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Ann Pediatr Endocrinol Metab > Volume 17(2); 2012 > Article
DOI: https://doi.org/10.6065/apem.2012.17.2.126   
Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome in a Child with Idiopathic Precocious Puberty.
Ben Kang, So Hyun Park, Dong Hyun Kim, Byoung Ick Lee, Mi Young Kim, Ji Eun Lee
1Department of Pediatrics, Inha University School of Medicine, Incheon, Korea. anicca@inha.ac.kr
2Department of Obstetrics and Gynecology, Inha University School of Medicine, Incheon, Korea.
3Department of Radiology, Inha University School of Medicine, Incheon, Korea.
Abstract
Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare congenital disorder characterized by the congenital absence or hypoplasia of the uterus and the upper two thirds of the vagina due to mullerian duct malformation during embryogenesis. MRKH syndrome usually presents as primary amenorrhea in adolescence in females showing normal development of secondary sexual characteristics and a normal 46, XX karyotype. For this reason, MRKH syndrome usually remains undiagnosed until primary amenorrhea or difficulty in sexual intercourse occurs. In this study, a case of MRKH syndrome diagnosed in a child with idiopathic precocious puberty is reported.
Keywords: Mayer-Rokitansky-Kuster-Hauser syndrome;Puberty, precocious


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