Component C. Urologic Management to Preserve Initial Renal Function Protocol for Young Children with Spina Bifida (UMPIRE Protocol)

摘要 青年人维持初始肾功能的泌尿外科处理方案 脊柱裂儿童（UMPIRE协议）的目标是实施和评估一个 通过收集数据， 仔细研究结果，以优化这些患者的长期肾脏健康。这 建议的目的是：1）执行和评估幼儿UMPIRE议定书 脊柱裂患者，2）支持泌尿科检查文件的标准化 结果，3）与其他网站进行高质量的合作研究项目，以及4） 改善CHLA SB患者的护理和结局。脊柱裂影响约 该国每年出生的约380万婴儿中有1600人， 28岁时神经管不完全闭合和脊髓膜突出 包括脊髓脊膜膨出、脂肪脊膜膨出和脊膜膨出。在 脊髓脊膜膨出，典型的损伤包括脑积水，基亚里II畸形， 神经源性肠、神经源性膀胱、活动能力下降和感觉缺失， 下肢和认知功能障碍神经源性膀胱患者在 复发性尿路感染、反流性肾病和 极端情况下，如果不治疗，肾衰竭。此外，患有神经源性膀胱的儿童 经常患有影响社会功能的严重尿失禁。这些 损伤可能对日常生活产生重大影响， 学校、工作和其他社群活动。认识到肾脏的重要性 功能不全和存活率是改善生活质量和生活的关键 期待UMPIRE协议是理解和 预防与SB相关的原发性和继发性疾病，特别是那些 与肾功能有关。从该协议产生的研究可以帮助确定最好的 可以被SB诊所社区普遍采用的做法，从而 从而改善对所有患者的护理。项目目标的实现将有助于 通过改善临床护理，增加有关人口的科学知识 通过强有力的研究工作确定最佳做法。我们的目标 改善护理，生活质量，长期健康，并充分融入所有要素， 这一重要群体的生活。

Abstract The Urologic Management to Preserve Initial Renal Function Protocol for Young Children with Spina Bifida (UMPIRE Protocol) goal is to implement and evaluate a urologic protocol for young children with spina bifida through the collection of data and careful study of results, to optimize the long term renal health of these patients. This proposal aims to: 1) Implement and evaluate the UMPIRE Protocol for young children with spina bifida, 2) Support standardization of the documentation of urology test results, 3) Conduct high quality, collaborative research projects with other sites, and 4) Improve the care and outcomes for SB patients at CHLA. Spina bifida affects about 1600 of the approximately 3.8 million babies born in the country each year, resulting from incomplete closure of the neural tube and protrusion of the spinal membrane at 28 days gestation, and includes myelomeningocele, lipomeningocele and meningocele. In myelomeningocele, typical impairments include hydrocephalus, Chiari II malformation, neurogenic bowel, neurogenic bladder, decreased mobility and lack of sensation in lower extremities, and cognitive dysfunction. Patients with neurogenic bladder are at high risk of developing recurrent urinary tract infections, reflux nephropathy and in extreme cases if untreated, renal failure. In addition, children with neurogenic bladder often suffer from significant incontinence of urine that effects social functioning. These impairments can have significant impact on day-to-day living and can interfere with school, work and other community activities. Realizing the importance of renal insufficiency and survival has been pivotal to improved quality of life and life expectancy. The UMPIRE Protocol is an important vehicle for understanding and preventing primary and secondary conditions associated with SB, in particular, those related to renal function. Research resulting from the protocol can help to identify best practices that can be universally adopted by the community of SB clinics, thereby leading to improved care for all patients. Achievement of project goals will serve to increase scientific knowledge about the population through improved clinical care resulting from identification of best practices through robust research efforts. Our goal is to improve the care, quality of life, long term health and full inclusion in all elements of community life for this important population.