Sickle Cell Disease
Conditions
Keywords
Erythrocytes, Sickle Cell Disease
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.
Interventions
None listed
Sponsors
Centre Hospitalier Intercommunal Creteil
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Months to 18 Years
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| number of dense red blood cells (DRBC) | 1 day | Evaluation of the number of dense red blood cells in the blood of affected children |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Number of patients with velocities > 200 cm/sec on transcranial doppler | 1 day | 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) |
| 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 | — |
| biological profile | 1 day | hemolytic parameters (LDH, bilirubin, Hemoglobin level) |
| Number of patients with history of ischemic lesions on magnetic resonance imaging (MRI) | 1 day | — |
| Number of patients with hydroxycarbamide treatment | 1 day | effect of hydroxycarbamide on the % DRBC |
| Number of dense red blood cells | 1 day | number of dense red blood cells in the pediatric population with no known blood condition |
| Number of patients with history of abnormal transcranial doppler (TCD) (≥ 200 cm/sec) | 1 day | — |
Countries
France
Outcome results
None listed