Document Detail


Pulmonary arterial hypertension in a patient with cowden syndrome and anorexigen exposure.
MedLine Citation:
PMID:  21972386     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
We report a case of pulmonary arterial hypertension (PAH) occurring in a patient with Cowden syndrome with a mutation in the phosphatase and tensin (PTEN) tumor suppressor gene, in the context of exposure to the appetite suppressant dexfenfluramine. Anorexigen exposure is known to be a risk factor for PAH. However, the role of PTEN in cell function and the development of pulmonary vascular remodeling and histopathologic signs of PAH in mice with a Pten depletion in smooth muscle cells suggest that the association of PAH and Cowden syndrome may be relevant. In this case report, we hypothesize that PTEN mutations may be a predisposing factor for the development of PAH, with anorexigen exposure as a potential trigger.
Authors:
Delphine Natali; Barbara Girerd; David Montani; Florent Soubrier; Gérald Simonneau; Marc Humbert; Olivier Sitbon
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Chest     Volume:  140     ISSN:  1931-3543     ISO Abbreviation:  Chest     Publication Date:  2011 Oct 
Date Detail:
Created Date:  2011-10-05     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0231335     Medline TA:  Chest     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1066-8     Citation Subset:  AIM; IM    
Affiliation:
Service de Pneumologie et Réanimation, Hôpital Antoine Béclère, 157 rue de la Porte de Trivaux, 92140 Clamart, France. olivier.sitbon@abc.aphp.fr.
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