非典型溶血尿毒综合征28例临床分析

Clinical analysis of 28 cases of atypical hemolytic uremic syndrome

目的探讨非典型溶血尿毒综合征（aHUS）急性期治疗方法及预后影响因素。方法回顾性分析首都医科大学附属北京儿童医院2011年1月至2015年3月收治的aHUS患儿28例，总结治疗前后病情转归。结果患儿给予血浆置换（PE）治疗，21例患儿予血液透析（HD）治疗，13例H因子（FH）抗体阳性患儿辅予激素治疗，1例患儿联合霉酚酸酯治疗，病程3个月时评价患儿各项指标，28例患儿中完全缓解13例；好转14例，其中6例合并高血压，4例出现肾功能减退。1例肾功能未恢复，未脱离HD。无死亡患儿。统计学分析结果显示遗留肾损害组与完全缓解组急性期血肌酐水平分别为（430.0±124.7） μmol/L与（265.6±90.8） μmol/L，2组比较差异有统计学意义（P〈0.01）；2组FH水平分别为（206.5±134.9） mg/L和（415.4±214.1） mg/L，差异有统计学意义（P〈0.01）。统计分析结果显示2组患儿年龄、血小板、补体C3、补体C4、血红蛋白及乳酸脱氢酶等差异均无统计学意义（P均〉0.05）。抗体阳性组血肌酐（346.4±112.5） μmol/L，阴性组血肌酐（297.0±89.3） μmol/L；抗体阳性组FH（307.4±187.3） mg/L，阴性组FH（379.8±203.5） mg/L，2组比较差异均无统计学意义（P均〉0.05）。结论PE为aHUS急性期的有效治疗手段，抗FH抗体阳性的患儿使用激素及免疫抑制剂治疗有效。急性期肾损伤严重者，遗留长期肾损伤的可能性大，血清FH水平低者，提示预后较差。

Objective To investigate the treatment of atypical hemolytic uremic syndrome （aHUS） in their acute phase and relate the prognostic factors.Methods Twenty-eight patients with aHUS treated in Department of Nephrology, Beijing Children′s Hospital Affiliated to Capital Medical University from January 2011 to March 2015 were collected, the outcomes of the treatment were summarized.Results All the patients were given plasma exchange （PE） treatment, 21 patients with hemodialysis （HD） treatment; 13 patients with H factor （FH） antibody were further treated with corticosteroid therapy, and 1 patient was treated with the combination of mycophenolate mofetil.Evaluation of patients′ indices were conducted at month 3.Out of the 28 patients, there were 13 patients with complete remission; of the 14 patients showed improvement, 6 patients were complicated with hypertension and 4 patients with renal dysfunction.One patient, with ongoing hemodialysis, did not show restoration of renal function.There was no death in children.Statistical analysis showed that the acute phase serum creatinine levels were （430.0±124.7） μmol/L and （265.6±90.8） μmol/L for the group with remaining kidney injury and the group with complete remission, respectively, the difference was statistically significant（P〈0.01）. The levels of FH for the 2 groups were （206.5±134.9） mg/L and（ 415.4±214.1） mg/L, and the difference was statistically significant（P〈0.01） as well.Statistical analysis showed that diffe-rences were no statistically significant between the 2 groups of patients on their age, platelets, complement C3, complement C4, hemoglobin and lactate dehydrogenase （all P〉0.05）. For the group with positive antibody, its average serum creatinine was （346.4±112.5） μmol/L.For the group with negative antibody, its average serum creatinine was（297.0±89.3） μmol/L.The FH for group with positive antibody and group with negative antibody were （307.4±187.3） mg/L and （379.8±203.5） mg/L, respectively.The differences were not statistically significant（all P〉0.05）.Conclusions PE is an effective treatment for aHUS at acute phase, and corticosteroid and immunosuppressive therapy are effective for patients with positive anti-FH antibody.For patients with severe kidney injury at acute phase, the possibility of long-term kidney damage is high.Low concentration of serum FH indicates a poor prognosis.