Sickle Cell Anemia and Cerebral Microcirculation : Multimodal Exploration (DREAM²)

Determinants of Cerebral Oxygenation and Perfusion in SCA Children Based on Combined ASL MRI, NIRS and Hemorheological Investigation

The aim of this study is to evaluate determinants of cerebral oxygenation and perfusion at the microcirculatory level in children with sickle cell anemia (SCA) using combined novel investigational tools: Arterial Spin Labeling (ASL) perfusion MR (Magnetic Resonnance) imaging, brain Near Infra-Red Spectroscopy (NIRS) and red blood cell (RBC) rheological properties.

Study Overview

• Status: Completed
• Conditions: Sickle Cell Disease
• Intervention / Treatment: Procedure: Physical exams and blood analyzes

Detailed Description

The investigators hypothesize that brain perfusion and/or oxygenation modifications may be evidenced in SCA children who have no microarteriopathy and may correlate with hemorheological abnormalities and impaired vasomotion. A multimodal approach designed to study a. cerebral perfusion and oxygenation, b. flow motion properties and c. blood rheological parameters might help to describe the different processes involved in cerebral ischemia.

• Study Type: Observational
• Enrollment (Actual): 64

Contacts and Locations

Study Locations

• France
  • Créteil, France
    • Centre Hospitalier Intercommunal
  • Paris, France
    • Necker - Enfants Malades Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 6 years to 16 years (CHILD)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

Patient included will be patients suffering from Sickle Cell Anemia, and coming at the study center in the frame of their annual follow-up visit.

Description

Inclusion Criteria:

• SS or S-beta° genotype;
• age 6-16 years;
• steady state;
• normal TCD (Transcranial Doppler);
• parental study approval and written informed consent.

Exclusion Criteria:

• SC, Sbeta+, SD Punjab genotype
• history of overt stroke,
• intracranial or cervical arterial stenosis,
• abnormal TCD at the time of the study.

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Sickle cell disease patients; Physical exams and blood analyzes	Procedure: Physical exams and blood analyzes; Blood samples collection (for DNA, plasma and cells analyzes) ; Hemorheologic analyzes ; ASL sequence on MRI ; Near Infra Red Spectroscopy (NIRS) and associated cardiofrequency analyze.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Number of patients on which we detect default of cerebral perfusion (in order to correlate them with other clinical or biological parameters)	ASL sequence (duration 4 min) : Regional brain tissue perfusion (expressed in mL/min/100g of tissue) will be measured in different lobes in both hemispheres and in the cerebellum. Pattern of perfusion will be analysed and measured.	1.5 years

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Bifrontal cerebral hemoglobin oxygen saturation	Bifrontal cerebral hemoglobin oxygen saturation monitored by NIRS (15 min). Spectral analyses (Fourrier transform) will be used to analyze the brain microvascular oxygen variability and calculate the flowmotion and vasomotion activities.	1.5 years
Description of the global assessment of RBC deformability	The description of the global assessment of RBC deformability will be done via a global association of several biological parameters : RBC deformability at several shear stresses by ektacytometry, RBC aggregation properties by syllectometry and blood viscosity by cone-plate viscosimetry. Measurements will be made according to the international guidelines for standardisation in hemorheology and within 4/5 hrs of sampling	1.5 years

Collaborators and Investigators

• Sponsor: Imagine Institute
• Collaborators: Hopital Universitaire Robert-Debre
• Investigators: Principal Investigator: Valentine Boursse, Hôpital Necker-Enfants Malades; Study Director: Suzanne Verlhac, Centre Hospitalier Intercommunal de Créteil

Study record dates

Study Major Dates

• Study Start (ACTUAL): March 2, 2015
• Primary Completion (ACTUAL): July 11, 2017
• Study Completion (ACTUAL): July 11, 2017

Study Registration Dates

• First Submitted: September 5, 2016
• First Submitted That Met QC Criteria: September 16, 2016
• First Posted (ESTIMATE): September 21, 2016

Study Record Updates

• Last Update Posted (ACTUAL): November 14, 2018
• Last Update Submitted That Met QC Criteria: November 12, 2018
• Last Verified: November 1, 2018

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Hematologic Diseases; Genetic Diseases, Inborn; Anemia; Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Hemoglobinopathies; Anemia, Sickle Cell
• Other Study ID Numbers: IMIS2014-04

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO