摘要目的 探讨儿童抗H因子自身抗体相关性非典型溶血尿毒综合征(aHUS)的临床及病理特点、治疗及预后.方法 纳入2010年11月至2011年5月北京大学第一医院收治的年龄＜18岁、临床符合aHUS诊断标准伴血H因子抗体阳性的4例患儿,其中男1例、女3例,年龄分别为11、5、7、8岁.分析4例患儿临床及病理特点、治疗及疗效.结果 4例患儿均伴有消化道症状如呕吐、腹痛或腹胀,均无腹泻;2例有高血压;1例有惊厥表现;1例既往有aHUS病史.实验室检查4例均有低补体C3血症和H因子自身抗体阳性,其血清H因子抗体滴度分别为1∶7068、1∶1110、1∶174、1∶869;其中3例发病时血清H因子水平低于正常(38.0、88.4、209.4 mg/L).3例行肾脏病理检查均符合血栓性微血管病表现.4例均给予激素联合霉酚酸酯治疗,2例联合血浆置换治疗.随访时间8 ～ 29个月,4例肾功能均恢复,蛋白尿均明显缓解,4例血清H因子抗体滴度分别降至1∶119、1∶170、1∶123和1∶674;3例血清低H因子水平者均较前恢复(405.8、155.8、438.4 mg/L).结论 本组H因子抗体相关aHUS患儿消化道症状常见,低补体血症突出,激素联合霉酚酸酯治疗有助于恢复,血清H因子抗体滴度动态监测有助于诊断和治疗.

abstractsObjective To investigate the clinical characteristics,renal pathology,treatment and prognosis of children with atypical hemolytic uremic syndrome associated with H factor antibody.Method Four children less than 18 yr of age admitted from Nov.2010 to May 2011 in Peking University First Hospital were included.They all met the criteria for atypical hemolytic uremic syndrome and with positive serum anti factor H antibody.They aged from 5 to 1 1 yr.Data on clinical manifestations,renal pathology,treatment and prognosis were analyzed.Result All of the 4 cases had gastrointestinal symptoms such as vomiting,abdominal pain,or abdominal distension.None of them had diarrhea.Two children had hypertension.One child had episodes of convulsion.One child had history of atypical hemolytic uremic syndrome.All of them had low serum complement C3.Three of them had low serum factor H (38.0,88.4,209.4 mg/L).All of them had serum antibody to factor H (1 ∶ 7 068,1 ∶ 1 l 10,1 ∶ 174,and 1 ∶ 869).Three of them received renal biopsy,all of them showed thrombotic microangiopathy.All of them were treated with steroid combined with mycophenolate mofetil.Two children received plasma exchange.They were followed up for 8 to 29 months.The renal function became normal and proteinuria relieved in all of them.The serum factor H concentration increased to 405.8,155.8 and 438.4 mg/L,respectively.The titer of anti factor H antibody decreased to 1 ∶ 119,1 ∶ 170,1 ∶ 123,and 1 ∶ 674,respectively.Conclusion Gastrointestinal symptom is common in children with atypical hemolytic uremic syndrome associated with H factor antibody.Hypocomplementemia was observed in all of them.Steroid combined with mycophenolate mofetil seemed to be effective for them.The monitoring of serum factor H and antibody to factor H may help diagnosis and treatment.