Kimura病并发肾病综合征临床特征  

Clinical characteristics of Kimura disease associated with nephrotic syndrome

作　　者：张雪晗 马杰[2] 焦洋[3] ZHANG Xue-han;MA Jie;JIAO Yang(Department of Health Care,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Nephrology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of General Internal Medicine,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院保健医疗部,北京100730 [2]中国医学科学院北京协和医学院北京协和医院肾内科,北京100730 [3]中国医学科学院北京协和医学院北京协和医院全科医学科(普通内科),北京100730

出　　处：《中华临床免疫和变态反应杂志》2021年第3期270-274,共5页Chinese Journal of Allergy & Clinical Immunology

基　　金：美国中华医学基金会公开竞标项目(20-384)。

摘　　要：目的总结Kimura病并发肾损害患者的临床特征,以提高临床医师对此疾病的认识。方法收集北京协和医院1980年1月至2020年6月确诊的Kimura病并发肾脏受累患者的临床资料,回顾性分析其流行病学特点、临床表现、实验室检查、诊治及转归。结果6例Kimura病患者中男性4例,女性2例,确诊Kimura病年龄14~52岁。1例患者同时出现肿块和蛋白尿,其余5例患者发现肿块早于出现蛋白尿,间隔时间2~48月(中位7.5月)。6例患者均表现为肾病综合征,24 h尿蛋白定量4.05~20.4 g(中位9.4 g),其中1例患者出现急性肾损伤。6例患者中5例行肾脏穿刺活检,病理分别为微小病变肾病(3例)和膜性肾病(2例)。所有患者经激素治疗后病情缓解。结论Kimura病累及肾脏出现肾病综合征可导致肾功能受损,甚至进展为终末期肾病。故应积极治疗,糖皮质激素可作为首选治疗药物。Objective To explore the clinical features of KD associated with nephrotic syndrome.Methods The clinical data of 6 patients with Kimura disease(KD)associated with nephrotic syndrome diagnosed in Peking Union Medical College Hospital from January 1980 to June 2020 were retrospectively analyzed.Results Of the 6 patients,there were 4 men and 2 women.The ages of diagnosis with KD were from 14 to 52 years.The mass preceded the renal manifestations in 5 patients,with a median interval of 7.5 months(2 to 48 months).In 1 patient,the symptoms occurred simultaneously.All 6 patients presented with nephrotic syndrome.The daily protein excretions varied from 4.05 to 20.4 g(median 9.4 g)per day.Serum creatinine level was elevated in 1 patient.Renal biopsies were carried out in 5 patients.The results demonstrated that 3 patients had minimal change disease,while the other 2 had membrane nephropathy.All patients recovered after treatment with oral steroids.Conclusions KD associated with nephrotic syndrome may progress to renal insufficiency and end-stage renal disease.Steroids could be a suitable choice of treatment for KD patients associated with nephrotic syndrome.

关 键 词：KIMURA病 蛋白尿 肾病综合征 肾功能受损 

分 类 号：R69[医药卫生—泌尿科学]