Xanthogranulomatous pyelonephritis in childhood: a rare but important clinical entity.
OBJECTIVE
Xanthogranulomatous pyelonephritis (XGPN) is extremely rare in children. The authors review their experience of this condition.


METHODS
Medical records were investigated to conduct a retrospective study of 4 patients with XGPN (3 boys, 1 girl; age range, 2 months to 7 years) at the authors' institute over the past 14 years.


RESULTS
Three of the 4 patients presented with fever of unknown origin and 1 with general fatigue. An abdominal mass was palpable in two cases at initial presentation. Although all patients had pyuria or hematuria, preoperative urine culture was positive in only 2 cases. Preoperative radiologic studies showed that 1 kidney was affected completely in 2 cases and affected partially in 2 cases. Preoperatively, the provisional diagnosis was XGPN in 3 cases, and Wilms' tumor in 1 case. Total nephrectomy was performed in 3 cases and enucleation in 1 case. XGPN was confirmed in all cases by histopathologic studies, but the underlying disease could be identified only in 1 case (cystinuria). All patients did well postoperatively and have had no further health problems over a mean follow-up period of 4.8 years.


CONCLUSIONS
XGPN should be considered when there is a history of recurrent or therapy-resistant pyelonephritis. Preoperative radiologic investigation is paramount for diagnosis, and nephrectomy is the treatment of choice, although partial resection or enucleation are adequate for partially affected kidneys.
Takamizawa
S
S
Department of Pediatric Surgery, Juntendo University School of Medicine, Tokyo, Japan.
Yamataka
A
A
Kaneko
K
K
Yanai
T
T
Yamashiro
Y
Y
Miyano
T
T
D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007668:Kidney / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D011705:Pyelonephritis, Xanthogranulomatous / Q000175:diagnosis* / Q000601:surgery*; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D013520:Urologic Surgical Procedures / Q000379:methods*