sickle cell anemia

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Related to sickle cell disease: sickle cell trait, Sickle cell crisis

sickle cell anemia

A chronic, severe, and sometimes fatal anemia marked by crescent-shaped red blood cells and characterized by fever, leg ulcers, jaundice, and episodic pain in the joints. The disease occurs in people who are homozygous for a gene that produces an abnormal form of hemoglobin, and it is found chiefly in people of African descent and in some Mediterranean, Middle Eastern, Central and South American, and South Asian populations. Also called sickle cell disease.

sick′le cell` ane′mia

a chronic hereditary blood disease, primarily affecting indigenous Africans and their descendants, in which an accumulation of oxygen-deficient sickle cells results in anemia, blood clotting, and joint pain.
Also called sicklemia.

sick·le cell anemia

A hereditary disease characterized by red blood cells that are sickle-shaped instead of round because of an abnormality in the hemoglobin, the protein that carries oxygen in the blood. Because of their shape, the cells can cause blockage of small blood vessels in the organs and bones, reducing the amount of oxygen available to those tissues.
Did You Know? Genetic mutations can be good or bad, and sometimes they can even be both. The mutation that causes sickle cell anemia is one example. It is harmful if a person inherits two copies of the mutated gene (one from each parent), but there is actually some benefit if only one copy of the gene is inherited. The defective gene causes red blood cells to be distorted into a sickle shape, which makes it hard for them to pass through the tiny blood vessels where they give oxygen to body tissues. If a person's chromosomes have two copies of the mutated gene, serious sickle cell anemia results, causing illness. With just one copy of the gene, though, only some mild sickling of the cells occurs. It so happens that this mild sickling is harmful to the parasite that causes malaria, and can protect a person from that disease. In a region like tropical Africa where malaria is common, people who have the mutation in one gene are more likely to ward off a malarial infection and to live long enough to have children, who then inherit the gene in turn. And because inheriting two copies of the gene is much less likely than inheriting just one, the benefits of the gene outweigh its risks for most people in these regions.
References in periodicals archive ?
Patients with sickle cell disease face a lifetime of challenges, and more research is desperately needed to address evidence gaps and improve our understanding of the best possible care for these patients," said Alexis Thompson, MD, MPH, moderator of the press conference and professor of pediatrics at the Ann & Robert H.
The sickle cell and thalassaemia conference is a forum for dialogue and interaction between the leading world experts in sickle cell disease and thalassaemia and health care professionals at the frontline of care, and is intended to update those working with patients across the world with SCD to consider efficient and safe ways to manage patients.
Patients with sickle cell disease often suffer from painful attacks known as vaso-occlusive crises, during which their sickle-shaped blood cells get stuck in tiny capillaries, depriving tissues of needed oxygen.
All TLOD chapters have programs that benefit sickle cell disease research and families throughout the United States.
The forensic medical examination reinforced the report by the hospital that the death was natural and caused by complications related to sickle cell disease, the statement said.
Diffusion-weighted MRI in 52 of these children showed evidence of acute silent infarction in 4 (18%) of 22 patients with sickle cell disease and in 2 (7%) of 30 patients without sickle cell disease, Dr.
One-quarter to one-third of children with sickle cell disease have evidence of silent strokes in their brains, said lead author Michael M.
A 2007 review in Bone Marrow Transplantation noted that: "Currently, hematopoietic SCT (HCT) is the only intervention that can restore normal hematopoiesis to provide a 'cure' in sickle cell disease.
Consultant geneticist and head of the Salmaniya Medical Complex (SMC) genetics department Dr Shaikha Al Arrayed, who represented Bahrain at the First Global Congress on Sickle Cell Disease, spoke about Bahrain winning the war against the potentially deadly disease after a 70 per cent fall in cases.
But the German researchers said in a letter published online Wednesday by the Journal of the American Medical Association that closer scrutiny of his foot bones pointed to sickle cell disease, in which red blood cells become dangerously misshaped.
Based on data from the 1997-2005 National Health Interview Survey, the study found black children with sickle cell disease were four times more likely to have fair or poor health status, were twice as likely to have recently visited a mental health professional and were more likely to have received special educational or early intervention services than black children without sickle cell disease.
Speaking on the eve of World Sickle Cell Day, which falls on Saturday, the official said while the incidence of sickle cell disease was 2.

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