Component B. National Spina Bifida Patient Registry (NSBPR)

摘要 这个项目的目标是继续参加全国脊柱裂患者 注册表本建议旨在：1）收集数据以纳入SB登记，包括 卫生状况、干预措施和成果，以更好地了解 人口，提高护理质量和优化健康; 2）有助于身体的 通过开展高质量的合作，了解SB人口 研究，利用现有的登记数据;和3）改善临床护理和结果 对于在CHLA脊柱裂计划中接受治疗的脊柱裂患者。脊柱裂 在该国出生的大约380万婴儿中， 据估计，美国目前有16万人生活在 条件（脊髓脊膜膨出、脂肪脊膜膨出和脊膜膨出）。在 脊髓脊膜膨出，典型的损害包括脑积水、基亚里II型畸形， 神经源性肠和/或膀胱，导致失禁、肌无力和缺乏 下肢的感觉和认知功能障碍。这些缺陷可能 对日常生活产生重大影响，并可能干扰学校、工作和 其他社群活动。考虑到SB中可能存在的无数症状， 病情会对生活质量产生重大影响。全国脊柱裂 患者登记是了解和预防原发性和 与SB相关的继发性疾病，可能导致健康改善， 降低医疗费用，提高生活质量。研究结果的使用 登记册数据的收集可有助于确定可普遍采用的最佳做法， SB诊所社区，从而改善对所有患者的护理。 项目目标的实现将有助于增加关于 通过继续招募患者，改善临床护理， 通过强有力的研究工作确定最佳做法。我们的目标是 改善护理、生活质量、长期健康，并充分融入社会的所有要素， 这一重要群体的生活。

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.