Pulmonary Capillary Hemangiomatosis and Pulmonary Veno-occlusive Disease.

Chaisson, Neal F; Dodson, Mark W; Elliott, Charles Gregory

Chaisson, Neal F; Dodson, Mark W; Elliott, Charles Gregory.

Afiliación

Chaisson NF; Respiratory Institute, Cleveland Clinic, Lerner College of Medicine of Case Western Reserve University, 9500 Euclid Avenue, A-90, Cleveland, OH 44195, USA.
Dodson MW; Pulmonary Division, University of Utah, 24 North 1900 East Wintrobe Building, Room 701, Salt Lake City, UT 84132, USA; Department of Medicine, Intermountain Medical Center, 5121 South Cottonwood Street, Suite 307, Murray, UT 84107, USA.
Elliott CG; Pulmonary Division, University of Utah, 24 North 1900 East Wintrobe Building, Room 701, Salt Lake City, UT 84132, USA; Department of Medicine, Intermountain Medical Center, 5121 South Cottonwood Street, Suite 307, Murray, UT 84107, USA. Electronic address: greg.elliott@imail.org.

Clin Chest Med ; 37(3): 523-34, 2016 09.

Article en En | MEDLINE | ID: mdl-27514598

RESUMEN

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.

Asunto(s)

Hemangioma Capilar/diagnóstico; Hipertensión Pulmonar/diagnóstico; Neoplasias Pulmonares/diagnóstico; Neoplasias Primarias Múltiples/diagnóstico; Enfermedad Veno-Oclusiva Pulmonar/diagnóstico; Diagnóstico Diferencial; Hemangioma Capilar/genética; Hemangioma Capilar/patología; Hemangioma Capilar/fisiopatología; Humanos; Hipertensión Pulmonar/fisiopatología; Neoplasias Pulmonares/genética; Neoplasias Pulmonares/patología; Neoplasias Pulmonares/fisiopatología; Mutación; Neoplasias Primarias Múltiples/genética; Neoplasias Primarias Múltiples/patología; Neoplasias Primarias Múltiples/fisiopatología; Proteínas Serina-Treonina Quinasas/genética; Enfermedad Veno-Oclusiva Pulmonar/genética; Enfermedad Veno-Oclusiva Pulmonar/patología; Enfermedad Veno-Oclusiva Pulmonar/fisiopatología

Palabras clave

Genetics; Pulmonary capillary hemangiomatosis; Pulmonary disorder; Pulmonary hypertension; Pulmonary veno-occlusive disease

Texto completo

Añadir a Mi BVS

Imprimir

XML

PubMed Links

Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedad Veno-Oclusiva Pulmonar / Hemangioma Capilar / Hipertensión Pulmonar / Neoplasias Pulmonares / Neoplasias Primarias Múltiples Tipo de estudio: Diagnostic_studies Límite: Humans Idioma: En Revista: Clin Chest Med Año: 2016 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Estados Unidos

Texto completo

Añadir a Mi BVS

Imprimir

XML

PubMed Links

Buscar en Google