CHRONIC NEPHROTIC SYNDROME AND CHRONIC-RENAL-FAILURE BY AMYLOIDOSIS SECONDARY TO XANTHOGRANULOMATOUS PYELONEPHRITIS

The association of xanthogranulomatous pyelonephritis (SPN) and system ic amyloidosis is extremely rare. This association has been described in only six cases. We present a 4-year-old male admitted with a histor y of anuria and abdominal pain. Investigations revealed multiple calcu li in both kidneys. A right pyelolithotomy and left nephrectomy were p erformed. Histological examination demonstrated XPN and amyloidosis. A t discharge serum creatinine had dropped to 1.1 mg/dl but after being lost to follow up for 9 years, the child was readmitted because of ede ma, Laboratory examination revealed a nephrotic syndrome and serum cre atinine of 2.3 mg/dl. Rectal biopsy showed the presence of amyloid. A treatment bq colchicine was unsuccesful, Fifteen months later, at the age of 15 years, the patient developed terminal renal failure (serum c reatinine 14 mg/dl).