Problem
Sickle cell disease
Other Names:
Sickle cell anaemia Nature:
A hereditary disease carried by a single recessive gene. When the gene is in homozygous (paired) condition in an individual, the disease is expressed. It causes the red blood cells to collapse and stack into columns under acidic blood conditions, as happens for example with physical exertion. The "rods" of stacked blood cells can clog blood vessels and limit the effective flow of oxygenated blood around the body. The shape of the "sickled" blood cells (as seen under a microscope) triggers a response in the body which very quickly destroys them, so adding to the symptoms of anaemia.
Incidence:
Sickle cell disease is endemic in parts of Africa, particularly west Africa. In other parts of the world, it is more prevalent among those of African extraction than among other racial groups.
It has a relatively high incidence because in its recessive state it affords protection against malaria. People with sickle cell anaemia, and heterozygous carriers of the gene, usually recover from childhood infection by malaria.
Broader Problems:
Hereditary haemolytic anaemias Reduced By:
Vector resistance to diseaseReferences:
Serjeant, Graham R: Sickle Cell DiseaseBohrer, Stanley P and Alavi, Abass: Bone Ischaemia and Infarction in Sickle Cell Disease Problem Type:
E: Emanations of other problems Date of last update
04.10.2020 – 22:48 CEST