Xanthogranulomatous Pyelonephritis in Childhood: Case Report and Review of English and Japanese Literature

Abstract

Xanthogranulomatous pyelonephritis is an uncommon disease that is usually seen in middle-aged women and is much rarer in children. We report a case of diffuse xanthogranulomatous pyelonephritis in a 3-year-old boy who had a nonfunctioning hydronephrotic right kidney on an excretory urogram. A review of the English and Japanese literature on xanthogranulomatous pyelonephritis in childhood revealed 2 remarkable differences regarding the incidence of sex and radiological findings. The majority of patients were girls in the English literature, whereas the opposite sex was seen in the majority of cases in Japan. The involved kidney was functioning on an excretory urogram in the majority of cases reported in the English literature, however, in all cases from Japan the involved kidney was non functioning. These findings further support the newer concept that xanthogranulomatous pyelonephritis in childhood exists in focal as well as diffuse forms.

Xanthogranulomatous Pyelonephritis: Clinical Experience with 62 Cases

“…Patients are typically in the fifth or sixth decade and the occurrence of pediatric cases, with peculiar aspects of the disease, is quite exceptional [29,38], Females are more frequently involved than males because of the higher incidence of urinary tract infections, and a 2:1 ratio has been reported [2], Renal cell carcinoma has occasionally been found to be associated with XGP [17,21,27].…”

“…Xanthogranulomatous tissue could arise from the im paired drainage of suppurative foci or from the extension of the inflammatory process to adipose tissue of the vas cular adventitia [7], It is currently believed that XGP originates as a local ized reaction, and subsequently extends to the entire kid ney [34], Pediatric cases, particularly those occurring in females, can remain limited [38] and, in the pediatric age group, the diffuse form is less frequently associated with stones, as opposed to adults [29],…”

Diffuse xanthogranulomatous pyelonephritis in a child with severe complications

“…About 200 pediatric cases have been reported since then in the English literature. The disease is usually unilateral [10,11], with only a few cases of bilateral involvement reported [1,11,12]. XGP is classified as focal or diffuse, depending upon the extent of parenchymal involvement.…”

“…However, cases associated with congenital problems such as ureteropelvic junction obstruction and vesicoureteral reflux have been reported [1,4]. Urinary tract infections with gram-negative bacteria, particularly Proteus species, followed by Escherichia coli, are most commonly associated with XGP [10,11,14,15,16]. Positive urine and/or exudate cultures have been reported in 70% of cases [14].…”

Xanthogranulomatous Pyelonephritis in Childhood

“…2 Up until 1982, only single case reports were described in children and, in all incidences, these were girls. [3][4][5][6][7][8][9][10][11][12] Since then, larger series of several cases have been reported. 13 14 Nephrectomy has been curative in all cases, with no long term sequelae.…”