儿童Kimura病合并肾病综合征临床、病理及预后分析

发布时间：2018-05-31 17:24

本文选题：肾病综合征 + Kimura病　；参考：《临床儿科杂志》2017年03期

【摘要】：目的探讨儿童Kimura病合并肾病综合征的临床特征。方法回顾分析4例Kimura病合并肾病综合征患儿的临床资料。结果 4例患儿均为男性,血清IgE均升高,外周血嗜酸性粒细胞均无升高;患儿的肾脏病理表现不一,2例出现急性肾小管损伤,1例重复肾活检出现病理转型,另1例肾脏间质见少许嗜酸性粒细胞浸润。4例患儿均对口服激素敏感。但除1例随访时间短,尚未复发,另3例均有复发,且均表现为尿蛋白阳性,不伴随淋巴结肿大。3例复发患儿加用免疫抑制剂治疗,其中2例加用他克莫司,复发次数较前减少。结论儿童Kimura病合并肾病综合征病程长,肾脏病理表现不一,对激素敏感,但易复发,需联合免疫抑制剂治疗,他克莫司可能对减少复发、延缓肾脏病理进展有一定疗效。
[Abstract]:Objective to investigate the clinical features of Kimura's disease complicated with nephrotic syndrome in children. Methods the clinical data of 4 cases of Kimura's disease complicated with nephrotic syndrome were retrospectively analyzed. Results all the 4 cases were male, the serum IgE level was increased, and the eosinophils in peripheral blood were not increased, the renal pathological manifestations were different in 2 cases and acute renal tubular injury occurred in 1 case, and the pathological transition was found in 1 case of repeated renal biopsy. In another case, a little eosinophil infiltration was found in renal stroma. 4 cases were sensitive to oral hormone. But except for one case with short follow-up time and no recurrence, the other 3 cases had recurrence, and all of them showed positive urinary protein. 3 cases of relapsed children without lymphadenopathy were treated with immunosuppressant, 2 cases of them were treated with tacrolimus, 2 cases were treated with tacrolimus. The number of recurrence was less than that before. Conclusion Children with Kimura's disease complicated with nephrotic syndrome have a long course of disease, different renal pathological manifestations, sensitive to hormones, but easy to relapse. Tacrolimus may have a certain effect on reducing recurrence and delaying renal pathological progress.
【作者单位】：西安市儿童医院肾脏科;陕西西北妇女儿童医院内三科;
【分类号】：R726.9;R725.5

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