Prodam Flavia, Bellone Simonetta, Grugni Graziano, Crinò Antonino, Ragusa Letizia, Franzese Adriana, Di Battista Eliana, Corrias Andrea, Walker Gillian, Rapa Anna, Aimaretti Gianluca, Bona Gianni
Division of Paediatrics, Department of Medical Science, University of Piemonte Orientale, Novara, Italy.
Clin Nutr. 2009 Feb;28(1):94-9. doi: 10.1016/j.clnu.2008.12.002. Epub 2009 Jan 17.
BACKGROUND & AIMS: Prader Willi syndrome (PWS) is a genetic syndrome characterized by hyperphagia, morbid obesity, relative hypoinsulinemia and normal insulin sensitivity. PWS presents higher total (TG) and acylated ghrelin (AG) levels. The cause of this increase as well as the modulation of ghrelin secretion in fasting and feeding in relation to other metabolic parameters and glucose tolerance in PWS is largely unknown.
We studied TG and AG at fasting in PWS children (14) and adults (18). We also studied TG and AG response to a mixed standardized light breakfast (SLB) in PWS adults without (AD-GT) and with glucose intolerance (AD-GI) at OGTT.
TG and AG were higher in children than in adults (p<0.05). AG was higher in adult males (p<0.001). Fasting AG and AG/TG ratio were lower in AD-GI than in AD-GT (p<0.05). TG, but not AG, decreased in AD-GT (p<0.006), whereas AG, but not TG, increased in AD-GI (p<0.03) post-SLB. Fasting TG and AG were negatively predicted by fasting insulin (p<0.05). Post-SLB AG was positively predicted by glucose during OGTT (p<0.04).
Fasting and post-meal AG levels are influenced by glucose tolerance in PWS, suggesting that AG derangement might have a role in the development of glucose intolerance.
普拉德-威利综合征（PWS）是一种遗传性综合征，其特征为食欲亢进、病态肥胖、相对低胰岛素血症以及正常的胰岛素敏感性。PWS患者的总生长激素释放肽（TG）和酰化生长激素释放肽（AG）水平较高。这种升高的原因以及PWS患者在禁食和进食期间生长激素释放肽分泌与其他代谢参数及葡萄糖耐量的关系在很大程度上尚不清楚。
我们研究了PWS儿童（14例）和成人（18例）禁食时的TG和AG。我们还研究了PWS成人在口服葡萄糖耐量试验（OGTT）中无葡萄糖不耐受（AD-GT）和有葡萄糖不耐受（AD-GI）的情况下，混合标准清淡早餐（SLB）后TG和AG的反应。
儿童的TG和AG高于成人（p<0.05）。成年男性的AG较高（p<0.001）。AD-GI患者的空腹AG和AG/TG比值低于AD-GT患者（p<0.05）。SLB后，AD-GT患者的TG降低（p<0.006），但AG未降低；而AD-GI患者的AG升高（p<0.03），但TG未升高。空腹胰岛素可负向预测空腹TG和AG（p<0.05）。OGTT期间的葡萄糖可正向预测SLB后的AG（p<0.04）。
PWS患者的葡萄糖耐量会影响空腹和餐后AG水平，提示AG紊乱可能在葡萄糖不耐受的发生中起作用。
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