Kirschstein M, Aksu F, Tegtmeyer F
Klinik für Pädiatrie, Medizinischen Universität zu Lübeck.
Monatsschr Kinderheilkd. 1991 Feb;139(2):105-9.
After a few days of prodromal illness with diarrhea and fever there was an abrupt onset of coma, seizures, severe shock, and hyperpyrexia in two infants; they than developed bleeding, pancytopenia, and impairment of liver and renal function. Both infants survived with major neurologic sequelae. The clinical symptoms and laboratory parameters were compatible with hemorrhagic shock and encephalopathy syndrome which was first described in 1983. The clinical course and biochemical features of this disease and its differential diagnoses are described, and etiology and pathogenesis are discussed. Furthermore it will be discussed, whether the hemorrhagic shock and encephalopathy syndrome is actually a "new" disease or just a new label for a known disorder.
在出现腹泻和发热的前驱疾病几天后，两名婴儿突然出现昏迷、惊厥、严重休克和高热；随后他们出现出血、全血细胞减少以及肝肾功能损害。两名婴儿均存活，但伴有严重的神经后遗症。临床症状和实验室检查结果与1983年首次描述的出血性休克和脑病综合征相符。本文描述了该疾病的临床病程、生化特征及其鉴别诊断，并讨论了病因和发病机制。此外，还将探讨出血性休克和脑病综合征究竟是一种“新”疾病，还是仅仅是一种已知疾病的新名称。
