Dezateux C A, Dinwiddie R, Helms P, Matthew D J
Arch Dis Child. 1986 Jul;61(7):647-51. doi: 10.1136/adc.61.7.647.
Reye's syndrome continues to be associated with a high mortality. Out of 12 cases treated on our intensive care unit over a four year period, seven died, one suffered minimal cerebral damage, and four were normal. Rapid progression through coma stages and high peak ammonia concentrations worsened prognosis. Reye's syndrome was suspected in only 50% of cases at the referring hospital and resulted in prompt referral to the intensive care unit in only one case. Late referral to the unit was associated with a poor outcome. Sudden neurological deterioration followed diagnostic lumbar puncture in six children. Papilloedema was an unreliable sign of raised intracranial pressure and was absent in all cases. While computed tomography of the brain was useful in providing additional evidence of raised intracranial pressure, this could only be confirmed by direct measurement. Lumbar puncture in the presence of rapidly progressive coma should be deferred until raised intracranial pressure has been excluded. To this end, early admission to a paediatric intensive care unit with facilities for computed tomography and monitoring of intracranial pressure is recommended.
瑞氏综合征的死亡率仍然很高。在我们重症监护病房四年间收治的12例患者中,7例死亡,1例有轻微脑损伤,4例恢复正常。昏迷阶段进展迅速以及血氨浓度峰值高会使预后恶化。在转诊医院,只有50%的病例怀疑为瑞氏综合征,且只有1例因此被迅速转诊至重症监护病房。延迟转诊至该病房与不良预后相关。6名儿童在诊断性腰椎穿刺后出现突然的神经功能恶化。视乳头水肿并非颅内压升高的可靠体征,所有病例均未出现。虽然脑部计算机断层扫描有助于提供颅内压升高的额外证据,但这只能通过直接测量来证实。在快速进展性昏迷的情况下,腰椎穿刺应推迟至排除颅内压升高后进行。为此,建议尽早入住具备计算机断层扫描和颅内压监测设施的儿科重症监护病房。