title: Vasculature and kidney complications in sickle cell disease.
abstract: 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.
authors.affiliation: Division of Nephrology and Hypertension, Internal Medicine, Department of Physiology and Biomedical Engineering, Mayo Clinic, Rochester, MN 55905, USA. nath.karl@mayo.edu
authors.forename: Karl A
authors.initials: KA
authors.lastname: Nath
authors.forename: Zvonimir S
authors.initials: ZS
authors.lastname: Katusic
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications* / Q000503:physiopathology; D000818:Animals; D042783:Endothelial Cells / Q000502:physiology; D051547:Heme Oxygenase-1 / Q000502:physiology; D006461:Hemolysis; D006801:Humans; D007674:Kidney Diseases / Q000209:etiology*; D014652:Vascular Diseases / Q000209:etiology*
chemical_list: D051547:Heme Oxygenase-1