Clinical Nephrology, cilt.49, sa.1, ss.62-65, 1998 (SCI-Expanded)
The association of xanthogranulomatous pyelonephritis (XPN) and systemic amyloidosis is extremely rare. This association has been described in only six cases. We present a 4-year-old male admitted with a history of anuria and abdominal pain. Investigations revealed multiple calculi in both kidneys. A right pyelolithotomy and left nephrectomy were performed. Histological examination demonstrated XPN and amyloidosis. At 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 edema. Laboratory examination revealed a nephrotic syndrome and serum creatinine of 2.3 mg/dl. Rectal biopsy showed the presence of amyloid. A treatment by colchicine was unsuccesful. Fifteen months later, at the age of 15 years, the patient developed terminal renal failure (serum creatinine 14 mg/dl).