EFFECT OF IGF-1 ON MALNOURISHED CHILDREN WITH CYSTIC FIBROSIS

我们的目标是确定胰岛素样生长因子（IGF-I）治疗是否能改善营养不良的囊性纤维化儿童的营养状况并促进其生长。 我们正在进行一项1-2期双盲交叉研究，比较IGF-I与安慰剂治疗营养不良的CF患者的效果。 营养不良对生存率有不利影响，在肺功能恶化的CF患者中很普遍。 营养补充通常需要使用胃造口术或鼻胃管。 然而，喂养很难管理，并可能进一步羞辱患有CF的儿童和青少年。在患有CF的儿童和青少年中，IGF-I（一种正常生长所必需的生长促进肽）的血液水平较低。 由于IGF-I治疗可改善营养不良患者的营养状况，因此存在评估营养不良CF儿童生长促进肽治疗的理由。 此外，IGF-I有助于调节血糖水平。 IGF-I对血糖控制的影响可能特别重要，因为CF患者患糖尿病的风险增加。 如果IGF-I改善营养状况、生长和血糖控制，那么它可能会改善营养不良的CF儿童的功能结局。IGF-1促进CF青春期前儿童的线性生长，同时降低基线和刺激胰岛素水平。 增强的线性生长可能反映了IGF-1的生长促进作用或与胰岛素敏感性增加相关的代谢效率提高。 未来的计划包括在未来一年内完成和分析这项研究。

Our goal was to determine if treatment with insulin-like growth factor (IGF-I) improves nutritional status and promotes growth in undernourished children with cystic fibrosis. We are in the midst of a phase 1-2 double blind crossover study that compares the treatment with IGF-I to placebo in undernourished patients with CF. Malnutrition adversely affects survival and is prevalent among patients with CF who have deteriorating pulmonary function. Nutritional supplementation often requires the use of a gastrostomy or nasogastric tube. However, the feedings are difficult to administer and may further stigmatize children and adolescents with CF. Blood levels of IGF-I, a growth-promoting peptide that is necessary for normal growth, are low in children and adolescents with CF. Since treatment with IGF-I improves nutritional status in undernourished patients, a rationale exists to assess treatment with growth-promoting peptides in those children with CF who are undernourished. Furthermore, IGF-I helps to regulate blood sugar levels. The effect of IGF-I on blood glucose control may be of particular importance since patients with CF have an increased risk to develop diabetes. If IGF-I improves nutritional status, growth, and glucose control, then it will likely enhance functional outcome in undernourished children with CF. IGF-1 promoted linear growth in prepubertal children with CF while decreasing baseline and stimulated insulin levels. Enhanced linear growth may have reflected the growth-promoting effects of IGF-1 or improved metabolic efficiency associated with increased insulin sensitivity. The future plans include completion and analysis of the study within the upcoming year.