ENDEMIC (BALKAN) NEPHROPATHY - INTERNATIONAL REGISTRY PRELIMINARY DATA FROM THE ERA EDTA REGISTRY AND TWO ENDEMIC FOCUSES

Autor: Vukovic Lela, Ivana, Noordzij, Marlies, Jager, Kitty, Bašić-Jukić, Nikolina, Rački, Sanjin, Leko, Ninoslav, Teskera, Tomislav, Stipančić, Želimir, Mikolašević, Ivana, Jelaković, Bojan
Jazyk: angličtina
Rok vydání: 2015
Předmět:
Popis: INTRODUCTION AND AIMS: Endemic (Balkan) nephropathy (EN) is a chronic tubulointerstital nephropathy associated with urothelial cancer caused by aristolochic acid and is an enviromental form of worldwide distributed arictolochic acid nephropathy. Disease is present in the rural areas alongside major rivers in Bosnia, Bulgaria, Croatia, Romania and Serbia. Prevalence of EN is from 0.4 - 12.3% with recent reports indicating a decrease in frequency of disease. In the recent decades technological improvement in agricultural practices significantly diminished the exposure to the environmental toxin. Therefore, it is expected that the proportion of EN patients on renal replacement therapy(RRT) will be decreasing. METHODS: For the first time, a uniform diagnostic criteria for EN were accepted, allowing the critical revision of EN patients in different countries with EN. Next critical step in investigating the changes in frequency and disease characteristics is a formation of international registry of EN patients. In that respect, a project Endemic (Balkan) Nephropathy in the ERA-EDTA Registry (EN-REG) was started. Of EN countries only Bulgaria was not included in the recent ERA EDTA registry reports. The aim of the project is to aquire already submitted data to the ERA EDTA registry along with specific data regarding EN including positive family history, longtime residence in endemic focus, history of urothelial malignancy and presence of proximal tubule damage in EN patients on renal replacement therapy from all EN countries. RESULTS: In the countries submitting individual data in the ERA EDTA registry, 632 patients with EN (322 Bosnia and Hercegovina, 237 Serbia, 88 Romania) were registered in the period form 1995-2011. Remaining patients were registered in Austria (N=14), Belgium (N=6) and France (N=9) emphasising the need for revision and analysis of submitted data to ascertain whether these are immigrants form endemic regions and to further improve accurate identification of patients with EN. To test the feasibility of the project, in the pilot study conducted in Croatia and Bosnia we collected data of EN patients that are currently on RRT or hav started in the period from 1995-2012. From 1995 a permanent increase of prevalence of EN patients on RRT was noted in Croatia (by 5.4% every year). Number of prevalent EN patients as well as prevalnce of EN patients on RRT is decreasing in Bosnia while the incidence is significantly rising by 5.5% every year. No significant difference in age at the start of RRT between two centres was found with a shift towards the older ages. Mortality was higher in Odzak (78.8% vs. 57.1 %) with no difference in the age of death (71.5 i 72.5 ; p 0.605). CONCLUSIONS: This project, based on the data aquired from the ERA EDTA registry for the period 1995-2012 and data from two dialysis centres is the first step in formation of a global EN registry followed in the future by formation of an aristolochic acid nephropathy registry while EN is the enviromental form of this toxic nephropathy.
Databáze: OpenAIRE