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

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 Mixed gonadal dysgenesis (MGD) is a rare disorder of sexual development. The management of MGD is challenging since the disease significantly impacts a patient's growth, hormone balance, and gonadal development. This article used a large population and a long follow-up period for its analysis. Objectives This study aims to summarize the gender determination basis and analyze the long-term follow-up of mixed gonadal dysgenesis. Methods A total of 45 patients' clinical data were summarized and analyzed. Patients were divided by gender. Next, we followed up regarding the occurrence of complications after surgery, the patients' satisfaction with external genitalia appearance, the growth of the patients, counting the surgical pattern the incidence of surgical complications and the development of the patients' growth. All patients included in this study underwent chromosomal karyotype analysis, abdomen exploration, and pathological biopsy. After sex determination, 7 patients who were raised as female underwent clitoroplasty, and bilateral gonadectomy. 38 male patients underwent urethroplasty & thorn; one-sided gonadectomy. Results Patient follow-up started in the third month after surgery. Female patients reported no surgeryrelated complications, while 14 male patients showed surgery -related complications. Additionally, 20 male patients (60.6 %) had a lower height compared to normal peers, 12 of which (36.4 %) were lower than the second standard deviation of the height of normal peers. Conclusion The clinical manifestations of mixed gonadal dysgenesis are variable, and the management is complicated. Children's gonadal function, external genital conditions, psychological evaluation, and parents' wishes should be considered before sex determination. In China, most patients are raised as males with a high incidence of postoperative complications. We found that short stature is a common feature in male patients, thus their height and growth should be carefully supervised. Patients should pay attention to their sexual function and sexual potential during adulthood.

Background: Concomitant anterior urethral valves (AUVs) and posterior urethral valves (PUVs) is an extremely rare congenital urologic anomaly, which may be easily overlooked in the clinic. Objective: This study assessed the prognosis of children with concomitant PUVs and AUVs. Methods: The clinical data of inpatients with concomitant AUVs and PUVs in our hospital were collected from January 1983 to June 2022. The clinical manifestations, auxiliary inspection, and treatment were described in detail. Results: In total, 6 cases of concomitant AUVs and PUVs in boys were found in our hospital, with ages ranging from 3 months to 9 years; the main clinical manifestation was abnormal urination. Four patients exhibited concomitant AUVs and PUVs preoperatively and underwent simultaneous anterior and posterior urethral valvotomy. Follow-up studies showed that 3 patients' clinical symptoms substantially improved with well-maintained renal function. One patient died of renal failure. In the other 2 patients, PUVs were initially identified and excised, but their clinical symptoms did not show substantial improvement. Following voiding cystourethrography (VCUG), the AUVs were found and obstructions were then completely relieved. However, 2 patients died of renal failure. Conclusions: If urinary symptoms cannot be substantially relieved after posterior urethral valvotomy, VCUG and cystoscopy should be repeated to shorten the interval between anterior and posterior urethral valvotomies to improve patient prognosis.

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 探讨女童陈旧骨盆骨折尿道外伤的治疗方法.方法 回顾性分析2015年4月至2023年4月北京儿童医院收治的10例陈旧骨盆骨折尿道外伤女童的临床资料.年龄(8.5±4.3)岁.7例合并尿道阴道瘘,1例合并膀胱阴道瘘;5例存在远端尿道闭锁或细小狭窄;5例合并阴道狭窄或部分闭锁.手术方式:经会阴尿道阴道瘘修补术1例,经耻骨尿道吻合术(端端吻合)2例,经耻骨尿道阴道瘘修补术+膀胱颈尿道吻合术1例,经耻骨尿道成形术(膀胱颈及尿道拖出)+尿道阴道瘘修补术+膀胱颈重建术4例,经耻骨尿道成形术+膀胱颈重建术1例,肠扩大膀胱阑尾代输出道可控性尿流改道术1例.术后观察排尿情况.每日使用尿垫数量＞1片为尿失禁,≤1片为控尿良好;主诉排尿费力,最大尿流率＜10ml/s为排尿困难.结果 本研究10例手术均顺利完成,术后无伤口感染、组织坏死等并发症.术后随访时间(52.0±26.2)个月.2例术后控尿良好,无排尿困难;7例出现并发症,其中2例因尿道狭窄致排尿困难,行尿道扩张后好转;5例尿道阴道瘘复发,其中3例再次行修补手术,再次手术后2例控尿良好,无排尿困难.1例尿流改道患者术后恢复良好.结论 女童陈旧骨盆骨折尿道外伤的处理较复杂,术后并发症发生率高,需根据年龄、病变严重程度和尿道、阴道条件选择合适的处理方式.

目的 探讨尿动力学(urodynamics,UDS)检查评估无神经系统及下尿路器质性病变的膀胱输尿管反流(vesicoureteral reflux,VUR)患儿下尿路功能障碍(lower urinary tract dysfunction,LUTD)的临床意义.方法 回顾性分析2017年1月至2021年12月首都医科大学附属北京儿童医院收治的106例VUR患儿UDS检查结果,按照有无LUTD将患儿分为有LUTD组(77例)和无LUTD组(29例),比较两组患儿膀胱容量、残余尿量(post voiding residual urine volume,PVR)以及最大尿流率等尿动力学指标.结果 有LUTD组残余尿量97.71±93.17 mL,明显高于无LUTD组11.81±9.17 mL(t=5.564,P=0.008);有 LUTD 组最大尿流率 8.82±5.01 mL/s,明显低于无 LUTD 组 15.04±7.75 mL/s(t=6.314,P=0.030).有LUTD组中,29例为低级别VUR(Ⅰ～Ⅲ级),48例为高级别VUR(Ⅳ～Ⅴ级).低级别VUR患儿中3例膀胱顺应性减低,高级别VUR患儿中19例顺应性减低,两者比较(x2=7.537,P=0.006)差异有统计学意义;低级别VUR患儿中7例PVR增加,高级别VUR患儿中23例PVR增加,两者比较(x2=4.298,t=0.038)差异有统计学意义.特发性逼尿肌过度活动(idiopathic detrusor overactivity disorder,IDOD)35例(35/77,45.5％),其中19例为低级别VUR,占低级别VUR总数的65.5％(19/29);逼尿肌括约肌协同失调(dysfunctional voiding,DV)33例,其中27例为高级别VUR,占高级别VUR总数的56.3％(27/48).结论 PVR增多、最大尿流率降低提示VUR患儿存在LUTD的可能.高级别VUR患儿中,PVR增加更为明显、且膀胱顺应性更差.IDOD在低级别VUR中常见,DV在高级别VUR中更为常见.

目的 探讨儿童肾盏憩室(calyceal diverticulum,CD)的临床特点、诊断及手术治疗方式.方法 回顾性分析2010年1月至2021年10月首都医科大学附属北京儿童医院泌尿外科与包头市第四医院小儿外科收治的7例肾盏憩室患儿临床资料,其中男5例,女2例;就诊年龄8个月至11岁,平均年龄3岁11个月.憩室位于左侧3例,右侧4例;肾上盏4例,肾中盏3例.憩室长径3～9cm,平均6.2 cm.产前超声发现肾脏囊性变3例,腹痛2例,无症状腹部包块1例,体检发现肾脏囊性病变合并高血压1例.3例术前经泌尿系超声检查确诊,4例术中确诊.7例均行手术治疗,其中开放肾盏憩室切除术2例,肾盏憩室剪裁+盏颈扩大术3例,开放和腹腔镜憩室壁翻瓣卷管输尿管端侧吻合术各1例.结果 7例均获随访,随访时间3个月至10年,患儿术后CD相关症状均消失,术前扩张的肾盏憩室均明显缩小,无一例复发;1例合并肾积水患儿术后积水明显减轻,但肾功能恢复较差,肾核素扫描显示分肾功能为20％,目前仍在随访中.结论 肾盏憩室易发生反复泌尿系感染、慢性腹痛、肉眼血尿、结石等,易误诊为单纯性肾囊肿,两者处理方式不同,需加以区分.应根据憩室位置、盏颈狭窄程度采取个体化治疗,包括肾盏憩室切除、盏颈扩大部分憩室切除、憩室壁翻瓣卷管输尿管端侧吻合术.如处理得当通常预后较好,少有复发.

PurposeWe aimed to explore whether Nephroblastomatosis (Nbm) has an effect on the prognosis of Wilms tumor (WT), and compare the relapse-free survival (RFS) and overall survival (OS) after surgery of WT patients with or without Nbm.MethodsWe retrospectively analyzed the clinical data of children with WT admitted to our department from April 2010 to September 2021. The enrolled patients were divided into two groups according to whether they were combined with Nbm histologically or not, the Nbm group for patients accompany with Nbm and the non-Nbm group for pure WT. All patients underwent a standardized comprehensive treatment according to National Wilms Tumor Study 5. Clinical variables, pathological results, and the prognosis were collected during follow-up.ResultsA total of 345 patients were finally enrolled in this study, of which 299 patients in the non-Nbm group and 46 patients in the Nbm group. Univariate Cox analysis revealed that Nbm was not the risk factor of either OS or RFS. The difference of postoperative RFS (P = 0.66) and OS (P = 0.68) between two groups was not significant. Subgroup analysis revealed that the RFS and OS between the non-Nbm group and the Nbm group had no statistical difference under the condition of same stage (low grade and high grade), same histology (favorable histology and unfavorable histology), same surgical approach (nephrectomy and nephron-sparing surgery), with or without lymph node metastasis (P > 0.05).ConclusionAlthough Nbm had a strong correlation with the occurrence of WT, a combined Nbm did not increase the risk of metastasis and poor prognosis of WT. After complete surgical resection followed by standardized treatment, the long-term RFS and OS were not different from pure WT.IEC-C-006-A04-V.06 retrospectively registered.

Introduction Current research on the posterior urethral valve (PUV) mainly focuses on the follow-up of bladder function after valve ablation. However, few studies exist on the changes in bladder function before and after valve ablation. Objectives To investigate the urodynamic changes before and after PUV ablation and determine the effect of operation on bladder function, in patients. Materials and methods The clinical records of 38 boys diagnosed with PUV and undergone urodynamic exams before and after valve ablation were retrospectively reviewed. In addition, differences in patients’ radiographic studies and urodynamic characteristics between pre- and post-operation were evaluated. Moreover, the urodynamic data was compared using the paired t-test and all the data was expressed as means ± SEM. Additionally, p values less than 0.05 were considered to be statistically significant. Results All the patients were diagnosed with PUV and the follow-up period after operation ranged between 9 and 114 months. The urodynamic exams were performed about 6 months after operation. The results revealed that bladder compliance improved from 8.49 ± 4.73 to 13.31 ± 6.78 ml/cmH 2 O while the maximum detrusor pressure decreased from 95.18 ± 37.59 to 50.71 ± 21.71 cmH 2 O, after valve ablation. Additionally, there were significant differences in the pre- and post-operation values of bladder compliance and maximum detrusor pressure ( p < 0.05). However, there were no significant differences in the pre- and post-operation values with regard to the residual urine volume, maximum bladder volume and maximum urinary flow rate ( p > 0.05). Conclusions The adequacy of the COPUM incision is necessary. But the study showed that endoscopic valve ablation couldn’t by itself completely improve the bladder function of patients diagnosed with PUV. However, it was able to improve bladder compliance and decrease maximum detrusor pressure to a certain extent. However, bladder compliance still couldn’t reach the normal level.

Objective:To summarize the clinical characteristics, treatments and prognoses of pediatric iatrogenic ureteral injury and lower the incidence of iatrogenic ureteral injury and the loss of long-term renal function.Methods:A total of 19 cases of iatrogenic ureteral injury from January 2005 to December 2020 were reviewed.There were 14 boys and 5 girls with an average age of 6 years.The involved side was left (n=7) and right (n=12). The causes, classifications, clinical manifestations, diagnostic modalities, management principles and long-term follow-up outcomes were recorded.Results:Among them, ureteral injury was caused by retroperitoneal or pelvic tumor operation (n=13) and appendectomy, high ligation of hernia sac and radical operation of Hirschsprung's disease (n=2). Six cases were treated promptly, including ureteropelvic anastomosis (n=2), end-to-end ureteral anastomosis (n=3) and ureteral replantation (n=1). Delayed diagnosis was made in 13 cases and time of postoperative diagnosis ranged from 4 days to 3 years.Two cases of hernia sac underwent ureteral reimplantation while the remainders had nephrostomy.After (3-6) months, pelvic ureteral anastomosis (n=1), ureteral end-to-end anastomosis (n=3), ureteral reimplantation (n=1), Boair surgery (n=2), appendix replacing ureter (n=2) and self-healing (n=2) were performed.During a follow-up period of (8-242) months, no obvious hydronephrosis was present in 2 cases of ureteral injury from ligation of hernia sac.The other 11 cases had varying degrees of hydronephrosis and remained stable during follow-ups.Conclusions:Iatrogenic ureteral injury in children occurs frequently during retroperitoneal, pelvic and inguinal operations.If detected timely and treated properly, long-term renal function may be preserved.

Objective:To evaluate the application value of distal ureter diameter ratio as a predictive factor for breakthrough urinary tract infection (BT-UTI) in children with primary vesicoureteral reflux (VUR).Methods:From January 2018 to December 2020, retrospective review was performed for the clinical features of 102 VUR children.Clinical data were collected for identifying the predictors of BT-UTI.Demographics, VUR grade, laterality, distal ureter diameter and UDR were examined by univariate and multivariable analyses.Primary outcome was BT-UTI.Results:Among them, 31 children (30.3%) experienced BT-UTI events.Based upon multivariate analysis, gender, maximal grade of reflux and laterality were not the predictors of BT-UTI.Ureteral diameter ratio (UDR)( P=0.0417, OR=11.4, 95% CI: 1.259-141.919) and age ( P=0.0467, OR=0.975, 95% CI: 0.949-0.997) were among the strongest predictors of early febrile BT-UTI in VUR children. Conclusion:Children with elevated distal UDR are at an elevated risk for BT-UTI independent of reflux grade.As an objective measurement of VUR, UDR provides valuable prognostic information for risks of recurrent pyelonephritis and may assist with clinical decision-making.

Objective:To improve the preoperative diagnosis of paratesticular tumor in children and to formulate an optimal and individualized treatment protocol.Methods:A total of 27 children of paratesticular tumor undergoing operations were reviewed retrospectively.The average age was 7.4(0.3-14.5) years.Tumor was located at left (n=12), right (n=14) and bilateral (n=1). All of them received preoperative physical and ultrasonic examinations.The average time from a discovery of testicular mass to surgery was 4.5 months (1 week to 3 years). Eight children underwent high ligation of spermatic cord through an inguinal incision during tumor orchiectomy.One child underwent resection of testicular para testicular masses through an inguinal incision.And 18 cases underwent resection of testicular simple para testicular masses through a scrotal incision.Without lymph node or distant metastasis, 6 low-risk children of parathyroid rhabdomyosarcoma received a combination of vincristine, actinomycin D and cyclophosphamide.One case of leukemic metastasis continued postoperative chemotherapy.The remainders received no further postoperative treatment.Results:The pathological examinations revealed benign (n=17) and malignant (n=10) tumors.Benign tumors included epididymal cyst (n=10), adrenal residual tumor (n=1), epididymal leiomyoma (n=1), infantile fibrous hamartoma (n=1), epididymal fibrous pseudotumor (n=1), lipoblastoma (n=1), splenic gland fusion (n=1) and lymphangioma (n=1). Malignant tumors (n=10) included paratesticular rhabdomyosarcoma (embryonic type) (n=6), infantile melanotic neuroectodermal tumor (n=1), well-differentiated liposarcoma (n=1), leukemic metastasis (n=1) and ganglionic neuroblastoma metastasis (n=1). The preoperative clinical diagnosis was basically consistent with the pathology.During an average follow-up period of 70(5-137) months, only 1 child of metastatic ganglioblastoma was re-operated for tumor recurrence on left face at Month 6 postoperatively.Conclusions:For paratesticular tumors in children, it is necessary to make a comprehensive judgment based upon medical history, laboratory tests and imaging examinations to make a definite preoperative diagnosis to avoid a delayed treatment of malignant tumors.

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.

PurposeEsophageal perforation caused by foreign body is common in Chinese medical institutions, and resultant deep neck infections (DNI) is quite different from typical DNI. The purpose of this article was to share our experience on management of this particular type of DNI.Material and methodsA retrospective review was conducted on a consecutive sample of such patients at Capital Medical University Beijing Friendship Hospital from 2015 to 2019.ResultsIn total, 24 cases were recorded. CT scan of the neck and upper thorax was the most useful tool for early diagnosis. Gas formation was not predictive of a worse clinical course. Eleven patients with minor DNI were treated with antibiotics and foreign body removal; while 13 patients with major DNI were treated with neck incision and drainage, ICU observation, and prolonged usage of antibiotics. Outcome was generally good, but major complications, including sepsis and lingual artery rupture, could occur.ConclusionsConservative management, focusing on prompt extraction of esophageal foreign body and adequate antibiotic coverage, can lead to good outcome for mild cases; while in addition to these measures, neck incision, cervical and superior mediastinal exploration, and high negative pressure drainage, should be performed for severe cases.

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.