Clinical staging in Reye syndrome.

Clinical staging in Reye syndrome.
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雷氏综合征的临床分期。

DOI:
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发表时间:
1974
期刊:
A M A Journal of Diseases of Children
影响因子:
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通讯作者:
P. Adams
P. Adams
中科院分区:
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文献类型:
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作者:
F. Lovejoy;A. Smith;M. Bresnan;J. N. Wood;D. I. Victor;P. Adams

文献摘要

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在三年的住院治疗期间,40名雷耶综合征患者中,17人死亡,而两名幸存者有严重的神经损伤。疾病的进展经历了五个阶段,每个阶段都与进行性的喙尾中枢神经系统受累相关。提示预后不良的临床指标包括:初始血氨浓度> 300μg/100 ml;通过前三个临床阶段的快速进展;第III期癫痫发作;IV期脑脊液压力升高;第三阶段的凝血酶原时间比对照组长13秒或更多。在病情超过III期的患者中,会发生死亡或严重的神经损伤。通过换血或降低颅内压升高的常规医学治疗手段,死亡率似乎并没有降低。
Of 40 patients with Reye syndrome hospitalized over a three-year period, 17 died, while two survivors have severe neurologic impairment. The illness progressed through five stages, each correlating with progressive rostral-caudal central nervous system involvement. Clinical indexes suggesting a poor outcome included an initial blood ammonia concentration > 300μg/100 ml; a rapid progression through the first three clinical stages; the onset of seizure activity in stage III; increased cerebrospinal fluid pressure in stage IV; and a prothrombin time in stage III that was 13 or more seconds longer than that of the control. In patients with an illness beyond stage III, death or severe neurological impairment occurred. The mortality did not seem to be lowered by exchange transfusion or conventional medical therapeutic means of reducing increased intracranial pressure.