NUTRITIONAL STATUS & PROGRESSION OF CF PULMONARY DISEASE

本研究的目的是确定更好的生长和营养状况是否可以减缓年幼CF患儿肺部疾病的进展。这是一项为期2年的前瞻性多中心研究，研究对象是基线时6至8岁的CF伴胰腺功能不全患儿。如果儿童有其他已知影响生长或营养状况的疾病，或者他们的肺功能低于预测的FEV1的40%，则排除在外。大约一半的受试者将被纳入营养干预计划，该计划先前显示对增加CF儿童的热量摄入和体重增加有效。该方案包括在基线、12和24个月时入院过夜，以评估静息和总能量消耗、生长、身体组成、性和骨骼成熟、饮食摄入和肺功能。在6个月和18个月时也安排了访问以进行生长评估。这些通常在家庭CF中心进行。协议从一开始就没有改变过。没有与研究方案相关的不良事件。然而，在一个案例中，在研究方案入院后发现，受试者并非处于研究方案所要求的正常健康状态，而是处于肺恶化的过程中。该疾病与研究方案的任何方面都没有关系。受试者在CHOP治疗病情恶化，随后转移到家中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.