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2013 ; 6
(3
): 257-65
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Balkan endemic nephropathy-current status and future perspectives
#MMPMID26064484
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Clin Kidney J
2013[Jun]; 6
(3
): 257-65
PMID26064484
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Balkan endemic nephropathy (BEN), originally described in 1956, is a unique
familial, chronic renal disease encountered with a high-prevalence rate in
Serbia, Bulgaria, Romania, Croatia and Bosnia and Herzegovina. The most prominent
features of the disease are its endemic nature, long-incubation period, familial
clustering of the disease and an unusually high incidence of associated upper
urothelial cancer (UUC). There are no clear-cut data on BEN incidence and
prevalence, since the studies carried out in different endemic areas yielded
contradictory information. In spite of intermittent variations, the incidence of
new cases has remained stable over time. It has been estimated that almost 100
000 people are at risk of BEN, whereas 25 000 have the disease. The clinical
signs and symptoms of BEN are non-specific and often remain unrecognized for
years. There are no pathognomonic diagnostic features of BEN, but the set of
epidemiological, clinical and biochemical data along with the pattern of
pathologic injury in the absence of any other renal diseases are highly
suggestive of this entity. Although the aetiology has been extensively studied,
fostering the publication of various hypotheses, only one of them has provided
conclusive evidence related to the aetiology of BEN. Studies conducted over the
past decade have provided particularly strong arguments that BEN and UUC are
caused by chronic poisoning with aristolochic acids (AAs). In light of these
later studies, one can raise the question whether AAs could be responsible for
previously and currently widespread unrecognized global renal disease and UUC.