Consensus statement on screening, diagnosis, classification and treatment of endemic (Balkan) nephropathy
Nephrol Dial Transplant (2014) 29: 2020–2027
doi: 10.1093/ndt/gft384
Advance Access publication 28 October 2013
Consensus statement on screening, diagnosis, classification
and treatment of endemic (Balkan) nephropathy
Bojan Jelaković1, Jovan Nikolić2, Zoran Radovanović3, Joelle Nortier4, Jean-Pierre Cosyns5, Arthur
P. Grollman6, Nikolina Bašić-Jukić1, Mladen Belicza7, Danica Bukvić8, Semra Čavaljuga9,
Dubravka Čvorišćec10, Ante Cvitković11, Živka Dika1, Plamen Dimitrov12, Ljubica Đukanović3,
Karen Edwards13, Dušan Ferluga14, Ljubica Fuštar-Preradović15, Gheorghe Gluhovschi16, Goran Imamović17,
Tratinčica Jakovina15, Petar Kes1, Ninoslav Leko18, Zvonimir Medverec19, Enisa Mesić17, Marica MiletićVladislav Stefanović25, Karla Tomić15, Senaid Trnačević17, Ivana Vuković Lela1 and Ranka Štern-Padovan26
1
School of Medicine, University of Zagreb, Department for Nephrology, Arterial Hypertension, Dialysis and Transplantation, University
Hospital Center Zagreb, Zagreb, Croatia, 2Clinic of Urology Institute of Urology and Nephrology, Clinical Centre of Serbia, Belgrade, Serbia,
Schools of Medicine, University of Belgrade, Belgrade, Serbia, 4Department of Nephrology, Erasme Hospital Universite Libre Bruxelles,
FULL REVIEW
3
Bruxelles, Belgium, 5Department of Pathology, Cliniques Universitaires St-Luc Université Catholique de Louvain Medical School, Brussels,
Belgium, 6Department of Pharmacological Sciences, State University of New York at Stony Brook, New York, USA, 7Department for Pathology,
Clinical Hospital ‘Sestre Milosrdnice’ University of Zagreb, School of Medicine, Zagreb, Croatia, 8Institute for Endemic Nephropathy, Lazarevac,
Serbia, 9Institute for Epidemiology, University of Sarajevo, Sarajevo, Bosnia and Hercegovina, 10Department of Clinical Laboratory Diagnostics,
University Hospital Center Zagreb, University of Zagreb, School of Medicine, Zagreb, Croatia, 11Institute for Public Health, Brodsko Posavska
County, Slavonski Brod, Croatia, 12Department of Biostatistics and Social Epidemiology, National Center for Public Health Protection, Sofia,
Bulgaria, 13Department of Epidemiology and Institute for Public Health Genetics, School of Public Health and Community Medicine University
of Washington, Seattle, WA, USA, 14School of Medicine, University of Ljubljana, Ljubljana, Slovenia, 15Department for Pathology and Forensic
Medicine, General Hospital ‘Dr.Josip Benčević’ Slavonski Brod, Croatia, 16University of Medicine and Pharmacy, Timisoara, Romania,
17
University Medical Center Tuzla, Tuzla, Bosnia and Herzegovina, 18Department for Nephrology, General Hospital ‘Dr.Josip Benčević’
Slavonski Brod, Slavonski Brod, Croatia, 19Department for Urology, General Hospital ‘Dr. Josip Benčević’ Slavonski Brod, Slavonski Brod,
Croatia, 20Department of Pathology, State University of New York at Stony Brook, New York, USA, 21Institute for Nephrology and
Hemodialysis, Clinical Center, University of Niš, Niš, Serbia, 22Department for Urology, School of Medicine University of Zagreb, University
Hospital Center Zagreb, Zagreb, Croatia, 23Department for Oncology, University Hospital Center Zagreb, University of Zagreb, School of
Medicine, Zagreb, Croatia, 24Department of Nephrology, University ‘Sts Ciril and Methodius’ Faculty of Medicine and Macedonian Academy of
Sciences and Arts, Skopje, Republic of Macedonia, 25Institute of Nephrology, Faculty of Medicine, Niš, Serbia and 26Department for Radiology,
University Hospital Center Zagreb, University of Zagreb, School of Medicine, Zagreb, Croatia
Correspondence and offprint requests to: Bojan Jelaković; E-mail:
A B S T R AC T
combinations of parameters, various cut-off values and many
of them are not in agreement with proposed international
guidelines. Leaders of EN centers began to address these problems at scientific meetings, and this paper is the outgrowth of
Currently used diagnostic criteria in different endemic
(Balkan) nephropathy (EN) centers involve different
© The Author 2013. Published by Oxford University Press
on behalf of ERA-EDTA. All rights reserved.
2020
Medved11, Frederick Miller20, Nikola Pavlović21, Josip Pasini22, Stjepko Pleština23, Momir Polenaković24,
those discussions. The main aim is to provide recommendations for clinical work on current knowledge and expertise.
This document is developed for use by general physicians, nephrologists, urologist, public health experts and epidemiologist, and it is hoped that it will be adopted by responsible
institutions in countries harboring EN. National medical providers should cover costs of screening and diagnostic procedures and treatment of EN patients with or without upper
urothelial cancers.
Keywords: consensus statement, diagnostic criteria, endemic
nephropathy, aristolochic acid nephropathy, upper urothelial
cancer
INTRODUCTION
Position Statement 1: Early detection of EN/UUC is important. Subjects who screen positive for EN should be
subjected to a diagnostic algorithm. Screening for EN is
not justified for children and teenagers, and screening out
of EN villages should be limited to sporadic forms of EN
and family members who moved from the endemic areas.
Ethical considerations should be taken into account in
screening surverys. Identification of new EN foci is not a
priority.
Determination of kidney impairment in EN
The Kidney Disease: Improving Global Outcomes
(KDIGO) guidelines recommend reporting estimated glomerular filtration rate (eGFR) in adults using the 2009 Chronic
Kidney Disease Epidemiology Collaboration (CKD-EPI) creatinine equation [24]. Serum creatinine should be measured
using assays with calibration traceable to the international
standard reference materials and minimally biased compared
Consensus statement on endemic (Balkan) nephropathy
2021
FULL REVIEW
Endemic (Balkan) nephropathy
EN, a chronic tubulointerstitial nephropathy characterized
by an insidious onset and gradual progression to end-stage
renal disease (ESRD), was first described 50 years ago and
remains an important medical, social and economic burden
for all countries harboring this devastating disease [1]. The
number of patients undergoing dialysis remains unchanged
[1–4]. However, a shift to older ages has been recorded among
newly diagnosed cases pointing to lower exposure. High prevalence of UUC is an important characteristic of EN [1, 4, 5].
A variety of environmental agents have been investigated
[1, 6–8]. The most widely studied ochratoxin A was rejected as
Methodolical issues in epidemiology of EN
This paper represents updated recommendations developed
during the ‘International workshop on diagnostic criteria on
Endemic Nephropathy’ held in Brač, Croatia, in 2008. The final
comments were made at a meeting organized in 2012 (Skopje,
Macedonia). The original aims of the workshop were to provide
recommendations for the screening, diagnosing and therapy of
patients with endemic (Balkan) nephropathy (EN) based on
current knowledge. Leading experts were invited to addr (...truncated)
