目的探讨儿童原发性肾病综合征低白蛋白血症诊断标准。方法收集1993年1月至2012年12月在北京大学第一医院住院的原发性肾病综合征患儿的临床资料,根据初次诊断肾病综合征时的最低血浆白蛋白水平将患儿分为<25 g·L~(-1)组和25~30 g·L~(-1)组。比较两组的肾脏病理类型、临床表型、激素效应、合并症和预后。结果进入本文分析患儿458例,白蛋白25~30 g·L~(-1)组28例,白蛋白<25 g·L~(-1)组430例,两组性别和年龄差异无统计学意义。(1)血浆白蛋白水平<25 g·L~(-1)组和25~30 g·L~(-1)组分别有12例和141例行肾脏病理分析,两组肾脏病理类型分布差异无统计学意义,均以局灶节段性肾小球硬化症为主,两组微小病变或轻微病变分别占21.9%和8.3%;(2)2组临床表型、免疫抑制剂的应用种类、严重合并症的发生情况和激素相关不良反应的发生情况差异均无统计学意义,2组激素效应分布差异有统计学意义,血浆白蛋白水平<25 g·L~(-1)组和25~30 g·L~(-1)组激素敏感比例分别为68.4%(294/430)和50.0%(14/28);(3)血浆白蛋白水平25~30 g·L~(-1)组严重预后发生率(14.3%,4/28)高于<25 g·L~(-1)组(4.4%,19/430)。结论具有大量蛋白尿伴有血浆白蛋白25~30 g·L~(-1)的患儿肾脏病理类型以非微小病变为主,其预后较血浆白蛋白<25 g·L~(-1)者更为严重,建议早期行肾活检。 Objective To investigate the diagnostic criteria of hypoalbuminemia in children with primary nephrotic syndrome. Methods The clinical data of children with primary nephrotic syndrome who were hospitalized in Peking University First Hospital from January 1993 to December 2012 were collected. According to the lowest plasma albumin level in primary nephrotic syndrome, children were divided into < 25 g · L -1 group and 25 ~ 30 g · L -1 group. The renal pathological types, clinical phenotypes, hormonal effects, complications and prognosis were compared between the two groups. Results In this study, 458 children were enrolled in this study. 28 cases of albumin 25 ~ 30 g · L -1 group and 430 cases of albumin <25 g · L -1 group showed no statistical difference in gender and age Significance of learning. (1) There were 12 cases and 141 cases of pathological analysis of renal albumin in 25 g · L ~ (-1) group and 25 ~ 30 g · L ~ (-1) group, respectively No statistical significance, were focal segmental glomerulosclerosis-based, two groups of minor lesions or minor lesions accounted for 21.9% and 8.3%; (2) 2 clinical phenotypes, types of immunosuppressants There was no significant difference in the incidence of serious complications and hormone-related adverse reactions between the two groups. There was significant difference in the distribution of hormones between the two groups. The levels of plasma albumin <25 g · L -1 and 25 The sensitivity of hormones to 30 g · L -1 group was 68.4% (294/430) and 50.0% (14/28), respectively. (3) The plasma albumin level was 25-30 g · L -1, Group severe prognosis (14.3%, 4/28) was higher than <25 g · L -1 group (4.4%, 19/430). Conclusions The pathological type of renal pathology in children with a large amount of albuminuria and albumin 25 ~ 30 g · L ~ (-1) is mainly non - minimal lesion, the prognosis is lower than that of plasma albumin <25 g · L ~ (-1) More serious, it is recommended early renal biopsy.