Balkan nephropathy
| Balkan nephropathy | ||
| ICD-10 | N15.0 | |
|---|---|---|
| OMIM | 124100 | |
| DiseasesDB | 31409 | |
| MeSH | C12.777.419.570.643.150 | |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2] Aarti Narayan, M.B.B.S [3]
Synonyms and keywords: Danubian endemic familial nephropathy; DEFN; Balkan endemic nephropathy; BEN; Nephropathica endemica
Overview
Balkan nephropathy is a form of chronic interstitial nephritis that is endemic to limited areas of Bulgaria, the former Yugoslavia, and Romania. It is characterized by a progressive shrinking of the kidneys that is often associated with uroepithelial tumors. It was first identified in the 1920s among several small, discrete communities along the Danube River and its major tributaries, in the modern countries of Croatia, Bosnia, Serbia, Romania and Bulgaria. A striking feature of the disease is its very localized occurrence. There are approximately ten small areas where it occurs, all of them more or less rural, but nothing seems to connect those areas other than the occurrence of this illness.
Historical Perspective
The first official publication was made by the Bulgarian nephrologist Dr. Yoto Tanchev and his team in 1956 in the Bulgarian Journal Savremenna Medizina,[2] a priority generally acknowledged by the international nephrological community.[3] Their study was based on a wide screening of inhabitants of the villages around the town of Vratsa, Bulgaria. Their contribution to the study of this unusual endemic disease of the kidneys consisted in the description of symptoms which were not typical for the common chronic nephritis, i.e., incidence only in adults (no children affected), lack of high blood pressure, xanthochromia of palms and soles (Tanchev's sign), early hypochromic anemia, a lack of proteinuria, and slow progression to chronic kidney failure.
A striking feature of the disease is its very localized occurrence. There are approximately ten small areas where it occurs, all of them more or less rural, but nothing seems to connect those areas, other than the occurrence of this illness. Tanchev and colleagues suggested the condition was a disease sui generis. Their initial hypothesis for the etiology was possible intoxication with heavy metals, because the villages affected were supplied with water coming from the nearby karst-type Vratsa Mountain.
The first name of the disease was "Vratsa nephritis". Later, following descriptions of similar ailments in Yugoslavia and Romania, the condition became known as Balkan endemic nephropathy.
Pathophysiology
- The etiology for Balkan nephropathy is currently unknown.[1].
- It has recently been hypothesized that chronic exposure to dietary aristolochic acid is a major risk for Balkan nephropathy. *Aristolochic acid may come from Aristolochia clematitis, a plant native to the endemic region, and its seeds may comingle with wheat used for bread.[2]
Causes
The etiology for DEFN is currently unknown.[3]. It has recently been hypothesized that chronic exposure to dietary aristolochic acid is a major risk for DEFN. Aristolochic acid may come from Aristolochia clematitis, a plant native to the endemic region, and its seeds may comingle with wheat used for bread.[4] This theory has recently gained further support through research by Arthur P. Grollman, cancer biologist and the director of Stony Brook University's chemical biology lab in New York, and Bojan Jelaković, an associate professor at the Zagreb University School of Medicine. [5]
Related Chapters
References
- ↑ Batuman V (2006). "Fifty years of Balkan endemic nephropathy: daunting questions, elusive answers". Kidney Int. 69 (4): 644–6. doi:10.1038/sj.ki.5000231. PMID 16467889.
- ↑ Grollman AP, Shibutani S, Moriya M; et al. (2007). "Aristolochic acid and the etiology of endemic (Balkan) nephropathy". doi:10.1073/pnas.0701248104. PMID 17620607.
- ↑ Batuman V (2006). "Fifty years of Balkan endemic nephropathy: daunting questions, elusive answers". Kidney Int. 69 (4): 644–6. doi:10.1038/sj.ki.5000231. PMID 16467889.
- ↑ Grollman AP, Shibutani S, Moriya M; et al. (2007). "Aristolochic acid and the etiology of endemic (Balkan) nephropathy". Proc. Natl. Acad. Sci. U.S.A. 104 (29): 12129–34. doi:10.1073/pnas.0701248104. PMID 17620607.
- ↑ Julia C. Mead (2007). "Manna from hell". The Scientist. 21 (11): 44.