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KMID : 1036920120170020126
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Annals of Pediatric Endocrinology & Metabolism
2012 Volume.17 No. 2 p.126 ~ p.129
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Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome in a Child with Idiopathic Precocious Puberty
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Kang Ben
Park So-Hyun
Kim Dong-Hyun
Lee Byoung-Ick
Kim Mi-Young
Lee Ji-Eun
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Abstract
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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.
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KEYWORD
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Mayer-Rokitansky-Kuster-Hauser syndrome, Puberty, precocious
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