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Liver histopathology in clinical Reye syndrome

Authors
Journal
Brain and Development
0387-7604
Publisher
Elsevier
Publication Date
Volume
13
Issue
2
Identifiers
DOI: 10.1016/s0387-7604(12)80114-8
Keywords
  • Reye Syndrome
  • Clinical Reye Syndrome
  • Reye-Like Disease
  • Microvesicular Fatty Liver
  • Acute Encephalopathy
Disciplines
  • Biology
  • Medicine

Abstract

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.

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