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>Journals >Ginecología y Obstetricia de México >Year 2015, Issue 03


Ávila-Vergara MA, León-Álvarez DA, López-Villegas MN, Quintero-Medrano SM, Angulo-Bueno GF, Vadillo-Ortega F
Mayer-Rokitansky-Küster-Hauser Syndrome: Two cases report
Ginecol Obstet Mex 2015; 83 (03)

Language: Espańol
References: 13
Page: 199-205
PDF: 348.71 Kb.


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ABSTRACT

Agenesia of the Müllerian ducts is a low-frequency congenital disease but with devastating effects on women’s reproductive health. In this paper we present two cases of women affected by Mayer-Rokitansky- Küster-Hauser syndrome (MRKH). First case was a 17-year-old woman with aplasia of the upper vagina and absence of uterus. No other defects were found and was classified as type 1-MRKH. Second case was 18-year-old woman with absence of uterus, escoliosis and polycystic ovary syndrome, classified as type II-MRKH. Patients were seen at the Hospital with primary amenorrhea and fully developed secondary sexual characteristics. A clinical follow-up protocol, including the use of high-resolution image studies was used for diagnosis. Diagnostic procedures and current medical approaches to the treatment of MRKH are discussed, including psychological advisory, surgical procedures and new tissue-engineering techniques.


Key words: Primary amenorrhea, uterine-vaginal aplasia, Mayer- Rokitansky-Küster-Hauser syndrome.


REFERENCIAS

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  2. Morcel K, Camboreioux L. Mayer-Rokitansky- Küster-Hauser (MRKH) syndrome. Orphanet J Rare Disease 2007;2:13 doi:10.1186/1750-1172-2-13.

  3. Oppelt PG,Lermann J,Strick R, Dittrich R, Strissel P, Rettig I, et al. Malformations in a cohort of 284 women with Mayer- Rokitansky-Küster-Hauser syndrome (MRKH). Reprod Biol Endocrinol 2012;10:57.

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  8. Mok-Lin EY, Wolfberg A, Hollinquist H, Laufer MR. Endometriosis in a patient with Mayer- Rokitansky-Küster-Hauser syndrome and complete uterine agenesis: Evidence to support the theory of coelomic metaplasia.

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  11. Brännström M, Johannesson L, Bokström H, Kvarnström N, Mölne J, Dahm-Kahler P, et al. Livebirth after uterus transplantation. Lancet 2014 (en prensa). Online: http:// dx.doi.org/10.1016/S0140-6736(14)61728-1

  12. Mattingly RF, Thompson JD.Te Linde´s Operative Gynecology. Sixth edition. Philadelphia: Lippincott Company;1985.p 355-359.

  13. Vecchietti G. Creation of an artificial vagina in Rokitansky- Küster-Hauser syndrome. Attual Ostet Ginecol 1965;11:131-147.






>Journals >Ginecología y Obstetricia de México >Year 2015, Issue 03
 

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