal Obstruction Syndrome Recruiting NCT03865589
26 Usefulness of Liver Stiffness Measurement in Predicting Hepatic Veno-Occlusive Disease Development in Patients Who Undergo HSCT: a Multicentric Prospective Study Recruiting NCT03426358
27 Vienna Vascular Liver Disease Study: Characterization of Patients With Vascular Liver Disease - a Registry With Biobank Recruiting NCT03541057
28 Pharmacogenetics of Veno-Occlusive Disease (VOD) in Children With Haematological Stem Cell Transplantation (HSCT) Recruiting NCT03664427
29 Biomarkers of Endothelial Dysfunction in Pediatric Patients Receiving High Intensity Chemotherapy/Irradiation Active, not recruiting NCT03132337
30 Pharmacogenetic Study of Antimitotic Therapies Involved in Hepatic Veno-occlusive Disease in Children With Nephroblastoma or Acute Lymphoblastic Leukemia Not yet recruiting NCT04168788
31 Compassionate Use of Defibrotide for Patients With Veno-Occlusive Disease No longer available NCT00143546 Defibrotide
32 Defibrotide Compassionate Use Protocol for Patients With Life Threatening Veno-Occlusive Disease of the Liver No longer available NCT00263978 Defibrotide
33 Portal Vein Thrombosis in Cirrhosis: A Cohort Sudy Withdrawn NCT02275585

Search NIH Clinical Center for Hepatic Veno-Occlusive Disease

Inferred drug relations via UMLS 70 / NDF-RT 51 :


Antithrombin III, Human
ANTITHROMBIN III,RECOMBINANT
Sources

Genetic Tests for Hepatic Veno-Occlusive Disease

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Anatomical Context for Hepatic Veno-Occlusive Disease

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MalaCards organs/tissues related to Hepatic Veno-Occlusive Disease:

40
Bone Marrow, Liver, Myeloid, Endothelial, Kidney, Heart, Bone
Sources

Publications for Hepatic Veno-Occlusive Disease

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Articles related to Hepatic Veno-Occlusive Disease:

(show top 50) (show all 2238)
# Title Authors PMID Year
1
The thiopurine methyltransferase genetic polymorphism is associated with thioguanine-related veno-occlusive disease of the liver in children with acute lymphoblastic leukemia. 54 61
17015055 2006
2
Costs of allogeneic hematopoietic stem cell transplantation. 54 61
16858272 2006
3
Control of hepatic veno-occlusive disease with an antithrombin-III concentrate-based therapy. 54 61
16490079 2006
4
Anti-thrombin III in the management of hematopoietic stem-cell transplantation-associated toxicity. 54 61
15113990 2004
5
Role of plasminogen activator inhibitor-1 (PAI-1) levels in the diagnosis of BMT-associated hepatic veno-occlusive disease and monitoring of subsequent therapy with defibrotide (DF). 61 54
12745658 2003
6
Plasminogen activator inhibitor-1 is an independent diagnostic marker as well as severity predictor of hepatic veno-occlusive disease after allogeneic bone marrow transplantation in adults conditioned with busulphan and cyclophosphamide. 61 54
12199790 2002
7
Multiple organ dysfunction syndrome in hematopoietic stem cell transplantation. 61 54
12004245 2002
8
Portal vein thrombosis after hematopoietic cell transplantation: frequency, treatment and outcome. 61 54
11896430 2002
9
Growth factors and hemostasis: differential effects of GM-CSF and G-CSF on coagulation activation--laboratory and clinical evidence. 61 54
11669301 2001
10
Antithrombin treatment of severe hepatic veno-occlusive disease in children with cancer. 54 61
10650858 1999
11
Tissue plasminogen activator (tPA) as therapy for hepatotoxicity following bone marrow transplantation. 54 61
10627640 1999
12
The relevance of plasminogen activator inhibitor 1 (PAI-1) as a marker for the diagnosis of hepatic veno-occlusive disease in patients after bone marrow transplantation. 61 54
10194118 1999
13
Relevance of proteins C and S, antithrombin III, von Willebrand factor, and factor VIII for the development of hepatic veno-occlusive disease in patients undergoing allogeneic bone marrow transplantation: a prospective study. 54 61
9827816 1998
14
Endothelial dysfunction after bone marrow transplantation: increase of soluble thrombomodulin and PAI-1 in patients with multiple transplant-related complications. 61 54
9540759 1998
15
Plasminogen activator inhibitor-1 confirms the diagnosis of hepatic veno-occlusive disease in patients with hyperbilirubinemia after bone marrow transplantation. 54 61
9058743 1997
16
Laboratory markers of veno-occlusive disease in the course of bone marrow and subsequent liver transplantation. 61 54
9052916 1997
17
Protein C, protein S and antithrombin III levels in the course of bone marrow and subsequent liver transplantation due to veno-occlusive disease. 61 54
9438165 1996
18
Recombinant human tissue plasminogen activator for the treatment of severe hepatic veno-occlusive disease in pediatric bone marrow transplant patients. 61 54
8807121 1996
19
Etoposide with/without G-CSF with busulfan and cyclophosphamide as conditioning for bone marrow transplantation. The BMT Team. 61 54
8602625 1996
20
Treatment of veno-occlusive disease of the liver with bolus tissue plasminogen activator and continuous infusion antithrombin III concentrate. 54 61
8704704 1996
21
Successful treatment of veno-occlusive disease with recombinant tissue plasminogen activator in a patient requiring peritoneal dialysis. 61 54
7858540 1994
22
Alteplase for hepatic veno-occlusive disease after bone marrow transplantation. 61 54
1354293 1992
23
Alteplase for hepatic veno-occlusive disease after bone-marrow transplantation. 61 54
1351153 1992
24
Alteplase for hepatic veno-occlusive disease complicating bone-marrow transplantation. 54 61
1349081 1992
25
Veno-occlusive Disease in HSCT Patients: Consensus-based Recommendations for Risk Assessment, Diagnosis, and Management by the GITMO Group. 61
33273315 2021
26
The Protégé Nitinol Self-Expanding Stent for the Treatment of Iliofemoral Veno-Occlusive Disease. 61
33506282 2021
27
Comparison of Total Body Irradiation-based Versus Chemotherapy-based Conditionings for Early Complications of Allogeneic Hematopoietic Stem Cell Transplantation in Children With ALL. 61
33625092 2021
28
Long-term follow-up of salvage therapy using a combination of inotuzumab ozogamicin and mini-hyper-CVD with or without blinatumomab in relapsed/refractory Philadelphia chromosome-negative acute lymphoblastic leukemia. 61
33740268 2021
29
β3-Adrenoreceptors as ROS Balancer in Hematopoietic Stem Cell Transplantation. 61
33799536 2021
30
The Relationship Between Busulfan Exposure and Achievement of Sustained Donor Myeloid Chimerism in Patients with Non-Malignant Disorders. 61
33781528 2021
31
Clinical outcomes of gemtuzumab ozogamicin for relapsed acute myeloid leukemia: single-institution experience. 61
33219461 2021
32
The role of cardiopulmonary exercise test in identifying pulmonary veno-occlusive disease. 61
33653804 2021
33
Embryonal rhabdomyosarcoma completely resected at diagnosis: The European paediatric Soft tissue sarcoma Study Group RMS2005 experience. 61
33567392 2021
34
Refined ultrasonographic criteria for sinusoidal obstruction syndrome after hematopoietic stem cell transplantation. 61
33763826 2021
35
Outcomes following hematopoietic stem cell transplantation in patients treated with standard chemotherapy with or without gemtuzumab ozogamicin for acute myeloid leukemia. 61
33564120 2021
36
Hepatic veno-occlusive disease in allogeneic stem cell transplant recipients with prior exposure to gemtuzumab ozogamicin or inotuzumab ozogamicin. 61
32988266 2021
37
Hepatic veno-occlusive disease/sinusoidal obstruction syndrome after hematopoietic stem cell transplantation for thalassemia major: incidence, management, and outcome. 61
33608657 2021
38
Early and late-onset veno-occlusive disease/sinusoidal syndrome post allogeneic stem cell transplantation - a real-world UK experience. 61
33037770 2021
39
[Screening for pulmonary veno-occlusive disease: A specific approach]. 61
33573875 2021
40
Time to Complete Response after Defibrotide Initiation in Hepatic Veno-Occlusive Disease: Is Less Actually More? 61
33781546 2021
41
Efficacy of low dose and short duration defibrotide prophylaxis for hepatic veno-occlusive disease after autologous haematopoietic stem cell transplantation. 61
32839533 2021
42
Incidence and risk factors of hepatic veno-occlusive disease/sinusoidal obstruction syndrome after allogeneic hematopoietic cell transplantation in adults with prophylactic ursodiol and intravenous heparin or prostaglandin E1. 61
33526915 2021
43
Clinical Predictors of Morbidity and Mortality in Hospitalized Pediatric Patients with Ascites. 61
33633084 2021
44
Predicting sinusoidal obstruction syndrome after allogeneic stem cell transplantation with the EASIX biomarker panel. 61
31974195 2021
45
Inotuzumab ozogamicin in infants and young children with relapsed or refractory acute lymphoblastic leukaemia: a case series. 61
33529389 2021
46
131 I-Meta-iodobenzylguanidine followed by busulfan and melphalan and autologous stem cell rescue in high-risk neuroblastoma. 61
33099289 2021
47
Early bilirubinemia after allogeneic stem cell transplantation-an endothelial complication. 61
33517355 2021
48
Profile of Hepatobiliary Dysfunction in Hematopoietic Stem Cell Transplant Recipients - An Indian Perspective. 61
33679044 2021
49
Characterization of the Relationship of Inotuzumab Ozogamicin Exposure With Efficacy and Safety End Points in Adults With Relapsed or Refractory Acute Lymphoblastic Leukemia. 61
32812370 2021
50
Outcome After Cord Blood Transplantation Using Busulfan Pharmacokinetics-Targeted Myeloablative Conditioning for Hurler Syndrome. 61
32961374 2021
Sources

Variations for Hepatic Veno-Occlusive Disease

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Expression for Hepatic Veno-Occlusive Disease

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Search GEO for disease gene expression data for Hepatic Veno-Occlusive Disease.
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Pathways for Hepatic Veno-Occlusive Disease

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Pathways related to Hepatic Veno-Occlusive Disease according to GeneCards Suite gene sharing:

(show all 12)
# Super pathways Score Top Affiliating Genes
1
Response to elevated platelet cytosolic Ca2+ 65
Show member pathways
 13.05 VEGFA THPO THBD SERPINE1 SERPINC1 PLG
2
Formation of Fibrin Clot (Clotting Cascade) 65
Show member pathways
 11.7 THBD SERPINE1 SERPINC1 PLG PLAT F8
3
Hematopoietic cell lineage 36
11.68 THPO CSF3 CD33
4
AGE-RAGE signaling pathway in diabetic complications 36
11.55 VEGFA THBD SERPINE1 F3 CXCL8
5
HIF-1-alpha transcription factor network 1
11.48 VEGFA SERPINE1 ABCB1
6
Endochondral Ossification 1
11.47 VEGFA PLAT FGF2
7
Cell adhesion_ECM remodeling 23
11.45 SERPINE1 PLG PLAT CXCL8
8
Lung fibrosis 1
11.43 FGF2 CXCL8 CSF3
9
Complement and coagulation cascades 36 1
11.33 THBD SERPINE1 SERPINC1 PLG PLAT F8
10
Cell adhesion_Plasmin signaling 23
11.25 VEGFA PLG PLAT FGF2
11
Glypican 1 network 1
11.02 VEGFA SERPINC1 FGF2
12
Dissolution of Fibrin Clot 65
10.69 SERPINE1 PLG PLAT
Sources

GO Terms for Hepatic Veno-Occlusive Disease

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Cellular components related to Hepatic Veno-Occlusive Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell surface GO:0009986 9.8 VEGFA THBD PLG PLAT F3 CD33
2 extracellular region GO:0005576 9.73 VEGFA THPO SERPINE1 SERPINC1 PLG PLAT
3 collagen-containing extracellular matrix GO:0062023 9.72 SERPINE1 SERPINC1 PLG PLAT F3
4 extracellular space GO:0005615 9.47 VEGFA THPO THBD SERPINE1 SERPINC1 PLG
5 platelet alpha granule lumen GO:0031093 9.46 VEGFA SERPINE1 PLG F8

Biological processes related to Hepatic Veno-Occlusive Disease according to GeneCards Suite gene sharing:

(show all 19)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.91 VEGFA HFE FGF2 F3 CXCL8 AZU1
2 cytokine-mediated signaling pathway GO:0019221 9.88 VEGFA FGF2 F3 CXCL8 CSF3
3 angiogenesis GO:0001525 9.85 VEGFA SERPINE1 FGF2 CXCL8
4 positive regulation of protein kinase B signaling GO:0051897 9.77 VEGFA THPO FGF2 F3 CSF3
5 platelet degranulation GO:0002576 9.76 VEGFA SERPINE1 PLG F8
6 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.74 VEGFA FGF2 CSF3
7 positive regulation of endothelial cell proliferation GO:0001938 9.7 VEGFA FGF2 F3
8 positive regulation of angiogenesis GO:0045766 9.65 VEGFA SERPINE1 FGF2 F3 CXCL8
9 positive regulation of DNA biosynthetic process GO:2000573 9.58 VEGFA FGF2
10 negative regulation of blood coagulation GO:0030195 9.58 THBD SERPINE1
11 fibrinolysis GO:0042730 9.58 SERPINE1 PLG PLAT
12 positive regulation of positive chemotaxis GO:0050927 9.56 VEGFA F3
13 negative regulation of cell-cell adhesion mediated by cadherin GO:2000048 9.54 VEGFA PLG
14 stem cell proliferation GO:0072089 9.51 FGF2 ABCB1
15 induction of positive chemotaxis GO:0050930 9.5 VEGFA CXCL8 AZU1
16 positive regulation of epithelial tube formation GO:1905278 9.49 VEGFA FGF2
17 blood coagulation GO:0007596 9.43 THBD SERPINC1 PLG PLAT F8 F3
18 negative regulation of fibrinolysis GO:0051918 9.33 THBD SERPINE1 PLG
19 hemostasis GO:0007599 9.02 THBD SERPINC1 PLG F8 F3

Molecular functions related to Hepatic Veno-Occlusive Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 10.35 VEGFA TPMT THBD SULT1A3 SP140 SP110
2 signaling receptor binding GO:0005102 9.72 THPO SERPINE1 PLG PLAT HFE
3 serine-type endopeptidase activity GO:0004252 9.56 PLG PLAT F3 AZU1
4 heparin binding GO:0008201 9.46 VEGFA SERPINC1 FGF2 AZU1
5 growth factor activity GO:0008083 9.26 VEGFA THPO FGF2 CSF3
6 cytokine activity GO:0005125 9.02 VEGFA THPO FGF2 CXCL8 CSF3
Sources

Sources for Hepatic Veno-Occlusive Disease

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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