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Distinctly abnormal brain metabolism in late-onset ornithine transcarbamylase deficiency
https://repo.qst.go.jp/records/44017
https://repo.qst.go.jp/records/440172ba04f32-45d7-4d1c-8931-f81dc7f93c42
| Item type | 学術雑誌論文 / Journal Article(1) | |||||
|---|---|---|---|---|---|---|
| 公開日 | 2006-03-16 | |||||
| タイトル | ||||||
| タイトル | Distinctly abnormal brain metabolism in late-onset ornithine transcarbamylase deficiency | |||||
| 言語 | ||||||
| 言語 | eng | |||||
| 資源タイプ | ||||||
| 資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||
| 資源タイプ | journal article | |||||
| アクセス権 | ||||||
| アクセス権 | metadata only access | |||||
| アクセス権URI | http://purl.org/coar/access_right/c_14cb | |||||
| 著者 |
Takanashi, Junichi
× Takanashi, Junichi× Ikehira, Hiroo× Tanada, Shuji× et.al× 高梨 潤一× 池平 博夫× 棚田 修二 |
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| 抄録 | ||||||
| 内容記述タイプ | Abstract | |||||
| 内容記述 | To assess alterations in brain metabolites in patients with late-onset ornithine transcarbamylase deficiency (OTCD). Methods:Six unrelated,asymptomatic Japanese late-onset OTCD patients were analyzed by proton MRS(1HMRS) using a point-resolved spectroscopy technique(repetition and echo times,5000 and 30 ms). Localized spectra for the centrum semiovale were acquired and absolute metabolite concentrations were calculated using an LCModel. Results: Compared with age-matched controls, N-acetylaspartate and creatine concentrations were normal in all patients. The glutamine(Gln) plus glutamate concentration was increased in four patients, which progressed in proportion to the clinical stage. myo-inositol(ml) could not be detected in five symptomatid patients. A decreased choline(Cho) concentration was detected in two clinically severe patients. 1HMRS after liver transplantation in one patient revealed the normalization of all metabolites. Conclusion:These findings suggest progression of neurochemical events in OTCD,i.e., ml depletion and Gln accumulation folloewd by Cho depletion, which is reverse of that in hepatic encephalopatht,i.e., Cho depletion followed by ml depletion and Gln accumulation. | |||||
| 書誌情報 |
Neurology 巻 59, 号 2, p. 210-214, 発行日 2002-07 |
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| ISSN | ||||||
| 収録物識別子タイプ | ISSN | |||||
| 収録物識別子 | 0028-3878 | |||||
