Associated Malformations and Renal Prognosis in Children with Congenital Renal HypoplasiaDysplasia Related Syndromes
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.
Renal hypoplasiaRenal dysplasiaMultiple malformationsRenal function
国家科技期刊平台
NSTL
万方数据
维普
