Dense Red Blood Cells in Sickle Cell Children

Study Overview
=================
Brief Summary
-----------------
Quantitative and prognostic evaluation of dense red blood cells in sickle cell children: preliminary single center study from the Creteil pediatric cohort.

Detailed Description
-----------------
An association between red blood cell density and hemolytic parameters, and clinical manifestations has been demonstrated in adults with sickle cell anemia. This factor has not been studied in children. The identification of predictive biomarkers of disease severity, especially of specific pediatric complications (cerebral vasculopathy, splenic sequestration, Dactylitis) would be useful for optimal care of the children and early intensification Red blood cell density might be one of these prognostic factors.

Official Title
-----------------
Quantitative and Prognostic Evaluation of Dense Red Blood Cells in Sickle Cell Children: Single-center Study From the Creteil (France) Pediatric Cohort

Conditions
-----------------
Sickle Cell Disease

Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Age: 18 months-18 years Patient with sickle cell disease namely SS, or S / Beta0 or S / Beta + Patient regularly followed in the pediatric cohort of the CHI Creteil Patient Hospitalized for an annual check-up With or without intensification by Hydroxycarbamide patient who haven't been transfused within 3 months Whose parents have given their informed consent Patients insured to the French social scheme Exclusion Criteria: Sickle cell SC disease Having received an allogeneic bone marrow transplantation Under regular transfusion program Having received a transfusion within 3 months

Ages Eligible for Study
-----------------
Minimum Age: 18 Months
Maximum Age: 18 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Common arm<br>Children with sickle cell anemia will be included. Blood samples of all the included patients will be collected during a day-hospitalization for a planned chek-up. For all these patients the number of dense erythrocytes will be evaluated | |

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| number of dense red blood cells (DRBC) | Evaluation of the number of dense red blood cells in the blood of affected children | 1 day | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| biological profile | hemolytic parameters (LDH, bilirubin, Hemoglobin level) | 1 day | 
| Number of patients with velocities > 200 cm/sec on transcranial doppler | Cerebral vasculopathy (correlation with patients with abnormal velocities on transcranial doppler (TCD)(> 200 cm/sec) History of dactylitis History of ischemic lesions on magnetic resonance imaging (MRI) | 1 day | 
| Number of patients with history of acute splenic sequestration, |  | 1 day | 
| Number of patients with history of acute chest syndrome |  | 1 day | 
| Number of patients with History of dactylitis |  | 1 day | 
| Number of patients with history of abnormal transcranial doppler (TCD) (≥ 200 cm/sec) |  | 1 day | 
| Number of patients with history of ischemic lesions on magnetic resonance imaging (MRI) |  | 1 day | 
| Number of patients with hydroxycarbamide treatment | effect of hydroxycarbamide on the % DRBC | 1 day | 
| Number of dense red blood cells | number of dense red blood cells in the pediatric population with no known blood condition | 1 day | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
Erythrocytes, Sickle Cell Disease