Quantifying Effects of Malnutrition on Cystic Fibrosis

描述(由申请人提供) 项目：囊性纤维化(CF)是最常见的，威胁生命的 常染色体隐性遗传病的发病率为1/2900白人 美国的新生儿。尽管营养不良很常见，而且已经被证明是一种 慢性萎缩性胃炎预后不良因素及营养不良与预后的关系 临床结果尚未量化。如此长期存在的知识差距 可归因于缺乏前瞻性的、长期随访的患者 队列，以及具有CF营养和综合知识的流行病学家 生物统计学。这项提议的总体目标是使用现代 统计和流行病学方法来量化之间的关系 营养不良和长期临床结果的决定因素，我们定义为 肺功能、进行性呼吸道感染和生存。独一无二的 利用两个丰富的持续不断的纵向数据库的机会 1985年，即儿童基金会患者登记处和威斯康星州儿童基金会新生儿 在筛选研究数据库时，我们热衷于检验以下假设 营养不良使CF患者患肺部疾病的风险增加 病情恶化，对女性患者的影响可能比男性患者更大。 具体目标是开发：1)预测结果的基线风险模型 根据确诊时的营养状况，2)短期风险 根据营养状况恶化来预测结果的模型 之前2-4年，以及3)利用营养的长期风险模型 以及来自整个患者病史的肺部数据来预测生存。 完成这个项目将产生关于如何 营养影响肺部表现，从而影响CF的存活率。是这样的 信息对于开发新的干预措施以改善生活至关重要 慢性萎缩性胃炎患者。候选人：赖博士是儿科博士后研究员 以及威斯康星大学麦迪逊分校的生物统计学。她有独特的经验结合基本的 营养研究、临床营养实践和生物统计学。这个项目 将使她能够获得高级培训，以增强她的职业生涯 在营养流行病学方面具有专业知识的独立调查员 慢性病。环境：该项目将在 菲利普博士指导下的儿科和生物统计学系 Farrell是一位公认的CF研究专家。候选人将会 受益于与麦迪逊大学多学科CF研究团队的合作 Cf中心以及来自各方面的积极和积极的研究参与 威斯康星大学麦迪逊分校的社区。

DESCRIPTION (provided by applicant) The project: Cystic fibrosis (CF) is the most common, life-threatening autosomal recessive genetic disorder with an incidence of 1 in 2900 white newborns in the US. Although malnutrition is common and has been shown to be an unfavorable prognostic factor in CF, the relationships between malnutrition and clinical outcomes have not been quantified. Such longstanding gap of knowledge can be attributed to lack of prospectively defined, long-term followed patient cohorts, as well as epidemiologists with combined expertise in CF nutrition and biostatistics. The overall objective of this proposal is to use modern statistical and epidemiological methods to quantify the relationships between malnutrition and determinants of long-term clinical outcome, which we define as lung function, progressive respiratory infection and survival. With the unique opportunity to utilize two rich ongoing longitudinal databases collected since 1985, namely, the CF Foundation Patient Registry and the Wisconsin CF Neonatal Screening Study database, we are enthusiastic to test the hypothesis that malnutrition predisposes CF patients to increased risks of pulmonary deterioration, with potentially greater effect on female than male patients. Specific aims are to develop: 1) baseline risk models, which predict outcomes based on nutritional status at the time of CF diagnosis, 2) short-term risk models, which predict outcomes based on nutritional deterioration in the preceding 2-4 years, and 3) long-term risk models, which utilize nutritional and pulmonary data from the entire patient history to predict survival. Accomplishing this project will generate important information regarding how nutrition affects pulmonary manifestations to impact CF survival. Such information is critical to developing new interventions to improve the life of patients with CF. The candidate: Dr. Lai is a postdoctoral fellow of Pediatrics and Biostatistics at UW-Madison. She has unique experiences combining basic nutrition research, clinical nutrition practice and biostatistics. This project will enable her to gain advanced training to enhance her career toward becoming an independent investigator with expertise in nutritional epidemiology of chronic diseases. The environment: This project will be performed in the Departments of Pediatrics and Biostatistics under the supervision of Dr. Philip Farrell, who is a well-recognized expert in CF research. The candidate will benefit from working with the multidisciplinary CF research team at the Madison CF center as well as from participation in the active and vigorous research community at UW-Madison.