Prognostic Factors of Acute Splenic Sequestration (SSADREPA)
Acute splenic sequestration is a frequent and life threatening complication occurring in approximately 10 % of homozygous children. Maximal incidence is between 6 and 18 months.
The investigators formulate the hypothesis that there are prognostic factors of acute splenic sequestration in SCD children . Thus the present research project aims at identifying in a forward-looking way prognostic factors of acute splenic sequestration in SCD children by analysing clinical, biological and genetic characteristics in a multicentric cohort of 150 SCD children.
|Study Design:||Intervention Model: Single Group Assignment
Masking: Open Label
Primary Purpose: Prevention
|Official Title:||Study of Prognostic Factors of Acute Splenic Sequestration in a Cohort of Sickle Cell Disease (SCD) Children Diagnosed at Birth|
- Study of prognostic factors of acute splenic sequestration [ Time Frame: after three years ] [ Designated as safety issue: No ]
- Complete blood count, reticulocyte count, haemoglobin level, VGM, TGMH, CCMH, hematocrit, % foetal haemoglobin (HbF), red blood cell densities (2 mL)
- Deformability of red blood cells
- Expression study of the cell surface molecule: Phosphatidyl serine, CD 36, READ B-CAM, CD 47, ICAM 4, VLA 4. (Cellulotheque = red blood cells frozen in cryopreservative conditions)
- Total LDH, Bilirubine (stigmas of hemolysis)
- Genetic Profil ( DNAtheque)
- Study of hematology parameters [ Time Frame: after three years ] [ Designated as safety issue: No ]
- Percentage of Howell Jolly's bodies (0,1 mL)
- Percentage of pitted cells (0,5 mL fixed in 4 %)formaldehyde
- Splenic volume, semi-quantitative measure in scintigraphy
- Antibody titers in answer to the antipneumococcic vaccination ( serotheque)
|Study Start Date:||August 2010|
|Estimated Study Completion Date:||November 2015|
|Estimated Primary Completion Date:||April 2015 (Final data collection date for primary outcome measure)|
Procedure: blood samples and scintigraphy
at 3 months, 6 months, 12 months, 18 months and 24 months will allow a clinical evaluation and an additional sampling of blood of 5 mL at each visit Scintigraphy at 6 months and 18 months
Other Name: blood samples and scintigraphy
A prospective multicentric analysis will be conducted in a cohort of 150 SS or S ß ° children diagnosed at birth, included at 3 -5 months and followed up to the age of 24 months.
Five visits, superimposed to the usual follow-up of SCD children, (Recommendations of the High Authority of Health) at 3 months, 6 months, 12 months, 18 months and 24 months will allow a clinical evaluation and an additional sampling of blood (5 mL) at each visit.
The samples will allow 1.analysis of the red blood cell phenotype (adhesion and deformability) and densities 2. the genetic profile 3.to establish a cell bank, a sera bank and a DNA bank, Spleen function in the cohort will be estimated by spleen scintigraphy, coupled with blood markers (pitted cells, Howell-Jolly bodies counts)
|Paris, France, 75015|
|Principal Investigator:||Valentine Brousse, MD, PhD||Assistance Publique - Hôpitaux de Paris|