方海宁+于飞

    [摘要] Prader-Willi综合征是一种极易引起儿童、青少年肥胖的基因组印记性疾病，由于过度进食、肥胖所引发的高血压、高血糖、血脂异常等代谢综合征症候群会严重影响患儿的生存率及生活质量。本文对一例经基因确诊的9岁Prader-Willi综合征并发代谢综合征的患儿使用人胰高血糖素样肽-1受体激动剂——艾塞那肽治疗前后血压、体重、生化、肝脏影像学、睡眠呼吸监测等数据进行分析。艾塞那肽治疗6个月后，患儿食欲减少，饱腹感增加，体重及体重指数下降，血压下降，低密度脂蛋白胆固醇下降，高密度脂蛋白胆固醇升高，肝脏CT值上升，呼吸暂停低通气量指数下降，耐受性好。本研究为此类患儿的治疗提供了新的可能的选择。

    [关键词] Prader-Willi综合征；代谢综合征；艾塞那肽；非酒精性脂肪性肝病；阻塞性睡眠呼吸暂停低通气量综合征

    [中图分类号] R596.1 [文献标识码] A [文章编号] 1673-7210(2018)01(c)-0159-04

    [Abstract] Prader-Willi syndrome is a genomic imprinting disease that is highly susceptible to childhood and adolescence obesity， multiplex syndromes of metabolic syndrome such as hypertension， hyperglycemia and dyslipidemia due to overfeeding and obesity will seriously affect the survival rate and quality of life of children. In this paper， a case of genetically diagnosed 9 years old Prader-Willi syndrome child complicated with metabolic syndrome is reported to analyse the data of blood pressure， body weight， biochemistry， liver imaging and sleep apnea monitoring before and after treatment of human glucagon like peptide -1 receptor agonist-exenatide ......