Study on relation between acute encephalopathy in childhood and causative disorders of organic and fatty acid metabolism

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Acute encephalopathy of various causes like hyperpyrexia or infection is not rare in childhood. We studied the relation between acute encephalopathy and disorders of organic and fatty acid metabolism to develop the biochemical and molecular diagnostic system, and made a diagnosis on over 74 cases with disorders of organic and fatty acid metabolism.We investigated the mechanism of acute encephalopathy in case of food poisoning in view of fatty acid oxidation impairment, and found that the acute encephalopathy was caused by temporary disturbance of fatty acid oxidation by the toxin. Furthermore, we developed a new therapy with bezafibrate for fatty acid oxidation disorders, using the in vitro probe assay, using cultured cells and tandem mass spectrometry.