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

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EP-HSPN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。

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

Objective: To investigate the clinicopathogical characteristics and outcome of Henoch-Schonlein purpura nephritis with endocapillary proliferation (EP-HSPN) in children. Methods: Retrospectively analyzed 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: ①In contrast to the 44 cases of non-EP-HSPN group, the 46 cases of EP-HSPN group showed younger, a shorter course of disease, joint and gastrointestinal tract more presented involvement, nephrotic level of proteinuria, hypoalbuminemia, nephrotic syndrome type, grade III lesion, a higher rate of crescent and capillary loops necrosis, but slighter damage of renal tubular interstitial. ②In 46 cases in children with EP-HSPN, compared with the 33 cases of non-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 hematuria and proteinuria type. ③For 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 differences 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.