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| ID | Type | Description | Link |
|---|---|---|---|
| 2009-A00142-55 | Other Identifier | AFSSAPS |
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| Name | Class |
|---|---|
| URC-CIC Paris Descartes Necker Cochin | OTHER |
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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 clinical, biological and genetic markers predictive of severe complications notably acute splenic sequestration in SCD children. The present research project thus aims at analyzing in a forward-looking way the profile of severity by analysing clinical, biological and genetic characteristics in a multicentric cohort of 60 SCD children
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)
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Intervention | Other |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| blood samples and scintigraphy | Procedure | 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 |
| Measure | Description | Time Frame |
|---|---|---|
| Study of prognostic factors of acute splenic sequestration |
| after three years |
| Measure | Description | Time Frame |
|---|---|---|
| Study of hematology parameters |
| after three years |
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Inclusion criteria :
Exclusion criteria :
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| Name | Affiliation | Role |
|---|---|---|
| Valentine Brousse, MD, PhD | Assistance Publique - Hôpitaux de Paris | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Necker Hospital | Paris | 75015 | France |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 31391165 | Result | El Hoss S, Cochet S, Marin M, Lapoumeroulie C, Dussiot M, Bouazza N, Elie C, de Montalembert M, Arnaud C, Guitton C, Pellegrino B, Odievre MH, Moati F, Le Van Kim C, Aronovicz YC, El Nemer W, Brousse V. Insights into determinants of spleen injury in sickle cell anemia. Blood Adv. 2019 Aug 13;3(15):2328-2336. doi: 10.1182/bloodadvances.2019000106. | |
| 30132969 |
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| ID | Term |
|---|---|
| D000740 | Anemia |
| ID | Term |
|---|---|
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
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| ID | Term |
|---|---|
| D001800 | Blood Specimen Collection |
| ID | Term |
|---|---|
| D013048 | Specimen Handling |
| D019411 | Clinical Laboratory Techniques |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
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| Brousse V, El Hoss S, Bouazza N, Arnaud C, Bernaudin F, Pellegrino B, Guitton C, Odievre-Montanie MH, Mames D, Brouzes C, Picard V, Nguyen-Khoa T, Pereira C, Lapoumeroulie C, Pissard S, Gardner K, Menzel S, Le Van Kim C, Colin-Aronovicz Y, Buffet P, Mohandas N, Elie C, Maier-Redelsperger M, El Nemer W, de Montalembert M. Prognostic factors of disease severity in infants with sickle cell anemia: A comprehensive longitudinal cohort study. Am J Hematol. 2018 Nov;93(11):1411-1419. doi: 10.1002/ajh.25260. Epub 2018 Sep 21. |
| 26137540 | Result | Brousse V, Colin Y, Pereira C, Arnaud C, Odievre MH, Boutemy A, Guitton C, de Montalembert M, Lapoumeroulie C, Picot J, Le Van Kim C, El Nemer W. Erythroid Adhesion Molecules in Sickle Cell Anaemia Infants: Insights Into Early Pathophysiology. EBioMedicine. 2014 Dec 18;2(2):154-7. doi: 10.1016/j.ebiom.2014.12.006. eCollection 2015. |
| D011677 | Punctures |
| D013514 | Surgical Procedures, Operative |
| D008919 | Investigative Techniques |