NUTRITIONAL STATUS OF CF PULMONARY DISEASE

根据1995年CF基金会患者登记数据， 的死亡率是由于心肺原因， 最常见的住院原因是肺部疾病加重 疾病 良好的营养和生长状况与较长的 生存率和肺功能改善或稳定， 营养不良的病人得到了营养。 1995年，20 CF患者的身高仍低于第5百分位数的百分比 25%的人体重低于第五百分位，这表明 严重的营养不良和发育异常。没有研究 足够的样本量，前瞻性地评估 维持良好的营养状态，防止肺部疾病进展 在一个随机的，长期的协议。 我们的目标是促进 一组CF儿童的正常生长和身体组成， 确定这是否减缓了CF肺病的进展， 两年的时间。 将从四个临床试验机构招募90名6至8岁的儿童， 以研究为导向的CF中心（见下文的性能网站）， 参与一项随机的营养干预与家庭护理 议定书 干预和数据收集活动将 由CHOP的研究团队进行，无论是在CHOP营养 和生长实验室，CHOP临床研究中心，或当地 CF中心，保证程序的统一性，数据的准确性 为家人提供方便。 营养学科 干预组将接受为期七周的家长计划， 孩子 该计划的目标是提高营养和CF知识， 与食物相关的行为管理、脂肪摄入量、总热量摄入量和 实现正常生长和身体组成。家庭护理受试者 组将接受基于当前实践的常规营养护理 他们的中心。 受试者将在两年内进行评估（基线， CHOP为12、24个月;当地研究中心为6和18个月）进行测量 包括：肺功能、营养状况（生长、身体 组成、骨骼成熟、血液值）、能量平衡 （休息、总能量消耗、粪便热量、体力活动）， 食物摄入和疾病严重程度（NIH，Schwachman，Brasfield评分， 请病假天数、住院天数、微生物学）。 我们假设能量平衡较好的孩子 如身高和体重z分数所示的生长改善， FEV 1显示肺功能更好。 这些数据将提供 早期有效营养干预科学基础 促进正常生长和身体组成作为标准CF护理。

According to the 1995 CF Foundation patient registry data, 84 percent of mortality in US patients was due to cardiopulmonary causes and the most common reason for hospitalization was an exacerbation of pulmonary disease. Good nutritional and growth status are associated with longer survival and with improvement or stabilization of pulmonary function in malnourished patients who have been renourished. Yet in 1995, 20 percent of patients with CF remain less than 5th percentile for height and 25 percent were less than 5th percentile for weight, indicating significant malnutrition and growth abnormalities. There are no studies of sufficient sample size which prospectively evaluate the effect of sustaining good nutritional status on progression of pulmonary disease in a randomized, long-term protocol. Our objective is to facilitate normal growth and body composition in a group of children with CF and determine if this slows the progression of CF pulmonary disease over a two year period. Ninety children, ages 6 to 8 years will be recruited from four clincial research oriented CF Centers (see below for Performance sites) to participate in a randomized, Nutrition Intervention versus Usual Care protocol. The intervention and data collection activities will be conducted by the CHOP-based research team, either at the CHOP Nutrition and Growth Laboratory, the CHOP Clinical Research Center, or the local CF Center, to ensure the uniformity of the program, accuracy of the data and convenience for the families. Subjects in the Nutrition Intervention group will receive a seven week program for parents and children. The program goals are to improve nutrition and CF knowledge, food-related behavioral management, fat intake, total caloric intake and achieve normal growth and body composition. Subjects in the Usual Care group will receive usual nutritional care based upon current practices of their Centers. Subjects will be assessed over two years (baseline, 12,24 months at CHOP;6 and 18 months at local sites) for measurements which include: pulmonary function, nutritional status (growth, body composition, skeletal maturation, blood values), energy balance (resting, total energy expenditure, stool calories, physical activity), food intake and disease severity (NIH, Schwachman, Brasfield scores, days sick from school, days in hospital, microbiology). We hypothesize that children with better energy balance will have improved growth as indicated by height and weight z scores and will have better pulmonary function as indicated by FEV1. These data will provide the scientific basis for early and effective nutritional intervention to promote normal growth and body composition as standard CF care.