Daugherty C C, Gartside P S, Heubi J E, Saalfeld K, Snyder J
Division of Pathology, Children's Hospital Medical Center, Cincinnati, OH 45229.
Am J Pathol. 1987 Nov;129(2):313-26.
Morphometric analysis of liver ultrastructure in 14 children with Reye's syndrome (RS) of varying morphologic severity was compared with that of 6 children with normal livers. Results showed reduced numbers of enlarged mitochondria in RS. Multivariant analysis identified correlations between increased mitochondrial size, decreased mitochondrial number, and severity of neurologic disease (stage). A disproportionate increase in mitochondrial area and perimeter in the RS cases with the most depressed mitochondrial number distinguished the 4 children with residual neurologic damage or death. Serum salicylate concentrations were negatively correlated with severity of morphologic alteration. Two cases of non-RS salicylate toxicity showed normal or near-normal mitochondrial size and number. In fatty liver from an autopsy specimen from a child, a child with carnitine deficiency, and a child on therapy for dermatomyositis, mitochondrial numbers were also normal. Decreased mitochondrial numbers are characteristic of RS and imply a pathophysiologic mechanism, possibly related to impaired mitochondrial replication. Synergism with other forms of mitochondrial injury, such as salicylates, hypoglycemia, or shock may influence clinical severity, progression, and outcome.
对14例不同形态学严重程度的瑞氏综合征（RS）患儿的肝脏超微结构进行形态计量分析，并与6例肝脏正常的儿童进行比较。结果显示，RS患儿中肿大线粒体数量减少。多变量分析确定了线粒体大小增加、线粒体数量减少与神经疾病严重程度（分期）之间的相关性。线粒体数量最少的RS病例中线粒体面积和周长不成比例地增加，这区分出了4例有残留神经损伤或死亡的患儿。血清水杨酸浓度与形态学改变的严重程度呈负相关。两例非RS水杨酸中毒病例的线粒体大小和数量正常或接近正常。在一名儿童的尸检标本、一名患有肉碱缺乏症的儿童和一名接受皮肌炎治疗的儿童的脂肪肝中，线粒体数量也正常。线粒体数量减少是RS的特征，这意味着一种病理生理机制，可能与线粒体复制受损有关。与其他形式的线粒体损伤（如水杨酸盐中毒、低血糖或休克）的协同作用可能会影响临床严重程度、病程和预后。
