# Neuroimaging, Neurocognitive, and Plasma Protein Markers of Brain Injury in Young Children with Sickle Cell Disease

> **NIH NIH R03** · HUGO W. MOSER RES INST KENNEDY KRIEGER · 2022 · $81,000

## Abstract

Project Summary/Abstract
 Sickle cell disease (SCD) is an inherited blood disorder with several neurological and developmental
complications. Young children with SCD between 2 and 5 years of age, in particular, have an increased risk for
ischemic stroke and silent cerebral infarctions (SCI). SCI are hyperintense T2 brain magnetic resonance
imaging (MRI) lesions with no accompanying acute neurological symptoms but with associated impaired
cognition and increased risk of future progressive or additional stroke/SCI. Neuroimaging and neurocognitive
profiles associated with SCI are well established in school-aged and adult SCD populations. This information is
largely unknown in young children under 6 years of age with SCD due to risk of sedation/anesthesia with MRI.
Our preliminary studies have shown that behavioral training can yield high quality neuroimaging data without
sedation in 3 to 4 years old children with SCD through a pilot study.
 The long term goal of this application is to develop a diagnostic battery using a combination of
unsedated neuroimaging measures, neurocognitive testing, and plasma brain injury protein levels to identify
young children with SCD at risk for SCI. Through a prospective longitudinal case-control study, we will collect
medical history and clinical data and perform neurological examination and blood draws annually in 100
children with SCD from study entry to study exit. Participants will also undergo initial neuroimaging battery at 3
to 4 years of age with repeat neuroimaging at study exit as well as longitudinal neurocognitive testing at study
entry, with initial neuroimaging, and with exit neuroimaging. This multi-disciplinary and multi-modality proposal
has three Aims. Aim 1 will identify the associated neuroimaging and neurocognitive findings in 3 to 4 year-old
children with SCD and SCI on initial MRI by comparing their results to children with SCD without SCI. Aim 2
will analyze which neuroimaging measures, neurocognitive test scores, and plasma protein levels may predict
future SCI risk, comparing longitudinal data from children with and without SCI on exit MRI. Aim 3 will explore
differences in the neuroimaging measures in children on different disease-modifying treatments (chronic
transfusion therapy, hydroxyurea) as determined by their clinical providers, through a secondary data analysis.
The proposed work will determine which neuroimaging measures and neurocognitive testing may predict SCI,
leading to a multi-site study to validate these findings in a larger regionally heterogenous SCD population. The
study PI, a neurodevelopmental physician, is an NHLBI K23 principal investigator well suited to expand her
existing study cohort with support from a team of co-investigators, including a senior behavioral psychologist
and neuroimaging physicist, as well as existing collaborators and staff.

## Key facts

- **NIH application ID:** 10470928
- **Project number:** 5R03HL158636-02
- **Recipient organization:** HUGO W. MOSER RES INST KENNEDY KRIEGER
- **Principal Investigator:** EBONI I LANCE
- **Activity code:** R03 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2022
- **Award amount:** $81,000
- **Award type:** 5
- **Project period:** 2021-09-01 → 2024-08-31

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10470928

## Citation

> US National Institutes of Health, RePORTER application 10470928, Neuroimaging, Neurocognitive, and Plasma Protein Markers of Brain Injury in Young Children with Sickle Cell Disease (5R03HL158636-02). Retrieved via AI Analytics 2026-05-23 from https://api.ai-analytics.org/grant/nih/10470928. Licensed CC0.

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