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）任何患有脊柱裂的年轻人失业或未充分就业，并且没有准备好迎接生活的挑战。这种成就不足在很大程度上可以追溯到他们早期的学术神经认知因素，这些因素导致了这些学习困难，但人们对这些因素知之甚少。同时，随着SB产前手术修复的增加，阐明其对神经认知结果的影响的兴趣也在增加。由于产前修复后症状性脊髓栓系可能导致神经功能下降，因此对这些儿童进行长期神经发育监测的重要性进一步得到强调。此外，神经管缺陷的患病率在西班牙裔中一直是最高的，并且在患有慢性健康状况（包括SB）的儿童中存在严重的种族和民族卫生保健差异。此外，SB患者肾脏疾病的患病率和严重程度尚未在反映当今美国人口统计数据的全国样本中进行研究。在美国，有超过7万人患有这种最常见的永久性先天缺陷。M挑战;然而，本研究的具体目的是探讨产前修复对脊髓栓系综合征发生率和神经认知发育的影响，并探讨种族和民族在使用预防性泌尿外科护理和获得纠正性泌尿外科干预方面的作用。对于我们的方法，我们将使用标准化的国家脊柱裂患者注册表和SB电子注册表来收集患者的数据，包括那些在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就诊的患者。此外，我们打算努力扩大和改进目前通过这个注册表数据收集工具获得的变量。我们预计这些结果将带来重要的结果，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，并将进一步我们对种族/民族作为不良泌尿系统结果的危险因素的理解。我们的长期目标是在临床实践中提出改变，以制定全民水平的脊柱裂护理管理国家标准，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.