摘要
目的分析儿童非典型溶血尿毒综合征(aHUS)临床特点及治疗情况。方法回顾性分析19例aHUS患儿的临床资料,包括临床表现、实验室检查、辅助检查、治疗及预后。结果患儿首发症状有贫血、皮肤出血点、咳嗽、发热、尿色异常、肾功能异常、血小板减少和抽搐等。19例患儿均有血红蛋白、血小板计数减少,乳酸脱氢酶(LDH)、尿素氮、肌酐升高,且有蛋白尿。18例患儿间接胆红素升高,17例患儿补体C3下降。19例骨髓检查显示骨髓增生活跃,红系明显增生,巨核细胞和血小板均减少。2例患儿肾穿刺病理结果提示血栓性微血管病变。19例患儿行血浆置换和糖皮质激素治疗,其他治疗包括输注洗涤红细胞纠正贫血、输注血小板、补液纠正电解质紊乱和抗感染等。18例患儿治疗后血小板计数升至正常范围,血红蛋白有明显好转,肾功能改善,LDH降至正常,补体升至正常。结论 aHUS患儿往往表现为多系统损害,容易误诊、漏诊,多数伴有补体C3下降。血浆置换联合激素治疗对aHUS有一定的疗效,能改善患儿的预后。
Objective To analyze the clinical characteristics and treatment of children with atypical hemolytic uremic syndrome(aHUS).Methods The clinical data of 19 children with aHUS were retrospectively analyzed,which included clinical characteristics,laboratory examination,auxiliary examination,treatment and prognosis.Results The initial symptoms of aHUS children were anemia,bleeding spots,cough,fever,abnormal urine color,abnormal renal function,thrombocytopenia and convulsion.The hemoglobin and platelet count were decreased,lactate dehydrogenase(LDH),urea and creatinine were increased,and albuminuria was seen in 19 cases.The indirect bilirubin was higher in 18 cases and complement C3 was lower in 17 cases.The bone marrow biopsy was performed in 19 cases,which showed that bone marrow hyperplasia was active,erythroid hyperplasia was obvious and megakaryocytes and platelets were decreased.The renal biopsy was performed in 2 cases,which showed thrombotic microangiopathy.The aHUS children were treated with plasma exchange and glucocorticoid.Other treatments included transfusion of red blood cells to correct anemia,transfusion of platelets,rehydration to correct electrolyte disorders,and anti-infection.After treatment,the platelet count of 18 cases was increased to normal range,hemoglobin and renal function were improved,LDH was decreased to normal and complement C3 was increased to normal.Conclusion The aHUS children often show multiple system damage and easy to be misdiagnosed and missed diagnose.Most aHUS children are with reduced complement C3 level.Plasma exchange combined with glucocorticoid therapy has a certain effect on aHUS,which can improve the prognosis of children.
作者
杨焕丹
张锐锋
彭倩倩
邱闪
周苏芹
YANG Huandan;ZHANG Ruifeng;PENG Qianqian(Department of Renal Rheumatoimmunology,Affiliated Xuzhou Children’s Hospital,Xuzhou Medical University,Xuzhou 221000,CHINA)
出处
《江苏医药》
CAS
2021年第6期611-613,618,共4页
Jiangsu Medical Journal
关键词
非典型溶血尿毒综合征
儿童
Atypical hemolytic uremic syndrome
Children
