用度拉鲁肽治疗一名普拉德-威利综合征患儿的早发 III 期糖尿病肾病:病例报告。

IF 1.5 4区 医学 Q2 PEDIATRICS Translational pediatrics Pub Date : 2024-05-31 Epub Date: 2024-05-21 DOI:10.21037/tp-23-518
Yonghua He, Rongrong Xu, Xueqing Ma, Jianhua Zhou, Liru Qiu
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引用次数: 0

摘要

背景:普拉德-威利综合征(PWS)是一种由染色体印记基因缺陷引起的多系统遗传性疾病,其中约 70% 的病例是由于父方第 15 号染色体区域缺失所致。主要临床特征包括严重的婴儿肌张力低下、早发的儿童肥胖症、多食和外生殖器发育不全。随着年龄的增长,PWS 患者可能会表现出易怒、社交功能障碍、性腺发育受损和代谢综合征。以往的文献显示,PWS 患者中 2 型糖尿病(T2DM)的发病率约为 7-24%。催产素是由下丘脑室旁核(PVN)和视上核(SON)分泌的一种神经肽,可调节能量代谢,与 PWS 有关。由于年龄的限制,很少有患者在儿童期发展为糖尿病肾病,儿童期典型的 PWS 糖尿病肾病的报道也极为罕见。杜拉鲁肽是一种胰高血糖素样肽-1(GLP-1)受体激动剂,可用于治疗T2DM:本文报道了一例PWS患儿并发Ⅲ期糖尿病肾病的病例,对诊断和治疗过程进行了回顾性分析,并对国内外文献进行了综述,以加深对该病的认识。本文还为合并糖尿病肾病的PWS患者提供了治疗思路:结论:加强对 PWS 的了解非常重要。结论:加强对 PWS 的认识非常重要,我们为儿童糖尿病肾病患者提供了新的诊断和可能的治疗方法。
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Early onset stage III diabetic nephropathy in a child with Prader-Willi syndrome treated with dulaglutide: a case report.

Background: Prader-Willi syndrome (PWS) is a multisystem genetic disorder caused by chromosomal imprinting gene defects, with approximately 70% of cases resulting from paternal deletion of the chromosomal region 15. The main clinical features include severe infantile hypotonia, early-onset childhood obesity, hyperphagia, and underdeveloped external genitalia. As individuals with PWS age, they may exhibit irritability, social dysfunction, impaired gonadal development, and metabolic syndrome. Previous literature places the prevalence of type 2 diabetes mellitus (T2DM) in PWS at approximately 7-24%. Oxytocin is a neuropeptide secreted by the paraventricular (PVN) and supraoptic (SON) nuclei of the hypothalamus and regulates energy metabolism, which is involved in PWS. Due to age limitations, very few patients progress to diabetic nephropathy during childhood, and reports of typical diabetic nephropathy in PWS during childhood are extremely rare. Dulaglutide is a glucagon-like peptide-1 (GLP-1) receptor agonist which can be used in the treatment of T2DM.

Case description: This article reports a case of a child with PWS complicated by stage III diabetic nephropathy, providing a retrospective analysis of the diagnosis and treatment process, as well as a review of domestic and international literature, to enhance understanding of this condition. And this article provides a treatment idea for PWS patients with diabetic nephropathy.

Conclusions: It is very important to enhance understanding of PWS. And we offer new diagnostic and possible therapeutic approaches for pediatric patients with diabetic nephropathy.

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来源期刊
Translational pediatrics
Translational pediatrics Medicine-Pediatrics, Perinatology and Child Health
CiteScore
4.50
自引率
5.00%
发文量
108
期刊介绍: Information not localized
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