Expert Consensus on Pediatric Urodynamics Reporting Using Modified Delphi Technique.

PURPOSE: Urodynamic testing (UDS) is an important tool in the management of pediatric lower urinary tract conditions. There have been notable efforts to standardize pediatric UDS nomenclature and technique, but no formal guidelines exist on essential elements to include in a clinical report. We sought to identify ideal structure and elements of a pediatric UDS assessment based on expert consensus. MATERIALS AND METHODS: Pediatric urologists regularly performing UDS were queried using a Delphi process. Participants were invited representing varied geographic, experience, and societal involvement. Participants underwent 3 rounds of questionnaires between November 2022 and August 2023 focusing on report organization, elements, definitions, and automated electronic health record clinical decision support. Professional billing requirements were also considered. Consensus was defined as 80% agreeing either in favor of or against a topic. Elements without consensus were discussed in subsequent rounds. RESULTS: A diverse sample of 30 providers, representing 27 institutions across 21 US states; Washington, District of Columbia; and Canada completed the study. Participants reported interpreting an average number of 5 UDS reports per week (range 1-22). The finalized consensus report identifies 93 elements that should be included in a pediatric UDS report based on applicable study conditions and findings. CONCLUSIONS: This consensus report details the key elements and structure agreed upon by an expert panel of pediatric urologists. Further standardization of documentation should aid collaboration and research for patients undergoing UDS. Based on this information, development of a standardized UDS report template using electronic health record implementation principles is underway, which will be openly available for pediatric urologists.

A scoping review on chordee correction in boys with ventral congenital penile curvature and hypospadias.

Introduction: Congenital penile curvature (PC), often concomitant with hypospadias, poses challenges in urology. Surgical correction techniques, including plication and corporotomy, lack standardized guidelines. This study aims to address the paucity of high-level evidence by comprehensively reviewing the outcomes of PC correction procedures in patients with and without hypospadias. This will inform clinical decision-making and provide insights for future research and meta-analyses. Methods: We conducted this scoping review in accordance with the JBI Manual for Evidence Synthesis and PRISMA-ScR guidelines. An extensive literature search was performed and comparative studies published in English up to June 2023 were included. The studies were divided into three categories: PC without hypospadias, PC with hypospadias, and studies comparing two or more materials for covering the ventral corporotomy. Data extraction comprised author details, patient characteristics, study design, interventions, outcomes, and complications. Methodological quality was assessed using the Newcastle-Ottawa Scale. Results: Forty-two studies were included in the review, which collectively comprised 3180 patients. Thirteen comparative studies reported the outcomes of surgery for congenital PC without hypospadias, 22 studies compared different techniques of PC correction in patients with hypospadias and 7 studies compared the type of materials for coverage following ventral corporotomy. In cases of PC without hypospadias, the most commonly reported surgery was the Nesbit's plication. For PC with hypospadias correction, the results of ventral corporotomy were superior to that of dorsal plication in most of the studies. The two-stage repair had better results when compared to the one-stage repair for patients with perineo-scrotal hypospadias. In studies comparing materials for coverage of ventral corporotomy, the tunica vaginalis flap or graft was utilized most commonly. The majority of the studies reported a success rate ranging from 85% to 100%. The methodological quality was high in all but four studies. Conclusion: Plication procedures are generally preferred for PC without hypospadias, but they result in penile shortening. For those with hypospadias, corporotomy is associated with superior outcomes than plication, especially for those with severe curvature and redo procedures. For ventral corporotomy coverage, the tunica vaginalis flap or graft is the most commonly reported tissue in the literature.

A systematic review of paediatric neurogenic lower urinary tract dysfunction guidelines using the Appraisal of Guidelines and Research Evaluation (AGREE) II instrument.

OBJECTIVE: To examine and critique current international clinical practice guidelines (CPGs) on management of paediatric neurogenic lower urinary tract dysfunction (NLUTD) and assess the applicability of these guidelines to clinical practice. MATERIALS AND METHODS: We conducted a systematic review of all CPGs on NLUTD published in English from the year 2010 to 2022. Six reviewers independently used the Appraisal of Guidelines and Research Evaluation (AGREE) II instrument to appraise all eligible CPGs. This instrument is comprised of 23 items organised into six quality domains. The scores for each item and domain were tabulated for each reviewer and interrater reliability was assessed for each domain using the intraclass correlation coefficient (ICC). RESULTS: Six CPGs were appraised and these included: National Institute for Health and Care Excellence (NICE), European Society for Paediatric Urology, International Children's Continence Society, Irish, Spina Bifida Association (SBA), and International Brazilian Journal of Urology guidelines. They had high mean standardised scores in the domain on 'scope and purpose' and 'clarity of presentation' but had low scores in the domain of 'applicability'. The top three CPGs based on overall score were the NICE, Irish and SBA guidelines and the reviewers had high degree of interrater reliability (ICC 0.912, P < 0.001). The mean scores in various domains for the top three guidelines were 95.8 (scope and purpose), 87.5 (stakeholder involvement), 69.1 (rigour of development), 94.0 (clarity of presentation), 68.4 (applicability), and 59.7 (editorial independence). The diagnostic and treatment recommendations of the top three guidelines were presented. CONCLUSION: The existing CPGs on paediatric NLUTD provide high-quality evidence based recommendations. The NICE, Irish and SBA guidelines were the top three CPGs identified. They scored high on most domains except applicability and editorial independence. These domains need to be considered for future updates to improve the utility.

Scoping review of neurogenic bladder patient-reported readiness and experience following care in a transitional urology clinic.

OBJECTIVE: To generate a scoping review that summarizes thematically on all reported patient perceptions on readiness and experiences during transitional urologic care for patients with neurogenic bladder and or congenital genitourinary conditions that require continuity of care into adulthood. METHODS: A systematic literature search was performed in October 2021. Records were screened and identified for studies relevant to reported readiness and experience in urologic transitional care among patients needing life-long urologic care. The methodological quality of the cross-sectional studies was assessed using AXIS. The included studies were clustered according to patient readiness in transition and patient experience-satisfaction in the urologic transition process. This scoping review was part of a systematic review registered on PROSPERO CRD42022306229 and was conducted in compliance with the PRISMA extension for scoping reviews. RESULTS: A total of 12 articles were included that assessed patients with neurogenic bladder that reported either readiness or patient experience following the transitional care process. The patient readiness was assessed in six studies, determined using the TRAQ score with a range of 3-4/5. Older age, high health literacy, and parental or families' transition process awareness were associated with readiness. Generally, patients experience better satisfaction with pediatric care than with adult care facilities. Most patients felt that sexuality and fertility were not adequately tackled during the transition. The reported barriers to successful transition were patient, provider, and system factors, including lack of insurance coverage/financial management, patient preference, long-term bond with the pediatric providers, and communication by the adult provider. Based on AXIS, all of the studies identified for this scoping review did not determine the sample size, and most of the studies did not categorize the responders, which could introduce bias to the interpretation of their results. CONCLUSION: This scoping review summarizes the readiness and experience of neurogenic bladder patients who underwent the urologic transitional process. Overall, understanding the patient, provider, and system factors associated with better readiness and enhancing the patient experience will ensure a better transition process.

Clinically and biologically relevant subgroups of Wilms tumour defined by genomic and epigenomic analyses.

BACKGROUND: Although cure rates for Wilms tumours (WT) are high, many patients receive therapy with attendant long-term complications. Our goal was to stratify WT using genome-wide analyses to identify candidate molecular features for patients who would benefit from a reduction in therapy. METHODS: We generated DNA methylation and exome sequencing data on WT-kidney pairs (n = 57) and unpaired tumours (n = 27) collected either at our centre or by the Children's Oncology Group. Samples were divided into a discovery set (n = 32) and validation set (n = 52). RESULTS: Analysis of DNA methylation revealed two subgroups of WT with distinct features. Subgroup A has a similar DNA methylation profile to mature kidney, while Subgroup B has genome-wide dysregulation of DNA methylation. The rate of non-synonymous missense mutations and segmental chromosomal aberrations was higher in Subgroup B tumours, suggesting that this group has genome instability related to its epigenetic state. Subgroup A had a higher proportion of cases of bilateral disease. Tumours with high-risk histology or from patients who relapsed were only found in Subgroup B. CONCLUSION: We have identified subgroup-specific molecular events that could inform future work supporting more targeted therapeutic approaches and patient stratification. We propose a novel developmental tumour model based on these findings.

Persistent myopathy despite release of partial obstruction: in vivo reversal of dysfunction and transcriptional responses using rapamycin.

Current and potential medical therapy for obstruction-induced myopathic bladder dysfunction (from benign prostatic hyperplasia or posterior urethral valves) focuses on symptoms. The persistent tissue pathology and dysfunction after release of obstruction is often deemed irreversible without any systematic therapeutic approaches. As rapamycin can attenuate bladder smooth muscle hypertrophy and dysfunction during the genesis of partial obstruction in vivo, we tested whether rapamycin could improve persistent function after release of obstruction (de-obstruction or REL). Female Sprague-Dawley rat bladders were partially obstructed (PBO) by suturing around both the urethra and a para-urethral steel rod, then removing the rod. One day prior to release of obstruction (preREL), voiding parameters and residual urine volume of preREL+future rapa, preREL+future veh groups were recorded. Release of obstruction (REL) was performed by suture removal following 6 weeks of PBO. For 4 more weeks after the de-obstruction, REL animals were randomized to rapamycin (REL+rapa) or vehicle (REL+veh). PBO for 6 weeks were used as positive controls. In shams, the urethra was exposed, but no suture tied. Voiding parameters and residual urine volume were measured prior to sacrifice of sham and REL+veh or REL+rapa, and PBO. Rapamycin efficacy was tested by pair-wise comparison of changes in individual voiding data from preREL+future veh or preREL+future rapa versus REL+veh or REL+rapa, respectively, as well as by comparisons of REL+veh to REL+rapa groups. Bladders were weighed and processed for a high-throughput QPCR array, and histopathology. Bladder/body mass ratios with PBO increased significantly and remained higher in the release phase in REL+veh animals. REL+rapa versus REL+veh improved residual volumes and micturition fractions toward sham levels. Three genes encoding extracellular proteins, BMP2, SOD3, and IGFBP7, correlated with functional improvement by Pearson's correlations. The promoters of these genes showed enrichment for several motifs including circadian E-boxes. While obstruction and REL augmented CLOCK and NPAS2 expression above sham levels, rapamycin treatment during release significantly blocked their expression. This experimental design of pharmaco-intervention during the de-obstruction phase revealed a novel pathway dysregulated during the clinically relevant treatment phase of obstructive bladder myopathy.

Do post-operative phone calls enhance family satisfaction and outcomes after outpatient pediatric urological surgeries? A prospective study.

INTRODUCTION: This study assesses whether post-operative check-in phone calls (POPC) performed within 48 h of outpatient pediatric urological surgeries by a non-medical professional (NMP) would increase patient/family satisfaction and minimize extraneous resource use by increasing email/telephone communication, while reducing emergency department (ED) visits within 30 days of that procedure. METHODS: Families of patients undergoing ambulatory pediatric urology surgeries were enrolled over 8 weeks. Group 1 did not receive POPC. Group 2 received a POPC within 48 h of their operation by a NMP. Both groups received a phone-call survey 2 weeks after surgery to assess families' perioperative satisfaction. RESULTS: In total, 74 families were enrolled (Group 1 = 44, Group 2 = 31). The response rates to phone surveys for Groups 1 and 2 were 59.1% and 77.4%, respectively. POPC did not improve perioperative satisfaction, nor did it significantly promote the use of nursing email/telephone communication (19.2% vs. 4.2%, p = 0.128) or reduce ED visits (15.4% vs. 0.0%, p = 0.111). However, all families in Group 2 thought POPC was timed appropriately and 79.1% perceived it to be helpful in reducing post-operative anxiety. CONCLUSION: POPC by a NMP within 48 h of surgery may not affect perioperative satisfaction of families of patients undergoing same-day pediatric urology surgery but may have an impact in reducing post-operative anxiety.

Turning a new "page": ways to decrease the number of pages after hours without compromising patient care

Background: Pages to house staff after hours, especially overnight, lead to interrupted sleep and fatigue the next day. Although some pages are urgent, others may not need an immediate response. In this study we aimed to identify unwarranted pages and to establish ways to reduce them. Methods: Over 2 months, all pages to the Department of Pediatric Urology at the Hospital for Sick Children in Toronto, Canada, during call hours were documented, including the assessment of the responding physicians of their medical necessity. After analyzing the reasons for inappropriate pages, we took several steps to try to reduce them without impairing patient care. One year later, pages were tracked again to evaluate the efficacy of our interventions. Results: In the initial measurement period, no calls from parents and approximately 50% of the in-hospital pages (15 of 36 pages from the wards, 27 of 49 pages from the emergency department, 17 of 31 pages requesting consultations, and 8 of 8 pages from the inhouse pharmacy and outside pharmacies) were considered medically urgent. The reasons for unwarranted pages were inconsistent parent teaching, lack of adequate triaging and prioritizing on the ward and lack of awareness of the structure of the on-call provisions among different services in the hospital. Several steps were taken to streamline the teaching of parents and nurses, standardize information, provide alternative means of communication within the hospital and restrict parents' access by phone to the urologist on call. One year later, the number of pages had decreased by 70%. Conclusion: Although physician coverage throughout the day and night is necessary for high-quality and safe patient care, communication with on-call physicians should be only for appropriate reasons. The provision of consistent teaching and alternative communication channels can improve patient care as well as decrease the number of after-hour pages.

Contexte: Les appels au personnel interne par téléavertisseur, surtout la nuit, perturbent le sommeil et entraînent de la fatigue le lendemain. Même si certains de ces appels sont urgents, d'autres ne nécessitent pas de réponse immédiate. Avec cette étude nous avons voulu identifier les appels par téléavertisseur qui sont injustifiés et trouver des façons d'en réduire le nombre. Méthodes: Sur une période 2 mois, nous avons documenté tous les appels par téléavertisseur adressés durant les heures de garde au service d'urologie pédiatrique de l'Hôpital SickKids de Toronto, au Canada, et demandé aux médecins y ayant répondu d'en évaluer le bien-fondé au plan médical. Après avoir analysé les raisons des appels jugés injustifiés, nous avons adopté plusieurs mesures pour en réduire le nombre sans compromettre les soins. Un an plus tard, nous avons de nouveau comptabilisé les appels par téléavertisseur pour mesurer l'efficacité de nos interventions. Résultats: Durant la période de mesure initiale, aucun appel des parents n'a été considéré médicalement urgent, tout comme environ 50 % des appels provenant de l'hôpital même (15 appels sur 36 des unités de soins, 27 appels sur 49 du service des urgences, 17 appels sur 31 pour des demandes de consultation et 8 appels sur 8 de la pharmacie de l'hôpital ou de pharmacies de l'extérieur). Les raisons des appels injustifiés étaient entre autres incohérence dans l'enseignement aux parents, triage et priorisation inadéquats à l'unité de soin et méconnaissance des différents services de l'hôpital quant à la structure et au fonctionnement du système de garde. Plusieurs mesures ont été prises pour simplifier l'enseignement aux parents et au personnel infirmier, standardiser l'information, fournir d'autres moyens de communication dans l'hôpital même et restreindre l'accès des parents à l'urologue de garde par téléphone. Un an plus tard, le nombre d'appels avait diminué de 70 %. Conclusion: Même si les médecins doivent être joignables jour et nuit pour assurer la qualité des soins et la sécurité des patients, les raisons de communiquer avec eux doivent être appropriées. Le rappel des consignes et le recours à d'autres canaux de communication peuvent améliorer les soins aux patients et réduire le nombre d'appels le soir et la nuit.

DNA Methylation Reduces the Yes-Associated Protein 1/WW Domain Containing Transcription Regulator 1 Pathway and Prevents Pathologic Remodeling during Bladder Obstruction by Limiting Expression of BDNF.

Chronic bladder obstruction and bladder smooth muscle cell (SMC) stretch provide fibrotic and mechanical environments that can lead to epigenetic change. Therefore, we examined the role of DNA methylation in bladder pathology and transcriptional control. Sprague-Dawley female rats underwent partial bladder obstruction by ligation of a silk suture around the proximal urethra next to a 0.9-mm steel rod. Sham operation comprised passing the suture around the urethra. After 2 weeks, rats were randomized to normal saline or DNA methyltransferase inhibitor, 5-aza-2-deoxycytidine (DAC) at 1 mg/kg, three times/week intraperitoneally. After 6 weeks, bladders were weighed and divided for histology and RNA analysis by high-throughput real-time quantitative PCR arrays. DAC treatment during obstruction in vivo profoundly augmented brain-derived neurotrophic factor (BDNF) expression compared with the obstruction with vehicle group, which was statistically correlated with pathophysiologic parameters. BDNF, cysteine rich angiogenic inducer 61 (CYR61), and connective tissue growth factor (CTGF) expression clustered tightly together using Pearson's correlation analysis. Their promoters were associated with the TEA domain family member 1 (TEAD1) and Yes-associated protein 1/WW domain containing transcription regulator 1 pathways. Interestingly, DAC treatment increased BDNF expression in bladder SMCs (P < 0.0002). Stretch-induced BDNF was inhibited by the YAP/WWTR1 inhibitor verteporfin. Verteporfin improved the SMC phenotype (proliferative markers and SMC marker expression), in part by reducing BDNF. Expression of BDNF is limited by DNA methylation and associated with pathophysiologic changes during partial bladder outlet obstruction and SMC phenotypic change in vitro.

Activated Hedgehog-GLI Signaling Causes Congenital Ureteropelvic Junction Obstruction.

Intrinsic ureteropelvic junction obstruction is the most common cause of congenital hydronephrosis, yet the underlying pathogenesis is undefined. Hedgehog proteins control morphogenesis by promoting GLI-dependent transcriptional activation and inhibiting the formation of the GLI3 transcriptional repressor. Hedgehog regulates differentiation and proliferation of ureteric smooth muscle progenitor cells during murine kidney-ureter development. Histopathologic findings of smooth muscle cell hypertrophy and stroma-like cells, consistently observed in obstructing tissue at the time of surgical correction, suggest that Hedgehog signaling is abnormally regulated during the genesis of congenital intrinsic ureteropelvic junction obstruction. Here, we demonstrate that constitutively active Hedgehog signaling in murine intermediate mesoderm-derived renal progenitors results in hydronephrosis and failure to develop a patent pelvic-ureteric junction. Tissue obstructing the ureteropelvic junction was marked as early as E13.5 by an ectopic population of cells expressing Ptch2, a Hedgehog signaling target. Constitutive expression of GLI3 repressor in Ptch1-deficient mice rescued ectopic Ptch2 expression and obstructive hydronephrosis. Whole transcriptome analysis of isolated Ptch2+ cells revealed coexpression of genes characteristic of stromal progenitor cells. Genetic lineage tracing indicated that stromal cells blocking the ureteropelvic junction were derived from intermediate mesoderm-derived renal progenitors and were distinct from the smooth muscle or epithelial lineages. Analysis of obstructive ureteric tissue resected from children with congenital intrinsic ureteropelvic junction obstruction revealed a molecular signature similar to that observed in Ptch1-deficient mice. Together, these results demonstrate a Hedgehog-dependent mechanism underlying mammalian intrinsic ureteropelvic junction obstruction.

Variability among Canadian pediatric surgeons and pediatric urologists in the management of cryptorchidism in boys before the publication of major guidelines: a retrospective review of a single tertiary centre

Background: Before 2014, there was a lack of recommendations on managing cryptorchidism, or undescended testis (UDT), from a large pediatric urological or surgical organization. We assessed the variability in management of UDT among pediatric urologists and pediatric surgeons at a single tertiary pediatric referral centre before publication of major guidelines. Methods: We performed a retrospective review of the electronic records of patients who underwent primary unilateral or bilateral orchidopexy at our centre between January 2012 and January 2014. Results: A total of 488 patients (616 testes) were identified, of whom 405 (83.0%) and 83 (17.0%) were managed by pediatric urologists and pediatric surgeons, respectively. There was no difference in baseline characteristics, including age seen in clinic or at surgery, testis location/palpability and availability of preoperative ultrasonograms, of patients seen by the 2 groups. Pediatric surgeons ordered preoperative ultrasonography more often than pediatric urologists (25.3% v. 3.7%, p < 0.001). With palpable UDTs, although both groups used open approaches, pediatric urologists preferred a scrotal approach (56.9%), and pediatric surgeons approached most testes inguinally (98.8%). With nonpalpable UDTs, laparoscopic approaches were preferred by both groups; however, pediatric urologists used a 2-stage Fowler­Stephens approach more often than pediatric surgeons (48.4% v. 15.8%, p < 0.001). Conclusion: There was wide variation in the management of primary UDT between pediatric urologists and pediatric surgeons before the publication of guidelines. The most prominent difference between the 2 groups was in the ordering of preoperative ultrasonography. Future assessment of change in practice patterns may elucidate whether guidelines are an effective tool for standardization of practice.

Contexte: Avant 2014, on ne disposait pas de recommandations émanant d'une grande organisation urologique ou chirurgicale pédiatrique pour la prise en charge de la cryptorchidie (absence d'un ou des deux testicules dans le scrotum). Nous avons évalué les divers types de prise en charge de la cryptorchidie chez les urologues et les chirurgiens pédiatriques dans un seul centre tertiaire de référence pédiatrique avant la publication de lignes directrices majeures. Méthodes: Nous avons procédé à une revue rétrospective des dossiers électroniques de patients ayant subi une orchidopexie unilatérale ou bilatérale primaire dans notre centre entre janvier 2012 et janvier 2014. Résultats: En tout, 488 patients (616 testicules) ont été identifiés, dont 405 (83,0 %) et 83 (17,0 %) ont été traités respectivement par des urologues et des chirurgiens pédiatriques. On n'a noté aucune différence quant aux caractéristiques de départ des patients vus par les 2 groupes, telles que l'âge lors de la consultation à la clinique ou lors de la chirurgie, la localisation/palpabilité des testicules et le recours à l'échographie préopératoire. Les chirurgiens pédiatriques ont demandé une échographie préopératoire plus souvent que les urologues pédiatriques (25,3 % c. 3,7 %, p < 0,001). En présence de cryptorchidie palpable, même si les 2 groupes ont utilisé une approche ouverte, les urologues pédiatriques ont préféré l'approche scrotale (55,4 %) et les chirurgiens pédiatriques l'approche inguinale (98,8 %). En présence de cryptorchidie non palpable, les approches laparoscopiques ont été privilégiées par les 2 groupes; toutefois, les urologues pédiatriques ont utilisé une approche Fowler­Stephens en 2 temps plus souvent que les chirurgiens pédiatriques (48,4 % c. 15,8 %, p < 0,001). Conclusion: On a noté une grande variation dans la prise en charge de la cryptorchidie primaire entre les urologues et les chirurgiens pédiatriques avant la publication des lignes directrices. La principale différence entre les 2 groupes concernait le recours à l'échographie préopératoire. L'évaluation future des changements affectant la pratique permettrait de déterminer si les lignes directrices sont un outil efficace pour sa standardisation.

Normothermic ex vivo kidney perfusion for graft quality assessment prior to transplantation.

Normothermic ex vivo kidney perfusion (NEVKP) represents a novel approach for graft preservation and functional improvement in kidney transplantation. We investigated whether NEVKP also allows graft quality assessment before transplantation. Kidneys from 30-kg pigs were recovered in a model of heart-beating donation (group A) after 30 minutes (group B) or 60 minutes (group C) (n = 5/group) of warm ischemia. After 8 hours of NEVKP, contralateral kidneys were resected, grafts were autotransplanted, and the pigs were followed for 3 days. After transplantation, renal function measured based on peak serum creatinine differed significantly among groups (P < .05). Throughout NEVKP, intrarenal resistance was lowest in group A and highest in group C (P < .05). intrarenal resistance at the initiation of NEVKP correlated with postoperative renal function (P < .001 at NEVKP hour 1). Markers of acid-base homeostasis (pH, HCO3- , base excess) differed among groups (P < .05) and correlated with posttransplantation renal function (P < .001 for pH at NEVKP hour 1). Similarly, lactate and aspartate aminotransferase were lowest in noninjured grafts versus donation after circulatory death kidneys (P < .05) and correlated with posttransplantation kidney function (P < .001 for lactate at NEVKP hour 1). In conclusion, assessment of perfusion characteristics and clinically available perfusate biomarkers during NEVKP allows the prediction of posttransplantation graft function. Thus, NEVKP might allow decision-making regarding whether grafts are suitable for transplantation.

Impact of Adjuvant Urinary Diversion versus Valve Ablation Alone on Progression from Chronic to End Stage Renal Disease in Posterior Urethral Valves: A Single Institution 15-Year Time-to-Event Analysis.

PURPOSE: Long-term progression to end stage renal disease of valve ablation alone vs ablation followed by additional urinary diversion were compared among children with stage 3 chronic kidney disease due to posterior urethral valves. MATERIALS AND METHODS: We performed a retrospective study of children with posterior urethral valves and stage 3 chronic kidney disease treated at a single institution between 1986 and 2011. The 3 treatment groups were classified as group 1-valve ablation alone, group 2-ablation plus subsequent vesicostomy and group 3-ablation followed by ureterostomies and/or pyelostomies. Baseline demographic characteristics were analyzed. Statistical analyses compared the incidence of time to end stage renal disease among the intervention groups using the Fisher-Freeman-Halton exact test and Kaplan-Meier analysis with the log rank test. Cox regression was used to determine predictors of end stage renal disease progression. RESULTS: A total of 40 eligible patients were included in the study (group 1-14 patients, group 2-13 patients, group 3-13 patients). Baseline characteristics and post-intervention estimated glomerular filtration rate revealed no significant between-group differences. A statistically significant difference in progression to end stage renal disease was noted within 1 year after diagnosis of stage 3 chronic kidney disease among the treatment groups (log rank test p=0.02). However, cumulative end stage renal disease incidence at 15-year followup showed no statistical difference (log rank test p=0.628). Cox regression analysis determined that bilateral renal dysplasia (HR 2.76, 95% CI 1.21-6.30) and estimated glomerular filtration rate 60 ml/minute/1.73 m2 or greater after intervention (HR 0.23, 95% CI 0.09-0.61) were predictive of the likelihood of progression to end stage renal disease. CONCLUSIONS: Urinary diversion following valve ablation in children with stage 3 chronic kidney disease associated with posterior urethral valves may temporarily delay progression to end stage renal disease. However, no long-term benefit was noted from diversion in the ultimate incidence of end stage renal disease, suggesting that these interventions should be seen as a temporizing measure. Bilateral renal dysplasia and post-intervention estimated glomerular filtration rate are independent variables predicting overall chronic kidney disease progression.

How can systems engineering inform the methods of programme evaluation in health professions education?

CONTEXT: We evaluate programmes in health professions education (HPE) to determine their effectiveness and value. Programme evaluation has evolved from use of reductionist frameworks to those addressing the complex interactions between programme factors. Researchers in HPE have recently suggested a 'holistic programme evaluation' aiming to better describe and understand the implications of 'emergent processes and outcomes'. FRAMEWORK: We propose a programme evaluation framework informed by principles and tools from systems engineering. Systems engineers conceptualise complexity and emergent elements in unique ways that may complement and extend contemporary programme evaluations in HPE. We demonstrate how the abstract decomposition space (ADS), an engineering knowledge elicitation tool, provides the foundation for a systems engineering informed programme evaluation designed to capture both planned and emergent programme elements. METHODS: We translate the ADS tool to use education-oriented language, and describe how evaluators can use it to create a programme-specific ADS through iterative refinement. We provide a conceptualisation of emergent elements and an equation that evaluators can use to identify the emergent elements in their programme. Using our framework, evaluators can analyse programmes not as isolated units with planned processes and planned outcomes, but as unfolding, complex interactive systems that will exhibit emergent processes and emergent outcomes. Subsequent analysis of these emergent elements will inform the evaluator as they seek to optimise and improve the programme. CONCLUSION: Our proposed systems engineering informed programme evaluation framework provides principles and tools for analysing the implications of planned and emergent elements, as well as their potential interactions. We acknowledge that our framework is preliminary and will require application and constant refinement. We suggest that our framework will also advance our understanding of the construct of 'emergence' in HPE research.

Treatment of daytime urinary incontinence: A standardization document from the International Children's Continence Society.

PURPOSE: This article is a standardization document of the International Children's Continence Society (ICCS); it represent a consensus of ICCS on the management of pediatric daytime urine incontinence (DUI). MATERIALS AND METHODS: This document was designed and written by a multi-disciplinary core group of authors appointed by the ICCS' board. RESULTS: Based on evidence of studies and the experience of experts, the treatment guideline of DUI is assembled in this standardization document. Guidelines and the algorithm of management include non-pharmacological treatment (urotherapy), as well as the pharmacological therapy and other modalities that are presented for DUI in general, as along with recommendations for individual conditions. CONCLUSION: The final document is not a systematic literature review. It includes relevant research when available as well as experts' opinion on the current understanding of daytime incontinence in children. This document illustrates that specific treatment of DUI based on an exact diagnosis is effective. The mainstay of treatment is urotherapy, but a combination of treatment modalities is often necessary. Neurourol. Urodynam. 36:43-50, 2017. © 2015 Wiley Periodicals, Inc.

ECLAMC Study: Prevalence patterns of hypospadias in South America: Multi-national analysis over a 24-year period.

OBJECTIVE: To evaluate prevalence trends of hypospadias in South-America it is essential to perform multicenter and multinational studies with the same methodology. Herein we present systematic data as part of an international multicenter initiative evaluating congenital malformations in South America over a 24-year period. MATERIALS AND METHODS: A nested case-control study was conducted using the Latin American Collaborative Study of Congenital Malformations (ECLAMC), between January 1989 and December 2012. Cases were stratified as isolated (IH) and non-isolated hypospadias (NIH). Global prevalence was calculated and discriminated by country. Associations between birth weight and gestational age, and NIH distribution by associated abnormality and severity of hypospadias, were analyzed. RESULTS: A total of 159 hospitals from six countries participated, reporting surveillance on 4.020.384 newborns. A total of 4.537 hypospadias cases were detected, with a global prevalence of 11.3/10.000 newborns. Trend analyses showed in Chile, Brazil and Uruguay a statistically significant increase in prevalence. Analysis of severity and associated anomalies did not to find an association for distal cases, but did for proximal (RR=1.64 [95% CI=1.33-2.03]). CONCLUSION: This is one of only a few Latin American multicenter studies reporting on the epidemiology of hypospadias in South America in the last two decades. Our data adds to evidence suggesting an increase in some countries in the region at different times. There were also variations in prevalence according to severity. This study adds to literature describing associated anomalies at a hospital-based level.

Measurement of Differential Renal Function by Scintigraphy in Hydronephrotic Kidneys: Importance of Conjugate Views for Accurate Evaluation.

PURPOSE: Traditionally a single posterior view is used to measure differential renal function during nuclear renal scintigraphy. Nevertheless, experimental data show important variation in this measurement in the setting of significant hydronephrosis. To date, the impact of degree of hydronephrosis on the accuracy of differential renal function determination has not been addressed. We evaluated the discrepancy between function measured by anterior and posterior views, and the relationship to varying degrees of hydronephrosis. MATERIALS AND METHODS: We retrospectively reviewed consecutive mercaptoacetyltriglycine renal scans from 2009 to 2011. Ultrasounds were reviewed and degree of hydronephrosis was recorded using anteroposterior pelvic diameter. Absolute percent difference in differential renal function between each view (anterior minus posterior) was calculated and correlated to anteroposterior pelvic diameter. Patients were stratified into 4 groups according to anteroposterior pelvic diameter, ie less than 10 mm, greater than 10 mm, greater than 15 mm and greater than 25 mm. RESULTS: A total of 519 scans with corresponding ultrasounds were analyzed. Median patient age was 2.26 years. Kidneys with a larger anteroposterior pelvic diameter had a greater discrepancy in function on anterior and posterior views. There was a meaningful discrepancy for anteroposterior pelvic diameter greater than 10 mm (p = 0.034) and greater than 25 mm (p = 0.032). Several statistical models were used to identify a meaningful cut point of 15 mm, where the discrepancy in anterior and posterior views became significant (p = 0.001). CONCLUSIONS: The use of single views during nuclear renography for grossly hydronephrotic kidneys is often inaccurate. The discrepancy in differential renal function obtained on the anterior and posterior views is adversely impacted especially as anteroposterior pelvic diameter increases beyond 15 mm. Therefore, we suggest incorporation of conjugate views for estimating differential renal function in patients with hydronephrosis.

Novel Strategy for Temporary Decompression of the Lower Urinary Tract in Neonates Using a Ureteral Stent.

PURPOSE: In children with congenital obstructive uropathy, including posterior urethral valves, lower urinary tract decompression is recommended pending definitive surgical intervention. Current options, which are limited to a feeding tube or Foley catheter, pose unappreciated constraints in luminal diameter and are associated with potential problems. We assess the impact of luminal diameter on the current draining options and present a novel alternative method, repurposing a widely available stent that optimizes drainage. MATERIALS AND METHODS: We retrospectively reviewed patients diagnosed with posterior urethral valves between January 2013 and December 2014. In all patients a 6Fr 12 cm Double-J ureteral stent was advanced over a guidewire in a retrograde fashion into the bladder. Luminal flow and cross-sectional areas were also assessed for each of 3 tubes for urinary drainage, ie 6Fr Double-J stent, 5Fr feeding tube and 6Fr Foley catheter. RESULTS: A total of 30 patients underwent uneventful bedside Double-J stent placement. Mean ± SD age at valve ablation was 28.5 ± 16.6 days. Mean ± SD peak serum creatinine was 2.23 ± 0.97 mg/dl after birth and 0.56 ± 0.22 mg/dl at the procedure. Urine output after stent placement was excellent in all patients. The Foley catheter and feeding tube drained approximately 18 and 6 times more slowly, respectively, and exhibited half the calculated cross-sectional luminal area compared to the Double-J stent. CONCLUSIONS: Use of Double-J stents in neonates with posterior urethral valves is a safe and effective alternative method for lower urinary tract decompression that optimizes the flow/lumen relationship compared to conventional drainage options.