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Sickle cell disease (SCD), also simply called sickle cell, is a group of hemoglobin-related blood disorders typically inherited. The most common type is known as sickle cell anemia. It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells.
Sickle cell disease. In persons with sickle cell disease, high levels of fetal hemoglobin as found in a newborn or as found abnormally in persons with hereditary persistence of fetal hemoglobin, the HbF causes the sickle cell disease to be less severe. In essence the HbF inhibits polymerization of HbS.
Sickle cell-Hb Lepore Boston syndrome is a type of sickle cell disease (HbS) that differs from homozygous sickle cell disease where both parents carry sickle hemoglobin. In this variant one parent has the sickle cell hemoglobin the second parent has Hb Lepore Boston, the only one of the three variants described in association with HbS.
Epidemiology. Hemoglobin C is found most abundantly in areas of West Africa, such as Nigeria, where Yorubas live. Hemoglobin C gene is found in 2-3% of African-Americans while 8% of African-Americans have hemoglobin S (Sickle) gene. Thus Hemoglobin SC disease is significantly more common than Hemoglobin CC disease.
Sickle cell trait describes a condition in which a person has one abnormal allele of the hemoglobin beta gene (is heterozygous), but does not display the severe symptoms of sickle cell disease that occur in a person who has two copies of that allele (is homozygous).
Appointed to the staff of the MRC Epidemiology Research Unit at UWI in November 1972, the ground work was laid for newborn screening of sickle cell disease made possible by the expertise of Beryl who adapted methods for the diagnosis of the disease on samples taken from the umbilical cord.
United States. President. Beverley Francis-Gibson. Website. www.sicklecelldisease.org. The Sickle Cell Disease Association of America, Inc. ( SCDAA) is a nonprofit organization with the sole purpose of supporting research, education and funding of individuals, families those who are impacted by sickle cell disease .
Epidemiology. Around 1 in 50 people are affected by a known single-gene disorder, while around 1 in 263 are affected by a chromosomal disorder. [7] Around 65% of people have some kind of health problem as a result of congenital genetic mutations. [7]
SCD-CARRE – Sickle Cell Disease and CardiovAscular Risk - Red cell Exchange Trial: The Sickle Cell Disease and Cardiovascular Risk—Red Cell Exchange (SCD-CARRE) randomized clinical trial will enroll 150 adult participants with SCD in the United States and Europe. All participants will be followed on a monthly basis for one year.
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.