• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

度拉糖肽治疗普拉德-威利综合征患儿的早发型III期糖尿病肾病:一例报告

Early onset stage III diabetic nephropathy in a child with Prader-Willi syndrome treated with dulaglutide: a case report.

作者信息

He Yonghua, Xu Rongrong, Ma Xueqing, Zhou Jianhua, Qiu Liru

机构信息

Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

Department of Pediatrics, The Central Hospital of Wuhan, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

出版信息

Transl Pediatr. 2024 May 31;13(5):833-839. doi: 10.21037/tp-23-518. Epub 2024 May 21.

DOI:10.21037/tp-23-518
PMID:38840685
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11148744/
Abstract

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.

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,约70%的病例是由于父源15号染色体区域缺失所致。主要临床特征包括严重的婴儿期肌张力减退、儿童期早发性肥胖、食欲亢进和外生殖器发育不全。随着PWS患者年龄增长,他们可能会出现易怒、社交功能障碍、性腺发育受损和代谢综合征。既往文献报道PWS患者2型糖尿病(T2DM)的患病率约为7%-24%。催产素是一种由下丘脑室旁核(PVN)和视上核(SON)分泌的神经肽,调节能量代谢,与PWS有关。由于年龄限制,儿童期进展为糖尿病肾病的患者非常少,关于儿童期PWS典型糖尿病肾病的报道极为罕见。度拉糖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。

病例描述

本文报告1例PWS合并III期糖尿病肾病患儿,对其诊断和治疗过程进行回顾性分析,并复习国内外文献,以提高对该疾病的认识。本文为PWS合并糖尿病肾病患者提供了一种治疗思路。

结论

加强对PWS的认识非常重要。我们为小儿糖尿病肾病患者提供了新的诊断和可能的治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3449/11148744/a7fc78f2a4ae/tp-13-05-833-f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3449/11148744/1e1a794ed905/tp-13-05-833-f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3449/11148744/87a82c48fd77/tp-13-05-833-f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3449/11148744/a7fc78f2a4ae/tp-13-05-833-f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3449/11148744/1e1a794ed905/tp-13-05-833-f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3449/11148744/87a82c48fd77/tp-13-05-833-f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3449/11148744/a7fc78f2a4ae/tp-13-05-833-f3.jpg

相似文献

1
Early onset stage III diabetic nephropathy in a child with Prader-Willi syndrome treated with dulaglutide: a case report.度拉糖肽治疗普拉德-威利综合征患儿的早发型III期糖尿病肾病:一例报告
Transl Pediatr. 2024 May 31;13(5):833-839. doi: 10.21037/tp-23-518. Epub 2024 May 21.
2
The changing purpose of Prader-Willi syndrome clinical diagnostic criteria and proposed revised criteria.普拉德-威利综合征临床诊断标准的目的变化及修订标准建议
Pediatrics. 2001 Nov;108(5):E92. doi: 10.1542/peds.108.5.e92.
3
Oxytocin's Regulation of Thermogenesis May Be the Link to Prader-Willi Syndrome.催产素对产热的调节可能是与普拉德-威利综合征的关联所在。
Curr Issues Mol Biol. 2023 Jun 6;45(6):4923-4935. doi: 10.3390/cimb45060313.
4
Is Oxytocin a Contributor to Behavioral and Metabolic Features in Prader-Willi Syndrome?催产素是普拉德-威利综合征行为和代谢特征的促成因素吗?
Curr Issues Mol Biol. 2024 Aug 13;46(8):8767-8779. doi: 10.3390/cimb46080518.
5
The effects of glucagon-like peptide (GLP)-1 receptor agonists on weight and glycaemic control in Prader-Willi syndrome: A systematic review.胰高血糖素样肽-1(GLP-1)受体激动剂在普拉德-威利综合征中对体重和血糖控制的影响:系统评价。
Clin Endocrinol (Oxf). 2022 Feb;96(2):144-154. doi: 10.1111/cen.14583. Epub 2021 Aug 26.
6
Effect of semaglutide on weight loss and glycaemic control in patients with Prader-Willi Syndrome and type 2 diabetes.司美格鲁肽对 Prader-Willi 综合征合并 2 型糖尿病患者体重减轻和血糖控制的影响。
Endocrinol Diabetes Nutr (Engl Ed). 2024 Feb;71(2):83-87. doi: 10.1016/j.endien.2023.12.001.
7
Clinical Trials in Prader-Willi Syndrome: A Review.普拉德-威利综合征的临床试验:综述。
Int J Mol Sci. 2023 Jan 21;24(3):2150. doi: 10.3390/ijms24032150.
8
Prevalence and risk factors for type 2 diabetes mellitus with Prader-Willi syndrome: a single center experience.普瑞德威利综合征 2 型糖尿病的患病率及危险因素:单中心经验
Orphanet J Rare Dis. 2017 Aug 30;12(1):146. doi: 10.1186/s13023-017-0702-5.
9
Weight Loss of Over 100 lbs in a Patient of Prader-Willi Syndrome Treated With Glucagon-Like Peptide-1 (GLP-1) Agonists.一名普拉德-威利综合征患者使用胰高血糖素样肽-1(GLP-1)激动剂治疗后体重减轻超过100磅。
Cureus. 2023 Feb 17;15(2):e35102. doi: 10.7759/cureus.35102. eCollection 2023 Feb.
10
Dysregulated adipose tissue expansion and impaired adipogenesis in Prader-Willi syndrome children before obesity-onset.普瑞德威利综合征儿童肥胖前脂肪组织过度扩张和脂肪生成受损。
Metabolism. 2022 Nov;136:155295. doi: 10.1016/j.metabol.2022.155295. Epub 2022 Aug 22.

本文引用的文献

1
Clinical Trials in Prader-Willi Syndrome: A Review.普拉德-威利综合征的临床试验:综述。
Int J Mol Sci. 2023 Jan 21;24(3):2150. doi: 10.3390/ijms24032150.
2
Effects of dulaglutide on endothelial progenitor cells and arterial elasticity in patients with type 2 diabetes mellitus.度拉糖肽对 2 型糖尿病患者内皮祖细胞及动脉弹性的影响。
Cardiovasc Diabetol. 2022 Oct 3;21(1):200. doi: 10.1186/s12933-022-01634-1.
3
Once-Weekly Dulaglutide for the Treatment of Youths with Type 2 Diabetes.每周一次度拉糖肽治疗青少年2型糖尿病
N Engl J Med. 2022 Aug 4;387(5):433-443. doi: 10.1056/NEJMoa2204601. Epub 2022 Jun 4.
4
Epigenetics in the pathogenesis of diabetic nephropathy.糖尿病肾病发病机制中的表观遗传学。
Acta Biochim Biophys Sin (Shanghai). 2022 Jan 25;54(2):163-172. doi: 10.3724/abbs.2021016.
5
Diabetic kidney disease in children and adolescents: an update.儿童和青少年糖尿病肾病:最新进展。
Pediatr Nephrol. 2022 Nov;37(11):2583-2597. doi: 10.1007/s00467-021-05347-7. Epub 2021 Dec 16.
6
The Spectrum of the Prader-Willi-like Pheno- and Genotype: A Review of the Literature.普拉德-威利样表型和基因型谱:文献综述
Endocr Rev. 2022 Jan 12;43(1):1-18. doi: 10.1210/endrev/bnab026.
7
Diabetes and kidney disease: emphasis on treatment with SGLT-2 inhibitors and GLP-1 receptor agonists.糖尿病与肾脏疾病:重点关注 SGLT-2 抑制剂和 GLP-1 受体激动剂的治疗作用。
Metabolism. 2021 Jul;120:154799. doi: 10.1016/j.metabol.2021.154799. Epub 2021 May 23.
8
Missed Diagnoses and Health Problems in Adults With Prader-Willi Syndrome: Recommendations for Screening and Treatment.普拉德-威利综合征成人漏诊和健康问题:筛查和治疗建议。
J Clin Endocrinol Metab. 2020 Dec 1;105(12):e4671-87. doi: 10.1210/clinem/dgaa621.
9
Dulaglutide: A Review in Type 2 Diabetes.度拉糖肽:用于 2 型糖尿病的治疗。
Drugs. 2020 Feb;80(2):197-208. doi: 10.1007/s40265-020-01260-9.
10
Dulaglutide and cardiovascular outcomes in type 2 diabetes (REWIND): a double-blind, randomised placebo-controlled trial.度拉糖肽与 2 型糖尿病患者的心血管结局(REWIND):一项双盲、随机、安慰剂对照试验。
Lancet. 2019 Jul 13;394(10193):121-130. doi: 10.1016/S0140-6736(19)31149-3. Epub 2019 Jun 9.