儿童木村病5例报告并文献复习

Kimura disease:5 cases report and literature review

目的探讨儿童木村病(kimura disease,KD)的临床特征、诊断及治疗。方法回顾性收集2000年1月至2016年12月在北京协和医院确诊为KD且发病年龄<18岁的患儿,对其临床表现、实验室检查、病理学检查和治疗方案等资料进行分析。结果对5例确诊KD的男性患儿进行分析,发病年龄9~13岁,中位年龄12岁,从发病至确诊KD的病程为2个月至3年。患儿均表现为头颈部淋巴结肿大或软组织肿块,外周血嗜酸性粒细胞增多,其中2例行血清Ig E检测,其水平明显升高;1例伴发肾病综合征;5例患儿均具有KD典型病理改变。3例患儿行手术切除联合局部放疗治疗,疗效满意未有复发。合并肾病者停用激素复发后,采用低剂量激素维持未复发。结论儿童KD发病率低,临床表现及实验室检查与成人相似,需依靠病理明确诊断,采用手术切除联合局部放疗疗效确切。

Objective To describe the clinical features, diagnosis and treatment of children with Kimura disease（KD）. Methods The clinical manifestation, lab examination results, pathological data, treatment and outcome of 5 children with KD diagnosed in Peking Union Medical College Hospital from January 2000 to December 2016 were retrospectively analyzed. Results All five KD pediatric patients were males, the age of onset varied from 9 to 13 years, the median age was 12 years. Time from onset to diagnosis ranged from 2 months to 3 years. All the patients presented with painless lymphadenopathy or subcutaneous masses in the head and neck region, and had peripheral blood eosinophilia. Elevated serum Ig E levels were detected in 2 patients tested. One had nephrotic syndrome. Biopsies were taken in all cases and the characteristic histopathological presentations were discovered. Three patients had been treated and responded well to surgical excision and local irradiation. The patient with nephrotic syndrome developed recurrence after discontinued steroid treatment, and maintained no recurrence after using low doses of glucocorticoid. Conclusion KD rarely occurs in children, and its clinical manifestations and examination results are similar in children and adult. Pathological examination is essential for diagnosis. Surgical excision and postoperative local irradiation has definite therapeutic effect on KD.