Preferred Name | pulmonary arterial hypertension | |
Synonyms |
PAH with overt features of venous/capillaries involvement PVOD/PCH idiopathic pulmonary hypertension PPH Ayerza's Syndrome Hypertension, Pulmonary pulmonary hypertension Pulmonary hypertension, NOS pulmonary hypertension, primary, 1 Syndrome, Ayerza-Arrilaga Ayerzas Syndrome Syndrome, Ayerza HYPERTENSION PULM pulmonary arterial hypertension PHT - Pulmonary hypertension PULM HYPERTENSION Ayerza-Arrilaga Syndrome pulmonary hypertension (disorder) Ayerza Arrilaga Syndrome Ayerza Syndrome PAH PPH1 |
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Definitions |
A syndrome with pathological changes in the pulmonary arteries and impaired PULMONARY CIRCULATION that can be the result of PULMONARY HYPERTENSION. Ayerza syndrome is characterized by slowly developing ASTHMA; BRONCHITIS; DYSPNEA; and CYANOSIS in association with POLYCYTHEMIA. Pulmonary arterial hypertension (PAH) is a group of diseases characterized by mean pulmonary artery pressure >20 mmHg and elevated pulmonary arterial resistance leading to right heart failure. PAH is progressive and potentially fatal. PAH may be idiopathic and/ or familial, have overt features of venous/capillary involvement (pulmonary veno-occlusive disease, PVOD/pulmonary capillary hemangiomatosis, PCH), induced by drug or toxin (drug-or toxin-induced PAH), or associated with other diseases like congenital heart disease, connective tissue disease, HIV, schistosomiasis, portal hypertension (PAH associated with other disease). Increased VASCULAR RESISTANCE in the PULMONARY CIRCULATION, usually secondary to HEART DISEASES or LUNG DISEASES. |
|
ID |
http://www.ebi.ac.uk/efo/EFO_0001361 |
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curated_content_resource |
https://search.clinicalgenome.org/kb/conditions/MONDO:0015924 |
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database_cross_reference |
NANDO:2200298 MEDGEN:425404 MedDRA:10064911 NANDO:1200425 NANDO:2100103 icd11.foundation:1931148955 SCTID:11399002 Orphanet:182090 SNOMEDCT:70995007 MONDO:0015924 MESH:D000081029 OMIM:615371 UMLS:C2973725 MeSH:D006976 DOID:6432 GARD:7501 NCIt:C3120 NORD:1634 |
|
definition |
A syndrome with pathological changes in the pulmonary arteries and impaired PULMONARY CIRCULATION that can be the result of PULMONARY HYPERTENSION. Ayerza syndrome is characterized by slowly developing ASTHMA; BRONCHITIS; DYSPNEA; and CYANOSIS in association with POLYCYTHEMIA. Pulmonary arterial hypertension (PAH) is a group of diseases characterized by mean pulmonary artery pressure >20 mmHg and elevated pulmonary arterial resistance leading to right heart failure. PAH is progressive and potentially fatal. PAH may be idiopathic and/ or familial, have overt features of venous/capillary involvement (pulmonary veno-occlusive disease, PVOD/pulmonary capillary hemangiomatosis, PCH), induced by drug or toxin (drug-or toxin-induced PAH), or associated with other diseases like congenital heart disease, connective tissue disease, HIV, schistosomiasis, portal hypertension (PAH associated with other disease). Increased VASCULAR RESISTANCE in the PULMONARY CIRCULATION, usually secondary to HEART DISEASES or LUNG DISEASES. |
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gwas_trait |
true |
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has_exact_synonym |
Ayerza's Syndrome Hypertension, Pulmonary pulmonary hypertension Pulmonary hypertension, NOS pulmonary hypertension, primary, 1 Syndrome, Ayerza-Arrilaga Ayerzas Syndrome Syndrome, Ayerza HYPERTENSION PULM pulmonary arterial hypertension PHT - Pulmonary hypertension PULM HYPERTENSION Ayerza-Arrilaga Syndrome pulmonary hypertension (disorder) Ayerza Arrilaga Syndrome Ayerza Syndrome PAH PPH1 |
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has_narrow_synonym |
PAH with overt features of venous/capillaries involvement PVOD/PCH |
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has_related_synonym |
idiopathic pulmonary hypertension PPH |
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IAO_0000233 | ||
id |
EFO:0001361 |
|
in_subset |
http://purl.obolibrary.org/obo/mondo/mondo-base#rare http://purl.obolibrary.org/obo/mondo/mondo-base#clingen http://purl.obolibrary.org/obo/mondo/mondo-base#otar http://purl.obolibrary.org/obo/mondo/mondo-base#gard_rare http://purl.obolibrary.org/obo/mondo/mondo-base#ordo_group_of_disorders http://purl.obolibrary.org/obo/mondo/mondo-base#disease_grouping |
|
label |
pulmonary arterial hypertension |
|
notation |
EFO:0001361 |
|
preferred label |
pulmonary arterial hypertension |
|
prefLabel |
pulmonary arterial hypertension |
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skos_closeMatch | ||
skos_exactMatch |
http://identifiers.org/mesh/D000081029 http://purl.obolibrary.org/obo/mondo/sources/icd11foundation/1931148955 http://identifiers.org/snomedct/11399002 http://purl.obolibrary.org/obo/Orphanet_182090 http://identifiers.org/medgen/425404 |
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term editor |
James Malone |
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subClassOf |