Study of Prognostic Factors of Acute Splenic Sequestration in a Cohort of Sickle Cell Disease (SCD) Children Diagnosed at Birth
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 58
- 试验地点
- 2
- 主要终点
- Study of prognostic factors of acute splenic sequestration
研究概览
简要总结
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
详细描述
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)
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Prevention
- 盲法
- None
入排标准
- 年龄范围
- 3 Months 至 6 Months（Child）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- 未提供
排除标准
- 未提供
研究组 & 干预措施
Intervention
干预措施: blood samples and scintigraphy (Procedure)
结局指标
主要结局
Study of prognostic factors of acute splenic sequestration
时间窗: after three years
* Complete blood count, reticulocyte count, haemoglobin level, VGM, TGMH, CCMH, hematocrit, % foetal haemoglobin (HbF), red blood cell densities (2 mL) * Deformability of red blood cells * Expression study of the cell surface molecule: Phosphatidyl serine, CD 36, READ B-CAM, CD 47, ICAM 4, VLA 4. (Cellulotheque = red blood cells frozen in cryopreservative conditions) * Total LDH, Bilirubine (stigmas of hemolysis) * Genetic Profil ( DNAtheque)
次要结局
- Study of hematology parameters(after three years)