Study of Biological Markers in Children With Sickle Cell Disease

Prospective Clinical Study on Early Inflammatory, Cell Adhesion and Hemostatic Plasmatic Markers of Endothelial Dysfunction in Children With Sickle Cell Disease (SCD)

Sickle cell disease is associated with significant morbi-mortality hence the interest in an early and targeted care. At present, there is no plasmatic marker able to identify infants at higher risk of developping severe complications later in life. However, recent studies have demonstrated a correlation between certain complications of the disease and biomarkers of the endothelial dysfunction characterizing it. Investigators prospectively followed a cohort of children diagnosed with SCD through the universal neonatal screening using inflammatory and haemostatic plasmatic markers to study their annual evolution. Investigators then will evaluate potential associations between these biological markers and the occurrence of SCD related complications. A secondary objective of this study is to evaluate the repercussions of therapeutic intervention on these markers. .

Sickle cell disease, children, thrombin generation, inflammation, cytokines, adhesion molecules, plasmatic markers, coagulation

Blood sampling before any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Plasmatic levels of IL-6 before the introduction of any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Plasmatic levels of IL-1ß before the introduction of any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Plasmatic levels of IL-8 before the introduction of any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Plasmatic levels of IL-10 before the introduction of any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Plasmatic levels of IL-12 before the introduction of any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Plasmatic levels of TNF alpha before the introduction of any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Plasmatic levels of ICAM-1 before the introduction of any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Plasmatic levels of VCAM-1 before the introduction of any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Measurement of plasmatic levels of VCAM-1 (pg/mL) by flow cytometric assay after in vitro stimulation with LPS

Plasmatic levels of E-selectine before the introduction of any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Plasmatic levels of P-selectine before the introduction of any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Plasmatic levels of VEGF before the introduction of any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Measurement of lag time (minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Measurement of lag time (minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Measurement of lag time (minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Measurement of lag time (minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Measurement of lag time (minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Lag time parameter in thrombin generation assay before the introduction of any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Measurement of lag time (minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Measurement of peak height (nM) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Measurement of peak height (nM) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Measurement of peak height (nM) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Measurement of peak height (nM) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Measurement of peak height (nM) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Peak height parameter in thrombin generation assay before the introduction of any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Measurement of peak height (nM) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Measurement of time to peak (minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method

Measurement of time to peak (minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method

Measurement of time to peak (minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method

Measurement of time to peak (minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method

Measurement of time to peak (minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method

Time to peak parameter in thrombin generation assay before the introduction of any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Measurement of time to peak (minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method

Measurement of endogenous thrombin potential (nM x minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Measurement of endogenous thrombin potential (nM x minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Measurement of endogenous thrombin potential (nM x minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Measurement of endogenous thrombin potential (nM x minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Measurement of endogenous thrombin potential (nM x minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Endogenous thrombin potential parameter in thrombin generation assay before the introduction of any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Measurement of endogenous thrombin potential (nM x minutes) parameter of thrombin generation in platelet poor plasma using the Calibrated Automated Thrombogram (CAT®) method.

Plasmatic levels of Factor VIII before the introduction of any new sickle cell disease treatment as determined by a physician according to the standard of care to which the hospital adheres

Inclusion Criteria: Patient aged less than 6 months Sickle cell syndrome SS, Sβthal or SC confirmed by hemoglobin electrophoresis Subjects legal representatives must have signed an informed consent document indicating that they understand the purpose of and procedures required for the study and are willing to let participate their child in the study Exclusion Criteria: Congenital abnormality other than sickle cell disease except for a glucose-6-phosphate-deshydrogenase Prematurity Initiation of the following therapies before enrollment: chronic transfusion regimen or bone marrow transplantation

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