MCID: PLM025
MIFTS: 44

Pulmonary Venoocclusive Disease

Categories: Rare diseases, Cardiovascular diseases, Genetic diseases, Respiratory diseases

Aliases & Classifications for Pulmonary Venoocclusive Disease

MalaCards integrated aliases for Pulmonary Venoocclusive Disease:

Name: Pulmonary Venoocclusive Disease 12 50 25 13 14
Pulmonary Veno-Occlusive Disease 12 25 29 52 42 69
Obstructive Disease of the Pulmonary Veins 50 25
Pvod 50 25
Pulmonary Venoocclusive Disease 1, Autosomal Dominant 69
Venous Form of Primary Pulmonary Hypertension 25
Isolated Pulmonary Venous Sclerosis 25

Classifications:



External Ids:

Disease Ontology 12 DOID:5453
MeSH 42 D011668
NCIt 47 C85039
SNOMED-CT 64 89420002
UMLS 69 C0034091

Summaries for Pulmonary Venoocclusive Disease

Genetics Home Reference : 25 Pulmonary veno-occlusive disease (PVOD) is characterized by the blockage (occlusion) of the blood vessels that carry oxygen-rich (oxygenated) blood from the lungs to the heart (the pulmonary veins). The occlusion is caused by a buildup of abnormal fibrous tissue in the small veins in the lungs, which narrows the vessels and impairs blood flow. Because blood flow through the lungs is difficult, pressure rises in the vessels that carry blood that needs to be oxygenated to the lungs from the heart (the pulmonary arteries). Increased pressure in these vessels is known as pulmonary arterial hypertension.

MalaCards based summary : Pulmonary Venoocclusive Disease, also known as pulmonary veno-occlusive disease, is related to pulmonary venoocclusive disease 1 and pulmonary venoocclusive disease 2, and has symptoms including hemoptysis An important gene associated with Pulmonary Venoocclusive Disease is BMPR2 (Bone Morphogenetic Protein Receptor Type 2), and among its related pathways/superpathways is ALK1 signaling events. The drugs Menadione and Phytonadione have been mentioned in the context of this disorder. Affiliated tissues include lung, heart and bone, and related phenotypes are Decreased substrate adherent cell growth and behavior/neurological

Wikipedia : 72 Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension caused by progressive... more...

Related Diseases for Pulmonary Venoocclusive Disease

Graphical network of the top 20 diseases related to Pulmonary Venoocclusive Disease:



Diseases related to Pulmonary Venoocclusive Disease

Symptoms & Phenotypes for Pulmonary Venoocclusive Disease

UMLS symptoms related to Pulmonary Venoocclusive Disease:


hemoptysis

GenomeRNAi Phenotypes related to Pulmonary Venoocclusive Disease according to GeneCards Suite gene sharing:

26
id Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased substrate adherent cell growth GR00193-A-1 9.23 ACVRL1 APLNR BMPR2
2 Decreased substrate adherent cell growth GR00193-A-3 9.23 APLNR
3 Decreased substrate adherent cell growth GR00193-A-4 9.23 ACVRL1 EIF2AK4
4 Decreased human cytomegalovirus (HCMV) strain AD169 replication GR00248-A 9.13 ACVRL1 BMPR2 EIF2AK4

MGI Mouse Phenotypes related to Pulmonary Venoocclusive Disease:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.63 ACVRL1 APLNR BMPR2 EIF2AK4 HTR2A KCNK3
2 muscle MP:0005369 9.35 ACVRL1 APLNR BMPR2 EIF2AK4 HTR2A
3 respiratory system MP:0005388 8.92 ACVRL1 APLNR BMPR2 KCNK3

Drugs & Therapeutics for Pulmonary Venoocclusive Disease

Drugs for Pulmonary Venoocclusive Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 54)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Menadione Approved, Nutraceutical Phase 4 58-27-5 4055
2
Phytonadione Approved, Nutraceutical Phase 4 84-80-0 4812 5284607
3 Anticoagulants Phase 4,Phase 3
4 Antifibrinolytic Agents Phase 4
5 Coagulants Phase 4
6 Fibrin Fibrinogen Degradation Products Phase 4
7 Fibrin fragment D Phase 4
8 Hemostatics Phase 4
9 Vitamins Phase 4
10 Menaquinone Nutraceutical Phase 4
11 naphthoquinone Nutraceutical Phase 4
12 Vitamin K Nutraceutical Phase 4
13
Dalteparin Approved Phase 3 9005-49-6
14
Heparin Approved, Investigational Phase 3 9005-49-6 772 46507594
15
Ethanol Approved Phase 3 64-17-5 702
16 calcium heparin Phase 3
17 Fibrinolytic Agents Phase 3
18 Heparin, Low-Molecular-Weight Phase 3
19 Cola Nutraceutical Phase 3
20
Ursodeoxycholic acid Approved, Investigational Phase 2 128-13-2 31401
21
Bevacizumab Approved, Investigational Phase 1, Phase 2 216974-75-3
22 Orange Approved, Nutraceutical Phase 1, Phase 2
23 Cholagogues and Choleretics Phase 2
24 Gastrointestinal Agents Phase 2
25 Angiogenesis Inhibitors Phase 1, Phase 2
26 Angiogenesis Modulating Agents Phase 1, Phase 2
27 Anti-Bacterial Agents Phase 1, Phase 2
28 Imatinib Mesylate Phase 1, Phase 2 123596
29 Protein Kinase Inhibitors Phase 1, Phase 2
30
Miconazole Approved, Investigational, Vet_approved Phase 1 22916-47-8 4189
31
Bradykinin Investigational Phase 1 58-82-2 439201
32 Antifungal Agents Phase 1
33 Anti-Infective Agents Phase 1
34 Cytochrome P-450 CYP2C9 Inhibitors Phase 1
35 Cytochrome P-450 CYP3A Inhibitors Phase 1
36 Cytochrome P-450 Enzyme Inhibitors Phase 1
37 Hormone Antagonists Phase 1
38 Hormones Phase 1
39 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 1
40 Kininogens Phase 1
41 omega-N-Methylarginine Phase 1
42 Steroid Synthesis Inhibitors Phase 1
43 Vasodilator Agents Phase 1
44
Bosentan Approved, Investigational 147536-97-8 104865
45
Cocaine Approved, Illicit 50-36-2 5760 446220
46
Methamphetamine Approved, Illicit 537-46-2 10836
47
Zinc Approved 7440-66-6 32051 23994
48
Acetylcholine Approved 51-84-3 187
49
Nitroprusside Approved 15078-28-1 11963622
50
Verapamil Approved 52-53-9 2520

Interventional clinical trials:

(show all 17)

id Name Status NCT ID Phase Drugs
1 DULCIS (D-dimer and ULtrasonography in Combination Italian Study) Completed NCT00954395 Phase 4
2 Standard Therapy With or Without Dalteparin in Treating Patients With Advanced Breast, Lung, Colorectal, or Prostate Cancer Completed NCT00003674 Phase 3 dalteparin;standard therapy
3 Effect of Aerobic EXercise on MiCroVAscular RarefacTION in Chinese Mild HyperteNsive Patients(EXCAVATION-CHN1) Recruiting NCT02817204 Phase 3
4 Ursodeoxycholic Acid in Chronic Heart Failure Unknown status NCT00285597 Phase 2 Ursodeoxycholic Acid
5 Chemotherapy Toxicity Reduction Via Urea Cycle Support Completed NCT00223730 Phase 2 Citrulline;Placebo
6 Pilot Study Using Avastin and Gleevec to Treat the Progression of Intraluminal Pulmonary Vein Stenosis Active, not recruiting NCT00891527 Phase 1, Phase 2 Bevacizumab (Avastin) and Imatinib Mesylate (Gleevec)
7 Effects of Miconazole on Blood Flow Completed NCT00004575 Phase 1 Miconazole, L-NMMA, Bradykinin
8 Technical Development of Cardiovascular Magnetic Resonance Imaging Recruiting NCT00064896 Phase 1
9 Effects on Glutamine on the Outcome of Allogeneic Bone Marrow Transplant Recipients Unknown status NCT00563498 Glutamine
10 Pharmacogenomics in Pulmonary Arterial Hypertension Unknown status NCT00593905 Sitaxsentan;Bosentan, Ambrisentan
11 Small Intestinal Absorption in Patients With Chronic Obstructive Pulmonary Disease and Cor Pulmonale Completed NCT03243994
12 Combustion Derived Air Pollution and Vascular Function Completed NCT00775099
13 Cardiovascular Effects of Chronic Snus Use Completed NCT02564289
14 The Effect of a Retrofit Particle Trap on the Vascular Effects of Diesel Exhaust Inhalation Completed NCT00745446
15 Registration Study for Rare Type of Pulmonary Hypertension Recruiting NCT03169010
16 Technical Development of Cardiovascular Magnetic Resonance Imaging Recruiting NCT00027170
17 A Non-Interventional, Patient Registry For The Collection Of Pre-Defined Safety Data In Patients Prescribed Thelin Terminated NCT00995566 Sitaxentan sodium

Search NIH Clinical Center for Pulmonary Venoocclusive Disease

Cochrane evidence based reviews: pulmonary veno-occlusive disease

Genetic Tests for Pulmonary Venoocclusive Disease

Genetic tests related to Pulmonary Venoocclusive Disease:

id Genetic test Affiliating Genes
1 Pulmonary Veno-Occlusive Disease 29

Anatomical Context for Pulmonary Venoocclusive Disease

MalaCards organs/tissues related to Pulmonary Venoocclusive Disease:

39
Lung, Heart, Bone, Bone Marrow, Prostate, Small Intestine

Publications for Pulmonary Venoocclusive Disease

Articles related to Pulmonary Venoocclusive Disease:

(show all 23)
id Title Authors Year
1
Small Sample Lung Biopsy Findings in Patients With Clinicoradiologic Suspicion of Pulmonary Venoocclusive Disease-Pulmonary Capillary Hemangiomatosis. ( 27623416 )
2016
2
Pulmonary venoocclusive disease in childhood. ( 24503954 )
2014
3
Characteristics of patients with pulmonary venoocclusive disease awaiting transplantation. ( 25296345 )
2014
4
Single-lobe lung transplantation for rapidly deteriorating pulmonary venoocclusive disease. ( 23336879 )
2013
5
Postradiofrequency ablation inflammatory pseudotumor associated with pulmonary venoocclusive disease: case report and review of the literature. ( 23352326 )
2013
6
Computed tomography findings of pulmonary venoocclusive disease in scleroderma patients presenting with precapillary pulmonary hypertension. ( 22549387 )
2012
7
Rapidly progressive pulmonary venoocclusive disease in young women taking oral contraceptives. ( 22884388 )
2012
8
Pulmonary venoocclusive disease and failure of specific therapy. ( 19809068 )
2009
9
Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. ( 15208112 )
2004
10
"High probability" perfusion lung scans in pulmonary venoocclusive disease. ( 11069842 )
2000
11
High-resolution CT of the chest in four patients with pulmonary capillary hemangiomatosis or pulmonary venoocclusive disease. ( 9798872 )
1998
12
Pulmonary venoocclusive disease: CT findings in eight patients. ( 8819387 )
1996
13
Segmental contour pattern in a case of pulmonary venoocclusive disease. ( 8403701 )
1993
14
Pulmonary venoocclusive disease: CT findings before and after single-lung transplantation. ( 8456659 )
1993
15
Pediatric case of the day. Pulmonary venoocclusive disease. ( 8498252 )
1993
16
Pulmonary venoocclusive disease in a patient with systemic lupus erythematosus. ( 8014950 )
1993
17
Fatal pulmonary venoocclusive disease after use of oral contraceptives. ( 1462933 )
1992
18
Fatal pulmonary venoocclusive disease secondary to a generalized venulopathy: a new syndrome presenting with facial swelling and pericardial tamponade. ( 1994922 )
1991
19
Pulmonary venoocclusive disease following bone marrow transplantation. ( 2660361 )
1989
20
CT findings in pulmonary venoocclusive disease. ( 6736380 )
1984
21
Pulmonary venoocclusive disease in pregnancy. ( 6738961 )
1984
22
Pulmonary venoocclusive disease: case report and review of the literature. ( 403796 )
1977
23
Pulmonary venoocclusive disease. ( 962409 )
1976

Variations for Pulmonary Venoocclusive Disease

ClinVar genetic disease variations for Pulmonary Venoocclusive Disease:

6
id Gene Variation Type Significance SNP ID Assembly Location
1 BMPR2 NM_001204.6(BMPR2): c.1120delA (p.I374*) deletion Pathogenic rs1085307300 GRCh37 Chromosome 2, 203395669: 203395669

Expression for Pulmonary Venoocclusive Disease

Search GEO for disease gene expression data for Pulmonary Venoocclusive Disease.

Pathways for Pulmonary Venoocclusive Disease

Pathways related to Pulmonary Venoocclusive Disease according to GeneCards Suite gene sharing:

id Super pathways Score Top Affiliating Genes
1
Show member pathways
10.05 ACVRL1 BMPR2

GO Terms for Pulmonary Venoocclusive Disease

Cellular components related to Pulmonary Venoocclusive Disease according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 dendrite GO:0030425 9.33 ACVRL1 BMPR2 HTR2A
2 caveola GO:0005901 9.26 BMPR2 HTR2A
3 neuronal cell body GO:0043025 9.13 ACVRL1 BMPR2 HTR2A
4 integral component of plasma membrane GO:0005887 9.02 ACVRL1 APLNR BMPR2 HTR2A KCNK3

Biological processes related to Pulmonary Venoocclusive Disease according to GeneCards Suite gene sharing:

(show all 18)
id Name GO ID Score Top Affiliating Genes
1 protein phosphorylation GO:0006468 9.73 ACVRL1 BMPR2 EIF2AK4
2 negative regulation of cell growth GO:0030308 9.58 ACVRL1 BMPR2
3 BMP signaling pathway GO:0030509 9.55 ACVRL1 BMPR2
4 positive regulation of endothelial cell proliferation GO:0001938 9.54 ACVRL1 BMPR2
5 signal transduction by protein phosphorylation GO:0023014 9.52 ACVRL1 BMPR2
6 positive regulation of pathway-restricted SMAD protein phosphorylation GO:0010862 9.51 ACVRL1 BMPR2
7 cellular response to BMP stimulus GO:0071773 9.49 ACVRL1 BMPR2
8 positive regulation of BMP signaling pathway GO:0030513 9.48 ACVRL1 BMPR2
9 blood vessel remodeling GO:0001974 9.46 ACVRL1 BMPR2
10 transmembrane receptor protein serine/threonine kinase signaling pathway GO:0007178 9.43 ACVRL1 BMPR2
11 activin receptor signaling pathway GO:0032924 9.4 ACVRL1 BMPR2
12 negative regulation of DNA biosynthetic process GO:2000279 9.37 ACVRL1 BMPR2
13 lymphangiogenesis GO:0001946 9.32 ACVRL1 BMPR2
14 positive regulation of angiogenesis GO:0045766 9.27 ACVRL1
15 artery development GO:0060840 9.26 ACVRL1 BMPR2
16 retina vasculature development in camera-type eye GO:0061298 9.16 ACVRL1 BMPR2
17 lymphatic endothelial cell differentiation GO:0060836 8.96 ACVRL1 BMPR2
18 venous blood vessel development GO:0060841 8.62 ACVRL1 BMPR2

Molecular functions related to Pulmonary Venoocclusive Disease according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 protein kinase activity GO:0004672 9.5 ACVRL1 BMPR2 EIF2AK4
2 protein serine/threonine kinase activity GO:0004674 9.43 ACVRL1 BMPR2 EIF2AK4
3 signal transducer, downstream of receptor, with serine/threonine kinase activity GO:0004702 9.16 ACVRL1 BMPR2
4 transmembrane receptor protein serine/threonine kinase activity GO:0004675 8.96 ACVRL1 BMPR2
5 BMP receptor activity GO:0098821 8.62 ACVRL1 BMPR2

Sources for Pulmonary Venoocclusive Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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