Survival and Analysis of Prognostic Factors for Bladder Malignancies in Children and Adolescents: A Population-based Study.

OBJECTIVE: To explore the clinicopathological features and prognosis of pediatric patients with malignant bladder tumors in a population-based cohort. METHODS: The database Surveillance, Epidemiology, and End Results was used to evaluate all pediatric patients diagnosed with malignant bladder tumors between 1975 and 2018. The log-rank test was used to compare survival curves. Kaplan-Meier estimations were used to create survival curves based on various parameters. The Cox proportional hazards model was utilized to determine the factors that were independently related to mortality. RESULTS: A total of 263 children and adolescents with bladder malignancies were assessed. Papillary urothelial neoplasms of low malignant potential were the most frequent histologic subtype (35.1%), while embryonal rhabdomyosarcoma was more common during the first decade of life. Survival rates varied significantly by age at diagnosis, with older patients showing better outcomes. When compared to other subtypes, papillary urothelial neoplasms of low malignant potential had the highest overall survival rates (3- and 5-year were 99.2% and 98.3%, respectively). Multivariate analysis of the entire cohort showed that Surveillance, Epidemiology, and End Results stage and surgery were significant independent predictors of progression to disease-specific death in this model. CONCLUSION: Bladder malignancies are rare in children and adolescents. The prognosis for them varies. The localized stage was independently associated with superior survival and surgery could extend survival time.

Minimally Invasive versus Open Ureteral Reimplantation in Children: A Systematic Review and Meta-Analysis.

PURPOSE: We performed a systematic review and meta-analysis to compare the safety and efficacy of minimally invasive surgery (MIS) versus open ureteral reimplantation (OUR) in children. METHODS: Literature searches were conducted to identify studies that compared MIS (laparoscopic ureteral reimplantation or robot-assisted laparoscopic ureteral replantation) and OUR in children. Parameters such as operative time, blood loss, length of hospital stay, success rate, postoperative urinary tract infection (UTI), urinary retention, postoperative hematuria, wound infection, and overall postoperative complications were pooled and compared by meta-analysis. RESULTS: Among the 7,882 pediatric participants in the 14 studies, 852 received MIS, and 7,030 received OUR. When compared with the OUR, the MIS approach resulted in shorter hospital stays (I 2 = 99%, weighted mean difference [WMD] -2.82, 95% confidence interval [CI] -4.22 to -1.41; p < 0.001), less blood loss (I 2 = 100%, WMD -12.65, 95% CI -24.82 to -0.48; p = 0.04), and less wound infection (I 2 = 0%, odds ratio 0.23, 95% CI 0.06-0.78; p = 0.02). However, no significant difference was found in operative time and secondary outcomes such as postoperative UTI, urinary retention, postoperative hematuria, and overall postoperative complications. CONCLUSION: MIS is a safe, feasible, and effective surgical procedure in children when compared with OUR. Compared with OUR, MIS has a shorter hospital stay, less blood loss, and less wound infection. Furthermore, MIS is equivalent to OUR in terms of success rate and secondary outcomes such as postoperative UTI, urinary retention, postoperative hematuria, and overall postoperative complications. We conclude that MIS should be considered an acceptable option for pediatric ureteral reimplantation.

Spongioplasty with Buck's fascia covering dorsal inlay graft urethroplasty for primary hypospadias repair.

INTRODUCTION: Neourethral covering is an essential technique for preventing complications such as fistula and glans dehiscence in hypospadias repairs. The spongioplasty has been reported for neourethral coverage about 20 years ago. However, reports of the outcome are limited. OBJECTIVE: This study aimed to retrospectively evaluate the short-term outcome of spongioplasty with Buck's fascia covering dorsal inlay graft urethroplasty (DIGU). METHODS: From December 2019 to December 2020, 50 patients with primary hypospadias (median age at surgery, 37 months; range, 10 months-12 years) were treated by a single pediatric urologist. The patients underwent spongioplasty with Buck's fascia covering dorsal inlay graft urethroplasty in single stage. The penile length, glans width, urethral plate width and length, and the location of the meatus of the patients were recorded preoperatively. The patients were followed up,complications noted, and postoperative uroflowmetries at the one-year follow-up time were evaluated. RESULTS: The average width of glans was 12.92 ± 1.86 mm. A minor penile curvature was observed in all patients (≤30°). The patients were followed up for 12-24 months, and 47 patients (94%) were free from complications. A neourethra formed with a slit-like meatus at the tip of the glans, and the urinary stream was straight. Three patients had coronal fistulae (3/50) and no glans dehiscence, and the mean ± SD Qmax of postoperative uroflowmetry was 8.13 ± 3.8 ml/s. DISCUSSION: This study estimated the short-term outcome of the DIGU covered using spongioplasty with Buck's fascia as the second layer in patients diagnosed with primary hypospadias with a relatively small glans (average width <14 mm). However, only a few reports emphasize spongioplasty with Buck's fascia as the second layer and the DIGU procedure performed on a relatively small glans. The major limitations of this study were its short follow-up time and the retrospective data collection. CONCLUSIONS: Dorsal inlay graft urethroplasty combined with spongioplasty with Buck's fascia as coverage is an effective procedure. In our study, this combination had good short-term outcomes for primary hypospadias repair.

Glutathione metabolism rewiring protects renal tubule cells against cisplatin-induced apoptosis and ferroptosis.

Renal proximal tubular cells are highly vulnerable to different types of assaults during filtration and reabsorption, leading to acute renal dysfunction and eventual chronic kidney diseases (CKD). The chemotherapeutic drug cisplatin elicits cytotoxicity causing renal tubular cell death, but its executing mechanisms of action are versatile and elusive. Here, we show that cisplatin induces renal tubular cell apoptosis and ferroptosis by disrupting glutathione (GSH) metabolism. Upon cisplatin treatment, GSH metabolism is impaired leading to GSH depletion as well as the execution of mitochondria-mediated apoptosis and lipid oxidation-related ferroptosis through activating IL6/JAK/STAT3 signaling. Inhibition of JAK/STAT3 signaling reversed cell apoptosis and ferroptosis in response to cisplatin induction. Using a cisplatin-induced acute kidney injury (CAKI) mouse model, we found that inhibition of JAK/STAT3 significantly mitigates cisplatin nephrotoxicity with a reduced level of serum BUN and creatinine as well as proximal tubular distortion. In addition, the GSH booster baicalein also reclaims cisplatin-induced renal tubular cell apoptosis and ferroptosis as well as the in vivo nephrotoxicity. In conclusion, cisplatin disrupts glutathione metabolism, leading to renal tubular cell apoptosis and ferroptosis. Rewiring glutathione metabolism represents a promising strategy for combating cisplatin nephrotoxicity.

Intrauterine neuromuscular and stromal dysplasia of the bladder in retinoic acid-induced myelomeningocele fetal rats.

OBJECTIVES: To observe the changes in the bladder of fetal rats with myelomeningocele (MMC) induced by all-trans retinoic acid (atRA) during the embryonic development stages. METHODS: The fetal rat model of MMC was induced by intragastric administration of atRA to pregnant rats on embryonic day 10 (E10). Fetal rats were harvested at E16, E18, E20, and E21 for observation and further testing. Those with MMC were classified as the MMC group, while those without MMC as the RA group. The areas of MMC skin defect, the crown-rump length (CRL), and body weight in different groups were compared. The histopathological changes in the bladder were compared. The expression levels of alpha-smooth muscle actin (αSMA), smooth muscle myosin heavy chain (SMMHC), connexin 43 (Cx43), desmin, ß3 tubulin, and vesicular acetylcholine transporter (VAChT) in the bladder were investigated by immunohistochemical staining and Western blotting. Pregnant rats given intragastric administration with olive oil (OIL group) at E10 were set as the blank control group. RESULTS: A total of 415 fetal rats of different gestational ages were harvested with an MMC incidence of 56.05 % (139/248). The incidence rate increased with embryonic days (p < 0.001). Compared with the other two control groups, the CRL and bodyweight of MMC fetal rats were significantly delayed at E21 (p < 0.001). The expression levels of αSMA, SMMHC, Cx43, desmin, ß3 tubulin and VAChT in the bladder of MMC fetal rats were significantly decreased at E21 (p < 0.05). CONCLUSIONS: In atRA-induced MMC fetal rats, there is neural, muscular, and stromal dysplasia in the bladder at an early gestational age. Further investigations on neurogenic bladder secondary to MMC are applicable using this animal model.

Tumor Suppressive Role of MUC6 in Wilms Tumor via Autophagy-Dependent ß-Catenin Degradation.

Wilms tumor is the most common renal malignancy in children. Known gene mutations account for about 40% of all wilms tumor cases, but the full map of genetic mutations in wilms tumor is far from clear. Whole genome sequencing and RNA sequencing were performed in 5 pairs of wilms tumor tissues and adjacent normal tissues to figure out important genetic mutations. Gene knock-down, CRISPR-induced mutations were used to investigate their potential effects in cell lines and in-vivo xenografted model. Mutations in seven novel genes (MUC6, GOLGA6L2, GPRIN2, MDN1, MUC4, OR4L1 and PDE4DIP) occurred in more than one patient. The most prevalent mutation was found in MUC6, which had 7 somatic exonic variants in 4 patients. In addition, TaqMan assay and immunoblot confirmed that MUC6 expression was reduced in WT tissues when compared with control tissues. Moreover, the results of MUC6 knock-down assay and CRISPR-induced MUC6 mutations showed that MUC6 inhibited tumor aggression via autophagy-dependent ß-catenin degradation while its mutations attenuated tumor-suppressive effects of MUC6. Seven novel mutated genes (MUC6, GOLGA6L2, GPRIN2, MDN1, MUC4, OR4L1 and PDE4DIP) were found in WT, among which MUC6 was the most prevalent one. MUC6 acted as a tumor suppressive gene through autophagy dependent ß-catenin pathway.

Cryptorchid testicular torsion in children: characteristics and treatment outcomes.

This study aimed to review and compare the characteristics and treatment outcomes of cryptorchid testicular torsion in pre- and postpubertal children. We reviewed the clinical data of 22 patients with testicular torsion complicated by cryptorchidism who were treated between January 2010 and December 2019. Patients were categorized into prepubertal (1 month to 9 years; n = 12) and postpubertal groups (10-16 years; n = 10). The age at presentation, clinical presentations, physical examination, and operation outcomes were assessed. The common clinical presentations in both groups were inguinal pain and a tender inguinal mass. Patients in the prepubertal group were significantly more likely to present with restlessness (33.3%) than those in the postpubertal group (0%; P = 0.044). After detorsion, testicular blood flow recovered during surgery in 25.0% of the prepubertal and 80.0% of the postpubertal patients (P = 0.010). Orchiectomy was required in 50.0% of the prepubertal and 20.0% of the postpubertal patients (P = 0.145). Of the 22 patients with follow-up data, the rates of testicular salvage were significantly different, at 16.7% in the prepubertal patients and 60.0% in the postpubertal patients (P = 0.035). Cryptorchid testicular torsion has various manifestations. Although an empty hemiscrotum and a painful groin mass were common in both groups, restlessness was more prevalent in the prepubertal patients during early testicular torsion onset than that in the postpubertal patients. Notably, the testicular salvage rate was significantly lower in the prepubertal patients than that in the postpubertal patients.

Genetic Architecture of Childhood Kidney and Urological Diseases in China.

Kidney disease is manifested in a wide variety of phenotypes, many of which have an important hereditary component. To delineate the genotypic and phenotypic spectrum of pediatric nephropathy, a multicenter registration system is being implemented based on the Chinese Children Genetic Kidney Disease Database (CCGKDD). In this study, all the patients with kidney and urological diseases were recruited from 2014 to 2020. Genetic analysis was conducted using exome sequencing for families with multiple affected individuals with nephropathy or clinical suspicion of a genetic kidney disease owing to early-onset or extrarenal features. The genetic diagnosis was confirmed in 883 of 2256 (39.1%) patients from 23 provinces in China. Phenotypic profiles showed that the primary diagnosis included steroid-resistant nephrotic syndrome (SRNS, 23.5%), glomerulonephritis (GN, 32.2%), congenital anomalies of the kidney and urinary tract (CAKUT, 21.2%), cystic renal disease (3.9%), renal calcinosis/stone (3.6%), tubulopathy (9.7%), and chronic kidney disease of unknown etiology (CKDu, 5.8%). The pathogenic variants of 105 monogenetic disorders were identified. Ten distinct genomic disorders were identified as pathogenic copy number variants (CNVs) in 11 patients. The diagnostic yield differed by subgroups, and was highest in those with cystic renal disease (66.3%), followed by tubulopathy (58.4%), GN (57.7%), CKDu (43.5%), SRNS (29.2%), renal calcinosis /stone (29.3%) and CAKUT (8.6%). Reverse phenotyping permitted correct identification in 40 cases with clinical reassessment and unexpected genetic conditions. We present the results of the largest cohort of children with kidney disease in China where diagnostic exome sequencing was performed. Our data demonstrate the utility of family-based exome sequencing, and indicate that the combined analysis of genotype and phenotype based on the national patient registry is pivotal to the genetic diagnosis of kidney disease. Supplementary Information: The online version contains supplementary material available at 10.1007/s43657-021-00014-1.

Application and efficacy of reconstructing forked corpus spongiosum in distal/midshaft hypospadias repair.

We reviewed our experience in reconstructing forked corpus spongiosum (FCS) in distal/midshaft hypospadias repair and analyzed the efficacy of this surgical technique. From August 2013 to December 2018, 137 consecutive cases of distal/midshaft hypospadias operated by the same surgeon in Urology Department, Children's Hospital of Fudan University (Shanghai, China), were retrospectively analyzed. Sixty-four patients who underwent routine tubularized incised plate (TIP) or onlay island flap (ONLAY) surgery were included in the nonreconstructing group, and 73 patients who underwent reconstructing FCS during TIP or ONLAY surgery were included as the reconstructing group. Thirty-eight cases underwent TIP, and 26 underwent ONLAY in the nonreconstructing group, with a median follow-up of 44 (range: 30-70) months. Twenty-seven cases underwent TIP, and 46 underwent ONLAY in the reconstructing group, with a median follow-up of 15 (range: 6-27) months. In the nonreconstructing/reconstructing groups, the mean age at the time of surgery was 37.55 (standard deviation [s.d.]: 29.65)/35.23 (s.d.: 31.27) months, the mean operation duration was 91.95 (s.d.: 12.17)/93.84 (s.d.: 14.91) min, the mean neourethral length was 1.88 (s.d.: 0.53)/1.94 (s.d.: 0.53) cm, and the mean glans width was 11.83 (s.d.: 1.32)/11.56 (s.d.: 1.83) mm. Twelve (18.8%)/5 (6.8%) postoperative complications occurred in the nonreconstructing/reconstructing groups. These included fistula (5/2), glans dehiscence (3/0), diverticulum (1/2), residual chordee (3/0), and meatus stenosis (0/1) in each group. There was a significant difference in the overall rate of complications (P= 0.035). These results indicate that the technique of reconstructing FCS provides excellent outcomes with fewer complications in distal/midshaft hypospadias repair.

Exploration of postnatal integrated management for prenatal renal and urinary tract anomalies in China.

Objective: The aim of this prospective observational study was to establish a suitable model for the postnatal follow-up and management of prenatal renal and urinary tract anomalies in Shanghai, China.Methods: Minhang and Changning maternal child health care hospitals were selected to establish the integrated management model. Newborns with prenatal renal and urinary tract anomalies in these two centers were eligible to participate in the study from 2015 to 2017. All newborns were classified into three groups based on prenatal findings: (1) severe/complex urinary tract dilatation (UTD) with ureterectasia, (2) other renal and urinary tract abnormalities, and (3) isolated mild to moderate UTD. The newborns underwent their first postnatal ultrasound and follow-up according to the presumed management strategy. Demographic and clinical data were collected from all institutes.Results: A total of 129 newborns fulfilled the study criteria, and 121 completed the postnatal evaluation. Ten newborns in group 1 (n = 13) were diagnosed with obstructive uropathy, including 9 with ureteropelvic junction obstruction (UPJO) and one with megaureter. All 13 newborns in group 2 had consistent postnatal results and were followed under previously established procedures. Sixty-seven cases in group 3 (n = 95) had a UTD at their first scan at 42 postnatal days, and two were diagnosed with UPJO. A total of 2 infants with UPJO underwent surgery, and 71 (65.7%, 71/108) of the UTD cases were resolved.Conclusions: The majority of the patients had a favorable outcome. Close multidisciplinary collaboration among obstetricians, neonatologists, pediatricians, and pediatric nephrologists and urologists is mandatory.

Posterior Urethral Valves with Lower Urinary Tract Symptoms: Perspective on Urodynamics.

BACKGROUND: Lower urinary tract symptoms (LUTs) are common in young boys with posterior urethral valves (PUVs). It is crucial to investigate the characteristics of PUV patients with and without LUTs after valve ablation. METHODS: Between January 2017 and December 2019, PUV patients visited Children's Hospital, Fudan University for following up were enrolled. Medical records and information from the patients' urodynamic studies (UDS) were reviewed. RESULTS: A total of 54 enrolled PUV patients were divided into symptomatic PUV group (28 cases) and non-symptomatic PUV group (26 cases) according to daytime incontinence or not, and 21 OAB cases without structural abnormalities were set as UDS control group. The non-symptomatic PUV patients had lager filling volume (135 ± 46% of EBC) than the symptomatic PUV patients and OAB patients (106.1 ± 44.4% of EBC, p = 0.0255 and 88.1 ± 39.6% of EBC, p = 0.0007, respectively). The detrusor pressure at 1/4 and 3/4 of full filling was higher in PUV groups than the control group, but no significant difference was found between the PUV groups. PUV patients with LUTs had a higher rate (19/28, 67.9%) of impaired bladder compliance than non-symptomatic PUV patients (11/26, 42.3%, p = 0.0489). The PUV patients with LUTs had a trend of worse kidney functions in lower GFR, higher serum creatinine and lower estimated GFR. CONCLUSION: PUV patients have higher detrusor pressure regardless of the presence or absence of LUT symptoms. Bladder function assessments are needed in boys with PUV, even without incontinence symptoms after valve ablation.

Early diagnosis of WT1 nephropathy and follow up in a Chinese multicenter cohort.

BACKGROUND: WT1 mutations cause a wide spectrum of renal and extrarenal manifestations concerning urogenital development and the development of tumors. METHODS: We retrospectively collected the information on the genotype and phenotype of WT1 nephropathy from the multicenter registry since 2014 to 2019. All patients were stratified by renal function decline status or by sequence timing. Rapid progressive group was defined as rapidly developing into ERSD within 12 months since disease onset. Early sequencing group was defined as gene mutation identified before ERSD. RESULTS: Thirty-three (3.5%) cases were identified with a WT1 mutation in patients with steroid resistant nephrotic syndrome (SRNS), proteinuria and chronic kidney disease (CKD) 3-5 stage of unknown origin. ESRD developed in twenty patients at a median age of 4.3 years old. Comparing study between the rapid progressive group (n = 8) and non-rapid progressive group (n = 25) showed no significant difference in age of onset, gender, syndrome phenotype, genotype and proteinuria except for initial estimated glomerular filtration rate (eGFR) (p = 0.021) or sequencing timing (p = 0.003). In multivariable logistic regression analysis, the delayed sequencing was associated with rapid renal function decline, even after adjusting for established clinical factors including syndromic phenotype, genotype, age onset and eGFR at initial stage (p = 0.019). The renal survival analysis did not show a significantly better outcome in early sequencing group than in delayed sequencing group (p > 0.05). CONCLUSION: Screening for WT1 mutations should be performed in children with Wilms' tumor, proteinuria/SRNS or CKD. Early diagnosis of WT1 nephropathy through clinical and genetic findings is warranted.

Outcomes of laparoscopic continent catheterizable conduit procedures in children with neurogenic bladder: An 8-year experience in a single center.

BACKGROUND: The continent catheterizeable conduit (CCC) procedure plays an important role in the treatment of children with neurogenic bladder. Laparoscopic procedures offer significant benefits; however, the potential complications should not be ignored. OBJECTIVE: By comparing the complications of CCC in laparoscopic and open neurogenic bladder operations in our center, we aimed to explore the advantages of laparoscopic CCC construction in children. STUDY DESIGN: All the cases of neurogenic bladder who had undergone laparoscopic or open lower urinary tract reconstruction at our hospital from June 2009 to December 2016 were retrospectively reviewed. We compared the rates of various complications in the laparoscopic and open groups as well as the appendix and Yang-Monti groups. RESULTS: 61 patients were included in this study. Laparoscopic CCC procedure was performed in 45 cases, 9 were converted to open owing to other combined procedures. Open CCC construction was performed in 16 cases. The mean follow-up time was 4.5 years. Catheterization was safe and uneventful in 72.1% of patients. Complications occurred in 17 cases, including stoma stenosis (n = 5), leakage (n = 5), mucosal prolapse (n = 3), conduit atresia (n = 3), and angulation of the conduit (n = 1). Eleven cases received surgical revision. There were no statistically significant differences in complication type or rate in the laparoscopic and open group, or in the appendix and Yang-Monti group. DISCUSSION: Although our study showed a notable complication rate associated with CCC, this did not differ for open and laparoscopic procedures, and laparoscopic procedures can achieve satisfactory results comparable to open procedures. CONCLUSION: From our single center's experience of lower urinary tract reconstruction, laparoscopic CCC surgery has a shorter hospital stay and no more complications than the open procedure. It is a safe and feasible procedure in children.

Reconstructing forked corpus spongiosum to correct glans droop in distal/midshaft hypospadias repair.

PURPOSE: We reviewed our experience and efficacy of reconstruction of a forked corpus spongiosum (FCS) to correct glans droop in distal/midshaft hypospadias repair. METHODS: Eighty-five consecutive patients who underwent distal/midshaft hypospadias repair by the same surgeon in our center from October 2015 to June 2018 were retrospectively analyzed. All cases were accompanied by different degrees of glans droop, which we corrected by cutting off and reconstructing the FCS along the plate. We recorded the degrees of glans droop, development of the FCS, and postoperative complications including residual chordee, fistula, diverticulum, glans dehiscence, meatus stenosis, and urethral stricture. RESULTS: The follow-up period ranged from 5 to 37 months (mean, 19.7 months). Two patients (2.3%) developed a coronal fistula and underwent a second repair. Two patients (2.3%) developed a mild urethral diverticulum and underwent continued observation. One patient (1.2%) developed a meatus stenosis that resolved after 1 month of meatus expansion combined with external mometasone furoate. No patients developed postoperative residual chordee or urethral stricture. CONCLUSIONS: The degree of glans droop is closely associated with the development of an FCS. Reconstructing the FCS to correct the glans droop can yield satisfactory outcomes and should be popularized in distal/midshaft hypospadias repair.

Genetic spectrum of renal disease for 1001 Chinese children based on a multicenter registration system.

To explore the approaches and diagnostic yield of genetic testing for renal disease in children, we describe the genotype and phenotype of the national cohort of children with renal disease from 13 different regions of China recruited from 2014 to 2018 by building up the multicenter registration system (Chinese Children Genetic Kidney Disease Database, CCGKDD). Genetic diagnosis was confirmed in 42.1% of our cohort of 1001 pediatric patients with clinical suspicion of a genetic renal disease. Of the 106 distinct monogenetic disorders detected, 15 accounted for 60.7% of genetic diagnoses. The diagnostic yield was 29.1% in steroid resistant nephritic syndrome (SRNS), 61.4% in cystic renal disease, 17.0% in congenital anomalies of the kidney and urinary tract (CAKUT), 62.3% in renal tubular disease/renal calcinosis, and 23.9% for chronic kidney disease (CKD) 3 to 5 stage with unknown origin. Genetic approaches of target gene sequence (TGS), singleton whole-exome sequencing (WES) and trio-WES were performed with diagnostic rates of 44.8%, 36.2%, and 42.6%, respectively. The early use of trio-WES could improve the diagnostic rate especially in renal tubular disease and calcinosis. We report the genetic spectrum of Chinese children with renal disease. Establishment of the CCGKDD will improve the genetic work on renal disease.

Early detection of congenital anomalies of the kidney and urinary tract: cross-sectional results of a community-based screening and referral study in China.

OBJECTIVE: To establish an effective screening model of congenital anomalies of the kidney and urinary tract (CAKUT) using ultrasound among neonates in Shanghai, China. DESIGN: Cross-sectional study. SETTING: A three-level screening model for CAKUT in neonates based on the child healthcare system was established since 2010 in Minhang District, Shanghai, China. PARTICIPANTS: During 2010-2015, neonates with criteria such as preterm, low birth weight and so on were eligible to participate in the study. Cases with renal pelvis dilatation (RPD) and other abnormal renal findings were managed based on presumed strategies. MAIN OUTCOME MEASURES: The proportion of RPD and other renal and urinary tract anomalies; number of diagnosed CAKUT under integrated management, especially obstructive uropathy. The anterior-posterior renal pelvic diameter (APRPD) cut-off points for likelihood of obstructive uropathy and need for surgery. RESULTS: A total of 8827 infants were consecutively screened. Absolute and relative rates of different degrees of RPD classified by APRPD were: mild (5-9.9 mm), 984 (11.1%); moderate (10-14.9 mm), 176 (2.0%); severe (≥15 mm), 20 (0.2%). Of 639 followed cases with RPD, 11 were diagnosed as obstructive uropathies. Of these, nine patients underwent surgery, at median age 2 months. A total 85.4% of mild, 62.5% of moderate and 30.0% of severe RPD cases resolved spontaneously. Other renal and urinary morphological abnormalities were diagnosed in 15 (0.2%) patients. The APRPD cut-off points for significant obstructive uropathy and need for surgery were 9.7 mm and 13.5 mm, respectively. CONCLUSIONS: This three-level screening model is an effective and feasible strategy for early detection and intervention of CAKUT in the early postnatal period, especially for patients with high-grade RPD and other renal and urinary malformations. This strategy could be useful in China and other developing areas with limited medical resources.

Separation of pygopagus, omphalopagus, and ischiopagus with the aid of three-dimensional models.

BACKGROUND: The three-dimensional (3D) technique provides with accurate anatomical information. We present the separation surgeries for three different kinds of conjoined twins with the aid of three-dimensional techniques. METHOD: For the pygopagus twins, a pelvic and lower vertebral model was made. For the omphalopagus and ischiopagus, their enhanced computed tomography (CT) scan images were transferred to the Computer-Assisted Surgery Planning System (CASP) (Hisense, Qingdao, China) to generate the 3D models. RESULT: In the case of the pygopagus twins, the 3D model clearly showed that their coccyges were joined at a 120°angle from each other horizontally which suggested that the blind-end orifice on their back was a pilonidal sinus, which separated the normal sphincter into two halves. In the omphalopagus, the 3D model revealed one of the branches of each twin's hepatic vein was connected with the other's. The 3D model of the ischiopagus twins revealed that both of the twins had duplicated bladders and each baby's duplicated bladders united with one of the bladders of the other baby and a single rectum passing through the middle of the four bladders. CONCLUSION: 3D techniques could provide more detailed anatomical information, which is helpful in planning procedures for such complicated separation surgery. LEVELS OF EVIDENCE: Level IV.

Epidemiologic study of 230 cases of testicular/paratesticular tumors or masses: 15-year experience of a single center.

PURPOSE: This study retrospectively investigated the cases of testicular tumors/masses treated in our center from 2002 to 2017 and analyzed their epidemiologic features. METHODS: Data were collected by searching our center's database using "testicular tumor" or "testicular mass" as keywords. Patients not operated in our hospital were excluded. Preoperative serum alpha-fetoprotein (AFP) levels were reviewed in germ cell tumor (GCT) cases and analyzed to predict malignancy in various age groups. RESULTS: In total, 230 cases were identified: 151 were benign (78 in the left, 72 in the right, and 1 bilateral) with 3.63years mean age during the operation, and 79 were malignant (42 in the left, 36 in the right, and 1 bilateral) with 2.21years mean age during the operation. Main pathological diagnoses were mature teratoma (92, 40.00%), yolk sac tumor (53, 23.04%), dermoid cyst (23, 10.00%), embryonic carcinoma (15, 6.53%), immature teratoma (14, 6.09%), benign cyst (8, 3.48%), Leydig cell tumor (6, 2.61%), and paratesticular rhabdomyosarcoma (5, 2.17%). All GCT cases with AFP >1000ng/ml, >100ng/ml, >20ng/ml were malignant in <7-, 7-9-, and ≥10-month-old groups, respectively. CONCLUSIONS: Radical inguinal orchiectomy without biopsy is suggested in 7-9- and ≥10-month-old cases with AFP >100ng/ml and >20ng/ml, respectively. TYPE OF STUDY: Retrospective Study. LEVEL OF EVIDENCE: Level III-IV.

In utero repair of fetal rat myelomeningocele affects neuromuscular development in the bladder.

Fetal repair of myelomeningocele (MMC) has been proven to be beneficial for the central nervous system development; however, the effect of fetal MMC repair on bladder function remains controversial. The objective of the present study was to establish an early timepoint for in utero MMC repair using a rat model, and to investigate the changes in bladder development subsequent to that intervention. Sprague Dawley rats were divided into the MMC, MMC repair and control groups. MMC rat fetus models were created by treating pregnant rats with all-trans retinoic acid. The MMC defect was then repaired in utero at embryonic day 17 (E17) using a chitosan-gelatin membrane patch. Fetal rat bladders were removed at E19 and E21 in each group, as well as at stage E17 in the MMC and control groups. Differential expression of ß-III-tubulin, α-smooth muscle actin (α-SMA), nerve growth factor (NGF) and acetylcholinesterase (AChE) mRNA, and ß-III-tubulin and α-SMA protein in the bladder following fetal repair was measured and compared among the three groups. In addition, the expression of NGF mRNA was significantly elevated at E21 in the MMC group compared with that of the control group, however, the level decreased in the repair group at stage E21. The expression of α-SMA mRNA significantly increased at E19 and then decreased at E21 in the repair group compared with that of the MMC group; however, there were no significant changes in α-SMA protein following the repair. Furthermore, the repair enhanced ß-III-tubulin mRNA expression at E19, but ameliorated the decrease of ß-III-tubulin protein at E21. The expression of AChE mRNA increased in the MMC group at E19 and E21 compared with that of the control group, although it was not significantly altered following repair as compared with that of the MMC group. In conclusion, in the current study, abnormal neuromuscular development was observed in the MMC bladder, which enabled a certain degree of improvement in the in utero MMC repair.