Unique wreath-like smooth muscle proliferation of the pulmonary vasculature in pulmonary veno-occlusive disease versus pulmonary arterial hypertension

Background/purpose: Pulmonary veno-occlusive disease (PVOD) is a rare but fatal cause of pulmonary hypertension reported to be linked to mutations of eukaryotic initiation factor 2 alpha kinase 4 (EIF2AK4), also known as general control nonderepressible 2 kinase (GCN2). PVOD is difficult to diagnose...

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Bibliographic Details
Main Authors: Ying-Ju Lai, Po-Ru Chen, Yen-Lin Huang, Hsao-Hsun Hsu
Format: Article
Language:English
Published: Elsevier 2020-01-01
Series:Journal of the Formosan Medical Association
Online Access:http://www.sciencedirect.com/science/article/pii/S0929664619302803