弥漫毛细血管内皮增生性紫癜性肾炎患儿是否伴发新月体形成的临床特征对比

Comparison of clinical characteristics between diffused capillary endothelial proliferative Henoch-Schonlein purpura nephritis children with and without crescents formation

目的对比弥漫毛细血管内皮增生性紫癜性肾炎(DEP-HSPN)患儿是否伴发新月体形成的临床特征。方法回顾性分析陕西省商洛市妇幼保健计划生育中心2013年2月至2017年6月经肾活检确诊的110例DEP-HSPN患儿的临床资料。其中,肾小球病理损害无新月体形成组24例(A组),肾小球病理损害有新月体形成组86例(B组),两组患儿均给予标准方案治疗,比较两组的临床特征指标及预后情况。结果B组Ⅴ型和肉眼血尿比例明显高于A组[59.3%(51/86)比0和83.7%(72/86)比29.2%(7/24)],尿红细胞计数、24h尿蛋白、血肌酐明显高于A组[(112.4±20.3)个/HP比(45.2±10.6)个/HP、(2471.6±242.0)mg比(1358.5±109.3)mg、(44.9±9.6)μmol/L比(32.3±5.2)μmol/L],血白蛋白明显低于A组[(22.8±3.8)g/L比(35.1±5.7)g/L],差异有统计学意义(P<0.01或<0.05);两组肾性蛋白尿比例、肾小球病理表现及免疫球蛋白沉积情况比较差异无统计学意义(P>0.05)。A组治疗1年临床完全缓解17例,无症状性血尿7例,B组分别为50和36例,两组比较差异无统计学意义(P>0.05)。结论当DEP-HSPN伴发新月体形成时,肉眼血尿、尿红细胞计数及大量蛋白尿比例明显增加;急性期给予免疫抑制剂联合治疗及缓解期长期序贯治疗,可获得良好预后。

Objective To investigate the clinical characteristics of diffused capillary endothelial proliferative Henoch-Sch?nlein purpura nephritis (DEP-HSPN) children with or without crescents formation. Methods The clinical data of 110 DEP-HSPN children diagnosed by renal biopsy from February 2013 to June 2017 in Shangluo Maternal and Child Health Family Planning Service Center were retrospectively analyzed. Among them, 24 cases had no crescents formation (group A), and 86 cases had crescents formation (group B). The children of 2 groups were treated with standard regimen, and the clinical characteristics and prognosis between 2 groups were compared. Results The rates of Ⅴ type and gross hematuria in group B were significantly higher than those in group A: 59.3%(51/86) vs. 0 and 83.7%(72/86) vs. 29.2%(7/24), the levels of urine red blood cell count, 24 h urine protein and serum creatinine were significantly higher than those in group A:(112.4 ± 20.3)/HP vs.(45.2 ± 10.6)/HP,(2 471.6 ± 242.0) mg vs.(1 358.5 ± 109.3) mg and (44.9 ± 9.6)μmol/L vs.(32.3 ± 5.2)μmol/L, the level of serum albumin was significantly lower than that in group A:(22.8 ± 3.8) g/L vs.(35.1 ± 5.7) g/L, and there were statistical differences (P<0.01 or <0.05). There were no statistical differences in rate of nephrogenous albuminuria, glomerular pathology type and immunoglobulin deposition condition between 2 groups (P>0.05). There were 17 cases of complete remission and 7 cases of asymptomatic hematuria in group A, and 50 and 36 cases in group B, respectively. There was no significant difference between 2 groups (P > 0.05). Conclusions When DEP-HSPN is accompanied by crescent formation, gross hematuria, urine red blood cell count and the proportion of massive albuminuria can increase significantly. Combination therapy with immunosuppressive agents in acute stage and long-term sequential treatment could achieve good prognosis.