INTERNATIONAL CONFERENCE ON PEDIATRICS AND HEALTHCARE
MAY 22-23, 2023 | DUBAI, UAE
Theme: Promoting excellence by examining the field of Pediatrics and Healthcare
Ariadne Carvalho Godinho
UNINASSAU Barreiras, BrazilTitle: Splenic sequestration in sickle cell disease
Abstract
Splenic Sequestration in Sickle Cell Disease: Sickle Cell Disease (SCD) is an inherited group of disorders characterized by the presence of Hemoglobin S (Hb S), either by homozygosity for the sickle cell mutation (Hb SS) or compound heterozygosity with another variant of beta globin ( eg, sickle-cell beta thalassemia, Hb SC disease). Sickle cell trait is a benign condition of the carrier. Hemoglobin S (HbS) results from the replacement of valine by glutamic acid as the seventh amino acid of the beta-globin chain, which produces a hemoglobin tetramer (alpha2/betaS2) that is poorly soluble when deoxygenated. The polymerization of deoxy HbS is essential for vaso-occlusive phenomena. The polymer takes the form of an elongated, rope-like fiber that often lines up with other fibers, resulting in the disruption of red blood cells enclosed in the classic crescent or sickle shape and a marked decrease in red blood cell deformability. Vaso-occlusive phenomena and hemolytic anemia are the clinical features of Sickle Cell Disease (SCD). Vaso-occlusion results in recurrent painful episodes and a variety of serious organ system complications that can lead to lifelong disabilities and even death. Splenic sequestration is an acute sensation of the greatest severity, being the cause of great morbidity and mortality in patients with sickle cell disease. It sets in suddenly, with a progressive drop in the blood values of hemoglobin and, if it does not remain, it evolves to hypovolemic shock. It is potentially fatal if not treated quickly. The objective of this presentation is to advise on the importance of early diagnosis of splenic sequestration and adequate treatment.
Biography
Ariadne Carvalho Godinho is a pediatric hematologist, coordinator of the Sickle Cell Disease Program at the Leonidia Ayres Health Center in Barreiras BA. she is a coordinator of the pediatric emergency Service at the Eurico Dutra Municipal Hospital in Barreiras BA.She is a professor of hematology and pediatrics at UNINASSAU in Barreiras, Brazil.
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