{"created":"2023-05-15T16:33:36.671186+00:00","id":5909,"links":{},"metadata":{"_buckets":{"deposit":"ca7830e1-16a1-438e-b941-1654b8d7b90f"},"_deposit":{"created_by":2,"id":"5909","owners":[2],"pid":{"revision_id":0,"type":"depid","value":"5909"},"status":"published"},"_oai":{"id":"oai:nagasaki-u.repo.nii.ac.jp:00005909","sets":["29:30"]},"author_link":["25537","25541","25539","25531","25533","25532","25534","25542","25535","25538","25540","25536"],"item_2_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2014-12-01","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"23","bibliographicPageEnd":"2709","bibliographicPageStart":"2705","bibliographicVolumeNumber":"53","bibliographic_titles":[{"bibliographic_title":"Internal Medicine"}]}]},"item_2_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"Hermansky-Pudlak syndrome (HPS) is a rare genetic disorder, the most common complication of which influencing the prognosis is pulmonary fibrosis. In the present report, we describe an autopsy case of a Japanese woman with HPS. The patient was diagnosed at 50 years of age based on the presence of oculocutane-ous albinism, hemorrhagic diathesis, ceroid-lipofuscin accumulation and pulmonary fibrosis. Although systemic steroids, immunosuppressants and pirfenidone were administered for pulmonary involvement, she died from respiratory failure two years later. Obtaining an early diagnosis and taking into consideration the need for lung transplantation is necessary in order to improve the prognosis of HPS. We herein report this very rare Japanese case of HPS with a review of the treatment approaches for HPS complicated with pulmonary fibrosis.","subitem_description_type":"Abstract"}]},"item_2_description_63":{"attribute_name":"引用","attribute_value_mlt":[{"subitem_description":"Internal Medicine, 53(23), pp.2705-2709; 2014","subitem_description_type":"Other"}]},"item_2_publisher_33":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"日本内科学会"}]},"item_2_relation_12":{"attribute_name":"DOI","attribute_value_mlt":[{"subitem_relation_type":"isIdenticalTo","subitem_relation_type_id":{"subitem_relation_type_id_text":"10.2169/internalmedicine.53.2239","subitem_relation_type_select":"DOI"}}]},"item_2_rights_13":{"attribute_name":"権利","attribute_value_mlt":[{"subitem_rights":"c 2014 by The Japanese Society of Internal Medicine"}]},"item_2_source_id_7":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"09182918","subitem_source_identifier_type":"ISSN"}]},"item_2_source_id_8":{"attribute_name":"EISSN","attribute_value_mlt":[{"subitem_source_identifier":"13497235","subitem_source_identifier_type":"ISSN"}]},"item_2_text_62":{"attribute_name":"出版者別言語","attribute_value_mlt":[{"subitem_text_value":"Japanese Society of Internal Medicine"}]},"item_2_version_type_16":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_version_resource":"http://purl.org/coar/version/c_970fb48d4fbd8a85","subitem_version_type":"VoR"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"Harada, Tatsuhiko"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Ishimatsu, Yuji"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Nakashima, Shota"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Miura, Shiro"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Tomonaga, Masaomi"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Kakugawa, Tomoyuki"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Hara, Shintaro"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Sakamoto, Noriho"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Yoshii, Chiharu"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Mukae, Hiroshi"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Kawabata, Yoshinori"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Kohno, Shigeru"}],"nameIdentifiers":[{}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2020-12-21"}],"displaytype":"detail","filename":"IntMed53_2705.pdf","filesize":[{"value":"2.4 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"IntMed53_2705.pdf","url":"https://nagasaki-u.repo.nii.ac.jp/record/5909/files/IntMed53_2705.pdf"},"version_id":"1a336fb7-a6b4-4e16-9fee-c6ad6a506191"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"Autopsy","subitem_subject_scheme":"Other"},{"subitem_subject":"Hermansky-pudlak syndrome","subitem_subject_scheme":"Other"},{"subitem_subject":"Lung transplantation","subitem_subject_scheme":"Other"},{"subitem_subject":"Pirfenidone","subitem_subject_scheme":"Other"},{"subitem_subject":"Pulmonary fibrosis","subitem_subject_scheme":"Other"}]},"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":"An Autopsy Case of Hermansky-Pudlak Syndrome: A Case Report and Review of the Literature on Treatment","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"An Autopsy Case of Hermansky-Pudlak Syndrome: A Case Report and Review of the Literature on Treatment"}]},"item_type_id":"2","owner":"2","path":["30"],"pubdate":{"attribute_name":"公開日","attribute_value":"2015-01-13"},"publish_date":"2015-01-13","publish_status":"0","recid":"5909","relation_version_is_last":true,"title":["An Autopsy Case of Hermansky-Pudlak Syndrome: A Case Report and Review of the Literature on Treatment"],"weko_creator_id":"2","weko_shared_id":-1},"updated":"2023-05-16T02:56:35.371518+00:00"}