{
    "abstract": "Evaluation of existing knowledge of etiopathogenesis, clinical manifestations and treatment options to increase quality of life in women with Mayer-Rokitansky-K\u00fcster-Hauser syndrome (MRKH).\nReview article.\nDepartment of Obstetrics and Gynaecology, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague.\nLiterature search using the Web of Science, Google Scholar and Medline databases with keywords (absolute uterine infertility factor, AUFI, Mayer-Rokitansky-K\u00fcster-Hauser syndrome, MRKH, uterine transplantation) and analysis of articles published in impact and reviewed journals.\nMRKH syndrome is defined as congenital agenesis of the upper two-thirds of vagina and uterus in women with normal secondary sexual characteristics and female karyotype (46, XX). The incidence of the syndrome is 1 : 4500 births of female sex children. It is the second most common cause of primary amenorrhea. Recent research has focused on elucidating the genetic origin of the disease, focusing on the research of candidate genes that could be participating in the genesis of M\u00fcllerian ducts and their derivatives.\nMRKH syndrome now appears as a\u00a0multifactorial congenital developmental defect based on a\u00a0combination of genetic predisposition and environmental factors. Modern medicine can help girls with MRKH syndrome to a\u00a0quality sexual life. It is also able to offer different possibilities of achieving motherhood. In the future, however, further research is needed, in particular on the etiology and pathogenesis of this syndrome to detect a\u00a0possible genetic basis of the disease.",
    "authors": [
        {
            "affiliation": null,
            "firstname": "R",
            "initials": "R",
            "lastname": "Chmel"
        },
        {
            "affiliation": null,
            "firstname": "Z",
            "initials": "Z",
            "lastname": "Pastor"
        },
        {
            "affiliation": null,
            "firstname": "M",
            "initials": "M",
            "lastname": "Mu\u017e\u00edk"
        },
        {
            "affiliation": null,
            "firstname": "T",
            "initials": "T",
            "lastname": "Brtnick\u00fd"
        },
        {
            "affiliation": null,
            "firstname": "M",
            "initials": "M",
            "lastname": "Nov\u00e1\u010dkov\u00e1"
        }
    ],
    "conclusions": "MRKH syndrome now appears as a\u00a0multifactorial congenital developmental defect based on a\u00a0combination of genetic predisposition and environmental factors. Modern medicine can help girls with MRKH syndrome to a\u00a0quality sexual life. It is also able to offer different possibilities of achieving motherhood. In the future, however, further research is needed, in particular on the etiology and pathogenesis of this syndrome to detect a\u00a0possible genetic basis of the disease.",
    "copyrights": null,
    "doi": null,
    "journal": "Ceska gynekologie",
    "keywords": [
        "AUFI",
        "MRKH",
        "Mayer-Rokitansky-K\u00fcster-Hauser syndrome",
        "absolute uterine factor infertility",
        "uterus transplantation"
    ],
    "methods": null,
    "publication_date": "2019-12-13",
    "pubmed_id": "31826637",
    "results": "MRKH syndrome is defined as congenital agenesis of the upper two-thirds of vagina and uterus in women with normal secondary sexual characteristics and female karyotype (46, XX). The incidence of the syndrome is 1 : 4500 births of female sex children. It is the second most common cause of primary amenorrhea. Recent research has focused on elucidating the genetic origin of the disease, focusing on the research of candidate genes that could be participating in the genesis of M\u00fcllerian ducts and their derivatives.",
    "title": "Syndrome Mayer-Rokitansky-K\u00fcster-Hauser - uterine and vaginal agenesis: current knowledge and therapeutic options.",
    "xml": "<Element 'PubmedArticle' at 0x78850387d030>"
}