Decreased percent change in renal pelvis diameter on diuretic functional magnetic resonance urography following administration of furosemide may help characterize unilateral uretero-pelvic junction obstruction.

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.

3D autofluorescence imaging of hydronephrosis and renal anatomical structure using cryo-micro-optical sectioning tomography.

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 mm3 and 13.01 ± 3.74 mm3 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.

Ureteral Obstruction Promotes Ureteral Inflammation and Fibrosis.

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.

Long non-coding RNA FABP5P3/miR-22 axis improves TGFß1-induced fatty acid oxidation deregulation and fibrotic changes in proximal tubular epithelial cells of renal fibrosis.

Severe hydronephrosis increases the risk of urinary tract infection and irretrievable renal fibrosis. While TGFß1-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 TGFß1 effects on lncRNA FABP5P3, CPT1A, RXRA, and NCOA1 expression and fibrotic changes in HK-2 cells and FABP5P3 overexpression effects on TGFß1-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 TGFß1-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 TGFß1 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 TGFß1-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. TGFß1 stimulation induced the activation of TGFß/SMAD and JAG/Notch signaling pathways; Nocth2 knockdown reversed TGFß1 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 TGFß1 stimulation.

High-Frequency US for BK Polyomavirus-associated Nephropathy after Kidney Transplant.

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.

Relation between Cajal Cell Density and Radiological and Scintigraphic Outcomes in Patients with Ureteropelvic Junction Obstruction.

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.

Unusual Clinical Presentation of Hodgkin Lymphoma in a Child: Both Spinal Cord Compression and Hydronephrosis.

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.

Graft kidney hydronephrosis caused by transplant ureter inguinal hernia: A case report.

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.

Consistent alteration of chain length-specific ceramides in human and mouse fibrotic kidneys.

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.

Pneumorenum-A Technique to Ease Laparoscopic Nephrectomy in Giant Hydronephrotic Nonfunctioning Kidney.

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.

Giant hydronephrosis secondary to ureterocele with duplex system in adults: Report of a case.

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.

Hounsfield unit attenuation value can differentiate pyonephrosis from hydronephrosis and predict septic complications in patients with obstructive uropathy.

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.

Endoscopic treatment of obstructive ureterohydronephrosis in children.

Obstructive ureterohydronephrosis in childhood population is a matter of debate between paediatric surgeons and paediatricians, as far as the therapeutic protocol that should be applied. Close observation, chemoprophylaxis, endoscopic and surgical approaches are the universally used techniques that provide quality of life in the paediatric patients. Undoubtedly, "the less is more" even when we have to encounter obstructive ureterohydronephrosis in children. Herein, we present a short case series where the endoscopic management of obstructive uropathies proved to be therapeutic without any need of surgical intervention.

Quantitative Proteome of Infant Stenotic Ureters Reveals Extracellular Matrix Organization and Oxidative Stress Dysregulation Underlying Ureteropelvic Junction Obstruction.

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.

Right hydronephrosis as a sign of complicated appendicitis.

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.

Ureteral cancer: Complete ureteral obstruction on computed tomography urography is associated with periureteral fat invasion.

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.

Antenatally detected urinary tract dilatation: a 12-15-year follow-up.

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.

Concomitant management of renal calculi and recurrent ureteropelvic junction obstruction with percutaneous nephrolithotomy and antegrade balloon dilation.

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.

A mutation affecting laminin alpha 5 polymerisation gives rise to a syndromic developmental disorder.

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/ß/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.