【摘要】 目的探讨儿童毛细血管内增生性紫癜性肾炎(DEP-HSPN)的临床、病理、治疗及预后。方法回顾性分析19例确诊为DEP-HSPN患儿的临床、病理、预后及其影响因素,随机选取同期入院行肾活检明确为HSPN但非DEP的55例患儿作为对照组。结果 19例DEP-HSPN患儿,男14例、女5例,平均年龄(10.6±2.6)岁;肾活检前病程(19.4±7.4)d;占同期肾活检紫癜性肾炎患儿3.92%。19例DEP-HSPN中10例为肾病综合征型,9例为血尿和蛋白尿型;均接受免疫抑制治疗,14例完全缓解,5例部分缓解;肾脏病理分级均为Ⅲb级,伴有6.38%~36.36%不等的新月体。DEP-HSPN患儿与病理均为Ⅲb级的55例年龄、性别相匹配的对照组患儿比较,肾穿前病程短、蛋白尿水平高、肾脏病理慢性损伤积分低,差异具有统计学意义(P均<0.05);新月体百分比两组差异无统计学意义(P>0.05)。结论儿童DEP-HSPN起病急,临床表现重,组织活动性病变多而慢性病变少;尚无证据表明毛细血管内增生性病变是影响HSPN预后的危险因素。更多还原

【Abstract】 Objective To explore the clinical and pathological features, treatment, and prognosis of capillary proliferative purpura nephritis(DEP-HSPN) in children. Methods The clinical data of 19 children diagnosed with DEP-HSPN were retrospectively analysis. Fifty-five children diagnosed with HSPN by renal biopsy were randomly selected as control group. Results The average age was 10.6±2.6 years old, and the average course of disease were 19.4±7.4 days before renal biopsy in 19 children with DEP-HSPN(14 males and 5 females) who make up 3.92% of anaphylactic purpura nephritis children confirmed by renal biopsy in the same period. In these 19 children, there were 10 cases having nephrotic syndrome and 9 case having hematuria and proteinuria type, all of whom were received immunosuppressive therapy. Finally, 14 cases achieved completely remission and 5 cases had partly remission. All of their classifications of renal pathology were Ⅲb levels, accompanied with 6.38% to 36.36% of crescents. Compared with 55 age and sex matched children with renal pathology classification of Ⅲb, the DEPHSPN children had shorter disease course, higher level of proteinuria, and lower pathological score of chronic renal injury(P all<0.05). There was similar percentage of crescent between two groups. Conclusions Children with DEP-HSPN usually have rapid onset, severe clinical manifestations, more active lesions and less chronic lesions. There is no evidence that the capillary proliferative lesion is a risk factor in the prognosis of HSPN so far.更多还原