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ClinicalTrials.gov - Information on Clinical Trials and Human Research Studies: Browse: Anemia, Sickle Cell
ClinicalTrials.gov - Information on Clinical Trials and Human Research Studies: Browse: Anemia, Sickle Cell
MedlinePlus
Medical Encyclopedia: Sickle cell anemia
Sickle Cell anemia is inherited as an autosomal recessive trait. This means it occurs in someone who has inherited hemoglobin S from both parents. Sickle Cell disease is much more common in certain ethnic groups, affecting approximately one out of every 500 African Americans. Someone who inherits hemoglobin S from one parent and normal hemoglobin (A) from the other parent will have Sickle Cell trait . Someone who inherits hemoglobin S from one parent and another type of abnormal hemoglobin from the other parent will have another form of Sickle Cell disease, such as thalassemia .
What Is Sickle Cell Anemia?
Sickle Cell trait is different from Sickle Cell anemia. People with Sickle Cell trait don t have the condition, but they have one of the genes that cause the condition. Like people with Sickle Cell anemia, people with Sickle Cell trait can pass the gene on when they have children. To learn more about Sickle Cell trait, see the section on causes of Sickle Cell anemia .
Anemia, sickle cell
Anemia, Sickle Cell
Sickle Cell Anemia Disease Profile
In general, both
children and adults with Sickle Cell anemia are more vulnerable to infections
and have a harder time fighting them off. This is the result of spleen
damage from sickled red cells, thus preventing the spleen from destroying
bacteria in the blood. Infants and young children, especially, are susceptible
to bacterial infections that can kill them in as little as 9 hours from
onset of fever. Pneumococcal infections used to be the principal cause
of death in children with Sickle Cell anemia until physicians began routinely
giving penicillin on a preventive basis to those who are diagnosed at
birth or in early infancy.
Sickle cell disease - Genetics Home Reference
The signs and symptoms of Sickle Cell disease are caused by the sickling of red blood cells. When red blood cells Sickle, they break down prematurely, which can lead to anemia. Anemia can cause shortness of breath, fatigue, and delayed growth and development in children. The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice. Painful episodes can occur when sickled red blood cells, which are stiff and inflexible, get stuck in small blood vessels. These episodes deprive tissues and organs of oxygen-rich blood and can lead to organ damage, especially in the lungs, kidneys, spleen, and brain. A particularly serious complication of Sickle Cell disease is high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension). Pulmonary hypertension occurs in about one-third of adults with Sickle Cell disease and can lead to heart failure.
New Hope for People with Sickle Cell Anemia (FDA Consumer Reprint)
Research continues on other possible ways of reducing the occurrence of painful Sickle Cell episodes by increasing the production of fetal hemoglobin. For example, NIH scientists are studying whether a combined regimen of hydroxyurea and erythropoietin, a hormone that increases the production of red blood cells, is less toxic and more effective than Hydrea alone. (Erythropoietin is licensed by FDA to treat anemia in certain patients.) Studies are also under way to determine the safety and efficacy of butyrate, an experimental drug that can stimulate production of fetal hemoglobin.
Minority Women's Health - African Americans - Sickle Cell Anemia
Sickle Cell anemia is a blood disorder that is passed down from parents to children. It involves problems with the red blood cells. Normal red blood cells are round and smooth and move through blood vessels easily. Sickle cells are hard and have a curved edge. These cells can't get through small blood vessels, which keeps the organs from getting blood. Your body destroys Sickle red cells quickly, but sometimes it can't make new red blood cells fast enough. This is a condition called anemia. Sickle Cell anemia can cause serious health problems, including:
MedlinePlus: Sickle Cell Anemia
A genetic problem causes Sickle Cell anemia. People with the disease are born with two
Sickle Cell genes, one from each parent. If you only have one Sickle Cell gene, it's
called Sickle Cell trait. About 1 in 12 African Americans has Sickle Cell trait. A blood
test can show if you have the trait or anemia. Most states test newborn babies as part of
their newborn screening programs.
What Causes Sickle Cell Anemia?
In Sickle Cell anemia, the abnormal hemoglobin sticks together when it gives up its oxygen to the tissues. These clumps are like liquid crystals that cause red blood cells to become stiff and shaped like a Sickle, or C. It takes two copies of the Sickle Cell gene for the body to make the abnormal hemoglobin found in Sickle Cell anemia.
Blood Diseases and Cancer - Evaluation, Treatment and Research at the National Heart, Lung and Blood Institute
This research protocol allows us to evaluate and treat you because you have Sickle Cell disease, Sickle Cell trait, or another anemia or red blood Cell disorder such as thalassemia. This will help us gain more understanding of your disease, painful attacks and lung complications often associated with these anemias. You will receive standard medical care for your blood disease and its complications. This means that you will not receive experimental treatment under this protocol.
NIH - Sickle Cell Anemia
What Is Sickle Cell Anemia?
Sickle Cell Anemia Gene
The structure of
normal deoxy hemoglobin is shown in Figure 1. The four gray clusters are
noncovalently bonded heme groups. Each heme serves as a contact site for
binding one molecule of oxygen. The two gold spheres near the top of the
molecule are phosphate groups. The green and blue chains are alpha chains,
and the gold and aqua chains are beta chains. The red box highlights the
general region of two beta chains where the sixth glutamic acid residue
is located. A genetic variant associated with this amino acid is the most
common cause of Sickle Cell anemia. Figure 2 shows the clumping together
of two deoxy hemoglobin molecules that contain this genetic variant.
Search of: Open Studies | "Anemia, Sickle Cell" - List Results - ClinicalTrials.gov
Sulfadoxine- Pyrimethamine Versus Weekly Chloroquine for Malaria Prevention in Children With Sickle Cell Anemia
Genomics|HuGENet|Reviews|Hb S Allele and Sickle Cell Disease|PubMed ID: 10791557
In 1972, the 92 nd congress of the US passed the National Sickle Cell Anemia Control Act (20). This law called for grant support for screening programs and in 1975, the first US state began a newborn screening program for Sickle Cell disease (21). However, it was the late 1980’s before most states were performing Sickle Cell, or hemoglobinopathy newborn screening (2). This was most likely due to the publication of a study in 1986 that showed that oral penicillin could significantly reduce the morbidity and mortality in children with Sickle Cell disease (22). In 1987, the National Institutes of Health held a conference that supported early diagnosis by newborn screening as being beneficial to infants with Sickle Cell disease (23). In 1993, another US agency, the Agency for Health Care Policy and Research (AHCPR), also concluded that newborn screening combined with comprehensive health care would significantly reduce the morbidity and mortality in infants with Sickle Cell disease (2). AHCPR further recommended that all infants should be tested for Sickle Cell disease, regardless of race (universal screening) since targeting high risk racial or ethnic groups would not identify all affected infants due to the inability to reliably determine the infants’ race by appearance, name or self-report. As of 1992, 40 of the states in the US, as well as the District of Columbia, Puerto Rico and the Virgin Islands, were performing hemoglobinopathy screening, although not necessarily on a universal basis (2).
genome.gov | Learning About Sickle Cell Disease
Sickle cells are destroyed rapidly in the bodies of people with the disease, causing anemia. This anemia is what gives the disease its commonly known name - Sickle Cell anemia.
Definition of sickle cell anemia - NCI Dictionary of Cancer Terms
An inherited disease in which the red blood cells have an abnormal crescent shape, block small blood vessels, and do not last as long as normal red blood cells. Sickle Cell anemia is caused by a mutation (change) in one of the genes for hemoglobin (the substance inside red blood cells that binds to oxygen and carries it from the lungs to the tissues). It is most common in people of West and Central African descent. Also called Sickle Cell disease.
Sickle Cell Anemia - Evaluation, Treatment and Research at the National Heart, Lung and Blood Institute
This research protocol allows us to evaluate and treat you because you have Sickle Cell disease, Sickle Cell trait, or another anemia or red blood Cell disorder such as thalassemia. This will help us gain more understanding of your disease, painful attacks and lung complications often associated with these anemias. You will receive standard medical care for your blood disease and its complications. This means that you will not receive experimental treatment under this protocol.
Sickle Cell Anemia and Stroke
The sickled cells in a child with Sickle Cell anemia have a hard time moving through the blood vessels in the brain. If some cells get "stuck" and can't move, other sickled cells pile up behind and cause a "log jam" that blocks the blood vessels. Oxygen can't get past the block to other parts of the brain, which causes the stroke.
OMIM - SICKLE CELL ANEMIA
OMIM - Sickle Cell ANEMIA
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