Department of Medicine, Boston University School of Medicine, Boston, Massachusetts 02118, USA.
J Cell Physiol. 2010 Sep;224(3):620-5. doi: 10.1002/jcp.22195.
Endothelial dysfunction and impaired nitric oxide bioavailability have been implicated in the pathogenesis of sickle cell anemia. Nitric oxide is a diatomic gas with a role in vascular homeostasis. Hemoglobin polymerization resulting from the HbS mutation produces erythrocyte deformation and hemolysis. Free hemoglobin, released into plasma by hemolysis scavenges on nitric oxide, and leads to reduced nitric oxide bioavailability. Pulmonary hypertension is a known consequence of sickle cell anemia. It occurs in 30-40% of patients with sickle cell anemia, and is associated with increased mortality. Several studies have implicated intravascular hemolysis, and impaired nitric oxide bioavailability in the pathogenesis of pulmonary hypertension. In this review, we summarize the mechanisms of altered nitric oxide bioavailability in sickle cell anemia and its possible role in the pathogenesis of pulmonary hypertension.
内皮功能障碍和一氧化氮生物利用度降低与镰状细胞贫血的发病机制有关。一氧化氮是一种具有血管稳态作用的双原子气体。由 HbS 突变引起的血红蛋白聚合导致红细胞变形和溶血。血红蛋白通过溶血释放到血浆中，清除一氧化氮，导致一氧化氮生物利用度降低。肺动脉高压是镰状细胞贫血的已知后果。它发生在 30-40%的镰状细胞贫血患者中，并与死亡率增加有关。几项研究表明，血管内溶血和一氧化氮生物利用度降低与肺动脉高压的发病机制有关。在这篇综述中，我们总结了镰状细胞贫血中一氧化氮生物利用度改变的机制及其在肺动脉高压发病机制中的可能作用。
