NEURODEVELOPMENTAL DEFICITS AMONG INFANTS AND TODDLERS WITH SICKLE CELL DISEASE

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目和 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。所列机构为 研究中心，而研究中心不一定是研究者所在的机构。 患有镰状细胞病（SCD）的幼儿基于疾病特异性和环境风险因素（例如较低的社会经济地位）而处于神经发育缺陷的增加的风险中（Schatz等人，2002;白色等人，2006年）。尽管存在这些风险，但对患有SCD的幼儿（小于4岁）的认知和发育缺陷的患病率和性质的认识不足。我们正在进行一项为期4年的混合横断面纵向研究，研究4岁以下SCD儿童的早期神经发育状况。 本研究的目的是（1）在控制SES的同时，描述婴儿和幼儿发育缺陷的患病率和性质;（2）检查镰状细胞表型、血液学严重程度和父母特征对发育结局的调节作用;（3）通过在SCD诊所的每次患者访视期间包括关于正常发育和行为的常规父母教育来改善患者结局。为了实现这些主要目标，研究有两个组成部分：神经发育评估组件和家长教育。在GCRC测试室完成的神经发育评估部分将确定特定年龄段（9、15、21、30和40个月）SCD幼儿的发育缺陷患病率。我们计划在5个年龄段中的每个年龄段至少进行45次评估（225次评估）。家长教育部分是一种基于SCD诊所的重复性干预措施，旨在影响患者的治疗结果。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Young children with sickle cell disease (SCD) are at increased risk for neurodevelopmental deficits based on both disease specific and environmental risk factors such as lower socioeconomic status (Schatz et al., 2002; White et al., 2006). Despite these risks, there is insufficient understanding of the prevalence and nature of cognitive and developmental deficits in young children (less than 4 years of age) who have SCD. We are conducting a 4-year mixed cross-sectional longitudinal study of early neurodevelopmental status in children younger than 4 years with SCD. The study aims are to (1) characterize the prevalence and nature of the developmental deficits in infants and toddlers while controlling for SES; (2) examine the moderating effects of sickle cell phenotype, hematologic severity, and parent characteristics on developmental outcome; (3) improve patient outcomes by including routine parent education about normal development and behavior during each patient visit at the SCD Clinic. To accomplish these primary aims the study has two components: Neurodevelopmental Evaluation Component and Parent Education. The Neurodevelopmental Evaluation component, completed in the GCRC testing room, will determine the prevalence of developmental deficits among young children with SCD at specific age levels (9, 15, 21, 30, and 40 months). We plan a minimum of 45 assessments at each of the 5 age levels (225 assessments). The Parent Education component is a recurrent, SCD clinic-based intervention intended to impact patient outcomes.