Molecular Screening of PAX2 Gene Polymorphism in Primary Vesicoureteral Reflux Patients in Taif Governorate, KSA.

<b>Background and Objective:</b> Primary Nonsyndromic Vesicoureteral Reflux (PVUR) is a widespread genetic malformation and considered a prevalent Congenital Abnormality of the Kidney and Urinary Tract (CAKUT). Mutations in the <i>PAX2 </i>gene have been associated with abnormalities in the kidney extending from CAKUT to oncogenic processes. The present study analyzes the <i>PAX2</i> polymorphisms and their association with primary VUR in Saudi children patients from the Taif governorate. <b>Materials and Methods:</b> Fifteen children with primary VUR were identified and screened for gene mutations in the <i>PAX2</i> gene by direct sequencing method of purified Polymerase Chain Reaction (PCR) products of all exons to elucidate the correlation between <i>PAX2</i> gene and VUR. <b>Results:</b> Seven new variants have been defined. Three polymorphic missense variants in homozygous genotype form were found in intron 8 and detected in eight patients, One missense mutation was found in exon 10 in the site of transactivation domain and detected in ten patients and <i>in-silico</i> analysis predicted it as a pathogenic one, Three mutations were found in exon 11 and detected in all patients as a compound homozygous. <b>Conclusion:</b> <i>PAX2</i>is important for normal kidney development and mutations in the gene possibly lead to disturbance in the protein structure and could be non-functional thus mutations in <i>PAX2</i> may be one of the causes of PVUR in Saudi Arabia. Further investigation is necessary to understand the aetiology of disease and maybe other genes implicated in VUR.

Contrast-enhanced voiding urosonography in the assessment of vesical-ureteral reflux: the time has come.

Vesicoureteral reflux (VUR) is a pathological condition contradistinguished by monolateral or bilateral retrograde flow of urine from the bladder to the ureter and to the kidney. If not properly recognized and treated, VUR can potentially be associated to several complications such as recurrent infections and possible secondary scars with Chronic Kidney Disease (CKD). Furthermore, it represents an important risk factor for nephrovascular hypertension. During the last 20 years, the diagnostic approach to this entity has passed through several, drastic changes: indeed, since its introduction in 1994 contrast-enhanced voiding urosonography (ceVUS) has gradually accompanied the voiding cystourethrography (VCUG) as alternative imaging technique for the diagnosis and staging of VUR. Despite a large number of papers has strongly encouraged its use in clinical practice, due to the lack of ionizing radiations and its high sensitivity rate, to date almost all the guidelines only include the VCUG for VUR diagnosis. The introduction of technologically advanced US software and the approval of the intravesical administration of ultrasound contrast agents by the Food and Drug Administration (FDA) and by the European Medicine Agency (EMA) have to induce the Scientific Community to a deep revaluation of the role of ceVUS in the diagnosis and follow-up of VUR: urosonography might extensively replace VCUG as the reference method, reserving to cystourethrography a role in the most complex anatomic settings for pre-surgical evaluation.

SARS-CoV-2 infection and recurrence of anti-glomerular basement disease: a case report.

BACKGROUND: Anti-glomerular basement membrane disease (GBM) disease is a rare autoimmune disease causing rapidly progressive glomerulonephritis and pulmonary haemorrhage. Recently, an association between COVID-19 and anti-glomerular basement membrane (anti-GBM) disease has been proposed. We report on a patient with recurrence of anti-GBM disease after SARS-CoV-2 infection. CASE PRESENTATION: The 31-year-old woman had a past medical history of anti-GBM disease, first diagnosed 11 years ago, and a first relapse 5 years ago. She was admitted with severe dyspnoea, haemoptysis, pulmonary infiltrates and acute on chronic kidney injury. A SARS-CoV-2 PCR was positive with a high cycle threshold. Anti-GBM autoantibodies were undetectable. A kidney biopsy revealed necrotising crescentic glomerulonephritis with linear deposits of IgG, IgM and C3 along the glomerular basement membrane, confirming a recurrence of anti-GBM disease. She was treated with steroids, plasma exchange and two doses of rituximab. Pulmonary disease resolved, but the patient remained dialysis-dependent. We propose that pulmonary involvement of COVID-19 caused exposure of alveolar basement membranes leading to the production of high avidity autoantibodies by long-lived plasma cells, resulting in severe pulmonary renal syndrome. CONCLUSION: Our case supports the assumption of a possible association between COVID-19 and anti-GBM disease.

Renal morphology and function from childhood to adulthood in Turner syndrome.

BACKGROUND: Turner syndrome (TS) is a chromosomal disorder with various complications, including congenital anomaly of the kidney and urinary tract (CAKUT). However, its renal function remains poorly known. Therefore, this study aimed to evaluate renal function in TS of various ages from childhood to adulthood. METHODS: We retrospectively analyzed 63 patients with TS who visited our hospital between 1989 and 2020, examined their renal morphology, and analyzed renal function by calculating the estimated glomerular filtration rate (eGFR) using formulas applicable for Japanese populations. RESULTS: Renal morphological abnormality was observed in 22 cases (35.0%) (horseshoe kidney, 7 [11.1%]; hydronephrosis, 11 [17.5%]; duplex collecting system, 3 [4.8%]; and single unilateral kidney, 1 [1.6%]). We evaluated the eGFR of 47 subjects aged 2.8-39.3 years and classified them into Group 1 (with CAKUT, n = 15) and Group 2 (without CAKUT, n = 32). The eGFR at the first visit and the final follow-up was not statistically different between these groups. In Group 1 with CAKUT, the eGFR was not significantly different between that at the first visit and that at the final follow-up (p = 0.21). During the observation period (median, 7.9 years), the eGFR of all individuals in both groups gradually decreased with age, but did not fall < 60 mL/min/1.73 m2, which defines chronic kidney disease (CKD). CONCLUSIONS: The renal function of TS remained normal in all cases during our investigation period, and no one developed CKD by the age of 40 years.

The Change in the Renal Pelvis Anterior-Posterior Diameter Between Prevoiding and Postvoiding Status, and Its Correlation With Vesicoureteral Reflux.

In this study, our aim was to evaluate the significance of the change in renal pelvis anterior-posterior diameter (RPAPD) before and after micturition between vesicoureteral reflux (VUR)-positive and -negative patients to whom had voiding cystourethrography (VCUG) was performed.In this study, 69 children, age ranging from 0 to 12 years, were included. Before the VCUG imaging, the RPAPD was measured first with a full bladder and then after urination via ultrasound (US). The differences between in RPAPD measurements were noted and values compared made among VUR-positive and -negative children. Data distribution was inhomogeneous, and the Wilcoxon Sign Rank test was utilized instead of Student t test. There was no statistically significant difference in prevoiding and postvoiding RPAPD in VUR (+) and VUR (-) patients (P = 0.672). There was no statistically significant relation between VUR and the presence of hydronephrosis (P = 0.126). Vesicoureteral reflux is more common in patients with urinary tract infections (UTI) (P = 0.001). There was no statistically significant relationship between prevoiding and postvoiding RPAPD change and VUR diagnosis (P = 0,164).Ultrasound is the modality of choice for urinary system evaluation. Diagnosis of hydronephrosis via US is not sufficient in predicting VUR; however, indirect findings may reveal the diagnosis. A decrease in RPAPD in postvoiding US evaluation may not rule out the VUR diagnosis for this reason further imaging modalities, such as VCUG, should be taken into consideration for the patients with clinical suspicion.

In utero myelomeningocele repair: The natural history of patients with incontinent pattern (sphincteric deficiency: leakage below 40 CMH20).

INTRODUCTION: Since 2011 we have been following prospectively myelomeningocele patients treated in utero with particular interest to patients with sphincter weakness/deficiency. We investigated the changes of bladder pattern and upper urinary tract with time in children who underwent in utero repair and had low-pressure incontinence based on urodynamic evaluation (UE). MATERIALS AND METHODS: From the 120 patients in our database, 117 had at least one UE. Of these, 30 were classified as incontinent when leaking at low pressure (<40 cmH20). We reviewed clinical evaluation, urinary tract ultrasound, voiding cystourethrography (VCUG), and other UE parameters at first and last evaluation. RESULTS: We found 30 cases (25.64%). Mean age at initial evaluation was 4.97 months followed by UE done initially at mean age of 5.73 months. Follow-up was 28.4 months. Febrile urinary tract infection has been found in four patients (13.3%), hydronephrosis in four patients, and bladder neck thickening in three (10%). The VCUG showed vesicoureteral reflux in three cases (3/27, 11.1%). A total of 90% of patients had detrusor overactivity with mean maximum detrusor pressure (33.37 cmH20). Only 16.67% of patients showed normal bladder capacity. From the 30 patients, 23 had at least two UE. We noticed a change of bladder pattern as follows: six patients became of high-risk pattern, five normal, and two with underactive bladder pattern. The average interval between the first and last UE was 25.5 months (median: 15 months). CONCLUSION: We concluded that 43.47% of patients with low DLPP have kept the incontinent pattern. If the initial LPP was below 30 cmH20, 70% remained with the incontinet pattern.

Urodynamic findings during the filling phase in neurogenic bladder patients with or without vesicoureteral reflux who have undergone sacral neuromodulation.

AIMS: To assess the urodynamic findings during the filling phase in neurogenic bladder patients with or without vesicoureteral reflux (VUR) who underwent sacral neuromodulation (SNM). METHODS: We retrospectively reviewed the records of 19 patients with neurogenic lower urinary tract dysfunction (NLUTD) who underwent SNM at our center from July 2018 to July 2019. Clinical data and video-urodynamic parameters were collected. VUR grading systems were used to evaluate upper urinary tract function. RESULTS: The mean test duration was 24 ± 8.2 days. The urodynamic evaluation showed a significant increase in the mean maximum cystometric capacity (136.3 ± 118.2 vs 216.5 ± 137.8 mL; P = .0071) and compliance (8.7 ± 8.52 vs18.3 + 16.47 mL/H2 O; P = .016), as well as a decrease in maximum intravesical pressure (57 ± 39.23 vs 36.58 ± 31.16 H2 O; P = .0064). In the voiding phase, none of the patients had automatic urination at the baseline and testing phases. In 8 of 19 patients who had detrusor overactivity (DO), the DO disappeared (four patients) or was delayed (four patients). The VUR in 3 of 12 ureter units disappeared. The grade of VUR or the volume before VUR improved in 8 ureter units, and the remaining 1 did not change significantly. An implant was performed in 16 cases. After permanent implantation, all patients needed intermittent catheterization to empty the bladder. CONCLUSIONS: This retrospective study indicates that SNM can improve the urinary storage function of the bladder in appropriate patients with NLUTD. For patients with VUR, SNM can cure or reduce VUR by improving DO and bladder compliance.

Accuracy of subjective vesicoureteral reflux timing assessment: supporting new voiding cystourethrogram guidelines.

BACKGROUND: Bladder volume at the onset of vesicoureteral reflux (VUR) is an important prognostic indicator of spontaneous resolution and the risk of pyelonephritis. OBJECTIVE: We aim to determine whether pediatric urologists and pediatric radiologists can accurately estimate the timing of reflux by examining voiding cystourethrogram (VCUG) images without prior knowledge of the instilled contrast volume. MATERIALS AND METHODS: Total bladder volume and the volume at the time of reflux were collected from VCUG reports to determine the volume at the onset of VUR. Thirty-nine patients were sorted into three groups: early-/mid-filling reflux, late-filling and voiding only. Thirty-nine images were shown to three pediatric urologists and two pediatric radiologists in a blinded fashion and they were then asked to estimate VUR timing based on the above categories. A weighted kappa statistic was calculated to assess rater agreement with the gold standard volume-based report of VUR timing. RESULTS: The mean patient age at VCUG was 3.1±2.9 months, the median VUR was grade 3, and 20 patients were female. Overall agreement among all five raters was moderate (k=0.43, 95% confidence interval [CI] 0.36-0.50). Individual agreement between rater and gold standard was slight to moderate with kappa values ranging from 0.13 to 0.43. CONCLUSION: Pediatric radiologists and urologists are unable to accurately and reliably characterize VUR timing on fluoroscopic VCUG. These findings support the recently published American Academy of Pediatrics protocol recommending the routine recording of bladder volume at the onset of VUR as a standard component of all VCUGs to assist in a more accurate assessment of the likelihood of resolution and risk of recurrent urinary tract infections.

A Primer on Congenital Anomalies of the Kidneys and Urinary Tracts (CAKUT).

Congenital anomalies of the kidneys and urinary tracts (CAKUT) are disorders caused by defects in the development of the kidneys and their outflow tracts. The formation of the kidneys begins at week 3 and nephrogenesis continues until week 36, therefore, the kidneys and outflow tracts are susceptible to environmental risk factors that perturb development throughout gestation. Many genes have been implicated in kidney and outflow tract development, and mutations have been identified in patients with CAKUT. In severe cases of CAKUT, when the kidneys do not form, the fetus will not survive. However, in less severe cases, the baby can survive with combined kidney and outflow tract defects or they may only be identified in adulthood. In this review, we will cover the clinical presentation of CAKUT, its epidemiology, and its long-term outcomes. We will then discuss risk factors for CAKUT, including genetic and environmental contributions. Although severe CAKUT is rare, low nephron number is a much more common disorder with its effect on kidney function increasingly apparent as a person ages. Low nephron number appears to arise by the same mechanisms as CAKUT, but it differs in terms of the magnitude of the insult and the timing of when it occurs during gestation. By understanding the causes of CAKUT and low nephron number, we can begin to identify preventive treatments and establish clinical guidelines for how these patients should be followed.

Lower Urinary Tract Dysfunction and Pediatric Renal Transplant.

OBJECTIVES: Lower urinary tract abnormalities have been considered to be contraindications for renal transplant. However, advancements in diagnosis and treatment in this area have allowed renal transplant as a treatment choice in selected cases. We evaluated clinical outcomes of pediatric renal transplant in patients with lower urinary tract dysfunction. MATERIALS AND METHODS: We retrospectively evaluated data from 165 pediatric renal transplant patients, and data were compared between patients with and without lower urinary tract dysfunction. Patient demographics, cause of chronic renal failure, acute rejection episodes, and graft loss were included in our analyses. RESULTS: Seventeen patients had lower urinary tract dysfunction, and the remaining 148 patients had functional lower urinary tracts. Patients with lower urinary tract dysfunction were younger than the other patient group at diagnosis of chronic renal failure. The mean follow-up after renal transplant in the 2 groups was similar. Differences with regard to donor type, immunosuppressive treatment, and acute rejection episodes were not significant between the 2 groups. Eight patients had lost their grafts during follow-up; however, only 1 of these patients was in the lower urinary tract dysfunction group. Graft loss rate was similar between the groups. CONCLUSIONS: Pediatric patients with lower urinary tract dysfunction had similar graft outcomes versus other pediatric renal transplant patients. Careful evaluation and preparation of the lower urinary tract are important factors for renal transplant success.

Effect of Early Oxybutynin Treatment on Posterior Urethral Valve Outcomes in Infants: A Randomized Controlled Trial.

PURPOSE: We studied the effect of oxybutynin on bladder and upper urinary tract outcomes in infants following posterior urethral valve ablation. MATERIALS AND METHODS: Patients younger than 12 months old who had undergone primary endoscopic valve ablation for posterior urethral valves were screened for eligibility. Patients who had undergone urinary diversion or had other conditions that could affect lower urinary tract function were excluded. Study patients were randomized to either oxybutynin (0.2 mg/kg 3 times daily) until toilet training or active observation. The study end points were serum creatinine, estimated glomerular filtration rate, hydronephrosis improvement, vesicoureteral reflux resolution, febrile urinary tract infection and toilet training. RESULTS: A total of 49 infants (24 receiving oxybutynin and 25 undergoing observation) were enrolled between December 2013 and September 2015 and completed at least 1 year of followup. Oxybutynin was discontinued before toilet training in 5 patients due to facial flushing in 2, bladder and upper tract dilatation in 2, and cognitive changes in 1. After a median followup of 44.2 months (range 12 to 57.6) median serum creatinine and estimated glomerular filtration rate were not significantly different between the groups (p=0.823 and p=0.722, respectively). Renal units in the oxybutynin group had a greater likelihood of hydronephrosis improvement (61.9% vs 34.8%, p=0.011) and resolution of vesicoureteral reflux (62.5% vs 25%, p=0.023). Febrile urinary tract infection (29.2% vs 40%, p=0.404), completion of toilet training (70.8% vs 76%, p=0.748) and age at toilet training (p=0.247) did not differ significantly between the oxybutynin and observation groups. CONCLUSIONS: Oxybutynin enhances hydronephrosis improvement and vesicoureteral reflux resolution following primary endoscopic valve ablation in infants but periodic monitoring is warranted.

[Diagnostic evaluation of reflux: significance of voiding cystourethrography]. / Refluxdiagnostik ­ Stellenwert des Miktionszystourethrogramms.

Radiological voiding cystourethrography has long been considered the gold standard for reflux testing in paediatric urology. Additional contrast-based procedures such as voiding urosonography or radionuclide cystography have been established in recent decades. Their main indication is reflux diagnosis after pyelonephritis in early infancy and childhood. The selection of the appropriate method is based on factors such as clinical question, availability, economic aspects, expenditure of human resources and time, and the goal of finding of method that provides as much information as possible while keeping radiation exposure to a minimum. Modern strategies aim for a risk-oriented, individual indication independent of the method of reflux testing.

[Long-term evolution of vesicoureteral reflux in children]. / L'évolution à long terme du reflux vésico-rénal chez l'enfant.

Vesicoureteral reflux is very common in children, requiring a long follow-up period to reduce its progression toward chronic renal failure. This study aims to analyze the epidemiological diagnostic features, the management of vesicoureteral reflux and long term course of patients with this disease. We conducted a retrospective study of 42 patients with suspected vesicoureteral reflux hospitalized in the Department of Visceral Paediatric Surgery at the Hassan II University Hospital of Fez over a period of 6 years from January 2010 to December 2015. Mean age of patients at diagnosis was 3 years and 2 months. The boy is to girl sex ratio was 1.8. Vesicoureteral reflux was isolated in 81% of patients and secondary or associated in 19% of patients. The most common initial manifestation was urinary tract infection (90.4%). Renal function was impaired in 54.8% of children. The treatment was based on antibiotics against diagnosed UTI (90.4%), antibiotic prophylaxis in the case of recurrence and Cohen reimplantation (97.62%). Surgical indication readily concerned all patients with grades IV and V vesicoureteral reflux (73.9%) as well as patients with impaired kidneys and 26.1% of patients after medical treatment. Early and late postoperative course was in general satisfactory: disappearance of vesicoureteral reflux in 92.68% of cases. Upper urinary tract regressed except for 9.52% of patients; 95.23% of patients had complete recovery of renal function. There was a reduction in UTI recurrence (in 19.04% of cases after surgery). The majority of parents judged positively the clinical course of their children (54.76%) after surgery.

Can distal ureteral diameter measurement predict primary vesicoureteral reflux clinical outcome and success of endoscopic injection?

OBJECTIVE: The objective of the study was to evaluate the predictive value of distal ureteral diameter ratio (UDR) on outcome of primary vesicoureteral reflux (VUR) and reflux resolution after endoscopic injection. PATIENTS AND METHODS: Three hundred eighty-three patients treated for primary VUR between January 2010 and October 2015 were retrospectively reviewed. The parameters analyzed were age at diagnosis, sex, grade and lateralite of VUR, complaints at admission (febrile urinary tract infection, antenatal hydronephrosis, family history),bladder-bowel dysfunction (urgency, incontinence, constipation), dimercaptosuccinic acid (DMSA) scintigraphy findings, follow-up period, clinical course (spontaneous resolution or surgical correction), time of spontaneous resolution, surgical treatment time and age, materials used for injection and success of endoscopic injection. Ureteral diameter ratio was calculated on the initial VCUG at the time of the diagnosis as the largest ureteral diameter within the false pelvis divided by the distance between L1-L3.The correlation between UDR, clinical outcome (spontaneous resolution/surgical correction) and success of endoscopic injection was evaluated by logistic regression analysis. To compare the effect of UDR and grade of reflux on spontaneous resolution, multivariate logistic regression analysis was performed in three models together with sex, age, resolution time, presence of febrile UTI and DMSA scan findings. RESULTS: Three hundred eighty-three patients were enrolled. There was a strong correlation between UDR and grade of reflux (p < 0,0001). Ureteral diameter ratio was higher in patients whose complaints at admission were family history and febrile UTI, but this correlation was not statistically significant (p > 0.05). When the correlation between UDR and the DMSA scan findings was evaluated, UDR was found to be significantly higher in patients with moderate and severe scarring. Bladder-bowel dysfunction was present in 111 patients (28.9%). There was no significant correlation between BBD and UDR (p > 0.05). 62 patients showed spontaneous resolution in a median duration of 1.55 years. The predictive value of UDR for spontaneous resolution was more significant than grade (p < 0.001).There was no spontaneous resolution in patients with UDR over 0.45.321 patients underwent operation (248 endoscopic injection, 17 ureteroneocystostomy, 56 endoscopic injection+ureteroneocystostomy). When the predictive value of the reflux grade and UDR in the success rate of endoscopic correction was compared, UDR was shown to be significantly more predictive than the grade of reflux (p < 0.05). Ureteral diameter ratio was significantly higher in patients whose injection treatment was unsuccessful. Each 0.05 unit increase in UDR affected the success of endoscopic injection negatively (95% CI:<0.001-0.071). CONCLUSION: Ureteral diameter ratio is an objective measurement of VUR and appears to be a new predictive tool for clinical outcome and success after endoscopic injection.

Voiding cystourethrography and 99MTC-MAG3 renal scintigraphy in pediatric vesicoureteral reflux: what is the role of indirect cystography?

BACKGROUND: Primary vesicoureteral reflux (VUR) is the most common urological anomaly in children. Voiding cystourethrography (VCUG) is considered the reference standard for the diagnosis of VUR. Even if it is a secure and standardized technique, it is still an invasive method, hence, the effort to find an alternative method to diagnose VUR. The aim of the study is to evaluate the diagnostic accuracy of 99mTC-MAG3 scintigraphy with indirect cystography in detecting VUR and to estimate any interobserver variability in 99mTC-MAG3 scintigraphy interpretation. METHODS: The authors retrospectively reviewed all the pediatric patients who underwent both a VCUG and a 99mTC-MAG3 renal scintigraphy at the study institution between 2012 and 2016. RESULTS: A total of 86 children (and 168 renal units) were included. MAG3 scan revealed a sensitivity of 54% and a specificity of 90% with positive predictive value of 79% and negative predictive value of 73%. Each MAG3 scintigraphy was then independently and blindly evaluated by a pediatric urologist and two nuclear physicians. After revision, the concordance between VCUG and MAG3 in reflux cases dropped from 54% to 27% (on average), and the reviewers reclassified most examinations as non-conclusive. CONCLUSIONS: 99mTC-MAG3 renal scintigraphy with indirect cystography showed low sensitivity in detecting VUR of any grade and cannot, therefore, be proposed as completely alternative to VCUG in the diagnosis of VUR. Moreover, MAG3 scintigraphy interpretation for the diagnosis of VUR has a very high interobserver variability, mostly because of the lack of a correct and complete voiding phase.

Clinical Spectrum of Congenital Anomalies of Kidney and Urinary Tract in Children.

OBJECTIVES: To evaluate the clinical spectrum and patterns of clinical presentation in congenital anomalies of kidney and urinary tract. METHODS: We enrolled 307 consecutively presenting children with congenital anomalies of kidney and urinary tract at the pediatric nephrology clinic. Patients were evaluated clinically, with serum biochemistry, appropriate imaging and radionuclide scans. RESULTS: The most common anomaly was primary vesicoureteric reflux (VUR) (87, 27.3%), followed by pelviureteral junction obstruction (PUJO) (62,20.1%), multicystic dysplastic kidney (51 16.6%), non-obstructive hydronephrosis (32, 10.4%) and posterior urethral valves (PUV) (23, 7.4%). 247 (80.4%) anomalies had been identified during the antenatal period. Another 33 (10.7%) were diagnosed during evaluation of urinary tract infection, and 21 (6.8%) during evaluation for hypertension at presentation. Obstructive anomalies presented earlier than non-obstructive (7 (3, 22.5) vs 10 (4, 24) mo: (P=0.01)). The median (IQR) ages of presentation for children with PUV (n=23), VUR (n=87) and PUJO (n=62) were 4 (2, 14) mo, 10 (5, 27) mo, and 7 (3, 22.5) mo, respectively. Nine (2.9%) children had extrarenal manifestations. CONCLUSIONS: The median age at clinical presentation for various subgroups of anomalies indicates delayed referral. We emphasize the need for prompt referral in order to initiate appropriate therapeutic strategies in children with congenital anomalies of kidney and urinary tract.

Bilateral duplex collecting system with bilateral vesicoureteral reflux: a case report.

BACKGROUND: A bilateral duplex collecting system is an unusual renal tract abnormality. Vesicoureteral reflux may be associated. We describe a rare case of bilateral duplex collecting system with bilateral vesicoureteral reflux in which the refluxing ureter on the left side drains the upper pole moiety contrary to what is often found. CASE PRESENTATION: A 24-year-old married Arab woman presented with ascending left-sided flank pain during micturition. She complained of recurrent urinary tract infections. A physical examination and laboratory tests were normal. Voiding cystourethrography and computed tomography scan detected bilateral duplex collecting system, grade IV vesicoureteral reflux on the left side, and grade I vesicoureteral reflux on the right. She underwent left heminephrectomy and dextranomer/hyaluronic acid injections on the right side. After a year of follow-up, a clinical examination and imaging findings were unremarkable. CONCLUSIONS: A bilateral duplex collecting system with refluxing upper pole moiety ureter is a very rare entity. The diagnosis should be suspected when exploring any flank pain with recurrent urinary tract infections to avoid subsequent renal impairment. Furthermore, this case shows how some common symptoms may lead to finding an unexpected urinary tract abnormality.

Utilidad de la clasificación de los patrones de disfunción miccional en el pronóstico del primer tratamiento endoscópico del reflujo vesicoureteral en niños mayores de 3 años / Usefulness of the classification of urinary dysfunction for the prognosis of the first endoscopic treatment of vesicoureteral reflux in children over the age of 3

Objetivos: Valorar el rendimiento diagnóstico de los patrones de micción disfuncional asociados al reflujo vesicoureteral (RVU) en niños mayores de 3 años, en función del resultado del primer tratamiento endoscópico (T1END), agrupados en una clasificación diseñada por nuestro grupo (CMD.URI-La Fe) y su comparación con otras clasificaciones existentes como la de Van Batavia et al. Material y métodos: Estudio transversal ambispectivo de una muestra de 50 niños. Criterios de exclusión: TEND previo, edad ≤ 3 años, anomalías anatómicas o neurológicas y antecedentes de cirugía uretral o abdominopélvica. Previamente al T1END se realizó una valoración de la función de vaciado vesical mediante flujometría + electromiografía (UF-EMG) y del residuo posmiccional (ecografía). Se obtuvieron otras variables procedentes del diario miccional, el espesor de la pared vesical premiccional (ecografía), así como variables clínicas. La corrección del RVU fue valorada mediante cistografía isotópica a los 3 meses del tratamiento. Se realizó una clasificación de los patrones miccionales en función de las variables significativas (URI-La Fe) y se valoró su rendimiento diagnóstico, comparándola al clasificar a los pacientes según propone Van Batavia et al. Resultados: Media de edad: 6,8 ± 2,28 años. Varones/mujeres (44%/56%). Grados de RVU: leve, moderado, severo. Tasa de corrección del RVU T1END: 77% (n = 38). Rendimiento diagnóstico (Van Batavia; URI-La Fe): predicción correcta (37,5%; 75%), sensibilidad (32,4%; 87,8%), especificidad (54,5%; 46,6%), valor predictivo positivo (70,6; 78,3%) y valor predictivo negativo (19,4%; 63,6%). Conclusiones: Nuestros resultados muestran la utilidad del estudio no invasivo y la clasificación de la disfunción miccional del niño mayor de 3 años previo al primer tratamiento endoscópico del RVU

Objectives: To assess the diagnostic performance of urinary dysfunction patterns associated with vesicoureteral reflux (VUR) in children over the age of 3 according to the result of the first endoscopic treatment (ENDT1), grouped into a classification designed by our group (CMD.URI-La Fe). Comparison with other current classifications such as that of Van Batavia et al. Material and methods: Ambispective cross-cutting study of a sample of 50 children. Exclusion criteria: previous ENDT, age ≤ 3 years, anatomical or neurological anomalies and a history of ureteral or abdominopelvic surgery. Prior to the ENDT1, a bladder voiding function assessment by uroflowmetry+electromyography (UF-EMG) and post-mictional residue (ultrasound). Other variables from the bladder diary, pre-mictional bladder wall thickness and other clinical variables. The correction of VUR was assessed by isotope cystography 3 months after the treatment. Urinary patterns were classified according to the significant variables (URI-La Fe), and the diagnostic performance of this classification was assessed, comparing it to the classification of patients as proposed by Van Batavia et al. Results: Mean age: 6.8±2.28 years. Males/females (44%/56%). Grades of VUR (mild/moderate/severe). ENDT1 VUR correction rate: 77% (n=38). Diagnostic performance (Van Batavia; URI-La Fe): correct prediction (37.5%; 75%), sensitivity (32.4%; 87.8%), specificity (54.5%; 46.6%), positive predictive value (70.6; 78.3%) and negative predictive value (19.4%; 63.6%). Conclusions: Our results show the usefulness of the non-invasive test and the classification of urinary dysfunction in children aged over 3 years prior to the first endoscopic treatment of VUR

Usefulness of the classification of urinary dysfunction for the prognosis of the first endoscopic treatment of vesicoureteral reflux in children over the age of 3. / Utilidad de la clasificación de los patrones de disfunción miccional en el pronóstico del primer tratamiento endoscópico del reflujo vesicoureteral en niños mayores de 3 años.

OBJECTIVES: To assess the diagnostic performance of urinary dysfunction patterns associated with vesicoureteral reflux (VUR) in children over the age of 3 according to the result of the first endoscopic treatment (ENDT1), grouped into a classification designed by our group (CMD.URI-La Fe). Comparison with other current classifications such as that of Van Batavia et al. MATERIAL AND METHODS: Ambispective cross-cutting study of a sample of 50 children. EXCLUSION CRITERIA: previous ENDT, age ≤ 3 years, anatomical or neurological anomalies and a history of ureteral or abdominopelvic surgery. Prior to the ENDT1, a bladder voiding function assessment by uroflowmetry+electromyography (UF-EMG) and post-mictional residue (ultrasound). Other variables from the bladder diary, pre-mictional bladder wall thickness and other clinical variables. The correction of VUR was assessed by isotope cystography 3 months after the treatment. Urinary patterns were classified according to the significant variables (URI-La Fe), and the diagnostic performance of this classification was assessed, comparing it to the classification of patients as proposed by Van Batavia et al. RESULTS: Mean age: 6.8±2.28 years. Males/females (44%/56%). Grades of VUR (mild/moderate/severe). ENDT1 VUR correction rate: 77% (n=38). Diagnostic performance (Van Batavia; URI-La Fe): correct prediction (37.5%; 75%), sensitivity (32.4%; 87.8%), specificity (54.5%; 46.6%), positive predictive value (70.6; 78.3%) and negative predictive value (19.4%; 63.6%). CONCLUSIONS: Our results show the usefulness of the non-invasive test and the classification of urinary dysfunction in children aged over 3 years prior to the first endoscopic treatment of VUR.

Can we predict vesicoureteral reflux resolution in patients with non-neurogenic lower urinary tract dysfunction?

OBJECTIVE: To analyze factors influencing reflux resolution in patients with the coexistence of non-neurogenic lower urinary tract dysfunction and vesicoureteral reflux. METHODS: The data of 153 children who were diagnosed with vesicoureteral reflux and accompanying non-neurogenic lower urinary tract dysfunction between 2010 and 2015 were retrospectively evaluated. Patients with neurogenic and anatomical malformations, monosymptomatic nocturnal enuresis, previous history of vesicoureteral reflux surgery, irregular and/or incomplete follow-up data were excluded. After exclusion of 55 patients, 98 patients were enrolled in this study. Patients were divided into two groups according to the presence of spontaneous vesicoureteral reflux resolution during the follow-up period. Group 1 consisted of 54 children with spontaneous vesicoureteral reflux resolution, whereas group 2 included 44 children without resolution. Medical history, physical examination, urinalysis, uroflowmetry combined with electromyography, ultrasonography, as well as the Dysfunctional Voiding and Incontinence Symptom Score questionnaire were also evaluated. RESULTS: The mean age at presentation was 7.57 ± 0.23 years (range 5-13 years), and the mean follow-up period was 28.3 months. Significant differences were noted between the two groups in terms of dysfunctional voiding and incontinence symptom score, bladder wall thickness, and the post-void residual urine volumes. In addition, lower urinary tract symptoms, namely frequency, urgency and daytime incontinence, were found to be higher in group 2. In multivariate analysis, post-void residual urine volume and Dysfunctional Voiding and Incontinence Symptom Score were found to affect reflux resolution rates (P = 0.002, P = 0.002, respectively). CONCLUSIONS: The absence of significant post-void residual urine volume, and a low Dysfunctional Voiding and Incontinence Symptom Score increase the likelihood of spontaneous resolution rates of vesicoureteral reflux in children with non-neurogenic lower urinary tract dysfunction.