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MCID: HPT046
MIFTS: 59

Hepatic Veno-Occlusive Disease

Categories: Cardiovascular diseases, Endocrine 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 76 53 59 55 44 15 73
Sinusoidal Obstruction Syndrome 53 59
Veno-Occlusive Disease 12 55
Hepatic Venoocclusive Disease 76
Hepatic Vein Thrombosis 73
Hepatic Vein Occlusion 73
Venoocclusive Disease 73
Budd-Chiari Syndrome 73

Characteristics:

Orphanet epidemiological data:

59
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 Endocrine diseases
See all MalaCards categories (disease lists)
ICD10: 33 34
Orphanet: 59  
Rare hepatic diseases


External Ids:

Disease Ontology 12 DOID:0080177
ICD10 33 K76.5
MeSH 44 D006504
Orphanet 59 ORPHA890
MESH via Orphanet 45 D006504
UMLS via Orphanet 74 C0019156
ICD10 via Orphanet 34 K76.5
Sources

Summaries for Hepatic Veno-Occlusive Disease

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NIH Rare Diseases : 53 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.Orpha Number: 890Disease definitionHepatic 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.EpidemiologyPrevalence 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 descriptionIt 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.EtiologyHepatic 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 methodsDiagnosis 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 diagnosisDifferential 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 treatmentNo 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.PrognosisSeverity 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.Visit the Orphanet disease page for more resources.

MalaCards based summary : Hepatic Veno-Occlusive Disease, also known as sinusoidal obstruction syndrome, is related to pulmonary hypertension and portal vein thrombosis. 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 Selenium Micronutrient Network. The drugs Busulfan and Fludarabine have been mentioned in the context of this disorder. Affiliated tissues include liver, bone and bone marrow, 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 : 76 Hepatic veno-occlusive disease or veno-occlusive disease with immunodeficiency (VODI) is a condition in... 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 240)
# Related Disease Score Top Affiliating Genes
1 pulmonary hypertension 30.9 F3 PLAT THBD
2 portal vein thrombosis 30.3 SERPINC1 SERPINE1
3 ischemic colitis 30.3 SERPINC1 SERPINE1
4 purpura 29.9 THPO THBD SERPINC1 F3
5 thrombotic thrombocytopenic purpura 29.9 F3 THBD
6 thrombosis 29.8 THBD SERPINE1 SERPINC1 PLAT F3
7 hepatic venoocclusive disease with immunodeficiency 12.5
8 pulmonary venoocclusive disease 12.5
9 pulmonary venoocclusive disease 1, autosomal dominant 12.3
10 hematopoietic stem cell transplantation 10.8
11 pulmonary hypertension, primary, 1 10.4
12 leukemia 10.4
13 scleroderma, familial progressive 10.3
14 leukemia, acute myeloid 10.3
15 myeloid leukemia 10.3
16 platelet aggregation, spontaneous 10.3 PLAT SERPINC1
17 plasminogen activator inhibitor-1 deficiency 10.2 PLAT SERPINE1
18 central serous chorioretinopathy 10.2 PLAT SERPINE1
19 posterior myocardial infarction 10.2 PLAT SERPINC1
20 encephalopathy, familial, with neuroserpin inclusion bodies 10.2 PLAT SERPINC1
21 mercury poisoning 10.2 PLAT THBD
22 aspirin allergy 10.2 SERPINC1 SERPINE1
23 sticky platelet syndrome 10.2 SERPINC1 SERPINE1
24 postaxial acrofacial dysostosis 10.2 PLAT THBD
25 retinal artery occlusion 10.2 SERPINC1 SERPINE1
26 subendocardial myocardial infarction 10.2 SERPINC1 SERPINE1
27 pleuropneumonia 10.2 PLAT SERPINC1
28 ligneous conjunctivitis 10.2 PLAT PLG
29 eisenmenger syndrome 10.2 PLAT THBD
30 post-thrombotic syndrome 10.2 SERPINC1 SERPINE1
31 inferior myocardial infarction 10.2 PLAT PLG
32 central retinal artery occlusion 10.2 PLAT PLG
33 hemorrhagic fever 10.2 F3 SERPINC1
34 alpha-2-plasmin inhibitor deficiency 10.2 PLG SERPINC1
35 occlusion precerebral artery 10.2 PLAT PLG
36 basilar artery occlusion 10.2 PLAT PLG
37 tuberculous empyema 10.2 PLG SERPINE1
38 chronic venous insufficiency 10.2 PLAT SERPINE1
39 venous insufficiency 10.2 PLAT SERPINE1
40 fournier gangrene 10.2 F3 SERPINC1
41 heparin-induced thrombocytopenia 10.2 F3 SERPINC1
42 angina pectoris 10.2 PLAT SERPINC1
43 lymphoma 10.2
44 colitis 10.2
45 retinal vascular occlusion 10.2 PLG SERPINC1
46 anterior spinal artery syndrome 10.2 PLG SERPINC1
47 sagittal sinus thrombosis 10.2 F3 SERPINC1
48 systemic lupus erythematosus 10.2
49 budd-chiari syndrome 10.2
50 graft-versus-host disease 10.2

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:

59 32 (show all 13)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hepatomegaly 59 32 hallmark (90%) Very frequent (99-80%) HP:0002240
2 renal insufficiency 59 32 hallmark (90%) Very frequent (99-80%) HP:0000083
3 ascites 59 32 hallmark (90%) Very frequent (99-80%) HP:0001541
4 abdominal pain 59 32 hallmark (90%) Very frequent (99-80%) HP:0002027
5 jaundice 59 32 hallmark (90%) Very frequent (99-80%) HP:0000952
6 respiratory failure 59 32 hallmark (90%) Very frequent (99-80%) HP:0002878
7 increased body weight 59 32 hallmark (90%) Very frequent (99-80%) HP:0004324
8 prolonged partial thromboplastin time 59 32 frequent (33%) Frequent (79-30%) HP:0003645
9 increased total bilirubin 59 32 hallmark (90%) Very frequent (99-80%) HP:0003573
10 hepatic encephalopathy 59 32 very rare (1%) Very rare (<4-1%) HP:0002480
11 elevated hepatic transaminases 59 Very frequent (99-80%)
12 abnormality of coagulation 59 Frequent (79-30%)
13 elevated hepatic transaminase 32 hallmark (90%) HP:0002910

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

26 (show all 13)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-103 9.5 SULT1A3
2 Increased shRNA abundance (Z-score > 2) GR00366-A-105 9.5 ABCB1
3 Increased shRNA abundance (Z-score > 2) GR00366-A-127 9.5 ABCB1 F3 SULT1A3
4 Increased shRNA abundance (Z-score > 2) GR00366-A-13 9.5 F3
5 Increased shRNA abundance (Z-score > 2) GR00366-A-146 9.5 SULT1A3
6 Increased shRNA abundance (Z-score > 2) GR00366-A-163 9.5 F3
7 Increased shRNA abundance (Z-score > 2) GR00366-A-168 9.5 SULT1A3
8 Increased shRNA abundance (Z-score > 2) GR00366-A-199 9.5 ABCB1
9 Increased shRNA abundance (Z-score > 2) GR00366-A-43 9.5 SULT1A3
10 Increased shRNA abundance (Z-score > 2) GR00366-A-74 9.5 F3
11 Increased shRNA abundance (Z-score > 2) GR00366-A-78 9.5 F3
12 Increased shRNA abundance (Z-score > 2) GR00366-A-85 9.5 SULT1A3
13 Increased shRNA abundance (Z-score > 2) GR00366-A-92 9.5 SULT1A3

MGI Mouse Phenotypes related to Hepatic Veno-Occlusive Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.92 ABCB1 F3 PLAT PLG SERPINC1 SERPINE1
2 cardiovascular system MP:0005385 9.91 ABCB1 F3 PLAT PLG SERPINC1 SERPINE1
3 homeostasis/metabolism MP:0005376 9.86 ABCB1 F3 PLAT PLG SERPINC1 SERPINE1
4 immune system MP:0005387 9.76 ABCB1 F3 PLAT PLG SERPINC1 SERPINE1
5 liver/biliary system MP:0005370 9.35 ABCB1 PLG SERPINC1 SERPINE1 THBD
6 respiratory system MP:0005388 9.02 F3 PLAT PLG SERPINE1 THBD
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 110)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Busulfan Approved, Investigational Phase 4,Phase 2,Not Applicable 55-98-1 2478
2
Fludarabine Approved Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 75607-67-9, 21679-14-1 30751
3
Vidarabine Approved, Investigational Phase 4,Phase 2,Phase 1,Not Applicable 24356-66-9 32326 21704
4 Antimetabolites, Antineoplastic Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
5 Antimetabolites Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
6 Immunologic Factors Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
7 Immunosuppressive Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
8 Antiviral Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
9 Antineoplastic Agents, Alkylating Phase 4,Phase 2,Not Applicable
10 Alkylating Agents Phase 4,Phase 2,Not Applicable
11 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
12
Levoleucovorin Approved, Investigational Phase 2, Phase 3 68538-85-2
13
Methotrexate Approved Phase 2, Phase 3,Phase 1 59-05-2, 1959-05-2 126941
14
leucovorin Approved Phase 2, Phase 3,Phase 3,Phase 1 58-05-9 6006 143
15
Defibrotide Approved, Investigational Phase 3,Phase 2 83712-60-1
16
Cytarabine Approved, Investigational Phase 3 147-94-4 6253
17
Mitoxantrone Approved, Investigational Phase 3 65271-80-9 4212
18
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2, Phase 3,Phase 1 59-30-3 6037
19 Micronutrients Phase 2, Phase 3
20 Antidotes Phase 2, Phase 3
21 Vitamin B Complex Phase 2, Phase 3,Phase 1
22 Trace Elements Phase 2, Phase 3
23 Vitamin B9 Phase 2, Phase 3,Phase 1
24 Vitamins Phase 2, Phase 3
25 Protective Agents Phase 2, Phase 3,Not Applicable
26 Antirheumatic Agents Phase 2, Phase 3,Phase 1,Not Applicable
27 Hematinics Phase 2, Phase 3
28 Folic Acid Antagonists Phase 2, Phase 3,Phase 1
29 Folate Phase 2, Phase 3,Phase 1
30 Dermatologic Agents Phase 2, Phase 3,Phase 1,Not Applicable
31 Nucleic Acid Synthesis Inhibitors Phase 2, Phase 3,Phase 1
32 Anticoagulants Phase 3,Phase 2
33 Fibrinolytic Agents Phase 3,Phase 2
34 Coagulants Phase 3
35 Anti-Inflammatory Agents Phase 3
36 Platelet Aggregation Inhibitors Phase 3,Phase 2
37 Liver Extracts Phase 3,Not Applicable
38 Peripheral Nervous System Agents Phase 3
39 Topoisomerase Inhibitors Phase 3,Phase 2,Not Applicable
40 Analgesics Phase 3
41
Etoposide Approved Phase 2,Not Applicable 33419-42-0 36462
42
Mechlorethamine Approved, Investigational Phase 2,Not Applicable 51-75-2 4033
43
Lenograstim Approved, Investigational Phase 2,Not Applicable 135968-09-1
44
Carboplatin Approved Phase 2 41575-94-4 10339178 38904 498142
45
Melphalan Approved Phase 2,Phase 1,Not Applicable 148-82-3 4053 460612
46
Clofarabine Approved, Investigational Phase 2 123318-82-1 119182
47
Panobinostat Approved, Investigational Phase 1, Phase 2 404950-80-7 6918837
48
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
49
Dactinomycin Approved, Investigational Phase 2 50-76-0 457193 2019
50
Vincristine Approved, Investigational Phase 2,Not Applicable 57-22-7, 2068-78-2 5978

Interventional clinical trials:

(show all 42)
# Name Status NCT ID Phase Drugs
1 Busulfan Safety/Efficacy as Conditioning Prior to Hematopoietic Cell Transplantation (HCT) Completed NCT00361140 Phase 4 Busulfan;Fludarabine
2 Busulfan Pharmacokinetic Analysis and GST Polymorphism in Adults Undergoing Hematological Stem Cell Transplantation Withdrawn NCT01071486 Phase 4 Hematological Stem Cell Transplantation
3 Ledipasvir/Sofosbuvir Treatment for Hepatitis C in HCT Recipients. Withdrawn NCT03279133 Phase 4 Ledipasvir 90mg/Sofosubvir 400mg
4 The Effect of Folinic Acid Rescue Following MTX GVHD Prophylaxis on Regimen Related Toxicity and Transplantation Outcome Unknown status NCT02506231 Phase 2, Phase 3 Folinic acid;Placebo
5 Defibrotide for Patients With Hepatic Veno-occlusive Disease: A Treatment IND Study Completed NCT00628498 Phase 3 Defibrotide;Defibrotide
6 Defibrotide for the Treatment of Severe Hepatic Veno-Occlusive Disease in Hematopoetic Stem Cell Transplant Patients Completed NCT00358501 Phase 3 Defibrotide
7 Pediatric Trial Investigating the Incidence & Outcome of Veno-Occlusive Disease With the Prophylactic Use of Defibrotide Completed NCT00272948 Phase 2, Phase 3 Defibrotide;Defibrotide;Defibrotide
8 A Study Of Inotuzumab Ozogamicin Versus Investigator's Choice Of Chemotherapy In Patients With Relapsed Or Refractory Acute Lymphoblastic Leukemia Completed NCT01564784 Phase 3 inotuzumab ozogamicin;FLAG (fludarabine, cytarabine and G-CSF);HIDAC (high dose cytarabine);cytarabine and mitoxantrone
9 Study Comparing Efficacy and Safety of Defibrotide vs Best Supportive Care in the Prevention of Hepatic Veno-Occlusive Disease in Adult and Pediatric Patients Recruiting NCT02851407 Phase 3 Defibrotide
10 HLA 10/10 Matched Unrelated Donor vs Haploidentical Allogenic Hematopoietic Stem Cell Transplantation Not yet recruiting NCT03655145 Phase 3
11 Antithrombin-III for Patients With Hepatic Veno-occlusive Diseases Following Hematopoietic Stem Cell Transplantation Unknown status NCT01886248 Phase 2 Freeze-dried Concentrated Human Antithrombin â…¢ 500 IU
12 Chemotherapy Toxicity Reduction Via Urea Cycle Support Completed NCT00223730 Phase 2 Citrulline;Placebo
13 N2001-02: I-MIBG With Intensive Chemotherapy and Autologous Stem Cell Rescue for High-Risk Neuroblastoma Completed NCT00253435 Phase 2 Carboplatin;Etoposide;Melphalan
14 Busulfan/Clofarabine + Allogeneic Stem Cell Transplantation Completed NCT01643668 Phase 2 Busulfan;Clofarabine
15 Histone Deacetylase Inhibitor LBH589 in Addition to Corticosteroids in Patients With Acute Graft Versus Host Disease (GVHD) Completed NCT01111526 Phase 1, Phase 2 Panobinostat (LBH589)
16 IV Busulfan Plus Bortezomib Conditioning Regimen for Second Autologous Stem Cell Transplant in Multiple Myeloma Patients Completed NCT01009840 Phase 2 IV busulfan;bortezomib
17 Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors Completed NCT00335556 Phase 2 Doxorubicin Hydrochloride;Irinotecan Hydrochloride;Cyclophosphamide;Etoposide;Carboplatin;Vincristine Sulfate
18 Total-Body Irradiation and Cyclophosphamide in Treating Patients Who Are Undergoing Donor Stem Cell Transplant for Hematologic Cancer and Other Diseases Completed NCT00317785 Phase 2 cyclophosphamide;cyclosporine;methotrexate;mycophenolate mofetil;sirolimus;tacrolimus
19 Bone Marrow Transplantation in Young Adults With Severe Sickle Cell Disease Completed NCT01565616 Phase 2 Conditioning Regimen with Bone Marrow Transplant
20 Defibrotide TMA Prophylaxis Pilot Trial Recruiting NCT03384693 Phase 2 Defibrotide
21 Thiopurine EnhAnced Maintenance Therapy Recruiting NCT02912676 Phase 1, Phase 2 Thioguanine (oral)
22 Allogeneic Stem Cell Transplantation in Relapsed/Refractory T-, NK/T-cell Lymphomas Recruiting NCT02859402 Phase 2 Busulfan;Fludarabine
23 HLA-Mismatched Unrelated Donor Bone Marrow Transplantation With Post-Transplantation Cyclophosphamide Recruiting NCT02793544 Phase 2 Fludarabine;Cyclophosphamide 14.5 mg/kg/day IV on Days -6, -5;Busulfan;Cyclophosphamide 50mg/kg/day IV on Days -2,-1;Cyclophosphamide 50mg/kg/day IV on Days -5,-4;Post-HCT Cyclophosphamide 50mg/kg IV on Day+3, +4;Sirolimus;Mycophenolate mofetil;G-CSF;Pre-HCT Mesna on Days -6 and -5;Pre-HCT Mesna on Days -2 and -1;Pre-HCT Mesna on Days -5 and -4;Post-HCT Mesna
24 IMRT-TMI With Fludarabine as Myeloablative Conditioning for Allogeneic HSCT Recruiting NCT03696537 Phase 1, Phase 2 Fludarabine
25 Conditioning SCID Infants Diagnosed Early Recruiting NCT03619551 Phase 2 Busulfan
26 Inotuzumab Ozogamicin and Blinatumomab in Treating Patients With Newly Diagnosed, Recurrent, or Refractory CD22-Positive B-Lineage Acute Lymphoblastic Leukemia Recruiting NCT03739814 Phase 2
27 Treosulfan-based Conditioning in Paediatric Patients With Haematological Malignancies Active, not recruiting NCT02333058 Phase 2 Treosulfan
28 Moxetumomab Pasudotox (CAT-8015, HA22) in Children With B-lineage Acute Lymphoblastic Leukemia and Minimal Residual Disease Prior to Allogeneic Hematopoietic Stem Cell Transplantation Terminated NCT02338050 Phase 2
29 Haploidentical Hematopoietic Stem Cell Transplantation Recruiting NCT02165007 Phase 1 peripheral blood stem cell graft that are CD34+ selected
30 Effects on Glutamine on the Outcome of Allogeneic Bone Marrow Transplant Recipients Unknown status NCT00563498 Not Applicable Glutamine
31 Prevention of the Hepatic Sinusoidal Obstruction Syndrome by Means of Anticoagulants Completed NCT00885950
32 Induction Therapy Including 131 I-MIBG and Chemotherapy in Treating Patients With Newly Diagnosed High-Risk Neuroblastoma Undergoing Stem Cell Transplant, Radiation Therapy, and Maintenance Therapy With Isotretinoin Completed NCT01175356 Not Applicable cyclophosphamide;topotecan hydrochloride;cisplatin;etoposide phosphate;vincristine sulfate;doxorubicin hydrochloride;busulfan;melphalan;isotretinoin
33 MRD Testing Before and After Hematopoietic Cell Transplantation for Pediatric Acute Myeloid Leukemia Completed NCT01385787
34 Using Ultrasound Elastography to Predict Development of SOS Recruiting NCT02483481
35 Sinusoidal Obstruction Syndrome for Stem Cell Transplant Patients Biomarker Study Recruiting NCT03132337
36 Liver Stiffness Measurement Predicts VOD/SOS Development (ELASTOVOD ITALIAN MULTICENTRIC STUDY) Recruiting NCT03426358 Not Applicable
37 Safety of Allogeneic Hematopoietic Cell Transplantation (HCT) For Patients With Classical Hodgkin Lymphoma (CHL) Treated With Nivolumab Recruiting NCT03200977
38 European VOD Registry Active, not recruiting NCT03032016
39 Study of Four Different Chemotherapy Regimens With or Without Total-Body Irradiation Followed by Umbilical Cord Blood Transplant in Treating Patients With Relapsed or Refractory Hematologic Cancer Active, not recruiting NCT00547196 Not Applicable busulfan;cyclophosphamide;cyclosporine;fludarabine phosphate;melphalan;mycophenolate mofetil
40 Compassionate Use of Defibrotide for Patients With Veno-occlusive Disease of the Liver No longer available NCT00143546 Defibrotide
41 Defibrotide Compassionate Use for Patients With Life Threatening Veno-Occlusive Disease of the Liver No longer available NCT00263978 Defibrotide
42 Busulfan, Melphalan, and Stem Cell Transplant After Chemotherapy in Treating Patients With Newly Diagnosed High-Risk Neuroblastoma Suspended NCT01798004 Not Applicable cyclophosphamide;topotecan hydrochloride;cisplatin;etoposide;vincristine sulfate;doxorubicin hydrochloride;busulfan;melphalan;mesna

Search NIH Clinical Center for Hepatic Veno-Occlusive Disease

Inferred drug relations via UMLS 73 / NDF-RT 51 :


Cochrane evidence based reviews: hepatic veno-occlusive disease

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:

41
Liver, Bone, Bone Marrow, Endothelial, Myeloid, Lung, Heart
Sources

Publications for Hepatic Veno-Occlusive Disease

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Articles related to Hepatic Veno-Occlusive Disease:

(show top 50) (show all 425)
# Title Authors Year
1
Defibrotide sodium for the treatment of hepatic veno-occlusive disease/sinusoidal obstruction syndrome. ( 29301447 )
2018
2
Hepatic veno-occlusive disease following sirolimus-based immune suppression. ( 29895929 )
2018
3
Evaluation of Therapeutic Effect of Rice Husk Silica Combined with Platelets Derived Growth Factor in Hepatic Veno- Occlusive Disease. ( 29301090 )
2018
4
Final results from a defibrotide treatment-IND study for patients with hepatic veno-occlusive disease/sinusoidal obstruction syndrome. ( 29767845 )
2018
5
Hepatic veno-occlusive disease during isavuconazole administration. ( 29278102 )
2018
6
Defibrotide for the treatment of hepatic veno-occlusive disease/sinusoidal obstruction syndrome following nontransplant-associated chemotherapy: Final results from a post hoc analysis of data from an expanded-access program. ( 29873895 )
2018
7
Late-Onset Hepatic Veno-Occlusive Disease after Allografting: Report of Two Cases with Atypical Clinical Features Successfully Treated with Defibrotide. ( 29326798 )
2018
8
Hepatobiliary and Pancreatic: Pregnancy induced hepatic veno-occlusive disease requiring liver transplantation. ( 29284078 )
2018
9
Hepatic veno-occlusive disease related to Gynura segetum: A case report. ( 29703039 )
2018
10
Gallbladder wall oedema and ascites are independent predictors of progression to hepatic veno-occlusive disease for children with hematopoietic stem cell transplantation. ( 29294155 )
2018
11
Sinusoidal Obstruction Syndrome/Veno-Occlusive Disease after Autologous or Allogeneic Hematopoietic Stem Cell Transplantation in Children: a retrospective study of the Italian Hematology-Oncology Association-Hematopoietic Stem Cell Transplantation Group. ( 30266674 )
2018
12
Clinical effects of recombinant thrombomodulin and defibrotide on sinusoidal obstruction syndrome after allogeneic hematopoietic stem cell transplantation. ( 30127466 )
2018
13
Novel Ultrasonographic Scoring System of Sinusoidal Obstruction Syndrome after Hematopoietic Stem Cell Transplantation. ( 29803752 )
2018
14
Identification and Management: Sinusoidal Obstruction Syndrome/Veno-Occlusive Disease Eelated to Hematopoietic Stem Cell Transplantation
. ( 29350698 )
2018
15
Early Clinical Predictors of Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome after Myeloablative Stem Cell Transplantation. ( 30081073 )
2018
16
Treatment of hepatic veno-occlusive disease in children with N-acetylcysteine. ( 30350914 )
2018
17
Risk factors for hepatic veno-occlusive disease caused by Gynura segetum: a retrospective study. ( 30367628 )
2018
18
Association of CTH variant with sinusoidal obstruction syndrome in children receiving intravenous busulfan and cyclophosphamide before hematopoietic stem cell transplantation. ( 27779248 )
2018
19
Diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in pediatric patients: a new classification from the European society for blood and marrow transplantation. ( 28759025 )
2018
20
Defibrotide: An Oligonucleotide for Sinusoidal Obstruction Syndrome. ( 28914546 )
2018
21
Phosphodiesterase III inhibitor attenuates rat sinusoidal obstruction syndrome through inhibition of platelet aggregation in Disse's space. ( 28960464 )
2018
22
Defibrotide for the treatment of sinusoidal obstruction syndrome: evaluation of response to therapy and patient outcomes. ( 29022099 )
2018
23
Abdominal computed tomography angiography in post-transplantation sinusoidal obstruction syndrome associated with R-DHAOX/BEAM toxicity. ( 29181839 )
2018
24
Using liver elastography to diagnose sinusoidal obstruction syndrome in pediatric patients undergoing hematopoetic stem cell transplant. ( 29335626 )
2018
25
High-dose methylprednisolone for the treatment of sinusoidal obstruction syndrome in adults. ( 29379167 )
2018
26
The role of continuous renal replacement therapy in the management of acute kidney injury associated with sinusoidal obstruction syndrome following hematopoietic cell transplantation. ( 29388370 )
2018
27
Re: Diagnostic performance of contrast-enhanced CT-scan in sinusoidal obstruction syndrome induced by chemotherapy of colorectal liver metastases: Radio-pathological correlation. ( 29395618 )
2018
28
Re: Diagnostic performance of contrast-enhanced CT-scan in sinusoidal obstruction syndrome induced by chemotherapy of colorectal liver metastases: Radio-pathological correlation. ( 29422354 )
2018
29
The involvement of Nrf2 antioxidant signalling pathway in the protection of monocrotaline-induced hepatic sinusoidal obstruction syndrome in rats by (+)-catechin hydrate. ( 29458270 )
2018
30
Oxaliplatin-induced hepatic sinusoidal obstruction syndrome in a patient with gastric cancer: A case report. ( 29468059 )
2018
31
Defibrotide for Sinusoidal Obstruction Syndrome/Veno-Occlusive Disease Prophylaxis in High-Risk Adult Patients: A Single-Center Experience Study. ( 29477779 )
2018
32
Azathioprine-induced hepatic sinusoidal obstruction syndrome. ( 29496246 )
2018
33
Sinusoidal obstruction syndrome/veno-occlusive disease complication in lymphoma patients treated with oxaliplatin-based regimen: A case series report. ( 29519713 )
2018
34
"Pre-emptive strike"-the case for early treatment of hepatic sinusoidal obstruction syndrome with defibrotide. ( 29537138 )
2018
35
Sinusoidal Obstruction Syndrome (Veno-occlusive Disease) Following Hematopoietic Stem Cell Transplant: Insights and Therapeutic Advances. ( 29715079 )
2018
36
Bevacizumab exacerbates sinusoidal obstruction syndrome (SOS) in the animal model and increases MMP 9 production. ( 29774103 )
2018
37
Hepatic sinusoidal obstruction syndrome following haematopoietic stem cell transplantation. A report of 33 cases. ( 29887172 )
2018
38
Fulminant hepatitis due to very severe sinusoidal obstruction syndrome (SOS/VOD) after autologous peripheral stem cell transplantation: a case report. ( 29970140 )
2018
39
Prophylactic recombinant thrombomodulin treatment prevents hepatic sinusoidal obstruction syndrome in high-risk pediatric patients that undergo hematopoietic stem cell transplants. ( 30003636 )
2018
40
Direct Intrahepatic Portocaval Shunt for Sinusoidal Obstruction Syndrome Associated with Hepatotoxicity of Pyrrolizidine Alkaloids. ( 30009181 )
2018
41
Comprehensive analysis of serum microRNAs in hepatic sinusoidal obstruction syndrome (SOS) in rats: implication as early phase biomarkers for SOS. ( 30019166 )
2018
42
New Ultrasound Techniques Challenge the Diagnosis of Sinusoidal Obstruction Syndrome. ( 30076031 )
2018
43
Soluble Thrombomodulin Attenuates Endothelial Cell Damage in Hepatic Sinusoidal Obstruction Syndrome. ( 30348695 )
2018
44
Gynura Rhizoma containing pyrrolizidine alkaloids induces the hepatic sinusoidal obstruction syndrome in mice via upregulating fibrosis-related factors. ( 30367152 )
2018
45
Treprostinil reduces endothelial damage in murine sinusoidal obstruction syndrome. ( 30535954 )
2018
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Hepatic veno-occlusive disease induced by Chinese medicinal herbs. ( 29032695 )
2017
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CT Features of Hepatic Veno-occlusive Disease: A Meta-analysis. ( 29191686 )
2017
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Low, fixed dose defibrotide in management of hepatic veno-occlusive disease post stem cell transplantation. ( 28460209 )
2017
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Defibrotide for Patients with Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome: Interim Results from a Treatment IND Study. ( 28285079 )
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Defibrotide for children and adults with hepatic veno-occlusive disease post hematopoietic cell transplantation. ( 28825848 )
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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+ 66
Show member pathways
 12.99 F3 PLAT PLG SERPINC1 SERPINE1 THBD
2
Selenium Micronutrient Network 1
Show member pathways
 12.01 PLAT PLG SERPINE1
3
IL-1 Family Signaling Pathways 65
Show member pathways
 11.9 CXCL8 F3 SERPINE1
4
Adhesion 4
11.7 F3 PLG SERPINE1
5
Formation of Fibrin Clot (Clotting Cascade) 66
Show member pathways
 11.63 F3 PLAT PLG SERPINC1 SERPINE1 THBD
6
AGE-RAGE signaling pathway in diabetic complications 37
11.62 CXCL8 F3 SERPINE1 THBD
7
Senescence and Autophagy in Cancer 1
11.61 CXCL8 PLAT SERPINE1
8
Cell adhesion_ECM remodeling 22
11.24 CXCL8 PLAT PLG SERPINE1
9
Complement and coagulation cascades 1 37
11.24 F3 PLAT PLG SERPINC1 SERPINE1 THBD
10
Validated transcriptional targets of AP1 family members Fra1 and Fra2 1
11.04 CXCL8 THBD
11
A-beta Pathways: Plaque Formation and APP Metabolism 65
10.82 PLAT PLG
12
Dissolution of Fibrin Clot 66
10.55 PLAT PLG SERPINE1
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 extracellular region GO:0005576 9.8 CXCL8 F3 PLAT PLG SERPINC1 SERPINE1
2 extracellular space GO:0005615 9.56 CXCL8 F3 PLAT PLG SERPINC1 SERPINE1
3 cell surface GO:0009986 9.55 ABCB1 F3 PLAT PLG THBD
4 collagen-containing extracellular matrix GO:0062023 9.02 F3 PLAT PLG SERPINC1 SERPINE1

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

# Name GO ID Score Top Affiliating Genes
1 blood coagulation GO:0007596 9.55 F3 PLAT PLG SERPINC1 THBD
2 positive regulation of angiogenesis GO:0045766 9.54 CXCL8 F3 SERPINE1
3 fibrinolysis GO:0042730 9.43 PLAT PLG SERPINE1
4 negative regulation of blood coagulation GO:0030195 9.32 SERPINE1 THBD
5 negative regulation of fibrinolysis GO:0051918 9.13 PLG SERPINE1 THBD
6 hemostasis GO:0007599 8.92 F3 PLG SERPINC1 THBD

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

# Name GO ID Score Top Affiliating Genes
1 serine-type endopeptidase activity GO:0004252 9.33 F3 PLAT PLG
2 signaling receptor binding GO:0005102 9.26 PLAT PLG SERPINE1 THPO
3 protease binding GO:0002020 8.8 F3 SERPINC1 SERPINE1
Sources

Sources for Hepatic Veno-Occlusive Disease

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 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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