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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.

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.