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

描述(申请人提供)：脊柱裂(SB)任何患有SB的年轻人都是失业或未充分就业的，没有准备好迎接生活中的挑战。这种成绩低下的现象在很大程度上可以追溯到他们早期的学术活动，导致这些学习困难的欧洲认知因素鲜为人知。同时，随着SB产前手术修复的激增，人们对阐明其对神经认知结果的影响的兴趣也增加了。这些儿童的长期神经发育监测的重要性因产前修复后症状性脊髓拴系可能导致的神经功能下降而进一步突出。此外，神经管缺陷的发生率一直被证明是拉美裔中最高的，在包括SB在内的患有慢性健康状况的儿童中存在严重的种族和民族保健差异。此外，还没有在反映当今美国人口统计学的全国样本中对SB患者的肾脏疾病的患病率和严重性进行研究。在美国，超过7万人生活在一起，这是最常见的永久性致残出生缺陷。然而，拟议研究的具体目的是探索产前修复可能对脊髓栓系综合征和神经认知发育的比率的作用，并探索种族和民族在使用预防性泌尿外科护理和获得矫正性泌尿外科手术干预方面的作用。对于我们的方法，我们将使用标准化的全国脊柱裂患者登记表和SB电子登记表来收集患者的数据，包括那些前往大型、种族多元化的德克萨斯州儿童医院/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，并将进一步加深我们对种族/民族作为不良泌尿系结果的风险因素的理解。我们的长期目标是建议改变临床实践，以制定人群水平的SB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.