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An Autopsy Case of Hermansky-Pudlak Syndrome: A Case Report and Review of the Literature on Treatment
http://hdl.handle.net/10069/35000
http://hdl.handle.net/10069/3500066b9dd9d-bc21-4b0e-a370-630087aaa5c9
| 名前 / ファイル | ライセンス | アクション |
|---|---|---|
IntMed53_2705.pdf (2.4 MB)
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| Item type | 学術雑誌論文 / Journal Article(1) | |||||
|---|---|---|---|---|---|---|
| 公開日 | 2015-01-13 | |||||
| タイトル | ||||||
| タイトル | An Autopsy Case of Hermansky-Pudlak Syndrome: A Case Report and Review of the Literature on Treatment | |||||
| 言語 | ||||||
| 言語 | eng | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | Autopsy | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | Hermansky-pudlak syndrome | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | Lung transplantation | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | Pirfenidone | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | Pulmonary fibrosis | |||||
| 資源タイプ | ||||||
| 資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||
| 資源タイプ | journal article | |||||
| 著者 |
Harada, Tatsuhiko
× Harada, Tatsuhiko× Ishimatsu, Yuji× Nakashima, Shota× Miura, Shiro× Tomonaga, Masaomi× Kakugawa, Tomoyuki× Hara, Shintaro× Sakamoto, Noriho× Yoshii, Chiharu× Mukae, Hiroshi× Kawabata, Yoshinori× Kohno, Shigeru |
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| 抄録 | ||||||
| 内容記述タイプ | Abstract | |||||
| 内容記述 | 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. | |||||
| 書誌情報 |
Internal Medicine 巻 53, 号 23, p. 2705-2709, 発行日 2014-12-01 |
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| 出版者 | ||||||
| 出版者 | 日本内科学会 | |||||
| 出版者別言語 | ||||||
| 値 | Japanese Society of Internal Medicine | |||||
| ISSN | ||||||
| 収録物識別子タイプ | ISSN | |||||
| 収録物識別子 | 09182918 | |||||
| EISSN | ||||||
| 収録物識別子タイプ | ISSN | |||||
| 収録物識別子 | 13497235 | |||||
| DOI | ||||||
| 関連タイプ | isIdenticalTo | |||||
| 識別子タイプ | DOI | |||||
| 関連識別子 | 10.2169/internalmedicine.53.2239 | |||||
| 権利 | ||||||
| 権利情報 | c 2014 by The Japanese Society of Internal Medicine | |||||
| 著者版フラグ | ||||||
| 出版タイプ | VoR | |||||
| 出版タイプResource | http://purl.org/coar/version/c_970fb48d4fbd8a85 | |||||
| 引用 | ||||||
| 内容記述タイプ | Other | |||||
| 内容記述 | Internal Medicine, 53(23), pp.2705-2709; 2014 | |||||
