0
Selected Reports |

Pulmonary Arterial Hypertension in a Patient With Cowden Syndrome and Anorexigen ExposurePulmonary Arterial Hypertension in Cowden Syndrome

Delphine Natali; Barbara Girerd, PhD; David Montani, MD, PhD; Florent Soubrier, MD, PhD; Gérald Simonneau, MD; Marc Humbert, MD, PhD; Olivier Sitbon, MD, PhD
Author and Funding Information

From the Faculté de Médecine (Drs Natali, Girerd, Montani, Simonneau, Humbert, and Sitbon), Université Paris-Sud, Kremlin-Bicêtre; the Service de Pneumologie et Réanimation (Drs Natali, Girerd, Montani, Simonneau, Humbert, and Sitbon), Centre de Référence de l’Hypertension Pulmonaire Sévère, Hôpital Antoine Béclère, Assistance Publique-Hôpitaux de Paris, Clamart; INSERM U999 (Drs Natali, Girerd, Montani, Simonneau, Humbert, and Sitbon), Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson; and Laboratoire d’Oncogénétique et Angiogénétique Moléculaire (Dr Soubrier), UMRS 956 INSERM, UPMC Univ Paris 06, Groupe Hospitalier Pitié-Salpétrière, Paris, France.

Correspondence to: Olivier Sitbon, MD, PhD, Service de Pneumologie et Réanimation, Hôpital Antoine Béclère, 157 rue de la Porte de Trivaux, 92140 Clamart, France; e-mail: olivier.sitbon@abc.aphp.fr

Drs Natali and Girerd contributed equally to this article.


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).


© 2011 American College of Chest Physicians


Chest. 2011;140(4):1066-1068. doi:10.1378/chest.10-2588
Text Size: A A A
Published online

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.

Figures in this Article

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Figures

Tables

References

NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
CHEST Journal Articles
PubMed Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543