Blättler W, Wegmann W, Herold H, Straub P W
Schweiz Med Wochenschr. 1975 Dec 20;105(51):1773-4.
The findings are reported in 4 siblings in whom death was due to typical hemolytic-uremic syndrome. In one, successive treatment with heparin, dipyridamole and aspirin was unsuccessful. 125I-fibrinogen half-life was 2.1 days under aspirin and 1.9 days under combined aspirin/heparin therapy. The parents and 4 surviving sibs had normal renal function and normal platelet and fibrinogen survival. The mother and 3 sibs had an increased percentage of megathrombocytes. Two exhibited renal accumulation of 51Cr-platelet radioactivity which was suppressed by platelet inhibitors. In both, renal biopsy disclosed definite ultrastructural endothelial alterations. These changes are probably responsible for the local platelet sequestration and appear to be the primary defect in this family with hemolytic-uremic syndrome.
研究结果报告于4名死于典型溶血性尿毒症综合征的兄弟姐妹。其中1例，先后使用肝素、双嘧达莫和阿司匹林治疗均未成功。服用阿司匹林时125I-纤维蛋白原半衰期为2.1天，联合使用阿司匹林/肝素治疗时为1.9天。父母及4名存活的兄弟姐妹肾功能正常，血小板和纤维蛋白原存活时间正常。母亲和3名兄弟姐妹的巨型血小板百分比增加。2例出现51Cr-血小板放射性在肾脏的蓄积，血小板抑制剂可抑制这种蓄积。2例肾脏活检均显示明确的超微结构内皮改变。这些变化可能是导致局部血小板滞留的原因，似乎是这个患有溶血性尿毒症综合征的家族的主要缺陷。
