伴毛细血管内增生的儿童紫癜性肾炎的临床病理及转归分析

Analysis of clinicopathology and outcome of childhood Henoch-Schonlein purpura nephritis with endocapillary proliferation

目的:探讨伴毛细血管内增生的儿童紫癜性肾炎(EP-HSPN)的临床、病理和疾病转归。方法:回顾性分析2005年1月-2013年6月本院收治的经肾活检确诊为EP-HSPN患儿的临床、病理和随访资料,选取同时段的非EP-HSPN患儿作为对照,将2组进行比较。进一步将EP-HSPN患儿毛细血管内增生的受累肾小球比例按≥50%、<50%分为DEP-HSPN组(弥漫性毛细血管内增生的HSPN)和非DEP-HSPN组,将2组进行比较。结果:1EP-HSPN组46例,非EP-HSPN组44例,与非EP-HSPN组患儿比较,EP-HSPN组患儿年龄小,起病较急,临床多见关节肿痛、胃肠道受累、肾病水平蛋白尿及低白蛋白血症,更多表现为肾病综合征型;病理上,EP-HSPN组患儿较多呈III级病理表现,新月体比例、毛细血管袢坏死比例高,肾小管间质病变损伤程度轻。246例EPHSPN患儿中,DEP-HSPN组13例,非DEP-HSPN组33例,与非DEP-HSPN组患儿相比,DEP-HSPN组患儿有较高的24 h尿蛋白量、血尿素氮,更多表现为肾病水平蛋白尿、低白蛋白血症和肾病综合征型,较少表现为血尿和蛋白尿型。3针对EP-HSPN患儿多采取激素联合环磷酰胺冲击治疗(占50.0%),非EP-HSPN患儿则较多采用对症治疗(占47.7%)。随访结果中,2组患儿在随访时间和疾病转归上差异无统计学意义。结论:EP-HSPN患儿临床表现和肾小球病理改变较重,早期积极予以激素联合免疫抑制剂干预可获得良好的短期疗效。

Objective： To investigate the clinicopathogical characteristics and outcome of Henoch-Schon-lein purpura nephritis with endocapillary proliferation （EP-HSPN） in children. Methods： Retrospectively ana- lyzed the clinicopathology and follow-up data of children whom were biopsy-proven EP-HSPN in our hospital from January 2005 to June 2013, and the contemporary non-EP-HSPN children were chosen as control group. Further comparisons among the EP-HSPN cases were performed. Results： in contrast to the 44 cases ofnon- EP-HSPN group, the 46 cases of EP-HSPN group showed younger, a shorter course of disease, joint and gastroin- testinal tract more presented involvement, nephrotic level of proteinuria, hypoalbuminemia, nephrotic syndrome type, grade II! lesion, a higher rate of crescent and capillary loops necrosis, but slighter damage of renal tubular interstitial. In 46 cases in children with EP-HSPN, compared with the 33 cases ofnon-DEP-HSPN, 13 cases of DEP-HSPN had heavy proteinuria, higher BUN, more showed nephrotic level of proteinuria, hypoalbuminemia and nephrotic syndrome type, less presented hernaturia and proteinuria type. OFor EP-HSPN children, more given steroid in combination with cyclophospamide treatment （50%）, whereas for non-EP-HSPN, more given symptomatic treatment （47.7%）. The follow-up results of two groups showed no statistically significant differ- ences in outcome. Conclusion： EP-HSPN shows severity of clinical manifestations and glomerular pathological changes. Immunosuppressant treatment in the early stage of disease is effective for a short term outcome.