血浆置换术后联合小剂量激素治疗溶血尿毒综合征

目的探讨血浆置换术后联合小剂量激素治疗溶血尿毒综合征(HUS)的疗效。方法回顾性分析近5 a来本院肾内科11例HUS患儿临床资料。男6例,女5例;年龄2～9岁,平均年龄5.2岁。重症10例,轻症1例。其中8例进行血浆置换治疗,每例2、3次,术后应用泼尼松(1.0～1.5 mg.kg-1)或甲泼尼龙(1 mg.kg-1)维持;其中1例联合血液透析治疗,1例联合连续性血液滤过治疗。轻症1例采用大剂量丙种球蛋白(丙球)治疗。1例重症HUS外院进行血液透析1个月余转本院继续血液透析治疗。1例重症HUS外院采用大剂量丙球冲击联合甲泼尼龙治疗后继续甲泼尼龙1 mg.kg-1治疗和肠道透析。结果 19例次血浆置换治疗均顺利实施,无明显并发症;8例血浆置换后联合小剂量激素治疗者中7例肝酶、心肌酶、肾功能恢复正常,尿常规镜下血尿或并轻中度蛋白尿出院,追踪观察2～26个月,复查肾功能均正常,尿蛋白阴性,5例镜下轻微血尿[RBC(9～36)×106L-1],1例感染后轻微镜下血尿,1例尿常规正常。轻症1例出院时尿常规和肾功能均正常,门诊随诊38个月尿常规正常。2例外院治疗的重症HUS患儿转入本科时病程1周～1个月,血小板已恢复正常,Hb无继续下降,尿常规示肉眼血尿和中量蛋白尿,处于肾衰竭期。其中1例血液透析6次,肾功能稍好转、肉眼血尿并中量蛋白尿,放弃治疗出院。1例经肠道透析和口服激素等措施后放弃治疗出院。结论重症HUS患儿宜早期应用血浆置换治疗,血浆置换治疗后联合小剂量激素治疗可改善重症HUS患儿预后,减少后遗症。

Effect of Piasmapheresis and Succedent Small Dosage of Hormonal Therapy for Haemolytic Uraemic Syndrome in Children

Objective To investigate the effect of plasmapheresis(PE) and succedent small dosage of hormonal therapy on haemolytic uraemic syndrome(HUS) in children.Methods Clinical data of hospitalized children with HUS in Department of Nephrology,Guangzhou Children’s Hospital,over the last 5 years were retrospectively analyzed.Of the 11 patients,with an average age of 5.2(range 2-9) years old,6 male and 5 female,10 cases were severe and 1 case was mild.Eight patients received PE 2 or 3 times respectively and succedent small dosage of hormonal therapy including 1 mg·kg-1 methylprednisolone or 1.0-1.5 mg·kg-1 prednisone.Of these patients,1 case received PE combined with hemodialysis(HD),1 case received PE combined with continuous veno-venous hemofiltration.One patient with mild haemolytic uraemic syndrome was treated by large dosage of intravenous immunoglobulin.One severe patient who received HD for more than 1 month in other hospital continued to receive HD in our hospital.One severe case who received large dosage of intravenous immunoglo-bulin and methylprednisolone in other hospital was treated by methylprednisolone and intestinal dialysis in our hospital.Results Nineteen times PE completed successfully without obvious complications.Of 8 patients who were treated by PE and succedent small dosage of hormonal therapy,7 patients discharged with liver function,heart function and renal function recovered and mild glass hematuria and mild proteinuria.The patients were followed-up for 2-26 months.During follow-up,all patients had normal renal function and negative proteinuria,5 cases had hematuria [red blood cell was(9-36)×106 L-1],1 case had slight hematuria only after infection,1 case had normal urine routine.One mild case discharged with normal renal function and urine routine,then followed-up for 38 months still had normal urine routine.The course of disease of 2 severe cases treated in other hospitals were more than 1 week when they were transferred to our hospital.They had gross hematuria and middle proteinuria and renal failure,normal platelet and hemoglobin.One case gave up and discharged with remissive gross hematuria,middle proteinuria and improved renal function after he received hemodialysis 6 times.One case gave up and discharged after he received methylprednisolone and intestinal dialysis therapy.Conclusions Severe HUS should receive PE early.PE and succedent small dosage of hormonal therapy can improve the prognosis and reduce the sequelae of HUS.