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儿童先天性肾发育不全/不良相关综合征的相关畸形和肾脏预后

李娜 毛建华

中国医学创新2024,Vol.21Issue(5):169-174,6.
中国医学创新2024,Vol.21Issue(5):169-174,6.DOI:10.3969/j.issn.1674-4985.2024.05.037

儿童先天性肾发育不全/不良相关综合征的相关畸形和肾脏预后

Associated Malformations and Renal Prognosis in Children with Congenital Renal HypoplasiaDysplasia Related Syndromes

李娜 1毛建华2

作者信息

  • 1. 浙江大学医学院 浙江 杭州 310058
  • 2. 浙江大学医学院附属儿童医院肾脏内科 浙江 杭州 310052
  • 折叠

摘要

Abstract

Renal hypoplasia/dysplasia(RHD)in children is often complicated by other congenital renal and urinary tract malformations and extrarenal malformations.Specific combinations of malformations are called RHD-related syndromes.These include monogenic syndromes such as renal coloboma syndrome,renal cyst-diabetic syndrome,hypoparathyroidism-deafness-renal dysplasia(HDR)syndrome,Townes-Brocks syndrome,branchio-oto-renal syndrom,etc.Syndromes with no single causative gene such as oblique vaginal septum syndrome,Mayer-Rokitansky-Küster-Hauser(MRKH)syndrome,Zinner syndrome,prune belly syndrome,VURD syndrome,VACTERL association,etc.The associated malformations and renal prognosis of different RHD-related syndromes vary greatly.This review focuses on the relatively common RHD-related syndromes in clinical practice.

关键词

肾发育不全/肾发育不良/多发畸形/肾功能

Key words

Renal hypoplasia/Renal dysplasia/Multiple malformations/Renal function

引用本文复制引用

李娜,毛建华..儿童先天性肾发育不全/不良相关综合征的相关畸形和肾脏预后[J].中国医学创新,2024,21(5):169-174,6.

中国医学创新

1674-4985

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