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obophenotype / human-phenotype-ontology

Ontology for the description of human clinical features
http://obophenotype.github.io/human-phenotype-ontology/
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Pulmonary artery adventitial fibrosis #6522

Closed eswietlik closed 3 years ago

eswietlik commented 3 years ago

Preferred term label: Pulmonary artery adventitial fibrosis

Synonyms

Definition (free text, please give PubMed ID) Accumulation of the fibroblasts in the adventitia PMID: 23737168

Parent term (use hpo.jax.org/app) Abnormal pulmonary artery morphology HP:0030966

Diseases characterized by this term? (e.g. Orphanet or OMIM number)

Idiopathic pulmonary arterial hypertension ORPHA:275766 Hereditary pulmonary arterial hypertension ORPHA:275777 Pulmonary arterial hypertension associated with congenital heart disease

Your nano-attribution (ORCID) https://orcid.org/0000-0002-4095-8489

pnrobinson commented 3 years ago

I think a better definition is

Increased amount of collagen, proteoglycans, and other extracellaulr matrix proteins resulting in scarring and thickening in the adventia of the pulmonary artery. (Question -- do we actually mean the pulmonary arterial tree rather than just the pulmonary artery sensu strictu? Should we cite this article for additional context: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6039811/).

eswietlik commented 3 years ago

Agree, sorry, was rushing through it and did not finish the definition. Yes, the adventitial fibrosis affects a significant proportion of pulmonary arterial tree (rather than just main, R and L PAs) and plays a significant role in disease pathogenesis PMID: 17008597; PMID: 23737168

pnrobinson commented 3 years ago

adding term, thanks!