Component B. National Spina Bifida Patient Registry (NSBPR)

摘要 该项目的目标是继续参与全国脊柱裂患者 注册表。这项建议的目的是：1)收集数据以纳入SB登记处，包括 健康状况、干预措施和结果，以更好地了解 人口，提高护理质量，优化健康；2)促进 通过高质量、协作性的行为了解SB人群 研究，利用现有的注册数据；以及3)改善临床护理和结果 适用于CHLA脊柱裂计划中照顾的脊柱裂患者。脊柱裂 在这个国家出生的大约380万名婴儿中，每个人都会影响到大约1600人 据估计，美国目前有16万人患有艾滋病 病情(脊髓脊膜膨出、脂肪脊膜脊膜膨出和脑膜膨出)。在……里面 脊髓脊膜膨出，典型的损害包括脑积水，Chiari II畸形， 神经源性肠道和/或膀胱导致大小便失禁、肌肉无力和缺乏 腿部感觉和认知功能障碍。这些损伤可能 对日常生活产生重大影响，并可能干扰学校、工作和 其他社区活动。考虑到某人可能出现的无数症状， 健康状况会对生活质量产生重大影响。国家脊柱裂 病人登记处是了解和预防原发和非传染性疾病的重要工具 与某人相关的继发性疾病，可能导致健康状况改善， 降低了医疗成本，提高了生活质量。从使用中得出的研究结果 可帮助确定可普遍采用的最佳做法 SB诊所的社区，从而改善了对所有患者的护理。 项目目标的实现将有助于增加对 通过继续招收患者来增加人口，从而改善临床护理 从确定最佳做法到强有力的研究努力。我们的目标是 改善护理、生活质量、长期健康和全面纳入 这一重要群体的社区生活。

Abstract The goal of this project is to continue to participate in the National Spina Bifida Patient Registry. This proposal aims to: 1) collect data for inclusion in the SB Registry including health status, interventions and outcomes, to better understand the needs of the population, improve the quality of care and optimize health; 2) contribute to the body of knowledge about the SB population through the conduct of high quality, collaborative research, utilizing existing Registry data; and 3) improve the clinical care and outcomes for patients with spina bifida cared for in the CHLA Spina Bifida Program. Spina bifida affects about 1600 of the approximately 3.8 million babies born in the country each year; an estimated 160,000 people in the United States are currently living with the condition (myelomeningocele, lipomeningocele and meningocele). In myelomeningocele, typical impairments include hydrocephalus, Chiari II malformation, neurogenic bowel and/or bladder resulting in incontinence, muscle weakness and lack of sensation in lower extremities, and cognitive dysfunction. These impairments can have significant impact on day-to-day living and can interfere with school, work and other community activities. Given the myriad symptoms that can be present in SB, the condition can have a significant impact on quality of life. The National Spina Bifida Patient Registry is an important vehicle for understanding and preventing primary and secondary conditions associated with SB, potentially leading to improved health, reduced health care costs and improved quality of life. Research resulting from the use of Registry data 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 continued enrollment of patients, and improve 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.