title: Chronic nephrotic syndrome and chronic renal failure by amyloidosis secondary to xanthogranulomatous pyelonephritis.
abstract: 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 unsuccessful. Fifteen months later, at the age of 15 years, the patient developed terminal renal failure (serum creatinine 14 mg/dl).
authors.affiliation: Ondokuz Mayis University, School of Medicine, Department of Pediatrics, Samsun, Turkey.
authors.forename: I
authors.initials: I
authors.lastname: Işlek
authors.forename: S
authors.initials: S
authors.lastname: Bariş
authors.forename: D
authors.initials: D
authors.lastname: Albayrak
authors.forename: R
authors.initials: R
authors.lastname: Büyükalpelli
authors.forename: R
authors.initials: R
authors.lastname: Sancak
mesh_terms: D000686:Amyloidosis / Q000150:complications / Q000209:etiology*; D002675:Child, Preschool; D002908:Chronic Disease; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology*; D008297:Male; D009404:Nephrotic Syndrome / Q000209:etiology*; D011705:Pyelonephritis, Xanthogranulomatous / Q000150:complications*