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与明显特发性血尿相关的肾小球基底膜异常:超微结构形态计量分析

Glomerular basement membrane abnormalities associated with apparently idiopathic hematuria: ultrastructural morphometric analysis.

作者信息

Coleman M, Haynes W D, Dimopoulos P, Barratt L J, Jarvis L R

出版信息

Hum Pathol. 1986 Oct;17(10):1022-30. doi: 10.1016/s0046-8177(86)80086-7.

Abstract

In a recent review of 480 renal biopsies, 41 cases were identified in which glomerular basement membrane (GBM) ultrastructural abnormalities were the major lesion. All of the patients had hematuria. None had evidence of immune-mediated glomerulonephritis. Positive family histories of renal disease were present in the majority of cases, and one case of Alport's syndrome was included. Subjectively, the GBM changes were variable but nearly always included membrane thinning. For objective characterization of this glomerular abnormality, a detailed morphometric study of GBM thickness was undertaken: 12 of these patients (study group) were compared with seven patients (control subjects) with subjectively normal glomeruli who underwent biopsy for reasons other than nonsurgical hematuria but who were also thought to have normal glomerular ultrastructure. The seven control subjects had a mean GBM thickness of 394 nm (SD, 19; range, 356 to 432 nm). Of the 12 study group patients, 11 had mean GBM thicknesses significantly different from control values (nine had mean GBM thinning: range, 235 to 327 nm; two had thickening: means, 440 and 469 nm). In the remaining case (Alport's syndrome) the overall mean was normal, but an abnormal distribution of very thin and very thick GBM regions was seen. Of the four apparently normal hematuric patients, significant mean GBM thinning (326 to 347 nm) was demonstrated in three, with an excess of thin GBM in the fourth case, although the mean thickness was normal. Thus, measurable abnormalities were defined in all of the cases of hematuria examined. The GBM measurements confirmed the subjective impression of membrane abnormality, usually attenuation, as the principal finding in this group of hematuric patients. Furthermore, morphometric analysis may reveal subtle changes of GBM thickness missed by subjective assessment.

摘要

在最近一项对480例肾活检的回顾中,确定了41例以肾小球基底膜(GBM)超微结构异常为主要病变的病例。所有患者均有血尿。均无免疫介导性肾小球肾炎的证据。大多数病例有肾病家族史阳性,其中包括1例Alport综合征。主观上,GBM变化各异,但几乎总是包括膜变薄。为了对这种肾小球异常进行客观表征,对GBM厚度进行了详细的形态计量学研究:将其中12例患者(研究组)与7例患者(对照对象)进行比较,后者因非手术性血尿以外的原因接受活检,但也被认为肾小球超微结构正常,且主观上肾小球正常。7例对照对象的GBM平均厚度为394 nm(标准差为19;范围为356至432 nm)。在12例研究组患者中,11例的GBM平均厚度与对照值有显著差异(9例平均GBM变薄:范围为235至327 nm;2例增厚:平均值分别为440和469 nm)。在其余病例(Alport综合征)中,总体平均值正常,但可见极薄和极厚GBM区域的异常分布。在4例明显正常的血尿患者中,3例显示出显著的GBM平均变薄(326至347 nm),第4例虽平均厚度正常,但薄GBM过多。因此,在所检查的所有血尿病例中均发现了可测量的异常。GBM测量结果证实了膜异常(通常为变薄)是这组血尿患者的主要发现这一主观印象。此外,形态计量学分析可能会揭示主观评估遗漏的GBM厚度的细微变化。

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