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Mayer-Rokitanski-Kuster-Hauser (MRKH) syndrome is a rare disease. A 27-year-old woman was admitted for primary amenorrhea and cyclic pelvic pain. Magnetic resonance imaging (MRI) revealed bilateral Müllerian remnants with functioning endometrium and a pelvic mass considered to be an endometriotic cyst. Bilateral Müllerian remnants were removed, and right ovarian cystectomy was performed at laparoscopic surgery. Accurate evaluation before the operation and informed consent are necessary to treat patients with MRKH syndrome.

A case of mucin-producing adenocarcinoma arising from a mature cystic teratoma of the right ovary is reported in a 67-year-old multiparous Japanese woman. The patient underwent total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and adjuvant chemotherapy containing cisplatin, Adriamycin, and cyclophosphamide with the diagnosis of ovarian cancer, probable stage Ic. Treatment was successful even though the tumor extended through the cyst wall.

Platelet-activating factor (PAF) has been implicated in a number of reproductive processes ranging from ovulation to parturition. To examine the role of PAF in the human periovulatory processes, the PAF-acetylhydrolase (PAF-AH) activity was assayed in the follicular fluid (FF) obtained in conjunction with the in vitro fertilization and embryo transfer (IVF-ET) procedure and the activity related to oocyte maturation. The PAF-AH activity was also related to the concentrations of estradiol (E2) and progesterone (P) in FF. PAF-AH activity was significantly lower in the FFs obtained from follicles of more than 20 mm in diameter. The enzyme activity was significantly lower in the FFs of patients with a successful outcome of their pregnancies. E2 concentrations were negatively correlated with PAF-AH activities in the FFs. No correlation was found between the PAF-AH activity and concentration of P in the FF. Significantly more mature oocytes were recovered in the group who subsequently become pregnant compared to the non-pregnant group. It is suggested that PAF may be increased following follicular maturation. The increase in PAF may contribute to oocyte maturation and to the successful outcome of pregnancy following fertilization. An additional function of the increased PAF in FF may also be the stimulation of the contraction of smooth muscle in the ovary, thereby assisting the extrusion of the oocyte cumulus cell mass and signaling the completion of ovulation.

Objective To examine the role of platelet-activating factor (PAF) metabolism in the periovulatory processes. Design The PAF-acetylhydrolase activity in the follicular fluid (FF) obtained in conjunction with IVF-ET procedure was assayed and its activity was related to oocyte maturation. The PAF-acetylhydrolase activity also was related to the concentration of various ovarian hormones. Setting All patients were managed and treated at Oita Medical University Hospital, Oita, Japan. Patients The study concerned 30 women between 28 and 36 years of age with tubal infertility. Main Outcome Measure The activity of PAF-acetylhydrolase in FF was assayed as well as E 2 and P. Oocyte maturation also was evaluated. Results Platelet-activating factor-acetylhydrolase activity was decreased significantly in the FFs of patients with a successful outcome of their pregnancies compared with the nonsuccessful group. Estradiol levels were negatively correlated with PAF-acetylhydrolase activities in the FFs. No correlation was found between the PAF-acetylhydrolase activity and P concentration in the FF. Significantly more mature and less immature oocytes were recovered in the group who subsequently became pregnant compared with the nonpregnant group. Conclusions It is suggested that the decrease in PAF-acetylhydrolase activity may result in an increase of PAF in the FFs, which in turn may contribute to a successful pregnancy. The determination of PAF-acetylhydrolase activity in FF may serve as a prognostic marker for the evaluation of oocytes that are utilized in IVF-ET procedure.

We evaluated the effect of transferrin on the regulation of granulosa cell function in humans by evaluating the production of progesterone (P) in the preovulatory phase in vivo, and in cultured porcine granulosa cells in vitro. Twenty-five women treated for in vitro fertilization and embryo transfer had their serum levels of 17 beta-estradiol (E2) and P determined on the day of administration of human chorionic gonadotropin. Transferrin concentrations were also determined in ovarian follicular fluid. In an in vitro study, porcine granulosa cells were cultured in the presence of follicle-stimulating hormone (FSH) and transferrin. Serum levels of P showed a significant negative correlation with those of transferrin (r = -0.53, p0.01), whereas serum levels of E2 did not (r = 0.14). When the subjects were divided into two groups by serum P concentration (low P1 ng/ml, high Por = 1 ng/ml) serum concentrations of transferrin were significantly increased in the group with the low versus the high level of p (p0.01). Production of P by porcine granulosa cells was suppressed by transferrin in the presence of various concentrations of FSH. Increasing the dose of transferrin significantly suppressed the production of P by those cells in a dose-dependent fashion. The production of P during the preovulatory phase may be suppressed by transferrin in the granulosa cells.

An ultrasonographic examination revealed increased fetal bladder size as well as fetal bilateral hydronephrosis at 13-weeks' gestation. Diagnosis of the fetal urethral obstruction was made. Repeated ultrasonography was undertaken from the 13th to through 16th weeks of gestation. Percutaneous puncture with aspiration and laboratory analysis of fetal urine was performed at 15-weeks' gestation. The fetal renal function seemed not to be damaged by use of these antenatal procedures. Following induced abortion at 16-weeks' gestation, an autopsy showed that the fetal urethral obstruction was caused by a posterior valve, with no histological evidence of fetal renal dysplasia.

A transverse vaginal septum is the most common congenital anomaly of the vagina. Vaginal atresia is seen less frequently. There have been some variations in cases of transverse vaginal septum and vaginal atresia. This is the first report of a patient with retrohymenal vaginal atresia and perforate transverse vaginal septum.