Pulmonary Arterial Hypertension in a Patient with Cowden Syndrome and the PTEN Mutation:

Pulmonary Circulation

DucHa¹ 

关键词: pulmonary hypertension;    PTEN mutation;    Cowden syndrome;   

DOI  :  10.1086/678552

学科分类：医学（综合）

来源: Sage Journals

【 摘 要 】

The pathogenesis of pulmonary arterial hypertension (PAH) exhibits many neoplastic-like features. Cowden syndrome is a difficult-to-recognize heritable cancer syndrome caused by a germline mutation in the phosphatase-and-tensin homolog deleted on the chromosome 10 (PTEN) gene. PTEN regulation has been implicated in cancer development and, more recently, PAH pathogenesis. Here we report a case of PAH in a patient with Cowden syndrome and the response to pulmonary vasodilators.

【 授权许可】

CC BY