Kidney transplantation in the patients with endemic nephropathy

Endemska nefropatija je sporo progresivna, kronična tubulointersticijska bolest bubrega. Bolest se pojavljuje sa visokom prevalencijom kod stanovnika endemskih ruralnih područja u Hrvatskoj, Srbiji, Bosni i Hercegovini, Bugarskoj. Kronična izloženost aristolohičnoj kiselini identificirana je kao glavni čimbenik rizika za EN te ima i ulogu u razvoju karcinoma urotrakta. Bolest nema specifičnih kliničkih simptoma te zbog toga ostaje neprepoznata dugi niz godina te u konačnici uzrokuje terminalni stadij bubrežne bolesti. Cilj ovog istraživanja bio je prikazati pacijente kojima su zbog EN transplantirani bubrezi. Retrospektivno smo analizirali bolesnike s endemskom nefropatijom u kojih je učinjena transplantacija bubrega u Kliničkom bolničkom centru Rijeka. Tijekom 12 godina na Klinici za urologiju KBC Rijeka transplantirana su 4 pacijenta s endemskom nefropatijom. Jedna pacijentica prezentirala se akutnom tubularnom nekrozom u ranom postoperativnom razdoblju sa sumnjom na odbacivanje grafta. U druge pacijentice došlo je do razvoja karcinoma mokraćnoga mjehura te je višestruko operirana. U niti jednog pacijenta nije došlo do značajnijeg pogoršanja bubrežne funkcije transplantata. Do sada su umrla 3 bolesnika od čega jedna bolesnica zbog metastatskog panurotelijalnog karcinoma. Najbolja terapijska metoda liječenja bolesnika s EN u terminalnom stadiju kronične bubrežne insuficijencije je transplantacija bubrega. Zbog povećane incidencije pojave karcinoma gornjeg dijela urotrakta preporuča se učiniti preventivnu bilateralnu nefroureterektomiju kao pripremu za transplantaciju bubrega.Endemic nephropathy is a slowly progressive, chronic tubulointerstitial renal disease. The occurrence has been recorded with high prevalence in residents of endemic rural regions in Croatia, Serbia, Bosnia and Herzegovina, Bulgaria. A chronic exposure to aristolochic acid has been identified as a major risk factor for EN and has a role in the development of urotract cancer. The clinical symptoms of EN are not specific and remain unrecognized for several years and disease finally can cause terminal stage of renal disease. The aim of this study was to present patients with transplanted kidneys who had an EN. The study was set up as a retrospective study of patients with endemic nephropathy in which kidney transplantation was performed at Clinical Hospital Center Rijeka. During the 12 years at the Clinic for Urology of KBC Rijeka, 4 patients with endemic nephropathy were transplanted. One patient presented with acute tubular necrosis with suspected graft rejection in the early postoperative period. The other patient develops urinary bladder cancer, which is why the multiple operations was done. There was no significant increase in renal function of the transplant in any patient. Up to now, 3 patients died of which one patient is due to metastatic panurothelial carcinoma. The best therapeutic treatment method of EN in the terminal stage of chronic renal insufficiency is kidney transplantation. The increased incidence of upper urothelial cancer in patients with EN has been reported and because of that a preventive bilateral nephroureterectomy is recommended