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Purpose: Genitourinary rhabdomyosarcoma (GU-RMS) often requires multimodal therapy treatment including radiation, chemotherapy, and radical surgery for disease control. The long-term effects of the disease and associated treatments are unclear. We sought to investigate the long-term genitourinary quality of life for adult survivors of pediatric GU-RMS., Materials and Methods: In total, 14 subjects (43% female, median age = 32.5 years [IQR = 23.25 - 39.25], range = 20 - 52 years, 2 bladder, 1 cervical, 5 paratesticular, 3 vaginal, 2 pelvic, 1 prostate RMS) agreed to interview about impact of GU-RMS treatment during childhood on quality of life. A semi-structured interview guide based on the Long-Term Service and Support quality of life model, and grounded in known GU-RMS experiences was created. Two coders independently coded using thematic analysis methodology., Results: Six themes emerged: (i) unknown fertility status, (ii) lack of education, (iii) relationships and difficult communication, (iv) incontinence, clean intermittent catheterization, and a bag, (v) lifestyle and learned adaptation, and (vi) threats to body image. Across these themes, participants reported insufficient knowledge regarding GU-RMS treatment and its impact on function. Participants were principally concerned with anatomical changes, fertility, pregnancy expectations, and survivorship challenges such as communication with romantic partners., Conclusions: Survivors of GU-RMS have significant urinary and sexual function concerns that are important to address in long-term survivorship. Clinicians can potentially improve survivors' quality of life through open and honest age-appropriate education on expectations for treatment, fertility preservation options, and long-term effects of treatment.

Background: North American Pediatric Urology fellowship programs underwent a structural change in 2021 that allows more flexibility in training. Given this opportunity as well as widespread concern about the development of contemporary surgical trainees, it is prudent to understand in detail the current state of preparedness of pediatric urology fellowship graduates for independent practice., Objective: The study aimed to determine recent pediatric urology graduates' reported levels of comfort both at graduation and following the start of clinical practice in performing select index procedures. We also queried the aspects of training and clinical practice perceived to be the most valuable for the development of surgical confidence., Study Design: Graduates of ACGME approved pediatric urology fellowships from 2016 to 2021 were surveyed. Index procedures were described via brief case vignettes. Respondents were asked to indicate their comfort level with each index procedure following fellowship graduation and at the current time point. Comfort levels were defined by the degree of support that respondents would seek from senior colleagues in preparation for case booking. Respondents were also asked about the most helpful operative settings during training and factors contributing to high and low comfort., Results: Fifty-three pediatric urologists (49%) completed the survey out of 109 invited. Most respondents practiced at an academic center. Perceived comfort was very high for low complexity procedures. The responses varied more widely for procedures of moderate and significant complexity (Figure). Across the cohort, there was a substantial increase in comfort between graduation and the current time point for all procedures queried. The most highly valued operative settings in fellowship were those offering real or simulated independence. Respondents most often attributed high comfort to robust case volumes and overall surgical skill gained in fellowship., Discussion: New pediatric urology faculty differ widely in surgical confidence, particularly for more complex procedures. There is meaningful growth in the confidence and self-perceived independence of pediatric urologists during their initial years of practice. The early years are a critical time of continuing maturation and development that should be supported with structured systems of mentorship. Future challenges include low case volumes for rare conditions and the centralization of complex care., Conclusion: These findings will provide valuable context for pediatric urology fellowship directors as they evaluate and redesign their programs under the new, more flexible structure. There are opportunities to formalize early practice mentorship to support the growth of new faculty., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: The thulium fiber laser is a promising new lithoptripsy technology never before studied in the pediatric population. Our center adopted the first platform in North America, the SuperPulsed thulium fiber laser (SPTF). We aimed to compare outcomes in pediatric ureteroscopy using the SPTF to those using the gold standard, low-power holmium:yttrium-aluminum-garnet (Ho:YAG) laser., Materials and Methods: This is a retrospective, consecutive cohort study of unilateral ureteroscopy with laser lithotripsy performed in pediatric patients from 2016 to 2021 as an early adopter of the SPTF. Thirty-day complications and stone-free status, defined as the absence of a stone fragment on followup imaging within 90 days, were analyzed using logistic regression. Operative times were compared using linear regression. Propensity scores for use of SPTF were used in regression analyses to account for potential cohort imbalance., Results: A total of 125 cases were performed in 109 pediatric patients: 93 with Ho:YAG and 32 with SPTF. No significant difference was noted in age (p=0.2), gender (p=0.6), stone burden (p >0.9) or stone location (p=0.1). The overall stone-free rate was 62%; 70% with SPTF and 59% with Ho:YAG. The odds of having a residual stone fragment were significantly lower with SPTF than with Ho:YAG (OR=0.39, 95% CI: 0.19-0.77, p=0.01). There was no significant difference in operative time (p=0.8). Seven (25%) complications were noted with SPTF and 19 (22%) with Ho:YAG (p=0.6)., Conclusions: The SPTF laser was associated with a higher stone-free rate than the low-power Ho:YAG laser without compromising operative time and safety.

Objective: To report and compare presentation and management of Fournier's Gangrene (FG) in female vs male patients at a single tertiary care center., Methods: Patient demographics, clinical characteristics, treatments and outcomes were summarized and compared between males and females who were treated for FG from 2011 to 2018 at a single institution., Results: Of the 143 patients treated for FG at our institution, 33 (23%) were female. Female patients were predominantly white (82%), with a median (IQR) age of 55 (46, 59). Median female boby mass index (BMI) was 42.1 (32, 50.4). Female patients' wound cultures were polymicrobial mix of gram positive and gram negative organisms. Median number of debridements for females was 2 (1,3). The most common anatomic region of gangrene involvement in females was labia (76%) followed by perineum (55%) and gluteus/buttocks (42%). Mortality rate during initial admission was 6% for females. Female patients had a higher median BMI than males (42.1 vs 33.7 respectively; P = .003). FG severity index, length of hospital stay, number of debridements, and wound cultures were comparable to males. The surgical team managing initial debridements differed with females managed primarily by general surgery and males primarily by urology. Mortality rate was comparable to men (6% vs 7%, P >.05)., Conclusion: Female patients with FG have greater BMI but similar clinical presentation, microbiologic characteristics and mortality rate compared to men. Urologists have little involvement during initial management for females at our institution., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: We evaluate the prevalent microorganisms, antibiotic sensitivity patterns and associated outcomes in patients with Fournier's gangrene., Materials and Methods: A retrospective chart review of patients with Fournier's gangrene was conducted between October 2011 and April 2018 at our institution. Univariate analysis was performed using the independent t-test or Kruskal-Wallis H test for continuous variables and exact test for categorical variables., Results: Of the 143 patients included in this study, wound culture was available in 131 (92%) patients with a median number of 3 microorganisms per wound. The most commonly grown pathogens were Staphylococcus species (66, 46%), Streptococcus species (53, 37%), Bacteroides species (34, 24%), Candida species (31, 22%), Escherichia coli (28, 20%) and Prevotella species (26, 18%). Most bacteria were sensitive to ampicillin-sulbactam, ceftriaxone, piperacillin-tazobactam, amikacin and cefepime, and resistant to ampicillin, trimethoprim-sulfamethoxazole, levofloxacin and clindamycin. Enterococcus faecalis and Streptococcus anginosus were resistant to vancomycin. The overall Fournier's gangrene mortality count was 14 (10%) patients. No association was noted between the type of infection and Fournier's gangrene severity index, length of hospital stay or mortality., Conclusions: At our institution Candida is a prevalent pathogen in the wound culture of patients with Fournier's gangrene. The resistance patterns for clindamycin and vancomycin are concerning. Addition of an antifungal agent to the empiric treatment should be considered based on clinical presentation.

Objective: To evaluate and profile ammonium acid urate (AAU) stone formers utilizing a large contemporary cohort., Materials and Methods: A retrospective review of all patients with documented AAU urolithiasis on stone analysis was performed from 1995 to 2013. A stone was considered AAU if it contained at least 10% of the total composition on stone analysis., Results: Of 12,421 stones, a total of 111 AAU stones were identified in 89 patients. Seventy-two patients (81%) had mixed composition stones. Of the 89 AAU patients, 10 (11%) had chronic kidney disease, 12 (13%) had recurrent urinary tract infections, 8 (9%) had diabetes mellitus, 25 (28%) had a history of ileostomy or bowel resection, and 11 (12%) had significant diarrhea or inflammatory bowel disease. Additionally, 20 (22%) had prior prostate surgery with bladder neck contracture and 8 (9%) were managed with a chronic indwelling catheter. Surgical intervention was required in 74 patients (83%). At the median follow-up of 4.9 years, 19 patients (21%) had stone recurrence with a median time to recurrence of 22 months (interquartile range: 10.5-42.0)., Conclusion: AAU stones are rare. In addition to previously described risk factors for AAU stone formation, patients with prior prostate surgery and bladder neck contracture or a surgically altered bladder may be at increased risk for formation. Furthermore, the long-term follow-up provided by this large AAU cohort demonstrates a significant risk for stone recurrence., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: To describe the endoscopic characteristics of renal papillae in struvite stone formers (SFs)., Materials and Methods: From 2009 to 2014, patients undergoing percutaneous nephrolithotomy were prospectively enrolled in our study. Endoscopic analysis and biopsy of papillae were performed to demonstrate the presence and percentage surface area (SA) of Randall's plaque or ductal plug. Comparison with idiopathic calcium oxalate (CaOx) SF and non-SF controls was performed., Results: We identified 29 struvite SFs to compare with 90 idiopathic CaOx SFs and 17 controls. On endoscopic mapping, 28 struvite SFs (97%) demonstrated Randall's plaque and 9 (31%) had plugging. The average mean SA of Randall's plaque in struvite SF (1.5 ± 1.4%) was less than CaOx SFs (3.7 ± 4.3%, P  =  .0018) and similar to controls (1.7 ± 2.7%, P  =  .76). Average mean plug SA was similar between struvite SFs, CaOx SFs, and controls. On metabolic assessment, 83% of struvite SFs had at least one urine abnormality, with urinary uric acid and oxalate levels significantly higher among struvite SFs compared to controls (P  =  .002). Despite lack of active urinary tract infection, interstitial inflammation was more prevalent in struvite SFs compared to CaOx SFs (43.5% vs 7.3%, P  =  .0001)., Conclusions: Our findings suggest a limited role for Randall's plaque in struvite stone formation. Struvite SFs have less plaque formation than CaOx SFs, but demonstrate evidence of severe parenchymal inflammation compared to other SFs. The role of this prominent interstitial inflammation requires further study., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objectives: Hemostatic clip migration into the lower urinary tract is a potential complication of radical prostatectomy that may cause symptoms, anxiety, and functional concern. Our objective was to evaluate initial presentation, endoscopic management, and outcomes of patients with hemostatic clip migration following radical prostatectomy., Patients and Methods: We retrospectively identified all patients with hemostatic clip migration at our institution from 1977 to 2012. Patient records were then reviewed to identify causative factors, presentation, and long-term functional outcomes., Results: Seventeen patients were identified with clip migration following radical prostatectomy. Eight (47%) patients had undergone open retropubic radical prostatectomy, and 9 (53%) had received robot-assisted radical prostatectomy. Hemostatic clip migration was diagnosed at a median of 8 (range, 1-252) months after prostatectomy. The majority of patients (n = 16, 94%) were symptomatic upon the diagnosis of clip migration. Symptoms included irritative urinary symptoms (n = 14, 82%), perineal pain (n = 3, 18%), hematuria (n = 2, 12%), and infection (n = 2, 12%). Five (29%) had concomitant bladder neck contracture. Fifteen (88%) underwent successful endoscopic clip removal, whereas 2 (13%) patients required a repeat operation for recurrent clip erosion. With a median follow-up of 1.6 years, the majority (n = 13, 87%) had complete symptom resolution after clip removal, although 2 patients had recurrent bladder neck contracture., Conclusions: Hemostatic clip migration after prostatectomy is often symptomatic with irritative voiding complaints, perineal pain, hematuria, infection, or bladder neck contracture. Fortunately, clips can be removed endoscopically with expected symptom resolution in the vast majority of patients.

Background/objectives: Epidemics of obesity and diabetes are escalating. High-calorie/high-fat food is a major cause for these global health issues, but molecular mechanisms underlying high-fat, diet-induced obesity are still not well understood. The aryl hydrocarbon receptor (AhR), a transcription factor that acts as a xenobiotic sensor, mediates environmental toxicant-induced obesity, insulin resistance and development of diabetes. AhR also influences lipid metabolism and diet-induced obesity. The effects of AhR deficiency on diet-induced obesity, hepatic steatosis and insulin resistance were examined., Methods: Male wild-type (WT), AhR null (AhR(-/-)) and AhR heterozygote (AhR(+/-)) mice were fed a normal chow diet (NCD, 10% kcal from fat) or a high-fat diet (HFD, 60% kcal from fat) for up to 14 weeks. Adiposity, adipose and liver morphology, insulin signaling, metabolic parameters and gene profiles were assessed., Results: AhR deficiency protected against HFD-induced obesity, hepatic steatosis, insulin resistance and inflammation. Moreover, AhR deficiency preserved insulin signaling in major metabolic tissues. These protective effects result from a higher energy expenditure in AhR-deficient mice compared with WT. Levels of transcript for both the thermogenic gene, uncoupling protein 1 (Ucp1), in brown adipose tissue and mitochondrial β-oxidation genes in muscle were significantly higher in AhR(-/-) and AhR(+/-) mice compared with WT., Conclusions: This work documents a physiologically relevant function for AhR in regulation of body weight, hepatic fat deposition, insulin sensitivity and energy expenditure under HFD exposure, suggesting that AhR signaling may be developed as a potential therapeutic target for treatment of obesity and metabolic disorders.

Objectives: To evaluate short-term outcomes of holmium laser enucleation of the prostate (HoLEP) and photoselective vaporisation of the prostate (PVP) in patients with benign prostatic hyperplasia (BPH) and chronic urinary retention (CUR)., Patients and Methods: A retrospective chart review was performed of all patients with CUR who underwent HoLEP or PVP at our institution over a 3-year period. CUR was defined as a persistent post-void residual urine volume (PVR) of >300 mL or refractory urinary retention requiring catheterisation., Results: We identified 72 patients with CUR who underwent HoLEP and 31 who underwent PVP. Preoperative parameters including median catheterisation duration (3 vs 5 months, P = 0.71), American Urological Association Symptom Index score (AUASI; 18 vs 21, P = 0.24), and PVR (555 vs 473 mL, P = 0.096) were similar between the HoLEP and PVP groups. The HoLEP group had a larger prostate volume (88.5 vs 49 mL, P < 0.001) and higher PSA concentration (4.5 vs 2.4 ng/mL, P = 0.001). At median 6-month follow-up, 71 (99%) HoLEP patients and 23 (74%) PVP patients were catheter-free (P < 0.001). Of the voiding patients, postoperative AUASI (3 vs 4, P = 0.06), maximum urinary flow rate (23 vs 18 mL/s, P = 0.28) and PVR (56.5 vs 54 mL, P = 1.0) were improved in both groups., Conclusions: Both HoLEP and PVP are effective at improving urinary parameters in men with CUR. Despite larger prostate volumes, HoLEP had a 99% successful deobstruction rate, thus rendering patients catheter-free., (© 2014 The Authors. BJU International © 2014 BJU International.)

Lymphoma may affect the ureter in cases of retroperitoneal involvement. We present a case of an adolescent male found to have non-Hodgkin lymphoma initially presenting as ureteral stricture evident on imaging. He was treated and responded to multiagent chemotherapy with resolution of both the lymphoma and the ureteral stricture. Although rare, non-Hodgkin lymphoma should be included in the differential diagnosis of pediatric patients with noncalculous, idiopathic ureteral strictures.

Objective: To evaluate comparative safety and outcomes in patients undergoing holmium laser enucleation of the prostate (HoLEP) with and without previous transurethral prostate surgery., Methods: We identified 37 patients who underwent HoLEP for persistent lower urinary tract symptoms (LUTS) after previous transurethral prostate surgery for benign prostatic hyperplasia (group I). Patients were then matched according to preoperative transrectal ultrasound prostate size to 74 patients without prior surgery who underwent HoLEP (group II). A retrospective chart review was performed., Results: In group I, no patients had previously undergone HoLEP, and 9 (24%) had undergone >1 surgery for benign prostatic hyperplasia (BPH). There were no significant differences in age, preoperative mean urinary flow, or preoperative prostate-specific antigen (PSA) value between groups I and II. There were no significant differences in perioperative parameters, including resected tissue weight (61.7 vs 63.9 grams, P = .83) or enucleation rate (1.32 vs 1.36 g/min, P = .76). There was no significant difference in mean catheterization time or length of hospital stay. Postoperative peak flow (23.4 vs 26.9 mL/sec, P = .14) and post-void residual (PVR, 50.9 vs 50.3 mL, P = .61) were improved and similar between groups. American Urological Association Symptom Indices (AUASIs) were improved in both groups, although the primary group was significantly lower (7.52 vs 5.21, P = .0060). There was no significant difference in adverse events. All patients were urinating at last follow-up (mean 18.4 and 15.1 months, P = .16)., Conclusion: HoLEP is safe and effective at relieving persistent LUTS after failed previous BPH surgery. Outcomes similar to those of a primary HoLEP procedure can be expected., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Activation of the aryl hydrocarbon receptor (AhR) by the highly toxic, prototypical ligand, 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD) or other dioxin-like compounds compromises ovarian function by altering follicle maturation and steroid synthesis. Although alteration of transcription after nuclear translocation and heterodimerization of AhR with its binding partner, aryl hydrocarbon nuclear transporter (ARNT), is often cited as a primary mechanism for mediating the toxic effects of dioxins, recent evidence indicates that crosstalk between AhR and several other signaling pathways also occurs. Like the circadian clock genes, AhR is a member of the basic helix-loop-helix, Per-ARNT-SIM (bHLH-PAS) domain family of proteins. Thus, these studies tested the hypothesis that TCDD can act to alter circadian clock regulation in the ovary. Adult female c57bl6/J mice entrained to a typical 12h light/12h dark cycle were exposed to a single 1 μg/kg dose of TCDD by gavage. Six days after exposure, animals were released into constant darkness and ovaries were collected every 4h over a 24h period. Quantitative real-time PCR and immunoblot analysis demonstrated that TCDD exposure alters expression of the canonical clock genes, Bmal1 and Per2 in the ovary. AhR transcript and protein, which displayed a circadian pattern of expression in the ovaries of control mice, were also altered after TCDD treatment. Immunohistochemistry studies revealed co-localization of AhR with BMAL1 in various ovarian cell types. Furthermore, co-immunoprecipitation demonstrated time-of-day dependent interactions of AhR with BMAL1 that were enhanced after TCDD treatment. Collectively these studies suggest that crosstalk between classical AhR signaling and the molecular circadian clockworks may be responsible for altered ovarian function after TCDD exposure., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Sandia National Laboratories, under the direction of the Office of Science and Technology, National Institute of Justice, conducted the chemical facility vulnerability assessment (CFVA) project. The primary objective of this project was to develop, test and validate a vulnerability assessment methodology (VAM) for determining the security of chemical facilities against terrorist or criminal attacks (VAM-CF). The project also included a report to the Department of Justice for Congress that in addition to describing the VAM-CF also addressed general observations related to security practices, threats and risks at chemical facilities and chemical transport. In the development of the VAM-CF Sandia leveraged the experience gained from the use and development of VAs in other areas and the input from the chemical industry and Federal agencies. The VAM-CF is a systematic, risk-based approach where risk is a function of the severity of consequences of an undesired event, the attack potential, and the likelihood of adversary success in causing the undesired event. For the purpose of the VAM-CF analyses Risk is a function of S, L(A), and L(AS), where S is the severity of consequence of an event, L(A) is the attack potential and L(AS) likelihood of adversary success in causing a catastrophic event. The VAM-CF consists of 13 basic steps. It involves an initial screening step, which helps to identify and prioritize facilities for further analysis. This step is similar to the prioritization approach developed by the American Chemistry Council (ACC). Other steps help to determine the components of the risk equation and ultimately the risk. The VAM-CF process involves identifying the hazardous chemicals and processes at a chemical facility. It helps chemical facilities to focus their attention on the most critical areas. The VAM-CF is not a quantitative analysis but, rather, compares relative security risks. If the risks are deemed too high, recommendations are developed for measures to reduce the risk. This paper will briefly discuss the CFVA project and VAM-CF process.