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Renal function, protein excretion, and pathology of Balkan endemic nephropathy. III, Light and electron microscopic studies
Ferluga, Dušan, 1934- ...
Renal functional alterations, urinary protein excretion, and pathology f Balkan endemic nephropathy. III. Light and electronmicroscopic studies. This study was performed to evaluate ...histomorphological features of BEN in 50 kidney biopsies from patients who met the epidemiologic, clinical and laboratory criteria for BEN. This is the first such study reported in detail. The patients were divided into three groups based on the DTPA clearance values: group 1, greather th. 99 ml/min, group 2,51 to 99 ml/min, and group 3,29 to 50 ml/min. All patients in all groups had an increase in proteinuria consisting of proteins less than 25,000 daltons. Multifocal interstitial sclerosis spreading from the superficial into the deep cortex was found in 49 (98 per cent ), tubular atrophy in 48 (96 per cent ), and global glomerular sclerosis with microvascular hyalinosis/sclerosis in 40 (80 per cent ). Not only the incidence but also the intensity of sclerotic and atrophic changes were significantly increased when compared to age-related standards. An accelerated aging process may be assumed to occur in BEN. More peculiar additional findings with ' much lower incidence and exlent included multifocal vascular and glomerular capillary changes resembling the chronic form ofthrombotic microangiopathy group of diseases. These findings, together with the presence of arteriolar hyalinosis and tubulointerstitial sclerosis seen in patients with cyclosporine nephrotoxicity suggest that the mechanism oftoxicity may be similar to BEN. We conclude that the histopathology as a unique disease. is predominantly tubulointerstitial sclerosis without infiltrates. The combination of the histology, tubular proteinuria, geographic distribution, familial occurrence, and the remarkable association with papillary transitional cell carcinoma ofthe renal pelvis and ureters, qualifies BEN
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