Comp C-Urologic Management to Preserve Renal Function Protocol - Duke Project

描述（由申请人提供）：脊柱裂（SB）是一种先天性神经系统缺陷，可影响胎儿、成长中的儿童和成人的多器官系统。在怀孕第一个月结束时，由于神经管关闭不完全和脊髓膜和神经突出而导致SB。脊髓脊膜膨出是SB最严重的形式，脊髓和神经穿过脊髓缺损暴露在表面。这会导致影响许多身体系统的大范围损伤。特别是在泌尿科，各中心之间的护理差异在出生和脊髓脊膜膨出闭合时就已被确认。众所周知，超过90%的脊髓脊膜膨出儿童患有神经性膀胱，如果不及时治疗，神经性膀胱的后遗症可能会导致肾功能衰竭。婴儿的泌尿科护理可以积极主动地识别并开始“敌对”膀胱的早期治疗，也可以更多地观察，只有当肾脏开始受到损害时才开始治疗。国家SB患者登记处（NSBPR）由美国疾病控制与预防中心（CDC）于2008年发起，目前已收集了来自全国19家SB诊所的4000多名患者的数据。该试点项目展示了多个SB中心协作登记SB患者并收集相关患者数据的能力，以共同改善SB患者的护理。登记处的下一个合乎逻辑的步骤是检查

DESCRIPTION (provided by applicant): Spina Bifida (SB) is a congenital neurologic defect that can affect multiple organ systems in the fetus, growing child, and adult. SB results from incomplete neural tube closure and protrusion of spinal membranes and nerves by the end of the first month of pregnancy. Myelomeningocele is the most severe form of SB in which the spinal cord and nerves come through the spinal defect and are exposed on the surface. This results in a wide range of impairments that affect many body systems. In urology specifically, variation in care between centers is recognized beginning at birth and myelomeningocele closure. It is well known that over 90% of children with myelomeningocele have neurogenic bladder, and if untreated, the sequelae of neurogenic bladder may result in renal failure over time. Urologic care in infants may be proactive to identify and initiate early treatment of "hostil" bladders or may be more observational with initiation of therapy only when kidneys begin to suffer insult. The National SB Patient Registry (NSBPR) was initiated in 2008 by the Centers for Disease Control and Prevention (CDC) and now has collected data on over 4000 patients from 19 SB clinics across the nation. This pilot project has demonstrated the ability of multiple SB centers to work collaboratively to enroll SB patient and collect pertinent patient data in an effor to improve care of SB patients collectively.(2) The next logical step of the registry is to examine critical issues in the care of SB patients by application of this model of multi-center collaboratie data collection. The CDC recently enlisted experts in pediatric urology and nephrology to develop a protocol for prospective standardized urologic management of newborns with myelomeningocele (Urologic Protocol for the Protection of Renal Function - UPPF) to be conducted over the first five years of life. The number of affected newborns across the U.S. is too small to make feasible a randomized, prospective controlled trial between proactive and observational management, but regular evaluation of a standardized protocol should allow determinations to be made about the necessity and regularity of interventions, such as imaging, urodynamic testing, and laboratory tests, to preserve renal function in the growing child. This project proposes to test the feasibility of implementation of the UPPF at Duke, assess the long term urologic outcomes of renal and bladder function in children following the UPPF over the first five years of life, and determine the necessity of following all components of the UPPF during the five years to optimally achieve the goal of preservation of renal function as a positive health outcome.