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

Sickle cell disease (SCD) substantially alters renal structure and function, and causes
various renal syndromes and diseases. Such diverse renal outcomes reflect the uniquely …

Sickle-cell disease is one of the most common severe monogenic disorders in the world.
Haemoglobin polymerisation, leading to erythrocyte rigidity and vaso-occlusion, is central to …

Background Sickle-cell anaemia is associated with substantial morbidity from acute
complications and organ dysfunction beginning in the first year of life. Hydroxycarbamide …

Sickle cell syndromes, including sickle cell disease (SCD) and sickle cell trait, are
associated with multiple kidney abnormalities. Young patients with SCD have elevated …

Sickle cell anemia is associated with unusual clinical heterogeneity for a Mendelian
disorder. Fetal hemoglobin concentration and coincident α thalassemia, both which directly …

Background Sickle cell disease (SCD) is one of the most common inherited diseases
worldwide. It is associated with lifelong morbidity and a reduced life expectancy …

In the 100 years since sickle cell anemia (SCA) was first described in the medical literature,
studies of its molecular and pathophysiological basis have been at the vanguard of scientific …

The sickle hemoglobin is an abnormal hemoglobin due to point mutation (GAG→ GTG) in
exon 1 of the β globin gene resulting in the substitution of glutamic acid by valine at position …

Sickle cell disease (SCD) is a group of inherited autosomal recessive disorders, including
sickle cell anemia, sickle beta-thalassemia, and other hemoglobinopathies, which are …