Inflammatory Response to Hydroxyurea Therapy in Sickle Cell Disease
Sickle Cell Disease
About this trial
This is an observational trial for Sickle Cell Disease focused on measuring Sickle cell disease, hydroxyurea, inflammation
Eligibility Criteria
INCLUSION CRITERIA:
Homozygous SS sickle cell children, aged > 3 years, of sub-Saharian Africa extraction, in a steady-state of disease (free of any infectious or vaso-occlusive events for the 4 weeks prior to and 2 weeks after blood sampling, and transfusion-free for 4 months prior to blood sampling), taken no drug except penicillin-V, folate or iron supplementation, hydroxyurea, divided into three groups :
- children treated with hydroxyurea 20-25 mg/kg/day since at least 3 months with clinical efficacy on vaso-occlusive events
- untreated children with major vaso-occlusive events
- children > 5 year-old without a history of vaso-occlusive events Signed informed consent obtained from the subjects (if possible) and their parents
- Controls : heterozygous AS parents or siblings of the patients, and AA siblings or healthy African unrelated subjects, aged > 3 years, taken no drug on the day of blood sampling.
Signed informed consent obtained from the subjects (if possible) and their parents
EXCLUSION CRITERIA:
- Children in a acute-phase of the disease
- Parent's or patient's refusal
- Taking any drug except penicillin-V, folate or iron supplementation, hydroxyurea
- Un-healthy control or taking drug
Sites / Locations
- Hopital Louis Mourier
Arms of the Study
Arm 1
Arm 2
Homozygous SS sickle cell children
Homozygous SS children
Hydroxycarbamide, Hydroxyurea (drug): Homozygous SS sickle cell children, aged > 3 years, of sub-Saharian Africa extraction, in a steady-state of disease , taken no drug except penicillin-V, folate or iron supplementation, hydroxyurea, divided into three groups : children treated with hydroxyurea 20-25 mg/kg/day since at least 3 months with clinical efficacy on vaso-occlusive events untreated children with major vaso-occlusive events children > 5 year-old without a history of vaso-occlusive events Controls : heterozygous AS parents or siblings of the patients, and AA siblings or healthy African unrelated subjects, aged > 3 years, taken no drug on the day of blood sampling.
Hydroxycarbamide, Hydroxyurea (drug): Homozygous SS children, aged > 3 years, of sub-Saharian Africa extraction, in a steady-state of disease, taken no drug except penicillin-V, folate or iron supplementation, hydroxyurea, divided into three groups : children treated with hydroxyurea 20-25 mg/kg/day since at least 3 months with clinical efficacy on vaso-occlusive events untreated children with major vaso-occlusive events children > 5 year-old without a history of vaso-occlusive events Controls : heterozygous AS parents or siblings of the patients, and AA siblings or healthy African unrelated subjects, aged > 3 years, taken no drug on the day of blood sampling.