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目的探讨儿童溶血尿毒综合征(HUS)患儿的临床特点,观察血浆置换在儿童HUS中的治疗效果和随访。方法研究对象为2001年1月至2007年11月在中国医科大学附属盛京医院儿肾科住院的9例HUS患儿,分析其首发症状和实验室检查,药物治疗经过,血浆置换或联合血液透析治疗的效果,病情好转后口服糖皮质激素治疗的转归及随访(0.5～6年)结果。结果腹泻相关的HUS(D+)和非典型HUS(D-)患儿临床特点和实验室检查无明显差异;在综合药物治疗不能缓解病情时应早期进行血浆置换,一般置换1次可以阻止病情进展,重症增加1次并联合血液透析可取得较好疗效;序贯口服糖皮质激素有助于降低尿蛋白,促进肾脏损伤的恢复。随访中1例出现蛋白尿,重复肾活检出现肾小管间质损伤和免疫物质增多;1例出现高血压。结论血浆置换治疗小儿HUS临床疗效显著,序贯口服糖皮质激素有助于降低尿蛋白,HUS患儿的长期随访需引起儿肾科医生的关注。
Objective To investigate the clinical features of pediatric patients with hemolysis-uremic syndrome (HUS) and observe the therapeutic effect and follow-up of plasma exchange in children with HUS. Methods The subjects were 9 HUS children hospitalized in Department of Pediatrics, Shengjing Hospital Affiliated to China Medical University from January 2001 to November 2007, and analyzed their initial symptoms and laboratory tests, drug treatment, plasma exchange or combined blood Dialysis treatment, the condition improved after oral glucocorticoid treatment and follow-up (0.5-6 years) results. Results The clinical features and laboratory tests of children with HUS (D +) and atypical HUS (D-) associated with diarrhea had no significant difference. Patients should be treated with plasma exchange in the early stage when the comprehensive medical treatment can not relieve the disease, and the general replacement can prevent the progression of the disease , Severe increase of 1 and combined hemodialysis can achieve better effect; sequential oral glucocorticoid helps to reduce urinary protein, promote the recovery of kidney injury. In one case, proteinuria occurred during follow-up, tubulointerstitial injury and immunologic substance increased in repeated renal biopsy, and hypertension occurred in 1 case. Conclusion The clinical effect of plasma exchange in the treatment of pediatric HUS is significant. Sequential oral administration of glucocorticoids can reduce urinary protein. The long-term follow-up of children with HUS needs attention of pediatric nephrologists.