Ureteropelvic Junction Obstruction Research Articles

Comparison of the safety and efficacy of laparoscopic single-incision triangulated umbilical surgery pyeloplasty with traditional three-hole surgery in a pediatric tertiary center.

To report the application of laparoscopic single-incision triangulated umbilical surgery (SITUS) pyeloplasty in children with ureteropelvic junction obstruction (UPJO) and compare its feasibility and efficacy with traditional three-hole laparoscopic pyeloplasty. Data from children with UPJO who underwent SITUS between July 2018 and August 2021 were included in this retrospective study and patients who were treated with traditional laparoscopic pyeloplasty were chosen for comparison. Thirty-two patients from SITUS group and 72 patients from traditional group were reviewed. The clinical characteristics, complications, and follow-up results were collected and compared. The preoperative demographic data and imaging parameters, including sex, surgical side, age, BMI, and preoperative anterior-posterior pelvic diameter (APD), showed no significant differences between the two groups. The median surgical time was 135 min (IQR: 119.75-160.5) in SITUS group, while 163.5 min (IQR: 141.25-187.5) in the traditional group (p = 0.0008). Two Clavien-Dindo III complications (6.25%) in SITUS group and 11 (15.3%) in the traditional group were recorded (p = 0.335). The success rate was 100% (32/32) in SITUS group and 94% (68/72) in the traditional group (p = 0.309). SITUS pyeloplasty is a feasible and effective laparo-endoscopic single-site (LESS) technique for pediatric patients with excellent cosmetic results comparable to the conventional laparoscopic pyeloplasty. More cases and longer follow-up periods are needed to determine the exact outcomes of the modified LESS technique.

Robot-assisted laparoscopic Anderson–Hynes pyeloplasty for ureteropelvic junction obstruction

ObjectivesTo explore surgical, functional, and symptomatic outcomes in a series of patients who underwent robot-assisted laparoscopic Anderson–Hynes pyeloplasty (RALP) for ureteropelvic junction obstruction using the DaVinci Si surgical robotic system.MethodsRetrospective study including patients aged 16 years or older who underwent RALP from June 2016 to December2021. The following outcomes were recorded: operative outcome and complications [classified according to the Clavien–Dindo Classification (CD)] within 30 days of the procedure as well as 1 year success rate and restenosis during follow-up.ResultsIn total, 194 patients were available for analyses with a median follow-up of 4.5 (IQR 3.0-6.0) years. The primary indications were loss of kidney function (45%), pain (36%), infection (11%), kidney stone (6%), and others (2%). The median operation time was 134 min (IQR 112-159), the median length of stay was 2 days (IQR 2-2), and the median time with double-j stent postoperatively was 24 days (IQR 22-27). Overall, 65 out of 194 patients (33%) experienced a postoperative complication (12% CD I, 13% CD II, 8% CD IIIa or IIIb). The 1 year success rate was 92% for patients treated because of deteriorating renal function, 78% for patients treated because of symptoms, 82% for patients treated because of infections, and 78% for patients treated because of kidney stones. Seven percent of the patients presented a recurrent ureteropelvic junction stricture during follow-up.ConclusionsIn our experience, robot-assisted laparoscopic Anderson–Hynes pyeloplasty performed with the DaVinci Si system is a safe with a few major complications and acceptable success rate.

Application of different CO2 pneumoperitoneum pressure in laparoscopic pyeloplasty for infants with ureteropelvic junction obstruction.

Laparoscopic pyeloplasty is a minimally invasive approach for the therapy of infant ureteropelvic junction obstruction (UPJO), reliant on CO2 pneumoperitoneum insufflation. While the impact of CO2 insufflation on adult and older pediatric populations has been studied, its effects on infants remain less explored. This prospective randomized controlled trial included infants with UPJO undergoing laparoscopic pyeloplasty. Patients were allocated to low pneumoperitoneum pressure (LPP, 5 mmHg) or high pneumoperitoneum pressure (HPP, 8 mmHg) groups. Surgical parameters, postoperative complications, acid-base balance, stress markers, inflammatory cytokines, and oxidative stress markers were evaluated and compared. A total of 116 infants were analyzed. Preoperative characteristics were comparable between LPP and HPP groups. No significant differences in blood loss, operation time, or hospitalization time were observed. Postoperative complications were similar between groups. Acid-base balance analysis revealed a decrease in pH after pneumoperitoneum in both groups, with greater reductions in actual base excess and standard base excess in the HPP group. Stress markers, cytokines, and oxidative stress markers increased postoperatively in both groups, with higher levels in the HPP group. HPP leads to more pronounced physiological responses, including acid-base alterations, stress reactions, and inflammatory cytokine elevations.

Ectopic pelvic kidney associated with uretero-pelvic junction obstruction: a case report

Ectopic pelvic kidney associated with uretero-pelvic junction obstruction: a case report

Role of Interstitial Cells of Cajal in Congenital Ureteropelvic Junction Obstruction.

Although congenital ureteropelvic junction (UPJ) obstruction is the most common cause of neonatal hydronephrosis, aetiopathogenesis is still inconclusive. Recently, the paucity of interstitial cells of Cajal (ICC) at the narrow adynamic part of UPJ has been implicated as a causative factor. This prospective study was conducted between October 2019 and March 2022 to find out the density of ICC by the immunohistochemical method using CD117 (c-kit) antibody, in resected segments of UPJ in obstruction patients and in renal tumour patients as control. ICC/high power field (hpf) was also studied from the margins of the resected segment in the obstruction group. The pre-operative and post-operative sonographic and renal scintigraphic features were compared. The median age of patients in the study group (n = 25) was 36 months and in the control group was 39 months. The mean ICC/hpf at the stenotic part of UPJ in the study group was 3.56 ± 1.26 and in the control group was 12.56 ± 1.89 (P = 0.0001). ICC density from the proximal and distal margins of the resected segment was 11.12 ± 2.12 and 11.68 ± 1.62, respectively (P < 0.001). The post-operative antero-posterior diameter of the renal pelvis and differential renal function showed significant improvement in comparison to the pre-operative value (P = 0.0045 and 0.0005, respectively). The significant decrease in the density of ICC at the stenotic part of UPJ compared to controls suggests a pacemaker role of these cells in ureteral peristalsis and the aetiopathogenesis of UPJ obstruction. Histopathological analysis of ICC should not only be limited to the stenotic part of UPJ but also should focus on the anastomosed ends of the ureter, which reflects post-pyeloplasty outcome.

Initial Experience of Robot Assisted Laparoscopic Pyeloplasty for Ureteropelvic Junction Obstruction Using the Hinotori Surgical Robot System.

This study aimed to investigate the perioperative outcomes of robot-assisted laparoscopic pyeloplasty (RLP) using the recently launched hinotori surgical robot system. This retrospective study compared the perioperative outcomes of 11 consecutive patients who underwent RLP with the hinotori surgical robot system from October 2022 to March 2024 (hinotori group) and 30 consecutive patients who underwent RLP with the da Vinci system from March 2019 to September 2022 (da Vinci group). The patient characteristics of the groups were similar. The median operative times in the hinotori and da Vinci groups were 236.0 and 231.5min, respectively (p=0.480). The success rates were 100.0% and 96.7%, respectively (p=1.000). Clavien-Dindo grade ≥3 complications occurred in one patient (9.1%) in the hinotori group and one patient (3.3%) in the da Vinci group (p=0.470). The perioperative outcomes in the hinotori group were not inferior to those in the da Vinci group.

Unsupervised Machine Learning to Identify Risk Factors of Pyeloplasty Failure in Ureteropelvic Junction Obstruction.

Introduction: In adult patients with ureteropelvic junction obstruction (UPJO), little data exist on predicting pyeloplasty outcome, and there is no unified definition of pyeloplasty success. As such, defining pyeloplasty success retrospectively is particularly vulnerable to bias, allowing researchers to choose significant outcomes with the benefit of hindsight. To mitigate these biases, we performed an unsupervised machine learning cluster analysis on a dataset of 216 pyeloplasty patients between 2015 and 2023 from a multihospital system to identify the defining risk factors of patients that experience worse outcomes. Methods: A KPrototypes model was fitted with pre- and perioperative data and blinded to postoperative outcomes. T-test and chi-square tests were performed to look at significant differences of characteristics between clusters. SHapley Additive exPlanation values were calculated from a random forest classifier to determine the most predictive features of cluster membership. A logistic regression model identified which of the most predictive variables remained significant after adjusting for confounding effects. Results: Two distinct clusters were identified. One cluster (denoted as "high-risk") contained 111 (51.4%) patients and was identified by having more comorbidities, such as old age (62.7 vs 35.7), high body mass index (BMI) (26.9 vs 23.8), hypertension (66.7% vs 17.1%), and previous abdominal surgery (72.1% vs 37.1%) and was found to have worse outcomes, such as more frequent severe postoperative complications (7.2% vs 1.0%). After adjusting for confounding effects, the most predictive features of high-risk cluster membership were old age, low preoperative estimated glomerular filtration rate (eGFR), hypertension, greater BMI, previous abdominal surgery, and left-sided UPJO. Conclusions: Adult UPJO patients with older age, lower eGFR, hypertension, greater BMI, previous abdominal surgery, and left-sided UPJO naturally cluster into to a group that more commonly suffers from perioperative complications and worse outcomes. Preoperative counseling and perioperative management for patients with these risk factors may need to be thought of or approached differently.

Predictive Value of the Postural Difference in Antero-Posterior Diameter of Renal Pelvis on Ultrasonography of Unilateral Ureteropelvic Junction Obstruction in Determining the Need For Surgery.

Ureteropelvic junction obstruction (UPJO) is the most common cause of antenatal hydronephrosis. Although majority of them improve with time, none of the existing diagnostic modalities can accurately predict which hydronephrotic kidney is at the risk of progressive renal damage and will benefit from early surgery. Postural variations in the anteroposterior pelvic diameter (APPD) of the hydronephrotic kidney in children during follow-up postnatal ultrasonography (USG) reflect the intrapelvic tension, which might help in predicting the need of surgery amongst these patients. We designed this prospective observational study in all unilateral UPJO patients on postural variation in the APPD of renal pelvis on ultrasonography. The mean age of all patients were 2.15 years (0-5 years) and managed at our institute at All India Institute of Medical Sciences, Bhubaneswar. The study duration was from July 2019 to May 2021. The management of these patients was done as per the standard institutional protocol and there was no deviation due to inclusion in this study. We documented the variations in postural APPD both amongst the conservatively managed group and the surgically managed group of patients. Linear correlation between two continuous variables was explored using Pearson's correlation (if the data were normally distributed) and Spearman's correlation (for non-normally distributed data). We found a higher prone APPD than supine APPD in all these patients indicating the obstruction at ureteropelvic junction. However, in the surgical group, there was less variation in the postural APPD compared to the conservative group, and when there was no variation in the postural APPD, the need of surgery was 100%. The limitation of our study was the small sample size (n = 36). A study involving a larger population or involving multiple institutions may further add significance to our findings. We found less postural variation in APPD on USG to be more likely associated with severe UPJO requiring early surgery. This may indicate a non-compliant renal pelvis. However, it was statistically not significant.

Robot‐assisted pyeloplasty for ureteropelvic junction obstruction complicating horseshoe kidney

IntroductionHorseshoe kidney is characterized by midline fusion of the lower poles of both kidneys. We report a case where robot‐assisted pyeloplasty was performed for hydronephrosis complicating horseshoe kidney.Case presentationA 31‐year‐old man repeatedly developed fever since childhood. He visited a local clinic for fever and left back pain, where he was diagnosed and treated for acute pyelonephritis. Abdominal computed tomography led to a diagnosis of horseshoe kidney and associated left ureteropelvic junction obstruction. He was referred to our hospital for pyeloplasty because of persistent left back pain. Robot‐assisted left pyeloplasty was performed using the Anderson–Hynes technique with four ports because crossing vessels were discovered intraoperatively. Isthmusectomy was not performed. The postoperative course was favorable. Computed tomography performed approximately 2 years post‐surgery showed improvement in hydronephrosis. No left back pain was reported.ConclusionOur case experience suggests the utility of robot‐assisted pyeloplasty for patients with horseshoe kidney.

Primary pyeloplasty for uretero-pelvic obstruction in the USA adult population with or without double-J indwelling ureteral stents. Insurance claims data on contemporary time to removal trends, perioperative complications, health care costs, and re-intervention rates.

Using a large population-based dataset, we primarily sought to compare postoperative complications, health-care expenditures, and re-intervention rates between patients diagnosed with ureteropelvic junction obstruction (UPJO) undergoing stented vs. non-stented pyeloplasty. The secondary objective was to investigate factors that influence the timing of DJ stent removal. Patients ≥18 years old with UPJO treated with primary open or minimally-invasive pyeloplasty were identified using the Merative™ Marketscan® Databases between 2007-2021. Multivariable modeling was implemented to investigate the association between Double-J (DJ) stent placement and post-pyeloplasty complications, hospital costs, and re-intervention rates and the role of the perioperative predictors on time to DJ stent removal. Subgroup analyses stratified by ureteral stenting duration were additionally performed. Out of 4872 patients who underwent primary pyeloplasty, 4154 (85.3%) had DJ placement. Postoperative complications were rare (N.=218, 4.47%) and not associated with ureteral stenting (odds ratio [OR]: 0.78, 95% confidence interval [CI]: 0.55-1.12). The median cost for in-hospital charges was $21,775, with DJ stent placement independently increasing the median aggregate amount (OR: 1.29, 95% CI: 1.09-1.53). Overall, re-interventions were performed in 21.18% of patients, with DJ stenting found to be protective (OR: 0.79, 95% CI: 0.66-0.96). Higher Charlson Comorbidity Index, longer hospital stay, and open surgical approach were independent predictors for prolonged DJ stenting time to removal. Our study suggests that patients undergoing stent-less pyeloplasty did have a higher rate of secondary procedures, but not higher complications when compared to those undergoing stented procedures. Concurrently, the non-stented approach is associated with decreased health-care expenditures, despite the increased rates of secondary procedures.

The Optimal Suture Bite Depth in Laparoscopic Pyeloplasty: A Comparative Study in Children.

Background: Modified Anderson-Hynes pyeloplasty is currently preferred for ureteropelvic junction obstruction (UPJO). Extravasation of urine and anastomotic stenosis are the most common complications after Anderson-Hynes pyeloplasty, which are closely linked with the technique for anastomosis. However, there are currently no clear guidelines for the suture bite depth in suturing the anastomosis during pyeloplasty. Objective: To analyze the optimal suture bite depth in laparoscopic Anderson-Hynes pyeloplasty. Study Design: A total of 90 children aged 4-14 years with UPJO-induced hydronephrosis who were surgically treated in the First People's Hospital of Lianyungang from July 2019 to July 2022 were prospectively recruited. All received laparoscopic Anderson-Hynes pyeloplasty using 5-0 Vicryl continuous sutures. According to the suture bite depth, the patients were divided into group A (depth 1 mm, n = 46) and group B (depth 0.5 mm, n = 44). Operation time, postoperative drainage volume, time of ureteral stent removal, incidence of postoperative complications, and time to hydronephrosis resolution were compared between groups. Results: Group A showed significantly less postoperative drainage volume, and shorter time of ureteral stent removal and hydronephrosis resolution (all P < .05). Four cases in group B received replacement of a double-J stent. Except for 1 patient receiving reoperation for anastomotic stenosis caused by massive extravasation of urine, the replaced double-J stent was successfully removed from the remaining 3 patients at 3 months, and the symptoms of anastomotic stenosis disappeared. No significant difference was detected in the operation time between groups (P > .05). Conclusion: An appropriate deeper suture bite depth for anastomosis may reduce postoperative urine extravasation and related complications in children who received laparoscopic pyeloplasty for UPJO-induced hydronephrosis.

PE007 Robotic-assisted laparoscopic pyeloplasty for ureteropelvic junction obstruction: A retrospective, high volume UK multi-centre review

PE007 Robotic-assisted laparoscopic pyeloplasty for ureteropelvic junction obstruction: A retrospective, high volume UK multi-centre review

Comparison of outcomes of open, laparoscopic and robot-assisted laparoscopic pyeloplasty in children with ureteropelvic junction obstruction: protocol for a systematic review and meta-analysis

IntroductionThe treatment of children with pelviureteric junction obstruction (PUJO) has naturally progressed from open, to minimally invasive approaches, including laparoscopic pyeloplasty and robot-assisted laparoscopic pyeloplasty (RALP). The RALP is now...

Comparative Study between Continuous and Interrupted Suturing in Laparoscopic Pyeloplasty

Background: Ureteropelvic junction obstruction (UPJO) is a common cause of symptomatic ureteral obstruction. Dismembered pyeloplasty is the most commonly employed procedure for the repair of UPJO. Both interrupted and continuous suturing is being practiced in laparoscopy. As satisfactory anastomosis determines the success of dismembered pyeloplasty and recovery of renal function, the success rate and the complications associated with the suturing technique need to be evaluated. Objective: To compare the outcome between continuous and interrupted suturing in laparoscopic pyeloplasty. Methods: This hospital-based, quasi-experimental study was done in the Department of Urology, Dhaka Medical College Hospital (DMCH), Dhaka and the National Institute of Kidney Diseases &amp; Urology (NIKDU), Dhaka from July 2022- June 2023. A total of 60 patients were purposively selected for the study. According to the suture method for ureteropelvic anastomosis, patients were divided into two groups: 30 patients (group A) underwent surgery with the continuous suture method, and 30 patients (group B) underwent surgery with the interrupted suture method. Data on suturing time, post-operative drain output, post-operative hospital stay and post-operative complications were collected. Patients were followed up at 01, 03 and 06 months. At 06 months, a DTPA renogram with split GFR was done to evaluate the patients. Results: The mean ages were 30.0 (±8.3) years for Group A and 33.9 (±9.3) years for Group B, with males comprising 56.7% and 63.3% of each group, respectively. Group B had significantly higher mean suturing time, post-operative drain output, and hospital stay compared to Group A (p&lt;0.001). No significant differences in GFR were noted between the groups (p&gt;0.05). Post-operative complications were observed in 10.0% of Group A and 23.3% of Group B (p=0.166). Conclusion: Continuous suturing technique for pyeloplasty is found to have a lower mean suturing time, mean drain output and post-operative hospital stay.

Value of Urine Neutrophil Gelatinase Associated Lipocalin for Prediction of Ureteropelvic Junction Obstruction in Children

Background: Congenital obstructive nephropathy is a significant cause of chronic kidney damage in childhood. Therefore, early diagnosis and management of urinary tract obstruction are necessary to prevent or reduce further renal damage. Various biomarkers have been investigated for the early identification of urinary tract obstruction. Objectives: The aim of this study was to evaluate the excretion of urine neutrophil gelatinase-associated lipocalin (Urine neutrophil gelatinase-associated lipocalin (uNGAL) &amp; uNGAL/Cr) as a useful, sensitive, and non-invasive diagnostic biomarker in children with ureteropelvic junction obstruction (UPJO). Methods: The study cohort consisted of 47 children with obstructive UPJO and 47 healthy children with normal renal ultrasounds. Ultrasonography and 99mTc DTPA radionuclide scans were used for diagnosing UPJO. All affected children had normal renal function based on GFR measurements. Urine neutrophil gelatinase-associated lipocalin was measured using Immunoenzymatic ELISA commercial kits. Results: Urine neutrophil gelatinase-associated lipocalin &amp; uNGAL/Cr levels were significantly higher in children with obstructive UPJO compared to the healthy control group. Additionally, both biomarkers demonstrated acceptable sensitivity and specificity for predicting for predicting UPJO. Conclusions: Based on these results, measuring uNGAL &amp; uNGAL/Cr is suggested for evaluating children with obstructive UPJO.

Electrochemical Detection of Neutrophil Gelatinase-Associated Lipocalin (NGAL) in Simulated Urine Using Au@Si/Ni-Porphyrin Nanocomposite for the Prediction of Kidney Injury

Kidney Injury is multifactorial out of which ureteropelvic junction obstruction is one of the major causes of concern. There are a multitude of methods for monitoring the deterioration of renal function like serum creatinine levels, ultrasound scans, renograms, and nuclear scans like radiolabelled MAG3 and DMSA which require repeat evaluation or sedation during the process. Many of the biomarkers utilized for the assessment of kidney injury are expressed at the later stages. Urine NGAL is a promising biomarker for risk stratification which is reported to be expressed at the onset of ureteropelvic junction obstruction (UPJO) and is a gold standard for the estimation of tubular injury. Urinary NGAL is also reported to be a specific marker for the prediction of hydronephrosis in children who require surgical intervention. The kidney injury cut-off for NGAL in urine is 185ng/mL and 48 ng/mL in the adult and pediatric population groups respectively. Electrochemical detection of NGAL in patient urine is a fast, reliable, non-invasive, accurate, and cost-effective method for monitoring kidney health to segregate patient groups that require surgical intervention.In this study, an electrochemical sensor was designed with Au(150nm) electrodes fabricated using thermal evaporation on a flexible substrate (PET; 250μm) by using Cr(20nm) as the seed layer. The electrode was immobilized with Au@Si core-shell decorated on Ni-porphyrin nanosheets functionalized with anti-NGAL using covalent chemistry. The nanocomposite was synthesized through a bottom-up approach where the Ni-tetrakis(4-carboxyphenyl) porphyrin nanosheets were synthesized using the solvothermal method in an organic phase (DMF) followed by purification and suspension in ethanol medium. The Si nanocrystals were synthesized in a citrate medium with APTMS as the precursor followed by purification via dialysis. The purified Si nanocrystals (60ppm) and Ni-porphyrin nanosheets (880ppm) were treated in chloroauric acid (0.25mM) and the reducing agent trisodium citrate (34mM) to form Au shell. The physicochemical and electronic properties of the synthesized nanocomposite were evaluated through XRD, XPS, microscopic analysis(TEM, SEM), AFM, UV-Vis, FTIR, and Raman spectroscopy. The resultant nanocomposite was purified and linked to anti-NGAL (100μg/mL) through EDC (0.4M)/NHS (0.1M) chemistry. The antibody-nanocomposite was immobilized on the fabricated Au electrodes (working electrode surface area: 0.35mm2) through BSA(1.2mM)/glutaraldehyde (2.5% v/v) crosslinking. The quantitative electrochemical estimation of NGAL antigen spiked in 0.1M PBS (pH=7.2±0.2) and simulated urine was performed using cyclic voltammetry (CV) and differential pulse voltammetry (DPV). The shelf life of the fabricated sensors was evaluated for 30 days (stored at 4˚ C until use) over the concentration range of 0-5000 ng/mL. The amount of test solution required per measurement for the miniaturized electrodes was 6μL.The sensing mechanism of the electrochemical platform was established through CV by varying the scan rate (0.05 V/s - 2.5 V/s) for 500ng/mL in 0.1M PBS and urine. With increasing scan rate a linear relationship between log ip and log ν was observed. For 0.1M PBS, the process was largely capacitive at various detected potentials (b=0.94 @ 0.5V; b=0.86 @ 0.3V; b=0.85 @ 0.07V). However, in the case of simulated urine, the electrochemical process was diffusion-limited at 0.6V (b=0.49). Therefore, to obtain higher current sensitivity in the diffusion-limited regime, DPV was adopted. With increasing concentration of antigen NGAL, a monotonic decrease in the redox current at 0.6V and 0.45V potential was observed. This signifies that the presence of NGAL antigen leads to the formation of antigen-antibody complex immobilized on the surface of the nanocomposite translating into an increase in the resistance at the electrode surface leading to a decrease in the redox current. The decrease in the current was proportional to the increase in the amount of the test antigen. The sensitivity of the sensor at 0.45V and 0.6V was 2.22 nA/ng mL-1/mm2 and 0.51 nA/ng mL-1/mm2 respectively with the lowest limit of NGAL detection as 12.5ng/mL in simulated urine by using DPV. Similarly, for CV the sensitivity of the sensor at 0.56V was 3.48 nA/ng mL-1/mm2 with 22ng/mL as the limit of detection. The sensor performance was evaluated without the use of nanocomposite (only antibody) as a control for comparison.Therefore, this study is a proof of concept for an effective electrochemical sensor platform design for urinary NGAL detection using a novel nanocomposite. It was established that the use of nanocomposite provides a better surface area to volume ratio through the core-shell nanostructures decorated on the nanosheets translating into higher electrochemical activity. The sensing platform was found to be specific, sensitive, and stable making it a perfect candidate for a point-of-care-testing(POCT) platform. Figure 1

Variable phenotype and genotype of pediatric patients with HNF1B nephropathy.

Hepatocyte nuclear factor 1β (HNF1B) mutations are the most common monogenic cause of congenital anomalies of the kidney and urinary tract (CAKUT). We aimed to investigate clinical and genetic characteristics of patients with HNF1B nephropathy to expand its phenotypic and genetic spectrum. This retrospective cohort study included 16 unrelated pediatric patients (6 females, 10 males) from 13 families with genetically confirmed HNF1B-related nephropathy. Abnormal prenatal kidney abnormalities were present in 13 patients (81.3%). The most common antenatal kidney abnormality was kidney cysts, which were observed in 8 patients (61.5%). Urinary system abnormalities (vesicoureteral reflux (VUR) and ureteropelvic junction obstruction (UPJO)) were present in 4 patients (25%). HNF1B analysis uncovered missense variants in 4 families (30.8%) as the most common genetic abnormality. In addition, 4 novel pathological variations have been defined. During follow-up, hypomagnesemia and hyperuricemia were observed in 7 (43.8%) and 5 patients (31.3%), respectively. None of the patients with a missense variant had hypomagnesemia. However, 7 out of 12 patients (58.3%) with a non-missense variant had hypomagnesemia (p=0.09). None of the patients had an HNF1B score below 8, and the mean score was 15.3±4.4. The mean follow-up period was 7.4±5.0 years. While 100% of patients (n=4) with missense variants were in various stages of CKD (CKD2: 2 patients, CKD3: 2 patients), 25% of those with non-missense variants had CKD (CKD2, 3, and 5; 1 patient, respectively) (p=0.026). Patients with HNF1B-associated disease have concomitant urinary system abnormalities such as VUR or UPJO. Missense variants seem to be the most common pathological variations in HNF1B gene and have higher risk of CKD.

Role of Ultrasonography and Dynamic Renal Scintigraphy Parameters in Decision Making Regarding Performance of Pyeloplasty in Children with Ureteropelvic Junction Obstruction

Role of Ultrasonography and Dynamic Renal Scintigraphy Parameters in Decision Making Regarding Performance of Pyeloplasty in Children with Ureteropelvic Junction Obstruction

Outcomes of robot-assisted laparoscopic pyeloplasty among pediatric patients with complex renal anatomy: A retrospective comparative study

Robot-assisted laparoscopic pyeloplasty (RALP) has been increasingly utilized in the treatment of pediatric ureteropelvic junction obstruction (UPJO) with reported success rates of >95%. Complex renal anatomy can make some cases challenging to reconstruct. To evaluate outcomes of children undergoing RALP with aberrant renal anatomy and compare it to those with simple renal anatomy. An IRB approved prospective registry was queried to retrospectively identify all patients who underwent robotic pyeloplasty at our institution from 2012 to 2022. Patients undergoing re-do pyeloplasty were excluded. Complex anatomy was defined as horseshoe kidney, ectopic/pelvic kidney, duplex collecting system, fully bifid renal pelvis and severe malrotation (≥180°). A comparative analysis of baseline demographics, pre-operative clinical/radiological characteristics, intra and post-operative details, and long-term success was performed between those patients with complex anatomy and those without. Of 405 total robotic pyeloplasty's, 375 patients (378 total pyeloplasty; 353 simple, 22 complex) met inclusion criteria. 27 re-do were excluded from analysis. The complex pyeloplasty cohort included 9 horseshoe kidneys, 8 duplex collecting systems, 3 ectopic/pelvic kidneys and 2 kidneys with severe malrotation. There was no difference in age (58 vs 31 months; p=0.38), procedure time (203 vs 207min; p=0.06), length of stay (1.4 vs 1.3 days; p=0.99), or success (91.6% vs 100%; p=0.24) between the simple and complex groups. Etiology of obstruction differed significantly between groups - high insertion was more common (3.9% vs 18.2%, p=0.02) and intrinsic narrowing was less common (60.1% vs 36.4%, p=0.04) in patients with complex anatomy. A multivariate logistic regression was adjusted for age, gender, etiology of obstruction, preoperative differential renal function and post-operative complications and found no difference in success between complex and simple RALP. The findings showed no significant differences in age, procedure time, length of hospital stay, or success rates between the two groups. Specifically, the success rates were 91.6% for the complex group and 100% for the simple group (p=0.24), indicating comparable efficacy. However, the etiology of obstruction varied significantly, with high ureteral insertion more common in the complex anatomy group (18.2% vs. 3.9%, p=0.02) and intrinsic narrowing less common (36.4% vs. 60.1%, p=0.04). Despite these differences, multivariate logistic regression, adjusted for confounders, confirmed no difference in success rates between the groups. RALP is a safe and efficacious approach in patients with complex anatomy with success rates comparable to index patients. High ureteral insertion does appear to be more common in patients with complex anatomy undergoing pyeloplasty.

Pathological characteristics analysis of children with intermittent and persistent hydronephrosis due to uretero-pelvic junction obstruction.

To analyze from a pathological perspective the differences between intermittent and persistent hydronephrosis in children with uretero-pelvic junction obstruction. 23 children who underwent unilateral dismembered pyeloplasty (Anderson-Hynes operation) for intermittent hydronephrosis from September 2017 to March 2024 were included in the observation group. They were compared with a control group consisting of 23 children with persistent hydronephrosis matched for age, gender, and affected side. All children had the narrowed segment surgically excised during the operation, while other obstructive causes (such as polyps, crossing vessels, or tumor compression) were excluded. The specimens were analyzed for muscle and collagen content using Masson's trichrome staining, and the collagen-to-muscle ratio (CMR) was calculated. The number of Cajal-like cells was quantified with c-kit immunohistochemical staining. For all slides, 10 random fields of view were selected under a 400× optical microscope to record pathological data and calculate mean values. Pathological indicators between the two groups were compared using the T-test and the Chi-square test, with P < 0.05 considered statistically significant. The observation group showed a significant difference in the number of fields with low, medium, and high densities of Cajal-like cells compared to the control group [132 (57.4%) vs. 173 (75.2%); 70 (30.4%) vs. 38 (16.5%); 28(12.2%) vs. 19 (8.3%), P < 0.001]. The uretero-pelvic junction in children with intermittent hydronephrosis had lower collagen content, higher muscle content, and a more regular arrangement. The collagen-muscle ratio was significantly lower than that in children with persistent hydronephrosis [(1.59 ± 0.65) vs. (3.98 ± 1.19), P < 0.001]. Compared with persistent hydronephrosis, the narrowed segment at the uretero-pelvic junction in children with intermittent hydronephrosis has a higher density of Cajal-like cells; lower collagen content, and higher muscle content (lower collagen-muscle ratio).