儿童毛细血管内皮增生性紫癜性肾炎50例临床与病理分析

The clinical and pathological features of capillary endothelial proliferative purpura nephritis in 50 children

目的探讨以弥漫性毛细血管内皮细胞增生为病理表现的紫癜性肾炎的临床及病理特点。方法回顾分析近5年来经肾活检确诊的50例毛细血管内皮增生性紫癜性肾炎(DEP-HSPN)患儿临床表现和病理。结果 50例患儿的病理损害包括单纯DEP-HSPN 11例(男7例、女4例)和毛细血管内皮细胞增生合并新月体形成(非单纯DEP-HSPN)39例(男27例、女12例),两组患儿起病至初诊时病程、年龄的差异无统计学意义(P>0.05)。11例单纯DEP-HSPN患儿临床均为Ⅲ型;39例非单纯DEP-HSPN患儿临床Ⅲ型16例,Ⅴ型23例。所有患儿均出现血尿、蛋白尿;非单纯DEP-HSPN患儿肉眼血尿的发生率、尿红细胞计数、24 h尿蛋白定量和血肌酐水平均高于单纯DEP-HSPN组,但血浆白蛋白水平低于单纯DEP-HSPN组,差异均有统计学意义(P<0.05)。DEP-HSPN易出现新月体,新月体形成率为11.1%(5.0%~27.6%),节段性病变、肾小管间质损害发生比例低;所有患儿系膜区均有非单纯Ig A沉积。50例患儿治疗1年时,22例临床完全缓解,28例为无症状性血尿,无活动性肾病及肾功能不全。32例非单纯DEP-HSPN患儿治疗前,治疗后1、3、6、12个月,各个时间点之间比较,24 h尿蛋白定量、血浆白蛋白水平、肉眼血尿以及镜下血尿比例的差异均有统计学意义(P<0.01);24 h尿蛋白定量、肉眼血尿的比例随治疗时间延长逐渐下降,而血浆白蛋白水平逐渐升高。结论 DEP-HSPN临床以肉眼血尿和大量蛋白尿为临床表现,起病急重,病理易合并新月体,当合并新月体形成,临床症状更重。急性期强有力的免疫抑制剂联合治疗、长期序贯随访治疗,该病可获得良好预后。

ObjectiveTo investigate the clinical and pathological characteristics of Henoch-Scho..nlein purpura nephritis with diffuse capillary endothelial cell proliferation as pathological manifestation. MethodsThe clinical manifestations and pathology of capillary endothelial proliferative purpura nephritis （DEP-HSPN） diagnosed by renal biopsy were retrospectively analyzed in 50 children in recent 5 years. ResultsThe pathological lesions in 50 cases included simple DEP-HSPN in 11 cases （7 males and 4 females） and capillary endothelial cell proliferation combined with crescents formation （non-simple DEP-HSPN） in 39 cases （27 males and 12 females）. There was no signifcant difference in the course of disease and age between the two groups （P〉0.05）. The clinical type of 11 cases of simple DEP-HSPN was type III. In 39 cases of non-simple DEP-HSPN, 16 cases were type III and 23 cases were type V. All of the children had hematuria and proteinuria. The incidence of gross hematuria, urine red blood cell count, 24 h urine protein, and serum creatinine levels in children with non-simple DEP-HSPN were signifcantly higher than those in simple DEP-HSPN group, but the plasma albumin level was signifcantly lower than that in simple DEP-HSPN group. It was easy to have crescent formation in DEP-HSPN, and the rate of crescent formation was 11.1% （5.0%-27.6%）. The incidence of segmental lesions and renal tubular interstitial damage was low. All children had non simple IgA deposits in the mesangial area. In the 50 children treated for 1 year, 22 had complete remission, 28 had asymptomatic hematuria, and none had active nephropathy and renal insuffciency. In 32 cases of non-simple DEP-HSPN, the 24 h urinary protein, plasma albuminlevel, and the incidences of gross hematuria and microscopic hematuria were statistically different before treatment and 1, 3, 6, 12 months after treatment （P〈0.01）. The 24 h urine protein and gross hematuria gradually decreased with the prolongation of treatment, while the level of plasma albumin was gradually increased. ConclusionsDEP-HSPN is characterized by grosshematuria and proteinuria. The onset is acute and it is easy to have crescent formation. When combined with crescent formation, the clinical symptoms are more severe. The combination of strong immunosuppressive agents and long-term sequential follow-up treatment is effective in acute stage. The prognosis is good.