Howie A J, Brewer D B
J Pathol. 1984 Mar;142(3):205-20. doi: 10.1002/path.1711420308.
We describe a distinctive and previously undescribed abnormality of the kidney. It consists of a combination of changes in the glomerulus and in the proximal convoluted tubule. The glomerular abnormality consists of a well-localized collection of intracapillary foam cells and marked vacuolation of the adjacent glomerular epithelial cells. The abnormality is always situated in the same position in the glomerular tuft namely adjacent to the origin of the proximal convoluted tubule, with adhesion to Bowman's capsule. The rest of the tuft appears normal by light microscopy but shows foot-process fusion by electron microscopy. The abnormality of the proximal convoluted tubular cells invariably affects the first part of the tubule adjacent to the glomerulus but in some cases involves the tubules more extensively in the cortex. Study of a series of 100 2-micron serial sections from one case indicated that probably every glomerulus had a lesion at the origin of the tubule. A study of 185 renal biopsies nearly all with segmental lesions revealed biopsies from 20 patients with this distinctive abnormality. There were 14 males, age 20-57, median 44 years, and six females age 19-65, median 22 years, all presenting with proteinuria, nearly all with the nephrotic syndrome. All except four were treated with steroids and in all those treated the proteinuria improved. Only one patient not treated with steroids had progressive renal impairment. Four patients died, none from renal failure. This lesion, the glomerular tip lesion, as we have called it, seems to be a well-defined and specific pathological entity. It has some similarities to minimal change nephropathy but there are clear and important differences. Previously it may have been included in series of cases of 'focal glomerulosclerosis' but that term is imprecise and is generally taken to have an unfavourable clinical course. For these reasons it is inappropriate to use 'focal glomerulosclerosis' as a name for the glomerular tip lesion.
我们描述了一种独特的、此前未被描述过的肾脏异常情况。它由肾小球和近端曲管的多种变化组合而成。肾小球异常表现为毛细血管内泡沫细胞的局限性聚集以及相邻肾小球上皮细胞的显著空泡化。该异常总是位于肾小球丛的同一位置，即紧邻近端曲管的起始处，并与鲍曼囊粘连。肾小球丛的其余部分在光学显微镜下看似正常，但在电子显微镜下显示足突融合。近端曲管细胞的异常总是影响紧邻肾小球的曲管第一部分，但在某些情况下会更广泛地累及皮质中的曲管。对一个病例的100张2微米连续切片进行研究表明，可能每个肾小球在曲管起始处都有病变。对185例几乎均为节段性病变的肾活检进行研究，发现有20例患者存在这种独特的异常。其中男性14例，年龄20 - 57岁，中位数44岁；女性6例，年龄19 - 65岁，中位数22岁，所有患者均有蛋白尿，几乎均为肾病综合征。除4例患者外，其余均接受了类固醇治疗，且所有接受治疗的患者蛋白尿均有所改善。只有1例未接受类固醇治疗的患者出现了进行性肾功能损害。4例患者死亡，均非死于肾衰竭。我们所称的这种病变，即肾小球顶端病变，似乎是一种明确且特定的病理实体。它与微小病变性肾病有一些相似之处，但也存在明显且重要的差异。此前它可能被纳入“局灶性肾小球硬化”病例系列中，但该术语并不精确，且一般认为其临床病程不佳。基于这些原因，用“局灶性肾小球硬化”来命名肾小球顶端病变是不合适的。
