Ann Pediatr Endocrinol Metab.  2012 Jun;17(2):126-129. 10.6065/apem.2012.17.2.126.

Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome in a Child with Idiopathic Precocious Puberty

• Kang B
• Park SH
• Kim DH
• Lee BI
• Kim MY
• Lee JE

• KMID: 1803844
• DOI: http://doi.org/10.6065/apem.2012.17.2.126

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.

Keyword

Mayer-Rokitansky-Kuster-Hauser syndrome; Puberty, precocious

MeSH Terms

Abnormalities, Multiple
Adolescent
Amenorrhea
Child
Coitus
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Embryonic Development
Female
Humans
Karyotype
Kidney
Mullerian Ducts
Pregnancy
Puberty, Precocious
Somites
Spine
Uterus
Vagina
Abnormalities, Multiple
Kidney
Mullerian Ducts
Somites
Spine
Uterus
Vagina