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MCID: HPT046
MIFTS: 60

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 77 54 60 56 45 15 74
Sinusoidal Obstruction Syndrome 54 60
Veno-Occlusive Disease 12 56
Hepatic Venoocclusive Disease 77
Hepatic Vein Thrombosis 74
Hepatic Vein Occlusion 74
Venoocclusive Disease 74
Budd-Chiari Syndrome 74

Characteristics:

Orphanet epidemiological data:

60
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: 34 35
Orphanet: 60  
Rare hepatic diseases


External Ids:

Disease Ontology 12 DOID:0080177
MeSH 45 D006504
ICD10 34 K76.5
MESH via Orphanet 46 D006504
ICD10 via Orphanet 35 K76.5
UMLS via Orphanet 75 C0019156
Orphanet 60 ORPHA890
Sources

Summaries for Hepatic Veno-Occlusive Disease

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NIH Rare Diseases : 54 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 Regulation of lipid metabolism Insulin signaling-generic cascades. The drugs Fludarabine and Vidarabine 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 : 77 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 260)
# Related Disease Score Top Affiliating Genes
1 pulmonary hypertension 31.0 F3 PLAT THBD
2 portal vein thrombosis 30.5 SERPINC1 SERPINE1
3 ischemic colitis 30.0 SERPINC1 SERPINE1 VEGFA
4 hypertension, essential 29.9 PLAT SERPINE1 THBD
5 purpura 29.8 F3 SERPINC1 THBD THPO
6 thrombosis 29.7 F3 PLAT SERPINC1 SERPINE1 THBD
7 hepatic venoocclusive disease with immunodeficiency 12.6
8 pulmonary venoocclusive disease 12.6
9 pulmonary venoocclusive disease 1, autosomal dominant 12.4
10 hematopoietic stem cell transplantation 10.8
11 pulmonary hypertension, primary, 1 10.4
12 leukemia 10.4
13 lymphoma 10.4
14 plasminogen activator inhibitor-1 deficiency 10.4 PLAT SERPINE1
15 platelet aggregation, spontaneous 10.4 PLAT SERPINC1
16 central serous chorioretinopathy 10.4 PLAT SERPINE1
17 posterior myocardial infarction 10.4 PLAT SERPINC1
18 encephalopathy, familial, with neuroserpin inclusion bodies 10.4 PLAT SERPINC1
19 mercury poisoning 10.4 PLAT THBD
20 aspirin allergy 10.4 SERPINC1 SERPINE1
21 postaxial acrofacial dysostosis 10.4 PLAT THBD
22 sticky platelet syndrome 10.4 SERPINC1 SERPINE1
23 scleroderma, familial progressive 10.4
24 subendocardial myocardial infarction 10.3 SERPINC1 SERPINE1
25 pleuropneumonia 10.3 PLAT SERPINC1
26 ligneous conjunctivitis 10.3 PLAT PLG
27 inferior myocardial infarction 10.3 PLAT PLG
28 central retinal artery occlusion 10.3 PLAT PLG
29 eisenmenger syndrome 10.3 PLAT THBD
30 post-thrombotic syndrome 10.3 SERPINC1 SERPINE1
31 alpha-2-plasmin inhibitor deficiency 10.3 PLG SERPINC1
32 occlusion precerebral artery 10.3 PLAT PLG
33 tuberculous empyema 10.3 PLG SERPINE1
34 basilar artery occlusion 10.3 PLAT PLG
35 fournier gangrene 10.3 F3 SERPINC1
36 heparin-induced thrombocytopenia 10.3 F3 SERPINC1
37 leukemia, acute myeloid 10.3
38 myeloid leukemia 10.3
39 sagittal sinus thrombosis 10.3 F3 SERPINC1
40 anterior spinal artery syndrome 10.3 PLG SERPINC1
41 spondyloocular syndrome 10.3
42 catastrophic antiphospholipid syndrome 10.3 F3 SERPINE1
43 purpura fulminans 10.3 F3 SERPINC1
44 chronic venous insufficiency 10.2 PLAT SERPINE1
45 hepatic infarction 10.2 F3 SERPINC1
46 venous insufficiency 10.2 PLAT SERPINE1
47 fibrinolytic defect 10.2 PLAT SERPINC1 SERPINE1
48 conjugate gaze palsy 10.2 F3 PLG
49 cerebral falx meningioma 10.2 F3 PLG
50 argentine hemorrhagic fever 10.2 PLAT SERPINC1 SERPINE1

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:

60 33 (show all 12)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hepatomegaly 60 33 hallmark (90%) Very frequent (99-80%) HP:0002240
2 renal insufficiency 60 33 hallmark (90%) Very frequent (99-80%) HP:0000083
3 ascites 60 33 hallmark (90%) Very frequent (99-80%) HP:0001541
4 abdominal pain 60 33 hallmark (90%) Very frequent (99-80%) HP:0002027
5 elevated hepatic transaminase 60 33 hallmark (90%) Very frequent (99-80%) HP:0002910
6 jaundice 60 33 hallmark (90%) Very frequent (99-80%) HP:0000952
7 respiratory failure 60 33 hallmark (90%) Very frequent (99-80%) HP:0002878
8 increased body weight 60 33 hallmark (90%) Very frequent (99-80%) HP:0004324
9 increased total bilirubin 60 33 hallmark (90%) Very frequent (99-80%) HP:0003573
10 prolonged partial thromboplastin time 60 33 frequent (33%) Frequent (79-30%) HP:0003645
11 hepatic encephalopathy 60 33 very rare (1%) Very rare (<4-1%) HP:0002480
12 abnormality of coagulation 60 Frequent (79-30%)

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

27 (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:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.09 ABCB1 F3 PLAT PLG SERPINC1 SERPINE1
2 cardiovascular system MP:0005385 10.06 ABCB1 F3 PLAT PLG SERPINC1 SERPINE1
3 homeostasis/metabolism MP:0005376 10.06 ABCB1 F3 PLAT PLG SERPINC1 SERPINE1
4 immune system MP:0005387 10.02 ABCB1 F3 PLAT PLG SERPINC1 SERPINE1
5 integument MP:0010771 9.8 F3 PLAT PLG SERPINE1 THBD VEGFA
6 liver/biliary system MP:0005370 9.73 ABCB1 PLG SERPINC1 SERPINE1 THBD VEGFA
7 renal/urinary system MP:0005367 9.55 PLG SERPINC1 SERPINE1 THBD VEGFA
8 reproductive system MP:0005389 9.43 ABCB1 PLAT PLG SERPINC1 THBD VEGFA
9 respiratory system MP:0005388 9.1 F3 PLAT PLG SERPINE1 THBD VEGFA
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 152)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Fludarabine Approved Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 75607-67-9, 21679-14-1 30751
2
Vidarabine Approved, Investigational Phase 4,Phase 1,Phase 2,Not Applicable 24356-66-9 21704 32326
3
Busulfan Approved, Investigational Phase 4,Phase 2,Not Applicable 55-98-1 2478
4 Antimetabolites Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
5 Antimetabolites, Antineoplastic Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
6 Immunosuppressive Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
7 Immunologic Factors Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
8 Antiviral Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
9 Alkylating Agents Phase 4,Phase 2,Not Applicable
10 Antineoplastic Agents, Alkylating Phase 4,Phase 2,Not Applicable
11 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
12
leucovorin Approved Phase 2, Phase 3,Phase 3,Phase 1 58-05-9 143 6006
13
Methotrexate Approved Phase 2, Phase 3,Phase 1 1959-05-2, 59-05-2 126941
14
Levoleucovorin Approved, Investigational Phase 2, Phase 3 68538-85-2
15
Defibrotide Approved, Investigational Phase 3,Phase 2 83712-60-1
16
Cytarabine Approved, Experimental, Investigational Phase 3,Phase 2 147-94-4, 65-46-3 6253
17
Mitoxantrone Approved, Investigational Phase 3 65271-80-9 4212
18
inotuzumab ozogamicin Approved, Investigational Phase 3,Phase 2 635715-01-4
19
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2, Phase 3,Phase 1 59-30-3 6037
20 Nucleic Acid Synthesis Inhibitors Phase 2, Phase 3,Phase 1
21 Vitamin B Complex Phase 2, Phase 3,Phase 1
22 Antirheumatic Agents Phase 2, Phase 3,Phase 1,Not Applicable
23 Antidotes Phase 2, Phase 3
24 Vitamins Phase 2, Phase 3
25 Folate Phase 2, Phase 3,Phase 1
26 Trace Elements Phase 2, Phase 3
27 Hematinics Phase 2, Phase 3
28 Nutrients Phase 2, Phase 3
29 Micronutrients Phase 2, Phase 3
30 Protective Agents Phase 2, Phase 3,Not Applicable
31 Folic Acid Antagonists Phase 2, Phase 3,Phase 1
32 Vitamin B9 Phase 2, Phase 3,Phase 1
33 Dermatologic Agents Phase 2, Phase 3,Phase 1,Not Applicable
34 Anticoagulants Phase 3,Phase 2
35 Fibrinolytic Agents Phase 3,Phase 2
36 Platelet Aggregation Inhibitors Phase 3,Phase 2
37 Anti-Inflammatory Agents Phase 3,Phase 2
38 Coagulants Phase 3
39 Liver Extracts Phase 3,Not Applicable
40 Peripheral Nervous System Agents Phase 3,Phase 2
41 Topoisomerase Inhibitors Phase 3,Phase 2,Not Applicable
42 Analgesics Phase 3,Phase 2
43
Thioguanine Approved Phase 1, Phase 2 154-42-7 2723601
44
Mercaptopurine Approved Phase 1, Phase 2 50-44-2 667490
45
carbamide peroxide Approved Phase 2 124-43-6
46
Etoposide Approved Phase 2,Not Applicable 33419-42-0 36462
47
Mechlorethamine Approved, Investigational Phase 2,Not Applicable 51-75-2 4033
48
Carboplatin Approved Phase 2 41575-94-4 10339178 38904 498142
49
Melphalan Approved Phase 2,Phase 1,Not Applicable 148-82-3 460612 4053
50
Lenograstim Approved, Investigational Phase 2,Not Applicable 135968-09-1

Interventional clinical trials:

(show all 46)
# 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 Ultrasound Elastography to Predict Development of SOS Recruiting NCT03858530 Phase 4
3 Busulfan Pharmacokinetic Analysis and GST Polymorphism in Adults Undergoing Hematological Stem Cell Transplantation Withdrawn NCT01071486 Phase 4 Hematological Stem Cell Transplantation
4 Ledipasvir/Sofosbuvir Treatment for Hepatitis C in HCT Recipients. Withdrawn NCT03279133 Phase 4 Ledipasvir 90mg/Sofosubvir 400mg
5 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
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 Completed NCT00358501 Phase 3 Defibrotide
8 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
9 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
10 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
11 HLA 10/10 Matched Unrelated Donor vs Haploidentical Allogenic Hematopoietic Stem Cell Transplantation Not yet recruiting NCT03655145 Phase 3
12 Thiopurine EnhAnced Maintenance Therapy Unknown status NCT02912676 Phase 1, Phase 2 Thioguanine (oral)
13 Chemotherapy Toxicity Reduction Via Urea Cycle Support Completed NCT00223730 Phase 2 Citrulline;Placebo
14 N2001-02: I-MIBG With Intensive Chemotherapy and Autologous Stem Cell Rescue for High-Risk Neuroblastoma Completed NCT00253435 Phase 2 Carboplatin;Etoposide;Melphalan
15 Busulfan/Clofarabine + Allogeneic Stem Cell Transplantation Completed NCT01643668 Phase 2 Busulfan;Clofarabine
16 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)
17 IV Busulfan Plus Bortezomib Conditioning Regimen for Second Autologous Stem Cell Transplant in Multiple Myeloma Patients Completed NCT01009840 Phase 2 IV busulfan;bortezomib
18 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
19 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
20 Bone Marrow Transplantation in Young Adults With Severe Sickle Cell Disease Completed NCT01565616 Phase 2 Conditioning Regimen with Bone Marrow Transplant
21 Defibrotide TMA Prophylaxis Pilot Trial Recruiting NCT03384693 Phase 2 Defibrotide
22 Inotuzumab Ozogamicin for Children With MRD Positive CD22+ Lymphoblastic Leukemia Recruiting NCT03913559 Phase 2 Inotuzumab ozogamicin;Methotrexate;Hydrocortisone;Cytarabine;Diphenhydramine;Acetaminophen;Methylprednisolone
23 Allogeneic Stem Cell Transplantation in Relapsed/Refractory T-, NK/T-cell Lymphomas Recruiting NCT02859402 Phase 2 Busulfan;Fludarabine
24 IMRT-TMI With Fludarabine as Myeloablative Conditioning for Allogeneic HSCT Recruiting NCT03696537 Phase 1, Phase 2 Fludarabine
25 Inotuzumab Ozogamicin in Treating Younger Patients With B-Lymphoblastic Lymphoma or Relapsed or Refractory CD22 Positive B Acute Lymphoblastic Leukemia Recruiting NCT02981628 Phase 2
26 Conditioning SCID Infants Diagnosed Early Recruiting NCT03619551 Phase 2 Busulfan
27 Inotuzumab Ozogamicin and Blinatumomab in Treating Patients With Newly Diagnosed, Recurrent, or Refractory CD22-Positive B-Lineage Acute Lymphoblastic Leukemia Recruiting NCT03739814 Phase 2
28 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
29 HLA-Mismatched Unrelated Donor Bone Marrow Transplantation With Post-Transplantation Cyclophosphamide Active, not 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
30 Treosulfan-based Conditioning in Paediatric Patients With Haematological Malignancies Active, not recruiting NCT02333058 Phase 2 Treosulfan
31 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
32 Haploidentical Hematopoietic Stem Cell Transplantation Recruiting NCT02165007 Phase 1 peripheral blood stem cell graft that are CD34+ selected
33 Effects on Glutamine on the Outcome of Allogeneic Bone Marrow Transplant Recipients Unknown status NCT00563498 Not Applicable Glutamine
34 Prevention of the Hepatic Sinusoidal Obstruction Syndrome by Means of Anticoagulants Completed NCT00885950
35 Using Ultrasound Elastography to Predict Development of SOS Completed NCT02483481
36 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
37 MRD Testing Before and After Hematopoietic Cell Transplantation for Pediatric Acute Myeloid Leukemia Completed NCT01385787
38 Sinusoidal Obstruction Syndrome for Stem Cell Transplant Patients Biomarker Study Recruiting NCT03132337
39 Liver Stiffness Measurement Predicts VOD/SOS Development (ELASTOVOD ITALIAN MULTICENTRIC STUDY) Recruiting NCT03426358 Not Applicable
40 Safety of Allogeneic Hematopoietic Cell Transplantation (HCT) For Patients With Classical Hodgkin Lymphoma (CHL) Treated With Nivolumab Recruiting NCT03200977
41 European VOD Registry Active, not recruiting NCT03032016
42 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
43 Jazz HCT Elastography Not yet recruiting NCT03865589 Not Applicable
44 Compassionate Use of Defibrotide for Patients With Veno-occlusive Disease of the Liver No longer available NCT00143546 Defibrotide
45 Defibrotide Compassionate Use for Patients With Life Threatening Veno-Occlusive Disease of the Liver No longer available NCT00263978 Defibrotide
46 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 74 / NDF-RT 52 :


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:

42
Liver, Bone, Bone Marrow, Endothelial, Myeloid, Kidney, Heart
Sources

Publications for Hepatic Veno-Occlusive Disease

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Articles related to Hepatic Veno-Occlusive Disease:

(show top 50) (show all 462)
# Title Authors Year
1
Liver sinusoidal obstruction syndrome associated with trastuzumab emtansine treatment for breast cancer. ( 31023619 )
2019
2
Liver Stiffness Measurement Allows Early Diagnosis of Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome in Adult Patients Who Undergo Hematopoietic Stem Cell Transplantation: Results from a Monocentric Prospective Study. ( 30660772 )
2019
3
The incidence and risk factors of hepatic veno-occlusive disease after hematopoietic stem cell transplantation in Taiwan. ( 30666432 )
2019
4
A Retrospective Analysis of Antithrombin III Replacement Therapy for the Treatment of Hepatic Sinusoidal Obstruction Syndrome in Children Following Hematopoietic Stem Cell Transplantation. ( 30676436 )
2019
5
Clinical effects of recombinant thrombomodulin and defibrotide on sinusoidal obstruction syndrome after allogeneic hematopoietic stem cell transplantation. ( 30127466 )
2019
6
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 )
2019
7
Hepatic veno-occlusive disease following sirolimus-based immune suppression. ( 29895929 )
2019
8
Early Clinical Predictors of Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome after Myeloablative Stem Cell Transplantation. ( 30081073 )
2019
9
Treatment of hepatic veno-occlusive disease in children with N-acetylcysteine. ( 30350914 )
2019
10
Ultrasound elastography techniques for diagnosis and follow-up of hepatic veno-occlusive disease. ( 30679827 )
2019
11
Risk Factors for Development of and Progression of Hepatic Veno-occlusive Disease/Sinusoidal Obstruction Syndrome. ( 30797942 )
2019
12
Treprostinil reduces endothelial damage in murine sinusoidal obstruction syndrome. ( 30535954 )
2019
13
Indirect costs associated with premature mortality among those with veno-occlusive disease/sinusoidal obstruction syndrome with multiorgan dysfunction post-hematopoietic stem-cell-transplant. ( 30588050 )
2019
14
Expert consensus on the clinical management of pyrrolizidine alkaloid-induced hepatic sinusoidal obstruction syndrome. ( 30669184 )
2019
15
Assessment of the proposed EBMT pediatric criteria for diagnosis and severity grading of sinusoidal obstruction syndrome. ( 30683907 )
2019
16
Gadoxetic Acid-Enhanced Hepatobiliary-Phase Magnetic Resonance Imaging for Pyrrolizidine Alkaloid-Induced Hepatic Sinusoidal Obstruction Syndrome and Association with Liver Function. ( 30718698 )
2019
17
Quantitative Analysis of CT Images in Patients with Pyrrolizidine Alkaloid-Induced Sinusoidal Obstruction Syndrome. ( 30778132 )
2019
18
Systematic review of defibrotide studies in the treatment of veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS). ( 30804485 )
2019
19
Validation of treatment outcomes according to revised severity criteria from European Society for Blood and Marrow Transplantation (EBMT) for sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD). ( 30809031 )
2019
20
(-)-Epicatechin attenuates hepatic sinusoidal obstruction syndrome by inhibiting liver oxidative and inflammatory injury. ( 30822691 )
2019
21
Peripheral hepatic sinusoidal obstruction syndrome due to oxaliplatin-based chemotherapy. ( 30847217 )
2019
22
Publisher Correction: Systematic review of defibrotide studies in the treatment of veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS). ( 30932018 )
2019
23
Incidence, risk factors and outcomes of sinusoidal obstruction syndrome after haploidentical allogeneic stem cell transplantation. ( 31053879 )
2019
24
A noninvasive diagnostic approach using per-rectal portal scintigraphy for sinusoidal obstruction syndrome after allogeneic hematopoietic cell transplantation. ( 31089276 )
2019
25
Assessment of hepatic sinusoidal obstruction syndrome with intravoxel incoherent motion diffusion-weighted imaging: An experimental study in a rat model. ( 31094055 )
2019
26
Hepatic sinusoidal obstruction syndrome following haematopoietic stem cell transplantation. A report of 33 cases. ( 29887172 )
2019
27
Sinusoidal obstruction syndrome/veno-occlusive disease after high-dose intravenous busulfan/melphalan conditioning therapy in high-risk Ewing Sarcoma. ( 29335623 )
2018
28
Novel Ultrasonographic Scoring System of Sinusoidal Obstruction Syndrome after Hematopoietic Stem Cell Transplantation. ( 29803752 )
2018
29
Gallbladder wall oedema and ascites are independent predictors of progression to hepatic veno-occlusive disease for children with hematopoietic stem cell transplantation. ( 29294155 )
2018
30
Identification and Management: Sinusoidal Obstruction Syndrome/Veno-Occlusive Disease Eelated to Hematopoietic Stem Cell Transplantation
. ( 29350698 )
2018
31
Association of CTH variant with sinusoidal obstruction syndrome in children receiving intravenous busulfan and cyclophosphamide before hematopoietic stem cell transplantation. ( 27779248 )
2018
32
Low, fixed dose defibrotide in management of hepatic veno-occlusive disease post stem cell transplantation. ( 28460209 )
2018
33
CT Features of Hepatic Veno-occlusive Disease: A Meta-analysis. ( 29191686 )
2018
34
Hepatic veno-occlusive disease during isavuconazole administration. ( 29278102 )
2018
35
Hepatobiliary and Pancreatic: Pregnancy induced hepatic veno-occlusive disease requiring liver transplantation. ( 29284078 )
2018
36
Evaluation of the therapeutic effects of rice husk nanosilica combined with platelet-derived growth factor in hepatic veno-occlusive disease. ( 29301090 )
2018
37
Defibrotide sodium for the treatment of hepatic veno-occlusive disease/sinusoidal obstruction syndrome. ( 29301447 )
2018
38
Late-Onset Hepatic Veno-Occlusive Disease after Allografting: Report of Two Cases with Atypical Clinical Features Successfully Treated with Defibrotide. ( 29326798 )
2018
39
Hepatic veno-occlusive disease related to Gynura segetum: A case report. ( 29703039 )
2018
40
Final results from a defibrotide treatment-IND study for patients with hepatic veno-occlusive disease/sinusoidal obstruction syndrome. ( 29767845 )
2018
41
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
42
Risk factors for hepatic veno-occlusive disease caused by Gynura segetum: a retrospective study. ( 30367628 )
2018
43
Comprehensive analysis of serum microRNAs in hepatic sinusoidal obstruction syndrome (SOS) in rats: implication as early phase biomarkers for SOS. ( 30019166 )
2018
44
New Ultrasound Techniques Challenge the Diagnosis of Sinusoidal Obstruction Syndrome. ( 30076031 )
2018
45
Soluble Thrombomodulin Attenuates Endothelial Cell Damage in Hepatic Sinusoidal Obstruction Syndrome. ( 30348695 )
2018
46
Gynura Rhizoma containing pyrrolizidine alkaloids induces the hepatic sinusoidal obstruction syndrome in mice via upregulating fibrosis-related factors. ( 30367152 )
2018
47
Role of Virtual Touch Tissue Imaging Quantification in the Assessment of Hepatic Sinusoidal Obstruction Syndrome in a Rat Model. ( 30561767 )
2018
48
Sinusoidal Obstruction Syndrome (SOS) in Multiple Myeloma with Renal Failure. ( 30631618 )
2018
49
Sinusoidal obstruction syndrome in a paediatric patient with acute lymphoblastic leukaemia after completion of reinduction therapy according to ALL Intercontinental Berlin-Frankfurt-Münster 2009. ( 30783392 )
2018
50
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 )
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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 17)
# Super pathways Score Top Affiliating Genes
1
Response to elevated platelet cytosolic Ca2+ 67
Show member pathways
 12.84 F3 PLAT PLG SERPINC1 SERPINE1 THBD
2
Regulation of lipid metabolism Insulin signaling-generic cascades 23
Show member pathways
 12.61 PLAT PLG SERPINE1 VEGFA
3
Selenium Micronutrient Network 1
Show member pathways
 12.06 PLAT PLG SERPINE1
4
Fluid shear stress and atherosclerosis 38
11.8 PLAT THBD VEGFA
5
Adhesion 4
11.72 F3 PLG SERPINE1
6
Formation of Fibrin Clot (Clotting Cascade) 67
Show member pathways
 11.63 F3 PLAT PLG SERPINC1 SERPINE1 THBD
7
AGE-RAGE signaling pathway in diabetic complications 38
11.62 F3 SERPINE1 THBD VEGFA
8
HIF-1-alpha transcription factor network 1
11.42 ABCB1 SERPINE1 VEGFA
9
Cell adhesion_ECM remodeling 23
11.37 PLAT PLG SERPINE1
10
Complement and coagulation cascades 1 38
11.24 F3 PLAT PLG SERPINC1 SERPINE1 THBD
11
Cell adhesion_Plasmin signaling 23
11.23 PLAT PLG VEGFA
12
Photodynamic therapy-induced HIF-1 survival signaling 1
11.14 SERPINE1 VEGFA
13
HIF-2-alpha transcription factor network 1
11.09 SERPINE1 VEGFA
14
Glypican 1 network 1
10.94 SERPINC1 VEGFA
15
A-beta Pathways: Plaque Formation and APP Metabolism 66
10.87 PLAT PLG
16
NOTCH1 regulation of human endothelial cell calcification 1
10.75 PLAT VEGFA
17
Dissolution of Fibrin Clot 67
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 9.87 F3 PLAT PLG SERPINC1 SERPINE1 THPO
2 extracellular space GO:0005615 9.76 F3 PLAT PLG SERPINC1 SERPINE1 THBD
3 platelet alpha granule lumen GO:0031093 9.43 PLG SERPINE1 VEGFA
4 cell surface GO:0009986 9.43 ABCB1 F3 PLAT PLG THBD VEGFA
5 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 positive regulation of angiogenesis GO:0045766 9.61 F3 SERPINE1 VEGFA
2 blood coagulation GO:0007596 9.55 F3 PLAT PLG SERPINC1 THBD
3 platelet degranulation GO:0002576 9.54 PLG SERPINE1 VEGFA
4 fibrinolysis GO:0042730 9.43 PLAT PLG SERPINE1
5 negative regulation of blood coagulation GO:0030195 9.4 SERPINE1 THBD
6 positive regulation of positive chemotaxis GO:0050927 9.32 F3 VEGFA
7 negative regulation of fibrinolysis GO:0051918 9.13 PLG SERPINE1 THBD
8 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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 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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