Congenital Ureteropelvic Junction Obstruction Research Articles

Determination of voiding pressure in infants with normal lower urinary tracts: Exploring the possible effect of sacral development.

To investigate the association between sacral underdevelopment, as defined by subnormal sacral ratio (SR) measurements, with increased maximum detrusor voiding pressure (P det. Max) in infants. In this 2007-2015 retrospective cohort study, the medical records of all infants who underwent a pyeloplasty due to congenital ureteropelvic junction obstruction were added. Their P det. Max was evaluated through the suprapubic catheter utilized for urinary drainage intraoperatively, without imposing any additional risk of urethral catheterization on the infant. SR was calculated via the plain kidney, ureter, and bladder (KUB) radiography film obtained during the voiding cystourethrogram (VCUG) evaluation before the surgery. Participants were categorized into SR < 0.74 or SR ≥ 0.74. P det. Max was subsequently compared between these two groups. A total of 45 patients were included in our analysis. Twenty-eight (62.2%) patients had a (SR < 0.74), while 17 (37.8%) had a (SR ≥ 0.74). P det. Max was shown to be significantly higher in the SR < 0.74 compared to the SR ≥ 0.74 group (167.5 ± 60.8 vs. 55.7 ± 17.9 cmH2O, p < 0.001). After adjusting for age and sex, SR remained a significant contributor to P det. Max (p < 0.001). Physiologic detrusor sphincter dyscoordination (PDSD) rate was significantly higher in the SR < 0.74 versus SR ≥ 0.74 group (100.0% vs. 70.6%, respectively; p = 0.005). Lumbosacral underdevelopment, as indicated by subnormal sacral ratios, is associated with sphincter-detrusor dyscoordination, which causes PDSD and can ultimately result in higher P det. Max in infants.

Surgical intervention and long-term renal outcomes of congenital ureteropelvic junction obstruction in a young adult cohort.

To evaluate the impact of surgical intervention on long-term renal outcomes for adult patients with congenital ureteropelvic junction obstruction (UPJO). We queried service members diagnosed with UPJO from the United States Military Health System electronic health records from 2005 to 2020. We assessed demographic, laboratory, radiology, surgical intervention, and outcome data. We evaluated the impact of surgical intervention on renal function based on the estimated glomerular filtration rate (eGFR), hypertension (HTN, defined as any prescription for blood pressure [BP] medication and/or average of two BP readings ≥ 130/80mmHg more than 2weeks apart), and changes in renal excretory function on radionuclide scans. We identified 108 individuals diagnosed with congenital UPJO; mean follow-up of 7years. Mean age at diagnosis was 25years; 95% male; 69% White, 15% Black. At diagnosis, median BP was 130/78mmHg and mean eGFR 93ml/min/1.73m2. Subsequently, 85% had pyeloplasty and 23% had stent placement. There were no significant differences in mean eGFR pre- and post-intervention (94 vs. 93ml/min/1.73m2, respectively; p = 0.15) and prevalence of defined HTN (59% vs. 61%, respectively; p = 0.20). Surgical intervention for right-sided UPJO significantly reduced the proportion of patients with delayed cortical excretion (54% pre vs. 35% post, p = 0.01) and T½ emptying time (35min vs. 19min, p = 0.009). Similar trends occurred with left-sided UPJO but were not significant. Surgical intervention was not associated with significant differences in the long-term outcomes of kidney function and HTN prevalence in our young adult cohort. However, renal excretory function improved on radionuclide scans.

Unique presentation of Congenital ureteropelvic junction obstruction in adult

Summary: A 34-year-old female, without comorbidities, presented to the emergency department with sudden right flank pain. Initial workup showed elevated WBC (14.5) and normal creatinine (0.81), but later developed fever spikes and rising CRP (59.50). Gentamicin treatment was initiated. Transferred for a Diuretic Renogram, confirming suboptimal function of the right kidney with obstruction at the renal pelvis/PUJ. Split functions indicated 32% for the right kidney and 68% for the left. Radiological workup included ultrasound, CT KUB with contrast, and CT Urography, revealing right renal acute hydronephrosis without evidence of stones. Management involved symptomatic treatment, but with persistent symptoms, Gentamicin prophylaxis was initiated. The patient was transferred for Renogram, detecting suboptimal right kidney function. A planned stent insertion followed by Robotic-assisted and laparoscopic pyeloplasty was scheduled. Discussion: Uretero Pelvic Junction Obstruction (UPJO) is a partial blockage where the ureter enters the kidney. Congenital UPJO can result from intrinsic narrowing, high ureter insertion, ureteral polyps, or accessory renal artery crossing vessels causing extrinsic compression. Most cases are detected on prenatal ultrasound, while postnatal presentations include UTI, abdominal mass, hematuria, and intermittent flank pain. Diagnosis involves CT urography and diuretic renogram, confirming suboptimal function (

Interstitial cells of Cajal in the ureteropelvic junction: does its expression have a role in the pathogenesis of congenital ureteropelvic junction obstruction?

Background One of the most frequent urological abnormalities in pediatric urology and the most frequent cause of hydronephrosis in the pediatric age group is uteropelvic &lt;ureteropelvic?&gt; junction obstruction (UPJO). Although its etiology has been the subject of several investigations, it is still unknown. Objective Comparing the quantitative changes in the interstitial cells of Cajal (ICC) between specimens with UPJO and specimens without hydronephrosis using light microscopy and correlating these changes to the etiology of the disease. Methods Patients with UPJO and patients without obstruction were compared immunohistochemically with c-kit (CD117) between February 2020 and February 2022 at Ain Shams University Hospital to quantify the ICC, which is situated close to the circular muscle layer and parallel to the muscle cells. Results Light microscopic analysis revealed that the control group’s Cajal cell density was much higher than the number of cells in the patients with UPJ blockage. Conclusion Our research found that the mean density of the ICC was lower in UPJO patients compared with controls, indicating that the absence of the ICC may contribute to the pathogenesis of UPJO.

Obstrucción congénita de la unión ureteropiélica. Estudio de casos en un centro de segundo nivel

Objective: To evaluate the incidence, clinical characteristics, diagnosis, and treatment of congenital ureteropelvic junction obstruction in pediatric patients (CUPBO) at the Hospital Infantil de Morelia. Material and methods: This was a retrospective, observational, descriptive, and cross-sectional study of pediatric patients diagnosed with CUPBO and treated in the urology service of a second-level care medical center from January 2018 to December 2021. Results: We reviewed 53 patients with prenatal hydronephrosis who underwent postnatal follow-up, of whom only 6 patients diagnosed with congenital ureteropelvic junction obstruction (CUPBO) were identified prenatally. Seven patients were diagnosed with hydronephrosis postnatally and subsequently diagnosed with CUPBO, resulting in a total sample of 13 diagnosed patients. The incidence of CUPBO was 3.4 patients per 100 first visits to the Pediatric Urology Service. Of the cases, 92.31% were male, and the most frequent clinical manifestations were skin infections and localized pain. Surgery was performed in all cases, with Anderson-Hynes pyeloplasty being the most frequent procedure. Complications included urinary tract infections, wound infections, and fistula, but most patients had satisfactory results with a reduction of hydronephrosis after surgery. Findings or conclusions: Our study found a low rate of early detection of CUPBO in patients with prenatal hydronephrosis follow-up. We recommend an intentional search for urologic alterations in structural ultrasound scans of prenatal control. We emphasize the importance of an accurate diagnosis and timely treatment of CUPBO to prevent renal complications.

IMMUNOHISTOCHEMICAL EXPRESSION OF C-KIT AND S-100 MARKERS IN URETEROPELVIC JUNCTION OBSTRUCTION AND ITS CORRELATION WITH HISTOPATHOLOGY AND MASSON'S TRICHROME STAINING

Background: Congenital obstructive nephropathy is the principal cause of end stage renal disease (ESRD) in children ( Beneldet al. 2003). Obstructive nephropathy leads to Hydronephrosis, dened clinically by an enlargement of the kidney as a result of urine accumulation in the renal pelvis or calyces. [1,2] Ureteropelvic junction (UPJ) obstruction is the most common cause of hydronephrosis with an estimated incidence of 1 in 1000–1500 newborns ( Changet al. 2004). [2,3] Aims: To evaluate the pathogenesis of congenital ureteropelvic junction obstruction (UPJO) by histopathology, IHC markers CD117 &amp; S100 and special stain Masson's Trichrome. Methods And Materials: The study group consists of 50 Pediatric cases presenting with intrinsic ureteropelvic junction obstruction and 15 patients with Wilms tumor with no UPJO formed the control group. All the cases and controls were subjected to histopathological examination, immunohistochemical staining with markers CD117 &amp; S100 and special stain Masson's Trichrome. Mann – Whitney U test and Pearson's chisquare test were used for statistical analysis. The cases had reduce Results: d CD117 and S100 expression, Irregularity of muscle bers, increased wall thickness &amp; muscle thickness and submucosal collagen deposition compared to controls. Decreased ICC interpreted by Conclusions: decreased CD117, decreased neural innervation interpreted by decreased S100, structural changes - Irregularity of muscle bers arrangement, increased submucosal collagen deposition and increased smooth muscle thickness possibly cause disruption of the mobility of UPJ and lead functional obstruction.

Assessment of Cases of Congenital Obstructive Uropathy in Children

Background: The aim is to assess cases of congenital obstructive uropathy in children.Material &amp; Methods:Sixty- five children in age ranged 5-12 years of either gender with congenital obstructive uropathy were recruited for the study. In all cases, ultrasonography was done in all cases. Growth of the child was monitored and height standard deviation score (Ht-SDS) was calculated annually. Glomerular filtration rate (ml/min/1.73 m2) was calculated.Results:Out of 65 patients, males were 40 and females were 25. Etiology found to be ureteropelvic junction obstruction (PUJO) in 12, posterior urethral valve (PUV) in 14, PUJO + PUV in 30 and obstructive megaureter in 9 cases. Symptoms were fever in 34, pain abdomen in 20, burning micturition in 45, poor urinary stream in 21 and flank pain in 15 cases. Treatment given was pyeloplasty in 14, nephrectomy in 20, PUV fulguration in 11, ureterocele decompression in 12 and ureteric reimplantation in 8 cases.Conclusions:Most common etiologies for congenital obstructive uropathy in children were congenital uretero-pelvic junction obstruction and posterior urethral valve. Male preponderance was seen with burning micturition.

Comparison of Surgical and Functional Outcome of Laparoscopic Pyeloplasty and Robot-assisted Pyeloplasty for Congenital Uretero Pelvic Junction Obstruction

Comparison of Surgical and Functional Outcome of Laparoscopic Pyeloplasty and Robot-assisted Pyeloplasty for Congenital Uretero Pelvic Junction Obstruction

Comparison of Drainage Methods After Pyeloplasty in Children: A 14-Year Study

Objective: To summarize our experiences with drainage methods after laparoscopic pyeloplasty with a 14-year study.Methods: We reviewed the data of the 838 children operated on for hydronephrosis due to congenital ureteropelvic junction obstruction (UPJO) between July 2007 and July 2020. Patients' demographics, perioperative details, postoperative drainage stents [including double-J stent, percutaneous trans-anastomotic (PU) stent, and trans-uretero-cystic external urethral stent (TEUS)], complications, hospital stay, and long-term follow-up outcomes were analyzed. Long-term follow-up was performed by outpatient visits and telephone follow-up. Moreover, we reviewed the details of nine cases of recurrence after laparoscopic pyeloplasty.Results: Comparison of preoperative general data among the three groups indicated that there was no statistical difference in age, gender, and surgical side of the three groups. Statistical differences were found in the incidence of postoperative complications from the three postoperative drainage method groups, especially the incidence of reoperations (p < 0.01): there were six cases (3.19%) of recurrences in the TEUS group, two cases (0.36%) in the DJ group, and one case (0.93%) in the PU group. In the six recurrent cases from the TEUS group, four cases (44.4%) were found to have stenosis, and two cases (22.2%) have iatrogenic valvular formation.Conclusion: Not all three types of drainage methods are suitable for drainage after pyeloplasty. Based on our findings, TEUS is not recommended.

Management of ureteropelvic junction obstruction in an era of minimally invasive surgery

IntroductionIn an era of minimally invasive surgery, laparoscopic pyeloplasty has emerged as an alternative to conventional open pyeloplasty. Although the open method boasts a success rate of over 90%, laparoscopic pyeloplasty far exceeds patient satisfaction due to its low perioperative morbidity. The aim of this study was to evaluate the outcome of patients who underwent laparoscopic pyeloplasty due to congenital ureteropelvic junction obstruction (UPJO). Material and Methods A retrospective observational study was conducted at Sri Jayawardenapura general hospital from March 2014 to December 2020. All patients were subjected to CT urogram and diuretic renogram (DTPA) scan. Clinical, imaging and biochemical findings were recorded. Patients included were those who underwent laparoscopic pyeloplasty based solely on the clinical severity of the disease, those who underwent lap-nephrectomy or open surgery was excluded. Follow up was based on clinical and DTPA scan findings. Improvement in symptomatology, split renal function(SRF) by 5% and/or diuretic curve pattern were considered successful outcomes. Results A total of 40 patients who underwent LP were evaluated with a mean age of 32 years and slight male preponderance. The majority (82.5%) were symptomatic with pain and/or infection. All were diagnosed with hydronephrosis with impaired DTPA. An SRF &lt; 40% in the affected kidney was observed in 22(55%) of patients. Concomitant pyelolithotomy was performed in 7(17.5%) patients. Mean hospital stay was 3.5 days. At mean follow up of 22-months, improvement in symptoms were noted in 35(87.5%) patients and SRF improvement (&gt;5%) were noted in 11(27.5%); the function remained stable in 27(67.5%) and deteriorated in 2(5%) of patients. Discussion Although LP is technically demanding, it offers excellent symptomatic relief and renal preservation. It offers less morbidity, better aesthetic and post-operative convalescence: a promising viable option replacing open surgery.

Minimally invasive management of massive giant hydronephrosis in tertiary care centre Northern Sri Lanka: A case report.

Introduction & importanceGiant hydronephrosis (GH) is defined as a kidney containing greater than 1000 ml of urine in its collecting system. It is a rarely presented condition in an adult. Common aetiology for GH is due to congenital ureteropelvic junction obstruction. We present a case of a late presentation of GH was being managed by a minimally invasive laparoscopic technique.Case presentationA 63-year-old Sri Lankan male presented with worsening of generalized abdominal swelling with vague abdominal symptoms for a six-month duration, found to have a GH of the left kidney on USS. The Contrast-enhanced computerized tomography (CECT) scan confirmed the diagnosis with 12l of fluid and possible ureteropelvic junction obstruction (UPJO). CT did not show any excretion of the left kidney. Hydronephrosis was drained one day before the surgery by a percutaneous nephrostomy tube. Laparoscopic transperitoneal nephrectomy was performed. The patient had a smooth and fast recovery.DiscussionA neglected congenital UPJO can present with GH in adults. It causes vague abdominal symptoms like abdominal distension, dyspepsia, and fatigue. CECT will give the diagnosis and identify the aetiology of GH. Non-functional GH kidneys can be treated with nephrectomy by open or laparoscopic surgical technique. Laparoscopic nephrectomy shoes less blood loss during surgery, less postoperative pain and early recovery after surgery. However, in GH, surgeons need the experience to overcome the challenges like adhesions and working in a small space.ConclusionsThe percutaneous nephrostomy decompression followed by laparoscopic transperitoneal nephrectomy is feasible for a massive GH.

Infant Robot-assisted Laparoscopic Pyeloplasty: Outcomes at a Single Institution, and Tips for Safety and Success

BackgroundSince its first description, multiple reports proved efficacy and safety of the robotic platform. Further progress has been made allowing for the application of robotic surgery to smaller patients, including infants. Despite the early favorable results, the use of robot surgery in infants is still controversial and more studies are needed to confirm its benefits. ObjectiveTo our knowledge, we present the largest single-institution case series of robot-assisted laparoscopic pyeloplasty (RAL-P) in infants, aiming to contribute to the current literature with a guide for key technical steps and safety tips for infant RAL-P. Design, setting, and participantsWe performed a retrospective review of a prospectively maintained database. The study protocol was approved by the institutional review board. Surgical procedureOnly infants (≤12 mo of age) with a diagnosis of congenital ureteropelvic junction obstruction (UPJO) undergoing primary robotic dismembered pyeloplasty were included in the study. MeasurementsWe critically reviewed the clinical outcomes, described the main steps of the operation, and shared tips for a safe approach. Results and limitationsFrom January 2012 to August 2019, 44 infants underwent RAL-P for UPJO––33 (75%) males and 11 (25%) females. All robotic cases were completed successfully, with no laparotomic conversions. The median age and weight were 4 (1–12) mo and 6.8 (3.8–10.5) kg, respectively. The mean operative time was 142 (±25) min. The mean estimated blood loss was 7 (±3.6) ml, and no intraoperative complications occurred. The mean length of hospital stay (LOS) was 1.4 (±0.7) d. Seven (15.6%) patients had postoperative complications—one (2%) ileus (Clavien-Dindo grade [CDG] I), four (9%) urinary tract infections (CDG II), and two (4.5%) port-site hernias (CDG III). At a median follow-up of 19 mo, the success rate was 100%. ConclusionsGiven the successful outcomes, benefits of decreased LOS, and improved cosmesis, RAL-P is an appealing management option for UPJO in infants. Market release of new systems, further miniaturization of instruments, and more affordable costs will hopefully be shedding light on more complex applications. Patient summaryInfants (≤12 mo of age) diagnosed with ureteropelvic junction obstruction undergoing primary robotic dismembered pyeloplasty were selected and included in this study. No intraoperative complications or conversion to an open approach occurred. Seven patients (16%) developed postoperative complications—one (2%) postoperative ileus, four (9%) urinary tract infections, and two (4.5%) port-site hernias. At a median follow-up of 19 (7–66) mo, the success rate was 100%.

Pyeloplasty in children with low differential renal function: Functional recoverability

Ureteropelvic junction obstruction (UPJO) is a common pathology in pediatric urology practice. Indications for intervention are well established while the ideal management of children with UPJO in poorly functioning kidneys is still debatable. We aimed in this study to evaluate the outcome of pyeloplasty in patients with UPJO and low DRF ≤20%. We retrospectively evaluated 218 children with congenital UPJO from two tertiary hospitals between 2008 and 2018. We included only those with primary unilateral UPJO and DRF ≤20% on diuretic renography. Open dismembered pyeloplasty with stenting was carried out in all. Patients with bilateral UPJO, solitary kidney, DRF above 20%, association with other urinary anomalies and patients who underwent previous renal or ureteric surgeries and patients with missed follow-up were excluded. Clinical and radiological outcomes of this subgroup of patients were assessed. A total of 21 patients (12 boys, 9 girls) with a median age at surgery of 2 years were included. Preoperative DRF ranged from 8 to 20% with a mean of 15%±3.5%. The median follow-up period was 2.5 years. Renal ultrasound showed improved HN in 16 patients (4 had a complete resolution) and stable in the remaining 5. Most of the improvement was noted in the first postoperative ultrasonography 16/21 (76%). Diuretic renography showed improved DRF by 10-15% in 8 patients (38%) while the remaining patients showed stable DRF with a non-obstructive curve with a mean improvement in DRF of 8±5%. No postoperative complications were encountered and none of our patients required reintervention during follow-up. The ideal management of children with UPJO and poorly functioning kidneys is still arguable. In our study we evaluated the outcome of pyeloplasty in patients with a low DRF ≤ 20% aiming to test the patients' variables that can affect the improvement of DRF and HN grade after surgical intervention, however, none of these preoperative variables seemed to be a significant predictor. Children with unilateral UPJO and DRF ≤ 20% have a favorable outcome after pyeloplasty. Improvement of HN in the first postoperative renal ultrasound is a good indicator for success.

Giant infected hydronephrosis: A kidney bag filled with 6300 cc pus

Giant hydronephrosis has been variably defined in literature as that one that contains more than 1 liter of fluid or comprising >1.6% of the body weight. It is also described as that occupying a hemiabdomen, crossing the midline and occupying more than five vertebral heights. Congenital uretero-pelvic junction obstruction is the most common cause of giant hydronephrosis in children and young adults. Other important causes include vesico-ureteric junction obstruction, ureteral atresia and primary obstructed megaureter. We herein report a rare case of giant hydronephrosis secondary to pelvi-ureteric junction obstruction presenting as an acute abdomen in a young female.

Outcome of Percutaneous Nephrostomy in Children With Ureteropelvic Junction Obstruction

Introduction and objectives: Congenital ureteropelvic junction obstruction (UPJO) is the most common cause of hydronephrosis. Management protocols are based on the presence of symptoms and when the patient is asymptomatic the function of the affected kidney determines the line of treatment. Percutaneous nephrostomy (PCN) became a widely accepted procedure in children in the 1990s. The aim of the study was to evaluate the results of performing percutaneous nephrostomy (PCN) in all patients with UPJO and split renal function (SRF) of less than 10% in the affected kidney, because the management of such cases is still under debate.&#x0D; Methods:This prospective clinical trial was carried out at Dhaka Medical College Hospital from January 2014 to December 2016. Eighteen consecutive patients who underwent PCN for the treatment of unilateral UPJO were evaluated prospectively. In these children, ultrasonography was used for puncture and catheter insertion. Local anesthesia with sedation or general anesthesia was used for puncture. Pig tail catheters were employed. The PCN remained in situ for at least 4 weeks, during which patients received low-dose cephalosporin prophylaxis. Repeat renography was done after 4 weeks. When there was no significant improvement in split renal function (10% or greater) and PCN drainage (greater than 200 ml per day) then nephrectomies were performed otherwise pyeloplasties were performed. The patients were followed up after pyeloplasty with renograms at 3 months and 6 months post operatively.&#x0D; Results: All the patients had severe hydronephrosis during diagnosis and 14 patients with unilateral UPJO were improved after PCN drainage and underwent pyeloplasty. The rest four patients that did not show improvement in the SRF and total volume of urine output underwent nephrectomy. In the patients with unilateral UPJO who improved after PCN drainage, the SRF was increased to 26.4% ±8.6% (mean± SD) after four weeks and pyeloplasty was performed. At three and six months follow-up, SRF value was 29.2% ±8.5% and 30.8.2% ±8.8% respectively.&#x0D; Conclusion: Before planning of nephrectomy in poorly functioning kidneys (SRF &lt; 10%) due to congenital UPJO, PCN drainage should be done to asses improvement of renal function.&#x0D; Bangladesh Journal of Urology, Vol. 20, No. 2, July 2017 p.61-64

Report of a de novo c.2605C > T (p.Pro869Ser) change in the MED13L gene and review of the literature for MED13L-related intellectual disability

BackgroundMED13L-related intellectual disability is a new syndrome that is characterized by intellectual disability (ID), motor developmental delay, speech impairment, hypotonia and facial dysmorphism. Both the MED13L haploinsufficiency mutation and missense mutation were reported to be causative. It has also been reported that patients carrying missense mutations have more frequent epilepsy and show a more severe phenotype.Case presentationWe report a child with ID, speech impairment, severe motor developmental delay, facial deformity, hypotonia, muscular atrophy, scoliosis, odontoprisis, abnormal electroencephalogram (EEG), and congenital ureteropelvic junction obstruction (UPJO) combined with high ureter attachment. We used whole-exome sequencing (WES) to detect the genetic aberration of the child and found a de novo mutation, c.2605C > T (p.Pro869Ser), in the MED13L gene. Neither of her parents carried the mutation. Additionally, we review the literature and summarize the phenotypes and features of reported missense mutations. After reviewing the literature, approximately 17 missense mutations in 20 patients have been reported thus far. For 18 patients (including our case) whose clinical manifestations were provided, 100% of the patients had ID or developmental delay (DD). A total of 88.9, 83.3 and 66.7% of the patients had speech impairment, delayed milestones and hypotonia, respectively. A total of 83.3% of the patients exhibited craniofacial deformity or other dysmorphic features. Behavioral difficulties and autistic features were observed in 55.6% of the patients. Cardiac anomalies were seen in only 27.8% of the patients. Of these patients, 44.4% had epileptic seizures. Of the 17 mutations, 2 were located in the N-terminal domain, 8 were located in the C-terminal domain, and 1 was located in an α-helical sequence stretch. One of them was located in the MID domain of the MedPIWI module.ConclusionsWe report a new patient with a reported missense mutation, c.2605C > T (p.Pro869Ser), who exhibited some infrequent manifestations except common phenotypes, which may broaden the known clinical spectrum. Additionally, by reviewing the literature, we also found that patients with missense mutations have a higher incidence of seizures, MRI abnormalities, autistic features and cardiac anomalies. They also have more severe ID and hypotonia. Our case further demonstrates that Pro869Ser is a hotspot mutation of the MED13L gene.

Selected Metal Matrix Metalloproteinases and Tissue Inhibitors of Metalloproteinases as Potential Biomarkers for Tubulointerstitial Fibrosis in Children with Unilateral Hydronephrosis.

Renal tubulointerstitial fibrosis caused by congenital ureteropelvic junction obstruction (UPJO) may lead to the development of obstructive nephropathy (ON) and the impairment of kidney function. Hence, the identification of early biomarkers of this condition might be of assistance in therapeutic decisions. This study evaluates serum and urinary metalloproteinases MMP-1, MMP-2, and MMP-9 and tissue inhibitors of metalloproteinases TIMP-1 and TIMP-2 as potential biomarkers of ON in children with congenital unilateral hydronephrosis (HN) caused by UPJO. Forty-five (45) children with congenital HN of different grades of severity and twenty-one (21) healthy controls were enrolled in the study. Urinary and serum concentrations of MMP-1, MMP-2, MMP-9, TIMP-1 and TIMP-2 were measured using specific ELISA kits. The urinary excretions were expressed as biomarker/creatinine (Cr) ratios. To evaluate the extracellular matrix remodelling process activity, the serum and urinary MMP-1, -2, -9/TIMP-1, -2 ratios were also calculated. In comparison with the controls, patients with HN, independent of the grade, showed significantly increased median serum MMP-9, TIMP-1, and TIMP-2, median urinary MMP-9/Cr, and TIMP-2/Cr ratios. Lower median values of serum MMP-2/TIMP-1, MMP-9/TIMP-1 in patients with HN were also revealed. Additionally, higher urinary MMP-2/Cr, lower urinary MMP-2/TIMP-2, and lower serum MMP-9/TIMP-2 ratios were observed in patients with HN grades 3 and 4. Patients with ON diagnosed by renal scintigraphy had a significantly higher median serum MMP-9 concentration and lower median serum MMP-9/TIMP-1, -2 ratios in comparison with those without this condition. Patients with nonglomerular proteinuria had a significantly higher median serum TIMP-1 concentration, a higher median urinary TIMP-2/Cr ratio, and a lower serum MMP-9/TIMP-1 ratio compared to those without this symptom. The relationship between the measured biomarkers and the relative function of the obstructed kidney showed no correlations. The ROC curve analysis showed a promising diagnostic profile for the detection of ON for serum MMP-9 and the serum MMP-9/TIMP-1 and MMP-9/TIMP-2 ratios. In conclusion, the results of this study suggest that patients with HN, particularly with grades 3 and 4, are at higher risk of renal tubulointerstitial fibrosis. The noninvasive markers of this condition considered are urinary MMP-2/Cr and MMP-9/Cr, serum MMP-9, serum and urinary MMP-2, MMP-9/TIMP-1, -2. Additionally, serum MMP-9 and MMP-9/TIMP-1, -2 may become promising markers of ON.

ITRAQ-based proteomics and in vitro experiments reveals essential roles of ACE and AP-N in the renin–angiotensin system-mediated congenital ureteropelvic junction obstruction

ObjectiveUreteropelvic junction obstruction (UPJO) is a common renal obstructive disorder, but its pathogenic mechanisms remain largely unclear. We aimed to investigate the potential involvement of the renin–angiotensin system in congenital UPJO pathogenesis. MethodsDifferentially expressed proteins in exosomes isolated from amniotic fluid of patients with congenital UPJO were characterized using iTRAQ (isobaric tags for relative and absolute quantification)-based proteomics. The expressions of angiotensin-converting enzyme (ACE) and aminopeptidase N (AP-N) in HK2 cells were inhibited by quinapril and siRNA, respectively. Cell proliferation and reactive oxygen species were measured by EdU staining and flow cytometry, respectively. Gene expression was detected by Western blot or qRT-PCR. The inflammatory factors were measured through ELISA. Mice that underwent unilateral ureteral obstruction were used as the animal model. ResultsThe identity of exosomes from amniotic fluids was confirmed by the expression of CD9 and CD26. In total, 633 differentially expressed proteins were identified in the amniotic fluid-derived exosomes from patients with UPJO, including 376 up- and 257 down-regulated proteins associated with multiple biological processes. Of them, ACE and AP-N were significantly decreased in the amniotic fluid exosomes. Inhibition of ACE and AP-N resulted in suppressed cell proliferation; repressed IARP, AT1R, and MAS1 expression; elevated ROS production; and increased IL-1β, TNF-α, and IL-6 levels in HK2 cells. Decreased ACE expression and elevated IL-1β levels were also observed in the mouse model. ConclusionSuppression of ACE and AP-N expression mediates congenital UPJO pathogenesis by repressing renal tubular epithelial proliferation, promoting ROS production, and enhancing inflammatory factor expression.

Case report: a 33-year-old man with recurrent ureteropelvic junction obstruction

Introduction: Ureteropelvic Junction Obstruction (UPJO) is the obstruction of upper urinary tract, present in fetal life until adulthood. Congenital UPJO mostly results from an intrinsic process with dilatation of renal collecting system. Acquired UPJO in adulthood may present without dilatation of renal collecting system, correlated with urolithiasis, infection, postoperative peripyelo-inflammatory fibrotic complication, and urothelial malignancy. In the past, Pyeloplasty, classic dismembered (Anderson-Hynes) or laparoscopic, is the gold standard treatment. In this study we would like to report and discuss comprehensive etiologies, diagnostic, and its treatment based from real case.Case Presentation: We report case: a 33-year-old Indonesian man with left flank colic pain (VAS:8/10), nausea, and hematuria that did not disappear with medication. He had stone history in right proximal ureter with hydronephrosis and hydroureter, Ureterorenoscopy had been performed two years ago. Other vital signs and hemodynamics were stable. Abdominal USG demonstrated bilateral hydronephrosis and hydroureter. Abdominal X-Ray showed right ureter stone. Right-Left Ureterorenoscopy + Double-J-Stent were performed. The diagnosis was Bilateral Nephrolithiasis, Stenosis, and Proximal Ureter Obstruction leading to Recurrent UPJO. Three months later there was still pain, the patient was undergone Cystoscopy + URS + Laser Endopyelotomy + DJ-Stent Replacement (from 6 to 7 Fr). One month later, DJ-stents were removed but the stenotic ureter remained so Laparoscopic End-to-End Proximal Ureter Anastomosis Resection and DJ-stent Insertion were finally performed. Patient’s complaints were slightly reduced.Conclusion: The Recurrent UPJO cause inadequate urinary flow leading to renal impairment. Clinical signs, symptoms, etiologies, and diagnostic imaging have significant role in determining the diagnosis. Early surgical intervention is the definitive treatment to reduce renal damage and its complications. Further researches to establish the causes, clinical manifestations, diagnostic imagings, and alternative treatments of UPJO are highly expected.

Use of double-J ureteric stents post-laparoscopic pyeloplasty to treat ureteropelvic junction obstruction in hydronephrosis for pediatric patients: a single-center experience.

ObjectivesWe aimed to investigate the safety concerns associated with placing double-J ureteric stents post-laparoscopic pyeloplasty surgery for congenital ureteropelvic junction obstruction (UPJO) and hydronephrosis.MethodsA total of 1349 patients with postoperative double-J stent placement at our center were included. Clinical variables for enrolled patients were collected by two independent authors. We compared clinical variables and the efficacy of stenting post-laparoscopic pyeloplasty.ResultsThe mean age of the patients was 4.23 ± 2.39 years. A total of 58.49% of patients were diagnosed with left UPJO with hydronephrosis and 33.95% were diagnosed with right UPJO. Furthermore, 7.56% of patients had bilateral UPJO. In all cases, 96.96% of indwelling double-J stents were successfully removed 4 weeks post-surgery. A total of 3.04% of the patients still required further management, including stent migration to the renal pelvis (0.37%), stent migration to the bladder (0.30%), prolapse of the stent through the ureter (0.15%), blockage of stents (1.85%), and fouling of stents (0.37%).ConclusionsDouble-J ureteric stents used after laparoscopic pyeloplasty for treating UPJO in hydronephrosis for pediatric patients is a safe, feasible, and beneficial method, which can be recommended for routine procedures. However, caution should be practiced for follow-up and removal using this method.