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Sickle cell disease (SCD), also simply called sickle cell, is a group of hemoglobin-related blood disorders that are typically inherited. [2] The most common type is known as sickle cell anemia. [2] Sickle cell anemia results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. [2]
The sickle cell trait provides a survival advantage against malaria fatality over people with normal hemoglobin in regions where malaria is endemic. The trait is known to cause significantly fewer deaths due to malaria, especially when Plasmodium falciparum is the causative organism.
The hemoglobin structural variants can be broadly classified as follows: [19] Sickle cell disorders, which are the most prevalent form of hemoglobinopathy. Sickle hemoglobin (HbS) is prone to polymerize when deoxygenated, precipitating within the red blood cell. This damages the RBC membrane resulting in its premature destruction and consequent ...
Sickle Cell Anemia, a Molecular Disease" is a 1949 scientific paper by Linus Pauling, Harvey A. Itano, Seymour J. Singer and Ibert C. Wells that established sickle-cell anemia as a genetic disease in which affected individuals have a different form of the metalloprotein hemoglobin in their blood.
Thus, it was not clear whether it was involved in sickle cell disease. Genetically, the abnormal hemoglobin was only in heterozygous condition. [22] The next year, Neel and his colleagues established that the hemoglobin is associated with sickle cell disease. [23] The hemoglobin was named hemoglobin III, [24] but hemoglobin C was eventually used.
A sickle cell health crisis can escalate into life-threatening complications, but patients still struggle to get seen quickly in emergency rooms and also to get pain medicine.
In children and adults red blood cell transfusion to increase the hemoglobin level to 100 g/L has been shown to decrease the risk of sickle cell-related complications. [2] However, this has not been seen in all studies, and has only been demonstrated for African haplotypes of Hemoglobin SS. [12] [13]
Sickle cell disease is an inherited long-term and potentially life-threatening condition which predominantly affects Black people, with around 15,000 people in England thought to live with the ...
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