MCID: PLM068
MIFTS: 29

Pulmonary Vein Stenosis malady

Cardiovascular diseases category

Summaries for Pulmonary Vein Stenosis

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42NIH Rare Diseases, 32MalaCards
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NIH Rare Diseases:42 Pulmonary vein stenosis is a very rare and serious condition in which there is a blockage in the blood vessels that bring oxygen-rich blood from the lungs back to the heart. this condition can be isolated to one vein, but often affects multiple veins. stenosis occurs when there is an abnormal thickening and narrowing of the walls of the veins. pulmonary vein stenosis is a progressive condition and may lead to total obstruction to a blood vessel. most commonly, all of the pulmonary veins of one lung are affected, causing pulmonary hypertension and pulmonary arterial hypertension. surgery and catheterization to widen the narrow veins is usually a short-term solution since the obstruction typically recurs. last updated: 9/1/2011

MalaCards: Pulmonary Vein Stenosis, also known as pulmonary veins stenosis, is related to vein disease and hypertension. An important gene associated with Pulmonary Vein Stenosis is F2RL1 (coagulation factor II (thrombin) receptor-like 1), and among its related pathways are Cardiac Progenitor Differentiation and Striated Muscle Contraction. The compounds cycloheximide and glucose have been mentioned in the context of this disorder. Affiliated tissues include heart and lung, and related mouse phenotypes are cardiovascular system and homeostasis/metabolism.

Aliases & Classifications for Pulmonary Vein Stenosis

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42NIH Rare Diseases, 60UMLS
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Classifications:

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Anatomical: Cardiovascular diseases


Aliases & Descriptions:

pulmonary vein stenosis 42 60
pulmonary veins stenosis 42


Related Diseases for Pulmonary Vein Stenosis

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17GeneCards, 18GeneDecks
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Diseases related to Pulmonary Vein Stenosis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 101)
idRelated DiseaseScoreTop Affiliating Genes
1vein disease10.5
2hypertension10.4
3mediastinitis10.3
4pulmonary embolism10.3
5primary pulmonary hypertension10.1
6scimitar syndrome10.1
7tetralogy of fallot10.1
8down syndrome10.1
9thoracic aortic aneurysm10.1
10patent ductus arteriosus10.1
11aortic aneurysm10.1
12coronary stenosis10.1
13esophagitis10.1
14lung cancer10.1
15pancreatitis10.0F2RL1
16cerebral primitive neuroectodermal tumor10.0CD99
17peritonitis10.0PECAM1
18peripheral primitive neuroectodermal tumor10.0CD99
19myoepithelioma10.0ACTC1
20meningioma10.0MKI67
21cervical cancer10.0TYMP
22carney triad10.0PDGFRA
23neurofibromatosis10.0PDGFRA
24osteosarcoma10.0PDGFRA
25myocardial infarction10.0PECAM1
26fasciitis10.0ACTC1
27pulmonary fibrosis10.0F2RL1
28uterine sarcoma10.0DES
29teratoma10.0ACTC1, CD99
30infantile myofibromatosis10.0DES
31hemangioma10.0PECAM1, ACTC1
32lipoma10.0DES
33hepatitis10.0TYMP, CD99
34pyogenic granuloma10.0PECAM1, ACTC1
35pituitary adenoma10.0MKI67
36pancreatic cancer10.0F2RL1, TYMP
37lymphangioma10.0PECAM1, ACTC1
38neurofibroma10.0PDGFRA, ACTC1
39granular cell tumor10.0DES
40mucoepidermoid carcinoma10.0ACTC1, MKI67
41endometrial carcinoma10.0TYMP, MKI67
42oligodendroglioma10.0MKI67, PDGFRA
43adenoid cystic carcinoma10.0MKI67, ACTC1
44malignant peripheral nerve sheath tumor10.0ACTC1, PDGFRA
45ovarian cancer10.0PDGFRA, TYMP
46colorectal cancer10.0PDGFRA, TYMP
47fibromatosis10.0ACTC1, DES
48adenosarcoma10.0DES, ACTC1
49gallbladder cancer10.0MKI67, TYMP
50dermatofibrosarcoma protuberans10.0ACTC1, DES

Graphical network of the top 20 diseases related to Pulmonary Vein Stenosis:



Diseases related to pulmonary vein stenosis

Clinical Features for Pulmonary Vein Stenosis

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Drugs & Therapeutics for Pulmonary Vein Stenosis

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Pulmonary Vein Stenosis

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Anatomical Context for Pulmonary Vein Stenosis

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32MalaCards
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MalaCards organs/tissues related to Pulmonary Vein Stenosis:

32
Heart, Lung

Animal Models for Pulmonary Vein Stenosis or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Pulmonary Vein Stenosis:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053858.2PECAM1, ACTC1, F2RL1, DES, PDGFRA
2MP:00053767.4PECAM1, PDGFRA, DES, TYMP, F2RL1, ACTC1

Publications for Pulmonary Vein Stenosis

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Genetic Variations for Pulmonary Vein Stenosis

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Expression for genes affiliated with Pulmonary Vein Stenosis

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Pulmonary Vein Stenosis

Search GEO for disease gene expression data for Pulmonary Vein Stenosis.

Pathways for genes affiliated with Pulmonary Vein Stenosis

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Sources:
37NCBI BioSystems Database, 53Reactome, 4Cell Signaling Technology
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Pathways related to Pulmonary Vein Stenosis according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.6ACTC1, PDGFRA
2
Hide members
9.5ACTC1, DES
39.1DES, F2RL1, ACTC1
48.7PECAM1, F2RL1, TYMP, PDGFRA

Compounds for genes affiliated with Pulmonary Vein Stenosis

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Sources:
44Novoseek, 24HMDB, 11DrugBank, 49PharmGKB, 59Tocris Bioscience, 28IUPHAR
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Compounds related to Pulmonary Vein Stenosis according to GeneCards/GeneDecks:

(show all 39)
idCompoundScoreTop Affiliating Genes
1cycloheximide4410.4TYMP
2glucose4410.0TYMP, ACTC1
3orcein449.9ACTC1, DES
4s 100449.8DES, ACTC1
5reticulin449.8ACTC1, DES
6mucicarmine449.8DES, ACTC1
7deoxyuridine44 2410.8TYMP, MKI67
8vinorelbine44 1110.8TYMP, MKI67
9b 723449.7ACTC1, DES
10p00001449.7TYMP, MKI67
11percoll449.7DES, PECAM1
12methanol44 2410.6ACTC1, MKI67
13prostacyclin449.6PECAM1, F2RL1, TYMP
14p003449.5PECAM1, TYMP, MKI67
15streptavidin449.5MKI67, DES
16matrigel449.5PECAM1, ACTC1, TYMP
17p005449.5PECAM1, TYMP, MKI67
18biotin44 11 2411.3ACTC1, MKI67, DES
19imatinib44 49 1111.3ACTC1, DES, PDGFRA
20folate449.3TYMP, MKI67, PDGFRA
21fibrinogen449.2DES, F2RL1, PECAM1
22actinomycin d449.0DES, TYMP, F2RL1, PECAM1
23hematoxylin449.0DES, MKI67, ACTC1, PECAM1
24estradiol44 11 2410.9TYMP, MKI67, DES
25p002448.9DES, MKI67, TYMP, PECAM1
26progesterone44 59 28 11 2412.8DES, MKI67, TYMP, ACTC1
27doxorubicin44 49 1110.8DES, MKI67, TYMP
28glycogen44 249.7ACTC1, MKI67, DES
29cysteine448.6PECAM1, F2RL1, TYMP, DES
30formaldehyde44 249.5DES, MKI67, TYMP, ACTC1, PECAM1
31nitric oxide44 11 2410.5DES, MKI67, TYMP, F2RL1, PECAM1
32retinoic acid44 249.4PDGFRA, DES, MKI67, ACTC1, PECAM1
33serine448.4DES, MKI67, TYMP, F2RL1, PECAM1
34oxygen44 249.1DES, MKI67, TYMP, F2RL1, ACTC1, PECAM1
35calcium44 49 11 2411.0PDGFRA, DES, MKI67, F2RL1, ACTC1, PECAM1
36thymidine44 249.0PDGFRA, DES, MKI67, TYMP, F2RL1, PECAM1
37tyrosine447.6PDGFRA, DES, MKI67, TYMP, F2RL1, ACTC1
38paraffin446.8PECAM1, PDGFRA, DES, MKI67, TYMP, CD99
39vegf446.8PDGFRA, DES, MKI67, TYMP, CD99, F2RL1

GO Terms for genes affiliated with Pulmonary Vein Stenosis

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16Gene Ontology
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Biological processes related to Pulmonary Vein Stenosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of phosphatidylinositol 3-kinase cascadeGO:0140689.6F2RL1, PDGFRA
2cardiac myofibril assemblyGO:0550039.5ACTC1, PDGFRA
3muscle filament slidingGO:0300499.2ACTC1, DES

Molecular functions related to Pulmonary Vein Stenosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1platelet-derived growth factor receptor bindingGO:0051619.5TYMP, PDGFRA

Products for genes affiliated with Pulmonary Vein Stenosis

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Sources for Pulmonary Vein Stenosis

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet