Egarter C, Schurz B, Fitz R, Grünberger W
I. Universitätsfrauenklinik Wien.
Geburtshilfe Frauenheilkd. 1988 Apr;48(4):235-9. doi: 10.1055/s-2008-1026494.
A characteristic and comparatively frequent malformation of the female genital tract is the congenital absence of the vagina and a rudimentary uterus, known as Mayer-Rokitansky-Küster syndrome. Since we employ the modified surgical technique according to Vecchetti we have a relatively large incidence of patients with this syndrome. Thus, a total of 15 patients were hospitalised for a thorough endocrinological examination including a GnRH test with 2 bolus injections of GnRH, TRH-test, the determination of testosterone, DHEAS, androstendione and gonadotropin as well as prolactin fluctuation over a 4-hour period. The values of 4 patients without any endocrinological disorder served as control group. 2 of the 15 patients showed an increase in androgenic hormones; 8 patients a slight hyperprolactinaemia disclosed by the TRH test. The gonadotropin fluctuation in all patients was restricted and the stimulation of the pituitary gland with GnRH bolus injections was diminished. The naloxone test led to a significant increase in peripheral gonadotropins. It remains to be determined whether this endocrinological disorder is the cause or the outcome of the rudimentary inner genitals.
女性生殖道一种典型且较为常见的畸形是先天性无阴道和残角子宫,即梅耶-罗基坦斯基-库斯特综合征。由于我们采用了根据韦凯蒂改良的手术技术,患有该综合征的患者比例相对较高。因此,共有15名患者住院接受全面的内分泌检查,包括注射两次促性腺激素释放激素(GnRH)进行GnRH试验、促甲状腺激素释放激素(TRH)试验、测定睾酮、硫酸脱氢表雄酮、雄烯二酮、促性腺激素以及4小时内催乳素的波动情况。4名无任何内分泌紊乱的患者的值作为对照组。15名患者中有2名显示雄激素水平升高;8名患者通过TRH试验发现有轻微高催乳素血症。所有患者的促性腺激素波动受限,GnRH推注对垂体的刺激减弱。纳洛酮试验导致外周促性腺激素显著增加。这种内分泌紊乱是残角内生殖器的原因还是结果,仍有待确定。