To analyze the clinical characteristics, complications and patients satisfaction of MIP hypospadias variant. A retrospective analysis was performed for 31 patients with MIP admitted to our hospital from January 2008 to February 2023. All enrolled patients underwent telephone follow-up and a survey was conducted on the satisfaction of patients and their families. Outcome analysis was focused on clinical data includes age, position of the meatus, penile curvature, type of repair, complications and satisfaction survey results. Statistical analyses were performed using R software. The average age of 31 MIP patients was 92.1 ± 40.7 months, and the median follow-up time was 49.0 [21.0–82.2] months. Twenty one patients (67.7

This study aims to evaluate the feasibility, safety and efficacy of robotic assisted laparoscopic pyeloplasty (RALP) with the novel SHURUI single-port (SR-SP) robotic surgical platform in children. Between November 2023 and April 2024, 20 pediatric patients diagnosed with ureteropelvic junction obstruction (UPJO) underwent transperitoneal RALP with the SR-SP surgical platform. Baseline characteristics, perioperative parameters and follow-up data were collected and analyzed. All surgical procedures were successfully performed without conversion to open or laparoscopic surgery. The median docking time, console time, and operative time were 3.5 min (range 3.5–6.0), 144.5 min (range 88.0–290.0), and 221.5 min (range 136.0–450.0), respectively. The median estimated blood loss was 5 ml (range 2–20) and the median length of hospital stays was 6 days (range 4–24). Through trans-umbilical incision, RALP was feasible in patients with a height of no less than 114 cm. The minimum incision length was 2 cm. No intraoperative complication was identified. Two cases were identified with the Clavien–Dindo Grade II postoperative complications and one with the Clavien–Dindo Grade III complications. The surgical success rate was 95

BACKGROUND:Varicocele (VC) is a leading cause of male infertility. Insufficient growth and development of the cremaster muscle may contribute to VC, but the underlying mechanism remains unclear. Cremaster muscle dysfunction may impair venous valve support, contributing to VC. The cremaster relies on satellite cells (SCs) for postnatal growth and damage repair. This study aimed to explore the mechanism of the cremaster muscle in the process of VC. METHODS:Ten male Sprague-Dawley (SD) rats were divided into two groups: the VC model group (5 rats) and the sham-control group (5 rats). After four weeks of observation, the cremaster muscles were collected. The diameters of the left and right spermatic veins were measured, and the left testis was isolated for morphological examination via H&E staining. SCs isolated from the left cremaster muscle were analyzed using multiple methods, including qPCR and Western blot. Data were analyzed using SPSS v.22.0. RESULTS:Compared to the control group, the model group showed decreased TFRC mRNA stability, decreased mitochondrial membrane potential, and decreased GSH and GSSG contents, as well as increased m6A modification levels and increased ROS, MDA, and Fe2+ contents. In addition, the model group also showed downregulation of transferrin receptor (TFRC, a key iron uptake protein involved in ferroptosis) expression and upregulated m6A methyltransferase and recognition proteins. Multiple biochemical test results indicated increased ferroptosis, characterized by changes such as decreased mitochondrial membrane potential and GSH and increased ROS, MDA, and Fe2+. CONCLUSION:This study suggests that SCs in the cremaster muscle is associated with impaired cremaster muscle repair and VC pathogenesis through m6A modification of TFRC mRNA. Our findings offer fresh insights into the role of cremaster SCs in VC and provide a foundation for future research on the potential therapeutic target of VC. STRENGTHS AND LIMITATIONS OF THIS STUDY:This study is the first to investigate the pathogenesis of varicocele from the perspective of the cremaster muscle, and some clues have been discovered from it. The causal relationship between m6A-TFRC axis and ferroptosis requires further validation using functional rescue experiments (e.g., METTL3 knockdown or ferroptosis inhibitors). The small sample size may limit statistical power; future studies with larger cohorts are warranted.

To investigate the long-term natural history of fetal hydronephrosis (FH) in a Chinese population, identify key prognostic factors, and optimize evidence-based postnatal management strategies using the Urinary Tract Dilation (UTD) classification system. A hybrid retrospective-prospective cohort study was conducted among 49,097 pregnant women who received prenatal screening at Beijing Children’s Hospital Shunyi Women’s and Children’s Hospital between January 2010 and March 2020. A total of 2,263 fetuses were diagnosed with hydronephrosis and followed for 5 years. Prenatal ultrasound parameters (anteroposterior renal pelvic diameter [APD], renal parenchymal thickness, and A/R ratio) and postnatal outcomes (surgery, urinary tract infections, hypertension) were analyzed. Cases were stratified into UTD A1 (low-risk) and A2 3 (high-risk) groups. Statistical analyses included generalized linear mixed-effects models (GLMM), cumulative link mixed models (CLMM), and generalized linear models (GLM). The incidence of fetal hydronephrosis was 4.61

To summarize our treatment experience of acute urethral trauma in pre-pubertal girls. We retrospectively analyzed six pre-pubertal girls with acute severe urethral trauma who were admitted to our hospital from April 2003 to April 2023 and followed up. All six patients had pelvic fractures and vaginal injuries, and one had a rectal injury. Five showed a large amount of fresh blood flowing from the perineum and an inability to urinate. However, the remaining patient had a more insidious onset, causing the emergency doctors to miss the diagnosis. Four had severe perineal tearing and had lost their normal urethral and vaginal openings, making urinary catheter insertion impossible. The diagnosis was very clear. The other two were diagnosed with urethrovaginal injury during cystourethroscopy. Five underwent urethral and vaginal repair surgery within 7 days after the injury, and two of them developed complications requiring endoscopy or reoperation. Postoperative questionnaire scoring showed that four patients had normal urinary function and two had mild dysfunction. The diagnosis of acute urethral injury in girls needs doctors’ attention. If the patient’s vital signs are stable, urgency urethrovaginal repair surgery can be performed. Although this is difficult and requires experienced pediatric urologists, it facilitates discovery of concurrent injuries.

Introduction:The incidence of pediatric nephrolithiasis has been increasing, and the role of genetic factors has garnered attention in recent years. This study aimed to explore the genetic basis underlying pediatric nephrolithiasis in Chinese population. Methods:Whole exome sequencing (WES) was conducted in a consecutive cohort of 456 children over a 11-year period. Clinical and genetic data were systematically collected, analyzed, and comprehensively compared. Results:Average age was 4.2 years with a male-to-female ratio of 2.2. A total of 260 causative variants in 16 genes were identified in 141 children, resulting in a positive molecular diagnosis rate of 31%. Of the causative variants, 43% were novel. The most prevalent diagnoses were primary hyperoxaluria (PH) (AGXT: 20%, GRHPR: 11%, and HOGA1: 24%) and cystinuria (SLC3A1: 18% and SLC7A9: 14%). Children with positive molecular diagnoses were more likely to have stone episodes, bilateral stones, multiple stones, or nephrocalcinosis (all P < 0.05). Children with AGXT defects were more prone to have severe clinical manifestations, and those with HOGA1 defects and males with SLC3A1 and SLC7A9 defects tended to be diagnosed at a younger age. The concordance rate between suspected clinical diagnoses and molecular diagnoses was 81%. At least 29% of children could benefit from additional clinical advice based on a molecular diagnosis. Conclusion:A genetic etiology was identified in 141 of 456 of pediatric patients (31%) with nephrolithiasis in a Chinese cohort. A positive molecular diagnosis is a risk factor for severe clinical presentation of pediatric nephrolithiasis. WES has the potential to be used to confirm or even modify clinical diagnoses, thereby facilitating individualized therapeutic and preventive interventions.

Purpose To summarize our treatment experience of acute urethral trauma in girls. Methods We retrospectively analyzed six girls with acute urethral trauma who were admitted to our hospital from April 2003 to April 2023 and followed up. Results All six patients had pelvic fractures and vaginal injuries, and one had a rectal injury. Five showed a large amount of fresh blood flowing from the perineum and an inability to urinate. However, the remaining patient had a more insidious onset, causing the emergency doctors to miss the diagnosis. Four had severe perineal tearing and had lost their normal urethral and vaginal openings, making urinary catheter insertion impossible. The diagnosis was very clear. The other two were diagnosed with urethrovaginal injury during cystourethroscopy. Five underwent urethral and vaginal repair surgery within 7 days after the injury, and two of them developed complications requiring endoscopy or reoperation. Postoperative questionnaire scoring showed that four patients had normal urinary function and two had mild dysfunction. Conclusion The diagnosis of acute urethral trauma in girls requires vigilance. If the patient’s vital signs are stable, emergency urethrovaginal repair surgery can be performed. Although this is difficult and requires experienced pediatric urologists, it facilitates discovery of concurrent injuries.

目的 分析选择男性性别、行尿道成形手术的性别发育异常(differences of sex development,DSD)患者临床特点.方法 回顾性分析首都医科大学附属北京儿童医院2016年1月至2019年12月收治的行尿道成形手术治疗的选择男性抚养的性别发育异常110例患儿病历资料,分析初次手术年龄、尿道缺损长度、尿道下裂分度、是否合并隐睾及阴茎阴囊转位、是否合并微小阴茎、性腺类型、染色体分型的分布特点.结果 初次手术年龄中位数为28个月(13～184个月),尿道缺损长度中位数为5cm(0.5～9cm).其中合并隐睾的患者70例(63.6％),合并阴茎阴囊转位患者37例(33.6％),其中既存在隐睾又合并阴茎阴囊转位患者9例(8.2％),性腺表现双侧睾丸的共55例(50.0％),其次为性腺发育不良的患儿32例(29.1％),卵睾的患儿22例(20.0％).在性腺发育不良病例中,性染色体型DSD病例占主导(65.6％),卵睾病例中,46XX DSD占主导(81.8％).染色体分型以46XY,DSD为主,其次为性染色体DSD,最后为46XX,DSD.结论 本研究中行尿道成形手术的男性抚养的DSD患者初次手术年龄要晚于尿道下裂推荐年龄,且患者多合并隐睾、阴茎阴囊转位及微小阴茎,阴茎阴囊转位以及小阴茎在性染色体型DSD中更为多见,其次为46XX DSD、46XY DSD.性腺表现各异,以睾丸为主,但性腺畸形病例中发育不良性腺占主导,而非卵睾.染色体分型以46XY DSD为主,其次为性染色体DSD,这与染色体分型作为性别决定的参考指标有关,且与染色体分型在性别选择的导向性一致.

Objective:To explore the clinical characteristics, therapeutic outcomes and prognoses of bilateral Wilms tumor (BWT) in children and to enhance its clinical awareness.Methods:From January 2008 to December 2019, clinical data were retrospectively reviewed for 43 hospitalized BWT children, including age, clinical manifestations, imaging findings, preoperative chemotherapy, surgical approaches, pathological diagnoses and prognoses. With a total of 86 kidneys, there were 24 boys and 19 girls with an onset age of 17 (3-69) months. Thirty children (60 sides) on preoperative chemotherapy were selected as preoperative chemotherapy group, 9 cases (18 sides) not on chemotherapy as non-chemotherapy group and the remaining 4 (8 sides) with unknown data not grouped. Six children underwent needle biopsy before chemotherapy. Among 30 children (60 sides) with preoperative chemotherapy, 26 (52 sides) had complete assessment data of preoperative chemotherapy response and 4 (8 sides) were missing. The duration of preoperative chemotherapy was 4 to 12 weeks. The major preoperative chemotherapy regimens were VA (vincristine+dactinomycin) (n=19, 38 sides) ; VAD (vincristine+ dactinomycin + adriamycin) (n=9, 18 sides) and vincristine (n=2, 4 sides). Survival curves of follow-up data were plotted with the Kaplan-Meier method and 4-year event-free and overall survival rates calculated by SPSS 24.0 software.Results:Nephron sparing surgery (NSS, n=30) and unilateral tumor nephrectomy plus NSS (n=13) were performed. Preoperative chemotherapy accounted for 80.0% (24/30) of NSS and there were 6 cases of NSS without chemotherapy. Cumulative residual renal parenchyma of kidney was greater than that of unilateral kidney [53.5% (23/43) ]while cumulative residual renal parenchyma of kidney was less than that of unilateral kidney [46.5% (20/43) ]. The follow-up period was 48 (8-158) months. And the follow-up age was 71(39-180) months. Except for 4 deaths, the remainders survived. Among survivors, there were renal failure (n=1) and surviving with tumor (n=4) . Among 9 cases of postoperative recurrence, there were death (n=3) , surviving with tumor (n=2) and surviving without tumor (n=4) . Based upon the Kaplan-Meier method, 4-year event-free survival rate was 85.6% and overall survival rate 90.4%. The highest unilateral stage was Ⅲ (n=4) and Ⅳ (n=5) ; recurrence sites were in situ (n=7) , lumbar muscle (n=1) and multiple retroperitoneal (n=1) . The median recurrence time was 11 (1-38) months. Seven cases underwent surgical resection of recurrent lesion and received postoperative intensive chemotherapy with etoposide, carboplatin and cyclophosphamide. Conclusions:WT has a decent prognosis after combining preoperative chemotherapy, NSS and postoperative stage-based interventions according to the specific pathological type.

Objective:To discuss the diagnosis and treatment 0f WAGR syndrome.Methods:The clinical data of 10 cases of WAGR syndrome children admitted to our hospital from January 2008 to November 2019 were respectively analyzed including the clinical features, diagnosis, and surgical treatments. There were 6 males and 4 females, aged from 13 to 36 months, with an average of 23.6 months. 9 cases were diagnosed as iris absence due to ocular abnormalities in infancy, and 1 case was diagnosed as iris absence due to ocular abnormalities by physical examination because of renal mass. There were 2 boys with cryptorchidism, and 2 boys with hypospadias, 1 of which did not received operation because of mild hypospadias, and another undergoing surgery. There were no abnormality of genitourinary system in the remaining 5 cases. There were 7 cases of unilateral nephroblastoma, with 1 case at the left and 6 cases at the right, and there were 3 cases of bilateral nephroblastoma. Abdominal doppler ultrasound and enhanced abdominal CT were performed for all patients. Abdominal doppler ultrasound indicated solid mass in renal parenchyma or non-uniform echo zone. Abdominal enhanced CT indicated renal tumor with diameter of 1.8 cm-12.7 cm and locally non-uniform enhanced echo. Among the 7 cases of unilateral nephroblastoma, 4 underwent nephrectomy, 1 underwent tumor enucleation, and 2 underwent tumor enucleation for unilateral tumor complicated with nephrogenic rests. There were 3 cases of bilateral nephroblastoma, 2 cases undergoing unilateral tumor enucleation firstly and contralateral tumor enucleation following chemotherapy. One case underwent unilateral tumor nephrectomy followed by contralateral tumor enucleation. One case of unilateral nephrogenic rests did not undergo renal tumor surgery. Preoperative chemotherapy was performed in 7 patients, including 3 bilateral nephroblastoma, 1 unilateral nephroblastoma combined with contralateral nephroblastoma, and 3 unilateral tumors larger enough to pass the midline. The chemotherapy regimen was VCR+ ACTD in 5 cases, VCR+ ACTD+ CTX+ DOX/CDDP+ VP16 and VCR+ CTX+ DOX in another 2 cases respectively.Results:All 10 cases were diagnosed as nephroblastoma. There were 3 patients without preoperative chemotherapy which belongs to COG stageⅠ(1 case) and STAGEⅢ(2 cases); Preoperative chemotherapy was performed in 2 patients with SIOP stage Ⅱ, 2 patients with SIOP stage Ⅲ, and 3 patients with SIOP stageⅤ. Nine children received regular chemotherapy after surgery, among which 1 child in stage Ⅰ received DD4A chemotherapy regimens, 2 children in stage Ⅱ received DD4A and EE4A regimen respectively, and 3 of the 4 children in stage Ⅲ received regular chemotherapy after surgery, including EE4A(1 case)and DD4A(2 cases). EE4A(1 case)and DD4A(2 cases) chemotherapy were performed in 3 patients with stage Ⅴ according to their unilateral tumor stage. Ten cases were followed up, with 9 of the 10 cases having no tumor recurrence or metastasis, and death in 1 case. At present, abdominal doppler ultrasound of 1 child with nephrogenic rests showed no obvious progress. The renal function of 9 children was not significantly abnormal during the regular follow-up. The results of intelligence screening showed that 6 of the 10 patients were significantly behind their peers, and 4 had no obvious abnormality compared with their peers. Gene tests were performed 3 times after surgery, and the results showed the deletion of 11p13 and adjacent distal genes.Conclusions:WAGR syndrome is rare in clinical practice, and renal ultrasound should be monitored after diagnosis to detect renal tumors in early stage. For bilateral cases, renal function should be preserved as long as possible in order to reduce the probability of renal failure. Long-term follow-up of nephroblastoma with this syndrome is particularly important.

Objective:To explore the diagnosis and management of ureteral obstruction at the level of iliac vessel.Methods:From January 2009 to December 2020, retrospective review was performed for clinical features, diagnoses and surgical managements of 29 surgical children of ureteral obstruction at the level of iliac vessel. There were 19 boys and 10 girls with an average age of 5.4 (0.5-14) years. IVP was performed. The relevant examinations included voiding cystourethrography (VCU) (n=6) , renal scan (n=6) and magnetic resonance urography (MRU, n=3). And the procedures were laparoscopic ureteroureterostomy (n=6) , open surgery (n=23) , pyeloplasty plus ureteroureterostomy for mid-ureteral stricture and ureteropelvic junction obstruction (UPJO, n=3).Results:During a median follow-up period of 12 (6-96) months, 3 children became lost and the remainders were tracked. Except for one case of nephrostomy, there were a marked relief of hydronephrosis and an improvement of split-renal function. No urinary tract infection occurred after operations.Conclusion:Ureteral obstruction at the level of iliac vessel is frequently accompanied by other urological abnormalities. Utrasound and MRU may be employed for determining the anatomy of urinary system and pinpointing the location of ureteral obstruction. And IVP and renal scan hint at renal function. After surgical intervention, clinical prognosis is generally decent.

目的 了解单侧肾盂输尿管连接部梗阻(ureteropelvic junction obstruction,UPJO)手术后短期患侧分肾功能(differential renal function,DRF)缓解效果. 方法 选择2019年3月至2019年10月首都医科大学附属北京儿童医院收治的单侧UPJO行肾盂成形术患者50例为研究对象,其中男37例,女13例,手术前后均行利尿性肾动态显像检查.根据术前DRF情况分为术前DRF降低组(DRF＜40％)、术前DRF正常组(40％ ＜DRF＜55％)和术前DRF超常组(DRF＞ 55％).分别对比各组手术前后患侧DRF及肾实质厚度(renal parenchymal thickness,PT)变化情况,进一步对比组间DRF缓解情况及术后DRF水平. 结果 三组患者手术年龄、方式及术后DRF差异具有统计学意义(P＜0.05);在术前DRF降低组及超常组患者中,手术前后DRF差异存在统计学意义(P＜0.05),术前DRF正常组患者手术前后DRF无明显变化,但肾实质厚度存在明显差异(P＜0.05).以术后DRF增加≥5％作为术后肾功能缓解标准(超常肾功能患者以术后DRF减少≥5％作为术后肾功能缓解标准),术前DRF不同组缓解率差异存在统计学意义(P＜0.05),术前DRF降低组患者术后肾功能水平仍低于术前DRF正常组及术前DRF超常组患者(P＜0.05). 结论 术前DRF＜ 40％的单侧UPJO患者术后短期DRF缓解显著,但其术后DRF低于术前DRF正常及超常患者.

目的:针对儿童肾积水诊断过程复杂,不同医疗机构的设备和医生技术经验不尽相同的问题,研发儿童肾积水诊断临床思维导向方案,实现智能化与高效化诊断.方法:纳入多类先天性肾积水诊断知识,并基于工作流和知识图谱关键节点推理技术给出将这些知识计算机临床思维化的方法,建立辅助导向系统,实现智能导诊.结果 与结论:形成儿童肾积水临床思维诊断辅助导向系统,对儿童肾积水精准临床思维诊断推广至基层具有重要意义.

Objective:To investigate the effect of timing of surgical treatment on renal function of children with solitary kidney and ureteropelvic junction obstruction (UPJO).Methods:The clinical data of patients with solitary kidney and UPJO admitted to Beijing Children′s Hospital, Capital Medical University and Shunyi Women′s and Children′s Hospital of Beijing Children′s Hospital from January 2006 to January 2018 were retrospectively analyzed.A total of 23 cases were enrolled, including 16 males and 7 females.The age of first visit ranged from 1 day to 15 years and 8 months (average: 2 years and 2 months). All the patients were conservatively treated for an average of 2 years and 9 months.SPSS 20.0 software was used for data analysis.Results:All patients received Anderson-Hynes pyeloplasty(A-H operation), and the age at operation ranged from 10 months to 16 years and 4 months, with an average of 4 years and 11 months.There were only 2 cases under 1 year old (10 months old and 11 months old, respectively). The double J stent was retained for 2 months after operation, and intravenous pyelography(IVP) was reexamined at 3 months after operation.The results showed that hydronephrosis either had no obvious change or was alleviated in different degrees.In 3 cases, IVP remained undetected for 40 minutes before operation.After operation, IVP was detected at 10-20 minutes.The follow-up period ranged from 1 year and 1 month to 10 years, with an average of 3 years and 9 months.Urinary ultrasound showed that the degree of pyeloplasty was less severe than that before operation.Conclusions:Close follow-up visits and conservative treatment of solitary kidney with UPJO are safe after 6 months.The first choice of operation is pyeloplasty.

The technological innovations of catheterization and catheterization materials have gone through a long history until the emergence of clean intermittent catheterization (CIC) technology.Neurogenic bladder (NB) is a major cause of lower urinary tract dysfunction in children.Children often have recurrent urinary tract infections, dysuria or urinary incontinence, resulting in hydroneureter and vesicoureteral reflux, renal impairment and death due to renal failure.With the global implementation of CIC, the mortality rate of NB children declined markedly and the quality-of-life improved.CIC has been recognized by International Continence Society as a first choice for bladder emptying and an important treatment for NB.Physicians, children and parents should cooperate to develop a personalized CIC program for NB children to maximize the value of CIC.

Objective:To explore the characteristics of urodynamic parameters of children with different lower urinary tract symptoms (LUTS) but without neurogenic or organic diseases, thus providing references for the clinical diagnosis and treatment.Method:Clinical data of LUTS children without neurogenic or organic diseases who underwent urodynamic tests in the Department of Urology, Beijing Children′s Hospital of Capital Medical University from January 2017 to December 2019 were retrospectively analyzed.A total of 70 LUTS children aged 5-12 years were recruited, involving 46 males and 24 females with the median age of 9.0 (7.0, 10.0) years.According to the main symptoms of LUTS, patients were divided into 4 groups: urinary frequency and urgency group, urinary incontinence group, enuresis group and others.All children underwent urodynamic tests, including noninvasive uroflow rate/pelvic floor electromyography and urethral catheterization cystometry.Urodynamic parameters were recorded for analysis.The results of urodynamic study were compared among urinary frequency and urgency group, urinary incontinence group and enuresis group.Results:There were no significant differences in the noninvasive uroflow rate and post-voiding resi-dual volume among the three groups.Contractions of pelvic floor muscles during urination in children with abnormal urine flow curve were more often observed than those with normal urine flow curve ( 17/38 cases vs.2/32 cases, χ2=13.012, P<0.05). The contractions in children with staccato-shaped curve were more often observed than those with plateau-shaped curve (13/22 cases vs.3/14 cases, χ2=36.000, P<0.05). There were no significant differences in the main invasive urodynamic parameters of storage and emptying phase among the three groups.The bladder compliance of children with detrusor overactivity (DO) was lower than those without DO [12.64(9.00, 21.11) mL/cmH 2O vs.32.22(21.81, 97.75) mL/cmH 2O, 1 cmH 2O=0.098 kPa, Z=-26.333, P<0.001]. The maximum urethral static pressure of children with enuresis was higher than those with urinary frequency and urgency, and urinary incontinence [(120.00±20.69) cmH 2O vs.(81.17±28.09) cmH 2O vs.(69.59±22.19) cmH 2O, F=12.170, P<0.05]. The maximum urethral closed pressure of children with enuresis was higher than those with urinary frequency and urgency, and urinary incontinence [(109.86±41.94) cmH 2O vs.(62.41±26.71) cmH 2O vs.(58.09±20.49) cmH 2O, F=11.804, P<0.05]. Conclusions:Urodynamic abnormalities are common in LUTS children without neurogenic or organic diseases.Urodynamic tests can be applied to identify the types and distributions of potential urodynamic abnormalities, and to guide the treatment and reflect curative effect.DO and decreased bladder compliance are the most common urodynamic abnormalities, which should be monitored and treated in time.

目的 明确无创尿流率同步体表盆底肌电图测定中的盆底肌电图滞后时间对于儿童特发性逼尿肌过度活跃的诊断价值. 方法 回顾性分析首都医科大学附属北京儿童医院69例因下尿路症状就诊但无神经、解剖异常的病例,由一名对无创盆底肌电图滞后时间结果未知的泌尿外科医师根据患儿经尿道置管膀胱测压检查结果作出逼尿肌过度活跃(detrusor overactivity,DO)的诊断,由另一名对经尿道置管膀胱测压结果未知的泌尿外科医师根据无创盆底肌电图滞后时间(＜2s判定为阳性)作出DO诊断.比较两者诊断儿童特发性DO的结果,检验两种诊断方法的一致性,计算盆底肌电图滞后时间诊断DO的敏感性和特异性. 结果 无创盆底肌电图滞后时间与经尿道置管膀胱测压诊断儿童特发性DO具有中等一致性(Kappa=0.548,P＜0.001),最佳诊断标准为≤1 s,敏感性为93.5％,特异性为63.2％,AUC=0.777,具有中等诊断价值. 结论 无创尿流率结合同步体表盆底肌电图测定中缩短的盆底肌电图滞后时间可初步诊断儿童特发性DO,有可能替代有创尿动力学检查识别特发性DO患儿.

目的 了解手术改善后尿道瓣膜症患儿膀胱功能的效果和手术前后膀胱功能的变化.方法 回顾性分析2014年1月 ～2016年12月在北京儿童医院及北京儿童医院顺义妇儿医院住院治疗并完成了手术前、后尿流动力学检查后尿道瓣膜症患者的临床资料.共7例患者,首诊年龄5个月 ～9岁2个月,平均年龄为4岁7个月,手术年龄5个月 ～9岁2个月,平均手术年龄4岁7个月.结果 7例患儿诊断为后尿道瓣膜症,随访时间3～96个月,平均随访时间32.7个月.术后复查尿流动力学时间为距术后3～21个月,平均6.9个月.手术前、后尿流动力学检查结果显示,最大膀胱测量容积138.86±116.13ml vs 174.71±100.20ml;残余尿量82.29±90.22ml vs 57.71±75.03ml;膀胱顺应性9.54±14.05ml/cmH2O vs 16.23±12.62ml/cmH2O;最大尿流率5.57±3.84ml/s vs 13.27±10.22ml/s.本组患儿最大膀胱容积、残余尿量、膀胱顺应性、最大尿流率在手术前、后两组数值变化比较,差异无统计学意义(P>0.05).结论 尿流动力学检查是监测后尿道瓣膜症病情变化的有效手段,瓣膜切开对改善最大膀胱容积、膀胱顺应性、残余尿量、最大尿流率并不理想.

目的 回顾性分析行尿道板纵切卷管尿道成形术(tubularized incised urethral plate ure-throplasty,TIP)治疗的尿道下裂患者阴茎的特点并对其进行远期随访,总结TIP手术的适应证,了解其远期排尿、性功能及社会心理状况,以提高对TIP手术的认识.方法 回顾性分析2004年1月至2011年12月初次行尿道下裂手术或尿道下裂术后再手术采用TIP手术治疗患者的临床资料,利用预先设计好的TIP手术后基本情况调查表通过电话随访及门诊复查,了解TIP手术远期疗效.结果 共266例尿道下裂患者纳入本研究(占同期行尿道成形术2993例的8.89％),行TIP手术时年龄1岁至16岁9个月,平均5岁7个月.阴茎条件:术前无明显阴茎下弯74例,轻度阴茎下弯仅需包皮脱套及松解腹侧纤维组织矫正阴茎下弯106例,中度阴茎下弯需加做背侧白膜紧缩矫正阴茎下弯86例,无重度阴茎下弯;尿道板宽0.6～1.0 cm,均血供丰富、弹性好,纵切后宽1.0～1.4 cm.随访113例,随访率42.5％,失访153例.平均随访时间12.5年,末次随访时患者平均年龄为17岁10个月.83.2％ 的患者对阴茎外观满意,20.3％ 的患者存在排尿方面的问题,6.2％ 的患者存在阴茎下弯,所有患者均不存在阴茎勃起功能障碍、勃起疼痛等情况,98.2％ 的患者社会心理适应良好.结论 TIP手术的应用需严格把握其适应证,阴茎下弯不严重的远端型和中间型尿道下裂,且尿道板发育良好为TIP手术的严格适应证.在正确把握其适应证的基础上,患者远期可获得较好的阴茎外观、排尿及性功能.

Objective:To explore the optimal treatment of children with ureterocele of upper pole moiety of duplex system with concurrent ipsilateral lower renal pole moiety or contralateral renal vesicoureteral reflux.Methods:Retrospective analysis was conducted for clinical data of 24 children with ureterocele of upper pole moiety of duplex system with concurrent ipsilateral lower renal pole moiety or contralateral renal vesicoureteral reflux from October 2007 to October 2019. There were 5 boys and 19 girls with an average age of 2 years. The involved sideness of duplication was left (n=13), right (n=6) and bilateral (n=5). Ureterocele of upper pole moiety of duplex system was left (n=15), right (n=9) and bilateral (n=0). Ipsilateral lower pole moiety of renal vesicoureteral reflux was left (n=13) and right (n=9); ipsilateral lower pole moiety and contralateral renal vesicoureteral reflux (n=2). Six children were diagnosed based upon abnormal ultrasound, 17 cases presented with urinary tract infection (UTI) and 1 case had intermittent abdominal pain. Twenty-two children with non-functional upper renal pole moiety underwent heminephrectomy. For two cases with functional upper kidneys, upper and lower ureteral anastomosis (n=1) and excision of ureterocele with reimplantation (n=1) were performed.Results:Among 22 patients with heminephrectomy, 19 were followed up and 3 were lost to follow-ups. Reflux disappeared (n=11), relieved (n=3) and stayed symptom-free (n=2). Three cases were re-operated for urinary tract infection because of non-relieving reflux. One case of upper/lower ureteral anastomosis had no clinical symptoms at 4 months. Reflux disappeared after an excision of ureterocele with reimplantation.Conclusions:For non-functional upper renal pole moiety with dysplasia after heminephrectomy, reflux disappeared in most cases and few required reoperation due to urinary tract infection of recurrent reflux. Functional upper kidney might be preserved.