血浆置换治疗儿童溶血尿毒综合征多中心流行病学调查  被引量:6

A multi- center epidemiological investigation on therapeutic plasma exchange for the children with hemolytic uremic syndrome

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作  者:中国医师协会儿科医师分会血液净化专家委员会 郑悦[2] 徐可[3] 雷晔飞[4] 冯仕品 王墨[6] 郝志宏[7] 高岩 李宇红 周萍[10] 肖政辉[11] 赵凯妹 夏正坤[13] 栾春丽 刘雪梅 赵丽君[16] 陆国平 张育才[18] 陈朝英[19] 栾江威 张建江[21] 刘翠华[22] 陈丽植[23] 赵成广[2] 吴玉斌[2] 姚勇[3] 曲强 谢敏 刘喜 万俊丽[6] 焦佳[6] 石咏琪 杨琴[6] 于力[7] 张瑶[7] 李颖杰 许自川 蒋新辉 邵晓珊 张思佳[10] 卢秀兰[11] 张岩[12] 马青山[12] 何旭[13] 史卓[13] 李倩[14] 孙书珍[14] 张洪霞 潘艳艳 仇三玲 蔡小狄 徐虹 朱艳[18] 史婧奕[18] 耿海云[19] 祝高红 戚畅 贾莉敏[21] 刘俊梅[21] 厉洪江 李广波 蒋小云[23] Pediatric Blood Purification Specialist Committee of Pediatric Association in Chinese Medical Doctor Association;Zheng Yue;Xu Ke;Lei Yefei;Feng Shipin;Wang Mo;Hao Zhihong;Gao Yan;Li Yuhong;Zhou Ping;Xiao Zhenghui;Zhao Kaishu;Xia Zhengkun;Luan Chunli;Liu Xuemei;Zhao Lijun;Lu Guoping;Zhang Yucai;Chen Chaoying;Luan Jiangwei;Zhang Jianjiang;Liu Cuihua;Chen Lizhi;Zhao Chengguang;Wu Yubin;Yao Yong;Qu Qiang;Xie Min;Liu X i;Wan Junli;Jiao Jia;Shi Yongqi;Yang Qin;Yu Li;Zhang Yao;Li Yingjie;Xu Z ichuan;Jiang Xinhui;Shao X iao-shah;Zhang Sijia;Lu Xiulan;Zhang Yah;Ma Qingshan;He Xu;Shi Zhuo;Li Qian;Sun Shuzhen;Zhang Hongxia;Pan Yanayan;Qiu Sanling;Cai Xiaodi;Xu Hong;Zhu Yan;Shi Jingyi;Geng Haiyun;Zhu Gaohong;Qi Chang;Jia Limin;Liu Junmei;Li Hongjiang;Li Guangbo;Jiang Xiaoyun(Department of Pediatric Nephrology and Rheumatology,Shengfing Hospital of China Medical University,Shenyang 110004,China;Department of Pediatrics,Peking University First Hospital,Beijing 100034,Chin;Department of Pediatrics,Chenzhou First People's Hospital,Chenzhou 423000,Hubei Province,China;Department of Pediatric Nephrology,Chengdu Women and Children's Center Hospital,Chengdu 610091,Chin;Department of Nephrology and Immunology,Children's Hospital of Chongqing Medical University,Chongqing 400014,Chin;Department of Pediatrics,Guangzhou First People's Hospital,Guangzhou 510180,China(Hao ZH,Yu L,Zhang Y;Department of Nephrology,Guangzhou Women and Children's Medical Center,Guangzhou 510120,Chin;Department of Nephrorheumatism,Guiyang Children's Hospital,Guiyang 550003,China(Li YH,Jiang XH,Shao XS;Department of Pediatrics,the Second Hospital of Ha' erbin Medical University,Ha'erbin 150086,Chin;Emergency Center,Hunan Children's Hospital,Changsha 410007,China;epartment of Pediatric Nephrology,Be-thune First Hospital of Jilin University,Jilin 130021,Chin;epartment of Pediatrics,Nanjing General Hospital of Nanjing Military Region,Nanjing 210002,Chin;epartment of Pediatric Nephrology and Rheumatology,Provincial Hospital of Shandong Universi

机构地区:[1]不详 [2]中国医科大学附属盛京医院小儿肾脏风湿免疫科,沈阳110004 [3]北京大学第一医院儿科,100034 [4]郴州市第一人民医院儿科,湖南郴州423000 [5]成都市妇女儿童中心医院肾内科,610091 [6]重庆医科大学附属儿童医院肾脏免疫科,400014 [7]广州市第一人民医院儿科,510180 [8]广州市妇女儿童医疗中心肾内科,510120 [9]贵阳市儿童医院肾脏风湿科,550003 [10]哈尔滨医科大学附属第二医院儿科,150086 [11]湖南省儿童医院急救中心,长沙410007 [12]吉林大学白求恩第一医院儿童肾病科,吉林130021 [13]南京军区南京总医院儿科,210002 [14]山东大学附属省立医院儿科肾脏风湿免疫科,济南250021 [15]山东大学齐鲁儿童医院肾内科,济南250022 [16]山西省儿童医院肾内科,太原030013 [17]复旦大学附属儿科医院重症医学科,上海201102 [18]上海市儿童医院重症医学科,200042 [19]首都儿科研究所附属儿童医院肾内科,100020 [20]武汉儿童医院肾内科,430016 [21]郑州大学第一附属医院儿内科,450052 [22]郑州大学附属儿童医院肾脏风湿科,450018 [23]中山大学附属第一医院儿科,广州510080

出  处:《中华实用儿科临床杂志》2018年第13期1022-1027,共6页Chinese Journal of Applied Clinical Pediatrics

摘  要:目的了解全国儿科血浆置换治疗溶血尿毒综合征(HUS)的现状,为进一步规范诊疗共识提供参考依据。方法中国医师协会儿科医师分会血液净化专家委员会讨论并制定调查表,以问卷调查的形式,收集22家单位2012年1月至2017年12月住院的146例行血浆置换的HUS患儿的病历资料进行统计学分析。结果1.共收集数据146例,其中男91例,女55例;平均年龄5.68岁(2个月~18岁);病因主要为感染(60例,41.1%),其次为补体缺陷(18例,12.3%)、药物(15例,10.3%)、风湿性疾病(9例,6.2%)及遗传代谢病(6例,4.1%)等。2.血液置换前患儿主要表现为少尿或无尿(101例),其次为意识障碍(45例)、高血压(58例)、消化道出血(42例)等症状,置换后明显缓解。3.血浆置换前实验室指标主要表现为血红蛋白[(68.26±18.17) g/L]、红细胞计数[(2.43±0.70) ×10^9/L]、血小板计数[(77.49±72.93) ×10^9/L]、补体C3[(0.70±0.26) g/L]、肾小球滤过率(GFR) [(26.98±17.16) mL/1.73 m2]下降,总胆红素[(44.33±42.07) μmol/L]、非结合胆红素[(30.78±26.68) μmol/L]、纤维蛋白降解产物(FDP)[(12.52±14.97) mg/L]、D-二聚体[(2 306.20±2 354.97) μg/L]、肌酐[(305.45±290.19) μmol/L]、24 h尿蛋白[(2.17±2.55) g/d]升高,置换后以上指标均显著好转[分别为(85.08±18.98) g/L、(2.80±0.98)×10^9/L、(183.80±131.95)×10^9/L、(0.85±0.25) g/L、(98.58±66.02) mL/1.73 m2、(19.71±33.24) μmol/L、(9.60±10.00) μmol/L、(7.33±9.81) mg/L、(1 322.05±1 595.23) μg/L、(139.28±163.75) μmol/L、(1.46±2.03) g/d],差异均有统计学意义(均P〈0.05)。4.出现并发症5例,2例低血压休克,管路堵塞、血小板减少、贫血加重各1例。5.经治疗后好转130例(89%),死亡16例(11%)。结论血浆置换治疗儿童HUS在国内已较广泛开展,ObjectiveTo study the current status of therapeutic plasma exchange(TPE) for the children with hemolytic uremic syndrome(HUS) in China, and to provide evidence for further standardizing the consensus of diagnosis and treatment.MethodsThe questionaires were designed after discussion by Pediatric Blood Purification Specialist Committee to investigate the data of 146 hospitalized HUS children with TPE, which were collected and analyzed from January 2012 to December 2017 in 22 units.Results(1)Among the 146 cases, there were 91 males and 55 females, with an average age of 5.68 years old (2 months to 18 years old). The causes included infection(41.1%, 60 cases), complement defects(12.3%, 18 cases), drugs(10.3%, 15 cases), rheumatic diseases(6.2%, 9 cases)and hereditary metabolic diseases(4.1%, 6 cases). (2)Before TPE, the main manifestations of the sick children were oliguria or anuria(101 cases), and disturbance of consciousness(45 cases), hypertension(52 cases), and gastrointestinal bleeding(42 cases), which were significantly relieved after TPE.(3)Before TPE, hemoglobin[(68.26±18.17) g/L], red blood cell count[(2.43±0.70) ×10^9/L], platelet count[(77.49±72.93) ×10^9/L], C3[(0.70±0.26) g/L], glomerular filtration rate(GFR)[(26.98±17.16) mL/1.73 m2]were all decreased, and total bilirubin[(44.33±42.07) μmol/L], unconjugated bilirubin [(30.78±26.68) μmol/L], fibrin breakdown products(FDP)[(12.52±14.97) mg/L], D-dimer[(2 306.20±2 354.97) μg/L], creatinine[(305.45±290.19) μmol/L], 24-hour urinary protein [(2.17±2.55) g/d] all increased, the above indicators were significantly improved after the TPE[ (85.08±18.98) g/L, (2.80±0.98) ×10^9/L, (183.80±131.95) ×10^9/L, (0.85±0.25) g/L, (98.58±66.02) mL/1.73 m^2, (19.71±33.24) μmol/L, (9.60±10.00) μmol/L, (7.33±9.81) mg/L, (1 322.05±1 595.23) μg/L, (139.28±163.75) μmol/L, (1.46±2.03) g/d, res

关 键 词:溶血尿毒综合征 血浆置换 儿童 流行病学 

分 类 号:R512.6[医药卫生—内科学]

 

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