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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。
Objective To investigate the clinical, pathological, therapeutic and prognosis of children’s capillary proliferative purpura nephritis (DEP-HSPN). Methods The clinical, pathological, prognostic factors and their influencing factors in 19 children with DEP-HSPN were retrospectively analyzed. Fifty-five children with renal biopsy confirmed as HSPN but not DEP were randomly selected as the control group. Results 19 cases of DEP-HSPN children, 14 males and 5 females, mean age (10.6 ± 2.6) years; pre-biopsy course (19.4 ± 7.4) d; accounted for 3.92% of children with renal biopsy in the same period. Among the 19 DEP-HSPNs, 10 were nephrotic syndromes and 9 were hematuria and proteinuria. All received immunosuppressive therapy, 14 were completely relieved and 5 were partially relieved. Nephridial pathological grade was grade IIIb with 6.38 % ~ 36.36% of the crescent. There was a statistically significant difference between the children with DEP-HSPN and those with pathological grade IIIb in 55 age and sex matched controls, with a short duration of pre-delivery, high proteinuria, and chronic kidney injury with a low level of proteinuria P <0.05). There was no significant difference in the percentage of crescent between the two groups (P> 0.05). Conclusions Children with acute onset of DEP-HSPN, clinical manifestations, tissue active lesions and less chronic lesions; there is no evidence that capillary proliferative lesions are risk factors for the prognosis of HSPN.