Joshua D. Roth , Diana Bowen , Molly E. Fuchs , Patricio C. Gargollo , Harrison Gottlich , David S. Hains , Andrew C. Strine , Konrad M. Szymanski
{"title":"End-stage and chronic kidney disease in classic bladder exstrophy: A retrospective muti-institutional cohort study","authors":"Joshua D. Roth , Diana Bowen , Molly E. Fuchs , Patricio C. Gargollo , Harrison Gottlich , David S. Hains , Andrew C. Strine , Konrad M. Szymanski","doi":"10.1016/j.jpurol.2024.10.016","DOIUrl":"10.1016/j.jpurol.2024.10.016","url":null,"abstract":"<div><h3>Introduction</h3><div>While most children with classic bladder exstrophy (CBE) are born with normal kidneys, some experience renal deterioration in adulthood. Little is known about the incidence of end-stage and chronic kidney disease (ESKD and CKD, respectively) in this population. Our group has recently published on surgical outcomes in a multi-institutional cohort of 216 people with CBE. Our aim was to describe the incidence of ESKD and prevalence of CKD in this cohort of people with CBE.</div></div><div><h3>Methods</h3><div>We retrospectively reviewed records of patients with CBE followed at five tertiary care centers described previously. The primary outcome was incidence of ESKD, defined as permanent peritoneal/hemodialysis or renal transplantation. The secondary outcome was prevalence of CKD stage 3 or higher (CKD3+, estimated glomerular filtration rate [eGFR]<60 ml/min/1.73 m<sup>2</sup>) at the last appointment. Creatinine-based eGFRs were calculated using the CKD-EPI Creatinine Equation (adults) and the Schwartz formula (children). Survival analysis and Fisher's exact test were used.</div></div><div><h3>Results</h3><div>A total of 201 patients (93 % of the original cohort) had renal function data available (63 % male). Four patients who had a primary urinary diversion remained diverted at a median follow-up of 20.1 years. None developed ESKD and one developed CKD3+. The remaining 197 patients had a primary bladder closure. At a median follow-up of 18.8 years old, 12 were diverted, 108 were augmented and 77 were neither. Three patients developed ESKD (1.5 %) at a median age of 23.4 years (1 hemodialysis, 2 transplantation). On survival analysis, the risk of ESKD was 0 % at 10 years, 1 % at 20 years and 5 % at 30 years (<strong>Figure 1</strong>). This was higher than the risk of 0.003 % at 21 years of age in the general population (p < 0.001). The median age of 141 individuals with eGFR data was 21.6 years old (65 % male). No children, 4 % of adolescents and 8 % of adults had CKD3+ (p = 0.45). On exploratory analyses, prevalence of CKD3+ did not differ by center or birth year (p ≥ 0.99).</div></div><div><h3>Conclusions</h3><div>The risk of ESKD and CKD among patients with CBE is not insignificant and appears to be more common than the general population. The potential role of modifiable contributing factors, such as increased bladder outlet resistance, warrants further investigation. Reliable long-term follow up is needed in this population to monitor for ESKD and CKD.</div></div>","PeriodicalId":16747,"journal":{"name":"Journal of Pediatric Urology","volume":"21 1","pages":"Pages 41-46"},"PeriodicalIF":2.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142590686","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Qingqing Tian, Chu Zhang, Xiang Zhao, Kechi Yu, Erhu Fang, Xuefeng Zhou, Yonghua Niu, Ning Li
{"title":"Potential impact of severe hydronephrosis secondary to ureteropelvic junction obstruction on pediatric blood pressure","authors":"Qingqing Tian, Chu Zhang, Xiang Zhao, Kechi Yu, Erhu Fang, Xuefeng Zhou, Yonghua Niu, Ning Li","doi":"10.1016/j.jpurol.2024.10.014","DOIUrl":"10.1016/j.jpurol.2024.10.014","url":null,"abstract":"<div><h3>Background</h3><div>Hydronephrosis could affect blood pressure (BP) according to published case reports and animal experiments. The impact on pediatric BP is often overlooked due to children's inherently lower BP, superior vascular elasticity and greater resistance to hypertension than adults.</div></div><div><h3>Objective</h3><div>This study aimed to prospectively observe the effects of hydronephrosis, secondary to ureteropelvic junction obstruction (UPJO), on pediatric BP.</div></div><div><h3>Methods</h3><div>Children with hydronephrosis secondary to UPJO who required pyeloplasty were categorized into five age groups: neonates, infants, toddlers, preschoolers, and school-aged children. Preoperative and postoperative systolic blood pressure (SBP), diastolic blood pressure (DBP), active renin concentration (ARC), and aldosterone concentration (AC) were compared among these age groups, followed by comparison with a control group of children without nephritis or cardiovascular conditions. The impact of severe hydronephrosis secondary to UPJO on pediatric BP and its association with the renin-angiotensin-aldosterone system (RAAS) were examined.</div></div><div><h3>Results</h3><div>This study enrolled 114 children with severe hydronephrosis secondary to UPJO and 153 without nephritic or cardiovascular conditions between September 2021 and June 2023. As the control group aged, SBP and DBP increased, whereas ARC and AC decreased. Overall, hydronephrosis group had higher SBP, DBP, ARC, and AC than the control<del>s</del> group. These differences differed between the age groups. After pyeloplasty, the postoperative BP of hydronephrosis group approximated that of the control group. Postoperative ARC levels were higher than those in the control group but were much lower than the preoperative levels. AC did not decrease significantly after surgery. The change in DBP in children with hydronephrosis before and after pyeloplasty showed a positive correlation with the change in AC.</div></div><div><h3>Conclusions</h3><div>Pediatric patients with severe hydronephrosis, a condition secondary to UPJO, displayed elevated BP, ARC, and AC. Following pyeloplasty, these patients noted a reduction in BP. The correlation between elevated blood pressure and the RAAS necessitates further comprehensive investigation.<span><figure><span><img><ol><li><span><span>Download: <span>Download high-res image (205KB)</span></span></span></li><li><span><span>Download: <span>Download full-size image</span></span></span></li></ol></span><span><span><p><span>Summary figure</span>. </p></span></span></figure></span></div></div>","PeriodicalId":16747,"journal":{"name":"Journal of Pediatric Urology","volume":"21 1","pages":"Pages 108-114"},"PeriodicalIF":2.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142621686","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Commentary on: “Onabotulinum toxin A injections: A novel option for management of refractory nocturnal enuresis” by Tyler Overholt, Davis Temple, Adam Cohen, Anthony Atala, Marc Colaco, Steve Hodges","authors":"Valeska Bidault-Jourdainne","doi":"10.1016/j.jpurol.2024.09.038","DOIUrl":"10.1016/j.jpurol.2024.09.038","url":null,"abstract":"","PeriodicalId":16747,"journal":{"name":"Journal of Pediatric Urology","volume":"21 1","pages":"Pages 53-54"},"PeriodicalIF":2.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142639255","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
David P. Adams , Etienne C. Gozlan , Nikhila Medikonda , Joanna J. Song , Arpan Sahoo , Michelle Yeagley , George Blanck
{"title":"Stratification of Wilms tumor patients using physicochemical properties of the adaptive immune receptor polypeptides, IGL and TRG","authors":"David P. Adams , Etienne C. Gozlan , Nikhila Medikonda , Joanna J. Song , Arpan Sahoo , Michelle Yeagley , George Blanck","doi":"10.1016/j.jpurol.2024.10.012","DOIUrl":"10.1016/j.jpurol.2024.10.012","url":null,"abstract":"<div><h3>Introduction</h3><div>Wilms tumor (WT) is the most common pediatric renal malignancy. Current guidelines that stratify WT risk and determine treatment courses are inadequate, as over 60 % of WT survivors develop treatment-related complications. Recently, numerous advances in establishing patient sub-groups with different clinical features have been realized by evaluating the adaptive immune receptor (IR) complementarity determining region-3 (CDR3) amino acid (AA) sequences, a reasonable series of successes, given the prominent role of the CDR3 in antigen binding, including tumor antigen binding. However, the possibility that adaptive IR chemical variability correlates with distinct survival outcomes for WT has not yet been explored.</div></div><div><h3>Objective</h3><div>The goal of this study was to isolate the T-cell receptor and B-cell receptor recombination, sequencing reads from WT RNAseq files, representing the actual tumor tissue, translate the sequences to AAs, identify the adaptive IR CDR3 domains, and determine whether the physicochemical properties of those CDR3 AA sequences correlated with survival probability distinctions.</div></div><div><h3>Study design</h3><div>WT RNA-seq files were mined to obtain the CDR3 AAs for various adaptive IRs. The physicochemical properties of these CDR3s were examined for trends in how those properties correlated with survival probabilities for WT patients, using a Kaplan–Meier analyses, verified via several approaches.</div></div><div><h3>Results</h3><div>The above processes indicated the association of the (a) IGL CDR3s′ instability index and the (b) TRG CDR3s’ fraction disorder promoting features with better outcomes. Additionally, the IGL CDR3 data were assessed using the Predictor of Natural Disordered Regions web tool, which strengthened the evidence for the association with the IGL CDR3 instability index with a better outcome.</div></div><div><h3>Discussion</h3><div>The approaches described here indicate that greater adaptive IR CDR3 instability and flexibility may serve as prognostic indicators; and may indicate the flexibility of CDR3 domains provides for greater opportunity to bind tumor antigens.</div></div><div><h3>Conclusion</h3><div>Further exploration and development of these approaches and findings may lead to new guidelines for more precise treatment regimens, or even watchful waiting periods, that could thereby decrease the lifetime occurrence of adverse events.<span><figure><span><img><ol><li><span><span>Download: <span>Download high-res image (196KB)</span></span></span></li><li><span><span>Download: <span>Download full-size image</span></span></span></li></ol></span><span><span><p><span>Summary figure</span>. </p></span></span></figure></span></div></div>","PeriodicalId":16747,"journal":{"name":"Journal of Pediatric Urology","volume":"21 1","pages":"Pages 130-135"},"PeriodicalIF":2.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142502580","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Olugbenga Awolaran , Ijeoma Nwachukwu , Anu Paul , Massimo Garriboli , Arash Taghizadeh , Sara Lobo , Karim Awad , Kate Burns , Mohamed Shalaby , Mark Woodward , Pankaj Mishra
{"title":"Endoscopic balloon dilatation of primary obstructive megaureter: An effective first line management in children","authors":"Olugbenga Awolaran , Ijeoma Nwachukwu , Anu Paul , Massimo Garriboli , Arash Taghizadeh , Sara Lobo , Karim Awad , Kate Burns , Mohamed Shalaby , Mark Woodward , Pankaj Mishra","doi":"10.1016/j.jpurol.2024.09.007","DOIUrl":"10.1016/j.jpurol.2024.09.007","url":null,"abstract":"<div><h3>Aim</h3><div>This study evaluates outcomes of endoscopic balloon dilatation (EBD) in the management of primary obstructive megaureter (POM) in children.</div></div><div><h3>Methods</h3><div>Retrospective data between 2013 and 2023 from two tertiary paediatric surgical centres in the UK were reviewed. Pre and post-operative clinical and imaging parameters of children managed with EBD were assessed. Failure of procedure was defined as requiring further intervention due to persistent/recurrent symptoms, upper tract dilatation and/or obstruction on MAG3 over the follow up period.</div></div><div><h3>Results</h3><div>55 children with 61 renal units were evaluated. Median age at treatment was 18 months with a median follow up of 24 months. There was significant reduction in upper tract ultrasound measurements following balloon dilatation but there was no significant difference between the pre and post-operative renal function on MAG3. No significance difference was demonstrated when the outcomes of cutting and non-cutting balloons were compared. No significant difference was shown when outcomes after EBD were compared between infants vs older children as well as ureteric dilatation less than or over 25 mm (p = 0.841). 87% were successfully treated with a single dilatation and this increased to 95% after second dilatation. The remaining 5% had ureteric re-implantation.</div></div><div><h3>Discussion</h3><div>Although a retrospective study, the patient population is relatively large. 87% success rate shown after EBD is comparable to similar studies. It has been suggested that children less than 12months and those with severe ureteric dilatation (>25 mm) may not be suitable for EBD. No significant difference was demonstrated when the outcomes of these categories of children were compared to other children with POM. All of the patients that had repeat balloon dilatation required no further intervention, a finding that has so far not been well evaluated in available literature.</div></div><div><h3>Conclusions</h3><div>This study demonstrates 87% success rate after single EBD in children with POM and this outcome increased to 95% following a second dilatation. EBD is shown to be an effective definitive surgical management option of POM. It can be safely offered as first line management in all patient groups and repeated if no initial response.<span><div><span><span><p><span>Summary table</span>. </p></span></span><div><table><thead><tr><td><span>Empty Cell</span></td><th>P-value</th></tr></thead><tbody><tr><th>Outcomes based on age (median post-op APD)</th></tr><tr><td><ul><li><span></span><span><div>- <12months</div></span></li></ul></td><td>20 units (13 mm)</td><td>0.433</td></tr><tr><td><ul><li><span></span><span><div>- >/ = 12mo</div></span></li></ul></td><td>41 units (13 mm)</td></tr><tr><th>Outcomes based on severity of DUD diameter (median post-op DUD)</th></tr><tr><td><ul><li><span></span><span><div>- <25 mm</div></span></li></ul","PeriodicalId":16747,"journal":{"name":"Journal of Pediatric Urology","volume":"21 1","pages":"Pages 124-129"},"PeriodicalIF":2.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142468273","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ashley W. Johnston , Mimi S. Kim , Paul Kokorowski , Devon J. Hensel , Patrice M. Yasuda , Richard C. Rink , Konrad M. Szymanski , Life with Congenital Adrenal Hyperplasia
{"title":"Experiences and preferences of women with CAH and parents about disclosure of childhood surgery","authors":"Ashley W. Johnston , Mimi S. Kim , Paul Kokorowski , Devon J. Hensel , Patrice M. Yasuda , Richard C. Rink , Konrad M. Szymanski , Life with Congenital Adrenal Hyperplasia","doi":"10.1016/j.jpurol.2024.09.030","DOIUrl":"10.1016/j.jpurol.2024.09.030","url":null,"abstract":"<div><h3>Introduction</h3><div>Complete disclosure of childhood genital surgery to patients with congenital adrenal hyperplasia (CAH) is a critical part of CAH care. There are no guidelines or uniform recommendations on the timing and content of surgical disclosure discussions.</div></div><div><h3>Objective</h3><div>Our objective was to describe the experiences and preferences of females with CAH and parents of females with CAH who underwent childhood genital surgery regarding surgical disclosure.</div></div><div><h3>Methods</h3><div>We conducted an anonymous cross-sectional online survey of females with CAH (46XX, ≥16 years [y] old) and parents of females with CAH who underwent genital surgery before age 4y in North America. Participants reported experiences, preferences, and advice about initial (“first time you were told”) and complete disclosure (“told all details”). Non-parametric statistics and qualitative analysis were used.</div></div><div><h3>Results</h3><div>Participants included 59 females with CAH (median age: 37y, 92% White, 93% non-Hispanic) and 41 parents (median: 36y, 85% White, 93% non-Hispanic, daughter median: 26y). The 76% of females who received complete disclosure were younger (median age: 33y) and underwent surgery more recently (median decade: 1980s) than the 14% who received only initial disclosure (median: 47y, 1970s) and the 10% who did not receive any disclosure (median: 60y, 1960s, p = 0.0003, <strong>Summary Figure</strong>). Females reported median ages of initial and complete disclosure as 7-10y and 11-13y, respectively. Disclosure was preferred by 98% of females with initial disclosure by age 14y and complete disclosure by 18y. Parents reported similar findings. Most disclosures were by mothers (initial: 82%, complete: 64%). Doctors were more involved in complete vs. initial disclosures (complete: 47%, initial: 13%, p < 0.001). Qualitative analysis of advice about surgical disclosure revealed 8 themes.</div></div><div><h3>Conclusions</h3><div>Disclosure of childhood genital surgery to women with CAH has increased over time. Although timing of disclosure varied, women preferred disclosure, and that it be initiated before age 14y and completed by age 18y.<span><figure><span><img><ol><li><span><span>Download: <span>Download high-res image (269KB)</span></span></span></li><li><span><span>Download: <span>Download full-size image</span></span></span></li></ol></span><span><span><p><span>Summary figure</span>. <!-->Surgical disclosure of females with CAH</p></span></span></figure></span></div></div>","PeriodicalId":16747,"journal":{"name":"Journal of Pediatric Urology","volume":"21 1","pages":"Pages 78-86"},"PeriodicalIF":2.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142468274","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Research on hypospadias and appeal to tradition fallacy","authors":"Hüseyin Özbey","doi":"10.1016/j.jpurol.2024.09.034","DOIUrl":"10.1016/j.jpurol.2024.09.034","url":null,"abstract":"","PeriodicalId":16747,"journal":{"name":"Journal of Pediatric Urology","volume":"21 1","pages":"Pages 55-56"},"PeriodicalIF":2.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142502578","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Response to letter to the editor re surgical and functional outcomes of dorsal inlay graft urethroplasty in revision vs primary hypospadias repair in the pediatric age","authors":"Michele Gnech, Carolina Bebi, Alfredo Berrettini, Dario Guido Minoli, Erika Adalgisa De Marco, Gianantonio Manzoni","doi":"10.1016/j.jpurol.2024.10.022","DOIUrl":"10.1016/j.jpurol.2024.10.022","url":null,"abstract":"","PeriodicalId":16747,"journal":{"name":"Journal of Pediatric Urology","volume":"21 1","pages":"Pages 241-242"},"PeriodicalIF":2.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142770049","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Julia Bhuiyan, Raghuveer Kavarthapu, Mary Soliman, G Thomas Brown, Jacqueline C. Yano, Tazim Dowlut-McElroy, Veronica Gomez-Lobo
{"title":"Histologic analysis of gonadal tissue in patients with Turner syndrome and Y chromosome material","authors":"Julia Bhuiyan, Raghuveer Kavarthapu, Mary Soliman, G Thomas Brown, Jacqueline C. Yano, Tazim Dowlut-McElroy, Veronica Gomez-Lobo","doi":"10.1016/j.jpurol.2024.08.007","DOIUrl":"10.1016/j.jpurol.2024.08.007","url":null,"abstract":"","PeriodicalId":16747,"journal":{"name":"Journal of Pediatric Urology","volume":"21 1","pages":"Pages 222-223"},"PeriodicalIF":2.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142140327","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}