Update on a family with hand-foot-genital syndrome: hypospadias and urinary tract abnormalities in two boys from the fourth generation.

We describe a fourth generation of involvement with hand-foot-genital syndrome. The first 3 generations of this family, which included 5 affected females and no affected males, were reported previously by Verp et al. [1983]. In the fourth generation, 2 affected males are identified. To our knowledge, the findings of bilateral vesicoureteral reflux in one boy and bilateral ureteropelvic junction obstruction in his cousin represent the first reports of urinary tract abnormalities in males with this syndrome.

An inexpensive operating microscope.

A fiberoptic colposcope was converted through minor alterations into an instrument suitable for use in gynecologic microsurgery. Magnification up to 24x may be obtained, and the extreme mobility, price consideration, and reduction in space at the operating table make it an attractive alternative to a bulky, expensive operating microscope.

Hypertension 6 weeks post partum in apparently normal women. A reappraisal and challenge.

Hypertensive blood pressure readings were recorded in 282 women from a group of 1025 consecutive patients returning for their sixth-week postpartum visit. Of the 282 women, 120 were classified as toxemic; however, 162 (58%) had had no elevation of blood pressure antepartum, intrapartum, or during the immediate postpartum period. In addition, 100 women with normal sixth-week postpartum blood pressures were randomly selected as controls. A retrospective analysis of their records showed sixth-week postpartum hypertension to occur much less frequently (13%) in nulliparous women, as compared to the preeclamptic nulliparous patients (31%). With the exception of proteinuria, all of the other data studied failed to reveal any significant abnormalities in the late postpartum hypertensive group of patients.

Factors affecting the yeild of urinary estriol.

PIP: Technical and maternal factors known to affect urinary estriol level or its measurement are discussed in view of the current use of serial estriol as an indicator of fetoplacental function. Technical artifacts can result from presence of methenamine mandelate, phenolphthalein, glucose, or high urine specific gravity. Aside from normal maternal factors such as inaccurate urine collection or variable fluid intake, estriol levels are depressed by corticosteroids and ampicillin. Pyelone phritis, anemia, hemoglobinopathy, abnormal renal status, malnutrition, and high altitude all depress estriol level or excretion. In some conditions such as pyelonephritis, amniotic estriol level may be a better indicator of fetal status.^ieng

Herpesvirus hominis type II infections in asymptomatic pregnant women.

This study was designed to determine the incidence of previous and current herpesvirus hominis Type II infections in asymptomatic pregnant women. Sera were obtained from 985 patients for detection of Type I and II herpesvirus hominis antibodies. In addition 770 cervical and 211 amniotic fluid cultures for herpesvirus hominis Type II were performed. Identification of previous herpesvirus hominis infection was determined in 352 patients (35.7%) by calculation of a Type II/I antibody index of 85 or greater. Type II herpesvirus hominis was isolated from five cervical cultures for a 0.65% antepartum incidence of infection. None of the amniotic fluid cultures demonstrated virus. The significance of these findings is discussed.

Gynecologic manifestations of chronic graft-versus-host disease.

Gynecologic manifestations of the clinicopathologic syndrome known as graft-versus-host disease seen in 5 patients treated with allogeneic bone transplantation are presented. The clinical symptoms and significant problems found in association with graft-versus-host disease may include extensive sclerosing vaginitis and stricture formation. Treatment and possible preventive therapy are discussed.

Factors affecting donor artificial insemination success rates.

Pregnancy was achieved at least once by 75 of 100 women treated with artificial insemination donor (AID). In all, 94 gestations were recorded, with a mean time to achieve pregnancy of 3.3 cycles. Success rates were studied relative to ovulatory function, cervical mucus parameters, previous reproductive history, and following pelvic surgery. A life-table analysis of conception according to actual inseminations performed during each cycle gave a 61% conception rate within 6 months, including patients who discontinued therapy after only one treatment cycle. Clomiphene citrate was used freely for ovulation induction. These patients took longer to conceive (5.5 cycles), but had an abortion rate similar to that of the general group. An effect of cervical mucus on the time necessary to initiate pregnancy was not demonstrated. Six of seven patients who had undergone reparative surgery for restoration or promotion of fertility conceived. The highest number of conceptions with double AID was found on cycle days 13 and 15.

Factors related to preferences in gamete donor sources.

OBJECTIVE: To survey fertile and infertile individuals concerning attitudes about donor insemination and ovum donation and to analyze whether gender, experience with infertility, and religion influences: [1] preference for choosing gamete donor over adoption or child-free living; [2] choice of selecting a known or anonymous donor; or [3] choice of disclosure or nondisclosure to any offspring. DESIGN: Patients in an infertility clinic and obstetrics and gynecology practice were asked to complete surveys. PARTICIPANTS: Three hundred nineteen individuals who completed surveys. MAIN OUTCOME MEASURES: A 14-item questionnaire (created for this study) with a demographic information section. RESULTS: No gender differences were observed for preference to use donor gametes, to adopt, or to live child free within the infertility group. In the obstetrics and gynecology group, men preferred adoption more than women and were less likely to use a donor. Choice of whether to use a donor, which donor source, and whether to disclose the use of a donor was influenced by gender, experience with infertility, and religion. CONCLUSIONS: Gender, experience with infertility, and religion influence an individual's choice in family building options as well as what information will be shared with the child. There is a wider acceptance of using known donor sources than has heretofore been seen and these options should be explored.

Resectoscopic myomectomy.

OBJECTIVE: To evaluate safety and efficacy of resectoscopic myomectomy for control of menorrhagia, dysmenorrhea, and/or reproductive wastage. DESIGN: Retrospective consecutive patient follow-up. SETTING: Private practice, outpatient, hospital. PATIENTS: All patients (92) having resectoscopic myomectomy between September 1, 1986 and December 31, 1989. INTERVENTIONS: Resectoscopic myomectomy with or without concomitant procedures such as endometrial ablation. MAIN OUTCOME MEASURES: Assessment of dysmenorrhea, menorrhagia, and reproductive outcome after surgery. RESULTS: Elimination of dysmenorrhea in 24 of 28, absence of menorrhagia in 65 of 80, and pregnancy in 10 of 13 with two abortions in a total of 11 gestations. One patient was admitted and treated with one unit of autologous blood and mechanical tamponade with an intrauterine balloon. Three cases of uterine perforation had no sequelae. One patient had mild endometritis responding to outpatient antibiotics. Two cases of leiomyosarcoma were diagnosed histologically and accounted for two of the three hysterectomies noted during follow-up. CONCLUSIONS: Resectoscopic myomectomy is a safe, effective, and relatively simple outpatient surgical procedure.

Long-term management of adenomyosis with a gonadotropin-releasing hormone agonist: a case report.

Leuprolide acetate was used to produce a constant hypoestrogenic environment in a young patient with histologically confirmed adenomyosis. Conservative medical therapy was initiated because of the patient's complaint of severe dysmenorrhea coupled with her strong desire for uterine conservation. The initial daily subcutaneous dose was eventually converted to monthly intramuscular depot formulation for patient convenience. A dramatic therapeutic response was observed with each course of therapy. This included a marked reduction in uterine size, amenorrhea, and complete resolution of pelvic pain. Cyclic use of an OC agent following LA was associated with a return of symptoms and uterine growth. The patient did, in fact, conceive immediately on cessation of analogue therapy.

Interruption of pregnancy by prostaglandin 15-methyl F2alpha.

The current study was formulated to investigate the abortifacient activity of prostaglandin 15-methyl F2alpha (15-methyl PGF2alpha) administered intramuscularly to 80 healthy women with gestations between 8 and 22 weeks. Goals were the establishment of an effective dosage schedule and assessment of the incidence and severity of side effects. All 80 gravidas were aborted, with a mean time to abortion of 15.70 hours (SD, 6.52). Gastrointestinal side effects occurred in 89% of the patients; temperature elevations greater than or equal to 100.6 degrees F were noted in 14 cases. No other significant complications were encountered. Transabdominal intra-amniotic pressure monitoring indicated the need to administer the drug at 2-hour intervals. The 15-methyl PGF2alpha patients were matched for parity and gestational length with 80 gravidas aborted with PGE2 20-mg vaginal suppositories. The difference in interval to abortion in the two groups was not statistically significant. While gastrointestinal side effects were more common with 15-methyl PGF2alpha, the frequency of drug-induced temperature elevations was reduced.

PIP: It has been reported that intra-amniotic administration of 15-methyl PGF2a (prostaglandin F2alpha) for abortion results in a high level of uterine contractility, a high rate of success, and a low incidence of side effects. This study assesses the abortifacient activity of 15-methyl PGF2alpha administered intramuscularly in 80 healthy women aged 14 to 40 with gestational ages between 8 and 22 weeks. 56 patients were nulliparious. Transabdominal intra-amniotic pressure monitoring was used to measure uterine contractility and to establish an effective dose schedule. 350 to 520 mcg of 15-methyl PGF2a were administered intramuscularly at 2-hour intervals until the onset of abortion. Intravenous oxytocin was infused in 6 cases to facilitate passage of retained placental tissue. Medications were given to reduce diarrhea, vomiting, and pain. All patients aborted. Total drug dose ranged from 900 to 8400 mcg; mean dose was 3254.32 mcg. Duration of treatment ranged from 4 to 34 hours. Induction-abortion time ranged from 5.5 to 35 hours, with mean interval of 15.70 hours. 89% of the patients experienced gastrointestinal side effects. 14 patients had temperature elevation more than or equal to 100.6 degrees F. There were no significant complications. The 15-methyl PGF2a patients were matched with 80 gravidas who had abortion using PGE2 20 mg vaginal suppositories. There were no statistical differences in interval to abortion between the 2 groups.

Abortion of early pregnancy on an outpatient basis using Silastic 15(S)-15-methyl prostaglandin F2alpha vaginal devices.

Hormonal changes associated with the use of 15(S)-15-methyl prostaglandin F2alpha in a Silastic device as an abortifacient were evaluated during very early first-trimester pregnancy. Five of six patients aborted completely with drug alone. Measurement of plasma samples demonstrated rapid drug absorption with apparent effective concentrations of 600 to 3000 pg/ml but without a pattern of sustained release and absorption. Routine serial determination of both serum chorionic gonadotropin (hCG) and beta-subunit hCG were used to assess the efficacy of treatment. Although more sensitive, beta-hCG measurement offered no advantage clinically over the standard hCG assay. Plasma progesterone values also reflected success or failure of the technique. Vomiting and diarrhea continue to be problems associated with the use of these compounds. The Silastic device may be the forerunner of a self-administered early abortifacient. Continued efforts are necessary to elucidate mechanisms of action and to find an analog specific for myometrial stimulation.

The luteal phase after ovulation induction with human menopausal gonadotropin and one versus two doses of a gonadotropin-releasing hormone agonist.

OBJECTIVE: To use a GnRH agonist (GnRH-a) to induce ovulation after priming with exogenous hMG. DESIGN: Prospective, randomized double-blind protocol using one or two doses of intranasal nafarelin. SETTING: Office-based ovulation induction program. PATIENTS, INTERVENTIONS: Infertile women not conceiving after use of clomiphene citrate for at least 6 months who were given hMG and nafarelin. No luteal support was given. MAIN OUTCOME MEASURES: Serum concentrations of FSH, LH, E2, and P acutely and at 6 days after GnRH-a administration. Duration of the luteal phase was assessed. RESULTS: Ovulation with elevation of both FSH and LH was achieved. The two-dose regimen was more effective than one dose for sustained LH release. Luteal phase P values and luteal phase duration were both less than usually seen with gonadotropin hCG therapy in the absence of luteal phase support. CONCLUSIONS: Ovulation induction with GnRH-a after hMG priming produces unacceptable luteal phase cycles in the absence of hormonal support.

The human sperm-hamster egg penetration assay: prognostic value.

Males from 227 infertile couples were evaluated using the human sperm-hamster egg penetration assay (SPA). Indications for the SPA were abnormal semen analyses, poor postcoital tests, documented autologous sperm antibodies, and long-term unexplained infertility. Normal results defined as greater than or equal to 11% penetration were seen in 58.6% of couples. Penetration rates of 1% to 10% were observed in 25.6%, and 15.9% failed to penetrate any of the oocytes. During the follow-up period, with a mean of 17.9 months, 26.9% conceived at least once with or without treatment. Monthly fecundity was 0.014 for normal SPA patients and 0.007 for the abnormal SPA group when calculated by the Kaplan-Meier survival curve with Cox correction for unequal follow-up. The monthly fecundity rate at any time during the 30-month interval of follow-up was twice as great for men with normal SPA values as for those with abnormal values, regardless of male or female diagnosis or therapy indicating the prognostic value of the SPA in an infertile population.

Insemination data on men with varicoceles.

OBJECTIVE: To determine possible benefits of sperm processing and intrauterine insemination (IUI) for a group of men with a varicocele history who had not achieved a pregnancy by natural coitus (mean duration of infertility 42.2 months). DESIGN: A retrospective study including infertile men with varicoceles who were classified by their semen analyses and sperm penetration assays (SPAs). SETTING: Private practice of infertility. PATIENTS: Seventy-one infertile couples. The husbands had a varicocele history and were grouped into four clinical categories (14 untreated, 5 medical treatment, 34 varicocelectomies, and 18 varicocelectomies plus medical treatment). The wives were studied or treated before IUI. INTERVENTIONS: Varicocelectomies were performed on the males when indicated. Female studies included laparoscopies as indicated intrauterine insemination with Tomcat catheter (Sherwood Medical, St. Louis, MO) was performed in all cases. MAIN OUTCOME MEASURES: Overall pregnancy rates (PRs) and fecundity rates with sperm processing and IUI. RESULTS: Six pregnancies occurred with 66 cycles of sperm processing and IUI among 28 men with normal SPAs (PR 21%, fecundity rate = 0.09). In contrast, there were no pregnancies with 121 cycles among 43 varicocele patients with abnormal SPA results or with low values for all three semen parameters. CONCLUSIONS: Sperm processing and IUI may be beneficial for selected patients with varicoceles who had not achieved a pregnancy by coitus.

Gynecologic endoscopic gas embolism.

OBJECTIVE: To alert gynecologic surgeons to the risk of room air embolism during endoscopy. DESIGN: Case reports. SETTING: Medico-legal consultations. PATIENTS: Five women having endoscopic procedures. INTERVENTIONS: Endoscopy followed by emergency resuscitative measures. RESULTS: Morbidity and mortality. CONCLUSIONS: The risk of room air embolism may be lessened by attention to the operative technique and by monitoring the end tidal carbon dioxide levels.

Serial beta-subunit human chorionic gonadotropin doubling time as a prognosticator of pregnancy outcome in an infertile population.

Prospective evaluation through the use of radioimmunoassay of the beta-subunit of human chorionic gonadotropin (beta-hCG) in blood samples obtained during the first 30 days of gestation was performed on an infertile population at high risk for pregnancy loss. Four hundred and fourteen samples in 281 pregnancies were analyzed. On the basis of single, random beta-hCG samples in asymptomatic patients, 77% of successful pregnancies and 59% of abortions were correctly identified. On the basis of beta-hCG doubling time (mean 2.2 days +/- 1.0 [2 SD]) computed from serial sampling, again in asymptomatic patients, 88% of successful pregnancies and 76% of abortions were correctly identified. beta-hCG doubling time appears to provide a reliable method of evaluating early pregnancy prognosis with significantly greater ability to identify problem pregnancies within the first 30 days of gestation than does single random hCG values.

Gestational carrier pregnancy.

OBJECTIVE: To evaluate the results of a gestational carrier program in terms of pregnancy rates in fresh and cryopreserved cycles. DESIGN: Retrospective analysis. SETTING: Private IVF program. PATIENT(S): Women with uterine or medical reasons for unsuccessful gestation. INTERVENTION(S): In vitro fertilization of oocytes with fresh or cryopreserved ET to gestational carriers. MAIN OUTCOME MEASURE(S): Pregnancy rates and deliveries. RESULT(S): A PR of 56.3% and a cycle rate of 30.8% was achieved in each patient <40 years of age in 117 cycles of fresh or cryopreserved ET. There were no pregnancies for nine patients >40 years of age in 27 cycles. CONCLUSION(S): Carrier gestation offers a satisfactory solution to childlessness caused by uterine or major medical factors.

Leuprolide acetate-prepared in vitro fertilization-gamete intrafallopian transfer cycles: efficacy versus controls and cost analysis.

OBJECTIVE: To evaluate the effect of gonadotropin-releasing hormone agonist (GnRH-a) when given through stimulation with gonadotropins versus stopping it as stimulation was initiated versus a control group unprepared in this manner. Also, to construct a cost analysis for this type of therapy in an in vitro fertilization-gamete intrafallopian transfer program (IVF-GIFT). DESIGN: Prospective, consecutive, randomized, with a control group. SETTING: An out-patient IVF-GIFT program. PATIENTS: One hundred new patients entering an IVF-GIFT program. INTERVENTIONS: In vitro fertilization or GIFT cycles with or without preparation (pituitary suppression) by GnRH-a administration in which suppression was continued or halted as gonadotropins were begun. MAIN OUTCOME MEASURES: Cancellation rates, ampules, and days of gonadotropin use, costs, estradiol (E2) level, egg numbers and quality, and pregnancy rates. RESULTS: Stopping GnRH-a (leuprolide acetate) therapy as stimulation was begun did not lessen the suppressive effect in reduction of spontaneous luteinizing hormone surge as seen with continued use of agonist. In contrast, the control group had a 30.3% cancellation rate before ovum retrieval. The E2 and egg data among the groups were similar, but as expected, agonist use led to the need for more and longer duration of gonadotropin stimulation. Pregnancy rates were not different by group. Costs were approximately even by the third initiated cycle.

Characteristics and attitudes of parents of children born with the use of assisted reproductive technology.

OBJECTIVE: To explore the medical issues, attitudes, concerns, and choices that parents have about their children born with the use of assisted reproductive technology (ART). DESIGN: Retrospective and prospective survey. SETTING: An academic medical center and a private practice. PATIENT(S): Participants who conceived and were delivered of infants in two ART programs. INTERVENTION(S): A total of 373 patients were mailed an anonymous survey, a consent form, and the Parent Child Relationship Inventory. The rate of response was approximately 49% for clinic A and 33% for clinic B. MAIN OUTCOME MEASURE(S): Pregnancy outcomes and attitudes about parenting. RESULT(S): Respondents' major concerns during pregnancy revolved around miscarriage and the infant's health; complications occurred in 38.9% of first pregnancies. Parents believed that their children were more appreciated, that their children were not emotionally different, that ART did not create ongoing medical or emotional problems, and they were not overprotective as parents. Gender differences were statistically significant on attitudinal variables. CONCLUSION(S): Parents had concerns about pregnancy. Overall, men and women felt positive about ART and their parenting. The ART experience is associated with complex choices, attitudes, and emotions.