MENTORED PATIENT-ORIENTED RESEARCH CAREER DEVELOPMENT

描述(由申请人提供)：系统性红斑狼疮儿童成年期存活率的提高导致了以前没有出现过的疾病 研究得很好。对这种发病率的有效评估需要纵向的 研究设计注意发病时的疾病严重程度和发病时间 疾病的发生与生长发育有关。因此，一个主要目标是 发展具有良好特色的北加州纵向儿科和 不同年龄段的青少年系统性红斑狼疮队列表现出多样性(至少7 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多个方面的资源 这一人群的长期结果。 本申请的职业发展部分包括完成 加州大学旧金山分校注重学习的临床研究硕士学位课程 用于分析重复测量、多变量和 分子流行病学，分析纵向多发性肺炎的所有重要技能 系统疾病数据集。 本应用程序的科学部分解决了 儿童起病的系统性红斑狼疮中的骨质疏松。发病时间和发病时间重合 达到正常骨量峰值可能会增加患骨质疏松症的风险 未来的骨质疏松症和骨折。系统性红斑狼疮相关疾病的年度测量 用双能X线评估骨密度的降低 骨密度测定(DEXA)和单能量定量计算机断层扫描(QCT) 将与疾病、治疗和宿主相关的因素相关， 相应的骨代谢标志物。骨骼与骨骼之间的关系 通过骨龄和骨密度纵向变化来评估成熟度将是 通过评估峰值骨量的时间和水平来确定，并且 疾病发生的时间对骨密度降低的贡献。 这个项目的发现可能会导致骨龄的发展- 儿童系统性红斑狼疮的具体治疗方案，并最终将有助于 改善儿童期患者的成人生活质量 SLE。此外，这一特征良好的系统性红斑狼疮队列的发展将 作为未来重要的资源，为学习奠定基础 儿童期起病的系统性红斑狼疮的许多终身影响。

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.