Korz R
Dtsch Med Wochenschr. 1984 Jan 27;109(4):132-6. doi: 10.1055/s-2008-1069153.
Three, previously healthy, patients developed profuse vomiting. In the further course of the disease there was a marked deterioration in their general condition with massive metabolic acidosis, cerebral symptoms and, finally, deep coma which ended in death, without any evidence of poisoning. Post-mortem examination revealed diffuse, droplet fatty infiltration of the liver of the degenerative type as well as cerebral oedema without inflammatory signs, findings of Reye's syndrome, rarely seen beyond childhood. The aetiology of this prognostically unfavourable disease remains uncertain, clinical features and laboratory results are very variable. In adults the disease is rarely diagnosed because the syndrome is not known. Only liver biopsy can confirm the diagnosis. Main aim of the symptomatic treatment is to counteract the cerebral oedema.
三名既往健康的患者出现了剧烈呕吐。在疾病的进一步发展过程中,他们的总体状况显著恶化,出现了严重的代谢性酸中毒、脑部症状,最终陷入深度昏迷并死亡,未发现任何中毒迹象。尸检显示肝脏有弥漫性、退行性类型的点状脂肪浸润以及无脑炎迹象的脑水肿,这是瑞氏综合征的表现,在儿童期以后很少见。这种预后不良疾病的病因仍不确定,临床特征和实验室结果差异很大。在成人中,由于不了解该综合征,这种疾病很少被诊断出来。只有肝活检才能确诊。对症治疗的主要目的是对抗脑水肿。