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Cerebrovascular Involvement in Sickle Cell Disease - Comprehensive Sickle Cell Center

Primary Purpose

Anemia, Sickle Cell, Blood Disease, Cerebrovascular Accident

Status
Completed
Phase
Locations
Study Type
Observational
Intervention
Sponsored by
National Heart, Lung, and Blood Institute (NHLBI)
About
Eligibility
Locations
Outcomes
Full info

About this trial

This is an observational trial for Anemia, Sickle Cell

Eligibility Criteria

undefined - 100 Years (Child, Adult, Older Adult)MaleDoes not accept healthy volunteers

No eligibility criteria

Sites / Locations

    Outcomes

    Primary Outcome Measures

    Secondary Outcome Measures

    Full Information

    First Posted
    May 25, 2000
    Last Updated
    March 15, 2016
    Sponsor
    National Heart, Lung, and Blood Institute (NHLBI)
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    1. Study Identification

    Unique Protocol Identification Number
    NCT00005326
    Brief Title
    Cerebrovascular Involvement in Sickle Cell Disease - Comprehensive Sickle Cell Center
    Study Type
    Observational

    2. Study Status

    Record Verification Date
    December 2004
    Overall Recruitment Status
    Completed
    Study Start Date
    January 1988 (undefined)
    Primary Completion Date
    undefined (undefined)
    Study Completion Date
    March 2004 (Actual)

    3. Sponsor/Collaborators

    Name of the Sponsor
    National Heart, Lung, and Blood Institute (NHLBI)

    4. Oversight

    5. Study Description

    Brief Summary
    To continue studies on the two major neurological complications of sickle cell disease (SCD): namely, stroke and chronic encephalopathy.
    Detailed Description
    BACKGROUND: 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. DESIGN NARRATIVE: 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. The study completion date listed in this record was obtained from the "End Date" entered in the Protocol Registration and Results System (PRS) record.

    6. Conditions and Keywords

    Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
    Anemia, Sickle Cell, Blood Disease, Cerebrovascular Accident

    7. Study Design

    10. Eligibility

    Sex
    Male
    Maximum Age & Unit of Time
    100 Years
    Accepts Healthy Volunteers
    No
    Eligibility Criteria
    No eligibility criteria
    Overall Study Officials:
    First Name & Middle Initial & Last Name & Degree
    Darryl DeVivo
    Organizational Affiliation
    Columbia University

    12. IPD Sharing Statement

    Citations:
    PubMed Identifier
    8042222
    Citation
    Hurlet-Jensen AM, Prohovnik I, Pavlakis SG, Piomelli S. Effects of total hemoglobin and hemoglobin S concentration on cerebral blood flow during transfusion therapy to prevent stroke in sickle cell disease. Stroke. 1994 Aug;25(8):1688-92. doi: 10.1161/01.str.25.8.1688.
    Results Reference
    background
    PubMed Identifier
    7974538
    Citation
    Venketasubramanian N, Prohovnik I, Hurlet A, Mohr JP, Piomelli S. Middle cerebral artery velocity changes during transfusion in sickle cell anemia. Stroke. 1994 Nov;25(11):2153-8. doi: 10.1161/01.str.25.11.2153.
    Results Reference
    background
    PubMed Identifier
    8503800
    Citation
    Kugler S, Anderson B, Cross D, Sharif Z, Sano M, Haggerty R, Prohovnik I, Hurlet-Jensen A, Hilal S, Mohr JP, et al. Abnormal cranial magnetic resonance imaging scans in sickle-cell disease. Neurological correlates and clinical implications. Arch Neurol. 1993 Jun;50(6):629-35. doi: 10.1001/archneur.1993.00540060059019.
    Results Reference
    background
    PubMed Identifier
    1987669
    Citation
    Brass LM, Prohovnik I, Pavlakis SG, DeVivo DC, Piomelli S, Mohr JP. Middle cerebral artery blood velocity and cerebral blood flow in sickle cell disease. Stroke. 1991 Jan;22(1):27-30. doi: 10.1161/01.str.22.1.27.
    Results Reference
    background

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