Division of Nephrology and Hypertension, Internal Medicine, Department of Physiology and Biomedical Engineering, Mayo Clinic, Rochester, MN 55905, USA.
J Am Soc Nephrol. 2012 May;23(5):781-4. doi: 10.1681/ASN.2011101019. Epub 2012 Mar 22.
Recent developments in sickle cell disease include the concept of a vasculopathic state and the classification of sickle cell disease into a hemolysis-endothelial dysfunction phenotype or a viscosity-vasoocclusion phenotype. The hemolysis-endothelial dysfunction phenotype largely reflects deficiency of or resistance to nitric oxide. In addition to discussing these areas, we suggest that the hemolysis-endothelial dysfunction phenotype also reflects the instability of sickle hemoglobin, the release of heme, and the induction of heme oxygenase-1. From these perspectives the renal complications of sickle cell disease are discussed and classified.
镰状细胞病的最新研究进展包括血管病变状态的概念和镰状细胞病分为溶血-内皮功能障碍表型或粘度-血管阻塞表型。溶血-内皮功能障碍表型在很大程度上反映了一氧化氮的缺乏或抵抗。除了讨论这些领域外，我们还认为溶血-内皮功能障碍表型也反映了镰状血红蛋白的不稳定性、血红素的释放以及血红素加氧酶-1 的诱导。从这些角度讨论并分类了镰状细胞病的肾脏并发症。
