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Clinics in chest medicine · Sep 2016
ReviewPulmonary Capillary Hemangiomatosis and Pulmonary Veno-occlusive Disease.
- Neal F Chaisson, Mark W Dodson, and Charles Gregory Elliott.
- Respiratory Institute, Cleveland Clinic, Lerner College of Medicine of Case Western Reserve University, 9500 Euclid Avenue, A-90, Cleveland, OH 44195, USA.
- Clin. Chest Med. 2016 Sep 1; 37 (3): 523-34.
AbstractThis 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.Copyright © 2016 Elsevier Inc. All rights reserved.
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