血浆置换治疗儿童溶血尿毒综合征多中心流行病学调查

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)×109/L]、血小板计数[(77.49 ± 72.93)×109/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)×109/L、(183.80 ± 131.95)× 109/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,有效性和安全性已得到证实,其治疗时机有待循证医学进一步证实.%Objective To 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 diagno-sis and treatment.Methods The 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 anu-ria(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)×109/L],platelet count[(77.49 ± 72.93)×109/L],C3[(0.70 ± 0.26)g/L],glomeru-lar 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 pro-tein [(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 )×109/L,(183.80 ± 131.95 )×109/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,respectively],and the differences were all statistically signifi-cant (all P＜0.05).(4)Complications occurred in 5 cases,including hypotension shock (2 cases),blockage of pipe (1 case),thrombocytopenia (1 case),anemia (1 case).(5)After TPE,130 cases (89%)were significantly effective, but 16 cases (11%)died.Conclusions TPE for children with HUS has been widely carried out in China,and its effectiveness and safety had been confirmed.The timing of treatment is further confirmed by evidence-based medicine.