title: A morphometric study of Reye's syndrome. Correlation of reduced mitochondrial numbers and increased mitochondrial size with clinical manifestations.
abstract: 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.
authors.affiliation: Division of Pathology, Children's Hospital Medical Center, Cincinnati, OH 45229.
authors.forename: C C
authors.initials: CC
authors.lastname: Daugherty
authors.forename: P S
authors.initials: PS
authors.lastname: Gartside
authors.forename: J E
authors.initials: JE
authors.lastname: Heubi
authors.forename: K
authors.initials: K
authors.lastname: Saalfeld
authors.forename: J
authors.initials: J
authors.lastname: Snyder
mesh_terms: D000704:Analysis of Variance; D002648:Child; D003576:Electron Transport Complex IV / Q000378:metabolism; D005260:Female; D006801:Humans; D008099:Liver / Q000166:cytology / Q000473:pathology; D008297:Male; D008854:Microscopy, Electron; D008930:Mitochondria, Liver / Q000648:ultrastructure*; D012016:Reference Values; D012202:Reye Syndrome / Q000473:pathology*
chemical_list: D003576:Electron Transport Complex IV