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Recurrence of pulmonary alveolar proteinosis after bilateral lung transplantation in a patient with a nonsense mutation in CSF2RB
http://hdl.handle.net/10069/37295
http://hdl.handle.net/10069/372954634dda8-8ef7-42e1-b2b8-502dd750f566
名前 / ファイル | ライセンス | アクション |
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RMCR19_89.pdf (2.3 MB)
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Item type | 学術雑誌論文 / Journal Article(1) | |||||
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公開日 | 2017-05-16 | |||||
タイトル | ||||||
タイトル | Recurrence of pulmonary alveolar proteinosis after bilateral lung transplantation in a patient with a nonsense mutation in CSF2RB | |||||
言語 | ||||||
言語 | eng | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Hereditary pulmonary alveolar proteinosis | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Lung transplantation | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Recurrence | |||||
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 |
Takaki, Masahiro
× Takaki, Masahiro× Tanaka, Takeshi× Komohara, Yoshihiro× Tsuchihashi, Yoshiko× Mori, Daisuke× Hayashi, Kentaro× Fukuoka, Junya× Yamasaki, Naoya× Nagayasu, Takeshi× Ariyoshi, Koya× Morimoto, Konosuke× Nakata, Koh |
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抄録 | ||||||
内容記述タイプ | Abstract | |||||
内容記述 | Hereditary pulmonary alveolar proteinosis (PAP) caused by mutations in CSF2RA or CSF2RB, which encode GM-CSF receptor α and β respectively, is a rare disease. Although some experimental therapeutic strategies have been proposed, no clinical evidence has yet been reported. We herein describe the clinical course and recurrence of hereditary PAP after lung transplantation. A 36-year-old woman developed PAP of unknown etiology. She underwent bilateral lung transplantation from living donors at the age of 42 years because of severe respiratory failure complicated by pulmonary fibrosis. However, PAP recurred after 9 months, and we found that donor-origin alveolar macrophages had been almost completely replaced with recipient-origin macrophages. We performed a genetic analysis and identified a point deletion in the CSF2RB gene that caused a GM-CSF receptor-mediated signaling defect. PAP progressed with fibrosis in both transplanted lungs, and the patient died of respiratory failure 5 years after the lung transplantation. Distinct from recent reports on pulmonary macrophage transplantation in mice, this case suggests that human alveolar macrophages might not maintain their population only by self-renewal but may depend on a supply of precursor cells from the circulation. Bone marrow transplantation should be considered for treatment of severe PAP with GM-CSF receptor gene deficiency. | |||||
書誌情報 |
Respiratory Medicine Case Reports 巻 19, p. 89-93, 発行日 2016-08-13 |
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出版者 | ||||||
出版者 | Elsevier Ltd. | |||||
ISSN | ||||||
収録物識別子タイプ | ISSN | |||||
収録物識別子 | 22130071 | |||||
DOI | ||||||
関連タイプ | isIdenticalTo | |||||
識別子タイプ | DOI | |||||
関連識別子 | 10.1016/j.rmcr.2016.06.011 | |||||
権利 | ||||||
権利情報 | c 2016 The Authors. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). | |||||
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 | Respiratory Medicine Case Reports, 19, pp.89-93; 2016 |