Lichtenstein P K, Heubi J E, Daugherty C C, Farrell M K, Sokol R J, Rothbaum R J, Suchy F J, Balistreri W F
N Engl J Med. 1983 Jul 21;309(3):133-9. doi: 10.1056/NEJM198307213090302.
In a one-year prospective study we assessed the incidence of Reye's syndrome in children presenting with the acute onset of vomiting after a prodromal upper-respiratory-tract infection or varicella, and with serum alanine or aspartate aminotransferase levels at least three times higher than normal, and a paucity of neurologic findings. Of 25 patients meeting the above criteria, 19 had liver biopsies yielding adequate tissue for diagnostic purposes. Biopsy specimens from 14 of these 19 patients (74 per cent) were diagnostic of Reye's syndrome, according to rigorous light-microscopical, histochemical, and ultrastructural criteria. None of the biopsy specimens contained evidence of other acute pathologic processes, including hepatitis. A wide spectrum of mitochondrial alterations existed at the ultrastructural level, ranging from mild to severe lesions that were indistinguishable from those seen in comatose patients with Reye's syndrome. Our findings suggest that the clinical complex of vomiting, hepatic dysfunction, and minimal neurologic impairment after varicella or an upper-respiratory-tract infection usually represents Reye's syndrome. This syndrome occurs more frequently than previously recognized.
在一项为期一年的前瞻性研究中,我们评估了前驱性上呼吸道感染或水痘后急性起病伴呕吐、血清丙氨酸或天冬氨酸转氨酶水平至少高于正常三倍且神经系统表现轻微的儿童中瑞氏综合征的发病率。在符合上述标准的25例患者中,19例进行了肝活检,获取了足够用于诊断的组织。根据严格的光学显微镜、组织化学和超微结构标准,这19例患者中的14例(74%)活检标本诊断为瑞氏综合征。所有活检标本均未发现包括肝炎在内的其他急性病理过程的证据。在超微结构水平上存在广泛的线粒体改变,从轻度到重度病变,与瑞氏综合征昏迷患者所见的病变无法区分。我们的研究结果表明,水痘或上呼吸道感染后出现呕吐、肝功能障碍和轻微神经功能损害的临床综合征通常为瑞氏综合征。该综合征的发生频率比以前认识到的更高。