普拉德-威利综合征患儿碳水化合物代谢改变的特征分析

Characterization of alterations in carbohydrate metabolism in children with Prader-Willi syndrome.

作者信息

Krochik Andrea Gabriela, Ozuna Blanca, Torrado María, Chertkoff Lilien, Mazza Carmen

机构信息

Nutrition Department, Hospital de Pediatría J P Garrahan, Buenos Aires, Argentina.

出版信息

J Pediatr Endocrinol Metab. 2006 Jul;19(7):911-8. doi: 10.1515/jpem.2006.19.7.911.

DOI:10.1515/jpem.2006.19.7.911

PMID:16995571

Abstract

OBJECTIVE

To study carbohydrate metabolism and insulin sensitivity and secretion in children and adolescents with Prader-Willi syndrome (PWS) compared with multifactorial obesity (MO) controls.

PATIENTS AND METHODS

Seventy-five patients with PWS and 395 controls with MO were studied by oral glucose tolerance test. Insulin resistance (IR) and beta-cell function were assessed by homeostasis model assessment (HOMA), insulin glucose index, fasting insulin and insulin sensitivity index.

RESULTS

The incidence of diabetes mellitus was 0% in PWS and 1.5% in MO, while carbohydrate intolerance was 9.3% in the former group and 7.6% in the latter (NS); basal insulin level (12 +/- 8.2 vs 22.3 +/- 25 mU/ml) and HOMA-IR (2.47 +/- 1.6 vs 4.18 +/- 5.05) were lower in PWS (p = 0.004 and 0.04, respectively), whereas HOMA beta-cell index was lower in PWS than in MO (59 +/- 42 vs 102 +/- 119, p = 0.03). ISI Composite was higher in PWS compared to MO (6 +/- 5.7 vs 4.18 +/- 5.05, p = 0.04).

CONCLUSION

Patients with PWS presented lower insulin resistance and a dissociation between beta-cell secretion and the degree of obesity.

摘要

目的

研究普拉德-威利综合征（PWS）患儿及青少年的碳水化合物代谢、胰岛素敏感性和分泌情况，并与多因素肥胖（MO）对照组进行比较。

患者与方法

通过口服葡萄糖耐量试验对75例PWS患者和395例MO对照组进行研究。采用稳态模型评估（HOMA）、胰岛素葡萄糖指数、空腹胰岛素和胰岛素敏感性指数评估胰岛素抵抗（IR）和β细胞功能。

结果

PWS患者的糖尿病发病率为0%，MO对照组为1.5%；而碳水化合物不耐受在PWS组为9.3%，MO组为7.6%（无显著性差异）；PWS患者的基础胰岛素水平（12±8.2对22.3±25 mU/ml）和HOMA-IR（2.47±1.6对4.18±5.05）较低（分别为p = 0.004和0.04），而PWS患者的HOMA β细胞指数低于MO组（59±42对102±119，p = 0.03）。与MO组相比，PWS患者的ISI Composite较高（6±5.7对4.18±5.05，p = 0.04）。结论：PWS患者表现出较低的胰岛素抵抗以及β细胞分泌与肥胖程度之间的分离。