MENTORED PATIENT-ORIENTED RESEARCH CAREER DEVELOPMENT

描述（由申请人提供）：增加SLE儿童的生存率 进入成年后，导致了以前没有的疾病的出现， 好好研究。 有效评估这种发病率需要纵向 研究设计关注疾病发作时的严重程度和 疾病发作与生长发育有关。 因此，主要目标是 发展一个充分表征的北方加州纵向儿科， 青少年SLE队列显示不同年龄（至少7岁）的多样性 年）、种族和性别以及相应的纵向组织库。 该队列将作为未来研究的许多方面的资源， 这一人群的长期结果。 此应用程序的职业发展部分包括完成 UCSF临床研究硕士学位课程，重点是学习 设计用于分析重复测量、多个变量和 分子流行病学，所有重要的技能，分析纵向多- 系统疾病数据集。 该应用程序的科学部分涉及开发 儿童期SLE患者的骨质疏松症 疾病发作的时间一致， 达到正常的峰值骨量可能会导致增加的风险， 未来的骨质疏松症和骨折。 SLE相关的年度测量结果 使用双能X射线评估的骨矿物质密度（BMD）降低 吸收测定法（DEXA）和单能量定量计算机断层扫描（QCT） 将与疾病、治疗和宿主相关因素相关， 相应的骨代谢标志物。 骨骼与 通过骨龄和BMD的纵向变化评估骨成熟度， 通过评估峰值骨量的时间和水平来确定， 疾病的时间对BMD降低的贡献。 该项目的发现可能会导致骨龄的发展- 儿童SLE的具体治疗方案，并最终将有助于 改善儿童期发病的人的成年生活质量 SLE。 此外，这一特征良好的SLE队列的发展将 作为未来的重要资源，为学习奠定基础 儿童期发病的SLE的许多终身影响。

DESCRIPTION (provided by applicant): Increased survival of children with SLE into adulthood has resulted in the emergence of morbidities not previously well studied. Effective evaluation of this morbidity requires longitudinal study designs with attention to disease severity at onset and timing of disease onset relative to growth and development. Thus, a primary goal is to develop a well characterized Northern California longitudinal pediatric and adolescent SLE cohort demonstrating diversity across age (minimum seven years), ethnicity, and gender with a corresponding longitudinal tissue bank. The cohort will serve as a resource for the future study of many aspects of long-term outcome in this population. The career development component of this application includes completion of the UCSF Master in Clinical Research degree program with a focus on learning methods designed to analyze repeated measurements, multiple variables, and molecular epidemiology, all important skills for analyzing longitudinal multi- system disease data-sets. The scientific component of this application addresses the development of osteoporosis in pediatric onset SLE. Coincident timing of disease onset and achievement of normal peak bone mass may result in an increased risk for future osteoporosis and fracture. Annual measurements of SLE-associated reductions of bone mineral density (BMD) assessed utilizing dual energy X-ray absorptiometry (DEXA) and single energy quantitative computed tomography (QCT) will be correlated with disease-, treatment- and host-related factors and corresponding markers of bone metabolism. The relationship between skeletal maturation assessed by bone age and longitudinal changes of BMD will be determined by evaluating the timing and level of peak bone mass, and the contribution of the timing of disease to reductions of BMD. The findings of this project may result in the development of bone age- specific treatment regimens for pediatric SLE, and ultimately will contribute to improving the quality of adult life for individuals with childhood-onset SLE. Furthermore, development of this well characterized SLE cohort will serve as an important future resource and builds a foundation for studying many lifelong effects of childhood-onset SLE.