儿童原发性肾病综合征合并肾上腺危象15例临床分析  

Clinical analysis of 15 children with primary nephrotic syndrome complicated with adrenal crisis

作　　者：孙智才[1] 刘玉玲[1] 李小琳[1] 潘晓芬[1] SUN Zhicai;LIU Yuling;LI Xiaolin;PAN Xiaofen(Department of Pediatrics,Boai Hospital of Zhongshan Zhongshan 528403,Guangdong,China)

机构地区：[1]广东省中山市博爱医院儿科,广东中山528403

出　　处：《临床儿科杂志》2023年第8期610-612,共3页Journal of Clinical Pediatrics

基　　金：中山市社会公益科技研究项目(No.2019B1010)。

摘　　要：目的探讨儿童原发性肾病综合征(PNS)合并肾上腺危象(AC)的临床特征及预后。方法对2010年1月至2022年3月就诊的原发性肾病综合征合并肾上腺危象患儿的临床资料进行回顾分析。结果15例患儿平均年龄(6.25±1.81)岁(4.75~12岁),其中男9例,女6例;病程(2.74±1.33)年(1~6年)。诱发因素为糖皮质激素减量或停药8例,呼吸道感染5例,2例无明显诱因。临床表现为腹痛、恶心、呕吐等消化道症状11例,低血压、少尿等循环系统衰竭表现7例,低钠血症8例,高钾血症4例,低血糖2例,所有患儿均伴有不同程度神经系统症状(头晕、乏力、精神萎靡或烦躁等),9例伴有消化道或休克早期症状。患儿随机血清皮质醇(37.6±15.7)nmol/L(15.3~52.7nmol/L),6例清晨8点空腹血清皮质醇(56.3±18.9)nmol/L(21.6~73.4 nmol/L)。所有患儿经静脉氢化可的松治疗2~3天后症状均得到有效控制,随访至2022年6月,11例PNS痊愈,4例PNS仍在治疗中,无AC再发病例。结论PNS合并AC多因激素减停或感染发病,以消化、循环系统症状为主要表现,早期识别、及时治疗预后良好。Objective To investigate the clinical characteristics and prognosis of primary nephrotic syndrome(PNS)complicated with adrenal crisis(AC)in children.Methods Clinical data of 15 patients with primary nephrotic syndrome complicated with adrenal crisis in the department of Pediatrics,Boai Hospital,Zhongshan City from January 2010 to March 2022 were retrospectively analyzed.Results The average age of the patients was 6.25±1.81 years old(4.75-12 years old),including 9 males and 6 females.The course of disease was 2.74±1.33 years(1-6 years).Inducement:Glucocorticoid dose reduction or withdrawal in 8 cases,respiratory tract infections in 5 cases,and no obvious triggers in 2 cases.Clinical manifestations:Gastrointestinal symptoms such as abdominal pain,nausea,and vomiting were found in 11 cases,circulatory failure manifestations such as low blood pressure and oliguria in 7 cases,hyponatremia in 8 cases,hyperkalemia in 4 cases,and hypoglycaemia in 2 cases,All the children were accompanied by neurological symptoms of varying degrees(such as dizziness,debilitation,malaise or irritability,etc.).Serum cortisol:Random serum cortisol level of 9 children with digestive tract or early shock symptoms was 37.6±15.7 nmol/L(15.3-52.7 nmol/L),fasting serum cortisol level was 56.3±18.9 nmol/L(21.6-73.4 nmol/L)at 8 am in 6 patients.Treatment and prognosis:Symptoms were effectively controlled in all children after 2-3 days of intravenous hydrocortisone treatment.After follow-up to June 2022,11 cases of PNS were cured,4 cases of PNS were still under treatment,and there was no recurrence of AC.Conclusion Primary nephrotic syndrome in children with adrenal crisis is mainly caused by hormone abatement or infection,and the symptoms of digestive and circulatory system are the main manifestations.Early identification and timely treatment have a good prognosis.

关 键 词：原发性肾病综合征 肾上腺危象 临床特征 预后 儿童 

分 类 号：R726.9[医药卫生—儿科]