XANTHOGRANULOMATOUS PYELONEPHRITIS IN A CHILD: A RARE ENTITY

Abstract

Xanthogranulomatous Pyelonephritis is chronic pyelonephritis uncommonly described in adults and rarely in children. It is characterised by replacement of renal parenchymal tissue by lipomatous cells making it non-functional and may be mistaken for pyonephrosis, cystic or solid tumor, and most commonly wilms tumour. It often presents as abdominal mass in children and more commonly involves left kidney. Imaging by CT scan is characteristic and histology is diagnostic. We are reporting a case in child who presented with prolonged febrile illness and documented urinary tract infection with nephrolithiasis without any abdominal lump and received antibiotics for multiple times without any improvement. USG was suggestive of pyonephrosis and multiple abscesses. However, CT scan done subsequently diagnosed as xanthogranulomatous pyelonephritis of right kidney which was confirmed on histology. After documenting no function in affected kidney with other being normal, unilateral nephrectomy was done resulting in rapid symptomatic improvement. Results for genetic analysis for suspected hereditary tubular disorders are pending.