Purpose: This study aimed to analyze the hormone profiles, to detect the rate of hyperandrogenemia and to investigate the potential effect of Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) on ovarian reserve, as reflected by the serum Anti-Mullerian hormone (AMH) levels. Clinical implications were analyzed by including our own experiences with three patients after ovarian stimulation in preparation for uterus transplantation.
Methods: Serum samples of 100 patients with MRKHS (50 patients with MRKHS type 1 and 50 with type 2) were analyzed and compared to 50 individually age-matched healthy controls. Blood samples for hormone analyses were collected routinely during the clinical visit.
Results: The mean age was 20.0 years for MRKHS type 1, MRKHS type 2 and healthy controls. Compared to healthy controls, there was no significant difference in AMH values in the MRKH patients. As shown in previous studies, the proportion of hyperandrogenemia without clinical symptoms was significantly higher in MRKHS type 1 (52%; p < 0.001) and type 2 (56%; p < 0.001) patients when compared to age-matched controls. In preparation for uterus transplantation, three patients were stimulated with FSH/hMG for mean 14.2 days and the mean number of aspirated oocytes was 13.2 (3-22), while 8.3 (2-10) oocytes could be fertilized and cryopreserved. The mean fertilization rate was 51.2% (30-67%).
Conclusion: The rate of hyperandrogenemia was significantly higher in MRKH patients compared to healthy age-matched controls. Though, ovarian reserve (AMH level) was not reduced compared to controls. Future studies are needed to identify optimal ovarian stimulation protocols as well as to implement a systematic multicenter reporting system.
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http://dx.doi.org/10.1007/s00404-017-4596-1 | DOI Listing |
Clin Case Rep
December 2024
Department of Obstetrics and Gynecology Tribhuvan University, Institute of Medicine Kathmandu Nepal.
Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is a rare congenital disorder characterized by agenesis of the uterus and upper two-thirds of the vagina. It affects around 1 in 4000-5000 females and is of two types: type 1, also known as isolated Müllerian aplasia or Rokitansky, which involves only uterovaginal agenesis, and type 2, presents as uterovaginal agenesis along with renal, cardiac, and other organ anomalies. Despite the absence of vaginal and uterine structures, individuals with MRKHS typically present with normal secondary sexual characteristics and ovarian functions.
View Article and Find Full Text PDFJ Clin Med
January 2024
Department of Obstetrics and Gynecology, University of Tübingen, 72076 Tübingen, Germany.
The Mayer-Rokitansky-Küster-Hauser syndrome is characterized by aplasia of the uterus and upper two-thirds of the vagina. While it can appear as an isolated genital malformation, it is often associated with extragenital abnormalities, with little still known about the pathogenetic background. To provide an overview of associated malformations and syndromes as well as to examine possible ties between the rudimentary tissue and patient characteristics, we analyzed a cohort of 469 patients with MRKHS as well as 298 uterine rudiments removed during surgery.
View Article and Find Full Text PDFCase Rep Obstet Gynecol
August 2023
Department of Obstetrics and Gynecology, Tottori University Faculty of Medicine, Yonago 683-8504, Japan.
Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is a rare congenital anomaly of the genital tract. Since the secretion of sex hormones from the ovaries is preserved, leiomyomas and adenomyomas, which are estrogen-dependent diseases, may develop from the uterine remnant. In contrast, patients with myotonic dystrophy type 1 (DM1), the most common dystrophy in adults, are considered to be at high risk for benign tumors of the female reproductive system, such as uterine leiomyomas and ovarian cysts.
View Article and Find Full Text PDFAnn Transl Med
October 2022
Department of Difficult Gynecological Disease, Xi'an People's Hospital (Xi'an Fourth Hospital), Xi'an, China.
Background: Although traditional multi-incision laparoscopic surgery is minimally invasive, it can still leave surgical scars on the lower abdomen. This study reports a modified Vecchietti-laparoendoscopic single-site (MVLESS) procedure using self-made instruments to create a neovagina for Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) and to determine the efficacy and safety.
Methods: We conducted a retrospective comparative cohort study analysis of the clinical data from MRKHS patients treated in the Department of Difficult Gynecological Disease at Xi'an People's Hospital between January 2010 and December 2020.
Ann Med Surg (Lond)
October 2022
Department of Gynecology and Obstetrics, Lumbini Provincial Hospital, Lumbini, 32914, Nepal.
Introduction: Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKHS) is a rare congenital disorder with an incidence of 1 in 5000 females. It is characterized by uterovaginal aplasia with normal secondary sexual characteristics and genetic karyotype 46XX. The exact etiology of MRKH syndrome is not known.
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