A study on clinical features and pathophysiology of drug-induced carnitine deficiency
| Project/Area Number |
25461552
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Pediatrics
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| Research Institution | Shimane University |
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Principal Investigator |
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| Project Period (FY) |
2013-04-01 – 2016-03-31
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| Project Status |
Completed (Fiscal Year 2015)
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| Budget Amount *help |
¥4,940,000 (Direct Cost: ¥3,800,000、Indirect Cost: ¥1,140,000)
Fiscal Year 2015: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2014: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2013: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
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| Keywords | カルニチン欠乏症 / タンデムマス / ピボキシル基 / シベレスタット / 遊離カルニチン / 全身性カルニチン欠乏症 / 急性脳症 / 低血糖 / in vitro probe assay / ピバロイルカルニチン / カルニチン / アシルカルニチン分析 / 薬剤性カルニチン欠乏症 / ピバリン酸 / バルプロ酸 |
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| Outline of Final Research Achievements |
In this study, we mainly focused on secondary carnitine deficiency due to pivalete-conjugated drugs. We retrospectively investigated the age, clinical manifestations, PCA administration period, and background of 25 patients who showed a decrease in free carnitine (C0, nmol/mL) concomitant with an increase in pivaloyl carnitine (C5) on acylcarnitine analysis with tandem mass spectrometry. Carnitine deficiency resulting from PCA treatment was most frequently observed in 1-year-old infants. Most patients manifested acute encephalopathy and/or hypoglycemia. Some patients developed carnitine deficiency after PCA administration for <14 days. We reported two patients who showed increased value of C5 by sivelestat sodium.
In the present study, we developed an in vitro probe assay for analyzing intra-and extra-cellular ACs.
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Report
(4 results)
Research Products
(7 results)
