Search Results (22)

The obstruction of the urinary tract is responsible for obstructive nephropathy (ON), also known as uropathy, which may then evolve in a renal parenchymal disease (hydronephrosis). Regarding the etiology of ON, it has been linked to the perturbation of processes occurring during the urinary tract development such as morphogenesis, maturation, and growth. Despite the research carried out in recent years, there is still a pressing need to elucidate the molecular processes underlying the disease. This may then result in the definition of novel biomarkers that can help in patient stratification and the monitoring of therapeutic choices. Matrix metalloproteinases (MMPs) are a family of zinc-dependent endopeptidases with key roles in extracellular matrix remodeling due to their wide cleavage specificity and ability to modulate the bioavailability of growth factors. Despite the known changes in the local tissue microenvironment at the site of the urinary tract obstruction, the role of MMPs in ureteropelvic junction obstruction (UPJO) and, therefore, in the pathogenesis of renal damage in ON is not well-documented. In this review, we underline the possible roles of MMPs both in the pathogenesis of UPJO and in the progression of related hydronephrosis. The potential use of MMPs as biomarkers detectable in bodily fluids (such as the patient’s urine) is also discussed. Full article

Background: Antenatal hydronephrosis (ANH) is the most common congenital renal and urinary tract anomaly, and parenchymal damage and renal fibrosis due to pathological hydronephrosis are the main causes of end-stage renal disease in children and chronic kidney disease in adults. At present, there is no validated biomarker for ANH, and diagnostic criteria other than prenatal ultrasonography (US) assessment are lacking. Therefore, we assessed to determine if biomarkers extracted from amniotic fluid small extracellular vesicles (sEVs) might be used as ANH diagnosis. Methods: With congenital ureteropelvic junction obstruction (UPJO) as the ultimate diagnosis, 10 pregnant women with Grade III-IV ANH and 10 normal pregnant women were recruited. The sEVs were extracted from amniotic fluid supernatant of all samples. Transcriptomic sequencing of sEVs in the discovery cohort identified the differential expression profiles for ANH. The known differentially expressed lncRNAs (DE-lncRNAs) were assessed by qRT–PCR in the validation cohort. Results: We explored the global RNA expression in sEVs from amniotic fluid. The differential expression profiles of both mRNAs and lncRNAs were related to fetal kidney development. Six known DE-lncRNAs were identified for ANH, and three of those with high expression were verified in more ANH samples. In particular, the upregulated LINC02863 and its target genes were associated with renal development and morphogenesis. The four predicted novel lncRNAs in high expression were also related to mesenchymal morphogenesis and the STAT3 signaling pathway and may play roles in ANH. Conclusions: We identified differentially expressed RNAs of all species in the sEVs from amniotic fluid, and the validated known DE-lncRNAs might serve as promising diagnostic biomarkers for ANH. Full article

Background: Chronic renal pain secondary to urological pathology can severely affect a patient’s quality of life. Utero Pelvic Junction Obstruction (UPJO) and Loin Pain Hematuria Syndrome (LPHS) are two conditions known to cause chronic renal pain that can be refractory to conventional treatments. Recent publications demonstrate neuromodulation as an effective treatment option for refractory renal pain. The purpose of this review is to (1) provide evidence from a patient case demonstrating the expanding clinical application of spinal cord stimulation (SCS) for refractory renal pain; (2) provide a review of the literature surrounding UPJO/LPHS and SCS; and (3) express a call to action to investigate the clinical applications of neuromodulation and SCS to treatment-resistant genitourinary pathologies. Methods: We performed a search across PubMed and Web of Science databases to search for patients with UPJO/LPHS who had chronic renal/flank pain and were treated with SCS. Results: In our review, we included five case reports/series (including our own) of eight patients who had chronic flank pain secondary to the genitourinary pathologies UPJO and LPHS. Some patients have been having renal pain for up to 20 years, with all patients experiencing significant or complete alleviation of pain after SCS despite multiple failed attempts with other modalities. Five patients had complete resolution of pain and four patients were able to discontinue their pain medications entirely. Conclusions: SCS is gaining traction in its versatility in the management of various chronic pathologies beyond just CNS conditions. Specifically, UPJO and LPHS are examples of urogenital conditions that may benefit from using SCS. Our case shows an exciting opportunity for managing clinical symptoms in patients who have these conditions but have not undergone surgery for many reasons. Our review and clinical report suggest more research is needed in the application of SCS to genitourinary conditions and those experiencing refractory chronic pain. Full article

Introduction: Single-port video-assisted techniques, such as one-trocar-assisted pyeloplasty (OTAP) or Round-Traction-Assisted Pyeloplasty (RoTAP), have proven helpful for correcting congenital ureteropelvic junction obstruction (UPJO), especially in infants. Surgical Technique: This manuscript presents a technical variation based on the gloveport system that allows bimanual endocavitary dissection and protects the surgical wound during the procedure without increasing the cost or technical difficulty. Comment: This new technique, called gloveport-assisted retroperitoneal pyeloplasty (GARP), could be a valuable alternative for this pathology. Full article

Urinary tract dilations (UTDs) are the most frequent prenatal renal anomaly. The spectrum of etiologies causing UTD ranges from mild spontaneously resolving obstruction to severe upper and lower urinary tract obstruction or reflux. The early recognition and management of these anomalies allows for improved renal endowment prenatally and ultimately better outcome for the child. The role of the general obstetrician and pediatrician is to recognize potential prenatal and postnatal cases addressed to their practice and to refer patients to specialized pediatric nephrology and urology centers with a sense of the urgency of such a referral. The aim of this paper is to offer clinical recommendations to clinicians regarding the management of neonates and children born with prenatally detected UTD, based on a consensus between Swiss pediatric nephrology centers. The aim is to give suggestions and recommendations based on the currently available literature regarding classifications and definitions of prenatal and postnatal UTD, etiologies, prenatal and postnatal renal function evaluation, investigations, antibiotic prophylaxis, and the need for referral to a pediatric nephrologist and/or urologist. The overarching goal of a systematic approach to UTD is to ultimately optimize kidney health during childhood and improve long-term renal function prognosis. Full article

Percutaneous renal surgery, although much less invasive than other procedures, is subject to several complications, which can occur at any time during the course of treatment, starting from the performance of the minimal nephrostomy procedure. We present an extremely rare vascular complication of percutaneous nephrostomy represented by arteriovenous fistula that occurred in a 24-year-old patient known to have right ureteropelvic junction obstruction operated with the absence of double-J catheter permeability and grade II-III hydronephrosis for which minimal percutaneous nephrostomy was urgently fitted. The arteriovenous fistula was resolved by supraselective artery embolization. Full article

Introduction: Etiopathogenesis and the symptomatology of ureteropelvic junction obstruction (UPJO) in the pediatric population has not yet been definitely clarified, suggesting a multifactorial nature of the condition. The aim was to analyze the association between the number of Interstitial Cells of Cajal (ICCs), as well as P₂X₃ receptors in ureteropelvic junction (UPJ) and the pain response in pediatric patients with hydronephrosis. Methods: 50 patients with congenital hydronephrosis underwent open or laparoscopic pyeloplasty at one of two departments of pediatric surgery and urology in Poland. Patients were divided into two groups according to the pain symptoms before surgery. A total of 50 samples of UPJ were obtained intraoperatively and underwent histopathological and immunohistochemical (IHC) analysis. Quantitative assessment of ICCs was based on the number of CD117(+) cells of adequate morphology in the subepithelial layer and the muscularis propria. Expression of P₂X₃ receptors was evaluated as the intensity of IHC staining. Results: Patients with hydronephrosis and accompanying pain were on average 60 months older (77 vs. 17 months) than children with asymptomatic hydronephrosis (p = 0.017). Symptomatic children revealed higher numbers of ICCs in both the subepithelial layer and in the lamina muscularis propria. In particular, symptomatic patients aged 2 years or more exhibited significantly higher numbers of ICCs in the subepithelial layer. Significant differences in the distribution of ICCs between the subepithelial layer and the lamina muscularis propria were observed in both groups. Expression of P₂X₃ receptors was limited to the urothelium and the muscle layer and correlated between these structures. There was no relationship between pain response and the expression of P₂X₃ receptors. Conclusions: ICCs and P₂X₃ receptors may participate in the pathogenesis of UPJO and in the modulation of pain response to a dilatation of the pyelocaliceal system. Explanation of the role of ICCs and P₂X₃ receptors in propagation of ureteral peristaltic wave and the modulation of pain stimuli requires further studies. Full article

Background: Robot-assisted pyeloplasty is widely used in pediatric surgery because of its well-known advantages over open or laparoscopic surgery. The aim is to explore our experience and evaluate the achievements we have made. Methods: We evaluated patients undergoing robotic pyeloplasty from January 2016 to November 2021, including those who presented with a ureteropelvic junction obstruction associated with other anomalies of the kidney. The parameters examined were: age, weight, associated renal malformations, conversion rate, operative time, and intra- and postoperative complications. Results: Of 39 patients, 7 (20%) were included, of whom 5 (71%) were male and 2 (29%) were female. The mean age at surgery was 84 months (range 36–180 months), and the mean weight at surgery was 24.4 kg (range 11–40 kg). In five (71%) patients the ureteropelvic junction obstruction (UPJO) was left-sided and in two (29%) it was right-sided. In four (57%) cases, UPJO was associated with a horseshoe kidney, right-sided in one (25%) patient, and left-sided in the other three (75%). A 180° rotation of the kidney was present in one (14%) patient. Nephrolithiasis was present in two (29%) patients. The mean operative time was 160 min (range 140–240 min). The average bladder catheter dwell time was 1 day (range 2–3 days), while the average abdominal drainage dwell time was 2 days (range 2–4 days). The mean hospitalization time was 4 days (range 3–9 days). On average, after 45 days (range 30–65) the JJ ureteral stent was removed cystoscopically. No intraoperative complications were reported, while one case of persistent macrohematuria with anemia requiring blood transfusion occurred postoperatively. Conclusions: Ureteropelvic junction obstruction might be associated with other congenital urinary tract anomalies such as a duplicated collecting system, horseshoe kidney, or pelvic kidney. These kinds of malformations can complicate surgery and require more attention and accuracy from the surgeon. Our experience shows that, with regards to the robotic learning curve required for pyeloplasty, the treatment of the ureteropelvic junction in these situations does not present insurmountable difficulties nor is burdened by complications. The application of robot-assisted surgery in pediatric urology makes difficult pyeloplasties easier. Full article

We designed a retrospective study to assess the surgical and economic outcomes of robot-assisted laparoscopic pyeloplasty (RALP) compared with open pyeloplasty (OP), including consecutive patients suffering from ureteropelvic junction obstruction and operated on from January 2012 to January 2022 at a single center. Preoperative, intraoperative, and postoperative outcomes, including costs, were comparatively analyzed. The primary outcome was 3-month success, defined as symptom resolution and no obstruction upon diuretic renal scintigraphy. Overall, 91 patients were included (48 OP and 43 RALP). The success rate at 3 months was 93.0% and 83.3% in the RALP and OP group, respectively (p = 0.178), and the results remained stable at the last follow-up (35.4 ± 22.8 months and 56.0 ± 28.1 months, respectively). Intraoperative blood loss (p < 0.001), need for postoperative analgesics (p = 0.019) and antibiotics (p = 0.004), and early postoperative complication rate (p = 0.009) were significantly lower in the RALP group. None of the assessed variables were a predictor for failure. The mean total direct cost per surgical procedure and related hospital stay was 2373 € higher in the RALP group. RALP is an effective and safe treatment for ureteropelvic junction obstruction; however, further studies are needed to evaluate the cost-effectiveness of RALP, accounting for indirect costs and cost-saving with new surgical platforms. Full article

Background: To explore the application value of intraoperative imaging by indocyanine green (ICG) injection through the collection system of the urinary tract for Da Vinci Xi robot navigation in complex surgeries on the upper urinary tract. Methods: Data of 14 patients who underwent complex surgeries of the upper urinary tract post-ICG injection through the collection system of the urinary tract in combination with Da Vinci Xi robot navigation in the Tianjin First Central Hospital between December 2019 and October 2021 were analyzed in this retrospective study. The operation duration, estimated blood loss, and exposure time of ureteral stricture to ICG were evaluated. The renal functions and tumor relapse were evaluated after surgery. Results: Of the fourteen patients, three had distal ureteral stricture, five had ureteropelvic junction obstruction, four presented duplicate kidney and ureter, one had a giant ureter, and one presented an ipsilateral native ureteral tumor after renal transplantation. The surgeries in all patients were successful, with no conversion to open surgery. In addition, no injury to the surrounding organs, anastomotic stenosis or leakage, or ICG injection-related side effects were detected. Imaging at 3 months post-operatively revealed improved renal functions compared to those before the operation. No tumor recurrence or metastasis was observed in patient 14. Conclusion: Fluorescence imaging compensating for the inadequacy of tactile feedback in the surgical operating system has advantages in identifying the ureter, determining the site of ureteral stricture, and protecting the blood flow for the ureter. Full article

The aim of the study was to evaluate whether serum aldosterone levels or plasmatic renin activity (PRA) measured early in life (1–3 months) could predict a future surgical intervention for obstructive congenital anomalies of kidney and urinary tract (CAKUT). Twenty babies aged 1–3 months of life with suspected obstructive CAKUT were prospectively enrolled. The patients underwent a 2-year follow-up and were classified as patients needing or not needing surgery. In all of the enrolled patients, PRA and serum aldosterone levels were measured at 1–3 months of life and were evaluated as predictors of surgery by receiver-operating characteristic (ROC) curve analysis. Patients undergoing surgery during follow-up showed significantly higher levels of aldosterone at 1–3 months of life compared to those who did not require surgery (p = 0.006). The ROC curve analysis of the aldosterone for obstructive CAKUT needing surgery showed an area under the ROC curve of 0.88 (95%CI = 0.71–0.95; p = 0.001). The aldosterone cut-off of 100 ng/dL presented 100% sensitivity and 64.3% specificity and predicted surgery in 100% of cases. The PRA at 1–3 months of life was not a predictor of surgery. In conclusion, serum aldosterone levels at 1–3 months could predict the need for surgery during obstructive CAKUT follow-up. Full article

The use of minimally invasive surgery (MIS) to treat ureteropelvic junction obstruction (UPJO) in children has significantly increased. Nowadays, open pyeloplasty (OP) seems to lose importance. The aim of this study is to evaluate the safety and efficacy of OP in infants < 1 year. Medical records of patients < 1 year with UPJO who had undergone retroperitoneal OP (January 2008–February 2022) at our institution were retrospectively analyzed. Included patients were operated on according to a modified Anderson–Hynes technique. All clinical, operative, and postoperative (1 month–5 years’ follow-up) data were collected. Additionally, a nonvalidated questionnaire was sent to the patients/parents. A total of 162 infants (124 boys) met the inclusion criteria. The median age at surgery was 3 months (range: 0–11 months). The median operation time was 106 min (range: 63–198 min). None of the patients had severe surgical complication (Clavien–Dindo > 3). The nonvalidated questionnaire showed a high impact of quality of life. Follow-up was in median 30.5 months (0–162 months). OP is still a reliable procedure with good long-term results especially in infants < 1 year of age, which can be performed in a variety of centers. Full article

Objective: To investigate dental anomalies and the molecular etiology of a patient with Ellis–van Creveld syndrome and two patients with Bardet–Biedl syndrome, two examples of ciliopathies. Patients and Methods: Clinical examination, radiographic evaluation, whole exome sequencing, and Sanger direct sequencing were performed. Results: Patient 1 had Ellis–van Creveld syndrome with delayed dental development or tooth agenesis, and multiple frenula, the feature found only in patients with mutations in ciliary genes. A novel homozygous mutation in EVC2 (c.703G>C; p.Ala235Pro) was identified. Patient 2 had Bardet–Biedl syndrome with a homozygous frameshift mutation (c.389_390delAC; p.Asn130ThrfsTer4) in BBS7. Patient 3 had Bardet–Biedl syndrome and carried a heterozygous mutation (c.389_390delAC; p.Asn130ThrfsTer4) in BBS7 and a homozygous mutation in BBS2 (c.209G>A; p.Ser70Asn). Her clinical findings included global developmental delay, disproportionate short stature, myopia, retinitis pigmentosa, obesity, pyometra with vaginal atresia, bilateral hydronephrosis with ureteropelvic junction obstruction, bilateral genu valgus, post-axial polydactyly feet, and small and thin fingernails and toenails, tooth agenesis, microdontia, taurodontism, and impaired dentin formation. Conclusions: EVC2, BBS2, and BBS7 mutations found in our patients were implicated in malformation syndromes with dental anomalies including tooth agenesis, microdontia, taurodontism, and impaired dentin formation. Full article

Background: Prenatal hydronephrosis is common and may vary in size. Although mostly unproblematic, it may be a sign of urinary tract obstruction of differing severity. Case Diagnosis/Treatment: We present a boy with prenatally detected bilateral giant hydronephrosis. A prenatal ultrasound showed the whole abdominal cavity of the fetus filled with urine. Kidney parenchyma could not be seen. The boy was born at 34 + 1 weeks’ gestation. After delivery, he showed a severely distended abdomen. Insertion of a nasogastric tube was not possible, and he had to be intubated due to respiratory distress. A bilateral percutaneous nephrostomy was performed immediately. After a few hours, he could be stabilized and extubated. An ultrasound on the following day showed two kidney units with normal kidney parenchyma of normal size. The initially slightly elevated serum creatinine level normalized within one week. An antegrade pyelography via the nephrostomy tubes showed bilateral ureteropelvic junction obstruction. Conclusion: Severe bilateral hydronephrosis may be associated with good outcome and well-preserved kidney function. Prenatal counseling should be done carefully, with discussion of different treatment possibilities and without definitive prediction of outcome. Full article

Background and Objectives: To clinically validate the computed tomography (CT)-based three-dimension (3D) model for treatment planning and intraoperative navigation of ureteropelvic junction obstruction (UPJO) complicated with renal calculi. Materials and Methods: We retrospectively collected the data of 26 patients with UPJO and renal calculi who were surgically treated in our institution from January 2019 to December 2021. Before surgery, 3D models based on preoperative CT scans were constructed in all patients. Additionally, the patients were divided into three groups according to the results of 3D models, distinguished by different treatment of renal calculi, that is, left untreated (1), pyelolithotomy (2), and endoscopic lithotomy (3). The quantitative analysis of renal calculi parameters, and perioperative and follow-up data were compared. Results: The mean number of involved renal calyces (p = 0.041), and the mean maximum cross-sectional area (p = 0.036) of renal stones were statistically different among the three groups. There were no significant differences among the three groups in the mean operative time, mean estimated blood loss, mean pararenal draining time, and mean hospital stay. The intraoperative stone clearance rates were 100% (11/11) and 77.8% (7/9) in group 2 and group 3, respectively. The trends of increased estimated glomerular filtration rate and decreased creatinine on the average levels after surgery were observed, although these changes were not statistically significant. At a mean follow-up of 19.4 ± 6.4 months, the overall surgical success rate of the UPJO was 96.2% (25/26), and the overall success rate of renal calculi removal was 80.8% (21/26). Renal stones in 66.7% (4/6) of patients in group 1 spontaneously passed out. Conclusions: Preoperative 3D CT models have exact clinical value in the surgical planning and intraoperative navigation of UPJO patients complicated with renal calculi. Full article