Balkan endemic nephropathy

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Disease Overview

Balkan endemic nephropathy is a kidney disease that affects people living in rural areas of Bosnia, Bulgaria, Croatia, Romania, and Serbia (areas along the Danube river and its tributaries). Affected individuals develop kidney damage that slowly progresses over 10 to 20 years to kidney failure. Many people with this condition also develop a type of bladder cancer known as upper urothelial carcinoma (UUC). Balkan endemic nephropathy is caused by chronic dietary exposure to low concentrations of a toxin called aristolochic acid, which comes from a plant called Aristolochia clematis.[3232][13664][13665] Genetics factors may also be involved.[13664][13665] There is no specific prevention or treatment of this condition. Management depends upon strategies that delay progression and manage the complications of the kidney disease. Dialysis or kidney transplantation may be needed.[13665]


Synonyms

  • BEN
  • Aristolochic acid nephropathy
  • AAN
  • Danubian endemic familial nephropathy
  • DEFN
  • Nephropathia epidemica

For more information, visit GARD.

National Organization for Rare Disorders