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As its clinical features remain unclear, we aimed to explore the characteristics of fatty liver disease associated with citrin deficiency. Methods: The prevalence of hepatic steatosis in 19 CTLN2 patients was examined, and clinical features were compared with those of non-alcoholic fatty liver disease (NAFLD) patients without known SLC25A13 gene mutations. Results: Seventeen (89%) CTLN2 patients had steatosis, and 4 (21%) had been diagnosed as having NAFLD before appearance of neuropsychological symptoms. One patient had steatohepatitis. Citrin deficiency-associated fatty livers showed a considerably lower prevalence of accompanying obesity and metabolic syndrome, higher prevalence of history of pancreatitis, and higher serum levels of pancreatic secretory trypsin inhibitor (PSTI) than fatty livers without the mutations. Receiver operating characteristic curve analyses revealed that a body mass index \u003c 20 kg/m(2) and serum PSTl \u003e 29 ng/mL were associated with citrin deficiency. Conclusions: Patients presenting with non-alcoholic fatty liver unrelated to obesity and metabolic syndrome might have citrin deficiency, and serum PSTI may be a useful indicator for distinguishing this from conventional NAFLD. (C) 2008 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved."}]}, "item_creator": {"attribute_name": "\u8457\u8005", "attribute_type": "creator", "attribute_value_mlt": [{"creatorNames": [{"creatorName": "Komatsu,  M", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8174", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Yazaki,  M", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8175", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Tanaka,  N", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8176", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Sano,  K", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8177", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Hashimoto,  E", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8178", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Taker,  YI", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8179", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Song,  YZ", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8180", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Tanaka,  E", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8181", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Kiyosawa,  K", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8182", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Saheki,  T", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8183", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Aoyama,  T", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8184", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Kobayashi,  K", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8185", "nameIdentifierScheme": "WEKO"}]}]}, "item_files": {"attribute_name": "\u30d5\u30a1\u30a4\u30eb\u60c5\u5831", "attribute_type": "file", "attribute_value_mlt": [{"accessrole": "open_date", "date": [{"dateType": "Available", "dateValue": "2015-09-24"}], "displaytype": "detail", "download_preview_message": "", "file_order": 0, "filename": "Citrin_deficiency_as.pdf", "filesize": [{"value": "169.8 kB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_note", "mimetype": "application/pdf", "size": 169800.0, "url": {"label": "Citrin_deficiency_as.pdf", "url": "https://soar-ir.repo.nii.ac.jp/record/3944/files/Citrin_deficiency_as.pdf"}, "version_id": "3178e8f2-a177-409c-8bac-0b97b24ae1d3"}]}, "item_language": {"attribute_name": "\u8a00\u8a9e", "attribute_value_mlt": [{"subitem_language": "eng"}]}, "item_resource_type": {"attribute_name": "\u8cc7\u6e90\u30bf\u30a4\u30d7", "attribute_value_mlt": [{"resourcetype": "journal article", "resourceuri": "http://purl.org/coar/resource_type/c_6501"}]}, "item_title": "Citrin deficiency as a cause of chronic liver disorder mimicking non-alcoholic fatty liver disease", "item_titles": {"attribute_name": "\u30bf\u30a4\u30c8\u30eb", "attribute_value_mlt": [{"subitem_title": "Citrin deficiency as a cause of chronic liver disorder mimicking non-alcoholic fatty liver disease", "subitem_title_language": "en"}]}, "item_type_id": "6", "owner": "1", "path": ["461/462"], "permalink_uri": "http://hdl.handle.net/10091/3078", "pubdate": {"attribute_name": "PubDate", "attribute_value": "2009-05-22"}, "publish_date": "2009-05-22", "publish_status": "0", "recid": "3944", "relation": {}, "relation_version_is_last": true, "title": ["Citrin deficiency as a cause of chronic liver disorder mimicking non-alcoholic fatty liver disease"], "weko_shared_id": -1}
  1. 050 医学部, 大学院医学系研究科
  2. 0501 学術論文

Citrin deficiency as a cause of chronic liver disorder mimicking non-alcoholic fatty liver disease

http://hdl.handle.net/10091/3078
33866990-fe81-4122-9793-5987b4ab6b6e
名前 / ファイル ライセンス アクション
Citrin_deficiency_as.pdf Citrin_deficiency_as.pdf (169.8 kB)
Item type 学術雑誌論文 / Journal Article(1)
公開日 2009-05-22
タイトル
言語 en
タイトル Citrin deficiency as a cause of chronic liver disorder mimicking non-alcoholic fatty liver disease
言語
言語 eng
資源タイプ
資源 http://purl.org/coar/resource_type/c_6501
タイプ journal article
著者 Komatsu, M

× Komatsu, M

WEKO 8174

en Komatsu, M

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Yazaki, M

× Yazaki, M

WEKO 8175

en Yazaki, M

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Tanaka, N

× Tanaka, N

WEKO 8176

en Tanaka, N

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Sano, K

× Sano, K

WEKO 8177

en Sano, K

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Hashimoto, E

× Hashimoto, E

WEKO 8178

en Hashimoto, E

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Taker, YI

× Taker, YI

WEKO 8179

en Taker, YI

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Song, YZ

× Song, YZ

WEKO 8180

en Song, YZ

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Tanaka, E

× Tanaka, E

WEKO 8181

en Tanaka, E

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Kiyosawa, K

× Kiyosawa, K

WEKO 8182

en Kiyosawa, K

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Saheki, T

× Saheki, T

WEKO 8183

en Saheki, T

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Aoyama, T

× Aoyama, T

WEKO 8184

en Aoyama, T

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Kobayashi, K

× Kobayashi, K

WEKO 8185

en Kobayashi, K

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信州大学研究者総覧へのリンク
氏名 Yazaki, M
URL http://soar-rd.shinshu-u.ac.jp/profile/ja.gpTauakh.html
出版者
出版者 ELSEVIER SCIENCE BV
引用
内容記述タイプ Other
内容記述 JOURNAL OF HEPATOLOGY. 49(5):810-820 (2008)
書誌情報 JOURNAL OF HEPATOLOGY

巻 49, 号 5, p. 810-820, 発行日 2008-11
資源タイプ(コンテンツの種類)
内容記述タイプ Other
内容記述 Article
ISSN
収録物識別子タイプ PISSN
収録物識別子 0168-8278
書誌レコードID
収録物識別子タイプ NCID
収録物識別子 AA10459105
DOI
関連識別子
識別子タイプ DOI
関連識別子 https://doi.org/10.1016/j.jhep.2008.05.016
関連名称
関連名称 10.1016/j.jhep.2008.05.016
出版タイプ
出版タイプ AM
出版タイプResource http://purl.org/coar/version/c_ab4af688f83e57aa
WoS
表示名 Web of Science
URL http://gateway.isiknowledge.com/gateway/Gateway.cgi?&GWVersion=2&SrcAuth=ShinshuUniv&SrcApp=ShinshuUniv&DestLinkType=FullRecord&DestApp=WOS&KeyUT=000261018000018
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