Antibiotic Prophylaxis in Infants with Grade III, IV, or V Vesicoureteral Reflux.

BACKGROUND: 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.).

Heterozygous loss of WBP11 function causes multiple congenital defects in humans and mice.

The genetic causes of multiple congenital anomalies are incompletely understood. Here, we report novel heterozygous predicted loss-of-function (LoF) and predicted damaging missense variants in the WW domain binding protein 11 (WBP11) gene in seven unrelated families with a variety of overlapping congenital malformations, including cardiac, vertebral, tracheo-esophageal, renal and limb defects. WBP11 encodes a component of the spliceosome with the ability to activate pre-messenger RNA splicing. We generated a Wbp11 null allele in mouse using CRISPR-Cas9 targeting. Wbp11 homozygous null embryos die prior to E8.5, indicating that Wbp11 is essential for development. Fewer Wbp11 heterozygous null mice are found than expected due to embryonic and postnatal death. Importantly, Wbp11 heterozygous null mice are small and exhibit defects in axial skeleton, kidneys and esophagus, similar to the affected individuals, supporting the role of WBP11 haploinsufficiency in the development of congenital malformations in humans. LoF WBP11 variants should be considered as a possible cause of VACTERL association as well as isolated Klippel-Feil syndrome, renal agenesis or esophageal atresia.

Serum and urine periostin and cytokeratin-18 in children with congenital obstructive nephropathy.

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%.

Urinary vanin-1 for predicting acute pyelonephritis in young children with urinary tract infection: a pilot study.

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.

Serum neutrophil gelatinase-associated lipocalin for predicting anemia of inflammation in children with urinary tract infection.

Anemia of inflammation (IA), the second most common cause of childhood anemia, results from macrophage iron sequestration and impaired erythropoiesis. Neutrophil gelatinase-associated lipocalin (NGAL) plays an important role in innate microbial immunity through its influence on intracellular iron homeostasis and inhibition of erythropoiesis. The predictive value of NGAL in IA was assessed in 74 children (age 6.30 ±3.64 months) with the first episode of urinary tract infection (UTI). Anemia of inflammation was found in 50% of children, including those with non-febrile UTI, and delayed onset of anemia was observed in 20% of children. There were no differences in NGAL levels between the anemic and non-anemic children, and no correlations between NGAL and hemoglobin (HGB) levels and red blood cell (RBC) count. In multivariate logistic regression analysis elevated C-reactive protein (CRP) was only independently associated with the presence of anemia in children with UTI [OR (95% CI): 1.128 (1.005-1.265), p = 0.040]. In stepwise multiple analysis age independently correlated with RBC (ß = 0.051, p = 0.001), while CRP independently correlated with HGB (ß = -0.037, p = 0.027) and RBC (ß = -0.022, p = 0.014). ROC analysis demonstrated better diagnostic profiles for CRP, procalcitonin (PCT) and fever duration for predicting the risk of IA than NGAL (AUC: 0.690, 0.669, 0.678 vs. 0.638, respectively). Despite the increase in HGB levels after 4-5 weeks from the onset of UTI, HGB values were still significantly lower in the anemic than in non-anemic children. NGAL was not useful for predicting IA in UTI, since its diagnostic value was inferior to conventional inflammatory markers.

Clinical profile of neonates with hypernatremic dehydration in a nephrology clinic.

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.

Massive thrombosis in an infant with suspected nephrocalcinosis: case report and literature review.

INTRODUCTION: Perinatal period is characterized by an increased risk of thrombosis due to low resources and limited compensatory capacity of the coagulation system in early stages of life. CASE REPORT: We report a case of a second pregnancy female infant born at 39 weeks by caesarean section, due to pre-labor rupture of membranes, with body weight of 3,570 γ and Apgar score 10. The pregnancy was complicated by hypothyroidism, uterine myoma, urinary tract infections, and mother's appendectomy at 16 Hbd. At 3 months, the girl was admitted to our hospital due to kidney calcifications, which were incidentally found during ultrasound scan. In laboratory workup, no abnormalities in calcium and phosphate homeostasis were detected. However, in ultrasound scan, linear calcifications along pyramids were visualized in both kidneys. Due to atypical location of nephrocalcinosis, Doppler scan was performed, showing lack of visible blood flow from renal veins to inferior vena cava (IVC), with compensatory flow from renal veins to paravertebral plexuses, and IVC obliteration with a massive calcification in the hepatic section. Magnetic resonance confirmed obliteration of IVC and common iliac veins, segmental dilatation of IVC, and compensatory blood flow from kidneys and lower limbs to paravertebral plexuses. Clinical picture and formation of collateral circulation suggested intrauterine thrombosis. Congenital thrombophilia was excluded in laboratory examination. CONCLUSIONS: The differential diagnosis of calcifications in renal parenchyma (nephrocalcinosis) should include renal vein thrombosis. Massive fetal and perinatal thrombosis can be asymptomatic due to high ability to form collateral circulation at the early stage of life.

Vitamin D and Calcium Homeostasis in Infants with Urolithiasis.

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 D3. 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.

Serum neutrophil gelatinase-associated lipocalin for predicting acute pyelonephritis in infants with urinary tract infection.

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.

Diagnostic accuracy of urine neutrophil gelatinase-associated lipocalin and urine kidney injury molecule-1 as predictors of acute pyelonephritis in young children with febrile urinary tract infection.

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.

Prognostic value of serum and urine kidney injury molecule-1 in infants with urinary tract infection.

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.

Neutrophil Gelatinase-Associated Lipocalin: A Biomarker for Early Diagnosis of Urinary Tract Infections in Infants.

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.

[High ureteral insertion as the cause of extreme hydronephrosis in a 5-year-old child with horseshoe kidney].

The incidence of horseshoe kidney is 1 per 400-800 live births. From 44-52% of the patients with horseshoe kidney have other coexisting abnormalities of the urinary tract, such as hydronephrosis, vesicoureteral reflux and a duplex collecting system. Our patient, a 5-year old boy, was admitted to a pediatric nephrology department because of abdominal pain and vomiting. He had ultrasonography of the abdomen performed for the first time at the age of 9-months and horseshoe kidney was shown. In a control ultrasonography, a mild dilatation of the pyelocalyceal system in the left kidney was described. On the day of admission, an abdominal ultrasound confirmed horseshoe kidney with large left hydronephrosis. Power Doppler ultrasonography showed two renal arteries to the left kidney and no arterial compression on the ureter. Dynamic scintigraphy (99mTc-EC) revealed left-sided hydronephrosis with high isotope intake up to 55% ERPF, a prolonged time of tissue perfusion and signs of subpelvic junction obstruction. Magnetic resonance urography presented an enlarged left kidney, with a diameter of up to 105 mm, a narrow renal cortex, a dilated renal pelvis up to 39 mm in diameter, dilated calyces up to 26-32 mm, and the high insertion of the ureter from the pelvis. The right kidney was normal. To facilitate drainage from the dilated collecting system of the left kidney, a double-J catheter was inserted. Pyeloplasty is planned as the next step of treatment. Conclusion: In a child with horseshoe kidney and a mild dilatation of the collecting system detected in infancy, long-term follow up is necessary, because of the increased risk of significant hydronephrosis in the future.

[Congenital megacalycosis in a girl with unilateral renal agenesis]. / Wrodzone kielichonercze u dziewczynki z jednostronna agenezja nerki.

Renal agenesis occurs in pediatric population with the incidence 1:500- 2000 children. It is more often diagnosed in boys and on the left side of the body. Renal agenesis may be isolated or it may be a part of complex malformation syndrome. Megacalycosis is a very rare anomaly of urinary tract associated with abnormal structure of the kidney pyramids. AIM: The aim of the study was to present for the first time in the medical literature the case of a girl with unilateral renal agenesis and megacalycosis. CASE REPORT: A girl, born at term in good general medical condition, and with normal birth weight was admitted to the hospital because of urinary tract infection caused by E.coli. Antenatal abdominal ultrasounds were normal. In a diagnostic, repeated ultrasound studies, unilateral, left renal agenesis and the righ-sided megacalycosis were found. The right kidney had dilated collecting system, with normal size of renal pelvis and enlarged calyces up to 26 mm. The kidney function was normal. Voiding cystourethrography excluded vesicoureteral reflux. Dynamic scintigraphy 99mTc-EC showed the lack of function of the left kidney, postinflammatory changes and dilation of collecting system without signs of obstruction. During two-years follow up we didn't observe clinical relapse of urinary tract infection. Blood pressure and kidney function were normal. CONCLUSIONS: Complex congenital anomalies of the kidney and the urinary tract (CAKUT) can be diagnosed at any age. Normal antenatal abdominal ultrasound does not exclude CAKUT. Every patient with congenital abnormalities of the kidney and the urinary tract requires long-term follow up, because of increased risk of chronic kidney disease.

[Dodatkowa tetnica nerkowa przyczyna skrajnego wodonercza u 5-letniego chlopca]. / Accessory renal artery as a cause of extreme hydronephrosis in 5-year old boy.

Hydronephrosis in children is most often due to an intrinsic ureteropelvic junction obstruction or by compression on ureter by accessory renal artery coming from the aorta to the lower pole of the kidney. AIM: The aim of study was to present a case with a late onset of hydronephrosis caused by accessory renal artery. CASE REPORT: 5-year old boy with a mild pyelectasia during first 10 months of age was admitted to hospital because of abdominal pain and vomiting. Abdominal ultrasound revealed a marked dilatation of the right pelvicalyceal system with renal pelvis measuring 23 mm in anterior-posterior (ap) diameter, enlargement of calyces to 10 mm and narrowed cortex to 5 mm. Dynamic scintigraphy (99mTc-EC) showed right-sided hydronephrosis with decreased isotope intake up to 31%, prolonged time of tissue perfusion and signs of ureteropelvic junction obstruction. Computed tomography urography with vascular option revealed right kidney length of 116 mm with narrow cortex, and dilated renal pelvis up to 53x52x28 (ap) mm and dilated calyces up to 16 mm. Apart from dilated collecting system, the computed tomography showed two renal arteries: normal artery coming from the aorta at the L1 level and the accessory renal artery, which originated from the aorta to the lower pole of the kidney at the L2/L3 level. The accessory renal artery compressed on the ureter causing hydronephrosis. The pyeloplasty modo Hynes-Anderson was performed. After 3 months an abdominal ultrasound revealed the right kidney of 89 mm in length with only moderate hydronephrosis: dilatation of renal pelvis up to 15-18 mm and calyces up to 7-8 mm. Scintigraphy showed isotope intake 48%. CONCLUSIONS: Hydronephrosis caused by accessory renal artery can be asymptomatic, with mild dilatation of pyelocalyceal system seen on abdominal ultrasonography. The first clinical symptoms may occur after several years and be associated with large hydronephrosis.

[Sterile leukocyturia - difficult diagnostic problem in children].

Sterile leukocyturia is an important and difficult clinical problem in children. In this paper, we described the most common nephrologic causes of sterile leukocyturia, including infectious, non-infectious and extrarenal etiology. We stressed an the importance of appropriate urine collection for urinalysis. There is a need for treatment of inflammation and also for diagnosis of potential anomalies of urethral orifice as causes of sterile leukocyturia in children.

Long-term follow up of a boy with unilateral autosomal dominant polycystic kidney disease and contralateral renal agenesis.

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.

[Renal staghorn calculi in small children - presentation of two cases]. / Kamica odlewowa nerki u malych dzieci-opis dwóch przypadków.

Urolithiasis in children occurs with the incidence of 0.1-5%. Risk factors such as metabolic disorders, recurrent urinary tract infections and/or congenital abnormalities of urinary tract are detected in 75-85% of children with urolithiasis. Staghorn calculi is associated with delayed diagnosis and treatment of urinary tract infection caused by specific organisms, which produce the enzyme urease, promoting generation of ammonia and hydroxide from urea. We present two boys with staghorn calculi recognized in 8th and 31st month of age. The reason for performing ultrasonography was urinary tract infection in both boys. The younger child was previously healthy, with no symptoms of urolithiasis, the older one had recurrent urinary tract infections caused by Proteus mirabilis, episodes of anxiety and abdominal pain. Laboratory test and imaging studies excluded congenital abnormalities in the urinary tract and typical metabolic causes of urolithiasis in both boys. Treatment of infection-related stones in the younger child included two extracorporeal shock-wave lithotripsy (ESWL). In the older child, both ESWL and operation were performed. Staghorn calculi were composed of mixtures of magnesium ammonium phosphate (struvite) and calcium carbonate (apatite) and confirmed to be identified as infection-related stones. During follow-up in a nephrology outpatient clinic, values of blood pressure, renal ultrasonography, kidney function test were normal and no symptoms of urinary tract infections were clinically present. In patients with recurrent urinary tract infections, urolithiasis should be taken into consideration. The majority of staghorn calculi is often asymptomatic and can be diagnosed with an ultrasonography study performed routine or during urinary tract infection.

Polydactyly and obesity - the clinical manifestation of ciliopathy: a boy with Bardet-Biedl syndrome.

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.

Extreme hydronephrosis due to uretropelvic junction obstruction in infant (case report).

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.