NUTRITIONAL STATUS & PROGRESSION OF CF PULMONARY DISEASE

这项研究的目的是确定更好的生长和营养状况是否可以减缓患有CF的幼儿肺部疾病的进展。这是一项为期两年的前瞻性多中心研究，研究对象为基线年龄的6至8岁患有慢性胰腺炎和胰腺功能不全的儿童。如果儿童有其他已知的影响生长或营养状况的疾病，或者如果他们的肺功能低于FEV1预测的40%，则儿童被排除在外。大约一半的受试者将参加一项营养干预计划，该计划以前被证明在增加CF儿童的卡路里摄入量和体重方面是有效的。该方案包括在基线、12个月和24个月入院过夜，以评估静息和总能量消耗、生长、身体成分、性和骨骼成熟、饮食摄入量和肺功能。还计划在6个月和18个月时进行访问，以进行增长评估。这些通常在家里的CF中心进行。该协议自一开始就没有任何变化。目前还没有与研究方案相关的不良事件。然而，在一个病例中，在入院接受研究方案后发现，受试者并不处于研究方案所要求的正常健康状态，而是处于肺部恶化之中。这种疾病与研究方案的任何方面都没有关系。受试者在CHOP接受了病情恶化的治疗，随后被转移到她家中的CF中心。

The purpose of this study is to determine whether better growth and nutritional status can slow the progression of pulmonary disease in young children with CF. This is a 2 year prospective multi-center study of children 6 to 8 years of age at baseline with CF and pancreatic insufficiency. Children are excluded if they have other medical conditions known to affect growth or nutritional status, or if their pulmonary function is less than 40 percent of predicted for FEV1. Approximately half of the subjects will be enrolled in a nutrition intervention program previously shown to be effective in increasing caloric intake and weight gain in children with CF. The protocol involves an overnight admission at baseline, 12 and 24 months to assess resting and total energy expenditure, growth, body composition, sexual and skeletal maturation, dietary intake and pulmonary function. Visits are also scheduled at 6 and 18 months for growth assessment. These are usually conducted at the home CF Center. There have been no changes to the protocol since it's inception. There have been no adverse events related to the study protocol. However, in one case, it was discovered following admission for the study protocol that the subject was not in a state of usual health as is required by the study protocol, but was in the midst of a pulmonary exacerbation. The illness was not related to any aspect of the study protocol. The subject was treated for the exacerbation at CHOP and subsequently transferred to her home CF Center.