Iványi B
University Institute of Pathology and Stereological Research Laboratory, Kommunehospitalet, University of Arhus, Denmark.
Arch Pathol Lab Med. 1993 Aug;117(8):837-40.
Glomerulotubular disconnections at the immediate postglomerular segment of the proximal tubules, accounting for an impaired renal function, were demonstrated recently in several chronic nonglomerular renal disorders. To analyze the glomerulotubular junctions in Bence Jones cast nephropathy, paraffin blocks from the kidneys of nine deceased patients with myeloma and chronic renal failure on whom autopsies were performed were studied in serial sections. Kidneys from seven deceased patients without renal disease on whom autopsies also were done served as controls. For stereological estimations, the percentages of patent and sclerosed glomerular profiles, the relative volume fractions, and the absolute volumes of the interstitium, tubules, and glomeruli were determined. In Bence Jones cast nephropathy, 96% of the glomerular profiles were patent, and the reconstruction of randomly chosen glomerulotubular junctions revealed that 84% of the patent glomeruli had normal tubular connections. There was an increased relative interstitial volume (fibrous tissue) and a decreased relative tubular volume. The absolute values indicated severe interstitial fibrosis, but not tubular atrophy. The results show that in contrast with other chronic nonglomerular nephropathies, chronic renal failure in Bence Jones cast nephropathy does not result from nephron disconnection at the immediate postglomerular segment of the proximal tubules. The main factor responsible for the decrease in renal function appears to be the progressive severe fibrosis of the interstitium.
近来在几种慢性非肾小球性肾脏疾病中已证实,近端肾小管紧邻肾小球后的节段出现肾小球肾小管分离,这会导致肾功能受损。为分析本-周蛋白管型肾病中的肾小球肾小管连接情况,对9例死于骨髓瘤和慢性肾衰竭且已进行尸检的患者肾脏石蜡块进行了连续切片研究。7例同样进行了尸检的无肾脏疾病的死者肾脏作为对照。为进行体视学评估,测定了开放和硬化肾小球轮廓的百分比、间质、肾小管和肾小球的相对体积分数以及绝对体积。在本-周蛋白管型肾病中,96%的肾小球轮廓是开放的,对随机选择的肾小球肾小管连接进行重建显示,84%的开放肾小球具有正常的肾小管连接。相对间质体积(纤维组织)增加,相对肾小管体积减少。绝对值表明存在严重的间质纤维化,但不存在肾小管萎缩。结果表明,与其他慢性非肾小球性肾病不同,本-周蛋白管型肾病中的慢性肾衰竭并非由近端肾小管紧邻肾小球后的节段的肾单位分离所致。导致肾功能下降的主要因素似乎是间质进行性严重纤维化。