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Cognitive impairment is a poorly understood, serious, and emerging complication for adult patients with sickle cell disease. Because there is extensive microvascular damage from oxidative damage in sickle cell disease, the investigators hypothesize that this is also present in the cerebral microvasculature to cause cognitive impairment. The investigators plan to test this by correlating markers of inflammation and oxidative damage with cognitive performance and 7 Tesla brain MRI microvascular findings in these patients, with the long term goal of understanding the mechanisms and risk factors of cognitive impairment in sickle cell disease.
The Neurovascular Determinants of Cognitive Function in Adults with Sickle Cell Disease is a study for adults 18 and older who have been diagnosed with Sickle Cell Disease (SCD). Many adult patients with SCD suffer from cognitive impairment (CI), a serious complication responsible for severe functional limitations, and whose pathogenesis, risk factors, and natural history are unknown. This research project will look at cognitive performance and 7 Tesla brain MRI findings along with markers of inflammation and oxidation to better understand the mechanism and risk factors. The investigators will complete a baseline assessment. SCD participants will be seen for follow-up at 3 and 5 years. Non-SCD participants will be seen for follow-up at 3 years. All assessments are completed at no cost and there is compensation for participation.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| SCD participant | No intervention | ||
| Non-SCD participant | No intervention |
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| Measure | Description | Time Frame |
|---|---|---|
| 7 Tesla MRI | Multiple conventional and novel markers of small vessel disease will be evaluated. | 5 years |
| Measure | Description | Time Frame |
|---|---|---|
| Inflammation | Blood biomarkers of inflammation will be collected | 5 years |
| Oxidative markers | Blood biomarkers of oxidative markers will be collected. |
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Inclusion Criteria:
For Non-SCD :
Exclusion Criteria:
Age less than 18 years
Current pregnancy or lactation
Any medical condition that may result in neurocognitive or brain dysfunction that is not secondary to SCD including:
Any contraindication to MRI scanning including:
Inability to provide direct consent
In a current medical or pain crisis as diagnosed by attending physician -
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150 participants with Sickle Cell Disease (SCD) 50 participants without Sickle Cell Disease
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| Name | Affiliation | Role |
|---|---|---|
| Enrico Novelli, MD | University of Pittsburgh | Principal Investigator |
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| ID | Term |
|---|---|
| D000755 | Anemia, Sickle Cell |
| ID | Term |
|---|---|
| D000745 | Anemia, Hemolytic, Congenital |
| D000743 | Anemia, Hemolytic |
| D000740 | Anemia |
| D006402 | Hematologic Diseases |
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blood biomarkers of inflammation and oxidative damage
| 5 years |
| D006425 |
| Hemic and Lymphatic Diseases |
| D006453 | Hemoglobinopathies |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |