From the age of 4 he had tracheostomy. Additionally boy received antihypertensive medication, anticoagulant (acenocumarol) for cardiological purposes, he also continued antiepileptic treatment. On admission he was pale, fatigued with marked dyspnoe. On examination artificial right eye bulb (after the rupture of congenitally deformed bulb), dry oral cavity mucosa, dry skin, red throat, postoperative scar in sternal area, additionally were found. No signs of cardiovascular insufficiency were noted. He remained in sitting position

on a wheel chair. Silmitasertib in vitro His intellectual development was slightly delayed. Laboratory test values showed anemia (Hb: 9.6 g/dl), high platelet count (595 G/L), high leukocyte count (15.2 G/L), metabolic acidosis (HCO3 6.3 mmol/L), hyperuricemia (675 μmol/L), hyponatremia (130 mmol/L), hypoproteinemia (50.2 g/L), hypoalbuminemia (21.2 g/L), higher creatinine (84 μmol/L) and urea values (11.7 mmol/L), high CRP concentration (79.3 mg/L), high grade proteinuria (3.6–14.0–27.0 g/L) without the features of nephrotic syndrome. Protrombin time (28.3 s), INR: 2.6 and APTT (46.2 s) were prolonged. The chest X-ray revealed bilateral pneumonia. Echocardiographic examination confirmed artificial mitral valve with maximum gradient 20 mmHg and tricuspid

valve insufficiency with pulmonary hypertension (maximum gradient 60–70 mmHg). Extrarenal cause of AKI was excluded. On ultrasound typically localized kidneys with mean length of 10 cm, blurred image and increased cortex echogenicity were shown. Conservative treatment of AKI was RG7204 nmr administered with no significant improvement. General edema persisted and hyperuricemia worsened. On day 4th rasburicase was applied at the dose 0.1 mg/kg body weight. Daily urine output and values of selected laboratory parameters on consecutive days are shown in Fig. 1. UA concentration significantly dropped ifenprodil during first 24 h after rasburicase administration and reached normal values. On day 12 furosemide and dopamine were withdrawn. Renal replacement therapy was not implemented. Creatinine and

urea values normalized at day 14. Alkalosis persisted from day 5 without supplementation. The increase of serum potassium concentration and the decrease of calcium were noted as shown on Fig. 1e and f. The treatment of pneumonia was finished at day 20 and the boy has been discharged. He remained under the strict nephrological control in out-patient clinic. Mild hyperuricemia was present during the time of further observation and was rather caused by chronic kidney disease than inherited defect of purine metabolism. Kidney function gradually deteriorated to reach the stadium 5 of chronic kidney disease after the 2.5 years from the episode of acute kidney injury. The conservative management of hyperuricaemia involves the use of allopurinol, high-volume hydration, diuretic therapy and urine alkalinization.