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MCID: HPT046
MIFTS: 57

Hepatic Veno-Occlusive Disease

Categories: Rare diseases, Gastrointestinal diseases, Liver diseases, Cardiovascular diseases, Endocrine diseases, Blood 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 Blood diseases
See all MalaCards categories (disease lists)
ICD10: 34 33
Orphanet: 59  
Rare hepatic diseases


External Ids:

Disease Ontology 12 DOID:0080177
MeSH 44 D006504
Orphanet 59 ORPHA890
MESH via Orphanet 45 D006504
UMLS via Orphanet 74 C0019156
ICD10 via Orphanet 34 K76.5
ICD10 33 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 portal vein thrombosis and thrombotic thrombocytopenic purpura. 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 Collagen chain trimerization. The drugs Busulfan and Fludarabine have been mentioned in the context of this disorder. Affiliated tissues include liver, bone marrow and bone, and related phenotypes are renal insufficiency and jaundice

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 150)
# Related Disease Score Top Affiliating Genes
1 portal vein thrombosis 30.8 SERPINC1 SERPINE1
2 thrombotic thrombocytopenic purpura 30.0 F3 THBD
3 purpura 29.3 F3 SERPINC1 THBD THPO
4 thrombosis 29.2 F3 PLAT SERPINC1 SERPINE1 THBD
5 hepatic venoocclusive disease with immunodeficiency 12.4
6 hepatitis 10.9
7 platelet aggregation, spontaneous 10.8 PLAT SERPINC1
8 plasminogen activator inhibitor-1 deficiency 10.8 PLAT SERPINE1
9 coronary restenosis 10.8 PLAT SERPINC1
10 central serous chorioretinopathy 10.8 PLAT SERPINE1
11 posterior myocardial infarction 10.8 PLAT SERPINC1
12 mercury poisoning 10.8 PLAT THBD
13 encephalopathy, familial, with neuroserpin inclusion bodies 10.8 PLAT SERPINC1
14 sticky platelet syndrome 10.8 SERPINC1 SERPINE1
15 postaxial acrofacial dysostosis 10.8 PLAT THBD
16 subendocardial myocardial infarction 10.7 SERPINC1 SERPINE1
17 eisenmenger syndrome 10.7 PLAT THBD
18 ligneous conjunctivitis 10.7 PLAT PLG
19 inferior myocardial infarction 10.7 PLAT PLG
20 central retinal artery occlusion 10.7 PLAT PLG
21 alpha-2-plasmin inhibitor deficiency 10.7 PLG SERPINC1
22 chronic venous insufficiency 10.6 PLAT SERPINE1
23 tuberculous empyema 10.6 PLG SERPINE1
24 occlusion precerebral artery 10.6 PLAT PLG
25 lung sarcoma 10.6 PLAT THBD
26 anterior spinal artery syndrome 10.6 PLG SERPINC1
27 basilar artery occlusion 10.6 PLAT PLG
28 heparin-induced thrombocytopenia 10.6 F3 SERPINC1
29 retinal vascular occlusion 10.6 PLG SERPINC1
30 sagittal sinus thrombosis 10.6 F3 SERPINC1
31 venous insufficiency 10.6 PLAT SERPINE1
32 purpura fulminans 10.5 F3 SERPINC1
33 placental abruption 10.5 SERPINC1 THBD
34 catastrophic antiphospholipid syndrome 10.5 F3 SERPINE1
35 hepatic infarction 10.5 F3 SERPINC1
36 carotid artery dissection 10.5 PLAT PLG
37 peripheral vertigo 10.5 F3 SERPINC1
38 dirofilariasis 10.5 PLAT PLG
39 protein c deficiency 10.5 SERPINC1 THBD
40 hemorrhagic fever 10.5 F3 SERPINC1
41 factor xii deficiency 10.5 F3 SERPINC1
42 fibrinolytic defect 10.5 PLAT SERPINC1 SERPINE1
43 conjugate gaze palsy 10.5 F3 PLG
44 hematopoietic stem cell transplantation 10.5
45 cerebral falx meningioma 10.5 F3 PLG
46 branch retinal artery occlusion 10.5 F3 SERPINC1
47 argentine hemorrhagic fever 10.5 PLAT SERPINC1 SERPINE1
48 pulmonary immaturity 10.5 F3 SERPINC1
49 protein s deficiency 10.4 F3 SERPINC1
50 femoral neuropathy 10.4 F3 PLG

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

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

26 (show all 14)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-103 9.53 SULT1A3
2 Increased shRNA abundance (Z-score > 2) GR00366-A-105 9.53 ABCB1
3 Increased shRNA abundance (Z-score > 2) GR00366-A-116 9.53 F3
4 Increased shRNA abundance (Z-score > 2) GR00366-A-127 9.53 ABCB1 F3 SULT1A3
5 Increased shRNA abundance (Z-score > 2) GR00366-A-13 9.53 F3
6 Increased shRNA abundance (Z-score > 2) GR00366-A-146 9.53 SULT1A3
7 Increased shRNA abundance (Z-score > 2) GR00366-A-163 9.53 F3
8 Increased shRNA abundance (Z-score > 2) GR00366-A-168 9.53 SULT1A3
9 Increased shRNA abundance (Z-score > 2) GR00366-A-199 9.53 ABCB1
10 Increased shRNA abundance (Z-score > 2) GR00366-A-43 9.53 SULT1A3
11 Increased shRNA abundance (Z-score > 2) GR00366-A-74 9.53 F3
12 Increased shRNA abundance (Z-score > 2) GR00366-A-78 9.53 F3
13 Increased shRNA abundance (Z-score > 2) GR00366-A-85 9.53 SULT1A3
14 Increased shRNA abundance (Z-score > 2) GR00366-A-92 9.53 SULT1A3

MGI Mouse Phenotypes related to Hepatic Veno-Occlusive Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.02 PLAT THPO PLG SERPINC1 ABCB1 SERPINE1
2 homeostasis/metabolism MP:0005376 9.97 F8 THBD PLAT THPO PLG SERPINC1
3 cardiovascular system MP:0005385 9.95 THBD PLAT PLG SERPINC1 ABCB1 SERPINE1
4 immune system MP:0005387 9.91 PLAT THPO PLG SERPINC1 ABCB1 SERPINE1
5 liver/biliary system MP:0005370 9.55 THBD PLG SERPINC1 ABCB1 SERPINE1
6 reproductive system MP:0005389 9.43 F8 THBD PLAT PLG SERPINC1 ABCB1
7 respiratory system MP:0005388 9.02 THBD PLAT PLG F3 SERPINE1
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 112)
# 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 21679-14-1, 75607-67-9 30751
3
Vidarabine Approved, Investigational Phase 4,Phase 2,Not Applicable 24356-66-9 32326 21704
4
Ledipasvir Approved Phase 4 1256388-51-8 67505836
5
Sofosbuvir Approved Phase 4 1190307-88-0 45375808
6 Antimetabolites Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
7 Antimetabolites, Antineoplastic Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
8 Immunosuppressive Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
9 Alkylating Agents Phase 4,Phase 2,Not Applicable
10 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
11 Antineoplastic Agents, Alkylating Phase 4,Phase 2,Not Applicable
12 Antiviral Agents Phase 4,Phase 3,Phase 2,Not Applicable
13 Ledipasvir, sofosbuvir drug combination Phase 4
14
Levoleucovorin Approved, Investigational Phase 2, Phase 3 68538-85-2
15
Methotrexate Approved Phase 2, Phase 3,Phase 1 1959-05-2, 59-05-2 126941
16 Defibrotide Approved, Investigational Phase 3,Phase 2 83712-60-1
17
Cytarabine Approved, Investigational Phase 3 147-94-4 6253
18
Mitoxantrone Approved, Investigational Phase 3 65271-80-9 4212
19
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2, Phase 3,Phase 1 59-30-3 6037
20
leucovorin Approved, Nutraceutical Phase 2, Phase 3,Phase 3,Phase 1 58-05-9 143 6006
21 Antidotes Phase 2, Phase 3
22 Antirheumatic Agents Phase 2, Phase 3,Phase 1,Not Applicable
23 Dermatologic Agents Phase 2, Phase 3,Phase 1,Not Applicable
24 Folic Acid Antagonists Phase 2, Phase 3,Phase 1
25 Hematinics Phase 2, Phase 3
26 Micronutrients Phase 2, Phase 3
27 Nucleic Acid Synthesis Inhibitors Phase 2, Phase 3,Phase 1
28 Protective Agents Phase 2, Phase 3,Not Applicable
29 Trace Elements Phase 2, Phase 3
30 Vitamin B Complex Phase 2, Phase 3,Phase 1
31 Vitamins Phase 2, Phase 3
32 Anticoagulants Phase 3,Phase 2
33 Anti-Inflammatory Agents Phase 3
34 Coagulants Phase 3
35 Fibrinolytic Agents Phase 3,Phase 2
36 Platelet Aggregation Inhibitors Phase 3,Phase 2
37 Liver Extracts Phase 3,Not Applicable
38 Analgesics Phase 3
39 Peripheral Nervous System Agents Phase 3
40 Topoisomerase Inhibitors Phase 3,Phase 2,Not Applicable
41 Folate Nutraceutical Phase 2, Phase 3,Phase 1
42 Vitamin B9 Nutraceutical Phase 2, Phase 3,Phase 1
43
Carboplatin Approved Phase 2 41575-94-4 10339178 498142 38904
44
Etoposide Approved Phase 2,Not Applicable 33419-42-0 36462
45
Lenograstim Approved, Investigational Phase 2,Not Applicable 135968-09-1
46
Mechlorethamine Approved, Investigational Phase 2,Not Applicable 51-75-2 4033
47
Melphalan Approved Phase 2,Phase 1,Not Applicable 148-82-3 4053 460612
48
Clofarabine Approved, Investigational Phase 2 123318-82-1 119182
49
Panobinostat Approved, Investigational Phase 1, Phase 2 404950-80-7 6918837
50
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860

Interventional clinical trials:

(show all 38)
# 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 Ledipasvir/Sofosbuvir Treatment for Hepatitis C in HCT Recipients. Recruiting NCT03279133 Phase 4 Ledipasvir 90mg/Sofosubvir 400mg
3 Busulfan Pharmacokinetic Analysis and GST Polymorphism in Adults Undergoing Hematological Stem Cell Transplantation Withdrawn NCT01071486 Phase 4 Hematological Stem Cell Transplantation
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 Chemotherapy Toxicity Reduction Via Urea Cycle Support Completed NCT00223730 Phase 2 Citrulline;Placebo
11 N2001-02: I-MIBG With Intensive Chemotherapy and Autologous Stem Cell Rescue for High-Risk Neuroblastoma Completed NCT00253435 Phase 2 Carboplatin;Etoposide;Melphalan
12 Busulfan/Clofarabine + Allogeneic Stem Cell Transplantation Completed NCT01643668 Phase 2 Busulfan;Clofarabine
13 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)
14 IV Busulfan Plus Bortezomib Conditioning Regimen for Second Autologous Stem Cell Transplant in Multiple Myeloma Patients Completed NCT01009840 Phase 2 IV busulfan;bortezomib
15 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
16 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
17 Bone Marrow Transplantation in Young Adults With Severe Sickle Cell Disease Completed NCT01565616 Phase 2 Conditioning Regimen with Bone Marrow Transplant
18 Defibrotide TMA Prophylaxis Pilot Trial Recruiting NCT03384693 Phase 2 Defibrotide
19 Thiopurine EnhAnced Maintenance Therapy Recruiting NCT02912676 Phase 1, Phase 2 Thioguanine (oral)
20 Allogeneic Stem Cell Transplantation in Relapsed/Refractory T-, NK/T-cell Lymphomas Recruiting NCT02859402 Phase 2 Busulfan;Fludarabine
21 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
22 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
23 Treosulfan-based Conditioning in Paediatric Patients With Haematological Malignancies Active, not recruiting NCT02333058 Phase 2 Treosulfan
24 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
25 Haploidentical Hematopoietic Stem Cell Transplantation Recruiting NCT02165007 Phase 1 peripheral blood stem cell graft that are CD34+ selected
26 Effects on Glutamine on the Outcome of Allogeneic Bone Marrow Transplant Recipients Unknown status NCT00563498 Not Applicable Glutamine
27 Prevention of the Hepatic Sinusoidal Obstruction Syndrome by Means of Anticoagulants Completed NCT00885950
28 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
29 MRD Testing Before and After Hematopoietic Cell Transplantation for Pediatric Acute Myeloid Leukemia Completed NCT01385787
30 Using Ultrasound Elastography to Predict Development of SOS Recruiting NCT02483481
31 Sinusoidal Obstruction Syndrome for Stem Cell Transplant Patients Biomarker Study Recruiting NCT03132337
32 European VOD Registry Recruiting NCT03032016
33 Liver Stiffness Measurement Predicts VOD/SOS Development (ELASTOVOD ITALIAN MULTICENTRIC STUDY) Recruiting NCT03426358 Not Applicable
34 Safety of Allogeneic Hematopoietic Cell Transplantation (HCT) For Patients With Classical Hodgkin Lymphoma (CHL) Treated With Nivolumab Recruiting NCT03200977
35 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
36 Compassionate Use of Defibrotide for Patients With Veno-occlusive Disease of the Liver No longer available NCT00143546 Defibrotide
37 Defibrotide Compassionate Use for Patients With Life Threatening Veno-Occlusive Disease of the Liver No longer available NCT00263978 Defibrotide
38 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 Marrow, Bone, Endothelial, T Cells, Testes, Kidney
Sources

Publications for Hepatic Veno-Occlusive Disease

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Articles related to Hepatic Veno-Occlusive Disease:

(show top 50) (show all 88)
# 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
Hepatic veno-occlusive disease induced by Chinese medicinal herbs. ( 29032695 )
2017
12
CT Features of Hepatic Veno-occlusive Disease: A Meta-analysis. ( 29191686 )
2017
13
Low, fixed dose defibrotide in management of hepatic veno-occlusive disease post stem cell transplantation. ( 28460209 )
2017
14
Defibrotide for Patients with Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome: Interim Results from a Treatment IND Study. ( 28285079 )
2017
15
Defibrotide for children and adults with hepatic veno-occlusive disease post hematopoietic cell transplantation. ( 28825848 )
2017
16
Usefulness of liver stiffness measurement in predicting hepatic veno-occlusive disease development in patients who undergo HSCT. ( 27941774 )
2016
17
In brief: Defibrotide (Defitelio) for hepatic veno-occlusive disease. ( 27603963 )
2016
18
Defibrotide in the treatment of hepatic veno-occlusive disease. ( 27843363 )
2016
19
Defibrotide for the Treatment of Hepatic Veno-Occlusive Disease: Final Results From the International Compassionate-Use Program. ( 27397724 )
2016
20
Prophylactic Treatment of Hepatic Veno-occlusive Disease During Stem Cell Transplantation. ( 27314223 )
2016
21
High Variability in the Reported Management of Hepatic Veno-Occlusive Disease in Children after Hematopoietic Stem Cell Transplantation. ( 27496218 )
2016
22
Long-awaited news for hepatic veno-occlusive disease. ( 27034420 )
2016
23
Hepatic Veno-Occlusive Disease after Hematopoietic Stem Cell Transplantation: Risk Factors and Stratification, Prophylaxis, and Treatment. ( 26431626 )
2016
24
Evaluation of the effects of preconditioning regimens on hepatic veno-occlusive disease in mice after hematopoietic stem cell transplantation. ( 25533545 )
2015
25
Hepatic veno-occlusive disease may develop in secondary iron overloaded mice after allogeneic hematopoietic stem cell transplantation with total body irradiation. ( 26457280 )
2015
26
Computed tomography findings of hepatic veno-occlusive disease caused by Sedum aizoon with histopathological correlation. ( 26517336 )
2015
27
Interventions for prophylaxis of hepatic veno-occlusive disease in people undergoing haematopoietic stem cell transplantation. ( 26017019 )
2015
28
Escalation to High-Dose Defibrotide in Patients with Hepatic Veno-Occlusive Disease. ( 26278046 )
2015
29
Interplay of Inflammation and Endothelial Dysfunction in Bone Marrow Transplantation: Focus on Hepatic Veno-Occlusive Disease. ( 26305238 )
2015
30
Defibrotide: a review of its use in severe hepatic veno-occlusive disease following haematopoietic stem cell transplantation. ( 25351934 )
2014
31
Experience from a single paediatric transplant centre with identification of some protective and risk factors concerning the development of hepatic veno-occlusive disease in children after allogeneic hematopoietic stem cell transplant. ( 24715523 )
2014
32
Sinusoidal obstruction syndrome (hepatic veno-occlusive disease). ( 25755580 )
2014
33
Graft Failure From Hepatic Veno-Occlusive Disease After a Liver Transplant: A Case Report. ( 25365427 )
2014
34
Experiences of t-PA use in moderate-to-severe hepatic veno-occlusive disease after hematopoietic SCT: is it still reasonable to use t-PA? ( 23892329 )
2013
35
Hepatic veno-occlusive disease after hematopoietic stem cell transplantation: novel insights to pathogenesis, current status of treatment, and future directions. ( 23089567 )
2013
36
Coexisting or underlying risk factors of hepatic veno-occlusive disease in pediatric hematopoietic stem cell transplant recipients receiving prophylaxis. ( 24128137 )
2013
37
Hepatic veno-occlusive disease resulting in tacrolimus toxicity after allogeneic hematopoietic stem cell transplantation. ( 23589797 )
2013
38
Hepatic veno-occlusive disease related to tacrolimus after pancreas transplantation. ( 23602212 )
2013
39
A murine model of hepatic veno-occlusive disease induced by allogeneic hematopoietic stem cell transplantation. ( 23579582 )
2013
40
Safety and efficacy of defibrotide for the treatment of severe hepatic veno-occlusive disease. ( 23606935 )
2012
41
Hepatic veno-occlusive disease with immunodeficiency (VODI): first reported case in the U.S. and identification of a unique mutation in Sp110. ( 22982295 )
2012
42
Plasma brain natriuretic peptide is associated with hepatic veno-occlusive disease and early mortality after allogeneic hematopoietic stem cell transplantation. ( 20173786 )
2010
43
Coagulation disturbances in paediatric patients with hepatic veno-occlusive disease after stem cells transplantation. ( 20229680 )
2010
44
Hepatic veno-occlusive disease and human herpes virus 7 infection in primary agammaglobulinemia. ( 19150678 )
2009
45
Early ultrasonographic diagnosis and clinical follow-up of hepatic veno-occlusive disease after allogeneic bone marrow transplantation in a patient with acute lymphoblastic leukemia. ( 19443980 )
2009
46
Late-onset hepatic veno-occlusive disease post autologous peripheral stem cell transplantation successfully treated with oral defibrotide. ( 20160371 )
2009
47
Hepatic veno-occlusive disease during chemotherapy for nephroblastoma: successful and safe treatment with defibrotide. Report of a clinical case. ( 19874245 )
2009
48
Angiogenesis alteration by defibrotide: implications for its mechanism of action in severe hepatic veno-occlusive disease. ( 18711003 )
2008
49
Hepatic veno-occlusive disease followed by esophageal varix rupture after hematopoietic stem cell transplantation in a 4-year-old boy with stage 4 neuroblastoma. ( 18176184 )
2008
50
Hepatic veno-occlusive disease after tandem autologous stem cell transplantation conditioned by melphalan. ( 18696182 )
2008
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:

# Super pathways Score Top Affiliating Genes
1
Response to elevated platelet cytosolic Ca2+ 66
Show member pathways
 12.84 F3 F8 PLAT PLG SERPINC1 SERPINE1
2
Collagen chain trimerization 66
Show member pathways
 12.48 F3 F8 SERPINC1 THBD
3
Folate Metabolism 1
Show member pathways
 11.99 PLAT PLG SERPINE1
4
Formation of Fibrin Clot (Clotting Cascade) 66
Show member pathways
 11.7 F3 F8 PLAT PLG SERPINC1 SERPINE1
5
Adhesion 4
11.67 F3 PLG SERPINE1
6
AGE-RAGE signaling pathway in diabetic complications 37
11.54 F3 SERPINE1 THBD
7
Complement and coagulation cascades 1 37
11.31 F3 F8 PLAT PLG SERPINC1 SERPINE1
8
Cell adhesion_ECM remodeling 22
11.28 PLAT PLG SERPINE1
9
A-beta Pathways: Plaque Formation and APP Metabolism 65
10.79 PLAT PLG
10
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 F3 F8 PLAT PLG SERPINC1 SERPINE1
2 cell surface GO:0009986 9.55 ABCB1 F3 PLAT PLG THBD
3 extracellular matrix GO:0031012 9.5 F3 PLAT SERPINE1
4 extracellular space GO:0005615 9.23 F3 F8 PLAT PLG SERPINC1 SERPINE1
5 platelet alpha granule lumen GO:0031093 9.13 F8 PLG SERPINE1

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

# Name GO ID Score Top Affiliating Genes
1 platelet degranulation GO:0002576 9.5 F8 PLG SERPINE1
2 fibrinolysis GO:0042730 9.43 PLAT PLG SERPINE1
3 blood coagulation GO:0007596 9.43 F3 F8 PLAT PLG SERPINC1 THBD
4 negative regulation of blood coagulation GO:0030195 9.37 SERPINE1 THBD
5 negative regulation of fibrinolysis GO:0051918 9.33 PLG SERPINE1 THBD
6 hemostasis GO:0007599 9.02 F3 F8 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 9.13 F3 PLAT PLG
2 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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