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为降低小儿溶血尿毒综合征 (HUS)的病死率 ,回顾性调查北京儿童医院35年 (1966～2000年 )期间HUS年住院患儿概况 ;分析了18例HUS死亡病例的临床表现、主要死因 ;并比较了前25年 (1966～1990年 )与近10年 (1991～2000年 )两个阶段住院患儿的病死率及主要治疗方法。结果1966～1990年共收治28例患儿 ,死亡12例 ,平均病死率高达42.9% ;而在1991～2000年收治的30例患儿中 ,死亡6例 ,平均病死率为20.0%。1997、1998年不仅是HUS发病高峰年 ,患儿数分别为9例、8例 (分别占同期住院的急性肾衰患儿总数的28.0 %、42.9% ) ,而且病死率高。近10年采用甲基泼尼松龙“冲击”配合抗凝疗法及早期实施预防性透析治疗对控制微血管性溶血性贫血和改善肾功能有效。提示35年来HUS呈散发的流行趋势 ,1997、1998年为流行高峰年。本病的主要死因是水中毒及感染。早期诊断、及时透析和针对内皮细胞损伤的发病机制进行治疗是降低本病病死率的有效措施
To reduce the mortality of children with hemolytic uremic syndrome (HUS), we retrospectively survey the hospitalized children with HUS during the 35 years (1966 ~ 2000) of Beijing Children’s Hospital. The clinical manifestations and the main causes of death of 18 HUS death cases were analyzed. The mortality and the main treatment of hospitalized children in the two stages of the first 25 years (1966-1990) and the last 10 years (1991-2000) were compared. Results A total of 28 children were treated from 1966 to 1990, with 12 deaths and an average mortality rate of 42.9%. Among 30 children admitted from 1991 to 2000, 6 died and the average mortality was 20.0%. In 1997 and 1998, it was not only the peak year of HUS but also the number of children was 9 and 8 (28.0% and 42.9% of the total number of acute renal failure hospitalized in the same period respectively), and the case fatality rate was high. Nearly 10 years with methylprednisolone “impact” with anticoagulant therapy and early implementation of prophylactic dialysis treatment of microvascular hemolytic anemia and improve renal function. HUS prompted the trend of 35 years has been distributed, 1997, 1998 is the peak year. The main cause of death of this disease is water poisoning and infection. Early diagnosis, timely dialysis and treatment of endothelial cell damage pathogenesis is an effective measure to reduce the mortality of this disease