Howie A J
Department of Pathology, University of Birmingham, Medical School, UK.
Pediatr Nephrol. 1993 Aug;7(4):370-4. doi: 10.1007/BF00857541.
Segmental sclerosing glomerular lesions are usually all grouped together and called focal segmental glomerulosclerosis. This has meant that the term that was originally used for a defined clinical entity is now applied to a variety of conditions in man and experimental animals, with the assumption that the morphological changes are the same in all conditions. Studies of the position of segmental lesions within glomeruli, the size of glomeruli and the proportion of glomeruli affected have shown that this assumption is wrong. Such studies have identified a disease that corresponds to the original clinical concept of focal segmental glomerulosclerosis. This begins with the nephrotic syndrome in patients whose renal biopsies show large glomeruli with mesangial hypercellularity and segmental lesions at every tubular origin. Later the biopsies have segmental lesions throughout the glomerular tuft. This disease differs clinically and pathologically from other conditions that have segmental sclerosing lesions, such as in patients with reduced renal mass or patients with hypertension and proteinuria. The term focal segmental glomerulosclerosis is now too ambiguous and unsatisfactory to be used without qualification.
节段性硬化性肾小球病变通常都被归为一类,称为局灶节段性肾小球硬化。这意味着最初用于定义临床实体的术语现在被应用于人类和实验动物的多种情况,假定在所有情况下形态学改变都是相同的。对肾小球内节段性病变的位置、肾小球大小以及受影响肾小球的比例进行的研究表明这一假定是错误的。此类研究已确定了一种与局灶节段性肾小球硬化的原始临床概念相对应的疾病。这种疾病始于肾病综合征患者,其肾活检显示肾小球较大,伴有系膜细胞增多以及每个肾小管起始处的节段性病变。后来活检显示整个肾小球毛细血管襻出现节段性病变。这种疾病在临床和病理上与其他具有节段性硬化病变的情况不同,比如肾实质减少的患者或高血压和蛋白尿患者。现在,局灶节段性肾小球硬化这个术语过于模糊且不尽人意,不能不加限定地使用。