ID | 118569 |
著者 |
Egashira, Ryoko
Saga University
Johkoh, Takeshi
Kansai Rosai Hospital
Terasaki, Yasuhiro
Nippon Medical School
Hebisawa, Akira
Asahi General Hospital
Abe, Kinya
Toyonaka Municipal Hospital
Baba, Tomohisa
Kanagawa Cardiovascular and Respiratory Center
Fujikura, Yuji
National Defense Medical College
Fujita, Etsuo
Hashimoto Municipal Hospital
Hamada, Naoki
Kyushu University
Handa, Tomohiro
Kyoto University
Hasegawa, Yoshinori
Osaka Saiseikai Nakatsu Hospital
Hidaka, Koko
National Hospital Organization Kokura Medical Center
Hisada, Takeshi
Gunma University
Hisata, Shu
Jichi Medical University
Honjo, Chisato
University of Fukui
Ichikado, Kazuya
Saiseikai Kumamoto Hospital
Inoue, Yoshikazu
National Hospital Organization Kinki-Chuo Chest Medical Center
Izumi, Shinyu
National Center for Global Health and Medicine
Kato, Motoyasu
Juntendo University
Kishimoto, Takumi
Okayama Rosai Hospital
Okamoto, Masaki
Kurume University
Miki, Keisuke
National Hospital Organization Osaka Toneyama Medical Center
Mineshita, Masamichi
St.Marianna University School of Medicine
Nakamura, Yutaro
Hamamatsu University School of Medicine
Sakamoto, Susumu
Toho University
Sano, Masaaki
Akita University
Tsukada, Yoshikazu
Soka Municipal Hospital
Yamasue, Mari
Oita University
Homma, Sakae
Toho University
Hagiwara, Koichi
Jichi Medical University
Suda, Takafumi
Hamamatsu University School of Medicine
Inase, Naohiko
Hiratsuka Kyosai Hospital
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資料タイプ |
学術雑誌論文
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抄録 | Background: Diffuse pulmonary ossification is a specific lung condition that is accompanied by underlying diseases. However, idiopathic dendriform pulmonary ossification (IDPO) is extremely rare, and the clinical features remain unclear. In this study, we aimed to report the clinical characteristics of IDPO.
Methods: We conducted a nationwide survey of patients with IDPO from 2017 to 2019 in Japan and evaluated the clinical, radiological, and histopathological findings of patients diagnosed with IDPO. Results: Twenty-two cases of IDPO were identified. Most subjects (82%) were male, aged 22-56 years (mean (SD), 37.9 (9.1)) at diagnosis. Nearly 80% of the subjects were asymptomatic, and the condition was discovered during a medical check-up. However, 36% of the subjects showed a decline in forced vital capacity (%FVC) predicted <80% at diagnosis. The typical radiological features of high-resolution CT (HRCT) are calcified branching structures that are predominantly distributed in the lower lung fields without any other conspicuous finding. Histopathological analysis also showed dendriform ossified lesions from the intraluminal areas to interstitial areas. Notably, during the follow-up period of 20 years, disease progression was found in 88% on HRCT and more than 50% on pulmonary function tests (FVC and/or forced expiratory volume in 1s). Two cases with rapid decline of 10% /year in %FVC predicted were observed.)) at diagnosis. Nearly 80% of the subjects were asymptomatic, and the condition was discovered during a medical check-up. However, 36% of the subjects showed a decline in forced vital capacity (%FVC) predicted <80% at diagnosis. The typical radiological features of high-resolution CT (HRCT) are calcified branching structures that are predominantly distributed in the lower lung fields without any other conspicuous finding. Histopathological analysis also showed dendriform ossified lesions from the intraluminal areas to interstitial areas. Notably, during the follow-up period of 20 years, disease progression was found in 88% on HRCT and more than 50% on pulmonary function tests (FVC and/or forced expiratory volume in 1s). Two cases with rapid decline of 10% /year in %FVC predicted were observed. )) at diagnosis. Nearly 80% of the subjects wereasymptomatic, and the condition was discovered during a medical check-up. However, 36% of the subjects showed a decline in forced vital capacity (%FVC) predicted <80% at diagnosis. The typical radiological features of high-resolution CT (HRCT) are calcified branching structures that are predominantly distributed in the lower lung fields without any other conspicuous finding. Histopathological analysis also showed dendriform ossified lesions from the intraluminal areas to interstitial areas. Notably, during the follow-up period of 20 years, disease progression was found in 88% on HRCT and more than 50% on pulmonary function tests (FVC and/or forced expiratory volume in 1s). Two cases with rapid decline of 10% /year in %FVC predicted were observed. Conclusions: IDPO develops at a young age with gradually progressive phenotype. Further research and long-term (>20 years) follow-up are required to clarify the pathogenesis and clinical findings in IDPO. |
掲載誌名 |
BMJ Open Respiratory Research
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ISSN | 20524439
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出版者 | BMJ Publishing Group
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巻 | 9
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号 | 1
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開始ページ | e001337
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発行日 | 2022-09-26
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権利情報 | This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.
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フルテキストファイル | |
言語 |
eng
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著者版フラグ |
出版社版
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部局 |
医学系
病院
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