sickle cell anemia

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

sickle cell anemia

n.
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



n.
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.
[1925–30]

sick·le cell anemia

(sĭk′əl)
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 ?
US-based therapeutics developer Emmaus Life Sciences's marketing authorization application for Xyndari has been fully validated and is now under assessment by the European Medicines Agency (EMA) for the treatment of sickle cell disease, the company said.
It is critical that we understand the full breadth of what were facing in the fight to cure Sickle Cell Disease, Senator Scott said.
Unless they can receive a bone marrow transplant, the average life expectancy of a child diagnosed with sickle cell disease is just 34 years.
06 ( ANI ): A study has recently warned that if Acute Chest Syndrome (ACS), a potentially severe lung complication of sickle cell disease, is not treated effectively in children then it may increase the risk of respiratory failure, chronic lung disease and prolonged hospitalisation.
The library being created by scientists at the Center for Regenerative Medicine (CReM) at Boston Medical Center (BMC) and Boston University School of Medicine (BUSM) comprises blood samples from ethnically diverse patients with sickle cell disease from around the world and represents the major genetic backgrounds on which the sickle cell mutation occurred.
Biopharmaceutical company Blood Therapeutics (NasdaqGS:GBT) reported on Wednesday the receipt of the European Commission's (EC) orphan medicinal disignation for GBT440 for the treatment of sickle cell disease (SCD).
To address the challenges, the American Society of Hematology in September launched the Sickle Cell Disease Coalition, of which APHA is a member, and released "State of Sickle Cell Disease: 2016 Report" to evaluate four priority areas: access to care, training and professional education, research and clinical trials, and global health.
html), which tracks health information of persons living with sickle cell disease in the United States throughout their lives.
Additionally, the report provides an overview of key players involved in therapeutic development for Sickle Cell Disease and features dormant and discontinued projects.
The research questions were 1) to what extent are children and adolescents with sickle cell disease at risk for depression and anxiety, 2) what is the quality of life in children and adolescents with sickle cell disease, and 3) what factors (age, gender, and number of pain episodes) have significant effects on risk for depression and/or anxiety and quality of life in children and adolescents with sickle cell disease?
Ofori-Acquah said, "Pre-clinical studies of NVX-508 suggest that the drug has great potential to ameliorate symptoms and reverse damage from hypoxia in VOC and ACS in sickle cell disease.
To study various surgical presentation in sickle cell disease.

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