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

被引：4

作者：

Natali, Delphine ^{[1
,2
,3
]}

Girerd, Barbara ^{[1
,2
,3
]}

Montani, David ^{[1
,2
,3
]}

Soubrier, Florent ^{[4
]}

Simonneau, Gerald ^{[1
,2
,3
]}

Humbert, Marc ^{[1
,2
,3
]}

Sitbon, Olivier ^{[1
,2
,3
]}

机构：

[1] Hop Antoine Beclere, AP HP, Serv Pneumol & Reanimat, Ctr Reference Hypertens Pulm Severe, F-92140 Clamart, France

[2] Univ Paris 11, Fac Med, Le Kremlin Bicetre, France

[3] Ctr Chirurg Marie Lannelongue, INSERM, U999, Le Plessis Robinson, France

[4] Univ Paris 06, INSERM, UPMC, Grp Hosp Pitie Salpetriere,UMRS 956,Lab Oncogenet, Paris, France

来源：

CHEST | 2011年 / 140卷 / 04期

关键词：

PTEN;

D O I：

10.1378/chest.10-2588

中图分类号：

R4 [临床医学];

学科分类号：

1002 ; 100602 ;

摘要：

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. CHEST 2011; 140(4):1066-1068

引用

页码：1066 / 1068

页数：3

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