Since the discovery of sickle cell disease (SCD) in 1910, enormous strides have been made
in the elucidation of the pathogenesis of its protean complications, which has inspired recent …

Hemolysis is a fundamental feature of sickle cell anemia that contributes to its
pathophysiology and phenotypic variability. Decompartmentalized hemoglobin, arginase 1 …

Background For children with sickle cell anaemia and high transcranial doppler (TCD) flow
velocities, regular blood transfusions can effectively prevent primary stroke, but must be …

The primary β-globin gene mutation that causes sickle cell disease (SCD) has significant
pathophysiological consequences that result in hemolytic events and the induction of the …

For over 100 years, clinicians and scientists have been unravelling the consequences of the
A to T substitution in the β-globin gene that produces haemoglobin S, which leads to the …

Acute chest syndrome (ACS) is a frequent cause of acute lung disease in children with sickle
cell disease (SCD). Patients may present with ACS or may develop this complication during …

Intravascular hemolysis, or the destruction of red blood cells in the circulation, can occur in
numerous diseases, including the acquired hemolytic anemias, sickle cell disease and β …

In sickle-cell disease, a point mutation in the β-globin chain causes haemoglobin to
polymerise within erythrocytes during deoxygenation, altering red blood cell rheology and …

Pulmonary hypertension affects∼ 10% of adult patients with sickle cell disease (SCD),
particularly those with the homozygous genotype. An increase in pulmonary artery systolic …

Sickle cell disease, a common single gene disorder, has a complex pathophysiology that at
its root is initiated by the polymerization of deoxy sickle hemoglobin. Sickle vasoocclusion …