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  1. 学術雑誌論文
  2. その他
  1. 部局別インデックス
  2. 医学部

Isovaleric acidemia : Therapeutic response to supplementation with glycine, l-carnitine, or both in combination and a 10-year follow-up case study

http://hdl.handle.net/20.500.12000/45971
http://hdl.handle.net/20.500.12000/45971
9cd68a4d-8976-4b09-b9ed-380809fa45ff
名前 / ファイル ライセンス アクション
1-s2.0-S2214426916300799-main.pdf 1-s2.0-S2214426916300799-main.pdf
Item type デフォルトアイテムタイプ(フル)(1)
公開日 2020-06-02
タイトル
タイトル Isovaleric acidemia : Therapeutic response to supplementation with glycine, l-carnitine, or both in combination and a 10-year follow-up case study
言語 en
作成者 Chinen, Yasutsugu

× Chinen, Yasutsugu

en Chinen, Yasutsugu

Nakamura, Sadao

× Nakamura, Sadao

en Nakamura, Sadao

Tamashiro, Kunihito

× Tamashiro, Kunihito

en Tamashiro, Kunihito

Sakamoto, Osamu

× Sakamoto, Osamu

en Sakamoto, Osamu

Tashiro, Kyoko

× Tashiro, Kyoko

en Tashiro, Kyoko

Inokuchi, Takahiro

× Inokuchi, Takahiro

en Inokuchi, Takahiro

Nakanishi, Koichi

× Nakanishi, Koichi

en Nakanishi, Koichi

アクセス権
アクセス権 open access
アクセス権URI http://purl.org/coar/access_right/c_abf2
権利情報
言語 en
権利情報 Creative Commons Attribution-NonCommercial-NoDerivatives 4.0
言語 en
権利情報Resource https://creativecommons.org/licenses/by-nc-nd/4.0/
権利情報 https://creativecommons.org/licenses/by-nc-nd/4.0/
主題
言語 en
主題Scheme Other
主題 Isovaleric acidemia
言語 en
主題Scheme Other
主題 Glycine
言語 en
主題Scheme Other
主題 L-carnitine
言語 en
主題Scheme Other
主題 Isovalerylcarnitine
言語 en
主題Scheme Other
主題 Isovalerylglycine
内容記述
内容記述タイプ Other
内容記述 Isovaleric acidemia (IVA) is an organic acid disease caused by a deficiency of isovaleryl-CoA dehydrogenase. Deficiency of this enzyme leads to accumulation of organic acids, such as isovalerylcarnitine and isovalerylglycine. The proposed IVA treatments include leucine restriction and l-carnitine and/or glycine supplementation, which convert isovaleric acid into non-toxic isovalerylcarnitine and isovalerylglycine, respectively. We examined the therapeutic response using the leucine load test and performed a 10-year follow-up in the patient. Methods:We evaluated the patient with IVA beginning at 5 years of age, when he presented with a mild to intermediate metabolic phenotype. Ammonia, free carnitine, isovalerylcarnitine, and isovalerylglycine were analyzed in the urine and blood after a meal consisting of 1600 mg leucine with glycine alone (250 mg/kg/day), l-carnitine alone (100 mg/kg/day), or both glycine and l-carnitine for four days each. Results:(Leucine load test) Three hours after the meal, serum ammonia levels increased most dramatically with glycine treatment alone, then with both in combination, and least with l-carnitine alone. Urinary isovalerylglycine levels increased 2-fold more with glycine supplementation than those following supplementation with both agents or with l-carnitine alone. Treatment with both agents resulted in a gradual increase in urinary acylcarnitine levels during the 6-h period following the leucine load, reaching concentrations comparable to those observed with l-carnitine alone. (Clinical course) After initiation of both glycine (200 mg/kg/day) and l-carnitine (100 mg/kg/day) supplementation at 5 years of age, doses were gradually reduced to 111.7 mg/kg/day and 55.8 mg/kg/day, respectively, at 15 years of age. His mind and body had developed without any sequelae. Discussion:We concluded that l-carnitine conjugated isovaleric acid earlier than glycine. Additionally, during the 10-year follow-up period, the patient displayed no clinical deterioration.
内容記述タイプ Other
内容記述 論文
出版者
言語 en
出版者 Elsevier
言語
言語 eng
資源タイプ
資源タイプ journal article
資源タイプ識別子 http://purl.org/coar/resource_type/c_6501
出版タイプ
出版タイプ VoR
出版タイプResource http://purl.org/coar/version/c_970fb48d4fbd8a85
識別子
識別子 http://hdl.handle.net/20.500.12000/45971
識別子タイプ HDL
関連情報
関連識別子
識別子タイプ DOI
関連識別子 https://doi.org/10.1016/j.ymgmr.2017.03.002
関連識別子
識別子タイプ DOI
関連識別子 https://doi.org/10.1016/j.ymgmr.2017.03.002
収録物識別子
収録物識別子タイプ ISSN
収録物識別子 2214-4269
収録物名
言語 en
収録物名 Molecular Genetics and Metabolism Reports
書誌情報
巻 11, p. 2-5
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