| Pulmonary veno-occlusive disease: advances in clinical management and treatments. | |
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MedLine Citation:
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PMID: 21510732 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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Pulmonary veno-occlusive disease (PVOD) is a rare disorder that can be misdiagnosed as idiopathic pulmonary arterial hypertension (PAH) and accounts for 5-10% of cases initially considered as idiopathic PAH. PVOD and idiopathic PAH share a similar clinical presentation, genetic background and hemodynamic profile. A definite diagnosis of PVOD necessitates a surgical biopsy, but since it represents a high-risk procedure in these patients, it is contraindicated. Therefore, a noninvasive diagnostic approach using chest high-resolution computed tomography, arterial blood gas analysis, pulmonary function tests and bronchoalveolar lavage is helpful to detect PVOD. PVOD is characterized by a poor prognosis and the possibility of developing severe pulmonary edema with specific PAH therapy. Lung transplantation remains the treatment of choice. |
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Authors:
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Alice Huertas; Barbara Girerd; Peter Dorfmuller; Dermot O'Callaghan; Marc Humbert; David Montani |
Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Expert review of respiratory medicine Volume: 5 ISSN: 1747-6356 ISO Abbreviation: Expert Rev Respir Med Publication Date: 2011 Apr |
Date Detail:
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Created Date: 2011-04-22 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101278196 Medline TA: Expert Rev Respir Med Country: England |
Other Details:
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Languages: eng Pagination: 217-31 Citation Subset: IM |
Affiliation:
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Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre, F-94276, France. david.montani@abc.aphp.fr. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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