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Stroke represents a focal brain insult whereas chronic encephalopathy represents a diffuse brain disturbance involving cognition and memory. The predisposition to stroke and the potential for dementia were increasingly apparent from our studies and the work of other investigators in sickle cell disease. The ontogeny of these two processes required further study before the mechanisms could be clearly articulated; and a coordinated assessment of cerebrovascular perfusion was needed to decipher the relationship between focal perfusion deficits and occlusion of large and small cerebral vessels. 150 subjects were enrolled in these studies. Improved understanding of the mechanisms underlying these two devastating neurological complications of SCD should result in prevention or effective treatment.
Three hypotheses were tested: (1) clinically-silent cranial magnetic resonance imaging (MRI) abnormalities represented the minimal expression of the neurovascular diathesis, and were the harbingers of clinically-overt strokes (study A); (2) sickle cell disease patients who developed cerebral infarctions had a predisposing risk factor(s) that contributed to this neurological complication (study B); and (3) sickle cell disease patients developed a chronic encephalopathy and dementia that was independent of the neurovascular diathesis (study C). Study A was a prospective evaluation of 50 SCD children aged 6 to 12 years attempting to identify a subgroup of patients at risk for the development of a clinically-apparent stroke. These patients were evaluated clinically, and underwent MRI scan, magnetic resonance angiography (MRA), and single photon emission computerized tomography (SPECT). Study B represented two studies designed to analyze risk factors for stroke. The first study was a retrospective case-control analysis of 25 young adults who suffered one or more strokes. These patients were age-matched to an SCD control group which had been clinically free of strokes and had MRI, MRA, and SPECT studies. The second study represented a prospective case-control analysis of children who were being followed in study A. Study C represented a prospective study of 50 SCD children, and 50 age-matched siblings or closest available relatives. Annual neurological examinations and neuropsychological evaluations were performed searching for evidence of chronic encephalopathy and dementia. The longitudinal study design was necessary to dissect out the subtle variables that contributed to the cognitive deficits. The study was renewed in FY 1998 to continue through FY 2004.
Note: Darryl DeVivo was PI on Subproject which began in FY 1988 as a competing renewal. The total grant began several years prior to that. Dollars for the subproject were estimated at 10% of the total funds awarded each year.
Observational Model: Natural History, Time Perspective: Longitudinal
Anemia, Sickle Cell
National Heart, Lung, and Blood Institute (NHLBI)
Published on BioPortfolio: 2014-08-27T03:57:53-0400
To conduct a prospective study aimed at the early detection and treatment of cerebral vascular disease prior to irreversible brain injury in young children with sickle cell anemia (SCA).
This study will summarized the clinical and laboratory data and the outcome of all the patients suffering from Sickle Cell Anemia (Including Sickle cell thalassemia) admitted to the pediat...
Sickle cell disease (SCD), also known as sickle cell anemia, is an inherited genetic disease that can cause intense pain episodes. This study will evaluate the effectiveness of the nutriti...
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 resp...
Children with sickle cell anemia (SCA) seem to have higher energy needs than children who do not have the disease. This may be the reason why children and teenagers with sickle cell anemia...
To evaluate different T -oxygenation calibrations for estimating venous oxygenation in people with sickle cell anemia (SCA).
Sickle cell anemia is the commonest genetic disorder in India, and the frequency of the sickle cell gene is very high in the remote tribal areas where facilities are generally limited. Therefore, a ra...
Sickle cell disease (SCD) is an autosomal recessive disorder caused by a mutation in β-globin (HBB) gene. We have generated an induced pluripotent stem cell (iPSC) line, IGIBi001-A from an Indian sic...
Present study was undertaken to study the association between sickle cell anemia (SCA) and glucose-6-phosphate dehydrogenase (G6PD) deficiency from Sahu and Kurmi population of Durg and Rajnandgaon di...
To describe the occurrence of both peripheral and central auditory system dysfunction in sickle cell anaemia (SCA) patients and discuss the different mechanisms hypothesized to be responsible for thes...
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.
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.
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.
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)
K-Cl cotransporter ubiquitously expressed with higher expression levels in ERYTHROCYTES of ANEMIA, SICKLE CELL. It mediates active potassium and chloride cotransport across the plasma membrane and contributes to cell volume homeostasis
Stroke - Cerebrovascular Disease (CVA)
A stroke is a serious medical condition that occurs when the blood supply to part of the brain is cut off. Strokes are a medical emergency and prompt treatment is essential because the sooner a person receives treatment for a stroke, the less damage is ...
Neurology - Central Nervous System (CNS)
Alzheimer's Disease Anesthesia Anxiety Disorders Autism Bipolar Disorders Dementia Epilepsy Multiple Sclerosis (MS) Neurology Pain Parkinson's Disease Sleep Disorders Neurology is the branch of me...