文献

J-GLOBAL ID：201702242797559159   整理番号：17A0268732

胆汁鬱滞および肝線維症のある早産児における4-フェニル酪酸治療の効果

Effects of 4-phenylbutyrate therapy in a preterm infant with cholestasis and liver fibrosis

著者 (11件)： Ito Shogo (Department of Pediatrics and Neonatology, Graduate School of Medical Sciences, Nagoya City University, Nagoya, Japan) ,  Hayashi Hisamitsu (Laboratory of Molecular Pharmacokinetics, Graduate School of Pharmaceutical Sciences, University of Tokyo, Tokyo, Japan) ,  Sugiura Tokio (Department of Pediatrics and Neonatology, Graduate School of Medical Sciences, Nagoya City University, Nagoya, Japan) ,  Ito Koichi (Department of Pediatrics and Neonatology, Graduate School of Medical Sciences, Nagoya City University, Nagoya, Japan) ,  Ueda Hiroko (Department of Pediatrics and Neonatology, Graduate School of Medical Sciences, Nagoya City University, Nagoya, Japan) ,  Togawa Takao (Department of Pediatrics and Neonatology, Graduate School of Medical Sciences, Nagoya City University, Nagoya, Japan) ,  Endo Takeshi (Department of Pediatrics and Neonatology, Graduate School of Medical Sciences, Nagoya City University, Nagoya, Japan) ,  Tanikawa Ken (Department of Diagnostic Pathology, Kurume University Hospital, Fukuoka, Japan) ,  Kage Masayoshi (Department of Diagnostic Pathology, Kurume University Hospital, Fukuoka, Japan) ,  Kusuhara Hiroyuki (Laboratory of Molecular Pharmacokinetics, Graduate School of Pharmaceutical Sciences, University of Tokyo, Tokyo, Japan) ,  Saitoh Shinji (Department of Pediatrics and Neonatology, Graduate School of Medical Sciences, Nagoya City University, Nagoya, Japan)
資料名： Pediatrics International (Web)  (Pediatrics International (Web))
巻： 58  号： 6  ページ： 506-509  発行年： 2016年 
JST資料番号： U0706A  ISSN： 1442-200X  資料種別： 逐次刊行物 (A)
記事区分： 原著論文  発行国： アメリカ合衆国 (USA)  言語： 英語 (EN)

抄録/ポイント： 胆汁酸塩輸送体は,肝細胞の細管膜に発現しており,胆汁酸塩の胆...

シソーラス用語： *胆汁うっ滞 ,...
準シソーラス用語： 胆汁酸輸送体 ,...

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著者キーワード (5件)： 4-phenylbutyrate ,  bile salt export pump ,  cholestasis ,  idiopathic neonatal hepatitis ,  preterm infant

分類 (3件)： 消化器疾患の薬物療法  (GH05020G) ,  その他の代謝作用薬の臨床への応用  (GW13070T) ,  小児科の治療  (GC08040I)

物質索引 (2件)： 4-フェニル酪酸 ,  ウルソデオキシコール酸

引用文献 (10件)：

• Hayashi H, Sugiyama Y. 4-phenylbutyrate enhances the cell surface expression and the transport capacity of wild-type and mutated bile salt export pumps. Hepatology 2007; 45: 1506-16.
• Gonzales E, Grosse B, Cassio D, Davit-Spraul A, Fabre M, Jacquemin E. Successful mutation-specific chaperone therapy with 4-phenylbutyrate in a child with progressive familial intrahepatic cholestasis type 2. J. Hepatol. 2012; 57: 695-8.
• Naoi S, Hayashi H, Inoue Tet al. Improved liver function and relieved pruritus after 4-phenylbutyrate therapy in a patient with progressive familial intrahepatic cholestasis type 2. J. Pediatr. 2014; 164: 1219-27.
• Balistreri WF, Bezerra JA, Jansen P, Karpen SJ, Shneider BL, Suchy FJ. Intrahepatic cholestasis: Summary of an AmericanAssociation for the Study of Liver Diseases single-topic conference. Hepatology 2005; 42: 222-35.
• Goto K, Sugiyama K, Sugiura Tet al. Bile salt export pump genemutations in two Japanese patients with progressive familial intrahepatic cholestasis. J. Pediatr. Gastroenterol. Nutr. 2003; 36: 647-50.

タイトルに関連する用語 (5件)： 胆汁鬱滞 ,  肝線維症 ,  早産児 ,  4-フェニル酪酸 ,  治療