Kimura S, Kobayashi T, Tanaka Y, Sasaki Y
Department of Pediatrics, Yokohama City University School of Medicine, Japan.
Brain Dev. 1991;13(2):95-100. doi: 10.1016/s0387-7604(12)80114-8.
Analysis of the liver histopathology in 19 children with clinical Reye syndrome (RS) revealed that nine had diffuse panlobular steatosis, one giant cell hepatitis, one a mild choledochal cyst with inflammation, two multifocal spotty necrosis and one multiple centrilobular necrosis, the other five being normal. Four of the nine patients with diffuse panlobular steatosis showed microvesicular fatty droplets with central nuclei, which was consistent with findings characteristic for typical RS. Two cases showed a periportal area dominant macrovesicular fatty change, which was highly suggestive for metabolic disorder. In the other three cases, the findings were so variable in terms of the size of lipid droplets and the location of nuclei in hepatocytes that it was not possible to provide any clue for defining a diagnosis. These results confirmed the legitimacy of the diagnostic criteria of RS which included a liver biopsy as one of the mandatory conditions. They also indicated that RS-mimicking clinical pictures can be presented by miscellaneous conditions in which liver histology does not necessarily helpful in establishing definite diagnosis.
对19例临床诊断为瑞氏综合征(RS)患儿的肝脏组织病理学分析显示,9例有弥漫性全小叶脂肪变性,1例为巨细胞肝炎,1例为轻度胆总管囊肿伴炎症,2例为多灶性斑点状坏死,1例为多灶性中央小叶坏死,其他5例正常。9例弥漫性全小叶脂肪变性患者中有4例显示有中央核的微泡性脂肪滴,这与典型RS的特征性表现一致。2例显示以门周区为主的大泡性脂肪改变,这强烈提示代谢紊乱。在其他3例中,肝细胞内脂滴大小和细胞核位置的表现差异很大,无法为明确诊断提供任何线索。这些结果证实了RS诊断标准的合理性,该标准包括肝活检作为强制性条件之一。它们还表明,多种情况可呈现类似RS的临床症状,其中肝脏组织学不一定有助于确立明确诊断。