Introduction: Karyomegalic tubulointerstitial nephritis is a rare entity which can lead to progressive renal failure. The pathogenesis of this disorder is unknown; however, a mitotic block linked to certain MHC genotypes has been proposed. Other potential etiologies include heavy metal or ochratoxin exposure and viral infections. Here, we report a 16-year-old liver allograft recipient who presented with progressive decline of renal function in the third year of the liver transplantation.

Case Report: A 16-year-old female who had liver transplantation at 2003 because of Wilson chirrosis, developed calcium-phosfate imbalance 3 years after her transplantation. Patient had only one episode of acute rejection during past 3 years. During follow-up, because of the elevated creatinine levels and the developing of glycosuria, a kidney biopsy was performed. The biopsy noted many tubular cells with striking nuclear enlargement with bizarre nuclear shapes. In addition, patchy fibrosis with tubular atrophy and a moderate chronic inflammatory infiltrate was observed in the biopsy. These findings were interpreted as karyomegalic nephropaty. EBV, Polyomavirus and CMV viral markers were negative in the biopsy. There was no exposure history to lead, cadmium, mercury or chromium but the patient’s copper level in the liver biopsy found highly elevated (112ug/gr). The patient denied a family history of kidney disease. Electrolyte replacement was applied and routinely controls are suggested.The patient is still at routine control with mildly elevated creatinine level (1,83mg/dl) and without liver allograft faliure.

Conclusions: Karyomegalic interstital nephritis is a rarely seen important condition. The clinicians should be alert for karyomegalic intestitial nephritis especially in patients with liver failure. Because, in the absence of viral infections and the other environmental factors, karyomegalic tubulointerstitial nephritis may be induced by altered copper metabolism.