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Transfusion independence and HMGA2 activation after gene therapy of human β-thalassaemia
The β-haemoglobinopathies are the most prevalent inherited disorders worldwide. Gene therapy of β-thalassaemia is particularly challenging given the requirement for massive haemoglobin production inExpand
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Impact of early transcranial Doppler screening and intensive therapy on cerebral vasculopathy outcome in a newborn sickle cell anemia cohort.
Transcranial Doppler (TCD) is used to detect children with sickle cell anemia (SCA) who are at risk for stroke, and transfusion programs significantly reduce stroke risk in patients with abnormalExpand
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The natural history of asymptomatic osteonecrosis of the femoral head in adults with sickle cell disease.
BACKGROUND Osteonecrosis of the femoral head is a frequent complication in adult patients with sickle cell disease. However, little is known about the natural history of asymptomatic lesions. Expand
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Endothelial dysfunction in patients with sickle cell disease is related to selective impairment of shear stress-mediated vasodilation.
Interactions between the endothelium and erythrocytes may contribute to the vascular complications of sickle cell disease (SCD). Endothelium-derived nitric oxide (NO) plays a major role in theExpand
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Influence of sickle cell disease and treatment with hydroxyurea on sperm parameters and fertility of human males
The use of hydroxyurea has considerably modified the prognosis of sickle cell disease and many more patients now reach reproductive age. This study shows alterations of semen parameters due to sickleExpand
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Glucose-6-Phosphate Dehydrogenase Deficiency and Homozygous Sickle Cell Disease in Congo
G6PD genotypes were determined in Brazzaville (Congo) on 188 HbSS patients (109 females, 79 males) and 210 controls (115 females and 95 males) with HbAA. DNA samples were analyzed by the polymeraseExpand
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Sickle-Cell Disease in Brazzaville, Congo: Genetical, Hematological, Biochemical and Clinical Aspects
Clinical, hematological and molecular features of 116 unrelated sickle-cell anemia patients from Brazzaville were investigated. The mean age of the patients was 9.4 ± 5.3 years. 232 βs-chromosomesExpand
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Erythrocyte density in sickle cell syndromes is associated with specific clinical manifestations and hemolysis.
Dense, dehydrated red blood cells (DRBCs) are a characteristic feature of sickle-cell disease (SCD). DRBCs play a role in the pathophysiology of SCD acute and chronic organ damage because ofExpand
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[Secondary iron overload].
Secondary iron overload (SIO) constitutes a growing clinical problem, particularly in haematological diseases in which the improvements of life expectancy give the iron overload enough duration toExpand
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Pyruvate kinase deficiency in France: a 3‐year study reveals 27 new mutations
Pyruvate kinase (PK) deficiency is the most common enzyme defect affecting the glycolytic pathway of the erythrocyte. Usually, it is clinically silent in heterozygotes but serious disorders areExpand
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