NEUROLOGICAL AND DEVELOPMENTAL OUTCOME IN TOF

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 目的1.比较TOF组和对照组在学业成绩、认知和行为方面的差异。 假设：与正常对照组相比，没有22q11微缺失的TOF修复儿童将表现出学习、认知和行为方面的缺陷。 主要结果将是韦克斯勒个人成就测试(WIAT)的综合总分。根据WIAT的评估，学业成功代表了包括智力、神经心理优势和弱点以及心理健康在内的因素的结果。 因此，次级结果将包括以下内容：(1)能力，通过韦氏儿童智力量表第三版(WISC-III)上的全量、言语和操作智商来衡量；(2)学习障碍，定义为儿童在特定学术领域的能力和成就之间的显著差异；(3)特定神经心理领域的表现，包括语言、记忆、注意力、执行功能和视觉-空间/运动整合；以及(4)精神健康，包括生活质量和全球情绪功能，以及具体的精神诊断，包括情绪、焦虑、注意力缺陷/破坏性行为和精神障碍。 目的2.比较TOF组和对照组在脑结构测量方面的差异。 假设：没有22q11缺失的TOF修复的儿童在结构MRI上的发现与匹配的对照组不同。 使用定量体积磁共振成像和分割的主要结果将是总脑组织体积。次要结果将是灰质(总灰质、皮质和皮质下灰质)、白质(有髓和无髓白质)和脑脊液的体积。 使用扩散张量MRI成像的主要结果变量将是局部脑白质微观结构，用相对各向异性(RA)来衡量。

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. AIM 1. To compare TOF and control groups on academic achievement, cognition and behavior. Hypothesis: Children with repaired TOF who do not possess 22q11 microdeletion will demonstrate academic, cognitive, and behavioral deficiencies relative to normal matched controls. The primary outcome will be Total Composite Score on the Wechsler Individual Achievement Test (WIAT). Academic success, as assessed by the WIAT, represents the outcome of factors including intelligence, neuropsychological strengths and weaknesses, and mental health. Therefore, secondary outcomes will include the following: (1) ability, measured by the Full-Scale, Verbal, and Performance IQ on the Wechsler Intelligence Scale for Children-Third Edition (WISC-III); (2) learning disability, defined as a significant discrepancy between a child's ability and achievement within particular academic domains; (3) performance in specific neuropsychologic domains, including language, memory, attention, executive functions, and visual-spatial/motor integration; and (4) mental health, including quality-of-life and global emotional functioning, as well as specific psychiatric diagnoses including mood, anxiety, attention-deficit/disruptive behavior, and psychotic disorders. AIM 2. To compare TOF and control groups on measures of brain structure. Hypothesis: Children with repaired TOF who do not possess the 22q11 deletion will have findings on structural MRI that differ from matched controls. The primary outcome using quantitative volumetric MRI imaging and segmentation will be total brain tissue volume. Secondary outcomes will be volumes of gray matter (total, cortical, and subcortical gray matter), white matter (myelinated and unmyelinated white matter), and cerebrospinal fluid. The primary outcome variable using diffusion tensor MRI imaging will be regional white matter microstructure, as measured by relative anisotropy (RA).