medric medric
[닫기]
잠시만 기다려 주세요. 로딩중입니다.

Long-term follow-up on MURCS (Mullerian duct, renal, cervical somite dysplasia) association and a review of the literature

Annals of Pediatric Endocrinology & Metabolism 2019년 24권 3호 p.207 ~ 211
 ( Kim Sun ) - Inha University Hospital Department of Pediatrics

 ( Lee Yeong-Seok ) - Inha University Hospital Department of Pediatrics
 ( Kim Dong-Hyun ) - Inha University Hospital Department of Pediatrics
 ( Yang A-Ram ) - Inha University Hospital Department of Pediatrics
 ( Lee Tack ) - Inha University College of Medicine Department of Urology
 ( Hwang Seun-Deuk ) - Inha University College of Medicine Department of Internal Medicine
 ( Kwon Dae-Gyu ) - Inha University Hospital Department of Orthopaedic Surgery

Abstract


Mullerian duct aplasia-renal aplasia-cervicothoracic somite dysplasia (MURCS) association is a unique development disorder with four common types of malformations that include uterine aplasia or hypoplasia, renal ectopy or agenesis, vertebral anomalies, and short stature. The majority of MURCS patients are diagnosed with primary amenorrhea from late-adolescence. However, a few cases with MURCS association are not well diagnosed during childhood and long-term outcomes are not well reported. We report a case of an 8-year-old girl with MURCS association who presented with recurrent urinary tract infections and multiple congenital malformations, and who was followed for 10 years until adulthood. MURCS association should be considered as one of the differential diagnoses when evaluating prepubertal females with vertebral and renal malformations.

키워드

Mullerian aplasia; Mullerian duct aplasia-renal aplasia-cervicothoracic somite dysplasia association; Mayer Rokitansky-Kuster-Hauser anomaly; Child
원문 및 링크아웃 정보
 
등재저널 정보