{"created":"2021-03-01T06:31:01.102200+00:00","id":25537,"links":{},"metadata":{"_buckets":{"deposit":"5533262c-94b3-4c04-9629-098f0ea2a954"},"_deposit":{"id":"25537","owners":[],"pid":{"revision_id":0,"type":"depid","value":"25537"},"status":"published"},"_oai":{"id":"oai:niigata-u.repo.nii.ac.jp:00025537","sets":["432:1291:1292:1304","453:456"]},"item_7_alternative_title_1":{"attribute_name":"その他のタイトル","attribute_value_mlt":[{"subitem_alternative_title":"A case of oral self-mutilation in Lesch-Nyhan syndrome"}]},"item_7_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2007-07","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"1","bibliographicPageEnd":"35","bibliographicPageStart":"31","bibliographicVolumeNumber":"37","bibliographic_titles":[{"bibliographic_title":"新潟歯学会雑誌"},{"bibliographic_title":"新潟歯学会雑誌","bibliographic_titleLang":"en"}]}]},"item_7_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"Lesch-Nyhan syndrome is a rare inborn error of purine metabolism caused of lack of hypoxanthine-guanine phosphoribosyl transferase (HGPRT), and is characterized by involuntary moments, mental retardation, cerebral palsy, and self-injurious behavior. Self-mutilation to lips and fingers are often observed, and prevention of self-mutilation and treatment to the destruction raise significant difficulties. We treated an 8 year-old boy with Lesch-Nyhan syndrome who had severe lower lip injuries with self-mutilation. The patient's central and lateral lower incisors were covered by resin at his first visit, and then treated by crown amputation and pulpectomy under general anesthesia. We also corrected form of his lower lip which was deeply wounded with self-mutilation. His self-injurious behavior is not seen for ten months after the treatment, but more follow-up examination is needed, as there are some reports that self-mutilation had restarted after the treatment.","subitem_description_type":"Abstract"},{"subitem_description":"Lesch-Nyhan症候群はhypoxanthine-guanine phosphoribosyl transferase (HGPRT) の欠損によりプリン体代謝の異常をきたす稀な先天性の疾患であり, 小児期に発症し, 不随意運動, 精神発達遅滞, 脳性麻痺, 自傷行為などの特徴的な臨床像を呈する疾患である。自傷行為は口唇や手指に多くみられ, その予防や治療には難渋することが多い。我々は今回下唇に自傷行為による深い潰瘍の見られた８歳のLesch-Nyhan症候群の男児に対し, 治療を行ったので報告する。初診時に下顎中切歯と側切歯をレジンで被覆し, その後全身麻酔下に下顎前歯の歯冠の削除と抜髄，下唇の形態修正を行った。術後10か月の間に自傷行為の再発は認めず, これらの治療法を組み合わせたことが自傷行為防止に有効であったと考えられた。しかしながら, さまざまな処置後に自傷行為の再発を認めたとの報告もあり, 引き続き注意深い経過観察が必要であると思われる。","subitem_description_type":"Abstract"}]},"item_7_full_name_3":{"attribute_name":"著者別名","attribute_value_mlt":[{"nameIdentifiers":[{"nameIdentifier":"150565","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Niimi, Kanae"}]},{"nameIdentifiers":[{"nameIdentifier":"150566","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Yoshizawa, Michiko"}]},{"nameIdentifiers":[{"nameIdentifier":"150567","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Izumi, Naoya"}]},{"nameIdentifiers":[{"nameIdentifier":"150568","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Takata, Yoshiyuki"}]},{"nameIdentifiers":[{"nameIdentifier":"150569","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Tomizawa, Mieko"}]},{"nameIdentifiers":[{"nameIdentifier":"150570","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Saito, Chikara"}]}]},"item_7_publisher_7":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"新潟歯学会"}]},"item_7_select_19":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_select_item":"publisher"}]},"item_7_source_id_11":{"attribute_name":"書誌レコードID","attribute_value_mlt":[{"subitem_source_identifier":"AN0018300X","subitem_source_identifier_type":"NCID"}]},"item_7_source_id_9":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"03850153","subitem_source_identifier_type":"ISSN"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"新美, 奏恵"}],"nameIdentifiers":[{"nameIdentifier":"150559","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"芳澤, 享子"}],"nameIdentifiers":[{"nameIdentifier":"150560","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"泉, 直也"}],"nameIdentifiers":[{"nameIdentifier":"150561","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"高田, 佳之"}],"nameIdentifiers":[{"nameIdentifier":"150562","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"富沢, 美惠子"}],"nameIdentifiers":[{"nameIdentifier":"150563","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"齊藤, 力"}],"nameIdentifiers":[{"nameIdentifier":"150564","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":"2019-08-19"}],"displaytype":"detail","filename":"NS_37(1)_31-35.pdf","filesize":[{"value":"364.8 kB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"NS_37(1)_31-35.pdf","url":"https://niigata-u.repo.nii.ac.jp/record/25537/files/NS_37(1)_31-35.pdf"},"version_id":"55b216c2-4e85-4fa9-beb5-850fc1d9c419"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"Lesch-Nyhan syndrome (Lesch-Nyhan 症候群)","subitem_subject_scheme":"Other"},{"subitem_subject":"self-mutilation (自傷行為)","subitem_subject_scheme":"Other"}]},"item_language":{"attribute_name":"言語","attribute_value_mlt":[{"subitem_language":"jpn"}]},"item_resource_type":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"resourcetype":"departmental bulletin paper","resourceuri":"http://purl.org/coar/resource_type/c_6501"}]},"item_title":"下唇への自傷行為を認めたLesch-Nyhan syndromeの1例","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"下唇への自傷行為を認めたLesch-Nyhan syndromeの1例"},{"subitem_title":"下唇への自傷行為を認めたLesch-Nyhan syndromeの1例","subitem_title_language":"en"}]},"item_type_id":"7","owner":"1","path":["456","1304"],"pubdate":{"attribute_name":"公開日","attribute_value":"2013-07-24"},"publish_date":"2013-07-24","publish_status":"0","recid":"25537","relation_version_is_last":true,"title":["下唇への自傷行為を認めたLesch-Nyhan syndromeの1例"],"weko_creator_id":"1","weko_shared_id":null},"updated":"2022-12-15T03:55:30.010607+00:00"}