Examining Cognitive Function and Brain Abnormalities in Adults With Sickle Cell Disease
Neuropsychological Dysfunction and Neuroimaging Abnormalities in Neurologically Intact Adults With Sickle Cell Disease
3 other identifiers
observational
212
1 country
12
Brief Summary
Sickle cell disease (SCD), also known as sickle cell anemia, is an inherited blood disease that can cause intense pain episodes and may lead to organ failure. Preliminary studies have shown that adults with SCD may have brain abnormalities that contribute to problems with cognitive functioning, including attention and memory difficulties. This study will use brain magnetic resonance imaging (MRI) and neuropsychological testing to examine the differences in cognitive functioning in adults with SCD and adults without SCD. 212 subjects participated in this cross-sectional study consisting of screening questionnaires, a neuropsychological testing battery, and MRI testing. Enrollment into this study ended in May 2008.
Trial Health
Trial Health Score
Automated assessment based on enrollment pace, timeline, and geographic reach
participants targeted
Target at P75+ for all trials
Started Dec 2004
Typical duration for all trials
12 active sites
Health score is calculated from publicly available data and should be used for screening purposes only.
Trial Relationships
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Study Timeline
Key milestones and dates
Study Start
First participant enrolled
December 1, 2004
CompletedFirst Submitted
Initial submission to the registry
September 10, 2007
CompletedFirst Posted
Study publicly available on registry
September 12, 2007
CompletedPrimary Completion
Last participant's last visit for primary outcome
May 1, 2008
CompletedStudy Completion
Last participant's last visit for all outcomes
May 1, 2008
CompletedResults Posted
Study results publicly available
November 26, 2009
CompletedMarch 21, 2017
February 1, 2017
3.4 years
September 10, 2007
August 28, 2009
February 14, 2017
Conditions
Keywords
Outcome Measures
Primary Outcomes (1)
Wechsler Adult Intelligence Scale (WAIS)-III Performance IQ
Extent of neurocognitive dysfunction in neurologically asymptomatic adult patients with sickle cell disease as measured by WAIS-III performance IQ. This quotient is based on an average of 100, with a standard deviation of 15. The Wechsler intelligence scales are not considered adequate measures of extremely high and low intelligence (IQ scores above 160 and below 40, respectively). The performance IQ is derived from scores on seven subtests: picture completion, picture arrangement, block design, object assembly, digit symbol, matrix reasoning, and symbol search.
Within 2 months of signing informed consent.
Secondary Outcomes (2)
Participants With Brain Lacunae as Measured by Clinical MRI
Within 2 months of informed consent
Volume of Total Cortical Gray Matter as Measured by Volumetric MRI.
Within 2 months of informed consent
Study Arms (2)
Cases (CLOSED)
These are patients diagnosed with sickle cell disease (confirmed by hemoglobin electrophoresis).
Controls (CLOSED)
These are persons that do not have sickle cell disease (confirmed by hemoglobin electrophoresis); matched to cases by age, gender, and education level
Interventions
Neuropsych Battery with 7 different tests that evaluate the patients neurological functioning.
The MRI is a standard procedure involving 30 minutes under the machine in order to obtain various images of the patients brain.
Eligibility Criteria
212 participants, 160 will have sickle cell anemia, 52 will be matched controls based on gender, age, and education level
You may qualify if:
- Individuals who meet all of the following criteria are eligible for enrollment as cases into the study:
- Adult between the ages of 21 and 55
- African descent
- Proficient/fluent in English
- Hemoglobin electrophoresis confirming hemoglobin SS or SB0 (%A \<= 15)
- Hemoglobin \<= 10 g/dL
- Capable of giving informed consent for the protocol
- Individuals who meet all of the following criteria are eligible for enrollment as community controls into the study:
- Adult between the ages of 21 and 55
- African descent
- Proficient/fluent in English
- Capable of giving informed consent for the protocol
You may not qualify if:
- Individuals who meet any of the following criteria are disqualified from enrollment in the case group of the study:
- Overt stroke
- Previous evidence of an abnormal MRI or CT other than small peri-ventricular or watershed lesions
- History of head injury that resulted in neurological symptoms or medical visit
- Abnormal neurologic exam with focal findings
- Mini-Mental Status Examination (MMSE) score of \< 20
- Profile of Mood States (POMS) score on the Depression-Dejection Subscale suggestive of a clinical depression (score \> 40)
- Alcohol consumption exceeding 14 drinks/week if female, 21 drinks/week if male
- Drug abuse, defined as using non-prescribed medication
- History of claustrophobia and/or presence of metallic implants such as pacemakers, surgical aneurysm clips, or known metal fragments embedded in the body
- Pregnancy
- Baseline blood pressure \> 140/90 on two repeated measurements. A second measurement is needed only if the first is \> 140/90
- History of uncontrolled hypertension
- Any chronic disorder that may result in neurocognitive or brain dysfunction that is not secondary to SCD including:
- Inflammatory arterial disorders (lupus, polyarteritis)
- +49 more criteria
Contact the study team to confirm eligibility.
Sponsors & Collaborators
Study Sites (12)
University of Southern California
Los Angeles, California, 90033, United States
Children's Hospital & Research Center at Oakland
Oakland, California, 94609, United States
Memorial Cancer Institute
Hollywood, Florida, 33021, United States
University of Miami Miller School of Medicine
Miami, Florida, 33136, United States
Medical College of Georgia
Augusta, Georgia, 30912, United States
Boston Medical Center
Boston, Massachusetts, 02118, United States
University of North Carolina at Chapel Hill
Chapel Hill, North Carolina, 27599, United States
Duke University Medical Center
Durham, North Carolina, 27705, United States
Cincinnati Children's Hospital
Cincinnati, Ohio, 45229, United States
University of Cincinnati Medical Center
Cincinnati, Ohio, 45267, United States
Children's Medical Center at Dallas
Dallas, Texas, 75390, United States
University of Texas Medical Branch
Galveston, Texas, 77555, United States
MeSH Terms
Conditions
Condition Hierarchy (Ancestors)
Limitations and Caveats
As a cross-sectional study, follow-up of patients not included. Age range of cases and controls not weighted enough to include elder population. Functional or perfusion brain measures not included. Biologic and genetic risk factors not included.
Results Point of Contact
- Title
- Elliott Vichinsky, MD
- Organization
- Children's Hospital of Oakland and Research Institute
Study Officials
- PRINCIPAL INVESTIGATOR
Elliott Vichinsky, MD
Northern California CSCC (Children's Hospital Oakland)
Publication Agreements
- PI is Sponsor Employee
- No
- Restrictive Agreement
- No
Study Design
- Study Type
- observational
- Observational Model
- CASE CONTROL
- Time Perspective
- CROSS SECTIONAL
- Sponsor Type
- OTHER
Study Record Dates
First Submitted
September 10, 2007
First Posted
September 12, 2007
Study Start
December 1, 2004
Primary Completion
May 1, 2008
Study Completion
May 1, 2008
Last Updated
March 21, 2017
Results First Posted
November 26, 2009
Record last verified: 2017-02