Establishment of Functional MRI Imaging Parameters for Use in the Evaluation of Sickle Cell Disease

Patients with sickle cell anemia (SCA) are at an increased risk for damage to brain tissue due to their disease. The investigators are interested in how blood flow and cerebral inflammation are different in SCA patients and how that affects brain tissue- the investigators will use a relatively new set of dynamic MRI techniques to evaluate these parameters. The investigators will image participants with both SCA and matched controls with non-invasive MRI.

Study Overview

• Status: Terminated
• Conditions: Sickle Cell Anemia

Detailed Description

Patients with sickle cell anemia (SCA) are at increased risk for episodes of stroke, both overt clinically evident and subclinical lesions only seen on imaging, which have associated morbidity and mortality. In addition, SCA patients demonstrate relatively poorer cognitive performance compared to their peers without SCA that is believed to be related to the episodes of stroke, but may be present even in their absence.

This study is designed to explore potential risk factors in patients with SCA that will identify predictors of cerebral damage that may also be modifiable. Elevated blood flow in cerebral arteries and increased inflammation are believed to be related to both ischemic lesions and cognitive findings but have not yet been clearly proven. We aim to use new MRI techniques which target cerebral blood flow and inflammation to identify differences in SCA patients and peers and follow this with an initial exploration of the association between these pathologic findings and cognitive deficits.

The investigator does not assign specific interventions to the subjects of the study.

• Study Type: Observational
• Enrollment (Actual): 26

Contacts and Locations

Study Locations

• United States
  • New York
    • Bronx, New York, United States, 10461
      • Albert Einstein College of Medicine
    • Bronx, New York, United States, 10461
      • Montefiore Medical Center (Einstein)

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 55 years (Adult)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

18-55 year old male and female patients with sickle cell anemia who are asymptomatic of previous CNS disease

Description

Inclusion Criteria:

• Patient with HbSS/HbSB0,
• age between 18 and 55 years or age/gender/race/education matched peer

Exclusion Criteria:

• Previous history of a stroke/transient ischemic attack,
• neurosurgery,
• head trauma,
• seizures,
• pulmonary embolism,
• deep-vein thrombosis,
• bleeding/clotting disorders,
• current or previous use of anticoagulation medications

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort
SCA Patients; Asymptomatic sickle cell anemia patients will undergo imaging/neurocognitive testing and be compared to controls
Control Patients; Asymptomatic sickle cell anemia patients will undergo imaging/neurocognitive testing and be compared to controls

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Cerebral blood flow (CBF) measured by continuous arterial spin labeling (CASL) MRI	CBF will be measured by continuous arterial spin labeling (CASL) MRI	Day 1

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Cogstate Testing	Exploration of the association between CBF values and cognitive performance deficits	Day 1
Mean diffusivity/Fractional anisotropy (MD/FA) evaluated by diffusion tensor imaging (DTI) MRI measurements	MD/FA will be evaluated by diffusion tensor imaging (DTI) MRI measurements	Day 1

Collaborators and Investigators

• Sponsor: Albert Einstein College of Medicine
• Investigators: Principal Investigator: Henny H Billett, MD, Montefiore Medical Center

Study record dates

Study Major Dates

• Study Start: November 1, 2014
• Primary Completion (Actual): May 4, 2020
• Study Completion (Actual): May 4, 2020

Study Registration Dates

• First Submitted: April 15, 2015
• First Submitted That Met QC Criteria: May 12, 2015
• First Posted (Estimate): May 15, 2015

Study Record Updates

• Last Update Posted (Actual): December 14, 2020
• Last Update Submitted That Met QC Criteria: December 11, 2020
• Last Verified: December 1, 2020

More Information

Terms related to this study

• Keywords: Inflammation; Cognition; Blood Flow
• Additional Relevant MeSH Terms: Hematologic Diseases; Genetic Diseases, Inborn; Anemia; Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Hemoglobinopathies; Anemia, Sickle Cell
• Other Study ID Numbers: 2014-3574

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: YES
• IPD Plan Description: Data will be made available after manuscript publication to those that request it
• IPD Sharing Time Frame: Time frame of study has ended. Only preliminary data will be available - no end date
• IPD Sharing Access Criteria: Other investigators
• IPD Sharing Supporting Information Type: STUDY_PROTOCOL; ICF; CSR