Primary Amenorrhea with Mayer-Rokitansky-Kuster-Hauser Syndrome

نویسندگان

چکیده

BACKGROUND: Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare disorder described as aplasia or hypoplasia of uterus and vagina due to an early arrest in the development Mullerian ducts. The etiology MRKH remains uncertain: Although at beginning, it was mentioned that this result sporadic abnormalities. It has recently been assumed genetic background cause increasing number familial cases. CASE REPORT: A 29-year-old female patient presented with amenorrhea. She also normal pubic axillary hair breast development. Routine investigations were normal. Genitalia inspection shows well developed, labia majora seen. Inspeculo show vaginal wall smooth, but portio not Vaginal examination result: unpalpable, both adnexa normal, parametrium laxed, Douglas pouch protruded. Rectal difficult identify. Transvaginal sonography revealed absence uterus, ovaries within diagnosed uterine aplasia. With this, provisional diagnosis made primary amenorrhea suspected syndrome, referred for follicle-stimulating hormone, estradiol, testosterone, prolactin levels by enzyme-linked immunosorbent assay limit followed karyotyping 46, XX. CONCLUSIONS: We report woman From our examination, syndrome. Uterus transplant alternative treat possibility get pregnant.

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ژورنال

عنوان ژورنال: Open Access Macedonian Journal of Medical Sciences

سال: 2021

ISSN: ['1857-9655']

DOI: https://doi.org/10.3889/oamjms.2021.6306