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Sickle-cell anaemia (also known as sickle-cell disorder or sickle-cell disease) is a common genetic condition due to a haemoglobin disorder – inheritance of mutant haemoglobin genes from both parents. Such haemoglobinopathies, mainly thalassaemias and sickle-cell anaemia, are globally widespread.
4 Σεπ 2023 · Sickle cell anemia is characterized by two major components: Hemolysis and vaso-occlusive crises (VOC). The defect in the beta-globin gene makes the sickle hemoglobin (HbS) molecule susceptible to converting into rigid, elongated polymers in a deoxygenated state.
INTRODUCTION. Hemoglobin has evolved to be an efficient oxygen (O 2) transporter. Its function, understood in terms of a two-state model of allostery, serves as a paradigm for many other proteins. A single β-globin gene (HBB glu6val) point mutation resulting in sickle hemoglobin (HbS) is the proximate cause of sickle cell disease (Chapter 19).
22 Αυγ 2012 · Sickle cell disease (SCD) 1 includes a group of related genetic diseases affecting hemoglobin structure, 2 and is considered the most common genetic blood disorder among children.
24 Ιαν 2019 · Based on current evidence, the pathobiology of SCD is considered to be a vicious cycle of four major processes, all the subject of active study and novel therapeutic targeting: (a) hemoglobin S...
Sickle cell disease (SCD) is an inherited blood disorder that affects red blood cells. Normal red blood cells are round and flexible, which lets them travel through small blood vessels to deliver oxygen to all parts of the body.
• Evidence-based treatment guidelines for sickle cell disease complications that affect the heart, lungs, and kidneys. • SCD affects multiple organs that require patients to seek care from doctors that
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