Posterior Urethral Valves Research Articles

Evaluation of predictive performance of fetal urinary inflammatory markers of postnatal kidney function in fetuses with posterior urethral valves

Abstract Background There are proposed roles for inflammation in the development of congenital obstructive uropathy in the setting of posterior urethral valves (PUV). However, the value of inflammatory proteins as predictive markers of postnatal kidney function, key in the management of fetuses with PUV, has not been explored. We screened fetal urine of fetuses with PUV with a panel of inflammatory proteins to determine their predictive value of postnatal kidney function. Methods Twenty-five different chemokines and cytokines were measured using a multiplex immunoassay in fetal urine of 79 PUV patients from retrospective cohorts, separated in discovery (n = 52) and validation (n = 27). The candidate markers were also quantified in amniotic fluid samples obtained from 16 PUV and 25 other congenital anomalies of the kidney and the urinary tract pregnancies. The performance of validated candidate inflammatory proteins was compared to the previously published 12PUV fetal urine peptide signature. Results Fetal urine chemokines CCL2 (MCP-1), CXCL9 (MIG), and CCL4 (MIP-1β) were identified as predictive of postnatal kidney failure in fetuses with PUV from the discovery cohort. Their predictive potential was confirmed in the validation cohort (AUCs of 0.87, 0.81, and 0.86, respectively). The performance of these individual chemokines was lower than the previously published 12PUV fetal urine peptide signature. However, the combination of the three chemokines performed similarly to 12PUV. In contrast, these three chemokines were not predictive of outcome in amniotic fluid. Conclusions We identified chemokines in fetal urine of PUV pregnancies that, after external validation, could serve as predictive biomarkers of postnatal outcome and contribute to improve prenatal PUV management. Graphical Abstract

Spectrum of congenital anomalies of the kidney and urinary tract in a pediatric nephrology clinic in Ethiopia and factors associated with advanced chronic kidney disease: A cross-sectional study.

Chronic kidney disease (CKD) is a significant health concern for children with congenital anomalies of the kidney and urinary tract (CAKUT). To address the limited data on CKD prevalence and risk factors in Ethiopia, this study focuses on a specific cohort of CAKUT patients at the pediatric renal unit of Tikur Anbessa specialized hospital. We aim to determine the prevalence of CKD in Ethiopian CAKUT patients, identify independent predictors of advanced stage CKD, and explore the distribution of CKD among different CAKUT phenotypes. Medical charts of children with CAKUT who visited pediatric renal unit were reviewed. Logistic regression analyses were performed to determine the independent predictors of advanced CKD. Statistical significance was set at p ≤ 0.05.The study included 129 male subjects (70.9%). Only 25.3% of CAKUT cases were diagnosed prenatally. About 52.2% of patients had unilateral CAKUT. Posterior urethral valves (PUV) were the most commonly diagnosed CAKUT phenotypes (27.5%). Fifty-three (29.1%) of the patients had CKD, and stage 3 was the most diagnosed CKD (37.7%). CKD development was observed more frequently in patients with isolated PUV (39.6%). Factors like low birthweight, prematurity at birth, and the presence of bilateral CAKUT emerged as independent risk factors for developing advanced stage CKD. This study reveals a significant burden of CKD in children and adolescents with CAKUT. Despite its importance, prenatal CAKUT detection remains low. Posterior urethral valves are the most prevalent phenotype, associated with a higher risk of CKD. The research also highlights independent predictors for advanced CKD development.

Predicting chronic kidney disease progression in children with posterior urethral valves

ObjectivePosterior urethral valves (PUV) leads to early chronic kidney disease (CKD) and renal failure in some children, while others may maintain preserved kidney function even into adulthood. Our goal was to assess the association between early imaging features and CKD progression in children with PUVs. We hypothesized that imaging features identified on PRUS and VCUG would be associated with CKD progression and could` be used in conjunction with nadir creatinine to predict future renal function. MethodsFollowing individual institutional research board approvals, we performed a retrospective cohort study at two institutions. Electronic medical records were queried to identify all patients with a history of PUVs treated between 1990 and 2022. Children who presented in the first year of life and had their initial renal bladder ultrasound (RBUS) and voiding cystourethrogram (VCUG) performed within the first 90 days of life were included. The primary outcome, CKD progression, was defined as development of ESKD requiring dialysis or renal transplant or a decline in eGFR of greater than 50%. Clinical variables of interest included: findings on initial RBUS (urinoma, cystic dysplasia, solitary kidney), vesicoureteral reflux on initial postnatal VCUG (degree and laterality), and nadir creatinine. Hazard ratios (HRs) were calculated from Cox proportional hazards regression for univariate and multivariable regression. ResultsOut of 537 patients with PUVs, 274 met our inclusion criteria. Median follow up was 5.84 years (IQR 2-10.2), and 55 patients (20%) reached our primary outcome. Multivariable analysis showed the presence of any degree of vesicoureteral reflux, HR 3.1 (95% CI 1.62-5.93), and nadir creatinine, HR 5.53 (95% CI 4-7.64), were predictive of CKD progression When nadir creatinine within the first year of life is used as a sole predictor of CKD progression, the area under the receiver-operator curve was 0.89 (95% CI .83-.91). ConclusionsThis study confirms nadir creatinine as a strong predictor for CKD progression and ESKD in patients with PUVs. In our cohort, the presence of any degree or laterality of vesicoureteral reflux, was also a significant predictor for CKD progression.

The effect of urinary diversion on kidney function in posterior urethral valves and ureterovesical obstruction.

Posterior urethral valves (PUV) and ureterovesical junction obstruction (UVJO) are common etiologies of lower urinary tract obstruction. The primary objective of therapy is to alleviate the obstruction in the urinary system. Temporary urinary diversion may be necessary in certain situations, such as Urinary tract infection (UTI), sepsis, and unchanging hydronephrosis. This study, aims to assess kidney parameters after urinary diversion (either pyelostomy or ureterostomy) in patients with PUV and UVJO. We conducted a retrospective analysis of all patients diagnosed with PUV or UVJO and treated with urinary diversion following urinary undiversion at our facility between 2015 and 2020. The following variables were collected: demographic details, surgical interventions (type of diversion), serum creatinine throughout follow-up, anterior-posterior diameter of the pelvis (APP), anterior-posterior diameter of the ureter (APU), and sonographic findings of renal parenchymal thickness (PT) as documented by a pediatric radiologist. Additionally, before and after urinary diversion, a Dimercapto succinic acid (DMSA) nuclear renal scan was conducted to assess renal function. We analyzed 67 patients, with a mean follow-up of 38.3months, undergoing either ureterostomy (42 patients) or pyelostomy (25 patients). This included 38 patients with PUV and 29 with UVJO. In PUV patients, significant improvements were observed in APP (MD = 5.56 ± 11.6, p = 0.0194), APU (MD = 5.57 ± 7.28, p < 0.001), and PT (MD = 3.66 ± 2.75, p < 0.001). Similarly, UVJO patients experienced significant improvements in APP (MD = 12.18 ± 18.63, p = 0.005), APU (MD = 7.82 ± 8.98, p = 0.001), and PT (MD = 2.79 ± 3.33, p = 0.001). DMSA scores did not significantly change in either group (p > 0.05). Notably, APP improved more in UVJO patients compared to PUV patients (p = 0.047). Our study suggests that urinary diversion could improve APP, APU, and renal parenchymal thickness in PUV and UVJO patients. The improvement of APP in UVJO patients was greater than in the PUV group. We propose further multi-center studies with longer durations of follow-up and more detailed additional data to support and confirm our results.

Care of children with posterior urethral valves after initial endoscopic incision/ablation: what a nephrologist needs to know.

Posterior urethral valves (PUV) are the most common cause of congenital urethral obstruction and are unique in the challenges they pose in management. Endoscopic ablation/incision of the valves is usually offered as the primary treatment of choice. Following this, a range of different clinical patterns are observed, each with varying bladder dysfunction and continence issues and associated with different grades of chronic kidney disease. This review outlines a systematic approach that could help pediatric nephrologists, pediatricians, and pediatric urologists, as well as nursing and allied health specialists, assess these children and develop well-informed management plans. The need for surveillance for bladder dysfunction (incidence approximately 55%), identification of red flags for progression to kidney failure (incidence approximately 20%), and multidisciplinary approach to care are presented, with a focus on reducing long-term morbidity in patients and difficulties for the families. Where possible, an alternative in resource-constrained situations is suggested. We also briefly outline the role of pharmacotherapy, assisted bladder emptying/drainage, and other interventions that have a role in the medium- to long-term management of these patients.

Postnatal Outcomes in Cases of Prenatally Detected Dilated Fetal Bladder

Objectives: To explore the long-term outcomes in foetuses presenting with an enlarged bladder antenatally. Methods - Design: A retrospective cohort study was conducted over a 15-year period between 2003 and 2018. Setting and participants: Foetuses suspected to have enlarged bladders antenatally were included. Outcome measurements and statistical analysis: Analyses were done based on the trimester the enlarged bladder was detected, live births, terminations, surgical interventions, creatinine levels and bladder measurements post-delivery, and subsequent health outcomes. Results: From the 50 foetuses identified prenatally, detections were as follows: first trimester (9 cases), second trimester (30 cases), and third trimester (11 cases). There were 14 terminations (28%), 5 stillbirths (10%), and 31 live births (62%). Among the terminated and stillbirth/neonatal death cases, the most frequent diagnosis in males was PUV (57%). Two female foetuses were terminated due to lumbosacral spina bifida and sacrococcygeal teratoma obstructing the ureters, respectively. In surviving males, PUV was predominant (48.3% of 29 males). In five cases megacystis had resolved by birth, including one following in-utero surgery. Among the two female cases, one issue was resolved by birth, and the second had bilateral ureteroceles. Post-birth, surgical intervention was needed in 61% of males and 50% of females. The commonest surgery was ablation of posterior urethral valves. The median age for surgery was 1.5 months with a median follow-up of 4 years and the longest follow-up of fourteen years. Six children had no postnatal renal or urological follow-up. The mean peak creatinine level was 132, dropping to 47 in the latest measurement. Half of the patients with pathology evident at birth exhibited abnormal bladder function. There was one neonatal death. Three patients necessitated a kidney transplant due to chronic kidney disease. Conclusion: Foetuses diagnosed with an enlarged bladder in the second trimester most frequently had the posterior urethral valve as the underlying cause. For patients continuing the pregnancy, most had live births. About 1 in 10 posterior urethral valve survivors developed chronic kidney conditions, leading to a renal transplant.

Think Tank 2: How Do We Precisely Define the "High Risk Bladder" and What Are the Interrelationships Between Inflammation, Blood Flow, Fibrosis, and Loss of Bladder Compliance?

Defining "high-risk bladder" or "high-pressure bladder" involves recognizing the potential for an unsafe lower urinary tract, where dysfunction in storage and micturition can threaten upper urinary tract health, leading to unfavorable outcomes like dialysis, recurrent infections, systemic impact, or mortality. ICI-RS was held in Bristol in June 2024, and Think Tank 2 aimed to define research priorities including identifying clinical predictors and developing prevention and monitoring strategies. Risk factors encompass both congenital and neurogenic lower urinary tract dysfunction, bladder outlet obstruction, vascular diseases, and inflammatory disorders, but a validated stratification risk is lacking. Reduced compliance and detrusor overactivity lead to high filling pressures and raised detrusor leak point pressure, playing urodynamic studies a crucial role in risk assessment, though further research is needed for different neurogenic populations. Congenital conditions such as spina bifida, posterior urethral valves, and bladder exstrophy also contribute to a high-risk bladder through fibrosis and reduced compliance. Inflammation and ischemia are key factors, with inflammation leading to fibrosis and impaired bladder storage and voiding function. Novel treatments, including sGC activators, PDE5 inhibitors, and regenerative therapies like stem cell injections and extracorporeal shock wave treatment, show promise in mitigating fibrosis and improving bladder compliance. Identifying and validating clinical risk stratification models, precise biomarkers and therapeutic windows remains essential for effective management and reversal of bladder fibrosis and dysfunction.

Role of MCU in the Management of Posterior Urethral Valves

Objective: In infancy, the most common cause of bladder outlet obstruction is Posterior urethral valves (PUV), which impair renal and bladder function. This study was planned to evaluate and record the role of MCU in the various clinical presentations and management, complications, surgical management, and long-term outcomes of PUV. Methods: In this study, 49 patients treated for PUV were evaluated at the Bangladesh Shishu Hospital and Institute from May 2018 to April 2021. Complete data were taken, paraclinical examinations were performed on each patient, and the diagnosis was confirmed by micturating-cysto-urethrography (MCU). Posterior urethral valves were ablated in all patients using an electric hook. Results: A total of 49 patients with a mean age at diagnosis of 42(±21) days were included in this study. Catheterization was done within one to 5 days of life in 29 patients. PUV was ablated by an electric hook in all 49 patients. The most common symptoms in our group were dribbling, poor stream (71%), and urinary tract infection (UTI) (46%). There was vesico-ureteral-reflux (VUR) in 52% and hydronephrosis in 92.5%. The most common associated anomaly was kidney anomalies (Renal agenesis/dysplasia and multicystic kidney disease) in 4(8.2%) patients. About ten patients had prenatal diagnosis of PUV. Complications occurred in two (4.2%) patients. Mortality occurred in 3 (6.4%) patients. The mean follow-up period was 3.2±0.8 years (1.5 months to 4 years). Conclusion: MCU is the gold-standard imaging modality for documenting PUV and Postoperative follow-up to assess the condition of the posterior urethra urinary drainage by catheter in the early days of infancy. The factors like renal dysplasia and UTI have their role in the outcome.

A new spectrum of neonatal urethral pathologies in the era of early vesicoamniotic shunting?

Intrauterine vesicoamniotic shunting (VAS) was shown to affect survival of male fetuses with megacystis in suspected lower urinary tract obstruction (LUTO). Data on postnatal management are largely lacking. We aim to describe the pathologies diagnosed in children born after vesicoamniotic shunt placement in early pregnancy for megacystis. All newborns with previous intrauterine VAS treated in our institution were analyzed retrospectively. We evaluated the clinical spectrum of urethral pathologies. We also compared patients who received a shunt before the 17th gestational week with those who received it later. Between 2014 and 2023, 26 patients (all male) with a history of VAS for suspected LUTO were treated in our institution postnatally. Five fetuses with dislocated shunts underwent re-implantation in utero. Overall, premature birth before the 38th week of gestation was observed in 14 patients. Seven patients received a Harrison® shunt whereas 19 received a Somatex® shunt. Twelve patients required surgical shunt removal under general anesthesia due to shunt migration/embedding. Posterior urethral valves were found in 10/26 patients, 10/26 patients showed a urethral hypoplasia [Fig.1] and two patients had urethral duplications. In two patients, we identified a prune belly syndrome. One patient had posterior and anterior urethral valves. One patient had a high grade bilateral vesicoureteral reflux without LUTO. The 11 patients shunted early (before 17GW) showed a trend towards a higher proportion of urethral hypoplasia in the early shunt group (54% vs. 26%) without statistical significance. In our observation, patients treated with VAS had a noticeable high proportion of complex urethral pathologies such as urethral hypoplasia. These data should be taken into consideration for prenatal counselling of parents and planning of postnatal management.

Posterior urethral valves: Clinical audits of presentation, diagnostic and therapeutic intervention in a nigerian teaching hospital.

We described our experience in the management of PUV at UNIOSUN Teaching Hospital, Osogbo, Osun State. This was a retrospective analysis of patients with diagnosis of PUV carried out over a 4year period (2019-2023). Demographic characteristics, clinical presentation, diagnosis and mode of therapeutic interventions were the variables analysed. A total of 17 patients with PUV were managed during the study period. Median age at presentation and surgery were 10months (range 3.0-48.0) and 13months (range 3.0-49.5), respectively. Most common presentation was poor urinary stream, 11 (64.7%). Mean PCV was 34.42%. Klebsiella aerogens was the predominant 9 (52.9%) organism isolated. A patient had prenatal USS diagnosis suggestive of PUV, majority (52.9%) had bilateral grade 1V hydronephrosis at presentation. Voiding cystogram was diagnostic in 14 patients, (82.4%) while urethrocystoscopy was done in 14 (82.4%) patients. Median creatinine level were 116, 76.5 and 51.0 (micromol/l) pre- and post-catheterization and 1month post-surgery, respectively. There was positive correlation between the age and post- surgery creatinine but a negative correlation between the PCV and grade of hydronephrosis. All patients had Mohans valvotomy. We had mortality in a patient from urosepsis. At 6months-1year follow-up, 15 patients had good urine stream with stable renal function. Early intervention assist in optimizing renal and bladder function and minimize risk of urosepsis. Where there is no facility for endoscopic valve ablation, Mohans valvotomy remains a viable treatment option.

Comparison of the Role of Anticholinergics and α-1 Adrenergic Blockers in Bladder Management in Posterior Urethral Valves: A Pilot Randomized Control Trial

Introduction: Posterior urethral valve (PUV) is a major cause of congenital bladder dysfunction, often persisting despite treatment. Emerging therapies, including anticholinergics and α-1 blockers, offer potential but lack clear guidelines. This study evaluates their effectiveness in improving bladder function after valve fulguration. Methods: Twenty PUV patients, aged ≥3 years, were randomized into anticholinergic (group A, n = 11) and α-1 adrenergic blocker (group B, n = 9) groups post-fulguration. Follow-up included clinical, radiological, and urodynamic assessments 6 months posttreatment initiation. Results: In group A, the mean maximum detrusor pressure (Pdet) decreased from 30.17 to 23.45 cm H2O (p = 0.033). Two patients normalized from high detrusor pressure (>40 cm H2O). In group B, 1 patient retained high detrusor pressure posttreatment. Group B improved in average urinary flow (Q avg) and maximum flow rate (Q max), with all patients having initially low Q avg (<10 mL/s). Two group B patients showed improved average flow rates posttreatment (p = 0.016); three in group A showed improvement but were not statistically significant (p = 0.197). Q max/flow time ratio was abnormal in all group B patients pretreatment. Two of the nine improved posttreatment, while only one in group A did. Conclusions: Anticholinergic medications positively impact cystometric parameters and are effective for detrusor instability and low compliance bladder. α-Adrenergic blockers influence uroflow parameters and can help treat bladder outflow obstruction. Consideration for a larger study with extended follow-up is warranted.

Utilizing artificial neural network system to predict the residual valve after endoscopic posterior urethral valve ablation.

To build, train, and assess the artificial neural network (ANN) system in estimatingthe residual valve rate after endoscopic valve ablationand compare the data obtained with conventional analysis. In a retrospective cross-sectional study between June 2010 and December 2020, 144 children with a history of posteriorurethral valve (PUV) who underwent endoscopic valve ablation were enrolled in the study. MATLAB software was used to design and train the network in a feed-forward backpropagationerror adjustment scheme. Preoperative and postoperative data from 101 patients (70%) (training set) were utilized to assessthe impact and relative significance of the necessity for repeatedablation. The validated suitably trained ANN was used to predict repeated ablation in the next 33 patients (22.9%) (test set)whose preoperative data were serially input into the system. To assess system accuracy in forecasting the requirement for repeatablation, projected values were compared to actual outcomes. The likelihood of predicting the residual valve was calculatedusing a three-layered backpropagating deep ANN using preoperativeand postoperative information. Of 144 operated cases, 33 (22.9%) had residual valvesand needs to repeated ablation. The ANN accuracy, sensitivity, and specificity for predicting the residual valve were 90.75%,92.73%, and 73.19%, respectively. Younger age at surgery, hyperechogenicity of the renal parenchyma, presence of vesicoureteralreflux (VUR), and grade of reflux before surgery were among the most significant characteristics that affectedpostoperative outcome variables, the need for repeated ablation, and were given the highest relative weight by the ANN system. Conclusions: The ANN is an integrated data-gathering tool for analyzing and finding relationships among variables as a complex non-linear statistical model. The results indicate that ANN is a valuable tool for outcome prediction of the residualvalveafter endoscopic valve ablation in patients with PUV.

Expression of Wnt signaling proteins in rare congenital bladder disorders

Introduction and AimsCongenital bladder anomalies are rare and are a leading cause of end stage renal failure in children. The Wnt signaling pathway, important during embryonic development, has been implicated in the pathogenesis of these conditions through regulation of gene expression, including essential transcription factors. We investigated the expression of four Wnt transcriptional targets, namely, Pygopus 1 (Pygo1), FRA1, TCF7L1 and Connexin 43 (Cx43) in three rare congenital bladder disorders: bladder exstrophy (BE), neurogenic bladder (NGB) and posterior urethral valves (PUV). MethodsBladder tissue samples were collected from patients at the Great Ormond Street Hospital for Sick Children, London, UK, with control (normally-functioning bladder, N=9), BE (N=15), NGB (N=6) and PUV (N=5). Histological analysis was performed using the van Gieson stain to differentiate smooth muscle (SM) and connective tissue (CT) compartments. An unbiased, automated, semi-quantitative immunofluorescence analysis was performed to measure the labelling intensity of four Wnt-related proteins in tissue from these four groups. Results and DiscussionThere was a significant (p<0.05) increase in the expression of Pygo1 in the smooth muscle of all anomalies examined and also in the connective tissue in PUV compared to control. Cx43 also showed overexpression in the smooth muscle across all conditions; however, there was a reduced expression in NGB and an increase in PUV in connective tissue. TCF7L1 showed a significant decrease in both tissue compartments for NGB, whereas FRA1 expression remained unchanged across all anomalies. We also measured colocalization of Wnt-related proteins. TCF7L1 exhibited increased colocalization with Pygo1 and FRA1 in exstrophy compared to control. These results suggest a complex dysregulation of the Wnt pathway in congenital bladder disorders. ConclusionWnt signaling-related proteins show dysregulation in congenital bladder disorders compared to control tissue. Understanding these mechanisms should help towards non-invasive early diagnosis, drug target discovery and development of treatment strategies for these conditions.Summary tableSummary of Wnt-related protein expression and colocalization in bladder exstrophy (BE), neurogenic bladder (NGB) and posterior urethral valves (PUV) disorder compared to normally-functioning control bladder. The table shows the levels of expression as significantly up (↑) down (↓) or no change (-) of Pygopus 1 (Pygo1), Connexin 43 (Cx43), FRA1 and TCF7L1 expression in smooth muscle and connective tissue compartments in BE, NGB and PUV relative to control bladder tissue samples. The table also shows colocalization of six protein pairs of Pygo1 and FRA1; Pygo1 and Cx43; Pygo1 and TCF7L1; FRA1 and Cx43; FRA1 and TCF7L1 and Cx43 and TCF7L1 in bladder disorders compared to control.Summary tableProtein Expression (Smooth muscle)Pygo1Cx43FRA1TCF7L1BE↑↑--NGB↑↑-↓PUV↑↑--Protein Expression (Connective tissue)BE----NGB-↓-↓PUV↑↑--ColocalizationPygo1/FRA1Pygo1/Cx43Pygo1/TCF7L1FRA1/Cx43FRA1/TCF7L1Cx43/TCF7L1Exstrophy--↑-↑-NGB------PUV------

Pediatric Kidney Transplantation in an Under-resourced Country: A Single-Center Experience.

Kidney transplant is the optimal method for managing children with end-stage kidney disease. This study aimed to present the experience and results of the pediatric kidney transplant program at our center. A single-center observational study was conducted at Children's University Hospital in Damascus, Syria. We reviewed the medical records of all kidney transplants performed between April 2018 and December 2022. All patients were under 14 years old when they underwent kidney transplant. All donors were living, as an effective deceased donor program is currently not an option in Syria. From April 2018 to December 2022, 33 pediatric kidney transplants were performed at our center. Most recipients were boys (n = 24; 72.7%), and 9 were girls (27.3%). Median age was 10 years (range, 4.5-14 y). The most frequent underlying diseases for kidney failure were renal dysplasia-hypoplasia (42.5%), reflux nephropathy (18.2%), and neurogenic bladder with posterior urethral valves (12%). In 10 patients, kidney transplant was performed preemptively (30.3%). Initial graft function was observed in all grafts (100%). During observation time (5 years), 28 patients (85%) retained adequate graft function. The 1-year graft and patient survival rates were 91%. Syria is still suffering from the consequences of the war, affecting organ transplantation in many ways. The good 1-year graft and patient survival in our center was encouraging. However, we admit that the number of patients was small and the follow-up duration was also not long enough; future research is needed to improve long-term outcomes.

Defining the Urine Proteome in Boys with Posterior Urethral Valves

Defining the Urine Proteome in Boys with Posterior Urethral Valves

The integral co-management role of the medical pediatric urologist: Improving the care of children with urological conditions

The complexities of modern medicine prompt a re-evaluation of traditional patient care models to enhance safety and quality. We implemented a one-year pilot Medical Pediatric Urology fellowship, jointly developed by Urology and Pediatric Hospital Medicine, where a pediatrician received postgraduate training in both specialties. This innovative program aimed to augment knowledge and expertise in non-surgical aspects of pediatric urology. Upon completion of the fellowship, the trained pediatrician assumed the role of medical pediatric urologist (MPU), integrating into both Pediatrics and Urology faculties. Unlike the traditional specialist consultation model, the MPU became integral member of the Urology Division, working full-time and sharing responsibilities for both inpatient and outpatient pediatric urology care at a tertiary pediatric hospital in Canada. This study aims to delineate the training curriculum, role, clinical outcomes, and educational impact of the MPU over eight years. We retrospectively analyzed clinical outcomes, including patient volumes seen annually by MPU, urologists, and nurse practitioners from 2021 to 2023. Clinic wait times and patient satisfaction were compared between MPU's community and tertiary hospital pediatric urology clinics. Educational impact was assessed through pediatric residents' ratings for the MPU as a clinical teacher during urology rotations using a Likert scale. Integration of MPU led to the establishment of specialized clinics, including those for neurogenic bladder and posterior urethral valves. MPU accounted for approximately 34.7% of all outpatient clinic visits. Subjective feedback highlighted enhanced quality of care with MPU co-management in the inpatient setting. Additionally, in a community-based medical urology clinic (CUC) staffed by the MPU and a pediatric urology nurse practitioner, patients experienced shorter wait times and higher satisfaction (53±36 days and 194±108 days) (p<0.01), with 97% vs. 91% of families reported feeling highly satisfied with care (p<0.01) compared with hospital clinics, respectively. The MPU received ratings of 4/5 or 5/5 as a clinical teacher from 81.82% of pediatric residents. Our novel co-management approach in pediatric urology integrates medical and surgical expertise. However, limitations include the retrospective design and single-center setting. Nonetheless, this framework presents a potential model for other surgical specialties, offering a unique practice niche for trainees interested in the medical management of surgical conditions. The medical pediatric urologist plays a pivotal role as an educator, hospitalist, and director of the outpatient clinic, effectively integrating medical and surgical expertise. The MPU model is promising for optimizing safety and quality of care in pediatric urology.

Posterior Urethral Valve Masquerading as Failure to Thrive

Posterior Urethral Valve Masquerading as Failure to Thrive

Distinguishing Features of Childhood Renal Dysplasia

Abstract Background Renal dysplasia (RD) is a major cause of chronic kidney disease (CKD) in childhood. Herein, we present a single-center experience about distinctive features of different subtypes of dysplasia. Methods All children with RD admitted between January 2018 and June 2019 were included in this cross-sectional study. Patients were classified as having unilateral RD, bilateral RD, posterior urethral valves (PUV), and multicystic-dysplastic kidneys (MCDK). Results A total of 198 patients (108 unilateral RD, 31 bilateral RD, 16 PUV, and 43 MCDK) with a follow-up period of 80 (1.5–240) months were included. The overall consanguinity rate was 22%, whereas it was 56% in bilateral RD patients. Two-thirds had additional urogenital anomalies. Hypertension was detected in 16% of the patients, and 20% had median proteinuria of 16 (5.2–173) mg/m2/h, which was significantly higher in the PUV and bilateral RD groups. The overall rate of CKD was 23%, which was more frequent in bilateral RD and PUV groups. The median age at end-stage renal disease was 91.5 (0.5–208) months, including 12 renal transplant patients. Conclusions As different subtypes of RD have distinct clinical and prognostic features, the follow-up of RD patients should be individualized, and modifiable complications such as proteinuria and HT should be monitored closely.

Comparison of Clinical, Etiological and Antibiotic Sensitivity Patterns of UTI in CAKUT and Non CAKUT Children

Background: Urinary tract infections (UTIs) are one of the most common bacterial infections in children. Congenital anomalies of the kidney and urinary tract (CAKUT) are a major cause of UTIs in this population. Objective: To compare clinical, etiological and antibiotic sensitivity patterns of UTIs in CAKUT and non-CAKUT children. Study Design: Prospective comparative study. Place and Duration: The study was conducted at the department of Pediatric Nephrology, The Children's Hospital Lahore from August 2023 to January 2024. Methods: 68 children with and without CAKUT having UTI were evaluated. Data regarding demographics, clinical presentation and antibiotic sensitivity pattern was collected. Susceptibility testing was done according to CLSI recommendations. Results: 34 patients with UTI in CAKUT group (Group A) had male predominance (73.5%) while children without CAKUT (Group B) were predominantly females (55.9%). Mean age in Group A (38.9 months) was less than that in Group B (53.9 months). The common presentations in both the groups were pyelonephritis and cystitis. Vesico-ureteric reflux (32.4%) and posterior urethral valves (29.4%) were the most common CAKUT findings. Majority of patients with CAKUT had Pseudomonas UTI (32.4%) while E. coli (35.3%) was common in children without CAKUT. Fosfomycin, Colistin, Linezolid and Polymyxin B had low resistance amongst both groups. The association between CAKUT and atypical uropathogens was not found to be statistically significant (p = 0.287). Conclusion: Differences exist in clinical presentation, etiology and antibiotic sensitivity patterns specific to UTI in CAKUT and non-CAKUT children. A high resistance pattern was found amongst common antibiotics.

Boys with bladder dysfunction may have posterior urethral valves – A simple framework to aid investigation

Bladder dysfunction (BD) is a common presenting complaint to paediatric urology outpatient clinics, accounting for up to 47% of referrals and affecting one in ten children of toilet training age. Boys with bladder dysfunction have been shown to have higher rates of posterior urethral valves (PUV) however when to consider cystourethroscopy in this group is unclear. Our primary aim was to assess the likelihood of detecting PUV in a cohort of boys of toilet training age presenting to a paediatric urologist with symptoms of bladder dysfunction. Our secondary aim was to assess whether resection of PUV was associated with improved clinical outcome. Retrospective review of male patients aged 4-16 (January 2007 to December 2023), referred to paediatric urologist in an Australian outpatient clinic with BD undergoing cystourethroscopy. Those with known renal tract or spinal pathology were excluded. Patients were divided into four groups according to their symptoms/investigation results. Patients were followed up to assess improvement in symptoms following resection of PUV. 247 boys were included in this study with a median age of 8.1 years. 81/247 (32.8%) had PUV on cystourethroscopy. The highest incidence of PUV 13/25 (52%) was in those patients with BD, haematuria or UTI and ultrasound (USS) changes. There was no difference in outcome improvement in those who underwent resection of PUV when compared with those without PUV. Our study findings concur with results from previous literature identifying higher rates of PUV in older boys with bladder dysfunction. We have utilised straightforward clinical criteria to provide a targeted framework for screening with cystourethroscopy. There was no difference in outcomes when PUV was resected, compared to those without PUV. Limitations of this study include the retrospective nature, possible selection and reporting biases. The likelihood of detecting PUV in our cohort of boys aged 4-16 with bladder dysfunction was 32.8%. The four groups provide clinicians with guidance on who may benefit from cystoscopy. This study provides future scope for a prospective interventional study of PUV in boys with BD.