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.
2012-05-01
Eng.
Journal of the American Society of Nephrology : JASN
Division of Nephrology and Hypertension, Internal Medicine, Department of Physiology and Biomedical Engineering, Mayo Clinic, Rochester, MN 55905, USA. nath.karl [at] mayo.edu
J Am Soc Nephrol. 2012 May;23(5):781-4
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