Please use this identifier to cite or link to this item: http://repositorio.unicamp.br/jspui/handle/REPOSIP/198199
Type: Artigo de periódico
Title: Newer Aspects Of The Pathophysiology Of Sickle Cell Disease Vaso-occlusion.
Author: Conran, Nicola
Franco-Penteado, Carla F
Costa, Fernando F
Abstract: Sickle cell disease is an inherited disorder of hemoglobin (Hb) synthesis, caused by a single nucleotide substitution (GTG>GAG) at the sixth codon of the beta-globin gene, leading to the production of a defective form of Hb, Hb S. When deoxygenated, Hb S polymerizes, damaging the sickle erythrocyte and it is this polymerization that is the primary indispensable event in the molecular pathogenesis of sickle cell disease. Hb S polymerization results in a series of cellular alterations in red cell morphology and function that shorten the red cell life span and leads to vascular occlusion. Sickle cell disease vaso-occlusion is now known to constitute a complex multifactorial process characterized by recurrent vaso-occlusion, ischemia-reperfusion injury, and oxidative stress with consequent vascular endothelial cell activation that induces a chronic inflammatory state in sickle cell disease individual and is propagated by elevated levels of circulating inflammatory cytokines. Activation of the endothelium results in the induction of endothelial adhesion molecule expression that mediates red and white cell adhesion to the vessel wall and the formation of heterocellular aggregates, followed by secondary red cell trapping, all of which contribute to reduced blood flow and eventually obstruction of the micro-circulation. Reduced nitric oxide bioavailability, caused principally by its consumption by cell-free Hb, liberated during intravascular hemolysis, contributes to this process by facilitating vasoconstriction and adhesion molecule activity.
Subject: Anemia, Sickle Cell
Endothelium, Vascular
Humans
Oxidative Stress
Reperfusion Injury
Vascular Diseases
Rights: fechado
Identifier DOI: 10.1080/03630260802625709
Address: http://www.ncbi.nlm.nih.gov/pubmed/19205968
Date Issue: 2009
Appears in Collections:Unicamp - Artigos e Outros Documentos

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