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目的探讨诊断遗传性肾炎（Ａｌｐｏｒｔ综合征）的新指标。方法应用超微病理检查结合免疫荧光方法，对４例电镜显示肾小球基底膜（ＧＢＭ）变薄或厚薄不均的患儿进行研究。结果２例Ａｌｐｏｒｔ综合征患儿的肾活检组织标本电镜下ＧＢＭ弥漫性厚薄不均，广泛变厚和分层，并与变薄的ＧＢＭ并存。间接免疫荧光检查ＧＢＭ中抗Ⅳ型胶原α３链、α５链抗体完全阴性，抗Ⅳ型胶原α１链抗体明显增强。而２例薄基底膜肾病患儿肾活检组织尽管电镜下ＧＢＭ也有变薄、断裂和分层现象，且其中１例有节段性增厚，但间接免疫荧光检查ＧＢＭ中抗Ⅳ型胶原成分均无改变。结论超微病理结合间接免疫荧光学检查，有助于诊断Ａｌｐｏｒｔ综合征，并能将其与薄基底膜肾病相鉴别
Objective To explore new diagnostic criteria for hereditary nephritis (Alport syndrome). Methods Ultrasonography and immunofluorescence were used to study the results of 4 electron microscopes in children with glomerular basement membrane (GBM) thinning or uneven thickness. Results The biopsies of 2 cases of Alport syndrome were diffusely thick, thin and thick under electron microscopy, extensively stratified and stratified, and coexisted with thinned GBM. Indirect immunofluorescence staining of anti-type Ⅳ collagen α3 chain in GBM, α5 chain antibody was completely negative, anti-type Ⅳ collagen α1 chain antibody was significantly enhanced. In the 2 cases of renal biopsy in children with thin basement membrane nephropathy, there were thinning, rupture and stratification of GBM under electron microscopy, and 1 case had segmental thickening. However, indirect immunofluorescence showed no change in anti-collagen Ⅳ in GBM . Conclusion The combination of indirect microscopy and indirect immunofluorescence can help diagnose Alport syndrome and differentiate it from thin basement membrane nephropathy