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Balkan endemic nephropathy-current status and future perspectives.

Pavlović NM - Clin Kidney J (2013)

Bottom Line: The clinical signs and symptoms of BEN are non-specific and often remain unrecognized for years.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).

View Article: PubMed Central - PubMed

Affiliation: Clinic of Nephrology, Clinical Centre , Nis , Serbia.

ABSTRACT
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.

No MeSH data available.


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Geographical distribution of endemic foci and pliocene lignite coal deposits.
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SFT049F1: Geographical distribution of endemic foci and pliocene lignite coal deposits.

Mentions: Balkan endemic nephropathy (BEN) is a familial, slowly progressive, chronic renal disease with insidious onset in the fifth decade of life and terminal renal failure in the sixth or seventh decade. The occurrence of BEN has been recorded with a high prevalence rate in Serbia, Bulgaria, Romania, Bosnia and Herzegovina and Croatia (Figure 1). The first cases were described in 1956 in Bulgaria, where Tanchev et al. [1] published the first detailed clinical description of the new entity. A year later, a renal disease with almost identical clinical and epidemiological characteristics was reported in Yugoslavia (Serbia) [2]. In 1961, it was found that a similar nephropathy was also prevalent in Romania [3]. The most prominent features of the disease are its endemic nature, the long latent period before development of the disease, familial clustering of the disease and the remarkably high incidence of upper urothelial cancer (UUC) associated with BEN or occurring in the population at risk [4–6]. No cases have been documented in children or adolescents. The aetiology of BEN has been the subject of many published studies resulting in the generation of several hypotheses. Although data published on these various hypotheses have presented some indication of their relevance to the aetiology of BEN, only one of them, dealing with chronic aristolochic acid (AA) poisoning, has provided conclusive evidence related to the aetiology of BEN and its clinical characteristics.Fig. 1.


Balkan endemic nephropathy-current status and future perspectives.

Pavlović NM - Clin Kidney J (2013)

Geographical distribution of endemic foci and pliocene lignite coal deposits.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4400492&req=5

SFT049F1: Geographical distribution of endemic foci and pliocene lignite coal deposits.
Mentions: Balkan endemic nephropathy (BEN) is a familial, slowly progressive, chronic renal disease with insidious onset in the fifth decade of life and terminal renal failure in the sixth or seventh decade. The occurrence of BEN has been recorded with a high prevalence rate in Serbia, Bulgaria, Romania, Bosnia and Herzegovina and Croatia (Figure 1). The first cases were described in 1956 in Bulgaria, where Tanchev et al. [1] published the first detailed clinical description of the new entity. A year later, a renal disease with almost identical clinical and epidemiological characteristics was reported in Yugoslavia (Serbia) [2]. In 1961, it was found that a similar nephropathy was also prevalent in Romania [3]. The most prominent features of the disease are its endemic nature, the long latent period before development of the disease, familial clustering of the disease and the remarkably high incidence of upper urothelial cancer (UUC) associated with BEN or occurring in the population at risk [4–6]. No cases have been documented in children or adolescents. The aetiology of BEN has been the subject of many published studies resulting in the generation of several hypotheses. Although data published on these various hypotheses have presented some indication of their relevance to the aetiology of BEN, only one of them, dealing with chronic aristolochic acid (AA) poisoning, has provided conclusive evidence related to the aetiology of BEN and its clinical characteristics.Fig. 1.

Bottom Line: The clinical signs and symptoms of BEN are non-specific and often remain unrecognized for years.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).

View Article: PubMed Central - PubMed

Affiliation: Clinic of Nephrology, Clinical Centre , Nis , Serbia.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus