Primary Children's Medical Center Spina Bifida Patient Registry

描述（由申请人提供）：SB是妊娠早期常见的脊柱先天性缺陷。自1998年以来，美国使用叶酸补充剂和强制性强化谷物已证明某些地区SB的患病率有所下降20。在犹他州，自2003年以来，所有神经管缺陷（NTD）的患病率实际上都有所增加，而SB 1的患病率没有减少。旨在满足SB患者复杂需求的多学科医疗保健计划仍然需要更深入地了解研究需求，以指导最佳实践并改善SB患者的生活。很少有前瞻性研究能够为研究或实践提供强有力的结论。该项目，国家SB患者登记示范项目，旨在促进人口统计学因素和临床干预措施对不同人群诊所中患者结局的影响的理解。该登记将用于确定前瞻性研究的领域，以指导最佳实践并改善SB患者的生活。我们位于犹他州，参与该项目，因为我们：1）全面的，全州范围的监测系统，为SB（犹他州出生缺陷网络），2）SB患者的集中医疗保健（SB计划，初级儿童医疗中心），3）作为一个主要的卫生保健机构（山间卫生保健）的儿科三级设施的位置，它提供了强大的数据集的联系。我们的目标是对2009年在犹他州SB诊所接受治疗的125名儿童进行标准化数据收集，以促进对SB患者护理的理解，并确定未来研究的领域。这些患者将在第2年和第3年观察。将为所有患者提供参与该项目的机会。该提案的具体目标是：1.在国家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; 5.制定将数据结果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.