The continuing medical mystery of Balkan Endemic Nephropathy

Journal of Rare Diseases
By: , and 



Balkan Endemic Nephropathy (BEN) is a disease of subtle onset and insidious progression that typically occurs between the 4th and 6th decade in long‐resident individuals in highly specific geographic locations of the Balkan region and affects 1 – 5% of the population. Though it does not follow typical Mendelian genetics, there is a familial pattern of occurrence. Although residents may live only a few kilometers apart, certain locations are highly affected while others close by, even as close as across the road, remain unscathed. Because of this geographic selectivity scientists have searched for an environmental cause. It is thought that exposure to the toxic plant Aristolochia clematitis is to blame. Genotoxic N‐heterocyclic or polycyclic aromatic containing coal water leachates entering cultivated soil and drinking water are also a possible cause due to the proximity and predictive power of endemic foci to coal deposits. Evidence for Ochratoxin A fungal poisoning also exists. High levels of phthalates have been measured in BEN‐endemic drinking water. BEN is a probably a multifactorial disease that may result from exposure through some of above‐mentioned environmental sources, with genetic factors contributing. This review will discuss recent research concerning the etiology, potential therapies for the treatment of nephropathy, and unexplored research directions for this chronic kidney disease.

Publication type Article
Publication Subtype Journal Article
Title The continuing medical mystery of Balkan Endemic Nephropathy
Series title Journal of Rare Diseases
Volume 3
Issue 2
Year Published 2015
Language English
Publisher Dowden Pub. Co.
Publisher location Montvale, NJ
Contributing office(s) Eastern Energy Resources Science Center
Description 16 p.
First page 22
Last page 37
Online Only (Y/N) N
Additional Online Files (Y/N) N
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