Comp B-Texas Children's Hospital and Baylor Coll of Med Spina Bifida Registry

描述（由申请人提供）：脊柱裂（SB）任何年轻的成年人与SB失业或就业不足，准备不足，以满足生活的挑战。这种学习成绩低下的现象可以追溯到他们早期的学业，导致这些学习困难的神经认知因素知之甚少。同时，随着SB产前手术修复的扩散，阐明其对神经认知结果的影响的兴趣增加了。这些儿童的长期神经发育监测的重要性进一步强调了可能的神经功能下降，由于症状性脊髓栓系后产前修复。此外，神经管缺陷的患病率一直被证明是最高的拉美裔，严重的种族和民族的健康护理的差异存在于儿童慢性健康状况，包括SB。此外，患病率和严重程度的肾脏疾病的个人SB尚未进行研究，在一个国家的样本，反映当今美国的人口统计。在美国，有超过7万人患有最常见的永久性致残性出生缺陷。M挑战;然而，这项研究的具体目的是探讨产前修复对脊髓栓系综合征和神经认知发育的影响，并探讨种族和民族在预防性泌尿外科护理和泌尿外科矫正手术中的作用。对于我们的方法，我们将使用标准化的国家脊柱裂患者登记表和SB电子登记表来收集患者的数据，包括那些参加大型和种族多样化的多学科德克萨斯儿童医院/贝勒医学院脊柱裂诊所的患者。此外，我们打算努力扩大和完善目前通过这一登记册数据收集工具获得的变量。我们预计这些结果将导致显著的结果，因为它们将指导未来关于产前SB修复和长期神经和神经认知结果的影响的研究，并将进一步加深我们对种族/种族作为不良泌尿系统结果的风险因素的理解。我们的长期目标是提出临床实践的改变，以制定SB护理管理的人群水平国家标准，并最终改善脊柱裂患者的生活。

DESCRIPTION (provided by applicant): Spina bifida (SB) any young adults with SB are unemployed or underemployed and ill-prepared to meet life's challenges. Much of this under-attainment can be traced back to their early academic eurocognitive factors contributing to these learning difficulties are poorly understood. Concurrently, with the proliferation of SB prenatal surgical repair, interest in elucidating its impact on neurocognitive outcomes has increased. The importance of long-term neurodevelopmental surveillance of these children is further underscored by the possible decline in neurologic function due to symptomatic cord tethering post prenatal repair. Moreover, the prevalence of neural tube defects has been shown consistently to be highest among Hispanics, and serious racial and ethnic health care disparities exist among children with chronic health conditions including SB. Further, the prevalence and severity of renal disease among individuals with SB have not been studied in a national sample reflecting present-day US demographics. More than 70,000 people in the United States live with, the most common permanently disabling birth defect. M challenges; yet, n The specific aims of the proposed study are to explore the role that prenatal repair may have on rates of tethered cord syndrome and neurocognitive development, and to explore the role that race and ethnicity play in the use of preventive urological care and in access to corrective urologic surgical interventions. For our methods, we will use the standardized National Spina Bifida Patient Registry forms and the SB electronic registry to collect data from patients, including those attending the large and racially diverse multi-disciplinary Texas Children's Hospital/Baylor College of Medicine Spina Bifida Clinic. Moreover, we intend to work towards the expansion and refinement of the variables currently obtained through this registry data collection instrument. We anticipate these results will lead to significant outcomes since they will guide future studies on the effects of prenatal SB repair and long-term neurologic and neurocognitive outcomes, and will further our understanding of race/ethnicity as a risk factor for adverse urologic outcomes. Our long-range goal is to propose change in clinical practice in order to develop population-level national standards for SB care management, and ultimately improve the lives of people with spina bifida.