| Project/Area Number |
62570433
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| Research Category |
Grant-in-Aid for General Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Research Field |
Pediatrics
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| Research Institution | Kumamoto University |
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Principal Investigator |
OHTANI Yoshinobu Kumamoto University Medical School,Instructor of Child Development, 医学部, 講師 (10168982)
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| Co-Investigator(Kenkyū-buntansha) |
KAI Yumiko Kumamoto University Medical Hospital,Assistant of Child Development, 附属病院, 助手 (10160954)
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| Project Period (FY) |
1987 – 1988
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| Project Status |
Completed (Fiscal Year 1988)
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| Budget Amount *help |
¥2,200,000 (Direct Cost: ¥2,200,000)
Fiscal Year 1988: ¥700,000 (Direct Cost: ¥700,000)
Fiscal Year 1987: ¥1,500,000 (Direct Cost: ¥1,500,000)
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| Keywords | Reye's syndrome / L-carnitine / mitochondrial dysfunction / Dicarboxylic aciduria / 4-pentenoic acid / バルプロ酸 / 4-penteroic acid / 尿中ジカルボン酸 |
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| Research Abstract |
We studied the carnitine metabolism in the adult rat model of Reye's syndrome and in the 3 cases of Reye's syndrome. Therapeutic effect of L-carnitine was also evaluated.
(1)Animal model of Reye's syndrome; 4-Pentenoic acid,administered intraperitoneally to rat by a single dose of 200mg/kg or by every 4 hours with 50mg/kg for 18 doses. could produced the features of typical Reye's syndrome. The administration of valproate(300-400mg/kg/day,intraperitoneally)for 7days could also produced the rat model of Reye's syndrome. The occurrence of Reye's syndrome in rat could be prevented with a prior administration of high doses of oral L-carnitine treatment(100-200mg/kg). But the recovery from the mitochondrial dysfunction,except for blood ammonia levels, was not obtained by the oral feeding of L-carnitine on the model rats of Reye's syndrome.
(2)L-carnitine treatment on early stage of Reye's syndrome; 3 patients of Reye's syndrome (2 cases of stage II and one case of stageIII) were treated with 200-400mg/kg/day of intravenously or orally administered L-carnitine. 2 cases of stage II were successfully treated, but only one case of stage III followed with some neurological seqela. After the treatment of L-carnitine,in these patients, carnitine metabolism was recovered and urinary dicarboxic acid was also disappeared. These data suggests that large doses of l-carnitine should be effective in early stage of Reye's syndrome.
(3)Therapeutic usefulness of large doses of L-carnitine on the patints of partial OTC deficiency(J.Pediatr. 1988.112;409-414) and on early stage of Reye's syndrome (Pediatr. Japonica (submitted)) was reported in the journal.
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