Nakada T, Shigematsu H, Bartter F C, Delea C S
Nephron. 1980;26(2):78-84. doi: 10.1159/000181956.
A study was made of two renal biopsy specimens obtained from a 22-year-old woman with Bartter's syndrome, the first to substantiate the diagnosis, the second 2 years later after the treatment with spironolactone. The first renal biopsy revealed remarkable hyperplasia and hypertrophy of the juxtaglomerular apparatus. The numerous proliferating cells were characterized by abundant lysosomal granules and dilated endoplasmic reticulum. The macula densa revealed slight proliferation, with narrow intercellular space. The ultrastructural picture of the second biopsy specimen showed increased lipofuscin-like granules in the juxtaglomerular cells, with epitheloid cells of the macula densa showing degeneration and irregular dilatation of the intercellular spaces. However, the light microscopic finding was compatible with that of the first. These findings suggest that the treatment was inadequate although clinical features or biochemical laboratory data were improved.
对一名患有巴特综合征的22岁女性的两份肾活检标本进行了研究,第一份标本用于确诊,第二份标本是在使用螺内酯治疗2年后获取的。第一次肾活检显示肾小球旁器显著增生和肥大。大量增殖细胞的特征是溶酶体颗粒丰富,内质网扩张。致密斑有轻微增殖,细胞间隙狭窄。第二次活检标本的超微结构显示肾小球旁细胞中脂褐素样颗粒增加,致密斑的上皮样细胞出现变性,细胞间隙不规则扩张。然而,光镜检查结果与第一次相符。这些发现表明,尽管临床特征或生化实验室数据有所改善,但治疗并不充分。