Amyloidosis secondary to xanthogranulomatous pyelonephritis: an unusual case

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Amyloidosis secondary to xanthogranulomatous pyelonephritis: an unusual case Key words: Amyloidosis - Xanthogranulomatous pyelonephritis - Nephrotic syndrome

Sirs, We read with great interest the clinical quiz of Flynn and Gill [1] in an earlier issue of the journal about a 3-year-old boy with xanthogranulomatous pyelonephritis (XPN) and amyloidosis. The association of XPN and systemic amyloidosis is extremely rare. To our knowledge, despite innumerable cases of XPN reported in the literature, this association has been described on only five occasions and two were children [1-4]. We present a child with XPN associated with secondary systemic amyloidosis and the nephrotic syndrome. A 6-year-old boy was admitted to our hospital with oedema, left upper quadrant pain and oliguria. He had had these symptoms for the past year when a kidney stone was detected. Physical examination revealed failure to thrive, hypertension, periorbital oedema, ascites, hepatosplenomegaly, a firm left upper quadrant mass and severe oedema of both lower extremities. On admission laboratory investigation revealed: haematocrit 38%, leucocyte count 19,700/ram 3, erythrocyte sedimentation rate 95 mm/h, blood urea nitrogen 19 mg/dl, serum creatinine 0.8 mg/dl, total serum protein 3.9 g/dl, serum albumin 1.5 g/dl, total serum lipids 465 mg/dl, serum cholesterol 176 mg/dl, serum C3 115 mg/dl, serum C4 38 mg/dl and fibrinogen 290 mg/dl. Urinalysis revealed microscopic haematuria, pyuria and proteinuria. The 24-h urinary protein excretion was 324 mg/m 2 per hour. Urine cultures for bacteria (including tuberculosis) were negative. No urinary acid-fast bacilli were detected. Urine cultures for fungi yielded Candida albicans. Intravenous urography revealed an enlarged and non-functioning left kidney with multiple stones in the left kidney and ureter. Ultrasound examination confrmed the enlargement of the left kidney (130• mm) and also revealed hepatosplenomegaly, ascites and an enlarged right kidney (119x54 mm). The right kidney parenchymal thickness was 2 0 - 2 5 mm. Computerised axial tomography confirmed the ultrasonographic findings. Left nephrostomy was performed, pyonephrosis drained and antibiotic treatment given. Pus cultures yielded more than 100,000 colonies/mm 3 of Acinetobacter baumani. Anterograde urography revealed a dilated left pelviureteric system.

No improvement in the function of the left kidney was observed a month later and the patient underwent left flank exploration and left-sided nephrectomy. Grossly, the kidney was severely inflamed and enlarged (12.5x7• cm) and showed moderate hydronephrosis. The capsule was thickened and adherent. The pelvis contained large amounts of green-yellow pus. The ureteropelvic junction was obstructed by a large calculus and also there were calculi in the kidney. The pelvic surface was eroded and covered by exudate. The renal parenchyma appeared thin and displayed numerous yellowish nodules and abscesses. Histologically, the prominent feature was a xanthogranulamatous reaction present in nodules or in bands outlining the calyces and the necrotic papillae. Foamy macrophages and multinuclear giant cells were the major component of this reaction. Congo red-positive birefringent material was seen in the glomerular tuft and along the peripheral capillary loops. Permanganate digestion studies suggested the diagnosis of an AA-type amyloid in the renal biopsy. Colchicine was started at a dose of 0.5 mg twice a day and after a month his clinical oedema resolved and 24-h urinary protein excretion decreased (53 mg/m 2 per hour). The long-term prognosis is difficult to forecast. However, since it is also difficult to estimate the interval between XPN and generalised amyloidosis we would like to emphasise the importance of early diagnosis and treatment of XPN. Aytiil Noyanl, Naci Dumanl, Giilfiliz G6nlii~en2, Ali Anarat 1, and Ilhan Tuncer 2

Departments of 1 Paediatric Nephrology and 2 Pathology School of Medicine, ~ukurova University, TR-01120 Adana, Turkey

References

1. Flynn MT, Gill DG (1992) Clinical quiz. Pediatr Nephrol 6:597-598 2. Querfeld U, Waldherr R, Twittenhoff W, M6hring K, Scharer K (1986) Generalized amyloidosis secondary to xanthogranulomatous pyelonephritis. Eur J Pediatr 145:565-568 3. Garber BB, Cendron M, Cohen R, Whitmore KE (1989) Xanthogranulomatous pyelonephritis and amyloidosis: a rare association. J Urol 142:114-116 4. Lauzurica R, Felip A, Serra A, Saladie JM, Montserrat E, Encabo B, Caralps A (1991) Xanthogranulomatous pyelonephritis and systemic amyloidosis: report of 2 new cases and the natural history of this association. J Urol 146: 1603-1606