Dilatation Of Collecting System Research Articles

Imaging of Vesicoureteral Reflux: AJR Expert Panel Narrative Review.

Vesicoureteral reflux (VUR) is a common congenital anomaly of the urinary tract that can present with collecting system dilation or as a febrile infection. VUR can lead to permanent renal sequelae requiring surgery but can also spontaneously resolve without complication. Therefore, recognizing patient populations who warrant imaging for screening, confirmation, or ongoing surveillance for VUR is important, as is avoiding overdiagnosis. In the appropriate patient populations, an accurate diagnosis of VUR allows early treatment and prevention of pyelonephritis and scarring. Various imaging modalities are available to diagnose and grade VUR, including voiding cystourethrography, radionucleotide cystography, and contrast-enhanced voiding urosonography (ceVUS). The objective of this article is to summarize the current understanding of VUR diagnosis and management and to discuss these imaging modalities' strengths and pitfalls. Considerations include indications for VUR imaging, patient preparation, conduct of the examination, issues related to radiologic reporting, and cost-effectiveness. An emphasis is placed on ceVUS, which is the most recently introduced of the three imaging modalities and is receiving growing support among pediatric radiologists.

A Study Assessing the Role of Renal Grayscale Ultrasonography and Flowmetry in Correlation With Renal Function Tests Across Various Renal Diseases.

Background Renal insufficiency, a critical concern in native and transplant kidneys, necessitates effective screening modalities for evaluation and management. Grayscale sonography has been a cornerstone in renal diagnostics, providing basic anatomical insights such as renal length, cortical thickness, and collecting system dilatation. Despite technological advancements, its impact on the differential diagnosis or management of renal disease remains limited, often showing normal findings in the presence of severe renal dysfunction. On the other hand, Doppler sonography, particularly the Doppler resistive index (RI), has shown potential in enhancing the assessment of renal dysfunction by quantifying alterations in renal blood flow and correlating with various renal pathologies and prognoses. Thus, this study aims to assess and compare the sensitivity of transabdominal and Doppler sonography as a diagnostic modality to evaluate medical renal diseases with altered renal function tests (RFTs). Methodology Participants included patients visiting the ultrasonography (USG)room at our hospital for USG of the kidneys, ureters, and bladder (USG KUB) and USG of the whole abdomen(USG W/A) with altered RFTs. Each underwent renal grayscale USG and RImeasurement, alongside standard RFTs, aiming to investigate the relationship between USG and RI findings and RFT outcomes to assess their predictive accuracy for renal function. Renal grayscale USG assessed parameters including renal dimensions, echogenicity, corticomedullary differentiation, and RI. Data management and charting were conducted with Microsoft Excel 2021 and Microsoft Word 2021. IBM SPSS Statistics for Windows, Version 24 (IBM Corp.,Armonk, NY) was utilized for data analysis. The analysis of variance (ANOVA) test examined relationships between renal parameters and RI across diagnostic groups. Furthermore, the chi-square test evaluated associations of renal ultrasound findings with RFTs and their significance. Results The study analyzed 93 patients with altered RFTs. Chronic kidney disease (CKD) affected 68 patients, primarily males in their fifth and sixth decades, showing reduced renal dimensions, increased cortical echogenicity, and elevated Doppler RI mean (RIm) with 83% sensitivity. Acute kidney injury (AKI) was found in 12 patients, mainly in their fourth decade, displaying increased renal parameters and elevated RIm with 75% sensitivity. Glomerular diseases, including nephrotic syndrome (NS) and nephritic syndrome (NeS), occurred in 9 patients, predominantly males in their fourth decade, with heightened renal cortical echogenicity and elevated RIm with 55.5% sensitivity. Lupus nephritis (LN) was detected in 4 female patients, despite normal renal parameters, showing elevated serum creatinine levels. Conclusions Doppler assessment of renal vascular waveforms effectively identifies chronic renal changes, aiding in the diagnosis of altered RFTs and guiding prognosis. While it detects typical changes like decreased size and parenchymal atrophy, it may not be as indicative of diabetic nephropathy. Key ultrasound indicators such as changes in echotexture and size, along with associated findings like ascites and effusions, help recognize altered renal function and minimize unnecessary interventions.

Retroperitoneal lymphadenopathy: An underappreciated contributor to acute kidney failure in patients with cancer

Introduction: Retroperitoneal lymphadenopathy represents a unique and infrequent complication within various malignancies. This case report exemplifies the intricate diagnostic challenges that arise when identifying nondilated obstructive nephropathy in the context of retroperitoneal lymphadenopathy due to diffuse large B-cell lymphoma (DLBCL), particularly when conventional imaging fails to reveal the hallmark feature of hydronephrosis. Case description: We present a 66-year-old male with relapsed chronic lymphocytic leukemia post-allogeneic stem cell transplantation. His direct admission to the hematology/oncology service was due to failure to thrive. Despite the absence of hydronephrosis on imaging, the patient developed volume overload and increased serum creatinine levels. An ultrasound unveiled mild bilateral collecting system dilation, while laboratory findings did not provide valuable insight into the underlying etiology. A Lasix renal scan confirmed split renal function with minimal bilateral excretion. High clinical suspicion resulted in an MRI scan showing interval enlargement of retroperitoneal lymph nodes and pelvicaliectasis, which prompted urological and interventional radiology involvement. Conclusions: This case underscores the complexity of diagnosing retroperitoneal lymphadenopathy and pelvicaliectasis, in DLBCL, particularly when hydronephrosis is absent on imaging. It emphasizes the significance of clinical acumen, collaboration among specialties, and the use of advanced imaging techniques. Timely recognition of nondilated obstructive nephropathy in the setting of retroperitoneal lymphadenopathy is pivotal for guiding appropriate management strategies and optimizing patient outcomes in the intricate clinical setting.

Regression of renal collecting system dilatation after pyeloplasty in children: evaluation of dynamics

Regression of renal collecting system dilatation after pyeloplasty in children: evaluation of dynamics

Pilot Study on the Molecular Pathogenesis of Pyeloureteral Junction Obstruction: Underdevelopment or Fibrosis?

Background and Objectives: Congenital ureteral stenosis is one of the leading causes of impaired urinary drainage and subsequent dilatation of the urinary collecting system, known as hydronephrosis or ureterohydronephrosis. The mechanism that leads to obstruction is not clearly known. Multiple studies in rat models have shown increased angiotensin II and TGFβ levels in obstructed ureteral tissue. The aim of the study is to investigate the expression of fibrosis-related genes in obstructive and normal ureteral tissue. Material and Methods: It is a monocentric pilot study in which nineteen patients were selected prospectively. 17 patients underwent Hynes-Anderson pyeloplasty due to the PUJO; two patients underwent ureteroneocystostomy due to ureterovesical junction obstruction (UVJO); and six patients were chosen for the control group: five underwent nephrectomies due to the kidney tumor and one underwent upper pole heminephrectomy due to the duplex kidney with normal pyeloureteric junctions in all. Tissue RNA was chemically extracted after freezing the biopsy samples in liquid nitrogen, with cDNA synthesis performed immediately after nucleic acid isolation. qPCR was performed to evaluate the relative expression of Tgfb1, Mmp1, Timp1, Pai1, Ctgf, and Vegfa. Expression levels of the Gapdh and Gpi genes (geometric average) were used to calculate the relative expression of the investigated genes. Outliers were removed prior to calculating confidence intervals for the experimental groups, and a Wilcoxon rank-sum test was performed to determine the statistical significance of the differences. Results: Significant differences between healthy and stenotic tissue samples in Ctgf gene expression levels were observed, with the samples from afflicted tissue showing lower expression. No statistical difference in expression levels of Tgfb1, Timp1, Vegfa, Mmp1, and Pai1 was found. Conclusions: These findings suggest that tissue fibrosis, similar to other tissues and organs, is not the leading cause of stenosis, at least at the moment of surgery. Decreased CTGF expression is indicative of the developmental origin of obstruction.

Contrast-enhanced ultrasound versus conventional ultrasound-guided percutaneous nephrolithotomy in patients with a non-dilated collecting system: results of a pooled analysis of randomized controlled trials

BackgroundContrast-enhanced ultrasound in percutaneous nephrolithotomy (CEUS-PCNL) is an economical and practical technique for the treatment of patients with renal stones without significant collecting system dilatation. The aim of this systematic review is to compare the safety and efficacy of CEUS-PCNL and conventional ultrasound (US)-guided (US-PCNL) treatment of patients with renal calculi without significant hydronephrosis.MethodsThis review was conducted with strict adherence to the PRISMA guidelines. Comparative studies on CEUS-PCNL and US-PCNL published in PubMed, SinoMed, Google Scholar, Embase, and Web of science until March 1, 2023, were systematically searched. RevMan 5.1 software was used for meta-analysis. Pooled odds ratios (ORs), weight mean differences (WMDs) and standard mean differences (SMDs) with 95% confidence intervals (CIs) were calculated using the fixed-effects or random-effects model. Publication bias was evaluated using funnel plots.ResultsFour randomized controlled trials involving 334 patients (168 with CEUS-guided PCNL and 166 with US-guided PCNL) were identified. There was no statistically significant difference between CEUS-guided PCNL and US-guided PCNL in terms of the operation time (SMD: − 0.14; 95% CI − 0.35 to 0.08; p = 0.21), minor complications (p = 0.48), major complications (p = 0.28) and overall complications (p = 0.25). However, CEUS-guided PCNL had a higher stone-free rate (OR: 2.22; 95% CI 1.2 to 4.12; p = 0.01), higher success rate of single-needle punctures (OR:3.29; 95% CI 1.82 to 5.95; p < 0.0001), shorter puncture time (SMD: − 1.35; 95% CI − 1.9 to − 0.79; p < 0.00001), shorter hospital stay (SMD: − 0.34; 95% CI − 0.55 to − 0.12; p = 0.002) and lesser hemoglobin loss (SMD: − 0.83; 95% CI − 1.06 to − 0.61; p < 0.00001) as compared with conventional US-guided PCNL.ConclusionsAccording to almost all pooled data, CEUS-guided PCNL is superior to US-guided PCNL in terms of the perioperative outcomes. However, many rigorous clinical randomized controlled studies are required to obtain more accurate results.Registration The study protocol was registered with PROSPERO (CRD42022367060).

MP70-10 PEDIATRIC SINGLE PORT ROBOTIC PYELOPLASTY COMPARED TO OPEN AND MULTIPORT COHORTS

You have accessJournal of UrologyCME1 Apr 2023MP70-10 PEDIATRIC SINGLE PORT ROBOTIC PYELOPLASTY COMPARED TO OPEN AND MULTIPORT COHORTS Jordan Smith, Alexandra Hernandez, Brian Wiseman, Christopher Bayne, and Romano Demarco Jordan SmithJordan Smith More articles by this author , Alexandra HernandezAlexandra Hernandez More articles by this author , Brian WisemanBrian Wiseman More articles by this author , Christopher BayneChristopher Bayne More articles by this author , and Romano DemarcoRomano Demarco More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003338.10AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: The Da Vinci SP® (single port) surgical system has been used widely in adult urology with comparable peri-operative outcomes compared to multi-port (MP) robotic surgery. However, reports in pediatric urology are limited. We sought to compare operative outcomes in a single pediatric urologist’s open, MP, and SP pyeloplasty experience. METHODS: A retrospective review was performed of all pyeloplasties by a single pediatric urologist in patients <18 y at a tertiary academic center from January 2021 to July 2022. Cases were categorized by approach: open, multiport (MP), or single port (SP). The primary outcomes were entire operative time (OT; defined as cystoscopy to incision close), length of hospital stay (LOS), inpatient opioid use, and post-operative complications (≤30 days). Surgical success (defined as improved nuclear renography drainage or improved renal collecting system dilatation on ultrasonography with resolution of pain) was evaluated as a secondary outcome. RESULTS: During the study period, 6 open, 5 MP, and 11 SP cases were performed. Open cases were performed via flank incision. MP cases were performed via mix of midline port placement and hidden incision endoscopic surgery (HIdES) approach with 3 or 4 trocars. Technique for SP cases evolved during the period. The SP cohort included older children (median 16 y) and larger children (60.1 kg) than open (2.3 mo; 5.7 kg) and MP (4 mo; 6.6 kg) cases. There was no difference in LOS, opioid use, or complications between groups. SP cases were notably longer (median 390 min [376–480 min]) than both open (240 min [240–360 min]) and MP (300 min [200–258 min]) cases. OT for SP cases improved over the last 5 cases (median 388 min) versus the initial 6 (median 405 min). All cases have continued to show a successful repair at a minimum 3 months. Due to sample size, inferential statistics were not able to be performed. CONCLUSIONS: In this single-surgeon experience during early adoption of the SP robot in pediatric urology, SP pyeloplasty cases took substantially longer. This may represent the early learning curve of developing a novel technique. There was no noted difference in other outcomes or complications. Multicenter collaboration of open, MP, and SP data would help account for individual surgeon differences and generate robust samples sizes for inferential statistical analysis. Knowledge of patient-centered opinions regarding the SP incision is warranted to justify SP pyeloplasty over current MP techniques. Source of Funding: None © 2023 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 209Issue Supplement 4April 2023Page: e1007 Advertisement Copyright & Permissions© 2023 by American Urological Association Education and Research, Inc.MetricsAuthor Information Jordan Smith More articles by this author Alexandra Hernandez More articles by this author Brian Wiseman More articles by this author Christopher Bayne More articles by this author Romano Demarco More articles by this author Expand All Advertisement PDF downloadLoading ...

The shape of the kidney pelvis in the fetus with hydronephrosis grade III as a predictor of surgical treatment in postnatal period

BACKGROUND: Fetal hydronephrosis is one of the most common malformations of the urinary system. The main goal of prenatal diagnostics is to identify a risk group with a dilatation of the renal collecting system due to mechanical obstruction of the pelvic-ureteral segment.&#x0D; AIM: The aim of the study is to definition of a prenatal risk group with a high probability of early surgical intervention in the postnatal period based on an assessment of the predictor properties of the pelvis shape in hydronephrosis grade III.&#x0D; MATERIALS AND METHODS: A prospective analysis of the results of prenatal ultrasound examination of 77 fetuses (82 renal units) with grade III hydronephrosis (SFU classification) was carried out. The anteroposterior diameter of the pelvis was measured in millimeters at the level of the kidney gate. With the ellipsoid shape of the pelvis, the compression ratio of the ellipse was calculated as the ratio of the small semi-axis of the ellipse to its large semi-axis. All parameters were recorded in the third trimester of pregnancy. The study included cases of hydronephrosis with parenchymal thickness not differing by more than two sigma deviations from the standard value. The first ultrasound examination in the postnatal period was performed during the first month of life and then at 3, 6 and 12 months.&#x0D; RESULTS: Prenatally, 57 kidneys had an elliptical pelvis and 25 funnel-shaped. The anteroposterior diameter of ellipsoid (16.5 [13; 20]) and funnel-shaped pelvis (15.0 [13; 17.8]) did not differ statistically significantly (p = 0.39). 36 (43.9%) patients were operated on, 29 of them were operated at the age of 13 months, due to the increase in APD from 17.56.0 to 27.98.2 (p = 0.001) and the transition of hydronephrosis to the IV degree by 1 month of life. The remaining 7 children had gradual progression of hydronephrosis and were operated at the age of 12 months and older. In 56.1%, resolution, regression or stabilization of hydronephrosis were noted during dynamic observation lasting 1 year. With a single-variant analysis, it was found that the configuration of the pelvis and the compression ratio of the ellipse statistically significantly correctly predicted the progression of hydronephrosis and the probability of surgery at 69.5% and 80.5%, respectively. At the same time, the compression ratio had a higher sensitivity and specificity.&#x0D; CONCLUSIONS: Fetal hydronephrosis with a funnel-shaped configuration of the pelvis, has a functional nature and regresses after birth during the first year of life. The antenatal ellipsoid configuration of the pelvis, approaching the circumference, is a statistically significant predictor of surgical intervention due to the progression of hydronephrosis due to external causes of obstruction.

Development of a Novel Clinical and Radiologic Risk Score to Predict Septic Complications After Urinary Decompression in Patients with Obstructive Uropathy.

Background: Well-defined clinical predictors of sepsis after upper tract drainage for obstructive uropathy are lacking. The study aim is to develop a data-driven score to predict risk of sepsis after decompression of the upper urinary tract. Materials and Methods: Complete clinical and radiologic data from 271 patients entering the emergency department for obstructive uropathy and submitted to stent/nephrostomy tube decompression were evaluated. The Charlson Comorbidity Index (CCI) was used to score comorbidities. The definition of sepsis was an increase in ≥2 Sequential Organ Failure Assessment points (or a postoperative persistently elevated score +1 additional increase) and documented blood or urine cultures. Descriptive statistics and stepwise multivariable logistic regression modeling with receiver operating characteristic analysis were performed to obtain a composite risk score to predict the risk of sepsis after surgery. This study was approved by our local Ethics Commitee (Prot. 25508). Results: Fifty-five (20.3%) patients developed sepsis. At multivariable analysis, CCI ≥2 (odds ratio [OR] 3.10; 95% confidence interval [CI] 1.36-7.04), maximum body temperature ≥38°C (OR 4.35; 95% CI 1.89-9.44), grade III-IV hydronephrosis (OR 2.37; 95% CI 1.10-4.98), HU of the dilated collecting system ≥7.0 (OR 4.47; 95% CI 2.03-9.81), white blood cells ≥15 × 103/mmc (OR 2.77; 95% CI 1.24-6.19), and C-reactive protein ≥10 (OR 3.27; 95% CI 1.41-7.56) were independently associated with sepsis. The positive predictive value of a true sepsis increased incrementally as a function of number of positive variables, ranging from 1.6% to 100.0% among patients with 1 and 6 positive variables, respectively. Conclusion: Our risk score identifies accurately patients with an increased risk of sepsis after urinary decompression for obstructive uropathy, hence improving clinical management.

A Comparability of Renal Length and Volume Measurements in MRI and Ultrasound in Children.

Introduction: Despite the significant increase in use of magnetic resonance imaging (MRI) in children, there is still a lack of normal reference values of renal size in this method and reference values are being interpolated from the ultrasound (US) studies. The study provides comparative analysis of agreement in renal length and volume measurements between MRI and ultrasound.Materials and Methods: Ninety-three children with a mean age of 8.0 ± 6.0 years, who had undergone both renal US and MRI exams, were included in the study. Participants were divided into three subgroups; each kidney was considered separately.Group 1 included 106 kidneys without any anomalies. Group 2 comprised 48 kidneys with a dilated collecting system. Group 3 included 32 kidneys with a duplicated collecting system. Measurements were taken in three dimensions, and renal volume was calculated from the ellipsoid formula.Results: We found no significant difference between US and MRI measurements in Group 1 and Group 2. In Group 3, the difference between measurements in both imaging methods was significant. The mean difference varied from 0.05% in Group 1, 2.95% in Group 2, to 4.99% in Group 3.Conclusion: The US and MRI are comparable methods in renal size measurements. The interpolation of sonographic renal length and volume reference values to the MRI in the pediatric population is justified, as there is a strong agreement between both methods. Both methods can be used interchangeably for following up of the renal size changes in the pediatric population.

Renal pelvic dilation

Renal pelvic dilation

Potential benefits of functional magnetic resonance urography (fMRU) over MAG3 renal scan in children with obstructive uropathy

Functional renal imaging, most commonly with MAG3 nuclear medicine renal scan, is recommended in the evaluation of children with urinary tract dilation (UTD) suspected of obstructive uropathy. Alternatively, renal function can be evaluated with functional Magnetic Resonance Urography (fMRU), which has superior anatomic detail. However, there are not enough data comparing both methods' equivalency. In this study, we compare the functional and obstruction parameters of fMRU and MAG3 in a pediatric cohort presenting with obstructive uropathy. This is an IRB-approved retrospective review of all children undergoing fMRU at a single, free-standing children's hospital between May 2008 and September 2017. Patients who also underwent a MAG3 renal scan within 6 months and who had no interval surgical intervention were included in the study. Bladder catheterization was performed prior to both imaging studies. 735 children had 988 fMRU studies performed during the study period. 37 unique patients (13 girls and 24 boys) with median age of 6 months (range: 2 mo-19y) were included in the final sample. Median time interval between studies was 70 days (range 6-179 days). The majority of participants (26/37, 70.3%) presented with UTD P3 and had diagnosis of uretero-pelvic junction obstruction (UPJO) in 21/37. Differential renal function (DRF) was used to group 10 fMRU and 9 MAG3 patients as normal; 9 fMRU and 11 MAG3 as mild; 11 fMRU and 6 MAG3 as moderate; and 7 fMRU and 6 MAG3 as severe; Wilcoxon signed-rank test (p=0.5106). Results were similar for DRF among patients with and without duplex kidneys. In the analysis of obstruction, using reference standard T½ MAG3≥20min, a greater or equal than 6min renal transit time (RTT) from fMRU showed a specificity of 94%, a sensitivity of 62%, and an AUC of 0.827. The differential renal function determined by MAG3 and fMRU in children was not statistically different, therefore we concluded that it was similar and potentially equivalent. Better correlation was shown in patients who had normal split kidney function. While the tests are clinically equivalent, the variability of DRF within each clinical category (i.e., normal, mild, moderate, severe) is not surprising, because MAG3 does not clearly differentiate the dilated collecting system from the functional parenchymal tissue, while fMRU does. Using MAG3 as the gold standard, fMRU was 94.74% specific and 5% more sensitive in detecting UPJO with a RTT of 6min vs. 8min.

Acute urinary retention in pediatric cerebral palsy: Is there an optimal management strategy?

Cerebral palsy (CP) patients commonly have lower urinary tract dysfunction. Urinary retention (UR), which has been associated with dysfunctional voiding in CP can correlate to chronic upper tract dysfunction. We sought to provide insight into the pediatric presentation of acute UR in patients with CP and subsequent outcomes in this at-risk population. All children with perinatally acquired CP presenting to a regional health network were identified from 2009 to 2019. Retrospective analysis of a hospitalized subset concurrently diagnosed with a first episode of acute UR was performed. Factors associated with new-onset UR are described, as well as management. Using follow-up data, we also assessed the risk for recurrent UR and/or abnormal renal imaging after an initial UR presentation. 3404 CP patients were analyzed with only 33 fulfilling inclusion criteria. Median age was 10(IQR 7.5-16; range 1-22) years, 87.9% were GMFCS-V. 39.4% had a reported history of decreased urinary frequency. At presentation, median maximal time without void prior to catheterization was 13h, and catheterized volume was a median 120% expected capacity-for-age. 84.8% of presentations were associated with a known transient/reversible etiology. 51.5% were post-anesthesia at median 3.5 days, 33.3% had associated constipation, 30.3% had received exacerbating medications. 11/33 were taught clean intermittent catheterization (CIC) after the initial presentation (all pro re nata [PRN] except one). At a median follow-up of 37 months: 50% of those without a CIC PRN no void plan had a repeat episode, at a median of 10.8 months later. Of the patients who had follow-up renal imaging at a median 22.0 months after presentation, 45% had abnormalities: 7 with debris or suspected stones and 2 with collecting system dilation. No factors associated with the initial UR presentation were found to be significantly predictive of recurrence or abnormal follow-up imaging. Patients with CP presenting with acute UR are often those with the most severe limitations and have a history of decreased urinary frequency. They usually have transient or reversible factors associated with UR presentation, however UR recurrence and abnormal imaging in this population subset is common. Pediatric patients with CP who present with acute UR usually present in the context of recent anesthesia and in the setting of exacerbating factors. They are at risk for recurrence and may be best managed with caretaker education of CIC PRN no void to address recurrent episodes. Providers should consider surveillance of these patients for the development of abnormal renal imaging.

MO167AN UNUSUAL COURSE OF A 2,8 DHA CRYSTAL INDUCED NEPHROPATHY

Abstract Background and Aims 2, 8 dihydroxyadenine (DHA) deposition is a less known etiology of crystal-induced nephropathy, caused by a deficiency in a purine salvage enzyme, the adenine phosphoribosyl transferase (APRT). DHA is an insoluble molecule in urine leading to crystal formation, tubular obstruction or stone formation. The disease manifests as a history of urolithiasis, chronic kidney disease and even loss of renal allograft when the disease is undiagnosed in native kidneys. The cornerstone of treatment is the inhibition of xanthine dehydrogenase reducing thus the formation of 2,8-DHA and its renal excretion. Method A 59-year-old obese Lebanese male patient, born to a consanguineously married couple, was admitted to another hospital with desaturation, a history of progressive shortness of breath and a creatinine level of 2,8 mg/dl. He had no hypertension nor diabetes. His family history was positive for a sister with ESRD of unknown etiology. He was discharged on oxygen and continuous airway positive pressure therapy for severe obstructive sleep apnea. His renal function deteriorated leading to a creatinine level of 9.8 mg/dl three months later. There were no signs of systemic disease, no gross hematuria, no fluid overload. His blood pressure was normal. Laboratory work up showed anemia, low grade proteinuria, intermittent microscopic hematuria and negative serological and immunological workup. Kidney ultrasound showed normal size kidneys with no evidence of collecting system dilatation or urolithiasis. Due to this atypical presentation, the patient was admitted for a renal biopsy with a creatinine level of 11 mg/dl upon admission. Results The renal biopsy showed tubulo-interstitial nephritis associated with numerous brown-green crystals by Haematoxylin and eosin of various shapes birefringent under polarized light with the characteristic “maltese cross”. Crystals were found within tubular lumens and cytoplasm, interstitium, and macrophages. These findings were characteristic of 2,8 DHA crystals deposition in the kidney. The patient was started on 120 mg of Febuxostat with a low purine and high fluid diet. A genetic testing showed a pathogenic homozygous variant in the APRT gene which causes an amino acid change from Glycine to Aspartate at position 63. Two weeks later the patient was admitted to the ICU with pneumonia, respiratory failure, a creatinine of 9 mg/dl and severe metabolic encephalopathy. He received 4 sessions of hemodialysis followed by an improvement in his kidney function with a creatinine level down to 3.2 mg/dl a month after his discharge and he remains off dialysis until now. Conclusion Around 400 cases are currently recognized worldwide, emphasizing the under recognition of this autosomal recessive disease. Considering that the homozygoty causing a complete APRT deficiency should range between 1/50 000 and 1/100 000 cases, this would translate in at least 80 000 cases worldwide. The variant found in our patient has previously been described as disease causing for APRT deficiency in four cases. Reviewing the phenotype of these cases we find differences in terms of presentation and evolution, highlighting the variability in the APRT deficiency phenotype and underlining the fact that no correlation between phenotype and genotype was reported to date even for the same type of mutation. This case report shows us that the initiation of an adequate therapy is necessary even at advanced stages of the disease since it can improve our kidney outcome.

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.

Foetal Ureteric Calculus Producing Hydronephrosis and Hydroureter.

Dilatation of foetal urinary collecting system is common. But cause of obstruction, if any, arise from soft tissues or developmental abnormalities of urinary tract or other paradox. A prenatal diagnosis of a 'calculus' inside ureter producing obstruction and ultimate back pressure changes in collecting system of a foetus is not reported so far. Other than inborn error of metabolism, what aetiological factors lead to this, are a matter of concern and great research. Here kidneys are not dysplastic. Amniotic fluid volume remains normal. Antenatal administration of calcium either orally or parenterally could be a contributory factor as it promotes nephrocalcinosis; and uretric calculus has it's origin in kidneys as believed. There is no maternal symptom related to this entity. Here we report a unique case of foetal ureterolithiasis producing hydroureter and hydronephrosis.

Diagnostic Value of Real-Time Ultrasound in Congenital Segmental Giant Megaureter: A Case Report

: Congenital segmental giant megaureter (CSGM), which refers to segmental cystic dilatation of the upper collecting system, is extremely rare in the pediatric population. Depending on the etiology, the condition could be categorized as primary or secondary megaureter with three subtypes including refluxing, obstructed, nonrefluxing and nonobstructed types. We report a case in which the CSGM presented as a giant abdominal cystic mass with associated right-sided hydronephrosis that was diagnosed by abdominal ultrasound. Cystic mass and the tubular structure at the caudal side of the cystic mass were demonstrated on abdominal ultrasound. We identified a connection between the cystic mass and the lumen of the tubular structure. Therefore, the cystic mass was reported as CSGM on abdominal ultrasound, especially categorized as the nonrefluxing and nonobstructed type. The percutaneous nephrostomy was performed to relieve hydronephrosis, followed by excision of the dilated segment. CSGM is an extremely rare presentation of megaureter, requiring variable imaging modalities and an appropriate intervention. Especially, careful real-time ultrasound was critical to identify the CSGM promptly and essential for the individual appropriate management.

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

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

CLINICAL COURSE OF FETAL PELVI-CALYCEAL DILATATION DIAGNOSED ON EARLY SECOND TRIMESTER ULTRASOUND.

Aims and Objectives: Incidental finding of fetal pelvi-calyceal dilatation is common finding on early second trimester anomaly scan. [18 to 23 weeks] It causes significant parental anxiety. The present study aims to apply stringent criteria for accurate assessment. It also studies the natural course of such findings, pathological cause for such findings and its postnatal outcomes.&#x0D; Study Design: This study includes 9500 cases that underwent anomaly at 18 to 23 weeks gestation period.&#x0D; &#x0D; The fetal pelvi-calyceal dilatation [PCD] was diagnosed and categorized according to criteria set by European Society for Pediatric Radiology [ 1]. PCD 0 – renal calyces and pelvis not or hardly visible. PCD 1 – renal calyces not visible and axial renal pelvis diameter is less than 7 mm. PCD 2 – some calices are visible but with normal forniceal and papillary shape, axial renal pelvis less than 10 mm. PCD 3 - marked dilatation of calices and pelvis. Pelvic axial width usually more than 10 mm with flattened papilla and rounded fornices but without parenchymal thinning. PCD 4- gross dilatation of entire collecting system and thinning of renal parenchyma. PCD 5- Only thin membrane like residual renal parenchymal rim.&#x0D; &#x0D; Result: Among 9500 women who underwent routine anomaly scan at 18 to 23 weeks gestation, total 390 had PCD findings [4.1 %] Single kidney taken as one case while bilateral findings were taken as 2 cases.&#x0D; 98 [25.1 %] pregnancies had bilateral PCD findings and rest 292[74.9%] had unilateral PCD changes. These findings correlate well with as shown by S Sairam et al, Livera et al, Gunn et al [2, 3, 4] &#x0D; PCD 1 findings were noted in 317 cases, PCD2 findings were noted in 38 cases, PCD3 findings in 23 cases and PCD4 findings in 12 cases. All these fetuses were followed till either PCD findings resolved naturally or till 2 years of their life. These findings also compare well with S Sairam et al, and Persutte et al [ 2, 5]&#x0D; About 00 cases of PCD1, 3 cases of PCD2 and 2 cases of PCD3 were lost for follow up for various reasons.&#x0D; Two cases of PCD1 were lost due to additional diagnosis of Down syndrome in one case and multiple anomalies in other cases. Both were excluded from the present study.&#x0D; Rest of them [383] were studied and analyzed for this paper.&#x0D; Out of 315 PCD1 cases, all turned out to be normal&#x0D; Out of 35 PCD2 cases, 19 were resolved, 12 remain unchanged at second year of child age rest were&#x0D; 4 worsened&#x0D; Out of 21 PCD 3, 2 cases resolved spontaneously, 5 remain unchanged and 14 cases worsened&#x0D; Out of 12 PCD 4 cases, all needed and underwent surgical intervention. 04 kidneys were saved and rest 8 had nephrectomies.

Frequency of urinary tract infection in children with antenatal diagnosis of urinary tract dilatation

BackgroundNeonates with congenital urinary tract dilatation (UTD) may have an increased risk of urinary tract infections (UTI). At present, the management of these patients is controversial and the utility of...