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The root cause of sickle cell disease is a single β-globin gene mutation coding for the sickle β-hemoglobin chain. Sickle hemoglobin tetramers polymerize when deoxygenated, damaging the sickle erythrocyte.
Martin H. Steinberg
doaj +1 more source
Prevalence of Haemoglobine s in Araraquara-SP Population.
The Sickle Cell Anemia is a genetic hemoglobionopathy, characterized by an alteration in the hemoglobin molecule struture (HbA1), called hemoglobin S (HbS), which causes a distortion in the erythocytes structure, changing from spherical shape to sickle ...
Bruno Rocha de Jesus +4 more
doaj +1 more source
Sickle cell disease is a hereditary disease that predominantly affects black people. It is very widespread in sub-Saharan Africa, particularly at the Lehmann “sickle belt” level, where the prevalence of the hemoglobin S involves at least 10% of the ...
Jeannette Bassimbié Kakou Danho +4 more
doaj +1 more source
CRISPR-Cas9 Editing of the HBG1/HBG2 Promoters to Treat Sickle Cell Disease
Background Sickle cell disease is caused by a defect in the β-globin subunit of adult hemoglobin (HbA). Sickle hemoglobin (HbS) polymerizes under hypoxic conditions producing deformed red blood cells (RBCs) that hemolyze and cause vaso-occlusion ...
Akshay Sharma +21 more
semanticscholar +1 more source
Understanding Sickle cell disease: Causes, symptoms, and treatment options
Sickle cell disease (SCD) is a hereditary blood disorder characterized by the production of abnormal hemoglobin molecules that cause red blood cells to take on a crescent or sickle shape.
C. Elendu +8 more
semanticscholar +1 more source
THE STATE OF HEMOGLOBIN IN SICKLED ERYTHROCYTES [PDF]
Electron microscopic and other evidence have provided support for the hypothesis that the sickling phenomenon is due to the intracellular formation of long slender crystals of reduced sickle hemoglobin. The rapid growth of these crystals causing tenting of the cell membrane is responsible for the bizarre distortion of the erythrocytes and presumably ...
openaire +2 more sources
Background Little is known about the effects of blood rheology on the occurrence of acute chest syndrome and painful vaso-occlusive crises in children with sickle cell anemia and hemoglobin SC disease.Design and Methods To address this issue, steady ...
Yann Lamarre +13 more
doaj +1 more source
Sickle Retinopathy in a Person with Hemoglobin S/New York Disease
A patient who presented with sickle retinopathy and hemoglobin electrophoresis results compatible with sickle cell trait was found, on further investigation, to be a compound heterozygote with hemoglobin S and hemoglobin New York disease.
Donovan Calder +4 more
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Objective: The aim of the present study was to characterize sickle cell disease retinopathy in children and teenagers from Bahia, the state in northeastern Brazil with the highest incidence and prevalence of sickle cell disease.
Dayse Cury de Almeida Oliveira +5 more
doaj +1 more source
Introduction: The benefits of manual versus automated red blood cell exchange have rarely been documented and studies in young sickle cell disease patients are scarce. We aim to describe and compare our experience in these two procedures.
Carlos Escobar +9 more
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