儿童系统性红斑狼疮相关肾小管酸中毒五例临床分析  

Clinical analysis of systemic lupus erythematosus complicated with renal tubular acidosis in five children

作　　者：简珊[1] 伍洁 文煜冰[3] 孙之星[1] 魏骐骄 何艳燕[1] 魏珉[1] Jian Shan;Wu Jie;Wen Yubing;Sun Zhixing;Wei Qijiao;He YanYan;Wei Min(Department of Pediatrics,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儿科,100730 [2]中国医学科学院北京协和医学院临床医学院,100730 [3]中国医学科学院北京协和医学院肾内科,100730

出　　处：《北京医学》2019年第11期1004-1006,共3页Beijing Medical Journal

基　　金：中国医学科学院医学与健康科技创新工程项目（2016-I2M-1-008）;北京协和医学院2017年教学质量工程项目（2017zlgc0104）

摘　　要：目的探讨儿童系统性红斑狼疮(systemic lupus erythematosus, SLE)相关肾小管酸中毒(renal tubular acidosis, RTA)患儿的临床特点。方法回顾性分析2012年1月至2017年6月北京协和医院收治的5例SLE相关RTA患儿的临床资料,并进行文献复习。结果 5例均为女童,均为远端RTA。确诊RTA年龄为7.0~17.1岁,平均(13.4±4.0)岁。随访时间为14~100个月,平均(62.0±33.6)个月。其中3例同时诊断SLE和RTA,1例为先诊断RTA后诊断SLE,另1例先诊断SLE后发现RTA。分析SLE和RTA的相关指标,发现起病时补体C3的水平越低,血碳酸氢盐水平越低,两者呈显著正相关(r=0.917,P <0.05)。2例曾行肾活检,均有肾小管间质改变。在积极治疗原发病同时予纠正酸中毒、补钾治疗;随着SLE病情缓解,RTA病情控制较好。结论病因不明的RTA患儿,尤其是青春期女孩,应注意排查SLE;SLE患儿应注意评估肾小管损害。对于SLE相关RTA患者,治疗原发病是关键,纠正酸中毒及补钾治疗可改善预后。Objective To analysis the clinical features of renal tubular acidosis(RTA) in the children with systemic lupus erythematosus(SLE). Methods The clinical and laboratory data of five pediatric SLE patients with RTA in Peking Union Medical College Hospital from January 2012 to June 2017 were analyzed retrospectively. Results All were girls with distal RTA. The diagnostic age of RTA ranged from 7.0-17.1 years with an average age of(13.4±4.0) years. Three cases were diagnosed as SLE with RTA at the same time, one was diagnosed as RTA before SLE, and the last one was diagnosed as SLE before RTA. It was found that the lower the level of C3, the lower the level of bicarbonate(r = 0.917, P < 0.05). Renal biopsies were performed in two cases, and obvious tubulointerstitial changes were found in both cases. While the primary diseases were treated, the patients also received treatment with citric acid mixture and potassium citrate. The follow-up time ranged from 14-100 months, with an average of(62.0 ± 33.6) months. With the alleviation of SLE, RTA was in remission. Conclusions For children of RTA with unknown etiology, especially adolescent girls, SLE should be considered;and for the patients with SLE, renal tubular damage should be assessed regularly. For the pediatric patients of SLE with RTA, primary disease treatment is important. Acid correcting agent and potassium supplementation are effective.

关 键 词：肾小管酸中毒 系统性红斑狼疮 儿童 

分 类 号：R59[医药卫生—内科学]