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目的总结儿童原发性肾病综合征(primary nephrotic syndrome,PNS)的临床特征。方法回顾性分析1996年8月1日至2006年7月31日964例(其中有747例新发病例)住院PNS患儿的临床及实验室资料。结果747例患儿中,男性568例,女性179例,年龄0.3~17.3岁,中位年龄4.7岁;1~5岁的PNS患儿有385例(51.5%)。与肾炎型肾病相比较,单纯型肾病患儿的血清白蛋白较低(P<0.05),而总胆固醇和低密度脂蛋白较高(P<0.05),两者24h尿蛋白量比较无统计学差异(P>0.05);PNS患儿的血清IgG比正常值低(P<0.01),IgM和IgE比正常值高(P<0.05),IgA和C3与正常值比较无统计学差异(P>0.05)。PNS复发的主要诱因是上呼吸道感染,其次是患儿不规则用药。结论儿童PNS以1~5岁好发,PNS存在体液免疫功能紊乱,降低血脂及预防、控制感染是治疗的关键,而加强宣传、指导对PNS的治疗也至关重要。
Objective To summarize the clinical features of primary nephrotic syndrome (PNS) in children. Methods The clinical and laboratory data of 964 inpatients with PNS (including 747 new cases) from August 1, 1996 to July 31, 2006 were retrospectively analyzed. Results Of the 747 children, 568 were male and 179 were female, ranging in age from 0.3 to 17.3 years, with a median age of 4.7 years; 385 (51.5%) were PNS children aged 1-5 years. Compared with nephritis nephropathy, serum albumin was lower (P <0.05) and total cholesterol and low density lipoprotein were higher in children with simple nephropathy (P <0.05), but there was no statistical difference between the two groups (P> 0.05). Serum IgG in PNS children was lower than normal (P <0.01), IgM and IgE were higher than normal (P <0.05), and IgA and C3 were not significantly different from normal (P> 0.05). PNS relapse is the main cause of upper respiratory tract infection, followed by children with irregular medication. Conclusions Children with PNS occur predominantly from 1 to 5 years old. PNS has humoral immune dysfunction, blood lipid lowering and prevention and control of infection are the keys to treatment. It is also important to strengthen publicity to guide the treatment of PNS.