Treatment of the Enlarged Clitoris

Management of the enlarged clitoris, because of its import for sexual function, has been and remains one of the most controversial topics in pediatric urology. Early controversy surrounding clitoroplasty resulted from many factors including an incomplete understanding of clitoral anatomy and incorrect assumptions of the role of the clitoris in sexual function. With a better understanding of anatomy and function, procedures have evolved to preserve clitoral tissue, especially with respect to the neurovascular bundles. These changes have been made in an effort to preserve clitoral sensation and preserve orgasmic potential. It is the goal of this manuscript to describe the different procedures that have been developed for the surgical management of clitoromegally, with emphasis on the risks and benefits of each. Equally important to any discussion of such a sensitive topic is an understanding of long-term patient outcomes. As we will see, despite its importance, there has been a dearth of data in this regard. Future work in the arena of patient satisfaction will undoubtedly play a major role in directing our surgical approach

Ultrastructure of Bone Marrow of Rats After Severe Hemodilution with Starch or Modified Hemoglobin

The ultrastructure of bone marrow of rats was studied 24 h after exchange-transfusion with solutions of starch or modified hemoglobin to a hematocrit of 10-15. Blood smears of the transfused rats had 17-20% reticulocytes as compared to 5-6% for sham operated controls. In the transfused rats marrow macrophages had numerous heterolysosomes apparently containing the starch or hemoglobin from the transfused solutions. Endothelial cells and reticular cells also possessed a few heterolysosomes thought to contain starch or hemoglobin. Reticular cells of the transfused rats contained numerous glycogen particles scattered throughout the cytoplasm or arranged in large masses. Synthesis of glycogen may indicate a metabolic change in reticular cells in response to tissue hypoxia induced by the exchange-transfusion procedure

Validation and Preliminary Results of the Parental Assessment of Children's External Genitalia Scale for Females (PACE-F) for Girls With Congenital Adrenal Hyperplasia

Objective To validate a parental assessment of children's external genitalia scale for females (PACE-F) for girls with congenital adrenal hyperplasia (CAH) by adapting the validated adult female genital self-image scale. Methods PACE-F was administered to parents of girls (Tanner 1, 2 months-12 years) with and without CAH. Final questions were determined by clinical relevance and psychometric properties (scores: 0-100). A reference range was established using 95% confidence interval among controls. Age-matched controls were compared to girls with CAH (1) <4 years old before and after female genital reconstruction surgery (FGRS), and (2) 4-12-year olds after FGRS. Nonparametric statistics were used. Results Participants included 56 parents of 41 girls with CAH (median 3.9 years old, 97.6% FGRS) and 139 parents of 130 girls without CAH. Face and content validity was established by families, experts, and factor analysis. Internal consistency was high (Cronbach's alpha: 0.83). Population reference score range was 66.7-100. Ten consecutive girls had pre- and post-FGRS PACE-F scores. All scores improved at 4 months after surgery and all preoperative scores were below reference range and lower than controls (P = .0001). All postoperative scores were within reference range, no different from controls (P = .18). Scores for girls with CAH after FGRS aged 4-12 years were no different from controls (100.0 vs 88.9, P = .77) and 90.0% were in reference range, as expected (P = .99). Conclusion We present a validated instrument for parental assessment of genital appearance in girls with CAH. We demonstrate improved parent-reported appearance after FGRS, with scores similar to age-matched controls

Long-term outcomes of catheterizable continent urinary channels: what do you use, where you put it and does it matter?

Introduction Appendicovesicostomy (APV) and Monti ileovesicostomy (Monti) are commonly used catheterizable channels with similar outcomes on short-term follow-up. Their relative long-term results have not been previously published. Objective Our goal was to assess long-term durability of APV and Monti channels in a large patient cohort. Study design In this retrospective cohort study, we retrospectively reviewed consecutive patients ≤21 years old undergoing APV and Monti surgery at our institution (1990–2013). We collected data on demographics, channel type, location, continence and stomal and subfascial revisions. Kaplan–Meier survival and Cox proportional hazards analysis were used. Results Of 510 patients meeting inclusion criteria, 214 patients had an APV and 296 had a Monti (50.5% spiral Monti). Median age at surgery was 7.4 years for APV (median follow-up: 5.7 years) and 8.7 years for Monti (follow-up: 7.7 years). Stomal stenosis, overall stomal revisions and channel continence were similar for APV and Monti (p ≥ 0.26). Fourteen APVs (6.5%) had subfascial revisions compared to 49 Montis (16.6%, p = 0.001). On survival analysis, subfascial revision risk at 10 years for APV was 8.6%, Monti channels excluding spiral umbilical Monti: 15.5% and spiral umbilical Monti: 32.3% (p < 0.0001, Figure). On multivariate regression, Monti was 2.09 times more likely than APV to undergo revision (p = 0.03). The spiral Monti to the umbilicus, in particular, was 4.23 times more likely than APV to undergo revision (p < 0.001). Concomitant surgery, gender, age and surgery date were not significant predictors of subfascial revision (p ≥ 0.17). Stomal location was significant only for spiral Montis. Discussion Our study has several limitations. Although controlling for surgery date was a limited way of adjusting for changing surgical techniques, residual confounding by surgical technique is unlikely, as channel implantation technique was typically unrelated to channel type. We did not include complications managed conservatively or endoscopically. In addition, while we did not capture patients who were lost to follow-up, we attempted to control for this through survival analysis. Conclusions We demonstrate, durable long-term results with the APV and Monti techniques. The risk of channel complications continues over the channel's lifetime, with no difference in stomal complications between channels. At 10 years after initial surgery, Monti channels were twice as likely to undergo a subfascial revision (1 in 6) than APV (1 in 12). The risk is even higher in for the spiral umbilical Monti (1 in 3)

The effect of the thioether-bridged, stabilized angiotensin-(1-7) analogue cyclic Ang-(1-7) on cardiac remodeling and endothelial function in rats with myocardial infarction

Modulation of renin-angiotensin system (RAS) by angiotensin-(17) (Ang-(17)) is an attractive approach to combat the detrimental consequences of myocardial infarction (MI). However Ang-(17) has limited clinical potential due to its unfavorable pharmacokinetic profile. We investigated effects of a stabilized, thioether-bridged analogue of Ang-(17) called cyclic Ang-(17) in rat model of myocardial infarction. Rats underwent coronary ligation or sham surgery. Two weeks thereafter infusion with 0.24 or 2.4 μg/kg/h cAng-(17) or saline was started for 8 weeks. Thereafter, cardiac morphometric and hemodynamic variables as wells as aortic endothelial function were measured. The average infarct size was 13.8 and was not changed by cAng-(17) treatment. MI increased heart weight and myocyte size, which was restored by cAng-(17) to sham levels. In addition, cAng-(17) lowered left ventricular end-diastolic pressure and improved endothelial function. The results suggest that cAng-(17) is a promising new agent in treatment of myocardial infarction and warrant further research.</p

The effect of the thioether-bridged, stabilized angiotensin-(1-7) analogue cyclic Ang-(1-7) on cardiac remodeling and endothelial function in rats with myocardial infarction

Modulation of renin-angiotensin system (RAS) by angiotensin-(17) (Ang-(17)) is an attractive approach to combat the detrimental consequences of myocardial infarction (MI). However Ang-(17) has limited clinical potential due to its unfavorable pharmacokinetic profile. We investigated effects of a stabilized, thioether-bridged analogue of Ang-(17) called cyclic Ang-(17) in rat model of myocardial infarction. Rats underwent coronary ligation or sham surgery. Two weeks thereafter infusion with 0.24 or 2.4 μg/kg/h cAng-(17) or saline was started for 8 weeks. Thereafter, cardiac morphometric and hemodynamic variables as wells as aortic endothelial function were measured. The average infarct size was 13.8 and was not changed by cAng-(17) treatment. MI increased heart weight and myocyte size, which was restored by cAng-(17) to sham levels. In addition, cAng-(17) lowered left ventricular end-diastolic pressure and improved endothelial function. The results suggest that cAng-(17) is a promising new agent in treatment of myocardial infarction and warrant further research.</p