Comp B-National SB Patient Registry at Vanderbilt Children's Hospital

描述（由申请人提供）：医疗保健的进步增加了脊柱裂婴儿的寿命，脊柱裂是美国最常见的永久性残疾出生缺陷。然而，长期的健康结果是有限的，实践模式各不相同，最佳做法没有得到很好的界定。CDC已经证明了国家脊柱裂患者登记处（NSBPR）的可行性。小门罗卡雷尔的脊柱裂项目。范德比尔特儿童医院（MCJCHV）每年评估200多名脊柱裂患者。MCJCHV的神经外科、骨科和泌尿外科项目为本研究设定了以下目标：1）在我们的项目中接近每一位合格的患者进行招募，2）在五年的研究期间为NSBPR提供关于同意患者的健康状况、临床护理和结局的纵向数据，3）评估NSBPR数据，以回答假设驱动的问题，特别是关于多-脊柱裂患者脊髓栓系的学科识别和处理，以及患者从青少年向成年过渡时的健康差异。PI和临床研究协调员将沿着确保提交给CDC的数据的质量控制。PI和高级/关键人员具有与其他国家数据库和联邦资助的赠款进行生产性研究的证明记录。这项研究的结果将有助于为脊柱裂的最佳实践临床指南提供信息。

DESCRIPTION (provided by applicant): Advances in medical care have increased longevity for babies born with spina bifida, the most common permanently disabling birth defect in the United States. However, long-term health outcomes are limited, practice patterns vary, and best practices are not well defined. The CDC has demonstrated the feasibility of a National Spina Bifida Patient Registry (NSBPR). The Spina Bifida Program at Monroe Carell Jr. Children's Hospital at Vanderbilt (MCJCHV) evaluates over 200 patients with spina bifida each year. The neurosurgery, orthopedics, and urology programs at MCJCHV have set the following goals for this study: 1) to approach each eligible patient in our program for enrollment, 2) to contribute longitudinal data on health status, clinical care, and outcomes for consented patients to the NSBPR over the five year study period, and 3) to evaluate NSBPR data to answer hypothesis-driven questions specifically about multi-disciplinary identification and management of tethered cord in spina bifida patients and about health disparities as patients transition from adolescence to adulthood. The PI along with the clinical research coordinator will ensure quality control of data submitted to the CDC. The PI and senior/key personnel have a demonstrated record of productive research with other national databases and federally funded grants. Published results from this study will help to inform best practice clinical guidelines for spina bifida.