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I级瑞氏综合征——进展至更深昏迷等级的结局及预测因素

Grade I Reye's syndrome--outcome and predictors of progression to deeper coma grades.

作者信息

Heubi J E, Daugherty C C, Partin J S, Partin J C, Schubert W K

出版信息

N Engl J Med. 1984 Dec 13;311(24):1539-42. doi: 10.1056/NEJM198412133112404.

Abstract

We studied 83 biopsy-proved cases of Grade I Reye's syndrome to determine the outcome, possible clinical or laboratory predictors of progression to deeper coma grades, and hepatic ultrastructural findings. Seventy-eight patients had no change in coma grade during hospitalization, whereas five (6 per cent) had progression to deeper coma grades. All the patients survived without sequelae except one who sustained severe brain damage. The mean (+/- S.E.) level of serum ammonia on admission was significantly higher (P = 0.005) in patients whose disease progressed to deeper neurologic grades (291 +/- 42 micrograms per deciliter) than in those whose disease did not so progress (53 +/- 5 micrograms per deciliter), and the corrected prothrombin time was significantly more prolonged (P = 0.005) in patients with progressing coma (3.9 +/- 0.5 seconds) than in those whose coma grade did not change (1.6 +/- 0.2 seconds). The combination of a prothrombin time 3 seconds or longer than that of the control and a serum level of ammonia on admission of 100 micrograms per deciliter or more correctly predicted progression in 71.5 per cent of the cases (sensitivity, 100 per cent; specificity, 97.6 per cent). Our findings suggest that the prognosis is excellent for survival without sequelae in Grade I Reye's syndrome (98.8 per cent) when management includes hospital surveillance and intravenous glucose and electrolyte infusion.

摘要

我们研究了83例经活检证实的I级瑞氏综合征病例,以确定其预后、可能进展至更深昏迷级别的临床或实验室预测指标以及肝脏超微结构表现。78例患者在住院期间昏迷级别无变化,而5例(6%)进展至更深的昏迷级别。除1例发生严重脑损伤外,所有患者均存活且无后遗症。疾病进展至更深神经学级别的患者入院时血清氨的平均(±标准误)水平(291±42微克/分升)显著高于疾病未进展的患者(53±5微克/分升)(P = 0.005),昏迷进展患者的校正凝血酶原时间(3.9±0.5秒)显著长于昏迷级别未变化的患者(1.6±0.2秒)(P = 0.005)。凝血酶原时间比对照长3秒或更长且入院时血清氨水平为100微克/分升或更高这一组合在71.5%的病例中能正确预测病情进展(敏感性为100%;特异性为97.6%)。我们的研究结果表明,当治疗包括住院监测以及静脉输注葡萄糖和电解质时,I级瑞氏综合征患者无后遗症存活的预后极佳(98.8%)。

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