| Literature DB >> 27514598 |
Neal F Chaisson1, Mark W Dodson2, Charles Gregory Elliott3.
Abstract
This article provides an overview of pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH), two disorders that challenge clinicians, radiologists, and pathologists because they often mimic pulmonary arterial hypertension (PAH). The article reviews the features that differentiate PVOD and PCH from PAH. The article also describes the overlap of PVOD and PCH, highlighted by recent reports of families diagnosed with PVOD or PCH caused by EIF2AK4 mutations. In addition, the article outlines current approaches to the diagnosis and treatment of PVOD and PCH.Entities:
Keywords: Genetics; Pulmonary capillary hemangiomatosis; Pulmonary disorder; Pulmonary hypertension; Pulmonary veno-occlusive disease
Mesh:
Substances:
Year: 2016 PMID: 27514598 DOI: 10.1016/j.ccm.2016.04.014
Source DB: PubMed Journal: Clin Chest Med ISSN: 0272-5231 Impact factor: 2.878