Titlebook: Sickle Cell Anemia; From Basic Science t Fernando Ferreira Costa,Nicola Conran Book 2016 Springer International Publishing Switzerland 2016

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Priapism in Sickle Cell Disease: New Aspects of Pathophysiology, male sickle cell disease (SCD) patients. It manifests either as an acute, severe event, or as recurrent, stuttering priapism, with very limited therapeutic options. Untreated priapism can cause irreversible erectile dysfunction and surgical treatment remains the only option for severe cases. The me

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Clinical Manifestations and Treatment of Adult Sickle Cell Disease,are known to affect virtually all organs and systems in the human body. Chronic hemolytic anemia and a systemic inflammatory state are the basic pathophysiological mechanisms that underlie the occurrence of both acute vaso-occlusive events (painful episodes, acute chest syndrome, priapism, stroke, e

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Future Perspectives for the Treatment of Sickle Cell Anemia,agents. These novel approaches target the diverse pathophysiology associated with SCD (e.g., increased blood cell adhesion, activated coagulation system, hyperinflammation, endothelial dysfunction). Potential therapies can be classified according to the “level” of the target intervention and related

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Book 2016and use this information as a basis to discuss its manifestations in childhood and adulthood. Current therapies and prospects for the development of new approaches for the management of the disease are also covered.

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Hemoglobin: Structure, Synthesis and Oxygen Transport,synthesis and globin gene expression/regulation, besides functional aspects of the hemoglobin molecule. The chapter also includes models that predict the mechanisms of Hb-O. ligation, mediated by the presence of allosteric effectors, such as H./CO., Cl. and organic phosphates, such as 2,3-biphosphoglycerate (2,3-BPG, from erythrocyte metabolism).

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