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The efficacy of continuous antibiotic prophylaxis in preventing urinary tract infection (UTI) in infants with grade III, IV, or V vesicoureteral reflux is controversial. METHODS In this investigator-initiated, randomized, open-label trial performed in 39 European centers, we randomly assigned infants 1 to 5 months of age with grade III, IV, or V vesicoureteral reflux and no previous UTIs to receive continuous antibiotic prophylaxis (prophylaxis group) or no treatment (untreated group) for 24 months. The primary outcome was the occurrence of the first UTI during the trial period. Secondary outcomes included new kidney scarring and the estimated glomerular filtration rate (GFR) at 24 months. RESULTS A total of 292 participants underwent randomization (146 per group). Approximately 75% of the participants were male; the median age was 3 months, and 235 participants (80.5%) had grade IV or V vesicoureteral reflux. In the intention-to-treat analysis, a first UTI occurred in 31 participants (21.2%) in the prophylaxis group and in 52 participants (35.6%) in the untreated group (hazard ratio, 0.55; 95% confidence interval [CI], 0.35 to 0.86; P=0.008); the number needed to treat for 2 years to prevent one UTI was 7 children (95% CI, 4 to 29). Among untreated participants, 64.4% had no UTI during the trial. The incidence of new kidney scars and the estimated GFR at 24 months did not differ substantially between the two groups. Pseudomonas species, other non-Escherichia coli organisms, and antibiotic resistance were more common in UTI isolates obtained from participants in the prophylaxis group than in isolates obtained from those in the untreated group. Serious adverse events were similar in the two groups. CONCLUSIONS In infants with grade III, IV, or V vesicoureteral reflux and no previous UTIs, continuous antibiotic prophylaxis provided a small but significant benefit in preventing a first UTI despite an increased occurrence of non-E. coli organisms and antibiotic resistance. (Funded by the Italian Ministry of Health and others; PREDICT ClinicalTrials.gov number, NCT02021006; EudraCT number, 2013-000309-21.) [ABSTRACT FROM AUTHOR]

Significance Statement: Congenital obstructive uropathy (COU) is a prevalent human developmental defect with highly heterogeneous clinical presentations and outcomes. Genetics may refine diagnosis, prognosis, and treatment, but the genomic architecture of COU is largely unknown. Comprehensive genomic screening study of 733 cases with three distinct COU subphenotypes revealed disease etiology in 10.0% of them. We detected no significant differences in the overall diagnostic yield among COU subphenotypes, with characteristic variable expressivity of several mutant genes. Our findings therefore may legitimize a genetic first diagnostic approach for COU, especially when burdening clinical and imaging characterization is not complete or available., Background: Congenital obstructive uropathy (COU) is a common cause of developmental defects of the urinary tract, with heterogeneous clinical presentation and outcome. Genetic analysis has the potential to elucidate the underlying diagnosis and help risk stratification., Methods: We performed a comprehensive genomic screen of 733 independent COU cases, which consisted of individuals with ureteropelvic junction obstruction ( n =321), ureterovesical junction obstruction/congenital megaureter ( n =178), and COU not otherwise specified (COU-NOS; n =234)., Results: We identified pathogenic single nucleotide variants (SNVs) in 53 (7.2%) cases and genomic disorders (GDs) in 23 (3.1%) cases. We detected no significant differences in the overall diagnostic yield between COU sub-phenotypes, and pathogenic SNVs in several genes were associated to any of the three categories. Hence, although COU may appear phenotypically heterogeneous, COU phenotypes are likely to share common molecular bases. On the other hand, mutations in TNXB were more often identified in COU-NOS cases, demonstrating the diagnostic challenge in discriminating COU from hydronephrosis secondary to vesicoureteral reflux, particularly when diagnostic imaging is incomplete. Pathogenic SNVs in only six genes were found in more than one individual, supporting high genetic heterogeneity. Finally, convergence between data on SNVs and GDs suggest MYH11 as a dosage-sensitive gene possibly correlating with severity of COU., Conclusions: We established a genomic diagnosis in 10.0% of COU individuals. The findings underscore the urgent need to identify novel genetic susceptibility factors to COU to better define the natural history of the remaining 90% of cases without a molecular diagnosis., (Copyright © 2023 by the American Society of Nephrology.)

Congenital obstructive nephropathy (CON) is one of the most common causes of chronic kidney disease in children. The aim of the study was to investigate serum and urine periostin and cytokeratin-18 (CK-18) in children with CON in relation to CON etiology, treatment, and kidney injury. We evaluated 81 children with CON secondary to ureteropelvic junction obstruction (UPJO), ureterovesical junction obstruction (UVJO), posterior urethral valves (PUV) and 60 controls. Neither biomarker demonstrated any relation to CON etiology. However, all patients showed significantly higher urine periostin (uPeriostin) and uPeriostin/Cr levels than the controls. Also, UVJO patients showed higher sCK-18 and uCK-18/Cr levels, and PUV patients showed higher uCK-18/Cr levels than the controls. Neither biomarker was found to have any relation to CON treatment. However, conservatively treated children and those before and after surgery showed significantly higher uPeriostin and uPeriostin/Cr levels than the controls. uPeriostin strongly correlated with differential renal function (DRF) < 40%. The ROC analysis demonstrated the best area under the curve (AUC) for uPeriostin (0.831) and uPeriostin/Cr (0.768), and low for sPeriostin (0.656) and uCK-18 (0.615) for detecting renal injury. In conclusion, although serum and urine periostin and CK-18 did not display any relation to etiology or the type of CON treatment, uPeriostin seems to be a useful tool for detecting renal injury in children with CON, especially due to its strong negative correlation with DRF < 40%., Competing Interests: The authors declare no conflict of interest., (Copyright © 2022 Termedia.)

Congenital obstructive nephropathy (CON) leads to renal fibrosis and chronic kidney disease. The aim of the study was to investigate the predictive value of urinary endoglin, periostin, cytokeratin-18, and transforming growth factor-β1 (TGF-β1) for assessing the severity of renal fibrosis in 81 children with CON and 60 controls. Children were divided into three subgroups: severe, moderate scars, and borderline lesions based on 99mTc-ethylenedicysteine scintigraphy results. Periostin, periostin/Cr, and cytokeratin-18 levels were significantly higher in the study group compared to the controls. Children with severe scars had significantly higher urinary periostin/Cr levels than those with borderline lesions. In multivariate analysis, only periostin and cytokeratin-18 were independently related to the presence of severe and moderate scars, and periostin was independently related to borderline lesions. However, periostin did not differentiate advanced scars from borderline lesions. In ROC analysis, periostin and periostin/Cr demonstrated better diagnostic profiles for detection of advanced scars than TGF-β1 and cytokeratin-18 (AUC 0.849; 0.810 vs. 0.630; 0.611, respectively) and periostin for detecting borderline lesions than endoglin and periostin/Cr (AUC 0.777 vs. 0.661; 0.658, respectively). In conclusion, periostin seems to be a promising, non-invasive marker for assessing renal fibrosis in children with CON. CK-18 and TGF-β1 demonstrated low utility, and endoglin was not useful for diagnosing advanced scars.

Background: Vanin-1, an epithelial glycosylphosphatidylolinositol (GPI)-anchored pantetheinase, is a valuable marker of renal injury., Purpose: The aim of this study was to assess the predictive value of vanin-1 in acute pyelonephritis (APN) in comparison to the conventional serum inflammatory markers in children aged 1-24 months with the first episode of urinary tract infection (UTI)., Material and Methods: Urinary vanin-1, vanin-1/Cr ratio, WBC, CRP, PCT were analysed in 58 children with febrile UTI and in 18 children with non-febrile UTI. Febrile UTI group was divided into APN subgroup ( n =  29) and non-APN subgroup ( n =  29), based on the results of Tc-99m-ethylenedicysteine scan., Results: The mean vanin-1 level was higher in the APN group compared to the non-febrile UTI group ( p =  0.02) and did not differ between APN and non-APN subgroup. In univariate analysis, vanin-1 ( p =  0.042), CRP ( p <  0.001), PCT ( p <  0.001), and WBC ( p =  0.022), were associated with APN, but only vanin-1 ( p =  0.048) and CRP ( p =  0.002) were independent markers of APN. In ROC analysis, vanin-1, with its best cut-off value of 16.53 ng/mL, had worse diagnostic profile (AUC 0.629, sensitivity 58,6%, specificity 63.8%) than CRP, PCT and WBC (AUC: 0.937; 0.880; 0.667, respectively)., Conclusions: Vanin-1 is not useful for predicting APN, since its diagnostic value is inferior to other conventional serum inflammatory markers.

Aim: The aim of the study was to assess clinical profile of neonates with hypernatremic dehydration (HD) and identify risk factors associated with acute kidney injury (AKI)., Materials and Methods: A retrospective study included 18 neonates with HD (serum Na ≥150 mmol/L) hospitalized in the Department of Pediatrics and Nephrology between the years 2009-2019., Results: The age at presentation was 7.5±4.7 days (range 2-18), weight loss was 15.9±8.3% (range 7.1-32.6) and serum Na range was 151- 192 mmol/L (median 155.5 mmol/L). In 12 (67%) neonates, breast or mixed fed, HD occurred due to inadequate milk intake, in 6 (33%) neonates feeding difficulties were secondary to an acute infection. There was positive correlation between serum Na level and percentage weight loss at presentation (r=0.88; p<0.001). In 6 (33%) patients serious complications of HD were found: AKI in 5 patients, convulsions in one. Percentage weight loss was significantly higher in neonates with HD-associated AKI than in neonates with HD without AKI (p<0.01). Serum Na level was marginally higher in neonates with AKI than in those without AKI (p=0.08). In univariate logistic regression analysis, higher percentage of weight loss and higher serum Na level at presentation were important diagnostic factors of AKI in neonates with HD (both p<0.05). ROC analysis determined good diagnostic profile only for percentage weight loss, with a best cut-off value of 24.8%, for predicting AKI in neonates with HD (AUC 0.862, sensitivity 80%, specificity 100%)., Conclusions: Neonatal HD mostly occurs due to inadequate milk intake in breast or mixed fed babies, and rarely due to feeding difficulties in babies affected by an acute infection. Percentage weight loss at presentation has strong association with neonatal HD and is the most important factor of AKI in neonates with HD., (© 2020 MEDPRESS.)

Introduction: Fever and elevated inflammatory markers have been used for diagnosis of acute pyelonephritis (APN) in infants with urinary tract infection (UTI).The aim of the study was to compare the usefulness of serum neutrophil gelatinase-associated lipocalin (sNGAL) with inflammatory markers for predicting APN in infants with UTI., Material and Methods: The prospective study included 46 infants with a first episode of UTI, divided into two groups (APN 23, lower UTI 23), according to the DMSA scan results. The following laboratory tests were performed: sNGAL, PCT, CRP, WBC, and ESR., Results: Significantly elevated levels of sNGAL, PCT, CRP, and ESR were observed in infants with APN compared to those with lower UTI. Higher sNGAL, CRP, and ESR values, presence of fever, and longer duration of fever before antibiotic treatment were associated with APN [odds ratio (OR) 1.02, 1.27, 1.03, 13.46, 2.12, respectively]. Receiver operating characteristic (ROC) analysis showed better diagnostic profiles for sNGAL, PCT, and CRP than for ESR for predicting APN [area under the curve (AUC) 0.808, 0.819, 0.841, and 0.750, respectively]. The appropriate cut-off values of sNGAL, PCT and CRP were 100.8 ng/ml, 0.15 ng/ml, 5.3 mg/dl (all sensitivity and specificity 82.6%), and that of ESR was 40 mm/h (sensitivity 78.3%, specificity 60.9%)., Conclusions: sNGAL shows similar usefulness as PCT and CRP for predicting APN in infants with UTI, the diagnostic value of ESR is smaller, and WBC is not useful at all. The presence of fever and longer duration of fever are important predictors of APN in infants with UTI., Competing Interests: The authors declare no conflict of interest.

Introduction: We assessed whether two urinary biomarkers of acute kidney injury, neutrophil gelatinase associated lipocalin (uNGAL) and kidney injury molecule-1 (uKIM-1), can be useful for predicting acute pyelonephritis (APN) in children aged 1-24 months with the first febrile urinary tract infection (UTI)., Material and Methods: A prospective study included 54 children divided into two groups (24 with APN, 30 with lower UTI), according to the dimercaptosuccinic acid (DMSA) renal scintigraphy results. Laboratory tests: uNGAL, uKIM-1, procalcitonin (PCT), C-reactive protein (CRP), white blood count (WBC) were performed., Results: We did not find significant differences in normalized and non-normalized values of uNGAL and uKIM-1 in children with APN and lower UTI. Positive correlations were determined between uNGAL and pyuria (r = 0.28, p < 0.05) and between uNGAL/uCr and uKIM-1/uCr (r = 0.53, p < 0.001) in the all UTI groups. Univariate logistic regression analysis demonstrated that only PCT (p < 0.0001) and CRP (p < 0.05) were important diagnostic factors of APN. Receiver operating curve (ROC) analysis showed good diagnostic profiles of PCT with the best cut-off value of 1.66 ng/ml and of CRP with the best cut-off value of 4.3 mg/dl for predicting APN (area under the curve [AUC]: 0.894 and 0.719, sensitivity: 75% and 96%, specificity: 93% and 43%, respectively)., Conclusions: uNGAL and uKIM-1 are not effective diagnostic markers for APN in young children with febrile UTI and cannot be used in clinical practice to differentiate APN from lower UTI., Competing Interests: The authors declare no conflict of interest.

The incidence of urolithiasis in infants is unknown. The aim of this study was to investigate clinical characteristics, nutrition, calcium, phosphate, 25-hydroxyvitamin D (25(OH)D), alkaline phosphate, and parathyroid hormone in infants with urolithiasis. There were 32 infants (23 boys and 9 girls) of the mean age of 6.4 ± 3.7 months (range 2-12 months), with diagnosis of urolithiasis enrolled into the study. Boys were younger than girls (5.3 vs. 9.1 months, respectively; p < 0.05). The infants were receiving prophylactic vitamin D 3 . Twenty-one of them were fed with milk formula, 9 were breastfed, and 2 were on a mixed diet. The major clinical symptoms consisted of irritability in 19 (59%) and urinary tract infection in 6 (19%) infants. Hypercalcemia and hyperphosphatemia were detected in the serum in 30 (94%) and 19 (60%) infants, respectively. The serum calcium level was higher in boys than girls (10.8 vs. 9.8 mg/dL, respectively; p < 0.05). Four (12.5%) infants had increased activity of alkaline phosphatase. The serum level of 25(OH)D was high in 3 (9%), low in 2 (6%), and normal in 27 (85%) infants. Parathyroid hormone was low in eight (25%) infants. Hypercalciuria and hyperphosphaturia were found in 11 (34%) boys and 8 (25%) girls. Family history of urolithiasis was positive in eight (25%) infants. We conclude that urolithiasis occurs in infancy more often in boys fed with milk formula and in those who received vitamin D supplementation. Hypercalcemia, hyperphosphatemia, and hypercalciuria are the most common changes present in clinical metabolic tests.

Introduction: Kidney injury molecule-1 (KIM-1) is an important diagnostic and prognostic marker in acute kidney injury and chronic kidney disease of various aetiologies. The aim of the study was to evaluate the usefulness of serum KIM-1 (sKIM-1) and urine KIM-1 (uKIM-1) for predicting febrile and non-febrile urinary tract infection (UTI) in infants., Material and Methods: A prospective study included 101 children divided into three groups: febrile UTI 49 children, non-febrile UTI 22 children, and healthy controls 30 children. The following laboratory tests were performed: sKIM-1, uKIM-1, white blood count (WBC), C-reactive protein (CRP), and procalcitonin (PCT)., Results: Median levels of sKIM-1 were significantly higher in the febrile and non-febrile UTI group compared to the healthy controls (both p < 0.05). Mean levels of uKIM-1 were significantly lower in the febrile UTI group compared to the non-febrile UTI group and healthy controls (p < 0.001 and p < 0.0001, respectively). Univariate logistic regression analysis has demonstrated a positive association of sKIM-1 with febrile and non-febrile UTI (both p < 0.05), and negative association uKIM-1 with febrile UTI (p < 0.0001). Receiver operating curve (ROC) analysis showed good diagnostic profiles of uKIM-1 with a best cut-off value of 2.4 ng/ml and sKIM-1 with a best cut-off value of 3.88 ng/ml for predicting febrile UTI (area under the curve [AUC] 0.82 and 0.67, sensitivity 73% and 63%, specificity 86% and 80%, respectively)., Conclusions: sKIM-1 can be useful for predicting febrile UTI. We do not recommended use of uKIM-1 as a marker of febrile UTI because of its negative association with febrile UTI. Both markers are not useful for predicting non-febrile UTI., Competing Interests: The authors declare no conflict of interest., (Copyright: © 2019 Polish Society of Experimental and Clinical Immunology.)

Pyonephrosis in the course of hydronephrosis usually provides to total or near-total loss of renal function. In adults pyonephrosis usually results from urolithiasis. In children usually congenital urinary tract anomalies are present as contributing factors., Case Report: 6-year old girl was admitted to the hospital because of 2-day history of high fever, abdominal pain, progressive deterioration of general condition and ultrasonographically (US) detected left hydronephrosis. US on admission showed grossly dilated left renal collecting system together with extensive echogenic debris and laboratory tests highly elevated inflammatory markers. Once diagnosis of pyonephrosis was established on the basis of clinical picture and US as well as laboratory tests results, nephrostomy tube was placed percutaneously. Pus culture obtained during placement of nephrostomy showed E.Coli ESBL (-) growth. Intensive antibiotic treatment (Meropenem) was continued for 3 weeks, nephrostomy was removed after 12 days after receiving normal urine. Further evaluation of urinary tract (US and computed tomography urography) showed large hydronephrosis due to ureteropelvic junction obstruction, while dynamic scintigraphy obstructive renogram with grossly diminished left kidney function to 20% of differential renal function. The girl was referred for operative treatment on urgent basis. Intraoperatively long distance utreteropelvic junction stenosis was found and dismembered Anderson- Hynes pyeloplasty was performed. During 2-years follow-up postoperatively US showed gradually decrease of dilatation of left renal collecting system, while dynamic scintigraphy revealed permanent improvement of drainage together with almost normal renal function (up to 45%). No urinary tract infections were noted., Conclusions: Proper management of pyonephrosis in hydronephrotic kidney due to congenital ureteropelvic junction obstruction enables good final result of treatment., (© 2018 MEDPRESS.)

Early diagnosis of urinary tract infection (UTI) is challenging in infants due to unspecific symptoms, difficulty in urine collection and possible contamination. The aim of this study was to assesses the usefulness of serum and urine neutrophil gelatinase-associated lipocalin (sNGAL and uNGAL, respectively) in the diagnosis of febrile and non-febrile UTI in infants. This prospective observational study enrolled 66 infants with the first episode of UTI and 18 healthy controls. At the time of enrollment, sNGAL, uNGAL, urinalysis, urine culture, white blood cell count (WBC), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), procalcitonin (PCT), and serum creatinine (sCr) were assessed. We found that, on average, both sNGAL and uNGAL levels were significantly higher in febrile UTI, compared to non-febrile UTI and controls. In turn, the mean sNGAL level, but not uNGAL, was significantly higher in the non-febrile UTI group compared to controls. sNGAL positively correlated with WBC, CRP, ESR and PCT, and uNGAL with CRP and leukocyturia. The receiver operating curves (ROC) demonstrate that the optimum cut-off of 76.2 ng/ml for sNGAL (sensitivity 92.9%, specificity 94.4%, and the area under the curve (AUC) of 0.98) and of 42.2 ng/ml for uNGAL (sensitivity 73.8%, specificity 72.2%, and AUC of 0.76) for diagnosing febrile UTI and 39.0 ng/ml for sNGAL (sensitivity 83.3%, specificity 55.6%, and AUC of 0.70) for diagnosing non-febrile UTI. In conclusion, serum NGAL is an excellent marker for the early diagnosis of febrile UTI, with sensitivity and specificity higher than those of urine NGAL. Diagnostic sensitivity of serum NGAL is smaller in non-febrile infants suffering from UTI, and urine NGAL is not useful for this purpose at all.

In patients with autosomal dominant polycystic kidney disease (ADPKD) coexisting abnormalities of the urinary tract are considered rare. Only a few patients with ADPKD and congenital abnormalities of the kidney and urinary tract- renal agenesis, hypoplasia, aplasia, horseshoe kidney, ectopic multicystic dysplasic kidney, or subpelvic junction obstruction were reported. Renal agenesis occurs in approximately 1 in 1.500.000-3.000.000 patients with ADPKD. We report a boy with ADPKD and renal agenesis diagnosed at the age of 12 years. ADPKD was diagnosed in some other members of the family. Additionally to kidney changes, mitral valve prolapse was found on echocardiography. At the age of 18 years high normal blood pressure was recognized and laboratory tests demonstrated: serum creatinine 1.0 mg/dl, glomerular filtrate rate 97.9 ml/min/1.73m2 , isotopic creatinine clearance (Tc-99mDTPA) 99 ml/min/1.73m2 , normal urinalysis, no microalbuminuria., Conclusions: In children with positive family history of ADPKD, screening ultrasonography of the kidney performed at the request of the family, allows the early diagnosis of sporadic present abnormalities of the kidney and urinary tract.

Introduction: The endoscopic correction of vesicoureteral reflux (VUR) in children is a currently well accepted therapy in many pediatric urology centers. Polyacrylate-polyalcohol copolymer (PPC), namely Vantris ® , is one of the tissue-augmenting substances used for endoscopic reflux therapy. The aim of this study was to evaluate the results with PPC in children., Material and Methods: From 2012 to 2016, 125 children (73 girls and 52 boys) aged 0.6-17.9 years (mean 4.9 ±3.58) were treated with PPC. VUR was unilateral in 64 and bilateral in 61 patients, comprising 197 renal refluxing units (RRUs) grades: II in 72, III in 50, IV in 33 and V in 42. Of these primary reflux was present in 132 RRUs and 65 were complex cases. Voiding cystourethrogram (VCUG) was done 3 months after procedure., Results: Follow-up was completed in 89.6% of patients (112 children), and 89.8% of RRUs (177 out of 197). Reflux resolved in 86.4% of RRUs after single injection, in 99.4% after second and in 100% after the third. The only significant, but serious complication observed was late ureteral obstruction after PPC injection correcting high grade reflux, which required ureteral re-implantation. This complication was found in 9 out of 112 children (8%), and in 11 out of 177 RRUs (6.2%), 1.1 -2.9 years (mean 2 ±0.7) after the PPC injection. The longest follow-up reaches 4.5 years., Conclusions: Our data show that the PPC injection is an effective procedure for treating all grades of VUR with high success rate. However, because of the possibility of late ureteral obstruction, which requires ureteroneocystostomy, long-term follow-up is mandatory.

Unlabelled: The prevalence of obesity in children is still rising all over the world. The most common reason for significant weight gain is a high-calorie diet and decreased physical activity. However, apart from environmental factors, genetic predisposition plays a crucial role in the pathomechanism of obesity. We present the case of a boy with pathological obesity and Bardet-Biedl syndrome (BBS). BBS is a ciliopathy, a heterogeneous group of rare disorders associated with defects in primary cilia. Other clinical signs and symptoms of BBS are: polydactyly, hypertension, hyperlipidemia, hypogonadotrophic hypogonadism, intellectual disability, rod-cone dystrophy, genitourinary and renal abnormalities., Conclusions: genetic factors of rapid weight gain should be taken into consideration in a child with obesity. Polydactyly can be associated with ciliopathy. A patient with Bardet-Biedl syndrome requires multi-specialist care.

Background: Hydronephrosis is the one of the most common congenital abnormalities of urinary tract. The left kidney is more commonly affected than the right side and is more common in males., Aim of the Study: To determine the role of ultrasonography, renal dynamic scintigraphy and lowerdose computed tomography urography in preoperative diagnostic workup of infant with extreme hydronephrosis., Case Report: We presented the boy with antenatally diagnosed hydronephrosis. In serial, postnatal ultrasonography, renal scintigraphy and computed tomography urography we observed slightly declining function in the dilated kidney and increasing pelvic dilatation. Pyeloplasty was performed at the age of four months with good result., Conclusions: Results of ultrasonography and renal dynamic scintigraphy in child with extreme hydronephrosis can be difficult to asses, therefore before the surgical procedure a lower-dose computed tomography urography should be performed.

Introduction: Urinary tract infection (UTI) occurs in 1.1% of girls and 1.4% of boys during the first year of life. Asymptomatic bacteriuria (ABU) is usually detected incidentally in 0.9% of girls and 2.5% of boys at this age. The aim of the study was to assess the usefulness of measurement of pro-inflammatory urine interleukin (IL)-6 and IL-8 concentrations and anti-inflammatory transforming growth factor β1 (TGF-β1) level in infants with febrile UTI, non-febrile UTI and ABU., Material and Methods: A total of 35 children, mean age 6.14 ±3.47 months, were divided into three groups: group I - febrile UTI (n = 13), group II - non-febrile UTI (n = 13) and group III - ABU (n = 9). At the time of enrollment urine IL-6, IL-8, TGF-β1 and serum C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and white blood cell count (WBC) were measured. Renal ultrasound was performed in all children, 99m Tc-dimercaptosuccinic acid scintigraphy (DMSA) and voiding cystourethrography in children with UTI., Results: Urine concentrations of IL-6 and IL-8 were significantly higher in febrile UTI compared to those with non-febrile UTI and ABU (p < 0.5, p < 0.01) and positively correlated with CRP, ESR and WBC (p < 0.01). Urine levels of TGF-β1 were significantly higher in children with febrile UTI compared to those with ABU (p < 0.05) and positively correlated with WBC (p < 0.01). Inflammatory changes in the DMSA scan were detected in 66.6% of children with UTI. No significant difference in frequency of an abnormal DMSA scan compared to a normal scan was found in groups with febrile and non-febrile UTI. No relations between urine cytokines, systemic inflammatory markers and changes in DMSA scan were observed. The cutoff value for detection of inflammatory changes in the DMSA scan for IL-8 was 120 pg/mg creatinine (Cr) and 40 pg/mg Cr for TGF-β1. Based on this value, the sensitivity for IL-8 was 58.3%, specificity 100% and for TGF-β1 66.7% and 83.7%, respectively., Conclusions: We found significant differences in children with febrile UTI and ABU regarding urine IL-6, IL-8 and TGF-β1 levels. Urine cytokines and systemic inflammatory markers do not differentiate between upper and lower UTI in infants.

Unlabelled: Improvement in the quality of life in Europe and North America in last decades caused that economical and social aspects of living conditions of the population have less effect and genetic defects of malabsorption of vitamin B12 became the main reason for cobalamin deficiency in children. Imerslund-Grasbeck syndrome (IGS) is characterized by vitamin B12 deficiency that leads usually to megaloblastic anemia and mild proteinuria. We described two pairs of siblings in two families with IGS. The diagnosis in first family (two brothers) was established at 33 and 22 months of age. The reason for diagnostic tests were proteinuria and anemia. Apart from respiratory tract infections, they didn't present other symptoms of cobalamin deficiency. In the second family IGS was diagnosed in children at 5 and 8 years of age. Diagnostic evaluation procedures wereperformedbecause ofneurologicalsigns, including weakness, loss of appetite, dysmorphia, psychomotor retardation. Laboratory tests revealed megaloblastic anemia, low concentration of vitamin B12 in serum and mild proteinuria. In the first pair low concentration of vitamin B12 was validated by the Schilling test, in the second pair methylomalonate acid was detected in the urinary metabolic test. All children were successfully treated with vitamin B12 and anemia and neurological signs disappeared. Long-term follow up showed failure to thrive in the girl and physical and mental retardation, microcephaly in her brother. Proteinuria in the range: 0.3-1.2 g/24 h was detected in each child, and the other laboratory tests were normal. Clinical symptoms, laboratory tests and good reaction to parenteral treatment with vitamin B12 allowed us to diagnose Imerslund-Grasbeck syndrome, even without genetic tests., Conclusion: A delayed diagnosis of congenital malabsorption of cobalamin can lead to physical and mental retardation in children. Children with megaloblastic anemia and proteinuria resistant to classical treatment should be tested for congenital malabsorbtion of cobalamin.

Unlabelled: Biliary sludge and/or biliary pseudolithiasis occur in patients treated with ceftriaxone with prevalence of 3-57%. Biliary obstruction can be the cause of the acute gallbladder enlargement. It is a minor complication, that usually does not give clinical symptoms and resolves once the drug is discontinued. The authors present a case of a 5-month old boy treated for the acute pyelonephritis. Routine ultrasound, performed on the 5th day of treatment with ceftriaxone, showed gallbladder enlargement. In the consecutive studies small gallblader sludge was visible. Patient had no symptoms related to the gallbladder enlargement. Ultrasound performed 6 weeks from the drug discontinuation was completely normal., Conclusions: Patients treated with ceftroiaxone should be monitored for biliary sludge and pseudolithiasis.

Unlabelled: Renal ectopia occurs in 1:3000-1:7000 children. Simple ectopia refers to the abnormal kidney location on the same side; in crossed ectopia the ureter crosses the midline of the body. In most cases ectopic kidney is found in the pelvis minor, in 5% of cases in the thoracic cavity. Kidney displacement does not give any clinical symptoms in the majority of patients, sometimes it may cause abdominal pain, urinary tract infection, chest pain or promote nephrolithiasis. Renal ectopia is usually discovered during routine abdominal ultrasound. We describe a boy with prenatal ultrasound diagnosis of left kidney agenesis. Right normal kidney and displaced to the thoracic cavity, smaller, with normal echo-structure left kidney were found in postnatal ultrasound. Left kidney ectopia was confirmed in renal scinthigraphy, radiography and computed tomography of the chest. Voiding cystourethrography (VCUG) excluded vesicoureteral re€ux., Conclusions: In case of kidney's absence in typical localization in the abdominal ultrasonography, the ectopic kidney should be suspected. Diagnosis of ectopic kidney requires further additional imaging examination to check renal function and other urinary tract anomalies.

Introduction of iterative reconstruction technique in modern computed tomography allowed to reduce the radiation dose and to extend indications for computed tomography urography in children. We describe the case of boy with congenital anomalies in urinary tract including bilateral double collecting system, dilatation of double collecting system in the right kidney and dilatation of lower pelvis in left kidney and bilateral dilatation of ureters. Anomalies were detected in prenatal and then confirmed in postnatal ultrasonography. Voiding cystourethrograhy, which was performed on the 4th day of life revealed grade IV vesicoureteral reflux to the lower part of double collecting system in the right kidney and grade V vesicoureteral reflux to the left kidney. In cystoscopy posterior urethral valves were excluded and presence of double ureters was confirmed. Dynamic scintigraphy (99mTc-EC) detected impaired parenchymal secretory function in the lower pole of left kidney. The patient was qualified for surgical treatment including left lower heminephoureterectomy. Before surgery procedure computed tomography urography was performed and revealed congenital anomalies in urinary tract: bilateral double collecting system, bilateral hydronephrosis of the lower part of kidneys, dilatation of ureters associated with high grade of vesicoureteral refluxes, narrow cortex of left kidney. Computed tomography urography with iterative reconstruction techniques enables precise visualization of urinary tracts with reduced radiation dose. It replaces conventional X-ray urography because of much better visualization of urinary tracts with comparable or even lower radiation dose.

Background: Conventional X-ray urography is one of the basic imaging techniques in urinary tract diseases in children. CT urography (CTU) employing standard Filtered Back Projection (FBP) reconstruction algorithms is connected with higher radiation dose. Advanced iterative reconstruction techniques enable lowering the radiation dose to the level comparable with conventional X-ray urography with better visualization of the urinary tract. Study protocol and indications for this modified technique should be discussed., Material/methods: Introduction of iterative image reconstruction techniques allowed to significantly reduce the radiation dose delivered during examinations performed at our Department, including CT examinations of urinary tract in children. During the last two years, CT urography replaced conventional X-ray urography and became the basic imaging technique in our Department. We discuss the study protocol regarding pediatric CTU examinations. The main goal is to receive an optimal image quality at reduced radiation dose., Results: CTU examinations performed using the standard filtered back projection (FBP) reconstruction technique are associated with radiation doses about 1.5 times higher than those in conventional X-ray urography. Implementation of iterative reconstruction algorithms in advanced CT scanners allow to reduce the radiation dose to a level comparable or even lower than that in X-ray urography. In addition, urinary tract can be evaluated more precisely in multiplanar reformatted (MPR) and volume rendered (VR) images., Conclusions: 1. Advanced iterative reconstruction techniques allow to reduce radiation dose in CT examinations and to extend indications for CT urography in children. 2. Urinary tract can be evaluated more precisely in multiplanar reformatted and volume rendered images. 3. CTU may replace conventional X-ray urography in children.

Key Clinical Message: The most common etiologies of acute scrotum in boys <1 year of age are torsion of the testis or an appendix, urogenital anomalies, and epididymitis. We report an infant with recurrent epididymitis associated with single-system ectopic ureter opening into the seminal vesicle and dysplastic right kidney. Treatment included nephroureterectomy.

This study aimed to determine the frequency of associated urological abnormalities in children with unilateral renal agenesis (RA) or multicystic dysplastic kidney (MCDK). In total, 38 children (10 girls, 28 boys) were studied: 21 with RA and 17 with MCDK. In 14 children (37%) anomalies of the urinary tract were suspected prenatally in ultrasound studies. In the remaining 24 children the diagnosis of RA/MCDK was made postnatally: in 13 (34%) in the first 7 days of life, in 11 (29%) at the age of 8 days to 34 months, mean 10.6+/-8.05 months. Voiding cystourethrography was done in 36 (95%) children, the isotopic 99mTc-EC/DMSA scan of the kidney in 29 (67%), and urography in 8. Urological anomalies were present in 11 (29%) children: in 7 (33%) with RA and in 4 (24%) with MCDK. Vesicoureteral reflux was diagnosed in 8 children: grade II in 4, III in 3, and IV in 1 (in 1 child to duplicated, in 1 to ectopic kidney); ureterovesical junction obstruction in 2 (9.5%); and ureteropelvic junction obstruction in 1 (4.8%). Among them, 2 children demanded surgery on the contralateral urinary tract: pyeloplasty in 1, antireflux procedure in 1; while 9 children were treated conservatively. Compensatory hypertrophy of the contralateral kidney was found in 90% of children. Thus due to an increased risk of pathological changes in the single functioning kidney, lifelong nephrological care is recommended in patients with unilateral RA/MCDK.

Background: The aim of this study was to assess relations between the clinical course of UTI, IL-6 and IL-8 levels, and the presence of inflammatory changes detected by renal scintigraphy using 99mTc-DMSA (DMSA)., Material/methods: We studied 33 children aged 1-24 months (mean 7.1+/-5.8 months) with first-time UTI. The subjects were divided in two groups: with fever (group I, n=10) and without fever (group II, n=23). Inflammatory markers (ESR, CRP, leukocyte count), urinary IL-6 and IL-8 level, and DMSA scan were evaluated in all children., Results: Urinary IL-6 and IL-8 levels [IL-6, IL-8/creatinine (pg/mg)] were significantly higher in group I than in group II (Il-6 level: 39.4+/-41.1 vs. 6.3+/-13.7, p<0.01; IL-8 level: 791.1+/-1143.6 vs. 36+/-87.9, p<0.001). We found positive correlation between urinary IL-6 and IL-8 levels and ESR, CRP, and leukocyte count (IL-6: r=0.43, p<0.05; r=0.46, p<0.05; and r=0.59, p<0.001, respectively; IL-8: r=0.55, p<0.05; r=0.72, p<0.0001; and r=0.44, p<0.05, respectively). We found no relation between urinary cytokine levels and the presence of inflammatory changes detected by DMSA scanning, despite slightly higher mean urinary cytokine levels in children with inflammatory changes in DMSA scan., Conclusions: We found significantly higher IL-6 and IL-8 levels in children with febrile UTI and elevated inflammatory markers. IL-6 and IL-8 levels do not differentiate between acute pyelonephritis and UTI in children to age 24 months.