库欣综合征合并食欲抑制剂暴露患者的肺动脉高压。
Pulmonary arterial hypertension in a patient with Cowden syndrome and anorexigen exposure.
机构信息
Faculté de Médecine, Université Paris-Sud, Kremlin-Bicêtre, France; Service de Pneumologie et Réanimation, Centre de Référence de l'Hypertension Pulmonaire Sévère, Hôpital Antoine Béclère, Assistance Publique-Hôpitaux de Paris, Clamart, France; INSERM U999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson, France.
Laboratoire d'Oncogénétique et Angiogénétique Moléculaire, UMRS 956 INSERM, UPMC Univ Paris 06, Groupe Hospitalier Pitié-Salpétrière, Paris, France.
出版信息
Chest. 2011 Oct;140(4):1066-1068. doi: 10.1378/chest.10-2588.
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.
我们报告了一例肺动脉高压（PAH）病例，该患者患有 Cowden 综合征，其磷酸酶和张力蛋白（PTEN）肿瘤抑制基因发生突变，同时暴露于食欲抑制剂右芬氟拉明。已知食欲抑制剂的暴露是 PAH 的一个危险因素。然而，PTEN 在细胞功能中的作用以及在平滑肌细胞中 Pten 耗竭的小鼠中肺血管重塑和 PAH 的组织病理学迹象表明，PAH 与 Cowden 综合征的关联可能是相关的。在本病例报告中，我们假设 PTEN 突变可能是 PAH 发展的一个易感因素，而食欲抑制剂的暴露可能是一个潜在的触发因素。
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