Balkan nephropathy
Encyclopedia
Danubian endemic familial nephropathy (DEFN), also called Balkan endemic nephropathy, is a form of interstitial nephritis
Interstitial nephritis
Interstitial nephritis is a form of nephritis affecting the interstitium of the kidneys surrounding the tubules...

. It was first identified in the 1920s among several small, discrete communities along the Danube
Danube
The Danube is a river in the Central Europe and the Europe's second longest river after the Volga. It is classified as an international waterway....

 River and its major tributaries, in the modern countries of Croatia
Croatia
Croatia , officially the Republic of Croatia , is a unitary democratic parliamentary republic in Europe at the crossroads of the Mitteleuropa, the Balkans, and the Mediterranean. Its capital and largest city is Zagreb. The country is divided into 20 counties and the city of Zagreb. Croatia covers ...

, Bosnia and Herzegovina
Bosnia and Herzegovina
Bosnia and Herzegovina , sometimes called Bosnia-Herzegovina or simply Bosnia, is a country in Southern Europe, on the Balkan Peninsula. Bordered by Croatia to the north, west and south, Serbia to the east, and Montenegro to the southeast, Bosnia and Herzegovina is almost landlocked, except for the...

, Serbia
Serbia
Serbia , officially the Republic of Serbia , is a landlocked country located at the crossroads of Central and Southeast Europe, covering the southern part of the Carpathian basin and the central part of the Balkans...

, Romania
Romania
Romania is a country located at the crossroads of Central and Southeastern Europe, on the Lower Danube, within and outside the Carpathian arch, bordering on the Black Sea...

 and Bulgaria
Bulgaria
Bulgaria , officially the Republic of Bulgaria , is a parliamentary democracy within a unitary constitutional republic in Southeast Europe. The country borders Romania to the north, Serbia and Macedonia to the west, Greece and Turkey to the south, as well as the Black Sea to the east...

.

The first official publication was made by the Bulgarian nephrologist Dr.Yoto Tanchev and his team in 1956 in the Bulgarian Journal "Savremenna Medizina",9, 1956, a priority generally acknowledged by the international nephrological community. 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 strange 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 anemia of hypochromous type, lack of proteinuria and slowly progression of the kidney failure. Tanchev et al. suggested this condition to be a disease sui generis. Their hypothesis for the etiology was related to a 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, an avalanche of research works and publications by Bulgarian and foreign scientists followed. Unfortunately, the etiology remains unknown till present. Dr. Yoto Tanchev died in the age of 83 in 2000.

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. It has recently been hypothesized that chronic exposure to dietary aristolochic acid
Aristolochic acid
Aristolochic acids are a family of carcinogenic, mutagenic, and nephrotoxic compounds commonly found in the Aristolochiaceae family of plants, including Aristolochia and Asarum, which are commonly used in Chinese herbal medicine. Aristolochic acid I is the most abundant of the aristolochic acids...

 is a major risk for DEFN. Aristolochic acid may come from Aristolochia clematitis
Aristolochia clematitis
Aristolochia clematitis, Birthwort, is a twining herbaceous plant in the Aristolochiaceae family, which is native to Europe. The leaves are heart shaped and the flowers are pale yellow and tubular in form...

, a plant native to the endemic region, and its seeds may comingle with wheat used for bread. 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.

External links

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