Propensity score matched analysis of treatment outcomes in pediatric ureteropelvic junction obstruction: a comparison between horseshoe and non-horseshoe kidneys.

BACKGROUND: Pediatric hydronephrosis poses distinct challenges, particularly in cases involving horseshoe kidneys (HSK). This retrospective study compares treatment outcomes between HSK and non-horseshoe kidneys (NHSK) in pediatric ureteropelvic junction obstruction (UPJO) patients. METHODS: A retrospective cohort study included 35 patients with HSK and 790 patients with NHSK undergoing pyeloplasty. Preoperative, intraoperative, and postoperative parameters were evaluated. Propensity score matching (PSM) balanced patient characteristics in the NHSK group. RESULTS: In comparison with NHSK, HSK exhibited a higher crossing vessel incidence (51.6% vs. 5.12%, P < 0.001) and smaller preoperative anteroposterior pelvic diameter (APD). Post 6 and 12 months, NHSK maintained a larger APD, with a higher P/C ratio at 12 months. PSM retained significantly higher crossing vessel incidence in HSK (51.6 vs. 3.61%, P < 0.001). Laparoscopic pyeloplasty (LP) in HSK showed lower postoperative length of stay (LOS). Postoperative ultrasound parameters favored NHSK. In HSK and NHSK with crossing vessels, HSK demonstrated higher complications even post-PSM (38.5% vs. 0%, P = 0.039). CONCLUSIONS: The study emphasizes the importance of recognizing crossing vessels in HSK-related hydronephrosis. Surgical success, although comparable between HSK and NHSK, requires tailored approaches. This investigation contributes valuable insights to pediatric urology, emphasizing personalized management for optimal outcomes.

Does bilateral Wilms' tumor involving the collecting system in children have a worse prognosis?

BACKGROUND: The literature on nephron-sparing surgery (NSS) in children with bilateral Wilms' tumors (BWT) involving the collection system is mostly comprised of case reports. The present study aimed to summarize the clinical characteristics, treatments, and prognosis of children with BWT involving the collecting system admitted to our pediatric surgery center compared with those whose tumors did not involve the collecting system. A secondary aim was to discuss how to preserve more kidney parenchyma and prevent long-term renal failure under the premise of preventing tumor recurrence. METHODS: Patients with BWT admitted to our pediatric surgery center between January 2008 and June 2022 were reviewed. All included patients were grouped according to the relationship between the tumor and collecting system according to the intraoperative findings. Group I included children with tumor infiltrating the collecting system, group II included children with tumor growing into the collecting system, and group III included children whose tumor did not involve the collecting system. The clinical features, treatments and prognosis of the patients were analyzed. RESULTS: Seventy patients were enrolled, including 20 patients with 25 sides of tumors infiltrating the collecting system in group I,10 patients with 13 sides of tumors growing into the collecting system in group II, and 40 patients in group III. There was no significant difference in patients age and gender between group I and group II. In total, 20 patients in group I and 9 patients in group II had partial response (PR) after neoadjuvant chemotherapy. In group I, 22 of 25 sides of tumors underwent NSS; in group II, 11 of 13 sides of tumors underwent NSS. During an average follow-up of 47 months, in group I, 6/20 patients relapsed and 2/20 patients died; in group II, 3/10 patients relapsed and 1/10 patient died. There was no significant difference in 4-year overall survival (OS) rate among groups I, II and III (86.36% vs. 85.71%vs. 91.40%, P = 0.902). CONCLUSIONS: To preserve renal parenchyma, NSS is feasible for children with BWT involving the collecting system. There was no significant difference in postoperative long-term OS between patients with BWT involving the collecting system and not involving the collecting system.

Urodynamic changes before and after endoscopic valve ablation in boys diagnosed with the posterior urethral valve without chronic renal failure.

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/cmH2O while the maximum detrusor pressure decreased from 95.18 ± 37.59 to 50.71 ± 21.71 cmH2O, 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.

Failure in Double-J stent inserting in laparoscopic pyeloplasty of ureteropelvic junction obstruction: the clinical features and outcomes.

BACKGROUND: Double-J (DJ) stent placement is an important procedure during laparoscopic pyeloplasty (LP). Failing to insert the DJ stent may indicate the patient was also complicated with uretero-vesical junction obstruction (UVJO), and surgeons have to change to another alternative drainage method. In the present study, we analyzed the risk factors of failure of DJ stent placement during the LP and reviewed the clinical outcomes of these challenging pyeloplasties. METHODS: We retrospectively analyzed the clinical data of patients with ureteropelvic junction obstruction (UPJO) who underwent LP in our department from January 2016 to September 2020. For patients who developed a difficult process of inserting the DJ stent, the externalized uretero-pyelostomy (EUP) stent was indwelled. Patients were finally divided into two groups: DJ group and EUP group. The primary outcomes were recurrent UPJO, postoperative uretero-vesical junction obstruction (UVJO) and complications. RESULTS: A total of 535 patients were included in the study, of which 37 patients (6.9%) failed to insert the DJ stent. Age was younger, and weight was lower (P < 0.05) in the EUP group. Within follow-up, recurrent UPJO occurred in ten (1.87%) patients, nine in the DJ group and one in the EUP group (P > 0.05). The incidence of postoperative UVJO in the EUP group was significantly higher than in the DJ group (10.8% vs. 0.2%, P < 0.01). 74 patients (13.8%) developed complications after surgery, 12 patients (32.4%) in the EUP group, significantly higher than that in the DJ group (32.4% vs. 12.4%, P < 0.01). Compared with the DJ group, the larger APD were observed in the EUP group at three months postoperatively (3.50 [3.02;4.58] vs. 2.20 [1.50;2.88], P < 0.05), but the difference vanished in further follow-up. CONCLUSION: The failure of DJ stent placement tends to occur in patients with younger age, lower weight, and larger preoperative APD. Failure may not increase the recurrent UPJO rate, but may indicate a higher probability of postoperative UVJO and may develop more postoperative complications and slower recovery.

Treatment of bilateral Wilms' tumor in children: how to improve the application of nephron-sparing surgery.

PURPOSE: To summarize the experience of nephron-sparing surgery (NSS) for bilateral Wilms tumors (BWT) in children. METHODS: This study included children with BWT admitted to our hospital between January 2008 and June 2022. The details of the treatments and outcomes were analyzed. RESULTS: In all, 70 patients (39 males and 31 females) were enrolled, including 66 patients with synchronous tumors and 4 patients with metachronous tumors. The median age at diagnosis was 13 (3-75) months. Overall, 59 patients received preoperative chemotherapy and 45.8% (54/118) of the 118 sides of WT achieved a partial response (PR). Of the 70 patients, 48 (68.6%) underwent bilateral NSS and 22 (31.4%) underwent unilateral NSS and contralateral total nephrectomy. The proportion of bilateral NSS in the preoperative chemotherapy group was significantly higher than in the non-chemotherapy group (P = 0.031). Additionally, there were 26, 25, 14, and 5 cases of stage I, stage II, stage III, and stage IV, respectively. Among the 70 children, 16 had a recurrence, and 8 died. The 4 years EFS and OS were 67.9% and 89.3%, respectively. CONCLUSIONS: The long-term survival rates of patients with BWT improved. Hence, preoperative chemotherapy should be administered to enhance the use of NSS in BWT.

Characterizations of three-dimensional root morphology and topological location of mandibular third molars by cone-beam computed tomography.

PURPOSE: This study aimed to delineate three-dimensional (3D) root morphology and topological locations of mandibular third molars (MTMs) by cone-beam computed tomography (CBCT) in a Chinese adult dental population. METHODS: Adult patients with MTMs were retrospectively screened based on CBCT images at our institution between January 2018 and December 2019. Root morphology and spatial locations of these teeth were defined based on CBCT 3D images. Potential associations with epidemiological and clinical/radiological parameters were analyzed using Chi-square or Fisher exact test. Two-tailed P values less than 0.05 were considered statistically significant. RESULTS: A total number of 2680 eligible patients (male/female:0.74; 35 ± 10 years old) with 4180 MTMs were enrolled. The majority of MTMs had 2 roots 3064 (73.30%), followed by 800 (19.14%) 1 root, 302 (7.22%) 3 roots, and 14 (0.33%) 4 roots. More than half of one-rooted MTMs were convergent, followed by club-shaped and C-shaped. Among MTMs with 2 roots, 2860 (93.34%) were M-D (mesio-distal) types. Most MTMs with 3 roots were M-2D (one root in mesial, two roots in distal) types, followed by 2M-D (two roots in mesial, one root in distal) types, and B-2L (one root in buccal, two roots in lingual) types. The presence of root configurations was significantly associated with the angulation, depth, and width classification in two-rooted MTMs (P < 0.05). CONCLUSIONS: Although the morphology and spatial locations of MTMs vary greatly, our results from a large dental population reconfirm that most MTMs have two roots with mesial-distal type of spatial distribution.

Analysis in the influence factors of urethroplasty in DSD.

BACKGROUND: At present, there is no specific research on the factors affecting the success rate of urethroplasty in patients with DSD. The purpose of this study is to explore the factors affecting the success of urethroplasty in DSD patients, and to provide some reference for the surgical treatment of DSD patients undergoing urethroplasty. METHOD: We reviewed patients with DSD who underwent urethroplasty from January 2016 to December 2019 retrospectively. Patients were divided into four groups: the successful group, the urethrocutaneous fistula group, the urethral diverticulum group, and the urethral stricture group. Risk factors were determined from the following data included the DSD classification, the age of first operation, length of urethral defect, degree of hypospadias, cryptorchidism, micropenis, gonad type, hormone therapy before operation, transposition of penis and scrotum, surgical strategy, urethral covering material, and postoperative catheter removal time. We explored the difference of each factor between four groups through the comparative study of single factor and multifactor logistic regression analysis of related factors. RESULT: 122 cases were enrolled in this group (n = 122), 12 cases were lost to follow-up. Median follow-up was 28 months (12-55 months).We found the success rate of operation decreased with longer urethral defect (B = - 0.473, P = 0.005). The success rate of operation was higher in staged operation and TPIT (TPIT = Transverse Preputial Island Tube operation)-related operation than primary operation (B = 1.238, P = 0.006) and TPIT-nonrelated operation (B = 2.293, P = 0.001). Although there was a significant difference between the age of the first operation and the occurrence of urethrocutaneous fistula (P = 0.006 < 0.05), there was no significant difference in logistic regression analysis (P = 0.161 > 0.05). The incidence of urethrocutaneous fistula was lower in TPIT-related operation than in TPIT-nonrelated operation (B = - 2.507, P = 0.000). The incidence of postoperative urethral diverticulum was lower in staged operation than in primary operation (B = - 1.737, P = 0.015). CONCLUSION: For patients with disorder of sex development undergoing urethroplasty, the length of urethral defect is an independent risk factor affecting both the success rate of operation and the urethrocutaneous fistula. The age of the first operation has a statistically significant effect on the occurrence of postoperative urethrocutaneous fistula, but it is not an independent factor. Urethrocutaneous fistula is less found in TPIT-related operation in the study. Staged operation is an independent protective factor for postoperative urethral diverticulum compared with one-stage operation but isn't related to urethrocutaneous fistula.

A multicenter study on surgical procedure selection and risk factor analysis of postoperative complications after TIP and Duckett hypospadias repair.

BACKGROUND: Hypospadias is a common congenital malformation in pediatric urology with surgery being the only curative treatment. Although there are hundreds of surgical methods for hypospadias, no single method can treat all types, and there are still high rates of postoperative complications. We performed this study to investigate surgical procedure selection and perform risk factor analysis of postoperative complications in hypospadias repair. METHODS: Retrospective analysis was performed of complete clinical and follow-up data of children with hypospadias who were treated and followed up at 15 children's clinical centers in Mainland China from December 2018 to December 2019. Children were divided into groups according to Barcat classification and surgical methods in order to analyze the surgical choice for different types of hypospadias and the influencing factors of different surgical methods for complications. RESULTS: In total, 1011 patients were followed up for 26 months. According to Barcat classification, there were 248 cases of distal type hypospadias, 214 of intermediate, and 549 of proximal type. Transverse preputial island flap urethroplasty (Duckett) and tubularized incised plate urethroplasty (TIP) were performed in 375 (37.1%) and 336 cases (33.2%), respectively. The postoperative complication rate of distal hypospadias was 23.4% (15.8-57.1%), mid shaft 29.0% (22.7-40.0%), and proximal 43.7% (30.2-52.9%). Among the 375 patients in Duckett group, 192 had complications. Multivariate logistic analysis showed that the length of prepuce island flap (OR = 3.506, 95% CI: 2.258-5.442) was an independent risk factor for complications after Duckett operation (P < 0.001). In TIP group, there were 336 cases with 84 complications. Multivariate logistic analysis showed that the width of urethral plate after longitudinal resection (OR = 0.836, 95% CI: 0.742-0.942) and glans width (OR = 0.851, 95% CI: 0.749-0.965) were independent risk factors for postoperative complications after TIP (P = 0.003, P = 0.012). CONCLUSION: Several anatomical features play a role during the selection process among the different surgical approaches, including glans size, urethral plate width, and the meatal position. The width of the urethral plate and glans width were risk factors for postoperative complications after TIP. The length of prepuce island flap was a risk factor for complications after Duckett operation.

A case report of renal calyceal diverticulum with hypertension in children and review of literature.

BACKGROUND: Renal calyx diverticulum refers to a cystic lesion covered with the transitional epithelium in the renal parenchyma. Although there is no clear evidence that calyx diverticulum can cause hypertension, there exists a close association between the two, and there are few related reports. Herein, we reported the case of a child with renal calyx diverticulum complicated with hypertension and summarized the diagnosis and treatment. CASE PRESENTATION: Physical examination of the patient, an 11-year-old child, revealed a left renal cyst with hypertension (155/116 mmHg). There were no related symptoms. Routine urine and blood biochemical examinations showed no abnormalities. Imaging revealed left renal cyst compression causing the hypertension. She underwent renal cyst fluid aspiration and injection of a sclerosing agent into the capsule, but her blood pressure increased again 3 days postoperatively. Color Doppler ultrasonography showed that the size of the left renal cyst was the same as that preoperatively. To further confirm the diagnosis, cystoscopic retrograde ureteropyelography was performed to confirm the diagnosis of renal calyx diverticulum. Subsequently, renal calyceal diverticulum resection and calyx neck enlargement were performed. The operation went smoothly and the blood pressure returned to normal postoperatively. No abnormalities were noted at the 7-month postoperative follow-up. CONCLUSION: There exists an association between renal calyx diverticulum and hypertension. Therefore, hypertension can be considered a surgical indication for renal calyx diverticulum. Moreover, renal calyceal diverticulum in children can be easily misdiagnosed as a renal cyst. Therefore, it is important to be vigilant to prevent a series of complications, such as postoperative urine leakage, in such cases.

Duplex kidney complicated with preoperative inferior nephroblastoma rupture in children: a case report and literature review.

BACKGROUND: We admitted a child with a duplex kidney combined with preoperative rupture of nephroblastoma and used this case to discuss the clinical features and treatment of this disease. CASE PRESENTATION: We retrospectively analyzed the clinical data of a 5-year-old girl with preoperative duplex kidney rupture combined with inferior nephroblastoma who was admitted to the Fourth Hospital of Baotou. In addition, we reviewed the relevant literature. The patient's details were as follows: weight, 17 kg; height, 108 cm; and body surface area, 0.7 m2. Abdominal ultrasound for abdominal pain revealed the presence of a left-sided renal mass; enhanced abdominal computed tomography further confirmed it to be a left-sided duplex kidney measuring approximately 6 × 5 × 5 cm, with a rupture originating from the lower kidney. The PubMed database was searched from 2010 to 2020 for the terms "Wilms' tumor" and "Duplex" and "Wilms' tumor" and "Rupture." The treatment plan was preoperative chemotherapy (vincristine/dactinomycin, VA regimen) + left kidney tumor radical surgery + postoperative chemotherapy (actinomycin-D/VCR/doxorubicin, AVD regimen). Postoperative pathology revealed an International Society of Pediatric Oncology intermediate-risk stage-3 nephroblastoma (mixed type) in the left kidney. Literature review was performed with 71 cases meeting the set criteria with an aim to analyze and summarize the clinical characteristics and treatment of patients with ruptured nephroblastoma and duplex kidney combined with nephroblastoma. CONCLUSIONS: To our knowledge, no previous studies have reported preoperative duplex kidney combined with nephroblastoma rupture. In patients with this condition, preoperative chemotherapy is recommended when the vital signs are stable and tumor resection can be performed after the tumor has shrunk to prevent secondary spread. If the patient's vital signs are unstable, emergency exploratory surgery is needed. If the nephroblastoma rupture is old and limited, surgery can be performed when the tumor size is small.

Testis-Preserving Tumor Enucleation Is Applicable in Children with Immature Testicular Teratoma.

INTRODUCTION: This study investigated the biological characteristics of immature testicular teratoma in children and explored the feasibility of testis-preserving tumor enucleation. METHODS: We retrospectively reviewed the cases of 23 children who received a pathologic diagnosis of immature testicular teratoma between January 2005 and December 2018. Ages ranged from 16 days to 13 months (mean: 6 months and 5 days). Painless testicular enlargement was the main clinical manifestation, and the course of disease ranged from 20 days to 4 months (mean: 1.4 months). The tumor volume ranged from 1.5 × 1.2 × 0.5 to 6 × 5 × 4.5 cm. Elevated levels of alpha-fetoprotein were measured in 21 patients. Preoperative ultrasound examination showed a cystic/solid mass with calcification. RESULTS: Excision of the affected testis was done in 10 patients and testis-preserving tumor excision in 13 patients. Postoperative chemotherapy was not employed. Nineteen patients were followed up for 1-10 years, and all showed disease-free survival without recurrence or metastasis. CONCLUSION: Immature testicular teratoma is found predominantly in children aged <1 year, and its biological characteristics are different from those in adults. Immature testicular teratoma is largely benign in children and can be managed by testis-preserving tumor enucleation, as for other benign tumors (such as mature teratoma). Postoperative monitoring and follow-up are necessary.

LINC00667 promotes Wilms' tumor metastasis and stemness by sponging miR-200b/c/429 family to regulate IKK-ß.

Wilms' tumor, also known as nephroblastoma, is a kind of pediatric renal cancer. Previous studies have indicated that microRNAs (miRNAs) regulate various cancers progression. However, whether miR-200 family regulated Wilms' tumor progression remains to be elucidated. In our study, miR-200b/c/429 expression was downregulated in Wilms' tumor tissue samples from 25 patients. And data from three independent analyses of quantitative real-time polymerase chain reaction revealed that the expression of miR-200b/c/429 was downregulated in Wilms' tumor cell lines. Functionally, Cell counting kit-8 assay revealed that cell viability was reduced by overexpressing miR-200b/c/429. Transwell assay manifested that cell migration and invasion was hindered by miR-200b/c/429 overexpression. Sphere-forming and western blot assays demonstrated that miR-200b/c/429 overexpression suppressed the sphere formation ability. Mechanically, nuclear factor-κB (NF-κB) pathway was confirmed to be associated with Wilms' tumor progression; miR-200b/c/429 overexpression inactivated NF-κB pathway as miR-200b/c/429 was identified to target IκB kinase ß (IKK-ß), an NF-κB pathway-related gene. Moreover, miR-200b/c/429 was sponged by LINC00667 in Wilms' tumor cells. LINC00667 competitively bound with miR-200b/c/429 to regulate IKK-ß expression and then activated NF-κB pathway in Wilms' tumor. Subsequently, rescue assays illustrated that silencing of IKK-ß could reverse the effect of miR-200b/c/429 inhibition on the progression of sh-LINC00667-transfected Wilms' tumor cells. In summary, LINC00667 promoted Wilms' tumor progression by sponging miR-200b/c/429 family to regulate IKK-ß.

Staged transverse preputial island flap urethroplasty for proximal hypospadias: a single-center experience.

PURPOSE: To evaluate the intermediate outcomes of our institution's experience with staged TPIF urethroplasty for proximal hypospadias repair. METHODS: We retrospectively evaluated the medical records of patients who underwent repair of proximal hypospadias using staged TPIF urethroplasty at our hospital from 2011 to 2017. RESULTS: One hundred and two patients were included in the present study. The mean follow-up was 52.4 months (range 13-74 months). The mean age at the time of the first surgery was 13.5 months (range 11-65 months). There were two main types of initial complications including meatal stenosis in four (3.9%) and urethrocutaneous fistula in three (2.9%) patients after the first stage. Surgical complications were seen in 15 patients after second stage, including urethrocutaneous fistulas in 8 (7.8%), urethral strictures in 5 (4.9%), urethral diverticula in 2 (1.9%). Overall complication rates after second stage were 14.7%. The incidence of fistulas was lower in patients who underwent repair with a tunica vaginalis flap (1/29, 3.4%) than with the dartos fascia (7/73, 9.6%; p = 0.435). CONCLUSIONS: Our results show that staged TPIF urethroplasty is a viable and durable technique for primary severe proximal hypospadias. This procedure was associated with a 14.7% complication rate in the present study. Staged TPIF urethroplasty can reduce the incidence of urethral strictures and diverticula associated with the second stage.

Ureteropelvic junction obstruction with polyps in children: clinical manifestations and supranormal preoperative differential renal function.

OBJECTIVE: To describe and analyze the clinical manifestation and pre-DRF of UPJO children with polyps and explore the possible influencing factors of supranormal pre-DRF. PATIENTS AND METHODS: All patients undergoing primary Anderson-Hynes pyeloplasty for UPJO due to polyp were retrospectively reviewed. Patients' characteristics, parameters of ultrasound and dynamic renograms (DR) were recorded in elaborate. Pre-DRF in groups of different age, weight, gender, pain, grade of hydronephrosis, anterio-posterior pelvic diameter (APD), length of kidney and postoperative ultrasonic parameters were compared. RESULTS: A total of 18 UPJO children with polyps were included. Five (27.78%) patients had SFU III grade of hydronephrosis. Seven (38.89%) patients were supranormal pre-DRF. All patients had pre-DRF > 40%. Drainage curve was delayed excretion in 12 (66.67%) patients and T1/2 < 20 min was in 4 (22.22%) patients. Among the 16 patients who underwent preoperative IVP examination, 15 (93.75%) patients had concentration of intrarenal pelvis contrast agent within 10 min. No significant difference in post-APD reduction rate and post-minPT increased was found between supranormal pre-DRF and non-supranormal pre-DRF groups. The supranormal pre-DRF was more likely to occur in young and low-weight children. CONCLUSION: The preoperative renal function of UPJO patients with polyps was well preserved, and 38.89% of them had supranormal pre-DRF. Patients with supranormal pre-DRF can be managed with the same strategies as those with normal renal function.

Urodynamic Abnormalities in Children with Primary Bilateral Vesicoureteral Reflux.

BACKGROUND: Bilateral vesicoureteral reflux (VUR) has been associated with poor long-term prognosis, and research focusing exclusively on its effect on urinary bladder function is lacking. This study aims to assess the urodynamic characteristics of children with bilateral VUR and identify factors influencing renal function. METHODS: A retrospective analysis was performed on children diagnosed with bilateral VUR who underwent urodynamic examination in the outpatient department of our institution from January 2019 to January 2023. All patients underwent comprehensive assessments, including voiding cystourethrography, urinary system ultrasound and urodynamic examination, to analyse the urodynamic features associated with different clinical characteristics. RESULTS: The study included 148 children with bilateral VUR, amongst whom 92 (62.2%) exhibited urodynamic abnormalities, including idiopathic detrusor overactivity (DO) in 30 cases (20.3%) and dysfunctional voiding (DV) in 58 cases (39.2%). DV was prevalent in children under 5 years of age, whereas idiopathic DO was predominantly observed in children older than 2 years of age. Children older than 5 years of age exhibited a trend towards reduced bladder volume, whereas those younger than 2 years of age often had increased bladder volume and residual urine. Multivariate analysis identified bilateral severe VUR as an independent risk factor for abnormal estimated glomerular filtration rate. CONCLUSIONS: DV tends to be more common in younger children with larger bladder volumes and increased residual urine than in older children. By contrast, older children are more susceptible to idiopathic DO associated with smaller bladder volumes than younger children. Bilateral severe reflux increases the likelihood of renal functional abnormalities, which appear to be a consequence of congenital scarring rather than abnormalities in bladder function.

Gender determination and long-time follow-up analysis of mixed gonadal dysgenesis.

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 + one-sided gonadectomy. RESULTS: Patient follow-up started in the third month after surgery. Female patients reported no surgery-related 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.

Nomogram for personalized prognostic assessment of children with favorable histology Wilms tumor: A retrospective analysis.

OBJECTIVE: This retrospective study aimed to construct and validate a nomogram for personalized prognostic assessment of favorable histology Wilms tumor (FHWT) based on clinical and pathological variables. METHODS AND MATERIALS: This was a retrospective study collected data from patients who underwent surgery for FHWT between March 2007 and November 2022 at Beijing Children's Hospital. Univariate and multivariate Cox proportional hazards regression analyses were conducted to determine the significance variables and constructed the nomogram in predicting event-free survival (EFS) in FHWT patients. RESULTS: A total of 401 FHWT patients were included in the study, with the median age of the patients was 3.4 years. The overall 1-, 3-, and 5-year OS rates were 98.2%, 96.3%, and 93.9%. The 1-, 3-, and 5-year EFS rates were 91.2%, 88.2%, and 86.6%. Subgroup analysis revealed age greater than 2 years was associated with a worse prognosis than age less than or equal to 2 years (P < 0.001), and patients with high-risk Wilms tumors were associated with a higher rate of recurrence and death (P < 0.001). Multivariate analysis showed that age (HR: 2.449, 95%CI: 1.004-5.973), stage (HR: 1.970, 95% CI:1.408-2.756), and histological risk (HR:9.414, 95% CI: 4.318-20.525) were identified as independent predictors of EFS (P < 0.05) and used to construct the nomogram. The prognostic nomogram demonstrated good calibration, great clinical utility, and the time-dependent receiver operating curve analysis showed that the nomogram had precise predictability, with area under the curve values of 0.85(95CI:0.796-0.913), 0.85(95CI:0.80-0.91), and 0.88(95CI:0.839-0.937) for 1-,3-year and 5-year EFS. CONCLUSION: This study provides valuable insights into the clinical characteristics and outcomes of FHWT patients. Accurate staging and histological risk assessment are important in predicting outcomes, and the prognostic nomogram we developed can be a useful tool for clinicians to assess patient prognosis and make informed treatment decisions.

Assessment of clinical prognosis improvement in children with concomitant anterior and posterior urethral valves: A case series report.

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.

Expression and clinical implications of PARs in the stenotic tissue of ureteropelvic junction obstruction.

Objective: To explore the expression and clinical implications of protease activated receptors (PARs) in the pathogenesis of children with ureteropelvic junction obstruction (UPJO). Material and methods: Immunohistochemistry was employed to investigate the distribution of PARs in both normal human ureteropelvic junction (UPJ) and cases of UPJO. Furthermore, PAR gene expression levels were assessed using real-time PCR (RT-PCR), and the patients in the UPJO group were stratified according to the Onen grading system. Subsequently, the clinical implications of PARs in UPJO were explored through RT-PCR analysis. Results: Immunofluorescence showed robust PAR2 expression in the control group compared with the UPJO group. The results of RT-PCR analysis revealed a significant decrease in the relative mRNA expression of PAR2 in the UPJO group compared to the control group. Notably, the relative RNA expression of PAR1 was significantly lower in the Onen-4 group compared to the control group. Furthermore, the relative mRNA expression of PAR2 exhibited a statistically significant difference among the Onen-3 group, Onen-4 group, and control group. Conclusions: PARs are widely distributed throughout the SIP syncytium of the UPJ and play a role in maintaining smooth muscle cells (SMCs) membrane potential by interacting with interstitial cells of Cajal (ICCs), as well as platelet-derived growth factor receptor alpha-positive cells (PDGFR α+ cells). The decreased expression of PAR1 suggests a higher preoperative Onen grade in UPJO patients. Furthermore, the downregulation of PAR2 effects at the UPJ may be involved in the loss of inhibitory neuromuscular transmission, disrupting the rhythmic peristalsis of the UPJ.