A Case of Mayer-Rokitansky-Küster-Hauser(MRKH) Syndrome with Imperforate Anus and Unilateral Renal Agenesis

Kim, Tae-Hyung; Kim, Jin-Hee; Kim, Soo-Yung

J Korean Soc Pediatr Nephrol > Volume 10(2); 2006 > Article

J Korean Soc Pediatr Nephrol 2006;10(2): 233-237.

편측 신장 무형성 및 쇄항을 동반한 Mayer-Rokitansky-Küster-Hauser(MRKH) 증후군 1례

김태형, 김진희, 김수영

¹부산대학교 의과대학 소아과학교실
²침례병원 소아과학교실
³부산대학교 의과대학 소아과학교실

A Case of Mayer-Rokitansky-Küster-Hauser(MRKH) Syndrome with Imperforate Anus and Unilateral Renal Agenesis

Tae-Hyung Kim, Jin-Hee Kim, Soo-Yung Kim

¹Department of Pediatrics, College of Medicine, Pusan National University
²Department of Pediatrics*, Wallace Memorial Baptist Hospital
³Department of Pediatrics*, Wallace Memorial Baptist Hospital

ABSTRACT

The clinical features of the Mayer-Rokitansky-Küster-Hauser(MRKH) syndrome include normal female secondary sex characteristics, normal genitalia, congenital absence of vagina, rudimentary or bipartite uterus, normal ovarian function and normal ovulation, 46, XX, karyotype, frequent association of renal, skeletal and other congenital anomalies. We experienced a case of a premature infant with MRKH syndrome who had imperforate anus and unilateral renal agenesis.

Key words: Mayer-Rokitansky-K | ster-Hauser syndrome | Imperforate anus | Renal agenesis