原发性膜性肾病(PMN)是儿童期罕见的一种肾病病理类型,临床表现为肾病综合征或无症状蛋白尿,常伴 镜下血尿、氮质血症或高血压;病理特征为肾小球基底膜增厚伴或不伴钉突形成,颗粒状IgG和补体C 3沿肾小球毛细血 管襻沉积。近年来儿童PMN发病率升高引起国内外学者的关注,但其发病机制尚不清楚,且无统一的治疗方案。文章综 述近年PMN的研究现状。
Primary membranous nephropathy (PMN) is a rare pathological type of nephropathy in childhood. The clinical manifestations are nephrotic syndrome or asymptomatic proteinuria, often accompanied by microscopic hematuria, azotemia or hypertension. The pathological features are glomerular basement membrane thickening with or without spike formation and deposits of granular IgG and complement C 3 along the glomerular capillary loops. In recent years, the incidence of PMN in children has increased, but the pathogenesis of PMN in children is still unclear, and there is no uniform therapeutic protocol. This article summarizes the research status of PMN in recent years.