Primary Children's Medical Center Spina Bifida Patient Registry

描述(申请人提供)：SB是一种常见的先天性脊柱缺陷，发生在妊娠早期。自1998年以来，美国使用叶酸补充剂和强制强化谷物，已证明某些地区的SB患病率有所下降20。在犹他州，自2003年以来，所有神经管缺陷(NTD)的患病率实际上一直在上升，而SB1的患病率并没有下降。为满足SB患者的复杂需求而设计的多学科医疗保健计划继续需要，同时需要对研究需求有更多的了解，以指导最佳实践并改善SB患者的生活。很少进行前瞻性研究，以得出强有力的结论，为研究或实践提供建议。这个项目，国家SB患者登记示范项目，旨在促进对人口因素和临床干预对患者结局的影响的理解，这些患者在一些不同人口的诊所看到。登记处将用于确定未来研究的领域，以指导最佳做法并改善与SB有关的个人的生活。我们之所以选择犹他州参与这一项目，是因为我们拥有：1)全州范围内的SB(犹他州出生缺陷网络)全面监测系统；2)针对SB(SB计划，初级儿童医疗中心)的个人集中医疗保健；3)作为主要医疗保健机构(山间医疗保健)的儿科三级机构，该机构提供强大的数据集以支持关联。我们的目标是在2009年对犹他州SB诊所治疗的125名儿童实施标准化数据收集，以帮助理解SB患者的护理并确定未来研究的领域。这些患者将在第二年和第三年接受治疗。所有患者都将有机会参与该项目。这项建议的具体目标是：1.在全国SB患者登记处实施标准化的数据收集流程；2.进行数据收集；3.实施分析计划；4.与其他获奖者一起参加协调委员会；以及5.制定将数据发现整合到临床实践中的计划。

DESCRIPTION (provided by applicant): SB is a common congenital defect of the spine occurring early in gestation. The use of folate supplementation and mandatory fortification of grains in the US since 1998 has demonstrated a decrease in the prevalence of SB in some areas20. In Utah, there has actually been an increase in the prevalence of all neural tube defects (NTD's) since 2003, and no reduction in SB1. Multidisciplinary health care programs designed to meet the complex needs of individuals with SB continue to be needed with greater understanding of research needs to direct best practice and improve the lives of individuals with SB. Few prospective studies have been carried out to make strong conclusions for recommendations for research or practice. This project, National SB Patient Registry Demonstration Project, seeks to advance the understanding of the influence of demographic factors and clinical interventions on outcome for patients seen in a number of clinics with diverse populations. The registry will be used to identify areas for prospective research to direct best practices and improve the lives of individuals with SB. We are positioned in Utah to participate in this project because of our: 1) Comprehensive, statewide surveillance system for SB (Utah Birth Defects Network), 2) Centralized health care for individuals with SB (SB Program, Primary Children's Medical Center), 3) Position as a pediatric tertiary facility of a major health care agency (Intermountain Health Care), which provides robust datasets for linkage. Our goal is to implement the standardized data collection with 125 children treated in the SB Clinic in Utah in 2009, in order to contribute to the understanding of care for patients with SB and identify areas of future research. These patients will be seen in year 2 and year 3. The opportunity to be involved in the project will be offered to all patients. The specific aims of this proposal are: 1. Implement standardized data collection process in the National SB Patient Registry; 2. Conduct data collection; 3. Implement an analytical plan; 4. Participate in a Coordinating Committee with other awardees; and 5. Develop a plan for integrating data findings into clinical practice.