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Pregnancy in women with Noonan syndrome: report of two cases.

BACKGROUND: Noonan syndrome is similar phenotypically to Turner syndrome, accounting for one in 1000-2500 live births. Two thirds of patients have cardiac anomalies, half with pulmonary stenosis. Two cases of Noonan syndrome in pregnancy are presented, each woman with a different cardiac anomaly. CASES: The first patient sought preconception counseling for pulmonary stenosis. She conceived within a year and had a cesarean because of prolonged rupture of membranes, transverse lie, and variable decelerations. The second patient presented at 14 weeks' gestation with an unrepaired coarctation of the aorta. She had an assisted vaginal delivery at 38 weeks. CONCLUSION: Successful pregnancy was possible in women with Noonan syndrome. A coordinated multidisciplinary team approach improved the likelihood of a successful outcome.

Adult↗

Antibiotic use in the emergency department. IV: Single-dose therapy and parenteral-loading dose therapy.

There are a number of infectious diseases that can be treated efficaciously with a single dose of an antimicrobial agent. Other infections that can be treated with oral antibiotics on an outpatient basis may resolve more quickly if a parenteral loading dose is given in the emergency department (ED) prior to discharge. This article reviews the supporting literature and indications for single-dose and parenteral first-dose-loading antimicrobial therapy in the ED. This approach may be appropriate for such diverse infections as streptococcal pharyngotonsillitis, otitis media, urinary tract infections, chlamydial genital infections, vaginitis due to yeast, bacteria, or trichomoniasis, pneumonia, gonorrhea and pelvic inflammatory disease, and pediatric fever without a source.

Acute Disease↗

Introducing external cephalic version at 36 weeks or more in a district general hospital: a review and an audit.

OBJECTIVE: To audit the effect of introducing external cephalic version at > or = 36 weeks on breech delivery rates and modes, and to assess factors that affect external cephalic version success rates. DESIGN: A prospective unblinded study over 12 months of factors affecting external cephalic version success. A retrospective review of breech deliveries for 12 months before introducing external cephalic version and the first 12 months of practising external cephalic versions. SETTING: Royal United Hospital, Bath. POPULATION: One hundred and three women (> or = 36 weeks) with breech presentation booked for external cephalic version, and 324 women with a singleton breech presentation at delivery between November 1992 and October 1994. METHODS: External cephalic version attempted on 89 women. Tocolysis was used on 41 of these women. MAIN OUTCOME MEASURES: External cephalic version success rate. Breech presentation rates (suitable for external cephalic version) at delivery. Delivery mode rates for breeches. RESULTS: External cephalic version was successful in 39% of women. The breech presentation rate (external cephalic version suitable) at delivery fell from 2.7% to 2.4% of all deliveries. The vaginal breech delivery rate fell from 0.98% to 0.51%. The caesarean section rate was 63% for breeches (external cephalic version suitable) before external cephalic version introduction, and 47% among women who had an attempted external cephalic version. CONCLUSIONS: Attempted external cephalic version reduces the woman's risk of vaginal breech delivery and caesarean section. It provides individual women with a third management option and the possibility of avoiding a vaginal breech delivery or a caesarean section. To avoid one caesarean section takes 5.9 attempted external cephalic versions. This approach is expensive in operator time. Operator, placental site, position of fetal back and amniotic fluid index had a significant effect on the success of external cephalic version.

Breech Presentation↗

[Urinary and anal incontinence: a taboo subject].

The prevalence of urinary incontinence is estimated at about 6 % in the overall population and may be as high as 40 % in women after menopause. For many women with stress incontinence, the new vaginal tape operations appear to offer cure rates comparable to those achieved with conventional procedures, but with less invasiveness and a shorter hospital stay. Pelvic organ prolapse and stress incontinence are two separate conditions which frequently coincide. Correcting prolapse alone may not cure stress incontinence - and may even unmask latent incompetence of the urethral sphincter. Depending on which definition is used, the prevalence of anal incontinence is between 1 and 5 %. In women, injuries sustained at vaginal delivery appear to be the most important risk factor. The prevalence of combined urinary and anal incontinence may approach 5-10 %. All women we see as gynecologists and obstetricians should be asked about symptoms of incontinence, and women with symptoms should be evaluated specifically and offered appropriate measures to improve their quality of life.

Cross-Sectional Studies↗

A systematic arrangement of laparoscopic total abdominal hysterectomy: a new technique.

This sequential, prospective, observational clinical trial evaluated a systematic arrangement of laparoscopic total abdominal hysterectomy and prophylactic, retroperitoneal posterior culdoplasty with vaginal vault suspension surgical techniques by suturing method. The uterus was extirpated laparoscopically in 25 consecutive patients using an extra- and intra-corporeal two-turn flat square knot method. Upon completion of uterine excision, a new prophylactic laparoscopic technique of retroperitoneal posterior culdoplasty and vaginal vault suspension were initiated to prevent pelvic relaxation. Retroperitoneal culdoplasty was performed using the anterior rectal fascia, the posterior uterovaginal fascia, and the deep layer retroperitoneal of the uterosacral ligaments. Vaginal vault suspension was performed using posteriorly the deep layer of the uterosacral ligaments; from a lateroposterior aspect, the vaginal vault was suspended to the cardinal ligaments bilaterally, and anteriorly, the vesicouterine fascia provided support for the vaginal apex. A systematic arrangement of surgical steps was evaluated. All predetermined samples of laparoscopic total abdominal hysterectomy with posterior retroperitoneal culdoplasty and vaginal vault suspension were accomplished in a prearranged systematic order. Neither technical failure nor conversion to laparotomy or transvaginal approach was encountered. This technique expedites uterine extirpation and prophylactic pelvic reconstruction with a low complication rate, can be executed with no transvaginal approach, and eliminates the morbidity and mortality associated with laparotomy itself.

Adult↗

Unreduced obturator dislocation of the hip. A case report.

A patient with a late unreduced obturator dislocation of the hip is reported and the literature reviewed. The diagnosis was confirmed by vaginal examination. An open reduction was necessary and it is suggested that this is best achieved through an anterior approach.

Adolescent↗

Reducing cesarean sections at a teaching hospital.

A department-wide effort to reduce the cesarean section rate at the University Medical Center of Jacksonville (Florida) began in 1987. University Medical Center, a teaching hospital with approximately 4500 annual deliveries, serves an almost exclusively indigent obstetric population. Overall, the cesarean section rate declined from 28 per 100 deliveries in 1986 to 11 per 100 in 1989. Decreasing the number of repeat cesarean sections played an important role in reducing total cesarean deliveries. In 1986 32% of women with prior cesarean sections underwent a trial of labor. By 1989 this proportion had increased to 84%. In 1986 65% of women undergoing a trial of labor were delivered vaginally. By 1989 this proportion had increased to 83%. For these reasons the proportion of patients who had repeat cesarean sections dropped from 8% in 1986 to 3% in 1989. Changing approaches to the evaluation and management of dystocia and fetal distress also helped to lower the overall cesarean section rate. In 1986 cesarean sections for at least one of these two indications accounted for 14% of all deliveries. By 1989 this percentage had dropped to 4%. Because selective criteria for vaginal delivery of fetuses in breech presentation were maintained, incremental increased rates of vaginal breech delivery had only a minimal impact on lower overall cesarean section rates. The reduction in the number of cesarean sections was accomplished without compromising neonatal outcomes. In fact, during this 4-year period neonatal mortality rates actually decreased; neonatal morbidity rates remained stable. Our experience suggests that cesarean section rates can be substantially reduced without compromising the newborn.

Birth Weight↗

[Vaginal fibromas (author's transl)].

The author describes the characteristics of vaginal fibromas, as observed in one personal case and after a rview of the published literature. These tumors, which affect women during the period of genital activity, are not of frequent occurrence, and the principal signs are dyspareunia and metrorrhagia. The histological appearance of the tumours is very similar to that of uterine fibromas, but their pathogenicity appears to be different, and they progress in a similar manner to that of benign tumors. Because of the existence of a cleavage line they can be easily enucleated, the main therapeutic problem being the choice of the surgical approach to be employed.

Adult↗

[Why women consult sexologists: an approach for practicing physicians].

Female sexual dysfunctions are less known than male ones because women are more discrete and introverted. Female genitals are mostly internal and women live sex more cerebrally and sentimentally than men. Still half the patients my current sexological consultation are women. They seek help due for lack of desire, frigidity, coital or clitoridal anorgasmy, dyspareuny, vaginism and problems related to pregnancy or the post-partum period. This article describes how to construct a precise clinical etiology following a "5 circles" scheme, which will help to determine the most adequate therapeutic approach.

Female↗

[Cortisol contents in maternal and umbilical artery blood during delivery of low birth weight fetuses].

Cortisol was measured in 50 parturients with low birthweight (LBW) fetuses in the course of vaginal delivery (n-18) and cesarean section (n-32). Twenty normal parturients whose pregnancy and labor were uncomplicated were taken as controls. In the main group, low birthweights corresponded to gestational age in 30 parturients, and fetal growth retardation (FGR) was diagnosed in 20. In the course of vaginal delivery, maternal cortisol level increased significantly, the rise being higher in parturients with the FGR syndrome. In both groups, blood cortisol levels were higher in the umbilical artery as compared to umbilical vein; in FGR cases, umbilical-artery cortisol level approached the respective values in control newborns. Following cesarean section, LBW fetuses showed considerably lower umbilical cortisol levels. Separate examination of cortisol fractions has demonstrated that free cortisol goes up in the maternal blood both at vaginal and abdominal delivery. The effect of corticosteroids, used to prevent respiratory distress syndrome, on maternal adrenal activity and LBW fetuses has been evaluated.

Adult↗

[Leiomyoma of the vagina: a report on 2 cases (author's transl)].

Two recent cases of vaginal fibroma are reported. These rare lesions are benign, and symptoms, often absent during the early stages, are due to compression from the growing tumor. Treatment is always surgical, the only problem being the most effective approach. This is either by the upper or lower route, depending on the location of the tumor.

Adult↗

Influence of vaginal versus abdominal hysterectomy on perioperative glucose metabolism.

The aim of this study was to investigate the metabolic effects of abdominal versus vaginal hysterectomy with specific regard to perioperative glucose metabolism. Fourteen patients received either abdominal (AH, n = 7) or vaginal hysterectomy (VH, n = 7). Hepatic glucose production was measured before and 2.5 h after the operation by stable isotope technique ([6,6-2H2]-glucose). Metabolic substrates (glucose, lactate, nonesterified fatty acids [NEFA], beta-hydroxybutyrate) and hormones (insulin, glucagon, cortisol, catecholamines) were determined pre-, intra-, and postoperatively. VH induced a higher postoperative glucose concentration than the abdominal approach (VH, 148 +/- 25 mg/dL; AH, 111 +/- 16 mg/dL; P < 0.05). Since postoperative enhancement of hepatic glucose production was comparable in both groups, glucose clearance was lower after the vaginal procedure (VH, 1.7 +/- 0.3 mL.kg-1.min-1; AH, 2.1 +/- 0.3 mL.kg-1.min-1; P < 0.05). NEFA, beta-hydroxybutyrate, and catecholamines similarily increased after surgery. Cortisol levels were more increased after VH (VH, 80 +/- 26 micrograms/dL; AH, 37 +/- 14 micrograms/dL; P < 0.001). Lactate, glucagon, and insulin concentrations did not change perioperatively. The more pronounced hyperglycemic response to VH was due to lower peripheral glucose use caused by higher postoperative cortisol values. The mechanisms responsible for this marked cortisol enhancement after the vaginal operation as well as the clinical significance for patients with preexisting impaired carbohydrate tolerance, however, remained unclear and warrant further investigation.

3-Hydroxybutyric Acid↗

Transvaginal repair of the posthysterectomy vesicovaginal fistula using a peritoneal flap: the gold standard.

OBJECTIVE: To evaluate the transvaginal approach to management of vesicouterine fistulas. STUDY DESIGN: Over a 10-year period, 7 cases of simple posthysterectomy vesicovaginal fistulas were identified. The surgical technique involved resection of the fistulous tract completely, performance of layered closure and placement of a peritoneal flap between the bladder and vaginal suture lines. RESULTS: One fistula closed spontaneously, and the remaining 6 were repaired transvaginally. Primary repair was successful in all cases, with no complications. CONCLUSION: The transvaginal repair described is the preferred method of repair, associated with an extremely high success rate, low morbidity and cost savings. Its approach should be considered the gold standard.

Adult↗

Touchdown General Primer (GP5+/GP6+) PCR and optimized sample DNA concentration support the sensitive detection of human papillomavirus.

BACKGROUND: The GP5+/GP6+ PCR assay is a well-established HPV detection technique. This study has examined the effects of incorporating 'hot start' and 'touchdown' steps into the protocol. In addition, dTTP was substituted with dUTP to permit contamination control measures against carry-over PCR product. METHODS: Firstly, HPV-16 was amplified from SiHa cell DNA (0.1 ng-100 ng) diluted in a background of C-33A DNA (100 ng-2 microg). Secondly, the detection of small quantities (15ag-1.5pg) of HPV recombinant plasmids (types 16, 31, 33, 45, 51, 52, and 56) diluted in C-33A DNA was investigated. Thirdly, clinical sample DNA extracts (cervical smears, formalin-fixed vaginal lesions and breast tumors) were tested for HPV. Six different PCR protocols were assessed. HPV was detected by gel electrophoresis, and by Southern and dot blot hybridization. RESULTS: HPV detection sensitivity was dependent on the total amount of DNA in a PCR. Touchdown protocols supported HPV-16 detection from 1 ng or 0.5 ng SiHa cell DNA in a background of 2 microg or 1 microg C-33A DNA respectively, and from 0.1 ng of SiHa cell DNA (approximately 28 copies HPV-16) in 500 ng or 100 ng background DNA. Under standard GP5+/GP6+ annealing conditions, HPV-16 went undetected when the DNA content of a PCR was 2 microg or 1 microg, and with 500 ng C-33A DNA the sensitivity limit was 1 ng SiHa cell DNA. HPV recombinant plasmids were each detected with high (albeit varying) sensitivity by a touchdown protocol. HPV-31 was better amplified under standard annealing conditions (1.5fg in 100 ng background DNA) than by a touchdown approach (15fg detection limit). HPV-52 was not amplified by the standard protocol at the dilutions tested. Seventeen different HPV types were demonstrated in 47/65 (72%) abnormal cytology samples recorded as HPV negative by standard GP5+/GP6+ conditions. Twenty-one different HPV types were recorded in 111/114 (97%) vaginal lesions. Multiple infections were also detectable using a touchdown approach. Of 26 breast tumors, 5 (19%) tested HPV positive by the standard assay and 15/26 (58%) using a touchdown protocol. CONCLUSION: Touchdown modification of the GP5+/GP6+ PCR assay enables the detection of HPV undetected under regular assay conditions. The use of standardized DNA quantities in a PCR rather than standard sample volumes containing arbitrary amounts of DNA is supported. A touchdown approach may be beneficial as an analytical test for the re-evaluation of (apparently) HPV negative abnormal cervical cytological or histological samples, and for investigating the association of HPV with disease conditions at diverse organ sites. The clinical utility of a touchdown approach for HPV detection requires further investigation as increased assay analytical sensitivity may not necessarily equate with improved clinical sensitivity or specificity.

Journal Article↗