Bernstein J
Am J Kidney Dis. 1987 Sep;10(3):222-9. doi: 10.1016/s0272-6386(87)80177-4.
The characteristic electron microscopic abnormality in Alport's syndrome is thickening of the glomerular basement membrane with splitting and lamellation of the lamina densa. It is commonly accompanied by severe attenuation of the basement membrane and sometimes by disruption of capillary walls. The ultrastructural abnormality is secondary to a more basic defect in the chemical structure of the glomerular basement membrane. Clues to chemical abnormalities have come from immunochemical studies, which suggest a lack of or alteration in that part of the noncollagenous portion of type 4 collagen related to Goodpasture antigen. Variability in the results of immunochemical studies suggest biochemical heterogeneity that still needs to be correlated with the known evidence of genetic heterogeneity in Alport's syndrome.
阿尔波特综合征典型的电子显微镜下异常表现为肾小球基底膜增厚，伴致密层分裂和分层。通常伴有基底膜严重变薄，有时还伴有毛细血管壁破坏。这种超微结构异常是肾小球基底膜化学结构更基本缺陷的继发表现。化学异常的线索来自免疫化学研究，该研究表明与抗肾小球基底膜抗体抗原相关的Ⅳ型胶原非胶原部分存在缺失或改变。免疫化学研究结果的变异性提示存在生化异质性，这仍需与阿尔波特综合征已知的遗传异质性证据相关联。
