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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.

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.

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.

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.

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.

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.

Introduction: The basis of the diagnosis of urinary tract infection in children is positive culture of properly collected urine sample. The reliability of the urine cultures depends on the method how the urine sample was taken and sometimes this may increase the risk of misdiagnosis., Aim of the Study: To determine the frequency of false positive urine cultures taken from midstream to a container or to a plastic collection bag in children under 2 years of age., Material and Methods: The study included 50 children (25 girls, 25 boys) aged 12 days to 24 months (mean age 7.26±6.51months) referred to the hospital with suspicion of urinary tract infection. The most frequent indications for urine analysis were: history of infection and/or abnormalities of urinary tract in 28 (56%) children, failure to thrive in 8 (16%) and fever in 6 (12%). Urine was taken from midstream to a container in 32 (64%) children and collected to a plastic bag in 18 (36%) children., Results: Hospital verifications of urine cultures were performed by suprapubic puncture culture in 24 (48% children) or by catheterization of the urinary bladder in 26 (52%) children. Urinary tract infection was confirmed in 11 (34%) among 32 children who had positive culture of urine form midstream. None of the children with positive urine culture from a plastic collection bag had urinary tract infection confirmed by suprapubic puncture or catheterization., Conclusions: Correct method of urine collection for bacteriological tests in children under two years of life can avoid the misdiagnosis of urinary tract infection and following unnecessary hospitalization, imaging procedures as well as potentially harmful treatment.