Coleman M, Stirling J W, Langford L R, Frith P A, Barratt L J
Department of Pathology, Repatriation General Hospital, Adelaide, Australia.
Am J Nephrol. 1994;14(1):47-54. doi: 10.1159/000168685.
Ultrastructural morphometric studies of glomerular basement membrane (GBM) thickness are described in two renal biopsy specimens from a patient who presented with hemoptysis and hematuria mimicking Goodpasture's syndrome. Significant GBM abnormality, with attenuation as the main lesion, identified in a biopsy specimen taken during active clinical disease appeared to have resolved in a second biopsy specimen taken during the recovery phase. There was no evidence of glomerulonephritis. Concurrent lung biopsy studies showed focal alveolar-capillary wall basal lamina changes of uncertain diagnostic significance. These observations suggest the alternative possibilities that GBM attenuation may be either an acquired consequence of systemic disease or may be part of an hitherto unrecognized primary multisystem abnormality of basal lamina affecting, in this case, glomerular and pulmonary laminae, resulting in hematuria and hemoptysis. The morphometric studies in this case indicate that simple-mean measurements of GBM thickness are inadequate alone for the quantitative study of this lamina because significant inter- and intraglomerular membrane variation, if irregularly distributed, can remain undetected.
对一名表现为咯血和血尿、疑似患古德帕斯彻综合征的患者的两份肾活检标本进行了肾小球基底膜(GBM)厚度的超微结构形态计量学研究。在临床疾病活动期采集的一份活检标本中发现了明显的GBM异常,主要病变为变薄,而在恢复期采集的另一份活检标本中,这种异常似乎已得到缓解。没有肾小球肾炎的证据。同时进行的肺活检研究显示,局灶性肺泡-毛细血管壁基底膜改变,其诊断意义尚不确定。这些观察结果提示了两种可能性:GBM变薄可能是全身性疾病的后天结果,也可能是一种迄今未被认识的原发性多系统基底膜异常的一部分,在本例中影响肾小球和肺基底膜,导致血尿和咯血。该病例的形态计量学研究表明,仅靠GBM厚度的简单平均测量不足以对该基底膜进行定量研究,因为如果肾小球间和肾小球内的膜变化分布不规则,可能仍无法检测到。