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成人瑞氏综合征。诊断要点。

Reye's syndrome in adults. Diagnostic considerations.

作者信息

Meythaler J M, Varma R R

出版信息

Arch Intern Med. 1987 Jan;147(1):61-4.

PMID:3800531
Abstract

Reye's syndrome (RS) is generally considered a childhood disease. We report our experience with RS in adults in the metropolitan Milwaukee area. Reye's syndrome was diagnosed in seven 18- to 46-year-old adults. The diagnostic criteria were as follows: viral prodrome followed by vomiting and encephalopathy without focal neurological signs, normal cerebrospinal fluid values, increased levels of serum aminotransferases (transaminase), prolonged prothrombin time, elevated blood ammonia levels, and characteristic microvesicular fatty liver and mitochondrial changes. None of the patients was hypoglycemic. The diagnosis of RS was entertained in 22 but confirmed in only seven patients. In cases of non-Reye's encephalopathy, drug ingestion presented as one of the most difficult differential diagnostic problems, which also included alcohol abuse, collagen vascular disease, and hepatitis B surface antigenemia. Clinical jaundice, distinctly uncommon in RS, was present in only one patient who presented to us in stage V coma. In adults, RS is more difficult to diagnose and should be suspected more frequently in patients with unexplained altered behavior following a viral illness and vomiting. Liver biopsy can be performed safely and is usually mandatory in adults. Patients with RS diagnosed during stage I or II coma and treated experienced an uneventful recovery.

摘要

瑞氏综合征(RS)通常被认为是一种儿童疾病。我们报告了在密尔沃基市区成年人中诊治RS的经验。7名18至46岁的成年人被诊断为瑞氏综合征。诊断标准如下:病毒前驱症状后出现呕吐和脑病,无局灶性神经体征,脑脊液值正常,血清转氨酶水平升高,凝血酶原时间延长,血氨水平升高,以及特征性的微泡性脂肪肝和线粒体改变。所有患者均无低血糖。22例患者曾考虑诊断为RS,但仅7例确诊。在非瑞氏综合征性脑病的病例中,药物摄入是最难鉴别的诊断问题之一,还包括酒精滥用、胶原血管病和乙肝表面抗原血症。临床黄疸在RS中明显少见,仅1例处于V期昏迷的患者出现黄疸。在成年人中,RS更难诊断,对于病毒感染后出现不明原因行为改变和呕吐的患者应更频繁地怀疑该病。肝活检可安全进行,在成年人中通常是必需的。在I期或II期昏迷时诊断并接受治疗的RS患者恢复顺利。

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