Kimura S, Ohtsuki N, Tanaka M, Nezu A
Department of Pediatrics, Urafune Hospital of Yokohama City University.
No To Hattatsu. 1999 Jul;31(4):355-8.
We reported here a 5-year-old girl with Sotos syndrome who developed acute shock encephalopathy syndrome (ASES), and differentiated ASES from Reye syndrome (RS). Abrupt onset of shock and status epilepticus developed and these were followed by disseminated intervascular coagulation (DIC) and liver damage. Gradual elevation of hepatic enzymes, high serum bilirubin value, and normal serum ammonia value in acute phase were incompatible with typical RS. Liver histology showed severe, diffuse necrosis of hepatocytes consisting of granular and vacuolar degeneration, which were quite different from those of RS. Thus, the disease process of ASES was shown to be different from those of RS.
我们在此报告一名患有索托斯综合征的5岁女孩，其发展为急性休克脑病综合征（ASES），并将ASES与瑞氏综合征（RS）进行了鉴别。休克和癫痫持续状态突然发作，随后出现弥散性血管内凝血（DIC）和肝损伤。急性期肝酶逐渐升高、血清胆红素值高以及血清氨值正常与典型的RS不相符。肝脏组织学显示肝细胞严重弥漫性坏死，包括颗粒样和空泡样变性，这与RS的情况截然不同。因此，ASES的疾病进程与RS不同。
