Prevalence of Balkan Endemic Nephropathy Has Not Changed Since 1971 in the Kolubara Region in Serbia

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Abstract

Background/Aims: Thirty-one years after the first cross-sectional study, the population of Vreoci, a Balkan endemic nephropathy (BEN) village, was reinvestigated in order to determine the current prevalence of BEN and the clinical and laboratory characteristics of BEN family members with detected signs of kidney disease. Methods: A total of 2,009 inhabitants (82% of the adult population) of the village were examined. Danilovic’s criteria were used for diagnosis and classification of BEN. Results: The prevalence of BEN (1.70%) was similar to that in 1971 (1.67%). Diagnosis of BEN was established in 19 BEN family members, suspected BEN in 23, proteinuria in 29, while 16 healthy members were examined as controls. Urine protein, alpha1-microglobulin levels and frequency of glucosuria were significantly higher and kidney length significantly smaller in the three patient groups than in healthy persons. Serum urea and creatinine levels were significantly higher, but creatinine clearance was lower in BEN and BEN suspected patients than in the other two groups. Conclusion: The prevalence of BEN remains stable over time in Vreoci village. Manifested disease was found in both BEN and BEN suspected patients. In persons with proteinuria but not enough criteria for BEN, tubular disorders and hypertension were frequently found.

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Alpha-1-microglobulin: an indicator protein for renal tubular function.

R A Crockson, Ann Cooper, Hsin-Chieh Yu … (1983)

A comparison of urinary alpha 1-microglobulin concentrations to the behaviour of other indicators of renal tubular disorders, beta 2-microglobulin, retinol-binding protein and N-acetyl-beta-D-glucosaminidase (NAG) has been made. In acute tubular disorders the concentrations of urinary beta 2M and RBP are highly correlated (r = 0.89) but this is less marked for alpha 1M and beta 2M (r = 0.55) and alpha 1M and RBP r = 0.48. NAG tends to run a parallel course to alpha 1M concentrations but lags behind the recovery of low molecular weight protein reabsorption following injury of the tubular cells. The concentrations of alpha 1M, and in particular its stability at low pH suggest that this protein may be useful in screening for tubular abnormalities and detecting chronic asymptomatic renal tubular dysfunction. Urinary alpha 1M greater than 15 mg/g creatinine is strongly suspicious of a proximal tubular dysfunction. The distinction between pure tubular proteinuria and mixed glomerular and tubular proteinuria requires further analysis.

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Recent data on endemic nephropathy and related urothelial tumors in Croatia.

Maja Peraica, Ana-Marija Domijan, M. Miletić (2005)

Endemic nephropathy (EN) is a renal disease of unknown etiology. In Croatia it occurs in the rural population in 14 villages located in the western part of Brodsko-Posavska county. This region also has an unusually high incidence of otherwise rare upper urothelial cancers. Between 1991 and 2002 the average general mortality for both sexes in the endemic region was 10.3 per thousand and the specific mortality for patients with EN was 0.65 per thousand (M 0.58/10((3)), F 0.72/10(3)). The average age of death of patients with EN was 69.2 years (M 67.7, F 70.3), which is similar to the life expectancy for the rest of the population in the county (67.8, M 64.4, F 71.8). This life expectancy is significantly higher than in the period 1957-1960 when the average age of EN-related death was 45.1 years. Between 1995 and 2002, in contrast to both Croatia as a whole and the respective county, the specific mortality with tumors of the pyelon and ureter in the endemic region was much higher in women than in men (9.020 and 4.697 per 100,000, respectively). The specific mortality of all patients with urothelial tumors was 14 times higher in the endemic region than in Brodsko-Posavska county and 55 times higher than in Croatia overall. The much higher specific urothelial-tumor mortality in women than in men (in contrast to the rest of the country) and the higher specific EN mortality indicate that the causative agent of the two nosological entities is the same.

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Clinical markers in adult offspring of families with and without Balkan Endemic Nephropathy.

W. Karmaus, A Bonev, V. Simeonov … (2006)

Balkan Endemic Nephropathy (BEN) is a kidney disease that progresses slowly. Only a few studies have investigated renal clinical markers in offspring of BEN families before the onset of the disease. This project aimed to determine whether kidney function and structure are altered in BEN offspring compared with non-BEN offspring. The study population consisted of 102 adult BEN offspring and a control group of 99 non-BEN offspring. We collected urine and blood samples, and conducted face-to-face interviews, physical examinations and ultrasound measurements of the kidney. Total protein, albumin, beta2-microglobulin and creatinine in urine, creatinine and urea in serum, and creatinine clearance (CCR) were determined. Two risk factors were assessed: first, the overall status of being an offspring from a BEN family, and second, the specific status of a mother and/or father with BEN. The data were analyzed using linear regression. After adjusting for confounders, we found that kidney length and minimal cortex width in BEN offspring were significantly decreased. Urine concentrations of total protein, albumin, and beta2-microglobulin were higher in BEN offspring. Regarding parental history, the associations were statistically significant only for the offspring of mothers who had BEN, with the exception of minimal cortex width, which showed no parental difference. For CCR, we did not identify a statistically significant effect for BEN offspring status nor for parental history. In conclusion, adult offspring of BEN families can be characterized by shorter kidney length and an increased excretion of albumin, total protein, and beta2-microglobulin, in particular, when the mother had BEN.