Stoyanov George S, Kobakova Ina, Petkova Lilyana, Dzhenkov Deyan L, Popov Hristo
General and Clinical Pathology/Forensic Medicine and Deontology, Medical University of Varna, Varna, BGR.
Cureus. 2021 Jan 1;13(1):e12415. doi: 10.7759/cureus.12415.
Balkan endemic nephropathy (BEN) is a form of interstitial nephritis seen only in certain geographical areas in the Balkan peninsula. Herein we describe the gross and histological changes in a diseased 42-year-old male Caucasian patient with BEN. All the changes fit the classically described alterations, with copper hue discoloration of the skin of the torso and orange discoloration of the soles and palms. Grossly, the kidneys were atrophic, with the left one weighing 31 grams and the right one 32 grams. Their surface was predominantly smooth with areas of fine granulations and cystic transformations. Histology revealed hyalinization of the glomeruli, predominantly in the external part of the cortex, severe vascular changes, interstitial fibrosis, and scant inflammatory cell infiltrate. The renal pelvis and ureters revealed multiple urothelial papillomas and atypical urothelial hyperplasia. BEN is only one geographical variant of interstitial nephritis caused by exposure to aristolochic acid. Other forms of this condition include Chinese herb nephropathy/aristolochic acid nephropathy, as well as several similar endemic conditions with a yet unestablished link to aristolochic acid.
巴尔干地方性肾病（BEN）是一种仅在巴尔干半岛某些地理区域出现的间质性肾炎。在此，我们描述了一名患有BEN的42岁男性白种病患者的大体和组织学变化。所有变化均符合经典描述的改变，躯干皮肤呈铜色，手掌和脚掌呈橙色。大体上，双肾萎缩，左肾重31克，右肾重32克。肾脏表面主要光滑，有细小颗粒状区域和囊性变。组织学检查显示肾小球玻璃样变，主要位于皮质外部，有严重的血管改变、间质纤维化以及少量炎症细胞浸润。肾盂和输尿管可见多个尿路上皮乳头状瘤和非典型尿路上皮增生。BEN只是因接触马兜铃酸而引起的间质性肾炎的一种地理变异型。这种疾病的其他形式包括中草药肾病/马兜铃酸肾病，以及几种与马兜铃酸尚未明确关联的类似地方性疾病。
