期刊
CLINICS IN CHEST MEDICINE
卷 22, 期 3, 页码 405-+出版社
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/S0272-5231(05)70280-X
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资金
- NHLBI NIH HHS [1RO1 HL60913-01, R01 HL43180-01A1, 1RO1 HL60195-01] Funding Source: Medline
Mild to moderate pulmonary hypertension (PH) is characterized histologically by medial smooth muscle cell hypertrophy. Sever PH (primary and secondary) is characterized by the presence of intraluminal clusters of endothelial cells in pulmonary arteries. We review the evolving concepts of pulmonary endothelial cell dysfunction in severe PH. Our most recent findings indicate that mutations underlie the endothelial cell proliferation seen in PPH, suggesting that endothelial progenitor-like cells may be activated to grow and eventually disrupt pulmonary artery flow.
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