engleski

Severe hematuria in a child with multiple renovascular anomalies of the left kidney and hemophilia B

Objectives and study: A child with Haemophila B, allergic to F IX medication and with plasma F IX inhibitors occasionally successfuly treated with Novoseven® (aFVIIr) was admitted in hospital with severe hematuria, urine clothing and fall of blood RBC counts.Methods: Clinical studies, abdominal ultrasound (US), US Doppler renal blood vessels imaging, MSCT kidney angiography, renal scintigraphy with Tc-99m-MAG3, Tc-99m-DTPA and Tc-99m-DMSA.Results: Treatment with Novoseven® with enhanced fluid parenteral intake completely reduced hematuria and stabilize RBC blood count. Repeated US kidney and Doppler imaging revealed after a few days unilateral enlargement of left kidney, pyelon dilatation and hyperechogenic inhomogenous content (pyelon clothing). MSCT kidney angiography revealed left kidney 3 independent passable arteries (one with irregular position) and 2 veins (one accessory) with no sign of thrombosis. Dinamic scintigraphy with deconvolution of renographic curves performed with MAG3 and DTPA find severe damaged left kidney. With DTPA we find afunctional renografic curve and with tubular radiopharmaceutical MAG3 renografic curve obstructed. After functional damaged secreted segment retention of activity in parenchyma in tubular cells but not in calyceal system was found. DMSA scintigraphy revealed the total decreased uptake of activity in tubular cells, predominantly in anterior part of the upper pole. Separate renal function with DMSA were left 27%/ and right 73% and are the same in scintigraphy with MAG3. Separate renal function determined with glomerular radiopharmaceutical DTPA were 8, 7% left and 91, 3% for right kidney.Conclusion: Although severe life-threathening hematuria was successfully treated with Novoseven® (aFVIIr), complications associated with congenital multiple vascular anomalies resulted in severe renal damage. With tubular agens we find damage on the parenchymal level, with glomerular agens DTPA even worse, reflecting the predominantly damage of the vascular bed of glomerulus. Possible recovery remains uncertain (future control scintigraphy).

hemophilia B; kidney; renovascular anomalies

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