Morita M, White R H, Coad N A, Raafat F
Department of Nephrology, Children's Hospital, Ladywood, Birmingham, UK.
Clin Nephrol. 1990 May;33(5):211-9.
Serial sections of renal biopsies obtained from 44 nephrotic children with focal segmental glomerulosclerosis (FSGS) were reviewed in order to determine the glomerular location of segmental lesions and relate the findings to the outcome of illness. There were 23 boys and 21 girls aged 0.9-14.2 years at onset. FSGS was classified as "hilar" in biopsies containing at least one lesion contiguous with or involving the hilum, regardless of the location of other lesions, and as "peripheral" in the absence of hilar lesions. Of the 44 initial biopsies, 33 were designated hilar and 6 peripheral; the remaining 5 were unclassifiable as it was not possible to determine the location of 1-3 lesions in each biopsy. Twenty-eight of the 33 hilar biopsies also contained peripheral lesions, including paratubular (glomerular "tip") lesions in 15 instances. Paratubular lesions as the predominant abnormality were observed in only four biopsies. Repeat biopsies showed that transition occurred from one type to another, and only 4 biopsies remained with a final designation of peripheral FSGS. After a follow-up period of 1.6-24.9 years (mean 9.3), there was no significant difference in outcome between hilar and peripheral FSGS, whether diagnosed on the initial or repeat biopsy. The division into separate categories is not clearcut, and the use of this as a prognostic aid does not justify the additional cost of preparing and examining numerous serial sections.
对44例患有局灶节段性肾小球硬化(FSGS)的肾病患儿的肾活检连续切片进行了回顾,以确定节段性病变的肾小球定位,并将结果与疾病转归相关联。发病时年龄为0.9 - 14.2岁,其中男孩23例,女孩21例。在含有至少一个与肾门相邻或累及肾门的病变的活检中,无论其他病变的位置如何,FSGS被分类为“肾门型”;在无肾门病变的情况下,分类为“外周型”。在44例初始活检中,33例被指定为肾门型,6例为外周型;其余5例无法分类,因为在每个活检中无法确定1 - 3个病变的位置。33例肾门型活检中的28例也含有外周病变,其中15例包括肾小管旁(肾小球“顶端”)病变。仅在4例活检中观察到以肾小管旁病变为主要异常。重复活检显示从一种类型转变为另一种类型,最终仅4例活检被指定为外周型FSGS。在1.6 - 24.9年(平均9.3年)的随访期后,肾门型和外周型FSGS在疾病转归方面无显著差异,无论在初始活检还是重复活检中诊断。分类并不明确,将其用作预后辅助手段并不能证明制备和检查大量连续切片所增加的成本是合理的。