Xantogranulomatous Pyelonephritis in children

Xanthogranulomatous Pyelonephritis (XP) is a renal chronic inflammatory disease infrequent during childhood produced by the association of long-term urinary tract obstruction with chronic infection. We report the cases of two children diagnosed of XP.

One of them consulted for cloudy urine. Hypergammaglobulinemia with the presence of a homogeneous band in the gamma fraction of the proteinogram stood out among laboratory findings. The other case was diagnosed while performing a urologic ultrasound in the follow up of kidney stone disease. In the latter, the renal inflammation was adhered to the perirenal capsule, colonic splenic flexure and descending colon. Both presented mild proteinuria and hematuria. None of them showed clinical sings of affectation of the general status and their renal function laboratory data as well as the infection markers were normal.

All the alterations disappeared after performing a nephrectomy of the affected kidney.

We present two cases of XP in a group of age rarely affected by this disease that also showed an uncommon presentation for their age. A high rate of suspicion must be held to diagnose and correctly treat this disease because the grade of renal and extra renal damage is closely related with the extension and duration of the disease.