Metabolic function and liver histopathology in Reye-like illnesses

Forty children with Reye syndrome (RS) or Reye-like illnesses were investigated to elucidate the underlying aetiologies. Extensive biochemical studies including patterns of organic acids and amino acids, liver histopathology, and, if available, a DNA approach were performed. In addition to classical...

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Published inActa pædiatrica (Oslo) Vol. 85; no. 9; p. 1053
Main Authors Hou, J W, Chou, S P, Wang, T R
Format Journal Article
LanguageEnglish
Published Norway 01.09.1996
Subjects
Acidosis - diagnosis
Child, Preschool
DNA - analysis
Female
Gas Chromatography-Mass Spectrometry
Humans
Infant
Infant, Newborn
Liver - pathology
Male
Metabolism, Inborn Errors - blood
Metabolism, Inborn Errors - diagnosis
Metabolism, Inborn Errors - urine
Reye Syndrome - etiology
Reye Syndrome - metabolism
Reye Syndrome - pathology
Reye Syndrome - physiopathology
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Summary:Forty children with Reye syndrome (RS) or Reye-like illnesses were investigated to elucidate the underlying aetiologies. Extensive biochemical studies including patterns of organic acids and amino acids, liver histopathology, and, if available, a DNA approach were performed. In addition to classical RS (n = 10), the causes of Reye-like conditions included hereditary organic acidaemias (n = 13), urea cycle defects (n = 4), mitochondrial disorders (n = 3), fulminant hepatitis (n = 2), tyrosinaemia (n = 1), valproate-associated hepatotoxicity (n = 1), and other non-specific generalized organic acid disorders (n = 6). It is important to collect specimens when encephalopathy with liver dysfunction of unknown causes is noted. When the underlying inherited metabolic disorders are confirmed, the prevention of the recurrence by adequate diet control and medications, and genetic counselling become possible.
ISSN:0803-5253
DOI:10.1111/j.1651-2227.1996.tb14216.x