NEUROPSYCHOLOGICAL DYSFUNCTION/NEUROIMAGING ABNORMALITIES IN ADULTS WITH SCD

这个子项目是许多利用 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。 这项研究的目的是确定患有镰状细胞病的成年人的记忆和注意力问题的程度。将入组年龄为21-55岁的神经系统完整的成人患者。这是没有经历过中风或其他脑损伤的成年人。本研究还将评价这些事件或状况与MRI异常之间的联系。这些发现将与具有类似背景的健康人进行比较。 主要目的是确定神经系统无症状的镰状细胞病成人患者神经认知功能障碍的程度。次要目的是确定神经认知功能障碍与影像学异常之间的相关性。 这是一项横断面调查试验，旨在比较成人神经功能正常的Hb SS/SBo患者与匹配的同龄对照组的神经心理功能。一项初步干预试验将从初步研究中随机抽取一部分患者，接受长期输血方案或单独接受标准治疗，为期6个月。WAIS III PIQ评分与标准数据（85）的标准差大于1的受试者子集将有资格在初步输血试验中随机分组。

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. The purpose of this research study is to determine the extent of memory and attention problems in adults with sickle cell disease. Neurologically intact adult patient's ages 21-55 years will be enrolled. That is adults who have not experienced a stroke or other brain damage. The study will also evaluate the link between these events or conditions and abnormal MRI. These findings will be compared to healthy people with similar backgrounds. The primary objective is to determine the extent of neurocognitive dysfunction in neurologically asymptomatic adult patients with sickle cell disease. The secondary objective to determine the association between neurocognitive dysfunction and imaging abnormalities. This is a cross-sectional investigation trial designed to compare neuropsychological function of adult neurologically normal Hb SS/SBo patients with matched-peer controls. A pilot intervention trial will randomize a subset of patients from the primary study to undergo either a chronic transfusion regimen or receive standard care alone, for six months. A subset of these subjects with WAIS III PIQ score greater than one standard deviation from the normative data (85) will be eligible for randomization in the pilot transfusion trial.