肾病综合征是儿科常见的肾病之一,是一组由各种病因引起的表现为大量蛋白尿、低白蛋白血症、高脂血症和不同程度水肿的临床症候群。根据全国协作调查资料,原发性肾病综合征约占儿科泌尿系统住院患儿的21%,发病率仅次于急性肾炎,居第2位。1病因和发病机制:肾病综合征可分为原发性、继发性和先天性3种类型,原发性肾病综合征占90%以上;继发性可见于系统性红斑狼疮、过敏性紫癜、乙型肝炎病毒相关性肾炎以及感染、药物、中毒、肿瘤或代谢病等;以往认为先天性肾病综合征少见,随着基因诊断技术的成熟,越来越多的先天性和(或)遗传性肾病综合征被发现。原发性肾病综合征的病因尚 Nephrotic syndrome is one of the most common pediatric kidney diseases. It is a group of clinical syndromes caused by a variety of etiologies, including massive proteinuria, hypoalbuminemia, hyperlipidemia and varying degrees of edema. According to the national collaborative survey data, primary nephrotic syndrome accounted for about 21% of pediatric urinary tract hospitalized children, the incidence rate is second only to acute nephritis, ranking No. 2. 1 Etiology and pathogenesis: Nephrotic syndrome can be divided into primary, secondary and congenital 3 types, primary nephrotic syndrome accounts for more than 90%; Secondary can be found in systemic lupus erythematosus, Henoch-Schonlein purpura , Hepatitis B virus-associated nephritis and infections, drugs, poisoning, cancer or metabolic diseases; In the past that congenital nephrotic syndrome rare, with the genetic diagnosis of mature technology, more and more congenital and (or) genetic Nephrotic syndrome was found. The etiology of primary nephrotic syndrome is still