Balkan endemic nephropathy

The patients are distinguished from those suffering from other causes of end-stage renal disease by showing an absence of high blood pressure, xanthochromia of palms and soles (Tanchev's sign), early hypochromic anemia, absence of proteinuria, and slow progression of kidney failure.{{cite journal |vauthors=Tanchev Y, Dorossiev D |title=The first clinical description of Balkan endemic nephropathy (1956) and its validity 35 years later |journal=IARC Sci. Publ. |issue=115 |pages=21–8 |year=1991 |pmid=1820335 }} There is no specific therapy; BEN causes end-stage renal disease, for which the only effective treatments are dialysis or a kidney transplant. In endemic areas BEN is responsible for up to 70% of end-stage renal disease. At least 25,000 individuals are known to have this form of the disease.The Epidemiology, Diagnosis, and Management of Aristolochic Acid Nephropathy: A Narrative Review [http://annals.org/article.aspx?articleid=1667269 Annals of Internal Medicine] 19 March 2013, Vol 158, No. 6

Patients with BEN have a greatly increased rate of transitional cell carcinoma of the upper urothelial tract, (the renal pelvis and ureters). (In populations without BEN, most urothelial cancer occurs in the bladder.{{cite news|title=Poisoned Land: On the trail of a mystery disease in the Balkans|url=http://www.newyorker.com/reporting/2013/08/12/130812fa_fact_batuman|access-date=August 23, 2013|newspaper=The New Yorker|date=August 12, 2013|author=Elif Batuman}} )

Dietary exposure to aristolochic acid is the cause of BEN and its attendant transitional cell cancers.{{cite journal |vauthors=Grollman AP, Shibutani S, Moriya M, et al |title=Aristolochic acid and the etiology of endemic (Balkan) nephropathy |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=104 |issue=29 |pages=12129–34 |year=2007 |pmid=17620607 |doi=10.1073/pnas.0701248104 |pmc=1913550|bibcode=2007PNAS..10412129G |doi-access=free }} Former hypotheses that included roles for ochratoxin, poisoning by organic compounds leached from lignite or by heavy metals, viruses, and trace-element deficiencies, are not supported by current evidence. Genetic factors may be involved in determining which persons exposed to aristolochic acid suffer from BEN and which do not.Balkan endemic nephropathy. U.S. Department of Health & Human Services. National Institutes of Health, National Center for Advancing Translational Sciences. https://rarediseases.info.nih.gov/diseases/8576/balkan-endemic-nephropathy

In the Balkan region, dietary aristolochic acid exposure may come from the consumption of the seeds of Aristolochia clematitis (European birthwort), a plant native to the endemic region, which grows among wheat plants and whose seeds mingle with the wheat used for bread.{{cite journal |author=Julia C. Mead |title=Manna from hell |journal=The Scientist |volume=21 |issue=11 |pages=44 |year=2007 |url=http://www.the-scientist.com/article/home/53787/ |access-date=2007-11-07 |archive-date=2008-09-06 |archive-url=https://web.archive.org/web/20080906165820/http://www.the-scientist.com/article/home/53787/ |url-status=dead }} Aristolochic-acid-containing herbal remedies used in traditional Chinese medicine are associated with a related—possibly identical—condition known as "Chinese herbs nephropathy".{{cite journal |author=De Broe ME |title=Chinese herbs nephropathy and Balkan endemic nephropathy: toward a single entity, aristolochic acid nephropathy |journal=Kidney Int. |volume=81 |issue=6 |pages=513–5 |date=March 2012 |pmid=22373701 |doi=10.1038/ki.2011.428 |doi-access=free }}

Exposure to aristolochic acid is associated with a high incidence of uroepithelial tumorigenesis.{{cite book|editor=Ronco, Claudio|title=Critical care nephrology|publisher=Elsevier Health Sciences|year=2008|isbn=978-1-4160-4252-5|page=1699|url=https://books.google.com/books?id=XkKn96HThzEC&pg=PA1699|display-editors=etal}}{{cite journal |vauthors=Chen CH, Dickman KG, Moriya M, Zavadil J, Sidorenko VS, Edwards KL, Gnatenko DV, Wu L, Turesky RJ, Wu XR, Pu YS, Grollman AP |title=Aristolochic acid-associated urothelial cancer in Taiwan |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=109 |issue=21 |pages=8241–6 |date=May 2012 |pmid=22493262 |pmc=3361449 |doi=10.1073/pnas.1119920109 |doi-access=free }}

Biopsy - For definitive diagnosis{{cn|date=July 2024}}

The first official published description of the disease was made by the Bulgarian nephrologist Dr. Yoto Tanchev (1917–2000) and his team in 1956 in the Bulgarian Journal Savremenna Medizina,Tanchev Y, Evstatiev Z, Dorossiev D, Pencheva J, Tzvetkova G. Studies on the nephritides in the District of Vratza. Savremena Medicina 1956; 7: 14–29 (Bulgarian). a priority generally acknowledged by the international nephrological community.

The disease was originally called "Vratsa nephritis," and became known as "Balkan endemic nephropathy" later, after people living in Yugoslavia and Romania were found to be suffering from it as well.

• Nephropathy
• Citrinin
• Ochratoxin A

{{reflist}}

{{Medical resources

| DiseasesDB = 31409

| ICD10 = {{ICD10|N|15|0|n|10}}

| ICD9 =

| ICDO =

| OMIM = 124100

| MedlinePlus =

| eMedicineSubj =

| eMedicineTopic =

| MeshID = D001449

}}

{{Nephrology}}

Category:Kidney diseases