Department of Screening Tests and Metabolic Diagnostics, Institute of Mother and Child, 01-211 Warsaw, Poland.
Department of Epidemiology and Biostatistics, Institute of Mother and Child, 01-211 Warsaw, Poland.
Nutrients. 2023 Mar 1;15(5):1240. doi: 10.3390/nu15051240.
Despite observable improvement in the treatment outcomes of patients with Prader-Willi syndrome (PWS), adequate weight control is still a clinical problem. Therefore, the aim of this study was to analyze the profiles of neuroendocrine peptides regulating appetite-mainly nesfatin-1 and spexin-in children with PWS undergoing growth hormone treatment and reduced energy intake.
Twenty-five non-obese children (aged 2-12 years) with PWS and 30 healthy children of the same age following an unrestricted age-appropriate diet were examined. Serum concentrations of nesfatin-1, spexin, leptin, leptin receptor, total adiponectin, high molecular weight adiponectin, proinsulin, insulin-like growth factor-I, and total and functional IGF-binding protein-3 concentrations were determined using immunoenzymatic methods.
The daily energy intake in children with PWS was lower by about 30% ( < 0.001) compared with the controls. Daily protein intake was similar in both groups, but carbohydrate and fat intakes were significantly lower in the patient group than the controls ( < 0.001). Similar values for nesfatin-1 in the PWS subgroup with BMI Z-score < -0.5 and the control group, while higher values in the PWS subgroup with BMI Z-score ≥ -0.5 ( < 0.001) were found. Spexin concentrations were significantly lower in both subgroups with PWS than the controls ( < 0.001; = 0.005). Significant differences in the lipid profile between the PWS subgroups and the controls were also observed. Nesfatin-1 and leptin were positively related with BMI ( = 0.018; = 0.001, respectively) and BMI Z-score ( = 0.031; = 0.027, respectively) in the whole group with PWS. Both neuropeptides also correlated positively in these patients ( = 0.042).
Altered profiles of anorexigenic peptides-especially nesfatin-1 and spexin-in non-obese children with Prader-Willi syndrome during growth hormone treatment and reduced energy intake were found. These differences may play a role in the etiology of metabolic disorders in Prader-Willi syndrome despite the applied therapy.
尽管普拉德-威利综合征(PWS)患者的治疗结果有明显改善,但适当的体重控制仍然是一个临床问题。因此,本研究的目的是分析接受生长激素治疗和减少能量摄入的 PWS 儿童中调节食欲的神经内分泌肽谱——主要是 nesfatin-1 和 spexin。
检查了 25 名非肥胖(年龄 2-12 岁)的 PWS 儿童和 30 名年龄匹配的接受不限量饮食的健康儿童。使用免疫酶法测定 nesfatin-1、spexin、瘦素、瘦素受体、总脂联素、高分子量脂联素、胰岛素原、胰岛素样生长因子-I 以及总和功能性 IGF 结合蛋白-3 的血清浓度。
PWS 患儿的每日能量摄入量比对照组低约 30%(<0.001)。两组患儿的每日蛋白质摄入量相似,但 PWS 患儿的碳水化合物和脂肪摄入量明显低于对照组(<0.001)。PWS 患儿 BMI Z 评分<-0.5 亚组和对照组的 nesfatin-1 值相似,而 BMI Z 评分≥-0.5 的 PWS 患儿亚组的 nesfatin-1 值较高(<0.001)。两个 PWS 亚组的 spexin 浓度均明显低于对照组(<0.001;=0.005)。PWS 亚组与对照组之间的血脂谱也存在显著差异。在整个 PWS 组中,nesfatin-1 和瘦素与 BMI(=0.018;=0.001)和 BMI Z 评分(=0.031;=0.027)呈正相关。在这些患者中,两种神经肽也呈正相关(=0.042)。
在接受生长激素治疗和减少能量摄入的非肥胖 PWS 儿童中发现了厌食肽谱的改变——特别是 nesfatin-1 和 spexin。尽管进行了治疗,但这些差异可能在 PWS 代谢紊乱的发病机制中发挥作用。