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1955年Gassez氏首次报告,在幼儿溶血性贫血和血小板减少症时,发生进行性的急性肾功衰竭。后来将该综合征称作溶血尿毒综合征(HUS)。目前,本病尚无特效疗法,病死率达50~60%。作者观察27例住院(血液透析科)的HUS患儿,其中男14例,女13例,24例年龄在3岁以下。检查了全部患儿急性期的止血系统改变(在住院后治疗开始前第1小时进行),并以18例同龄正常儿作为对照。全部患儿入院时均处于急性肾功衰竭的无尿期,持续无尿3~7天,全部诊断为HUS重型。
Gassez’s first report in 1955 showed progressive acute renal failure in young children with hemolytic anemia and thrombocytopenia. This syndrome was later referred to as hemolytic uremic syndrome (HUS). At present, there is no effective treatment for this disease, the mortality rate is 50 ~ 60%. The authors looked at 27 HUS children hospitalized (hemodialysis) with 14 males and 13 females, 24 under the age of 3. The hemostatic changes were examined in all children at acute stage (1 hour before hospitalization) and 18 normal children of the same age as controls. All children admitted to the hospital were in an anuria of acute renal failure, continued anuria for 3 to 7 days, all diagnosed as HUS heavy.