{"_buckets": {"deposit": "adc03fa0-4588-47bc-93a2-9792f80a128a"}, "_deposit": {"id": "3944", "owners": [], "pid": {"revision_id": 0, "type": "depid", "value": "3944"}, "status": "published"}, "_oai": {"id": "oai:soar-ir.repo.nii.ac.jp:00003944", "sets": ["462"]}, "author_link": ["8174", "8175", "8176", "8177", "8178", "8179", "8180", "8181", "8182", "8183", "8184", "8185"], "item_1628147817048": {"attribute_name": "出版タイプ", "attribute_value_mlt": [{"subitem_version_resource": "http://purl.org/coar/version/c_ab4af688f83e57aa", "subitem_version_type": "AM"}]}, "item_6_biblio_info_6": {"attribute_name": "書誌情報", "attribute_value_mlt": [{"bibliographicIssueDates": {"bibliographicIssueDate": "2008-11", "bibliographicIssueDateType": "Issued"}, "bibliographicIssueNumber": "5", "bibliographicPageEnd": "820", "bibliographicPageStart": "810", "bibliographicVolumeNumber": "49", "bibliographic_titles": [{"bibliographic_title": "JOURNAL OF HEPATOLOGY"}]}]}, "item_6_description_30": {"attribute_name": "資源タイプ（コンテンツの種類）", "attribute_value_mlt": [{"subitem_description": "Article", "subitem_description_type": "Other"}]}, "item_6_description_31": {"attribute_name": "フォーマット：mimeタイプ", "attribute_value_mlt": [{"subitem_description": "application/pdf", "subitem_description_type": "Other"}]}, "item_6_description_5": {"attribute_name": "引用", "attribute_value_mlt": [{"subitem_description": "JOURNAL OF HEPATOLOGY. 49(5):810-820 (2008)", "subitem_description_type": "Other"}]}, "item_6_link_3": {"attribute_name": "信州大学研究者総覧へのリンク", "attribute_value_mlt": [{"subitem_link_text": "Yazaki, M", "subitem_link_url": "http://soar-rd.shinshu-u.ac.jp/profile/ja.gpTauakh.html"}]}, "item_6_link_67": {"attribute_name": "WoS", "attribute_value_mlt": [{"subitem_link_text": "Web of Science", "subitem_link_url": "http://gateway.isiknowledge.com/gateway/Gateway.cgi?\u0026GWVersion=2\u0026SrcAuth=ShinshuUniv\u0026SrcApp=ShinshuUniv\u0026DestLinkType=FullRecord\u0026DestApp=WOS\u0026KeyUT=000261018000018"}]}, "item_6_publisher_4": {"attribute_name": "出版者", "attribute_value_mlt": [{"subitem_publisher": "ELSEVIER SCIENCE BV"}]}, "item_6_relation_48": {"attribute_name": "DOI", "attribute_value_mlt": [{"subitem_relation_name": [{"subitem_relation_name_text": "10.1016/j.jhep.2008.05.016"}], "subitem_relation_type_id": {"subitem_relation_type_id_text": "https://doi.org/10.1016/j.jhep.2008.05.016", "subitem_relation_type_select": "DOI"}}]}, "item_6_select_64": {"attribute_name": "著者版フラグ", "attribute_value_mlt": [{"subitem_select_item": "author"}]}, "item_6_source_id_35": {"attribute_name": "ISSN", "attribute_value_mlt": [{"subitem_source_identifier": "0168-8278", "subitem_source_identifier_type": "PISSN"}]}, "item_6_source_id_39": {"attribute_name": "NII ISSN", "attribute_value_mlt": [{"subitem_source_identifier": "0168-8278", "subitem_source_identifier_type": "PISSN"}]}, "item_6_source_id_40": {"attribute_name": "書誌レコードID", "attribute_value_mlt": [{"subitem_source_identifier": "AA10459105", "subitem_source_identifier_type": "NCID"}]}, "item_6_text_69": {"attribute_name": "wosonly authkey", "attribute_value_mlt": [{"subitem_text_value": "Adult-onset type II citrullinemia; Body mass index; Non-alcoholic fatty liver disease; Pancreatic secretory trypsin inhibitor"}]}, "item_6_text_70": {"attribute_name": "wosonly keywords", "attribute_value_mlt": [{"subitem_text_value": "NEONATAL INTRAHEPATIC CHOLESTASIS; II CITRULLINEMIA CTLN2; HOMOZYGOUS SLC25A13 MUTATION; SECRETORY TRYPSIN-INHIBITOR; ASPARTATE-GLUTAMATE CARRIER; ADULT-ONSET CITRULLINEMIA; HEPATOCELLULAR-CARCINOMA; JAPANESE POPULATION; CLINICAL-PICTURES; PPAR-ALPHA"}]}, "item_6_textarea_68": {"attribute_name": "wosonly abstract", "attribute_value_mlt": [{"subitem_textarea_value": "Background/Aims: Citrin deficiency caused by SLC25A13 gene mutations develops into adult-onset type II citrullinemia (CTLN2) and may be accompanied with hepatic steatosis and steatohepatitis. 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": "著者", "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": "ファイル情報", "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": "言語", "attribute_value_mlt": [{"subitem_language": "eng"}]}, "item_resource_type": {"attribute_name": "資源タイプ", "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": "タイトル", "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": ["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}