遗传性肾小球疾病是一组具有基因突变背景,主要累及肾小球的疾病。除累及肾脏外,常伴身体其他器官受累。狭义的遗传性肾炎指Alport综合征(AS),广义的还包括Fabry病、先天性肾病综合征、薄基膜肾病,指甲-髌骨综合征,部分或全身脂肪发育不良等遗传性肾小球疾病。Alport综合征(AS) Hereditary glomerular disease is a group of diseases that have a gene mutation background that mainly affects the glomerulus. In addition to involving the kidneys, often accompanied by other body organs involved. Narrow genetic hereditary nephritis refers to Alport syndrome (AS) and broadly includes hereditary glomerular diseases such as Fabry’s disease, congenital nephrotic syndrome, thin-base membranous nephropathy, nail-patella syndrome, partial or total systemic dysplasia . Alport syndrome (AS)