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Background: A well-established definition of obstruction in the setting of congenital hydronephrosis is lacking. Multiple imaging modalities and radiographic characteristics or parameters have been described to help confirm the diagnosis of ureteropelvic junction obstruction (UPJO). We sought to evaluate the change in anterior-posterior renal pelvic diameter (APRPD) following furosemide administration in patients with unilateral hydronephrosis and confirmed UPJO on functional magnetic resonance urography (fMRU) who underwent pyeloplasty., Materials and Methods: There were 49 patients who met inclusion criteria (11 females, 38 males; average age 2.2 years, SD 3.4 years) from February 2006 to September 2020, diagnosed with unilateral hydronephrosis (SFU Grade 3-4) who underwent fMRU prior to pyeloplasty for confirmed UPJO. 29 of the included patients also underwent a post-pyeloplasty fMRU. A weight-adjusted dose of 1 mg/kg of furosemide (max 20 mg/kg) was administered intravenously. Two board-certified pediatric radiologists measured the APRPD of the obstructed and non-dilated kidneys prior to pyeloplasty and APRPD of the hydronephrotic kidneys on the post-pyeloplasty follow up fMRUs. Measurements were performed on images prior to and approximately 30 min following furosemide injection., Results: The average APRPD before furosemide injection in the obstructed kidney prior to pyeloplasty was 26.3 mm (SD 9.0 mm) compared to the non-dilated (not obstructed) kidney measurement of 5.1 mm (SD 3.6 mm) (p < 0.001). Following administration of furosemide, the average APRPD was 31.4 mm (SD 8.8 mm) in the obstructed kidney, and 7.8 mm (SD 4.1 mm) in the non-dilated kidney (p < 0.001). After pyeloplasty, the pre-furosemide APRPD measurement was 17.8 mm (SD 11 mm), which was significantly less compared to the pre-pyeloplasty APRPD (p < 0.001). The post-pyeloplasty, post-furosemide APRPD measurement was 25.8 mm (SD 12 mm), also significantly less compared to the pre-pyeloplasty measurement (p = 0.02). The changes in APRPD in the obstructed kidney prior to pyeloplasty was 5.1 mm (SD 3.5 mm) and after pyeloplasty was 8 mm (SD 4.6 mm) (p = 0.002). Change in APRPD in the non-dilated kidney was 2.7 mm (SD 2.3 mm). Percent APRPD change in the obstructed kidney was 22.9% (SD 18.5%), which was significantly less than 33.3% (SD 22.1%) in the post-pyeloplasty kidney (p = 0.028) and 82.8% (SD 87.9%) in the non-dilated kidney (p < 0.001)., Conclusions: A relatively smaller change in APRPD on fMRU following administration of furosemide in the setting of UPJO may serve as another predictive characteristic of obstructed kidneys., Competing Interests: Conflict of interest Dr. Kirsch is a consultant for Palette Life Sciences, a speaker for Intuitive Surgical, and is the Co-founder and CEO of Global Continence, Inc. Dr. Linam, Dr. Janssen, Dr. Cho, and Ms. Stone have no disclosures., (Copyright © 2023 Journal of Pediatric Urology Company. All rights reserved.)

Rationale: Mesoscopic visualization of the main anatomical structures of the whole kidney in vivo plays an important role in the pathological diagnosis and exploration of the etiology of hydronephrosis. However, traditional imaging methods cannot achieve whole-kidney imaging with micron resolution under conditions representing in vivo perfusion. Methods: We used in vivo cryofixation (IVCF) to fix acute obstructive hydronephrosis (unilateral ureteral obstruction, UUO), chronic spontaneous hydronephrosis (db/db mice), and their control mouse kidneys for cryo-micro-optical sectioning tomography (cryo-MOST) autofluorescence imaging. We quantitatively assessed the kidney-wide pathological changes in the main anatomical structures, including hydronephrosis, renal subregions, arteries, veins, glomeruli, renal tubules, and peritubular functional capillaries. Results: By comparison with microcomputed tomography imaging, we confirmed that IVCF can maintain the status of the kidney in vivo . Cryo-MOST autofluorescence imaging can display the main renal anatomical structures with a cellular resolution without contrast agents. The hydronephrosis volume reached 26.11 ± 6.00 mm 3 and 13.01 ± 3.74 mm 3 in 3 days after UUO and in 15-week-old db/db mouse kidneys, respectively. The volume of the cortex and inner stripe of the outer medulla (ISOM) increased while that of the inner medulla (IM) decreased in UUO mouse kidneys. Db/db mice also showed an increase in the volume of the cortex and ISOM volume but no atrophy in the IM. The diameter of the proximal convoluted tubule and proximal straight tubule increased in both UUO and db/db mouse kidneys, indicating that proximal tubules were damaged. However, some renal tubules showed abnormal central bulge highlighting in the UUO mice, but the morphology of renal tubules was normal in the db/db mice, suggesting differences in the pathology and severity of hydronephrosis between the two models. UUO mouse kidneys also showed vascular damage, including segmental artery and vein atrophy and arcuate vein dilation, and the density of peritubular functional capillaries in the cortex and IM was reduced by 37.2% and 49.5%, respectively, suggesting renal hypoxia. In contrast, db/db mouse kidneys showed a normal vascular morphology and peritubular functional capillary density. Finally, we found that the db/db mice displayed vesicoureteral reflux and bladder overactivity, which may be the cause of hydronephrosis formation. Conclusions: We observed and compared main renal structural changes in hydronephrosis under conditions representing in vivo perfusion in UUO, db/db, and control mice through cryo-MOST autofluorescence imaging. The results indicate that cryo-MOST with IVCF can serve as a simple and powerful tool to quantitatively evaluate the in vivo pathological changes in three dimensions, especially the distribution of body fluids in the whole kidney. This method is potentially applicable to the three-dimensional visualization of other tissues, organs, and even the whole body, which may provide new insights into pathological changes in diseases., Competing Interests: Competing Interests: The authors have declared that no competing interest exists., (© The author(s).)

Background: Hydronephrosis and renal impairment may persist even after relieving an obstruction, particularly in cases of chronic obstruction. Obstruction can cause fibrotic changes of the ureter, potentially contributing to long-term kidney damage., Objective: To characterise pathophysiological changes of obstructed ureters with focus on inflammatory responses triggering fibrosis and potential impairment of ureteral function., Design, Setting, and Participants: Eighty-eight mice were randomly assigned to unilateral ureteral obstruction (UUO) for 2 d, UUO for 7 d, and UUO for 7 d followed by 8 d of recovery, or a control group (no prior surgical intervention)., Outcome Measurements and Statistical Analysis: Peristaltic rate was determined over 2 min by direct visualisation with a microscope, while hydronephrosis was assessed by ultrasound. Obstructed and contralateral ureters were harvested, and underwent histopathological evaluation. We quantified 44 cytokines/chemokines, and five matrix metalloproteases using Luminex technology. Cell composition was characterised via immunofluorescence. Statistical significance was assessed using Welch analysis of variance, Kruskal-Wallis test, and Dunnett's T3 multiple comparison test., Results and Limitations: Obstruction resulted in hydronephrosis and significantly impaired peristalsis. Marked fibrosis was observed in lamina propria, muscle layer, and adventitia. Connective tissue in obstructed ureters showed hyperaemia and leucocyte infiltration. Unsupervised hierarchical clustering demonstrated different cytokine/chemokine patterns between groups. Ureters obstructed for 7 d followed by recovery were notably different from other groups. Inflammatory cytokines, chemoattractants, and matrix metalloproteases increased significantly in obstructed ureters. Contralateral unobstructed ureters showed significantly increased levels of chemokines and matrix metalloproteases. Immunofluorescence confirmed activation of T cells, Th1 and Th2 cells, and M1 macrophages in obstructed and contralateral ureters, and a shift to M2 macrophages following prolonged obstruction., Conclusions: Ureteral obstruction triggers severe inflammation and fibrosis, which may irreversibly impair ureteral functionality. Function of the unobstructed contralateral ureter may be regulated by a systemic immune response as a result of the obstruction., Patient Summary: Here, we studied in more detail the way the ureter responds to being blocked. We conclude that a strong immune response is activated by the blockage, leading to changes in the structure of the ureter possibly impacting function, which may not be reversible. This immune response also spreads to the opposite ureter, possibly allowing it to change its function to compensate for the reduced functionality of the blocked ureter., (Copyright © 2022 European Association of Urology. Published by Elsevier B.V. All rights reserved.)

Severe hydronephrosis increases the risk of urinary tract infection and irretrievable renal fibrosis. While TGFβ1-mediated fibrotic changes in proximal tubular epithelial cells and fatty acid oxidation (FAO) deregulation contribute to renal fibrosis and hydronephrosis. Firstly, a few elements were analyzed in this paper, including differentially-expressed long non-coding RNAs (lncRNAs), and miRNAs correlated to CPT1A, RXRA, and NCOA1. This paper investigated TGFβ1 effects on lncRNA FABP5P3, CPT1A, RXRA, and NCOA1 expression and fibrotic changes in HK-2 cells and FABP5P3 overexpression effects on TGFβ1-induced changes. Moreover, this paper predicted and proved that miR-22 binding to lncRNA FABP5P3, 3'UTR of CPT1A, RXRA, and NCOA1 was validated. The dynamic effects of the FABP5P3/miR-22 axis on TGFβ1-induced changes were investigated. A Renal fibrosis model was established in unilateral ureteral obstruction (UUO) mice, and FABP5P3 effects were investigated. Eventually, this paper concluded that TGFβ1 inhibited lncRNA FABP5P3, CPT1A, RXRA, and NCOA1 expression, induced fibrotic changes in HK-2 cells, and induced metabolic reprogramming within HK-2 cells, especially lower FAO. FABP5P3 overexpression partially reversed TGFβ1-induced changes. miR-22 targeted lncRNA FABP5P3, CPT1A, RXRA, and NCOA1. LncRNA FABP5P3 counteracted miR-22 inhibition of CPT1A, NCOA1, and RXRA through competitive binding. TGFβ1 stimulation induced the activation of TGFβ/SMAD and JAG/Notch signaling pathways; Nocth2 knockdown reversed TGFβ1 suppression on lncRNA FABP5P3. FABP5P3 overexpression attenuated renal fibrosis in unilateral ureteral obstruction mice. The LncRNA FABP5P3/miR-22 axis might be a potent target for improving the FAO deregulation and fibrotic changes in proximal TECs under TGFβ1 stimulation.

Background BK polyomavirus-associated nephropathy (BKPyVAN) is an important cause of chronic renal allograft dysfunction. However, US features indicative of BKPyVAN have not been fully evaluated. Purpose To assess the value of high-frequency US for the diagnosis of BKPyVAN in kidney transplant recipients. Materials and Methods In this prospective cohort study, participants who tested positive for BK viruria after kidney transplant from September 2019 to January 2021 were evaluated with high-frequency US 1 day before biopsy. Clinical characteristics and US features were compared between participants with and without BKPyVAN. Significant predictors associated with BKPyVAN were determined using logistic regression analyses. The area under the receiver operating characteristic curve (AUC) was used to evaluate diagnostic performance. Results A total of 105 participants who underwent kidney transplant (mean age, 38 years ± 11 [SD]; 63 men) were evaluated; 45 participants were diagnosed with BKPyVAN. Multivariable analysis demonstrated that eccentric hydronephrosis and subcapsular hypoechoic areas were independent factors for BKPyVAN. The AUC for predicting BKPyVAN according to subcapsular hypoechoic areas was 0.66 (95% CI: 0.55, 0.77), with a specificity of 92% (55 of 60 participants). The AUC of combined US (eccentric hydronephrosis plus subcapsular hypoechoic area) and clinical (urine BKPyV DNA load [BKPyV-DNA] plus BK viremia) features was 0.90, with a specificity of 92% (55 of 60 participants). Parenchymal hyperechoic and subcapsular hypoechoic areas were independent factors for differentiating BKPyVAN from transplant rejection. The pooled specificity of subcapsular hypoechoic areas was 96% (21 of 22 participants), with an AUC of 0.67 (95% CI: 0.54, 0.80). For the combination of US (parenchymal echogenicity plus subcapsular hypoechoic area) and clinical (urine BKPyV-DNA plus time since transplant) features, the AUC reached 0.92 and specificity was 82% (18 of 22 participants). Conclusion High-frequency US characteristics are valuable for diagnosing BK polyomavirus-associated nephropathy (BKPyVAN) and distinguishing BKPyVAN from rejection in kidney transplant recipients. © RSNA, 2022 Online supplemental material is available for this article .

Background: Hodgkin lymphoma (HL) is predominantly a nodal disease with extranodal presentation being uncommon. Presentation with neurological symptoms is not uncommon in adult patients with HL. Subdiaphragmatic involvements are less common especially in childhood. In the literature, there has been no case which presented with both spinal cord compression and bilateral hydronephrosis in pediatric patients with HL., Observation: We report a 9-year-old boy diagnosed with HL who presented with bilateral hydronephrosis and epidural involvement., Conclusion: Differential diagnosis of abdominal mass in patients presenting with spinal cord compression and/or hydronephrosis should include HL. Retrograde J ureteral stenting is the treatment of choice for malignant ureteral obstruction., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Rationale: Ureteral obstruction of the graft kidney is a common complication of kidney transplantation. However, ureteral obstruction caused by inguinal hernia has rarely been reported. We present a rare case of ureteral obstruction with allograft dysfunction caused by an inguinal hernia., Patient Concerns: A 76-year-old man, who was a renal transplant recipient, presented with bilateral pitting oedema, reduced urine output, and right inguinal hernia., Diagnoses: Abdominal computed tomography revealed severe hydroureteronephrosis of the kidney allograft. A right inguinal hernia with ureteral incarceration was observed., Interventions: The patient underwent graft percutaneous nephrostomy, followed by antegrade insertion of a double-J tube (DJ). Gradual improvement was observed in his renal function. Right inguinal herniorrhaphy was performed 5 days later., Outcomes: The renal function returned to normal after percutaneous nephrostomy and insertion of the DJ. A right inguinal direct-type hernia with ureter adhesion to the hernial sac was observed during the surgery. The posterior wall defect was repaired by the McVay technique. The DJ was removed after 1 month. The patient's renal function remained stable at 6-month follow-up., Lessons: The orientation of the graft kidney has a significant influence on the location of the ureter. Upward orientation of the hilum will result in superficial location of the ureter, rendering it close to the hernial sac and susceptible to incarceration. The transplant surgeon should be aware of such a presentation of graft dysfunction with inguinal hernia to prevent a delay in the diagnosis and graft loss., Competing Interests: The authors have no funding and conflicts of interests to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Laparoscopic nephrectomy in a giant hydronephrotic nonfunctioning kidney occupying the most of the abdomen is challenging task. To overcome the difficulty in laparoscopic dissection of such kidneys we described a technique "Pneumorenum," wherein the kidney is decompressed by suction through a 5 mm trocar inserted directly into kidney under vision. Following suction decompression, the kidney is inflated later during dissection with carbon dioxide gas to moderate size to do the dissection in an easy manner., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Introduction: In this study, we aimed to investigate the correlation between Cajal cell density and preoperative and postoperative radiological and scintigraphic parameters in ureteropelvic junction obstruction (UPJO)., Methods: The study group consisted of 41 renal units (38 consecutive patients; 13 female and 25 male) surgically treated for UPJO. UPJ specimens from patients were immuno-stained with CD117 (c-kit) antibody for interstitial Cajal cells (ICCs). The relation between Cajal cell density and preoperative and postoperative radiological and scintigraphic parameters was evaluated., Results: The mean age of the patients was 8.52 ± 8.86 (0-35) years. The density of Cajal cells was defined in 2 groups for convenient analysis as 0-5 cells (low) in 19 (46.3%) patients and >5 cells (moderate-high) in 22 (53.6%). There was significant difference between the preoperative and postoperative anteroposterior diameters of the related kidneys in both Cajal groups (p = 0.001-low, p = 0.000-moderate-high) independent of Cajal cell density. Regression in hydronephrosis postoperatively was determined in both Cajal groups (77.8%-low, 64.7%-moderate-high); however, there was no difference between them (p = 0.39). Preoperative T1/2 was significantly longer in the low Cajal group (p = 0.02). Postoperative T1/2 decreased in both low (p = 0.000) and moderate-high (p = 0.001) Cajal groups, but no difference was found between them (p = 0.24). There was significant improvement in the kidney differential function after surgery in the low Cajal density group (p = 0.015) while there was no correlation between the scintigraphic success or improvement and Cajal cell density (p = 0.51)., Discussion/conclusion: ICC deficiency/density could not be shown as a predictive factor for the determination of success rate of pyeloplasty. Despite the lack of any evidence for the degree of deficiency as an indicator for the severity of obstruction and prediction of surgical success, further studies are needed for confirmation., (© 2021 S. Karger AG, Basel.)

Background: Several studies revealed alterations of single sphingolipid species, such as chain length-specific ceramides, in plasma and serum of patients with kidney diseases. Here, we investigated whether such alterations occur in kidney tissue from patients and mice suffering from renal fibrosis, the common endpoint of chronic kidney diseases., Methods: Human fibrotic kidney samples were collected from nephrectomy specimens with hydronephrosis and/or pyelonephritis. Healthy parts from tumor nephrectomies served as nonfibrotic controls. Mouse fibrotic kidney samples were collected from male C57BL/6J mice treated with an adenine-rich diet for 14 days or were subjected to 7 days of unilateral ureteral obstruction (UUO). Kidneys of untreated mice and contralateral kidneys (UUO) served as respective controls. Sphingolipid levels were detected by LC-MS/MS. Fibrotic markers were analyzed by TaqMan® analysis and immunohistology., Results: Very long-chain ceramides Cer d18:1/24:0 and Cer d18:1/24:1 were significantly downregulated in both fibrotic human kidney cortex and fibrotic murine kidney compared to respective control samples. These effects correlate with upregulation of COL1α1, COL3α1 and αSMA expression in fibrotic human kidney cortex and fibrotic mouse kidney., Conclusion: We have shown that very long-chain ceramides Cer d18:1/24:0 and Cer d18:1/24:1 are consistently downregulated in fibrotic kidney samples from human and mouse. Our findings support the use of in vivo murine models as appropriate translational means to understand the involvement of ceramides in human kidney diseases. In addition, our study raises interesting questions about the possible manipulation of ceramide metabolism to prevent progression of fibrosis and the use of ceramides as potential biomarkers of chronic kidney disease., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Giant hydronephrosis in adults is a rare entity. It is defined as an extensive dilatation of the pyelocaliceal cavities occupying a large part of the abdominal cavity. Giant hydronephrosis is usually due to pelvi-ureteric junction obstruction and is usually diagnosed in children and infants. Ureterocele, which is a cystic dilatation of the terminal ureter, often drains the upper part of the kidney in patients with a duplex system. Massive hydronephrosis in a patient with duplex system and obstructive ureterocele was described by Aeron et al. in 2017. A thorough search of the major medical databases disclosed that no other cases have been reported since. We describe a second case of unilateral complete duplex system with ureterocele and massive hydronephrosis of the upper moiety in an adult man with intermittent abdominal pain associated with constipation and a decrease in appetite. The renal function of the left kidney was 8% of total function by radionuclide renal scan. The patient subsequently underwent left laparoscopic nephrectomy.

Antenatal hydronephrosis (AHN) is the most frequently detected abnormality by prenatal ultrasonography. Differential diagnosis of AHN includes a wide variety of congenital abnormalities of the kidney and urinary tract ranging from mild abnormalities such as transient or isolated AHN to more important ones as high-grade congenital vesicoureteral reflux or ureteropelvic junction obstruction. It is well known that the outcome depends on the underlying etiology. Various grading systems have been proposed for the classification of AHN on prenatal and postnatal ultrasonography. Mild isolated AHN represents up to 80% of cases, is considered to be benign, and majority of them resolve, stabilize, or improve during follow-up. Controversies exist regarding the diagnosis and management of some important and severe causes of AHN such as high-grade vesicoureteral reflux and ureteropelvic junction obstruction. Current approach is becoming increasingly conservative during diagnosis and follow-up of these patients with less imaging and close follow-up. However, there is still no consensus regarding the clinical significance, postnatal evaluation, and management of infants with AHN. The aim of this review is to discuss the controversies and provide an overview on the management of AHN.

Competing Interests: Competing interests: None declared.

Purpose: Ureteropelvic junction obstruction (UPJO) is the most frequent cause of congenital hydronephrosis in child. To better investigate the molecular mechanisms of this pathological process, the stenotic ureter proteome of UPJO in infants is compared with their own normal pre-stenotic segments., Experimental Design: Data independent acquisition-based proteomics are performed to compare proteome between pre-stenotic and stenotic ureter from nine UPJO infants. Gene ontology analysis, hierarchical cluster analysis, and network interaction are performed to characterize biological functions of significantly altered proteins. Selected significantly altered proteins are validated by western blot on another three UPJO infants., Results: 15 proteins are up-regulated and 33 proteins are down-regulated during stenotic pathology. Significantly altered proteins are involved in decreased extracellular matrix and cytoskeleton organization, increased regulation of oxidative activity, and altered inflammatory associated exocytosis. Significant expression of biglycan, fibulin-1, myosin-10, cytochrome b5 are validated providing possible mechanism in UPJO which could be associated impaired smooth muscle cell, epithelial integrity, and increased oxidative stress. CONCLUSIONS AND CLINICAL RELEVANCE: This study provides molecular evidence of dysregulated extracellular matrix organization, impaired smooth muscle cell, and oxidative stress during UPJO pathology, indicating that biglycan, fibulin-1, myosin-10, cytochrome b5 might reflect the pathology of UPJO., (© 2020 Wiley-VCH GmbH.)

Background: Antenatally diagnosed urinary tract dilatation (UTD) still burdens healthcare providers and parents. This study was conducted to establish long-term outcome in an unselected group of children with antenatally detected UTD., Methods: Seventy-one out of 103 children born in 2003-2005 and diagnosed with antenatal UTD agreed to participate in a 12-15-year follow-up including blood and urine samples, a kidney ultrasound exam, and kidney scintigraphy. The records were searched for previous urinary tract infections., Results: Among children with an anteroposterior diameter (APD) ≤ 7 mm and no calyceal, kidney, ureteral, or bladder pathology in the early postnatal period, no one tested had reduced estimated glomerular filtration rate (eGFR), albuminuria, or UTD at the follow-up at a mean age of 13.6 years. One child had kidney damage not affecting kidney function. Among children with postnatal APD > 7 mm and/or kidney, calyceal, ureteral, or bladder pathology, 15% had persistent UTD and 32-39% (depending on the method used) had kidney damage. Major postnatal urinary tract ultrasound abnormalities and a congenital anomalies of the kidney and urinary tract (CAKUT) diagnosis were factors associated with an increased risk for permanent kidney damage (odds ratios 8.9, p = 0.016; and 14.0, p = 0.002, respectively). No one had reduced eGFR. One child (1/71, 1%) had a febrile urinary tract infection after the age of 2., Conclusions: We conclude that in children with postnatal APD ≤ 7 mm, no calyceal dilatation, normal bladder, ureters, and kidney parenchyma, the outcome is excellent. There is no need for long-term follow-up in these patients.

We aimed to assess the role of computerized tomography attenuation values (Hounsfield unit-HU) for differentiating pyonephrosis from hydronephrosis and for predicting postoperative infectious complications in patients with obstructive uropathy. We analysed data from 122 patients who underwent nephrostomy tube or ureteral catheter placement for obstructive uropathy. A radiologist drew the region of interest for quantitative measurement of the HU values in the hydronephrotic region of the affected kidney. Descriptive statistics and logistic regression models tested the predictive value of HU determination in differentiating pyonephrosis from hydronephrosis and in predicting postoperative sepsis. A HU cut-off value of 6.3 could diagnose the presence of pyonephrosis with 71.6% sensitivity and 71.5% specificity (AUC 0.76; 95%CI: 0.66-0.85). At multivariable logistic regression analysis HU ≥ 6.3 (p ≤ 0.001) was independently associated with pyonephrosis. Patients who developed sepsis had higher HU values (p ≤ 0.001) than those without sepsis. A HU cut-off value of 7.3 could diagnose the presence of sepsis with 76.5% sensitivity and 74.3% specificity (AUC 0.79; 95%CI: 0.71-0.90). At multivariable logistic regression analysis, HU ≥ 7.3 (p ≤ 0.001) was independently associated with sepsis, after accounting for clinical and laboratory parameters. Measuring HU values of the fluid of the dilated collecting system may be useful to differentiate pyonephrosis from hydronephrosis and to predict septic complications in patients with obstructive uropathy.

Objective: Acute appendicitis is known for one of the most common cause of acute abdomen. However, it is still challenging to clearly distinguish complicated from uncomplicated appendicitis preoperatively. We investigated the association between right hydronephrosis and complicated appendicitis compared with other computed tomography (CT) findings of acute appendicitis., Materials and Methods: 1275 patients who pathologically diagnosed with acute appendicitis were included. They were classified into the complicated appendicitis and uncomplicated appendicitis groups based on their histopathological results. We reviewed their CT findings, including right hydronephrosis, appendiceal wall defect, periappendiceal abscess, extraluminal or intraluminal free air, appendicolith, appendiceal dilatation, appendiceal wall thickening, periappendiceal infiltration, periappendiceal fluid collection, and ascites. Comparison between the two groups was performed and the diagnostic performance of each CT finding was evaluated. The location of appendix and periureteral extension of periappendiceal infiltration were evaluated in patients with right hydronephrosis., Results: Out of 1275 patients, 29 (2.3 %) patients showed right hydronephrosis. Right hydronephrosis was significantly related to complicated appendicitis with the odds ratio of 4.90 (95 % confidence interval, 1.62-14.83; P = 0.005). Right hydronephrosis yielded specificity of 99.3 %. All patients with right hydronephrosis accompanied with periappendiceal infiltration and 28 (96.6 %) patients showed periureteral extension. Subcecal and pelvic areas are the most common locations, with 11 (37.9 %) patients each., Conclusion: The presence of right hydronephrosis was specific finding for complicated appendicitis and it could be considered as one of indirect sign of complicated appendicitis., (Copyright © 2020. Published by Elsevier B.V.)

Objective: To determine predictive factors for periureteral fat invasion of ureteral cancer on computed tomography (CT) urography., Materials and Methods: Consecutive 77 patients with CT urography and surgically proven ureteral cancer were retrospectively evaluated. Three independent radiologists with different experiences in interpreting CT urography analyzed radiologic findings as follows: (a) periureteral infiltration (absent; equivocal; or present); (b) ureteral obstruction (complete obstruction or not); (c) lesion length; (d) lesion width; (e) hydronephrosis grade; and (f) renal atrophy (present or not). We analyzed univariate and multivariate logistic regression. We assessed sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of significant parameters., Results: Periureteral fat invasion was surgically identified in 33.8 % (26/77). For reader 1, periureteral infiltration (odds ratio [OR] = 3.330), complete ureteral obstruction (OR = 18.965), and renal atrophy (OR = 3.116) were significant in univariate analysis (p < 0.05), while only complete ureteral obstruction was significant in multivariate analysis (OR = 12.779; p = 0.018). For reader 2 (OR = 4.172; p = 0.019) and 3 (OR = 9.459; p = 0.035), only complete ureteral obstruction was significant in univariate analysis. Sensitivity, specificity, PPV, and NPV of complete ureteral obstruction were 96.2 % (25/26), 43.1 % (22/51), 46.3 % (25/54), and 95.7 % (22/23) for reader 1, 84.6 % (22/26), 43.1 % (22/51), 43.1 % (22/51), and 84.6 % (22/26) for reader 2, and 96.2 % (25/26), 27.5 % (14/51), 40.3 % (25/62), and 93.3 % (14/15) for reader 3, respectively., Conclusion: Complete ureteral obstruction on CT urography is highly sensitive for extra-ureteral extension of ureteral cancer. Ureteral cancer without complete ureteral obstruction may be less likely to invade periureteral fat., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Laminin alpha 5 (LAMA5) is a member of a large family of proteins that trimerise and then polymerise to form a central component of all basement membranes. Consequently, the protein plays an instrumental role in shaping the normal development of the kidney, skin, neural tube, lung and limb, and many other organs and tissues. Pathogenic mutations in some laminins have been shown to cause a range of largely syndromic conditions affecting the competency of the basement membranes to which they contribute. We report the identification of a mutation in the polymerisation domain of LAMA5 in a patient with a complex syndromic disease characterised by defects in kidney, craniofacial and limb development, and by a range of other congenital defects. Using CRISPR-generated mouse models and biochemical assays, we demonstrate the pathogenicity of this variant, showing that the change results in a failure of the polymerisation of α/β/γ laminin trimers. Comparing these in vivo phenotypes with those apparent upon gene deletion in mice provides insights into the specific functional importance of laminin polymerisation during development and tissue homeostasis., Competing Interests: Competing interestsA.J.M. has received research grant funding from Sanofi-Genzyme and has membership of an Advisory Board for Otsuka, neither of which are related to this project or work., (© 2020. Published by The Company of Biologists Ltd.)

A 66-year-old man was found dead at his home address. He had a history of poor nutrition with recent episodes of vomiting. At autopsy the decedent was cachexic with a body mass index (BMI) of 16.1. The major findings were of marked prostatomegaly (165 g) with obstruction to urine outflow resulting in dilatation and trabeculation of the bladder and bilateral hydroureteronephrosis. The bladder contained 1.5 l of yellow urine and the kidneys weighed only 109 g on the left and 125 g on the right (N = 140-160 g). Histological examination of the prostate revealed benign nodular hyperplasia. Biochemical analysis of vitreous humor demonstrated a creatinine of 1579 μmol/L (normal 45-90) and a urea of 133.4 mmol/L (normal 2.5-7.1). Despite genitourinary causes of sudden and/or unexpected death being rare in routine forensic practice, this case demonstrates significant underlying obstructive renal disease that resulted in sudden death and that had remained undiagnosed until the time of autopsy.

Objective: This study aimed to present our experience of concomitant management of renal calculi and recurrent ureteropelvic junction obstruction (UPJO) with percutaneous nephrolithotomy (PCNL) and antegrade balloon dilation., Methods: We retrospectively reviewed 31 patients who underwent PCNL and antegrade balloon dilation for treatment of renal calculi and recurrent UPJO. The inclusion criterion was the presence of UPJO after failed pyeloplasty with ipsilateral renal calculi. Success was defined as achievement of both symptomatic and radiographic resolution of any stones and obstruction., Results: All operations were successful without grade III or higher postoperative complications. A stone-free status was observed in all patients and the overall success rate of the procedure was 87.1% (27/31). The success rate of the procedure was significantly higher in patients with mild or moderate preoperative hydronephrosis (96%) than in those with high-grade preoperative hydronephrosis (50%). Moreover, the success rate of the procedure was lower in patients with poor preoperative renal function (0%) than in those with good or moderate renal function (93.1%)., Conclusion: Combined PCNL and antegrade balloon dilation management represents a safe and effective approach for patients with renal calculi and recurrent UPJO after failed pyeloplasty.

Background: Desmoid tumors (or aggressive fibromatosis) are locally infiltrative connective-tissue tumors that can arise in any anatomic location; they can be asymptomatic, or they can result in pain, deformity, swelling, and loss of mobility and/or threaten visceral organs with bowel perforation, hydronephrosis, neurovascular damage, and other complications. Existing clinical trial endpoints such as the Response Evaluation Criteria in Solid Tumors (version 1.1) and progression-free survival are inadequate in capturing treatment efficacy. This study was designed to develop a novel clinical trial endpoint by capturing patient-reported outcomes (PROs)., Methods: Following best practices in qualitative methodology, this study used concept elicitation (CE) interviews to explore desmoid patients' perspectives on key disease-related symptoms and impacts. Qualitative analysis was performed to determine the relative frequency and disturbance of symptoms and impacts as well as other characteristics of these concepts. A draft PRO scale was then developed and tested with cognitive interviewing. Information from the interviews was subsequently incorporated into the refined PRO scale., Results: CE interviews with desmoid patients (n = 31) helped to identify salient concepts and led to a draft scale that included symptom and impact scales. Cognitive interviews were completed with additional patients (n = 15) across 3 phases. Patient input was used to refine instructions, revise and/or remove items, and modify the response scale. This resulted in an 11-item symptom scale and a 17-item impact scale., Conclusions: This is the first disease-specific PRO instrument developed for desmoid tumors. The instrument is available as an exploratory endpoint in clinical trials. This study highlights the feasibility and challenges of developing PRO instruments for rare diseases., (© 2019 American Cancer Society.)

Purpose: Hydronephrosis due to ureteric obstruction (UO) is stage-defining at cervical cancer presentation but may occur after primary staging. We aimed to determine the incidence and review the presentation and management of UO in women with cervical cancer attending our center. Particular attention was paid to the evolving role of interventional radiology (IR) in management., Methods: Women with a new diagnosis of cervical cancer between January 2012 and December 2016 formed the cohort that was retrospectively reviewed from the oncology database and patient records., Results: There were 310 women diagnosed with cervical cancer; 240 were stages I/II and 70 were stages III/IV. Primary treatments were chemoradiotherapy (n = 168; 54.2%), surgery (n = 121; 39.0%), and palliative care alone (n = 21; 6.8%). UO occurred in 74 (23.9%); present at primary staging in 53 (71.6%) and arising after staging in 21 (28.4%). Primary interventions for hydronephrosis were IR (n = 50; 67.6%), cystoscopic stenting (n = 19; 25.7%), bowel urinary conduit construction (n = 2; 2.7%), and none (n = 3; 4.1%). For those who attended IR, the mean number of IR procedures was 2.2, range 1-7. Maximum serum creatinine was 303 μmol/L for women with UO at primary staging compared with 252 μmol/L for UO after staging (P = 0.267). Thirty-eight women experienced substantial morbidity related to UO. Stage-adjusted mortality risk was 2.3 times higher for UO cases compared with those without UO., Conclusions: UO is associated with substantial morbidity and survival disadvantage in cervical cancer and may present after primary cancer staging. We recommend renal biochemistry during routine follow-up. A majority of cervical cancer-associated UO cases are managed with IR in our center.

Background: Gastrointestinal atresias and urological defects are main causes of pediatric surgery in infants. As copy number variants (CNVs) have been shown to be involved in the development of congenital malformations, the aim of our study was to investigate the presence of CNVs in patients with gastrointestinal and urological malformations as well as the possibility of tissue-specific mosaicism for CNVs in the cohort., Methods: We have collected tissue and/or blood samples from 25 patients with anorectal malformations, esophageal atresia, or hydronephrosis, and screened for pathogenic CNVs using array comparative genomic hybridization (array-CGH)., Results: We detected pathogenic aberrations in 2/25 patients (8%) and report a novel possible susceptibility region for esophageal atresia on 15q26.3. CNV analysis in different tissues from the same patients did not reveal evidence of tissue-specific mosaicism., Conclusion: Our study shows that it is important to perform clinical genetic investigations, including CNV analysis, in patients with congenital gastrointestinal malformations since this leads to improved information to families as well as an increased understanding of the pathogenesis., (© 2019 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.)

The aim of this study is to evaluate the significance of renal pelvis aspiration (RPA) in the management of antenatal hydronephrosis (AHN). This study enrolled 15 AHN cases (one twin pregnancy) that necessitated RPA for AHN. Chromosomal abnormalities, gene disorders, and additional life-threatening congenital abnormalities were eliminated prior to intrauterine interventions. Urine analysis were performed for the evaluation of renal function. Normal renal function was observed in six neonates/infants (40%) (group 1), whereas impaired renal function and various type of urinary system anomalies were observed in 9 neonates/infants (60%) (group 2) during the short-term and longitudinal follow-up periods. There were statistically significant differences in the oligohydroamniosis rate, mean fetal urine sodium value, mean fetal urine β2-microglobulin, mean gestational week at birth, and mean birthweight values between the groups (P = 0.007, P < 0.001, P = 0.035, P < 0.001, and P = 0.001, respectively). Renal pelvis aspiration and urine analysis were substantial for the management of AHN in necessary cases. β2-microglobulin and sodium are clinically useful markers to detect the presence of severe renal damage due to obstructive uropathy and thus, important adjuvants in the proper selection of fetuses for further antenatal interventions., (© 2019 Japanese Teratology Society.)

Aim to illuminate the correlation between fetal hydronephrosis and postnatal urological abnormalities by a new classification system-"urinary tract dilation (UTD)" grade.Of 659 pregnancies screened by ultrasound, 34 cases were found with isolated fetal hydronephrosis, and enrolled in our study from 2017 to 2019. These 34 infants had been prospectively followed up to 6 months after birth by 3 times of urinary tract ultrasound. Hydronephrosis was graded in accordance with the UTD classification system.Among 34 infants with isolated fetal hydronephrosis. Twenty-four (70.6%) were classified as UTD A1 grade (mild hydronephrosis), and the other 10 (29.4%) were UTD A2-3 grade (moderate to severe hydronephrosis) by antenatal evaluation. After birth, all of the 24 infants (70.6%) with UTD A1 grade had normal ultrasonic findings of urinary tract; while the other 10 cases with UTD A2-3 grade had persistent abnormalities of urinary tract by postnatal assessment, sorted as UTD P1 grade (mild hydronephrosis) in 6 cases (17.6%), UTD P2 grade (moderate hydronephrosis) in 2 cases (5.9%) and UTD P3 grade (severe hydronephrosis) in 2 cases (5.9%). The most common postnatal urological abnormality of isolated fetal hydronephrosis was vesicoureteral reflux. During the follow-up period, 7 infants (20.6%) had urinary tract infection episodes and very few obtained positive cultures of the urine. Only 2 infants (5.9%) with the diagnosis of ureterovesical junction obstruction required surgery intervention, who were both classified as UTD P3 grade. None of the children with UTD P1 and P2 grades needed operation.The UTD classification system described the urinary tract both antenatally and postnatally, which could point out the correlation between fetal hydronephrosis and postnatal urological abnormalities. This new classification system is expected to be a good prognostic marker for fetal hydronephrosis.

The hydronephrosis, characterized by the dilation of the renal pelvicalyceal system with possible functional damage to the renal parenchyma, is the most common congenital abnormality of the urinary system detected in utero through the prenatal ultrasound screening. (...).

Bladder-sparing therapies for the treatment of nonmetastatic muscle-invasive bladder cancers are included in both American and European guidelines. Numerous treatment approaches have been described, including partial cystectomy, radiation monotherapy, and radical transurethral resection. However, the most oncologically favorable and well-studied regimen employs a multimodal approach that consists of maximal transurethral resection of the bladder tumor followed by concurrent radiosensitizing chemotherapy and radiotherapy. This sequence, referred to as trimodal therapy (TMT), has been evaluated with robust retrospective comparative studies and prospective series, although a randomized trial comparing TMT with radical cystectomy has not been performed. Despite promising reports of 5-year overall survival rates of 50% to 70% in well-selected patients, relatively few patients qualify as ideal candidates for TMT. Specifically, contemporary series exclude patients who have clinical stage T3 disease, multifocal tumors, coexisting carcinoma in situ, or hydronephrosis. Herein, we review all forms of bladder-preserving therapies with an emphasis on TMT, highlighting the rationale of each component, survival outcomes, and future directions.

Objective: To present a rare case of "huge" hydronephrosis causing distortion of large vessels and formation of a thrombus in the inferior vena cava. Multidisciplinary treatment was applied with particular focus on pyeloplasty utilizing a robot-assisted laparoscopic approach., Methods: A 20-month-old male presented to the emergency room severely ill with abdominal pain, nausea, vomiting, and fever and was subsequently transferred to the intensive care unit, in septic shock. An abdominal ultrasound revealed a large multilobular cystic structure in the right hemiabdomen, which was initially interpreted as an infected mesenteric cyst. CT scan revealed a huge hydronephrotic kidney crossing the midline, causing a mass effect that compressed and distorted the vena cava laterally, in addition to a thrombus between the hepatic vein and right renal vein. Intravenous Ceftriaxone and Amikacin, as well as anticoagulation therapy with low molecular weight heparin (Enoxaparin) were initiated. A nephrostomy tube was inserted that drained 900 mL of purulent urine. A full hematology investigation including protein C, S, and antithrombin III was carried out, excluding factor V Leiden and prothrombin mutation. All values were in the normal range. Dimercaptosuccinic Acid (DMSA) scan showed 30% function on the affected kidney and Voiding Cystourethrogram (VCUG) excluded any bladder pathology or reflux. Subcutaneous Enoxaparin was continued for 3 months, maintaining antifactor Xa in the therapeutic range (0.7-1 IU/mL). Ultrasound Doppler of the vena cava showed full resolution of the thrombus. Robot-assisted laparoscopic pyeloplasty was performed and significant reduction of the renal pelvis was carried out, taking care to preserve the calyces. Postoperative ultrasound 4 months after surgery showed a complete resolution of the hydronephrosis., Conclusion: Giant hydronephrosis is a rare finding. Distortion of adjacent veins and formation of thrombosis should be kept in mind, as they are life threatening. A multidisciplinary collaboration is mandatory to ensure optimal treatment., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Abdominal and pelvic computed tomography (CT) scans can be complex to interpret but sometimes significant abnormalities are relatively easy to recognize. In emergencies it is beneficial if physicians and surgeons can identify significant pathology that may immediately change patient management. Early image interpretation will allow clinicians to alert radiologists to provide prompt urgent reports, facilitate early referral to other specialities or expedite emergency surgery. This article provides non-radiologists with a systematic approach to identifying emergency pathology on abdominal and pelvic CT scans. It reviews the relevant cross-sectional anatomy and discusses the CT appearances of bowel perforation, bowel obstruction, bowel ischaemia (gangrene), bleeding, appendicitis and hydronephrosis using illustrative examples from the authors' clinical practice. Underlying causes for these conditions and the importance of interpreting the radiological appearances in conjunction with the patient's clinical condition and history are discussed. The authors hope that by using the POGBAH acronym and a systematic approach readers will be able to identify emergency pathology on abdominal and pelvic CT which may improve patient care.

Background: There are limited data on the predictive value of the consensus urinary tract dilation (UTD) score with respect to subsequent clinical diagnoses. We sought to define the relationship between postnatal UTD risk score and clinical outcomes during childhood., Methods: Complete ultrasound image sets from a random selection of infants aged 0-90 days undergoing initial ultrasound at a single institution for prenatal hydronephrosis between 2012 and 2014 were assigned a UTD score by 1 pediatric urologist and 1 pediatric radiologist. Urinary tract dilation risk score was analyzed for association with a composite outcome comprising urinary tract infection, vesicoureteral reflux (VUR), ureteropelvic junction obstruction, non-refluxing megaureter (NRM), ureterocele, bladder outlet obstruction (BOO), and chronic kidney disease. Surgical intervention and resolution of UTD were evaluated separately. Descriptive and survival analyses were performed., Results: Urinary tract dilation scores for 494 subjects were P0 in 23.5%, P1 in 26.5%, P2 in 23.5%, and P3 in 26.5%. Seventy-four percent were male. Median age at initial imaging was 28 days; median follow-up was 19.8 months. The composite outcome occurred in 138 of 494 patients (27.9%) and varied significantly (p < 0.001) by UTD score: 11.2% for P0, 10.7% for P1, 29.3% for P2, and 58.8% for P3. On survival analysis (Summary Figure), higher UTD grade was significantly associated with the composite outcome (hazard ratio for P3 vs. P0 was 7.4 [95% CI: 3.44-15.92, p < 0.001]). Urinary tract infection and VUR diagnosis varied by UTD score (p = 0.03 and p < 0.001, respectively). Ureteropelvic junction obstruction was diagnosed (based on MAG3 results) in 6.3% of patients, 84% of whom were P3. Non-refluxing megaureter was diagnosed in 7.7%. Ureterocele and BOO were uncommon (1.4%, and 0.6%, respectively). Surgical intervention was also associated with UTD risk, with 46% of P3 undergoing surgery vs. 1% of P0, 1% of P1, and 6% of P2 (p < 0.001). Resolution of UTD occurred in 41% (median 10.1 months) and varied significantly by UTD risk (p < 0.001)., Discussion: Urinary tract dilation risk score is associated with clinical events, although ascertainment bias may influence some of the differences in outcomes, particularly for VUR, because VCUG utilization varied by the UTD group. The lack of any significant difference in outcomes between patients with UTD P0 versus P1 suggests that the P1 category could be eliminated as it does not meaningfully distinguish between outcome risk., Conclusions: Higher UTD risk scores are strongly associated with genitourinary diagnoses during the first two years of life., (Copyright © 2019 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

As a member of the cysteine protease family, cathepsin S (CTSS) serves an important role in diseases such as cancer, arthritis and atherosclerosis. Nevertheless, its role in renal fibrosis is unknown. In the present study, the effects of CTSS on renal fibrosis in mild (group M) and severe (group S) hydronephrosis were studied by reverse transcription‑-quantitative PCR (RT‑qPCR), western blot analysis (WB), Masson's trichrome staining and immunohistochemical staining in mouse models. The effects of CTSS on extracellular matrix (ECM) deposition and epithelial‑mesenchymal transition (EMT) and the potential mechanisms were further studied by RT‑qPCR and WB in transforming growth factor (TGF‑β1)‑stimulated TCMK‑1 cells. Compared with group N (no hydronephrosis), the expression levels of CTSS in the M and S groups were significantly higher, and a significant increase in ECM deposition was observed in the S group. In addition, compared with group N, the expression levels of TGF‑β1, α‑smooth muscle actin (α‑SMA), SMAD2, SMAD3, phosphorylated (p)SMAD2 and pSMAD3 in groups M and S were significantly higher, whereas the expression of E‑cadherin was significantly lower. Inhibition of CTSS expression increased the expression levels of TGF‑β1, α‑SMA, fibronectin, collagen‑I, SMAD2, SMAD3, pSMAD2 and pSMAD3, whereas E‑cadherin expression decreased. A significant increase in CTSS was observed in the TGF‑β1‑stimulated TCMK‑1 cell line. ECM deposition and EMT were also intensified. The opposite outcomes occurred after intervention with small interfering RNA targeting CTSS. In conclusion, CTSS affected EMT and the deposition of ECM. CTSS may mediate the regulation of fibrosis by the TGF‑β/SMAD signaling pathway. CTSS may serve an important role in the treatment of renal fibrosis.

Data regarding spontaneous resolution of mild hydronephrosis consist of different etiologies, and hence, it is heterogeneous. The aim of this study is to evaluate spontaneous resolution rates and the time to complete resolution of antenatally detected isolated hydronephrosis in patients with renal pelvis antero-posterior diameter (RPAPD) ≤ 20 mm. Retrospective chart review of patients who were admitted to our pediatric nephrology clinic for the evaluation of antenatal hydronephrosis between 2011 and 2017 was conducted. Patients that had hydronephrosis with RPAPD ≤ 20 mm, < SFU (Society for Fetal Urology) grade IV on initial postnatal evaluation, and ≥ 3 months of follow-up with at least two renal ultrasounds (US) were included. Complete resolution of hydronephrosis was regarded as SFU grade 0 with a RPAPD of < 7 mm. Patients with ureteric and lower urinary tract abnormalities were excluded. There were a total of 276 patients who met the inclusion criteria. Median follow-up was 16 months (range 3-96 months). Hydronephrosis completely resolved in 198 patients (71.7%). Surgery was not required in any of the patients; however, nine patients (3.3%) showed progression to higher grades of hydronephrosis or increase in AP diameter. Median time to complete resolution of hydronephrosis was 6 months (range 2-35 months) in our study. Those with AP diameter < 10 mm became normal in median of 5 months, while it took median 11 months for patients with AP diameter 10-20 mm to become normalized (p < 0.001).Conclusion: Isolated antenatal hydronephrosis with RPAPD ≤ 20 mm would spontaneously resolve in 71.7% of the cases. Resolution may be expected to happen in 3 years, while the majority will take place in the first year of life. What is Known: • Antenatal hydronephrosis comprises an important amount of clinical visits. • Spontaneous resolution rates differ for various etiologies. What is New: • Isolated antenatal hydronephrosis with RPAPD ≤ 20 mm completely resolves within 3 years in approximately 72% of the cases. • Resolution can be observed within the first year of life in most of the cases.

Pelvic lipomatosis is an uncommon disease with no clear etiology and it occurs secondary to deposition of a large amount of fatty tissue in the pelvis. This deposition causes compression to the rectum, bladder, and venous structures. Because of this compression, various symptoms, such as recurrent urinary infections, dysuria, tenesmus, and constipation, have mostly been reported. However, iliac or superior vena cava thrombosis secondary to vascular compression of pelvic lipomatosis is rare. This report describes a case of pelvic lipomatosis, which was associated with right-sided mild hydronephrosis and portal vein thrombosis.

Centromere-binding protein F (CENP-F) is a large and complex protein shown to play critical roles in mitosis and various other interphase functions. Previous studies have shown that the disruption of CENP-F function leads to detrimental effects on human development. Still, it is important to note the lack of studies focusing on the effects that the loss of this essential protein may have on specific adult organs. In the current study, we used a novel global knockout murine model to analyze the potential consequences deletion of CENP-F has on adult kidney structure and function. We discovered several structural abnormalities including loss of ciliary structure, tubule dilation, and disruption of the glomerulus. Along with these structural irregularities, renal dysfunction was also detected suggesting hydronephrosis and acute kidney injury in these knockout organs. Importantly, this is the first study linking CENP-F to kidney disease and hopefully these data will serve as a platform to further investigate the molecular mechanisms disrupted in the kidney by the loss of CENP-F. Anat Rec, 302:163-170, 2019. © 2018 Wiley Periodicals, Inc., (© 2018 Wiley Periodicals, Inc.)

Aim: The study aimed to analyze the musculature and innervations of pelviureteric junction (PUJ) complex and upper ureter by morphometry and immunohistochemistry in surgically resected specimens of PUJ obstruction and compares the findings with normal controls., Materials and Methods: Specimens of the PUJ were obtained from twenty patients with hydronephrosis due to PUJ obstruction undergoing surgical treatment in this unit from 2013 to 2015. In the control group, seven PUJ complexes were taken from patients who had nephrectomy due to trauma and Wilms tumor. The sections were stained with H and E stain and elastic von Gieson stain. For immunohistochemistry, the tissue sections were immunostained by an indirect immunoperoxidase method for S-100 and neuron-specific enolase., Results: Histopathologically, the predominant arrangement of muscle fibers was layered, i.e., a thick circular layer surrounded by two longitudinal layers on either side in PUJ of obstruction patients. S-100 immunoreactivity in the muscle layer showed dense neural innervation in PUJ complex of obstructed patients., Conclusions: Circular enhanced musculature may cause a sphincter-like activity with holding up of urine. The higher density of neuronal expression in PUJ might be responsible for causing constant contraction and intrinsic obstruction., Competing Interests: None

The primary aim of the present study was to investigate the potential effect of high‑pressure carbon dioxide (CO2) pneumoperitoneum on kidneys with severe hydronephrosis and to investigate the possible underlying mechanism. A total of 18 rabbits underwent a surgical procedure inducing severe hydronephrosis. Rabbits were then divided at random into three groups (n=6 each) and subjected to intraabdominal pressure of 0, 8 or 18 mmHg, respectively. CO2 inflation lasted for 90 min in the pneumoperitoneum groups. Oxidative stress was assessed by measurements of reactive oxygen species (ROS). Activation of apoptosis was analyzed by western blot analysis of B‑cell lymphoma 2 (Bcl‑2), Bcl‑2‑associated x protein (Bax), cytochrome c (Cyt c), caspase‑3 and caspase‑9 levels. In addition, TUNEL assay, hematoxylin and eosin (H&E) staining, measurement of mitochondrial membrane potential (MMP) and detection of changes to kidney ultramicrostructure were performed. In the 0 and 8 mmHg groups, all results were normal and similar. However, in the 18 mmHg group, the kidneys suffered oxidative damage and mitochondrial injuries, and increased ROS levels, lower MMP and mitochondrial vacuolization were observed. Furthermore, the mitochondrial/caspase‑dependent pathway of apoptosis was activated, as indicated by the apoptotic index, and the expression levels and translocation of Bax, Bcl‑2, Cyt c, caspase‑3 and caspase‑9. Therefore, it is concluded that high‑pressure CO2 pneumoperitoneum induces oxidative damage and apoptosis in rabbit kidneys with severe hydronephrosis, which is associated with the mitochondrial apoptotic pathway.

Congenital obstructive nephropathy is a major cause of chronic kidney disease (CKD) in children. The contribution of changes in the identity of renal cells to the pathology of obstructive nephropathy is poorly understood. Using a partial unilateral ureteral obstruction (pUUO) model in genetically modified neonatal mice, we traced the fate of cells derived from the renal stroma, cap mesenchyme, ureteric bud (UB) epithelium, and podocytes using Foxd1Cre, Six2Cre, HoxB7Cre , and Podocyte.Cre mice respectively, crossed with double fluorescent reporter (membrane-targetted tandem dimer Tomato (mT)/membrane-targetted GFP (mG)) mice. Persistent obstruction leads to a significant loss of tubular epithelium, rarefaction of the renal vasculature, and decreased renal blood flow (RBF). In addition, Forkhead Box D1 (Foxd1)-derived pericytes significantly expanded in the interstitial space, acquiring a myofibroblast phenotype. Degeneration of Sine Oculis Homeobox Homolog 2 (Six2) and HoxB7-derived cells resulted in significant loss of glomeruli, nephron tubules, and collecting ducts. Surgical release of obstruction resulted in striking regeneration of tubules, arterioles, interstitium accompanied by an increase in blood flow to the level of sham animals. Contralateral kidneys with remarkable compensatory response to kidney injury showed an increase in density of arteriolar branches. Deciphering the mechanisms involved in kidney repair and regeneration post relief of obstruction has potential therapeutic implications for infants and children and the growing number of adults suffering from CKD., (© 2018 The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society.)

Rationale: Since the introduction of radionuclide research methods into clinical practice, they occupy an importantplace in the diagnosis of hydronephrosis and, at the same time, sufficiently objective, sensitive and atraumaticmethods of investigation., Objective: On the basis of retrospective data, to investigate the diagnostic role of radionuclide renography (RRG)and the method of indirect radionuclide renangiography (IRAG) in clean-up workers of Chornobyl accident withhydronephrosis., Materials and Methods: A total of 257 patients with hydronephrosis (140 women and 117 men) aged 15 to 77 yearswere examined by the RRG and the IRAG. The RRG technique consists of intravenous administration of a solution of131I-hypurane (2.5 kBq/kg) and continuous registration for 20 minutes of the level of radioactivity above the kid-neys with the help of sensors of the renograph UR 1-1. The IRAG was conducted for 30-45 seconds with exposure1 frame per second after intravenous administration of a solution of 99mTc-pentatech (2 MBq/kg)., Results: The results of the radionuclide study of the hemodynamics of patients with different stages ofhydronephrosis made it possible to draw a conclusion regarding the expediency of taking into account the condi-tion of the cup-pelvic system in the preoperative period, as well as the parameters of the arterial and venous circu-lation., Conclusions: Combined use of X-ray and radionuclide methods allows establishing the cause and consequences ofhydronephrosis, to develop a rational treatment plan. RRG and IRAG are reliable methods of dynamic control in post-operative observation of clean-up workers of Chornobyl accident with hydronephrosis., (P. O. Korol, M. M. Tkachenko.)

Purpose: We assessed the diagnostic value of anteroposterior diameter of the fetal renal pelvis in predicting postnatal surgery., Materials and Methods: PubMed®, Embase® and Cochrane Library databases were searched for articles comparing patients who underwent surgery or conservative therapy with 15 mm anteroposterior diameter as a cutoff value. Data on sensitivity, specificity, positive likelihood ratio, negative likelihood ratio, diagnostic OR and area under the summary ROC curve were used to evaluate the diagnostic value of anteroposterior diameter of the fetal renal pelvis in predicting postnatal surgery., Results: Five studies involving a total of 1,159 patients met the inclusion criteria. The summary sensitivity and specificity were 0.81 (95% CI 0.60 to 0.92) and 0.78 (95% CI 0.68 to 0.86), respectively. Positive and negative likelihood ratios of anteroposterior diameter were 3.73 (95% CI 2.75 to 5.06) and 0.24 (95% CI 0.11 to 0.52), respectively. Diagnostic OR of anteroposterior diameter for predicting postnatal surgery was 13.33 (95% CI 6.61 to 26.89) and area under the summary ROC curve was 0.85 (95% CI 0.82 to 0.88). Subgroup analysis suggested postnatal anteroposterior diameter was associated with higher diagnostic OR compared to prenatal anteroposterior diameter for predicting postnatal surgery (ratio between prenatal and postnatal anteroposterior diameter 0.27, 95% CI 0.09 to 0.86, p = 0.026)., Conclusions: Using 15 mm anteroposterior diameter of the fetal renal pelvis as a cutoff showed moderate diagnostic value for predicting postnatal surgery., (Copyright © 2018 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

The association between tumor of the urinary tract and hydronephrosis caused by a syndrome of the pyelo-ureteral junction is rare. Indeed, tumors of the upper urinary tract and hydronephrosis have generally a cause-effect relationship. This last is due, more often, to an obstruction caused by a tumor of the ureter or of the pyelo-ureteral junction. We report the case of a 66-year old patient with a history of smoking and right pyelonephritis, presenting with right lumbar pain intermittently evolving over several months without haematuria. Ultrasound showed a dilation of the pyelocaliceal cavities with major reduction of the corticomedullary index of the right kidney. Uroscan was in favor of cystic dysplasia of the right kidney as well as of right hydronephrosis associated with syndrome of pyelo-ureteral junction with budding intrarenal images leading to the suspicion of pyelic tumor. The assessment was completed by urinary cytology which was positive. Right laparoscopic nephroureterectomy was performed and pathologic examination confirmed the diagnosis of urothelial carcinoma of the upper urinary tract.

A 60-year-old man presented to an outpatient pain management clinic with antalgic gait and left lower extremity (LLE) radiculopathy from an unknown aetiology. A lumbar MRI revealed minimal disc protrusion at L3 and a partially visualised left-sided kidney abnormality. Abdominal and pelvic CT demonstrated severe hydronephrosis of the left kidney compressing the left psoas major. The patient was immediately referred to an outside hospital for nephrology workup, and following nephrostomy tube, his radicular pain resolved. He remained asymptomatic at 4 weeks follow-up. We found two cases of postsurgical, retroperitoneal fluid collection that caused lumbar radiculopathy, but none associated with hydronephrosis. To our knowledge, this is the first case in the literature to report hydronephrosis as the potential aetiology of lumbar radiculopathy, which highlights an important clinical reminder: to consider extraspinal aetiologies in all patients who present with lumbar radiculopathy, when clinical symptoms, examination findings and diagnostic studies present with unusual characteristics., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.)

Urinary tract obstruction and the subsequent development of hydronephrosis can cause kidney injuries, which results in chronic kidney disease. Although it is important to detect kidney injuries at an early stage, new biomarkers of hydronephrosis have not been identified. In this study, we examined whether vanin-1 could be a potential biomarker for hydronephrosis. Male Sprague-Dawley rats were subjected to unilateral ureteral obstruction (UUO). On day 7 after UUO, when the histopathological renal tubular injuries became obvious, the vanin-1 level in the renal pelvic urine was significantly higher than that in voided urine from sham-operated rats. Furthermore, vanin-1 remained at the same level until day 14. There was no significant difference in the serum vanin-1 level between sham-operated rats and rats with UUO. In the kidney tissue, the mRNA and protein expressions of vanin-1 significantly decreased, whereas there was increased expression of transforming growth factor (TGF)-β1 and Snail-1, which plays a pivotal role in the pathogenesis of renal fibrosis via epithelial-to-mesenchymal transition (EMT). These results suggest that vanin-1 in the renal pelvic urine is released from the renal tubular cells of UUO rats and reflects renal tubular injuries at an early stage. Urinary vanin-1 may serve as a candidate biomarker of renal tubular injury due to hydronephrosis.

Ureteral obstruction will lead clinically to hydronephrosis, which may further develop into partial or complete loss of kidney function and even cause permanent histological damage. However, there is little knowledge of metabolic responses during the obstructed process and its recoverability. In this study, a complete unilateral ureteral obstruction (CUUO) model was established in the rabbit, and 1 H NMR-based metabolomic analysis of urine was used to reveal the metabolic perturbations in rabbits caused by CUUO and the metabolic recovery after the CUUO was relieved. Univariate and multivariate statistical analyses were used to identify metabolic characteristics. The gradually decreased levels of 3-hydroxykynurenine, 3-methylhistidine, creatinine, guanidoacetate, meta- and para-hydroxyphenylacetate, and phenylacetylglycine and the gradually increased levels of acetate, alanine, citrate, glycine, lactate, and methionine in urine could be regarded as potential biomarkers for the occurrence and severity of ureteral obstruction. And the reduced levels of 3-methylhistidine, creatinine, guanidoacetate, hippurate, meta-hydroxyphenylacetate, and methylguanidine and the elevated levels of 2-aminoisobutyrate, acetylcholine, citrate, lactate, lysine, valine, and α-ketoglutarate in urine compared with the obstructed level could characterize the metabolic recovery of ureteral obstruction. Our results depicted the disturbed biochemical pathways involved in ureteral obstruction and demonstrated the practicability of recovering renal functions for the patients with severe hydronephrosis in clinical practice by removing causes for obstruction.

Background: To improve the prediction of outcomes in patients who will undergo radical nephroureterectomy (RNU) for upper tract urothelial carcinoma (UTUC), we examined the preoperative prognostic factors and established a risk classification model., Methods: A total of 148 patients who underwent RNU without history of neo-adjuvant chemotherapy between 1999 and 2016 in Hiroshima University Hospital were retrospectively reviewed. Associations between preoperative/clinicopathological factors and outcomes including cancer-specific survival (CSS) and recurrence-free survival (RFS) were assessed. We specifically looked at neutrophil-lymphocyte ratio (NLR) due to growing evidence on its predictive role in cancer prognosis prediction., Results: Preoperative elevated neutrophil-lymphocyte ratio (pre-op NLR, ≥3.0) and hydronephrosis (≥grade 2) were associated with advanced pathological stage; and were identified as independent predictive factors of shorter CSS and RFS in univariate and multivariate analysis. We classified the patients in three groups using preoperative factors and found that the 5-year CSS was 94.5, 75.9 and 44.7% and the 5-year RFS was 74.3, 57.6 and 28.7% in the low-risk group (neither pre-op NLR nor hydronephrosis), intermediate-risk group (either pre-op NLR or hydronephrosis) and high-risk group (pre-op NLR and hydronephrosis), respectively. High-risk group had significantly worse CSS (P = 0.0172) and RFS (P = 0.0014) than intermediate-risk group and low-risk group (CSS (P < 0.0001) and RFS (P < 0.0001))., Conclusions: Elevated pre-op NLR and hydronephrosis were identified as independent prognostic factors in patients with UTUC. These simple preoperative factors can stratify three prognostic groups and may help urologists in clinical decision-making before RNU.

Symptomatic urinary obstruction, characterised by decreased or absent urinary outflow, is a common concomitant disease in patients with urological malignancies or carcinomas forming abdominal metastases. While acute urinary obstruction is associated with renal colics, chronic urinary obstruction often goes unnoticed for a long time. In addition to intrinsic causes such as upper urinary tract tumours, this condition is often caused by external compression of the ureter by tumour masses. Placement of a ureteral JJ stent or a percutaneous nephrostomy tube results in early disobstruction. Depending on the prognosis of the underlying disease, a definitive surgical restoration may be considered., Competing Interests: Die Autoren geben an, dass keine Interessenkonflikte bestehen., (© Georg Thieme Verlag KG Stuttgart · New York.)

Introduction: Antenatal hydronephrosis is a steady source of urology referrals since the era of routine fetal ultrasonography. Although most resolve, there are no guidelines for follow-up., Objective: Our goal is to define safe parameters with which patients can be discharged early and avoid unnecessary follow-up., Methods: We retrospectively reviewed all patients referred to a single children's referral hospital center for isolated antenetal hydronephrosis between 2010 and 2012. We looked at patients and renal units separately and divided the cohort into two groups for comparison. Our analysis endpoint is progression. That is, if the initial postnatal anterior-posterior diameter (APD) is less than 10 mm, progression occurs if the APD increases to 10 mm or above upon follow-up. Conversely, if the initial APD is 10 mm or more in at least one renal unit, progression occurs if the APD remains at 10 mm or above upon follow-up., Results: There majority of the 186 patients and 308 renal units included in the analysis, were classified in the APD less than 10 mm group. Most renal units in the APD of less than 10 mm group were of SFU grades 0-2 (92.1%) and most of the renal units in the APD of 10 mm or greater group were of SFU grades 3-4 (60%) (Table). Only 19 renal units (6.2%) underwent pyeloplasty, and they were all from the APD of 10 mm or greater group and classified as SFU grade 3-4. No renal unit with an APD of less than 10 mm, nor any with an APD of 10 mm or greater and a SFU grade 0-2 underwent pyeloplasty. More than half of the renal units' hydronephrosis resolved in the APD of 10 mm or greater group, in comparison with 96.1% of the APD of less than 10 mm group. On multivariate analysis, patients with an APD of 10 mm or greater were 7.76 times more likely to show progression (p = 0.0006)., Conclusion: An initial postnatal APD of 10 mm or greater, with a SFU grade 3-4, merits follow-up. However, all patients with an APD of less than 10 mm, especially when with a SFU grade 1-2, can be safely discharged as they are unlikely to experience complications., (Copyright © 2018 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)