Danubian endemic familial nephropathy: Difference between revisions

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   OMIM          = 124100 |
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{{SK}} DEFN; Balkan endemic nephropathy
{{SK}} DEFN; Balkan endemic nephropathy; endemic nephropathy, Balkan; nephropathy, Balkan


==Overview==
==Overview==

Revision as of 20:00, 28 September 2012

Danubian endemic familial nephropathy
DiseasesDB 31409
MeSH D001449

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: DEFN; Balkan endemic nephropathy; endemic nephropathy, Balkan; nephropathy, Balkan

Overview

Danubian endemic familial nephropathy (DEFN), sometimes called Balkan endemic nephropathy,[1] is a form of interstitial nephritis. 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.

Causes

The etiology for DEFN is currently unknown.[2]. 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.[3] 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. [4]

References

  1. Online Mendelian Inheritance in Man (OMIM) 124100
  2. 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.
  3. 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.
  4. Julia C. Mead (2007). "Manna from hell". The Scientist. 21 (11): 44.

Related Chapters

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