Xanthogranulomatous Pyelonephritis in Childhood

Abstract

Background-Xanthogranulomatous pyelonephritis is a severe, atypical form of chronic renal parenchymal infection accounting for 6/1000 surgically proved cases of chronic pyelonephritis. Its manifestations mimic those of neoplastic and other inflammatory renal parenchymal diseases and, consequently, it is often misdiagnosed preoperatively. Conclusions-Increasing awareness of this condition should lead to the diagnosis being suspected preoperatively. (Arch Dis Child 1999;81:483-486)

Generalized amyloidosis secondary to xanthogranulomatous pyelonephritis

“…Up to 1980, 42 cases of XPN in children had been published [10]. More recently, additional series of 567 children with this condition have been reported [4,11]. The typical clinical picture of XPN in childhood is characterized by recurrent episodes of symptomatic pyelonephritis accompanied by a nonfunctioning kidney shown by X-ray examination.…”

“…The radiological picture is rather characteristic [1]. In almost all cases the disease was unilateral [3,4,7,11]. The most important differential diagnosis is Wilms tumour [11].…”

“…The existence of Miillerian duct derivatives in otherwise normal males constitutes a rare but well recognized disorder Offprint requests to: J. P. Fryns of sexual development called internal male pseudo-hermaphroditism [10,11,15] or persistent M/illerian duct syndrome [4,16].…”

Serendipitous diagnosis of childhood xanthogranulomatous pyelonephritis in a child with osteomyelitis

“…An unusual feature of our patient was the occult nature of the renal disease, masked by the concomitant osteomyelitis until discovered serendipitously on a bone scan. There is at least one other report of a case of childhood XGP that was initially diagnosed as osteomyelitis [4].…”

“…There have been increasing numbers of reports of a rare childhood form that differs in many respects from its adult counterpart [2][3][4]. In both types, there is no single test by which one can establish a definitive preoperative diagnosis [5].…”