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)治疗是否改善了患有囊性纤维化的营养不良儿童的营养状况并促进了他们的生长。我们正在进行一项1-2期双盲交叉研究，将营养不良的CF患者的IGF-I治疗与安慰剂治疗进行比较。营养不良影响存活率，在肺功能恶化的CF患者中很常见。营养补充通常需要使用胃造口术或鼻胃管。然而，这种喂养很难管理，可能会进一步使患有CF的儿童和青少年蒙受耻辱。在患有CF的儿童和青少年中，IGF-I的血液水平很低，IGF-I是一种正常生长所必需的促生长肽。由于IGF-I治疗改善了营养不良患者的营养状况，因此有理由对营养不良的CF儿童使用促生长多肽治疗进行评估。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。在降低基线和刺激的胰岛素水平的同时，IGF-1促进了患有CF的青春期前儿童的直线生长。增强的线性生长可能反映了IGF-1的生长促进作用，或者与胰岛素敏感性增加相关的代谢效率的提高。未来计划包括在即将到来的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.