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Zinner syndrome in childhood and adolescence: Report of four cases and review of the literature. 儿童和青少年时期的津纳综合征:四例病例报告和文献综述。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-09 DOI: 10.1016/j.jpurol.2024.10.001
Ivana Dasic, Djordje Topalovic, Polina Pavicevic, Sofija Cvejic, Sasa Milivojevic

Zinner syndrome is a rare congenital malformation of the urinary system that occurs exclusively in males. It consists of renal agenesis, ipsilateral obstruction of the ejaculatory duct, and cystic changes in the seminal vesicles. It is rarely described in the pediatric population due to the absence of symptoms, as well as the failure to recognize it due to masking by other morphological changes and conditions. Four patients from the pediatric population are presented. Two patients were asymptomatic, and the changes were detected incidentally or during other diagnostic procedures. The other two patients exhibited symptoms and signs such as testicular pain, abdominal pain, hematuria, and hematospermia, along with megaureter. Ultrasound serves as the initial diagnostic method, while confirmation of Zinner's syndrome is established by MR urography. In cases that ultrasound verified kidney agenesis and ipsilateral anechoic, avascular tubulocystic structures, it is necessary to perform follow-up MR urography to confirm or exclude Zinner's syndrome. Following diagnosis, asymptomatic pediatric patients should be monitored every 6 months to a year, using ultrasound, with particular attention to cyst size and content.

津纳综合征是一种罕见的先天性泌尿系统畸形,只发生在男性身上。它包括肾脏发育不全、同侧射精管阻塞和精囊囊性改变。由于没有症状,以及被其他形态变化和疾病所掩盖而无法识别,这种病在儿童群体中很少见。本文介绍了四名儿童患者。其中两名患者没有症状,这些变化是偶然发现的,或者是在其他诊断过程中发现的。另外两名患者表现出睾丸疼痛、腹痛、血尿、血精以及巨输尿管等症状和体征。超声波是初步诊断方法,而 Zinner's 综合征的确诊则要通过磁共振尿路造影术。如果超声波检查证实肾脏缺如和同侧无回声、无血管的肾小管囊性结构,则有必要进行后续的磁共振尿路造影检查,以确诊或排除锌纳氏综合征。确诊后,无症状的儿科患者应每 6 个月至 1 年接受一次超声波监测,尤其要注意囊肿的大小和内容物。
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引用次数: 0
Urologist burnout: Prevalence, impact, and strategies for resilience. 泌尿科医生的职业倦怠:泌尿科医生的职业倦怠:发生率、影响和应对策略。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-09 DOI: 10.1016/j.jpurol.2024.09.033
Yoshiyasu Takefuji
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引用次数: 0
Is robot-assisted appendicovesicostomy equivalent to the current gold standard open procedure? A comparative analysis. 机器人辅助阑尾造口术是否等同于目前的黄金标准开放手术?对比分析。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-08 DOI: 10.1016/j.jpurol.2024.09.027
Suhaib Abdulfattah, Sahar Eftekharzadeh, Emily Ai, Aznive Aghababian, Maya Overland, Sameer Mittal, Arun K Srinivasan, Aseem R Shukla

Introduction: Robot-assisted Laparoscopic Appendicovesicostomy (RALAPV) is increasingly performed as a minimally invasive alternative to the open appendicovesicostomy (OPAV), but questions remain regarding the efficacy of the RALAPV compared to OPAV.

Objective: To assess and compare outcomes for non-augmented RALAPV to the open surgical approach.

Materials and methods: An IRB approved prospective registry was retrospectively examined to abstract all patients who underwent APV without augment between 2012 and 2023. Baseline demographics, intra and post-operative characteristics, and long-term outcomes were aggregated and compared. P-values were two sided and a p-value <0.05 was considered statistically significant.

Results: 52 children were identified, 19 (36.5%) OAPV and 33 (63.5%) RALAPV. The median age at surgery was 8.5 years for OAPV and 9.3 years for RALAPV (p = 0.29). Longer operative time was noted in the RALPAV group (346 min vs 289 min, p = 0.04), with a lower estimated blood loss (5 cc vs 30 cc, p = 0.003), shorter median length of hospital stay (4 days vs 5 days, p = 0.07), and lower IV morphine administration (0.04 mg/kg/d vs 0.09 mg/kg/d, p = 0.01). Similar surgical reintervention rate was seen in both cohorts (42% OAPV vs 36% RALAPV, p = 0.77). At the end of follow-up, continence was achieved in 18 (95%) OAPV and 33 (100%) RALAPV patients (p = 0.37) CONCLUSIONS: RALAPV shows comparable success to patients who underwent OPAV procedures. The longer follow-up interval for OPAV highlights minimally invasive surgery as a recent adoption.

导言:机器人辅助腹腔镜阑尾造口术(RALAPV)作为开放式阑尾造口术(OPAV)的微创替代方法,越来越多地应用于临床,但与OPAV相比,RALAPV的疗效仍存在疑问:目的:评估和比较非增强型 RALAPV 与开放手术方法的疗效:回顾性研究了经 IRB 批准的前瞻性登记处,抽取了 2012 年至 2023 年期间接受无增强 APV 的所有患者。对基线人口统计学、术中和术后特征以及长期疗效进行了汇总和比较。P值为双侧,P值结果:共发现 52 名儿童,其中 19 名(36.5%)为 OAPV,33 名(63.5%)为 RALAPV。OAPV手术的中位年龄为8.5岁,RALAPV手术的中位年龄为9.3岁(P = 0.29)。RALAPV 组的手术时间更长(346 分钟 vs 289 分钟,p = 0.04),估计失血量更少(5 毫升 vs 30 毫升,p = 0.003),中位住院时间更短(4 天 vs 5 天,p = 0.07),静脉注射吗啡用量更少(0.04 mg/kg/d vs 0.09 mg/kg/d,p = 0.01)。两组患者的手术再干预率相似(42% OAPV vs 36% RALAPV,p = 0.77)。在随访结束时,18 名(95%)OAPV 患者和 33 名(100%)RALAPV 患者实现了排尿通畅(p = 0.37):RALAPV 的成功率与 OPAV 患者相当。OPAV 的随访间隔时间较长,这说明微创手术是最近才被采用的。
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引用次数: 0
The mediating effects of parental hope and psychological resilience on social support and decision conflict in children with hypospadias. 尿道下裂患儿父母的希望和心理复原力对社会支持和决策冲突的中介效应。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-05 DOI: 10.1016/j.jpurol.2024.09.026
BoXin Liu, SuFang Li, YaNan Xu, Shanshan Ding, Pei Ning

Introduction: The most common congenital genital abnormality of the urinary system in infants is hypospadias; its frequency is rising to 0.33 percent globally. Surgical reconstruction is the most effective treatment for hypospadias.Parents, as surrogate decision-makers for children, frequently experience decision-making conflicts when making decisions. Stress is neither a straightforward stimulus nor a simple response. However, a stressor (stimulus) caused by life events and influenced by numerous intermediate factors including personality, social support, and psychosomatic symptoms or diseases is a multi-factor process of stress response (response). The surgical decision conflict of parents of children with hypospadias is a type of stress response, and social support may have an effect on parents' hope, psychological resilience, and decision conflict, with hope and psychological resilience serving as mediators between social support and decision conflict.

Aim: To investigate the structural relationship between parental social support, hope, psychological resilience, and parents decision conflict in children with hypospadias.

Study design: A cross-sectional design was used to collect the data of 210 parents of children with hypospadias from Department of Urology, Shenzhen Children's Hospital from April 2022 to March 2023.

Results: Social support indirectly affects decision conflict through hope and psychological resilience, with a total indrect effect of -0.511 (P < 0.05).

Discussion: Decision conflicts and regret can be reduced if medical professionals provide adequate and clear information, decision assistance, and meet support requirements during the decision-making process. Improving the level of psychological resilience, hope, and social support among parents of children with hypospadias is a crucial strategy for reducing the level of decision conflict they experience. In the process of making surgical decisions for children with hypospadias, the more sufficient social support from relatives, friends, and medical staff can stimulate their hope, boost their belief in the success of surgery, and the greater their psychological resilience, the more effectively decision conflict can be reduced. Some potential limitations of this study should be considered. Firstly, this study was a cross-sectional survey design. It may have some limitations in providing causal relationships between selected variables. Second, the survey investigated only one hospital, which reduces the generalizability of the findings.

Conclusion: The data support the proposed model and confirm the structural relationship among the four study variables. This study provides new information on the mediating role of hope and psychological resilience between social support and decision-making conflict.

导言:尿道下裂是婴儿泌尿系统最常见的先天性生殖器畸形,全球发病率上升至 0.33%。手术重建是治疗尿道下裂最有效的方法。父母作为孩子的代理决策者,在做决定时经常会遇到决策冲突。压力既不是直接的刺激,也不是简单的反应。然而,由生活事件引起的压力源(刺激),并受到人格、社会支持、心身症状或疾病等众多中间因素的影响,是一个多因素的压力反应(响应)过程。尿道下裂患儿家长的手术决策冲突是应激反应的一种,而社会支持可能会对家长的希望、心理复原力和决策冲突产生影响,其中希望和心理复原力是社会支持和决策冲突之间的中介。研究目的:探讨尿道下裂患儿家长的社会支持、希望、心理复原力和决策冲突之间的结构关系:研究设计:采用横断面设计,收集2022年4月至2023年3月深圳市儿童医院泌尿外科210名尿道下裂患儿家长的数据:社会支持通过希望和心理弹性间接影响决策冲突,总的不正确效应为-0.511(P 讨论):如果医务人员在决策过程中提供充分和明确的信息、决策帮助并满足支持要求,就可以减少决策冲突和遗憾。提高尿道下裂患儿家长的心理承受能力、希望和社会支持水平是减少其决策冲突的重要策略。在尿道下裂患儿的手术决策过程中,来自亲友和医务人员的社会支持越充分,就越能激发他们的希望,增强他们对手术成功的信念,提高他们的心理承受力,就越能有效减少决策冲突。本研究可能存在一些局限性。首先,本研究采用的是横断面调查设计。它在提供所选变量之间的因果关系方面可能存在一些局限性。其次,调查只涉及一家医院,这降低了研究结果的普遍性:数据支持所提出的模型,并证实了四个研究变量之间的结构关系。本研究为希望和心理弹性在社会支持与决策冲突之间的中介作用提供了新的信息。
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引用次数: 0
Parental regret following decision for sons to undergo elective post-neonatal circumcision. 父母在决定让儿子接受选择性新生儿包皮环切术后的遗憾。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-05 DOI: 10.1016/j.jpurol.2024.10.005
Tomer Bashi, Hadas Rorman, Ziv Savin, Noam Bar-Yaakov, Snir Dekalo, Jacob Ben-Chaim, Yuval Bar-Yosef

Introduction: The reasons for performing a circumcision among males after the neonatal period are usually cultural or medical. We aimed to evaluate parental regret for providing consent and to identify factors associated with such regret.

Methods: Included were the parents of males aged 6 months to 18 years who underwent circumcision under general anesthesia at a single center between 2/2017 and 01/2023. Those who underwent additional surgical procedures during the same session were excluded. Parents responded telephonically to the Decision Regret Scale (DRS) questionnaire. Regret was classified as none (0 points), mild (1-25) or moderate-to-strong (26-100). Surgical and demographic data were retrieved for comparison to DRS scores and identification of predictors of parental regret.

Results: In total, 201 of the 265 suitable patients met the inclusion criteria. Parents of 130 patients (65% response rate) whose average age was 5.06 (IQR 1.58,7.53) years completed the DRS questionnaire (study group). The average time since surgery was 41.8 (IQR 25.4,59.3) months. Forty surgeries were undertaken for cultural reasons and 90 for medical considerations. Eighteen parents reported regret (15 mild and 3 moderate-to-strong) for their decision to consent to their son's circumcision. The time from responding since surgery was the only significant variable in the DRS scores, with a 33-month gap predicting no regret (p = 0.02 compared to shorter gaps). The reasons for circumcision did not significantly differ between the "regret" and "no-regret" groups (p = 0.23).

Discussion: Our current investigation revealed a lower incidence of parental regret when compared to previous reports following distal hypospadias repair, likely attributable to the lower complication rate associated with circumcision. Our data reflect the experience of a single center in a country where neonatal male circumcision is routinely performed for cultural and religious reasons, thus precluding the generalization of our findings to places where post-natal circumcision is less commonplace.

Conclusion: Consent to their son's post-neonatal circumcision was regretted by 13.8% of parents. Time since surgery significantly influenced the reduction of their negative attitudes.

导言:对新生儿期后的男性实施包皮环切术的原因通常是文化或医学方面的。我们的目的是评估父母对同意进行包皮环切术的后悔程度,并确定与这种后悔相关的因素:研究对象包括 2017 年 2 月 2 日至 2023 年 1 月 1 日期间在一个中心接受全身麻醉下包皮环切术的 6 个月至 18 岁男性的父母。不包括在同一疗程中接受其他外科手术的患者。家长通过电话回答了决定后悔量表(DRS)问卷。后悔程度分为无(0 分)、轻度(1-25 分)或中度至重度(26-100 分)。我们还检索了手术和人口统计学数据,以便与 DRS 评分进行比较,并确定父母后悔的预测因素:在 265 位合适的患者中,共有 201 位符合纳入标准。平均年龄为 5.06(IQR 1.58,7.53)岁的 130 名患者(回复率为 65%)的父母填写了 DRS 问卷(研究组)。平均手术时间为 41.8 个月 (IQR 25.4,59.3) 。40 例手术是出于文化原因,90 例是出于医疗考虑。有 18 位家长对同意儿子接受包皮环切手术的决定表示后悔(15 位轻度后悔,3 位中度到强烈后悔)。自手术以来作出反应的时间是 DRS 评分中唯一显著的变量,33 个月的间隔预示着不会后悔(与较短的间隔相比,p = 0.02)。包皮环切术的原因在 "后悔 "组和 "不后悔 "组之间没有显著差异(p = 0.23):我们目前的调查显示,与之前的报告相比,远端尿道下裂修复术后家长后悔的发生率较低,这可能是由于包皮环切术的并发症发生率较低。我们的数据反映的是一个国家的一个中心的经验,在这个国家,出于文化和宗教原因,新生儿男性包皮环切术是常规手术,因此我们的研究结果不能推广到那些产后包皮环切术不那么普遍的地方:13.8%的父母对同意其儿子在新生儿期后接受包皮环切手术感到后悔。手术后的时间明显影响了他们消极态度的减少。
{"title":"Parental regret following decision for sons to undergo elective post-neonatal circumcision.","authors":"Tomer Bashi, Hadas Rorman, Ziv Savin, Noam Bar-Yaakov, Snir Dekalo, Jacob Ben-Chaim, Yuval Bar-Yosef","doi":"10.1016/j.jpurol.2024.10.005","DOIUrl":"https://doi.org/10.1016/j.jpurol.2024.10.005","url":null,"abstract":"<p><strong>Introduction: </strong>The reasons for performing a circumcision among males after the neonatal period are usually cultural or medical. We aimed to evaluate parental regret for providing consent and to identify factors associated with such regret.</p><p><strong>Methods: </strong>Included were the parents of males aged 6 months to 18 years who underwent circumcision under general anesthesia at a single center between 2/2017 and 01/2023. Those who underwent additional surgical procedures during the same session were excluded. Parents responded telephonically to the Decision Regret Scale (DRS) questionnaire. Regret was classified as none (0 points), mild (1-25) or moderate-to-strong (26-100). Surgical and demographic data were retrieved for comparison to DRS scores and identification of predictors of parental regret.</p><p><strong>Results: </strong>In total, 201 of the 265 suitable patients met the inclusion criteria. Parents of 130 patients (65% response rate) whose average age was 5.06 (IQR 1.58,7.53) years completed the DRS questionnaire (study group). The average time since surgery was 41.8 (IQR 25.4,59.3) months. Forty surgeries were undertaken for cultural reasons and 90 for medical considerations. Eighteen parents reported regret (15 mild and 3 moderate-to-strong) for their decision to consent to their son's circumcision. The time from responding since surgery was the only significant variable in the DRS scores, with a 33-month gap predicting no regret (p = 0.02 compared to shorter gaps). The reasons for circumcision did not significantly differ between the \"regret\" and \"no-regret\" groups (p = 0.23).</p><p><strong>Discussion: </strong>Our current investigation revealed a lower incidence of parental regret when compared to previous reports following distal hypospadias repair, likely attributable to the lower complication rate associated with circumcision. Our data reflect the experience of a single center in a country where neonatal male circumcision is routinely performed for cultural and religious reasons, thus precluding the generalization of our findings to places where post-natal circumcision is less commonplace.</p><p><strong>Conclusion: </strong>Consent to their son's post-neonatal circumcision was regretted by 13.8% of parents. Time since surgery significantly influenced the reduction of their negative attitudes.</p>","PeriodicalId":16747,"journal":{"name":"Journal of Pediatric Urology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142566535","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}
引用次数: 0
AI-PEDURO - Artificial intelligence in pediatric urology: Protocol for a living scoping review and online repository. AI-PEDURO - 儿科泌尿学中的人工智能:活范围审查协议和在线资料库。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-05 DOI: 10.1016/j.jpurol.2024.10.003
Adree Khondker, Jethro C C Kwong, Mandy Rickard, Lauren Erdman, Andrew T Gabrielson, David-Dan Nguyen, Jin Kyu Kim, Tariq Abbas, Nicolas Fernandez, Katherine Fischer, Lisette A 't Hoen, Daniel T Keefe, Caleb P Nelson, Bernarda Viteri, Hsin-Hsiao Scott Wang, John Weaver, Priyank Yadav, Armando J Lorenzo

Background: Artificial intelligence (AI) and machine learning (ML) methods are increasingly being applied in pediatric urology across a growing number of settings, with more extensive databases and wider interest for use in clinical practice. More than 30 ML models have been published in the pediatric urology literature, but many lack items required by contemporary reporting frameworks to be high quality. For example, most studies lack multi-institution validation, validation over time, and validation within the clinical environment, resulting in a large discrepancy between the number of models developed versus the number of models deployed in a clinical setting, a phenomenon known as the AI chasm. Furthermore, pediatric urology is a unique subspecialty of urology with low frequency conditions and complex phenotypes where clinical management can rely on a lower quality of evidence.

Objective: To establish the AI in PEDiatric UROlogy (AI-PEDURO) collaborative, which will carry out a living scoping review and create an online repository (www.aipeduro.com) for models in the field and facilitate an evidence synthesis of AI models in pediatric urology.

Methods and analysis: The scoping review will follow PRISMA-ScR guidelines. We will include ML models identified through standardized search methods of four databases, hand-search papers, and user-submitted models. Retrieved records will be included if they involve ML algorithms for prediction, classification, or risk stratification for pediatric urology conditions. The results will be tabulated and assessed for trends within the literature. Based on data availability, models will be divided into clinical disease sections (e.g. hydronephrosis, hypospadias, vesicoureteral reflux). A risk assessment will be performed using the APPRAISE-AI tool. The retrieved model cards (brief summary model characteristics in table form) will be uploaded to the online repository for open access to clinicians, patients, and data scientists, and will be linked to the Digital Object Identifier (DOI) for each article.

Discussion and conclusion: We hope this living scoping review and online repository will offer a valuable reference for pediatric urologists to assess disease-specific ML models' scope, validity, and credibility to encourage opportunities for collaboration, external validation, clinical testing, and responsible deployment. In addition, the repository may aid in identifying areas in need of further research.

背景:人工智能(AI)和机器学习(ML)方法正越来越多地应用于小儿泌尿外科领域,数据库越来越广泛,临床实践中的应用兴趣也越来越浓厚。小儿泌尿外科文献中已发表了 30 多个 ML 模型,但其中许多模型缺乏当代报告框架所要求的高质量项目。例如,大多数研究缺乏多机构验证、时间验证和临床环境验证,导致开发的模型数量与临床环境中部署的模型数量之间存在巨大差异,这种现象被称为人工智能鸿沟。此外,小儿泌尿外科是泌尿外科的一个独特亚专科,病情发生频率低,表型复杂,临床管理可依赖的证据质量较低:建立儿科泌尿外科人工智能(AI-PEDURO)合作组织,该组织将开展一项活范围审查,并为该领域的模型创建一个在线存储库(www.aipeduro.com),促进儿科泌尿外科人工智能模型的证据综合。方法与分析:范围界定审查将遵循 PRISMA-ScR 指南。我们将纳入通过四个数据库的标准化检索方法确定的 ML 模型、手工检索论文和用户提交的模型。如果检索到的记录涉及小儿泌尿科疾病的预测、分类或风险分层的 ML 算法,则将纳入检索记录。结果将以表格形式列出,并对文献中的趋势进行评估。根据数据可用性,模型将分为临床疾病部分(如肾积水、尿道下裂、膀胱输尿管反流)。将使用 APPRAISE-AI 工具进行风险评估。检索到的模型卡片(以表格形式简要概括模型特征)将上传到在线资料库,供临床医生、患者和数据科学家开放访问,并将链接到每篇文章的数字对象标识符(DOI):我们希望这份活范围综述和在线资料库能为儿科泌尿科医生评估疾病特异性 ML 模型的范围、有效性和可信度提供有价值的参考,从而鼓励合作、外部验证、临床测试和负责任的部署。此外,该资料库还有助于确定需要进一步研究的领域。
{"title":"AI-PEDURO - Artificial intelligence in pediatric urology: Protocol for a living scoping review and online repository.","authors":"Adree Khondker, Jethro C C Kwong, Mandy Rickard, Lauren Erdman, Andrew T Gabrielson, David-Dan Nguyen, Jin Kyu Kim, Tariq Abbas, Nicolas Fernandez, Katherine Fischer, Lisette A 't Hoen, Daniel T Keefe, Caleb P Nelson, Bernarda Viteri, Hsin-Hsiao Scott Wang, John Weaver, Priyank Yadav, Armando J Lorenzo","doi":"10.1016/j.jpurol.2024.10.003","DOIUrl":"https://doi.org/10.1016/j.jpurol.2024.10.003","url":null,"abstract":"<p><strong>Background: </strong>Artificial intelligence (AI) and machine learning (ML) methods are increasingly being applied in pediatric urology across a growing number of settings, with more extensive databases and wider interest for use in clinical practice. More than 30 ML models have been published in the pediatric urology literature, but many lack items required by contemporary reporting frameworks to be high quality. For example, most studies lack multi-institution validation, validation over time, and validation within the clinical environment, resulting in a large discrepancy between the number of models developed versus the number of models deployed in a clinical setting, a phenomenon known as the AI chasm. Furthermore, pediatric urology is a unique subspecialty of urology with low frequency conditions and complex phenotypes where clinical management can rely on a lower quality of evidence.</p><p><strong>Objective: </strong>To establish the AI in PEDiatric UROlogy (AI-PEDURO) collaborative, which will carry out a living scoping review and create an online repository (www.aipeduro.com) for models in the field and facilitate an evidence synthesis of AI models in pediatric urology.</p><p><strong>Methods and analysis: </strong>The scoping review will follow PRISMA-ScR guidelines. We will include ML models identified through standardized search methods of four databases, hand-search papers, and user-submitted models. Retrieved records will be included if they involve ML algorithms for prediction, classification, or risk stratification for pediatric urology conditions. The results will be tabulated and assessed for trends within the literature. Based on data availability, models will be divided into clinical disease sections (e.g. hydronephrosis, hypospadias, vesicoureteral reflux). A risk assessment will be performed using the APPRAISE-AI tool. The retrieved model cards (brief summary model characteristics in table form) will be uploaded to the online repository for open access to clinicians, patients, and data scientists, and will be linked to the Digital Object Identifier (DOI) for each article.</p><p><strong>Discussion and conclusion: </strong>We hope this living scoping review and online repository will offer a valuable reference for pediatric urologists to assess disease-specific ML models' scope, validity, and credibility to encourage opportunities for collaboration, external validation, clinical testing, and responsible deployment. In addition, the repository may aid in identifying areas in need of further research.</p>","PeriodicalId":16747,"journal":{"name":"Journal of Pediatric Urology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142468300","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}
引用次数: 0
Expression of Wnt signaling proteins in rare congenital bladder disorders. 罕见先天性膀胱疾病中 Wnt 信号蛋白的表达。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-05 DOI: 10.1016/j.jpurol.2024.09.029
Boyu Xie, Michael Millar, Callum Arthurs, Navroop Johal, Christopher Fry, Aamir Ahmed

Introduction and aims: Congenital 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), Connexin 43 (Cx43), FRA1 and TCF7L1 in three rare congenital bladder disorders: bladder exstrophy (BE), neurogenic bladder (NGB) and posterior urethral valves (PUV).

Methods: Bladder 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 discussion: There 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.

Conclusion: Wnt 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.

导言和目的:先天性膀胱异常非常罕见,是导致儿童终末期肾衰竭的主要原因之一。Wnt 信号通路在胚胎发育过程中非常重要,它通过调控基因表达(包括重要转录因子)被认为与这些疾病的发病机制有关。我们研究了四种 Wnt 转录靶标(即 Pygopus 1 (Pygo1)、Connexin 43 (Cx43)、FRA1 和 TCF7L1)在三种罕见先天性膀胱疾病(膀胱外萎症(BE)、神经源性膀胱(NGB)和后尿道瓣膜(PUV))中的表达情况:膀胱组织样本取自英国伦敦大奥蒙德街病童医院(Great Ormond Street Hospital for Sick Children)的对照组(功能正常膀胱,9 例)、BE(15 例)、NGB(6 例)和 PUV(5 例)患者。使用 van Gieson 染色法进行组织学分析,以区分平滑肌 (SM) 和结缔组织 (CT) 区。对这四组组织中的四种 Wnt 相关蛋白的标记强度进行了无偏见、自动化、半定量的免疫荧光分析:结果和讨论:四组组织中的四种 Wnt 相关蛋白的标记强度存在明显差异(P与对照组组织相比,先天性膀胱疾病患者体内的 Wnt 信号相关蛋白出现失调。了解这些机制将有助于对先天性膀胱疾病进行无创早期诊断、发现药物靶点并制定治疗策略。
{"title":"Expression of Wnt signaling proteins in rare congenital bladder disorders.","authors":"Boyu Xie, Michael Millar, Callum Arthurs, Navroop Johal, Christopher Fry, Aamir Ahmed","doi":"10.1016/j.jpurol.2024.09.029","DOIUrl":"https://doi.org/10.1016/j.jpurol.2024.09.029","url":null,"abstract":"<p><strong>Introduction and aims: </strong>Congenital 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), Connexin 43 (Cx43), FRA1 and TCF7L1 in three rare congenital bladder disorders: bladder exstrophy (BE), neurogenic bladder (NGB) and posterior urethral valves (PUV).</p><p><strong>Methods: </strong>Bladder 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.</p><p><strong>Results and discussion: </strong>There 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.</p><p><strong>Conclusion: </strong>Wnt 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.</p>","PeriodicalId":16747,"journal":{"name":"Journal of Pediatric Urology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142581162","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}
引用次数: 0
Mental health and health-related quality of life in adolescents surgically treated for hypospadias in childhood. 接受尿道下裂手术治疗的青少年的心理健康和与健康相关的生活质量。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-05 DOI: 10.1016/j.jpurol.2024.09.031
Eirik Gulseth, Andreas Urdal, Marit Helen Andersen, Ragnhild Sørum Falk, Gunnar Aksnes, Ragnhild Emblem, Trond Diseth, Anne Wæhre
<p><strong>Background: </strong>There is limited research and conflicting results on the mental health and health-related quality of life of individuals surgically treated for hypospadias in childhood. Furthermore, the significance of patient-reported outcomes (PRO) in hypospadias is growing. More research is necessary on these topics, particularly in adolescents.</p><p><strong>Objective: </strong>We aimed to compare mental health, health-related quality of life, body satisfaction, self-esteem, penile appearance, and sexual function outcomes in 16-year-old adolescents who had surgery for hypospadias in childhood with a healthy comparison group.</p><p><strong>Study design: </strong>The study involved 16-year-old adolescents who had surgery for hypospadias in one Norwegian hospital. An age-matched comparison group of healthy adolescents was previously recruited. The study assessed self-reported outcomes on mental health using the Strengths and Difficulties Questionnaire, health-related quality of life using the Pediatric Quality of Life Inventory, body satisfaction and self-esteem using the Self-Perception Profile for Adolescents, and genital perception and sexual function using the Pediatric Penile Perception Score and a non-validated structured interview.</p><p><strong>Results: </strong>In total, 117 adolescents operated for hypospadias and 61 healthy adolescents were included. The outcomes for mental health and health-related quality of life were not statistically different between groups. However, 18 (16%) hypospadias patients were classified as cases/borderline on mental health outcomes, compared to 2 (3%) in the comparison group (p = 0.01). Patients classified as cases/borderline on mental health reported lower health-related quality of life, self-esteem, and more stressful life events. Self-reported penile appearance and sexual function were comparable between groups, however the proximal hypospadias group displayed lower satisfaction.</p><p><strong>Discussion: </strong>Although the present study had generally positive outcomes, a significant finding was that a higher number of adolescents who had previously undergone hypospadias surgery during childhood were classified as cases or borderline cases with respect to mental health issues. Hypospadias surgery in childhood may pose challenges for some patients during adolescence, particularly in the proximal group. Regular monitoring by healthcare professionals is crucial in providing necessary support. A limitation is the small size of the subgroups.</p><p><strong>Conclusion: </strong>Most patients report good mental health, quality of life, and self-esteem even though patients with hypospadias report more mental health issues than the comparison group. Proximal hypospadias were less satisfied with penile appearance and sexual function. However, due to the small sample size, conclusions about this group should be treated cautiously. Assessing self-reported outcomes in follow-ups is important to
背景:关于儿童尿道下裂手术治疗者的心理健康和与健康相关的生活质量的研究有限,结果也相互矛盾。此外,尿道下裂患者报告结果(PRO)的重要性日益凸显。有必要就这些主题开展更多研究,尤其是针对青少年:我们的目的是比较儿童时期接受尿道下裂手术的 16 岁青少年与健康对比组的心理健康、健康相关生活质量、身体满意度、自尊、阴茎外观和性功能结果:研究对象为在挪威一家医院接受尿道下裂手术的 16 岁青少年。此前还招募了一个年龄匹配的健康青少年对比组。研究使用优势与困难问卷评估心理健康方面的自我报告结果,使用儿科生活质量量表评估与健康相关的生活质量,使用青少年自我感知档案评估身体满意度和自尊,使用儿科阴茎感知评分和未经验证的结构化访谈评估生殖器感知和性功能:共纳入了117名接受尿道下裂手术的青少年和61名健康青少年。两组在心理健康和与健康相关的生活质量方面没有统计学差异。然而,有18名尿道下裂患者(16%)在心理健康结果方面被归类为病例/边缘,而对比组只有2人(3%)(P = 0.01)。在心理健康方面被归类为病例/边缘的患者报告的健康相关生活质量、自尊和生活压力事件均较低。两组患者自我报告的阴茎外观和性功能相当,但尿道下裂近端患者的满意度较低:讨论:尽管本研究的结果总体上是积极的,但一个重要发现是,在心理健康问题方面,曾在童年时期接受尿道下裂手术的青少年中,有较多人被归类为病例或边缘病例。儿童时期接受尿道下裂手术的患者在青春期可能会面临一些挑战,尤其是近端组患者。医护人员的定期监测对于提供必要的支持至关重要。不足之处在于分组规模较小:尽管尿道下裂患者比对比组患者有更多的心理健康问题,但大多数患者都表示心理健康、生活质量和自尊心良好。近端尿道下裂患者对阴茎外观和性功能的满意度较低。不过,由于样本量较小,对这一群体的结论应谨慎对待。在随访中评估自我报告的结果对于识别接受尿道下裂手术并面临心理健康下降风险的青少年非常重要。
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引用次数: 0
Response to commentary: "Experimental study of the effectiveness of warm ischemia and cold ischemia during testis-sparing surgery in rats". 对评论的回应:"大鼠保睾手术中温缺血和冷缺血有效性的实验研究"。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-04 DOI: 10.1016/j.jpurol.2024.09.028
Emine Doğan, Ayşe Karaman
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引用次数: 0
The integral co-management role of the medical pediatric urologist: Improving the care of children with urological conditions. 儿科泌尿科医生不可或缺的共同管理角色:改善对泌尿系统疾病患儿的护理。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-02 DOI: 10.1016/j.jpurol.2024.09.024
Joana Dos Santos, Walid A Farhat, Michelle Shouldice, Joao Pippi Salle, Michael Chua, Mandy Rickard, Abby Varghese, Darius Bagli, Armando J Lorenzo, Martin A Koyle
<p><strong>Introduction: </strong>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.</p><p><strong>Objective: </strong>This study aims to delineate the training curriculum, role, clinical outcomes, and educational impact of the MPU over eight years.</p><p><strong>Study design: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Discussion: </strong>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.</p><p><strong>Conclusion: </strong>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
简介:现代医学的复杂性促使人们重新评估传统的病人护理模式,以提高安全性和质量:现代医学的复杂性促使我们重新评估传统的病人护理模式,以提高安全性和质量。我们开展了为期一年的小儿泌尿外科医学奖学金试点项目,该项目由泌尿外科和儿科医院医学部共同开发,一名儿科医生同时接受两个专业的研究生培训。这项创新计划旨在增强儿科泌尿外科非手术方面的知识和专业技能。完成研究金培训后,受训儿科医生将担任儿科泌尿科内科医生(MPU),同时兼任儿科和泌尿科医生。与传统的专家会诊模式不同,MPU 成为了泌尿科不可或缺的成员,在加拿大一家三级儿科医院全职工作,并分担儿科泌尿科住院和门诊护理的责任:本研究旨在阐述 MPU 八年来的培训课程、作用、临床成果和教育影响:我们回顾性地分析了临床结果,包括 2021 年至 2023 年期间每年由 MPU、泌尿科医生和执业护士接诊的患者数量。比较了 MPU 社区儿科泌尿科诊所和三级医院儿科泌尿科诊所的候诊时间和患者满意度。在泌尿科轮转期间,儿科住院医师采用李克特量表对作为临床教师的 MPU 进行评分,以此评估其对教育的影响:结果:整合 MPU 后,建立了神经源性膀胱和后尿道瓣膜等专科门诊。重症监护病房的门诊量约占门诊总量的 34.7%。主观反馈强调,住院环境中的 MPU 共同管理提高了护理质量。此外,在社区医疗泌尿科诊所(CUC)中,由 MPU 和儿科泌尿科护士共同管理,患者的等待时间更短,满意度更高(53 ± 36 天和 194 ± 108 天)(P 讨论):我们在小儿泌尿科采用的新型共同管理方法整合了内科和外科的专业知识。然而,该方法存在回顾性设计和单中心设置等局限性。不过,这一框架为其他外科专科提供了一个潜在的模式,为对外科疾病的内科管理感兴趣的学员提供了一个独特的实践机会:内科小儿泌尿科医生作为教育者、住院医生和门诊主任发挥着关键作用,有效地整合了内科和外科的专业知识。MPU 模式有望优化小儿泌尿外科的医疗安全和质量。
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引用次数: 0
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Journal of Pediatric Urology
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