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Sickle cell disease is an inherited blood disorder that affects red blood cells (RBCs). People with sickle cell disease frequently experience anemia, or a low number of RBCs. RBCs are responsible for carrying oxygen to the brain and other body tissues that need oxygen to function properly. The purpose of this study is to determine what changes, which were possibly caused by anemia, exist in the brains of individuals with sickle cell disease.
The role of RBCs is to carry oxygen from the lungs to the brain and other body tissues. Individuals with anemia have unusually low numbers of RBCs. They also often have difficulty concentrating and remembering information, which is likely caused by a reduced oxygen supply to the brain. Previous research has shown that correcting anemia in patients without sickle cell disease improved their memory and attention. The purpose of this study is to examine any abnormal changes in the brains of individuals with sickle cell disease and to determine whether these changes are related to reduced memory and attention capabilities.
Participants will attend one study visit. During this visit, a brain magnetic resonance image (MRI) will be performed while participants complete neuropsychological tests that measure memory, attention, and organizational ability. There will be no follow-up visits.
Observational Model: Case Control, Primary Purpose: Screening, Time Perspective: Cross-Sectional, Time Perspective: Retrospective/Prospective
Sickle Cell Disease
University of California San Francisco
National Heart, Lung, and Blood Institute (NHLBI)
Published on BioPortfolio: 2014-08-27T03:39:29-0400
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One of the sickle cell disorders characterized by the presence of both hemoglobin S and hemoglobin C. It is similar to, but less severe than sickle cell anemia.
An abnormal hemoglobin resulting from the substitution of valine for glutamic acid at position 6 of the beta chain of the globin moiety. The heterozygous state results in sickle cell trait, the homozygous in sickle cell anemia.
An acute purulent infection of the meninges and subarachnoid space caused by Streptococcus pneumoniae, most prevalent in children and adults over the age of 60. This illness may be associated with OTITIS MEDIA; MASTOIDITIS; SINUSITIS; RESPIRATORY TRACT INFECTIONS; sickle cell disease (ANEMIA, SICKLE CELL); skull fractures; and other disorders. Clinical manifestations include FEVER; HEADACHE; neck stiffness; and somnolence followed by SEIZURES; focal neurologic deficits (notably DEAFNESS); and COMA. (From Miller et al., Merritt's Textbook of Neurology, 9th ed, p111)
A disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs. It is the clinical expression of homozygosity for hemoglobin S.
The condition of being heterozygous for hemoglobin S.
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