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The recruiting centre was no longer presenting new patients for inclusion
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Quantitative and prognostic evaluation of dense red blood cells in sickle cell children: preliminary single center study from the Creteil pediatric cohort.
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Common arm | 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 |
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| 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 |
| 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) |
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Inclusion Criteria:
Exclusion Criteria:
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patients with sickle cell disease followed regularly in the CHI creteil hospital aged from 18 months to 18 years old.
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| Name | Affiliation | Role |
|---|---|---|
| Corinne Pondarre, MD PhD | CHI Créteil | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| CHI Creteil | Créteil | 94000 | France |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 10631276 | Background | Miller ST, Sleeper LA, Pegelow CH, Enos LE, Wang WC, Weiner SJ, Wethers DL, Smith J, Kinney TR. Prediction of adverse outcomes in children with sickle cell disease. N Engl J Med. 2000 Jan 13;342(2):83-9. doi: 10.1056/NEJM200001133420203. | |
| 24117340 | Background | Benkerrou M, Alberti C, Couque N, Haouari Z, Ba A, Missud F, Boizeau P, Holvoet L, Ithier G, Elion J, Baruchel A, Ducrocq R. Impact of glucose-6-phosphate dehydrogenase deficiency on sickle cell anaemia expression in infancy and early childhood: a prospective study. Br J Haematol. 2013 Dec;163(5):646-54. doi: 10.1111/bjh.12590. Epub 2013 Oct 10. |
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| ID | Term |
|---|---|
| D000755 | Anemia, Sickle Cell |
| ID | Term |
|---|---|
| D000745 | Anemia, Hemolytic, Congenital |
| D000743 | Anemia, Hemolytic |
| D000740 | Anemia |
| D006402 | Hematologic Diseases |
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blood samples to study dense red blood cells
| 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 |
| 16940426 | Background | Quinn CT, Shull EP, Ahmad N, Lee NJ, Rogers ZR, Buchanan GR. Prognostic significance of early vaso-occlusive complications in children with sickle cell anemia. Blood. 2007 Jan 1;109(1):40-5. doi: 10.1182/blood-2006-02-005082. Epub 2006 Aug 29. |
| 21068435 | Background | Bernaudin F, Verlhac S, Arnaud C, Kamdem A, Chevret S, Hau I, Coic L, Leveille E, Lemarchand E, Lesprit E, Abadie I, Medejel N, Madhi F, Lemerle S, Biscardi S, Bardakdjian J, Galacteros F, Torres M, Kuentz M, Ferry C, Socie G, Reinert P, Delacourt C. Impact of early transcranial Doppler screening and intensive therapy on cerebral vasculopathy outcome in a newborn sickle cell anemia cohort. Blood. 2011 Jan 27;117(4):1130-40; quiz 1436. doi: 10.1182/blood-2010-06-293514. Epub 2010 Nov 10. |
| 22919030 | Background | Bartolucci P, Brugnara C, Teixeira-Pinto A, Pissard S, Moradkhani K, Jouault H, Galacteros F. Erythrocyte density in sickle cell syndromes is associated with specific clinical manifestations and hemolysis. Blood. 2012 Oct 11;120(15):3136-41. doi: 10.1182/blood-2012-04-424184. Epub 2012 Aug 23. |
| 22915643 | Background | Thornburg CD, Files BA, Luo Z, Miller ST, Kalpatthi R, Iyer R, Seaman P, Lebensburger J, Alvarez O, Thompson B, Ware RE, Wang WC; BABY HUG Investigators. Impact of hydroxyurea on clinical events in the BABY HUG trial. Blood. 2012 Nov 22;120(22):4304-10; quiz 4448. doi: 10.1182/blood-2012-03-419879. Epub 2012 Aug 22. |
| 25203083 | Background | Yawn BP, Buchanan GR, Afenyi-Annan AN, Ballas SK, Hassell KL, James AH, Jordan L, Lanzkron SM, Lottenberg R, Savage WJ, Tanabe PJ, Ware RE, Murad MH, Goldsmith JC, Ortiz E, Fulwood R, Horton A, John-Sowah J. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA. 2014 Sep 10;312(10):1033-48. doi: 10.1001/jama.2014.10517. |
| D006425 |
| Hemic and Lymphatic Diseases |
| D006453 | Hemoglobinopathies |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |