Sickle Cell Disease: A New Vision for an Old Problem

Darío Acuña-Castroviejo, MD, PhD and Iryna Rusanova, PhD (Editors)
Institute of Biotechnology, Biomedical Research Center, Health Sciences Technology Park, University of Granada, Granada, Spain

Series: Recent Advances in Hematology Research
BISAC: MED038000

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Volume 10

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Special issue: Resilience in breaking the cycle of children’s environmental health disparities
Edited by I Leslie Rubin, Robert J Geller, Abby Mutic, Benjamin A Gitterman, Nathan Mutic, Wayne Garfinkel, Claire D Coles, Kurt Martinuzzi, and Joav Merrick

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Sickle cell hemoglobin (HbS) is the result of a single nucleotide change (GAG ¨ GTG) in the ƒÀ-globin gene, where valine replaces glutamic acid at the sixth amino acid position in the ƒÀ-globin chain. Sickle cell disease is a growing global health problem. The World Health Organization has estimated that 7% of the world population has the mutation and 300,000-400,000 affected children are born every year.

The disease progresses towards a severe chronic hemolytic anemia, and it shows a heterogeneous clinical course, related with different genetic factors. Despite the fact that all subjects with sickle cell disease (SCD) have the same single base pair mutation in the DNA, we further confirmed here that the severity of the clinical and hematological manifestations is extremely variable. Increasing evidence has indicated a role of oxidative stress in the vascular pathophysiology of SCD. The vascular endothelium is central to disease pathogenesis because it displays adhesion molecules for blood cells, balances procoagulant and anticoagulant properties of the vessel wall and regulates vascular homeostasis by synthesizing vasoconstricting and vasodilating substances.

In addition, recent studies support the existence of a hyperoxidative status in SCD patients that may account, at least in part, for the clinical manifestations of these patients. Moreover, SCD patients with mild clinical outcomes were associated with low oxidative status, whereas high oxidative stress was related to severe phenotypes. Thus, the use of oxidative stress biomarkers may be important in the evaluation of the clinical condition of SCD patients, whereas the use of therapies to improve their redox status and increase NO bioavailability would be beneficial to reduce the severity of sickle disease.

The global burden of SCD is now significantly increased and, thus, it is currently a public health problem around the world. This disease has passed from being a problem of the developing countries to affect many people in developed countries. This book summarizes the current epidemiology status and the latest discoveries in the pathophysiology of SCD, and the potential therapies that may improve the clinical course of this disease. (Imprint: Nova Biomedical )

Preface

Chapter 1. Epidemiology of Sickle Cell Disease (SCD)
(Iryna Rusanova, Departamento de Biomédica, Universidad Especializada de las Américas, Panamá, República de Panamá, and others)

Chapter 2. The Phenotype Diversity and Hematological Status in Sickle Cell Anemia
(Iryna Rusanova, F. Javier Perea-Díaz and Dario Acuña-Castroviejo, Departamento de Biomédica, Universidad Especializada de las Américas,
Panamá, República de Panamá, and others)

Chapter 3. Pathophysiological Consequences of Hemolysis in Sickle Cell Disease
(Rajiv Lochan Tiwari, Gowtham Kumar Annarapu and Prasenjit Guchhait, Regional Centre for Biotechnology, Delhi NCR, India, and others)

Chapter 4. Genotypes of Sickle Cell Disease
(F. Javier Perea-Díaz and Bertha Ibarra-Cortés, División de Genética, Centro de Investigación Biomédica de Occidente, Centro Medico de Occidente, Instituto Mexicano del Seguro Social, Guadalajara, Jalisco, México, and others)

Chapter 5. Vasculopathy, Chronic Oxidative Stress and Endothelial Dysfunction in SCD
(Clara Y. Lo and Claudia R. Morris, Department of Pediatrics, Division of Hematology-Oncology, Lucile Packard Childrenfs Hospital, Stanford University School of Medicine, Palo Alto, CA, USA, and others)

Chapter 6. Biology of Nitric Oxide Relevant to Sickle Cell Disease
(Iryna Rusanova and Dario Acuña-Castroviejo, Departamento de Biomédica, Universidad Especializada de las Américas, Panamá, República de Panamá, and others)

Chapter 7. Therapy of Sickle Cell Disease
(S. Eridani, G. Graziadei, A. Marcon and M.D. Cappellini, Department of Medico-Surgical Physiopathology, University of Milano, and others)

Index

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