Dense Red Blood Cells in Sickle Cell Children

Centre Hospitalier Intercommunal Creteil82 enrolled

Overview

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.

Study Type

OBSERVATIONAL

Enrollment

Conditions

Sickle Cell Disease

Eligibility

Sex: ALLMin age: 18 MonthsMax age: 18 Years

Medical Language ↔ Plain English

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

Locations (1)

CHI Creteil

Créteil, France

Outcomes

Primary Outcomes

number of dense red blood cells (DRBC)

Evaluation of the number of dense red blood cells in the blood of affected children

Time frame: 1 day

Secondary Outcomes

biological profile

hemolytic parameters (LDH, bilirubin, Hemoglobin level)

Time frame: 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)

Time frame: 1 day

Number of patients with history of acute splenic sequestration,

Time frame: 1 day

Number of patients with history of acute chest syndrome

Time frame: 1 day

Number of patients with History of dactylitis

Time frame: 1 day

Number of patients with history of abnormal transcranial doppler (TCD) (≥ 200 cm/sec)

Time frame: 1 day

Number of patients with history of ischemic lesions on magnetic resonance imaging (MRI)

Time frame: 1 day

Number of patients with hydroxycarbamide treatment

effect of hydroxycarbamide on the % DRBC

Time frame: 1 day

Data from ClinicalTrials.gov

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