Abstract：
Recent progress in glomerular immune complex and complement-mediated diseases have refined diagnostic categories and informed mechanistic understanding of disease development in pediatric patients. Herein, we discuss selected advances in 3 categories. First, membranous nephropathy antigens are increasingly utilized to characterize disease in pediatric patients and include phospholipase A2 receptor (PLA2R), Semaphorin 3B (Sema3B), neural epidermal growth factor-like 1 (NELL1), and protocadherin FAT1, as well as the lupus membranous-associated antigens exostosin 1/2 (EXT1/2), neural cell adhesion molecule 1 (NCAM1), and transforming growth factor beta receptor 3 (TGFBR3). Second, we examine advances in techniques for paraffin and light chain immunofluorescence (IF), including the former\'s function as a salvage technique and their necessity for diagnosis in adolescent cases of membranous-like glomerulopathy with masked IgG kappa deposits (MGMID) and proliferative glomerulonephritis with monotypic Ig deposits (PGNMID), respectively. Finally, progress in understanding the roles of complement in pediatric glomerular disease is reviewed, with specific attention to overlapping clinical, histologic, and genetic or functional alternative complement pathway (AP) abnormalities among C3 glomerulopathy (C3G), infection-related and post-infectious GN, \"atypical\" post-infectious GN, immune complex mediated membranoproliferative glomerulonephritis (IC-MPGN), and atypical hemolytic uremic syndrome (aHUS).
摘要：
肾小球免疫复合物和补体介导的疾病的最新进展已经完善了儿科患者的诊断类别和对疾病发展的知情机制理解。在这里，我们讨论3类的选定进展。首先,膜性肾病抗原越来越多地用于表征儿科患者的疾病，包括磷脂酶A2受体（PLA2R），信号3B(Sema3B)，神经表皮生长因子样1（NELL1），和原钙粘蛋白FAT1，以及狼疮膜相关抗原外生菌素1/2（EXT1/2），神经细胞粘附分子1（NCAM1），和转化生长因子β受体3（TGFBR3）。第二,我们研究了石蜡和轻链免疫荧光(IF)技术的进展，包括前者作为挽救技术的功能及其在青少年病例中诊断为具有掩盖的IgGκ沉积（MGMID）的膜样肾小球病和具有单型Ig沉积（PGNMID）的增生性肾小球肾炎的必要性，分别。最后,综述了补体在小儿肾小球疾病中作用的研究进展，特别注意重叠的临床，组织学，和C3肾小球病（C3G）中的遗传或功能替代补体途径（AP）异常，感染相关和感染后GN，“非典型”感染后GN，免疫复合物介导的膜增殖性肾小球肾炎（IC-MPGN），非典型溶血性尿毒综合征（aHUS）。
