论文部分内容阅读
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。
Objective To investigate the efficacy of combined plasma and small dose hormone therapy for hemolytic uremic syndrome (HUS). Methods The clinical data of 11 HUS children with nephrology in our hospital during the past 5 years were retrospectively analyzed. 6 males and 5 females; aged 2 to 9 years, mean age 5.2 years. Severe in 10 cases, mild cases in 1 case. Eight of them were treated with plasma exchange, two or three times in each case. Postoperatively, they were maintained with prednisone (1.0-1.5 mg.kg-1) or methylprednisolone (1 mg.kg-1) Dialysis treatment, 1 case of combined continuous hemofiltration treatment. 1 case of mild disease using high-dose gamma globulin (C-ball) treatment. One case of severe HUS outside the hospital for hemodialysis more than 1 month transfer to continue hemodialysis hospital. One case of severe HUS outside the hospital using high-dose c-ball impact with methylprednisolone treatment continued methylprednisolone 1 mg.kg-1 treatment and intestinal dialysis. Results All the 19 cases of plasmapheresis were successfully performed with no obvious complication. Among the 8 patients who underwent plasmapheresis and small-dose steroid treatment, 7 cases of liver enzymes returned to normal with normal myocardial enzymes and renal function, Degree of urinary albuminuria was discharged from the hospital and was followed up for 2 to 26 months. Renal function was normal and urinary protein was negative. Five patients had mild hematuria [RBC (9 ~ 36) × 106L-1], one microscopic hematuria , 1 case of normal urine. 1 case of mild urinary routine urinary discharge and renal function were normal, outpatient follow-up of 38 months urine normal. 2 cases of severe hospital outpatient HUS admitted to undergraduate course of disease duration of 1 week to 1 month, platelets have returned to normal, Hb no further decline in urinary routine showed gross hematuria and moderate proteinuria in renal failure. One case of hemodialysis 6 times, slightly improved renal function, gross hematuria and proteinuria, give up treatment and discharge. 1 case of intestinal dialysis and oral hormones and other measures to give up treatment and discharge. Conclusions The patients with severe HUS should be treated with plasma exchange early and plasma combined with small dose of hormone therapy can improve the prognosis and reduce the sequelae of severe HUS.