Abstract:

Objective　To improve the level of diagnosis and treatment of children with Kimura’s disease complicated with nephrotic syndrome. Methods　In order to understand the current pathogenesis，diagnosis and treatment of children with Kimura’s disease complicated with nephrotic syndrome，we reviewed 3 cases of  Kimura’s disease in our department from 2011 to 2015，and studied the related literature at home and abroad at the same time. Results　Kimura’s disease is a rare chronic immune inflammatory disease which mainly damaged lymph nodes，soft tissue and salivary glands. It mostly occurs in Asian people between the age of 28 and 32，especially male. At present，the cause of the disease and its pathogenesis is still unclear. Histopathology is the only way to diagnose Kimura’s disease. As the golden standard of kidney disease diagnosis，renal biopsy is a big help for the diagnosis and treatment of renal damage of Kimura’s disease. Current treatments mainly depend on medication，and generally hormone combined immune inhibitors are used. Conclusion　Kimura’s disease is a chronic disease，which is a benign lesion but easy to recur. There usually is no malignant change.

Key words: Kimura&rsquo, s disease；nephrotic syndrome；lymph node

摘要：

目的    提高对儿童木村病合并肾病综合征的诊断及治疗水平。方法　对西安市儿童医院肾内科2011—2015年所诊断的3例木村病合并肾病综合征进行病例回顾，同时查找相关国内外文献资料，以了解目前儿童木村病的发病机制、诊断及治疗方案。结果　木村病是一种罕见的以淋巴结、软组织及唾液腺损害为主的慢性免疫炎性疾病，多见于28～32岁的亚洲人群，好发于男性。目前对此病的病因及其发病机制尚不清楚。组织病理是诊断木村病的惟一手段，肾活检作为肾脏疾病诊断的金标准，对于诊断及治疗木村病肾损害有很大帮助。目前的治疗主要依赖药物治疗，一般都需要用激素联合免疫抑制剂。结论　木村病属慢性起病，呈良性病程，但易复发，一般无恶变倾向。

关键词: 木村病, 肾病综合征, 淋巴结