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MMPH is usually complicated with tuberous sclerosis complex (TSC). MMPH patients tend to be asymptomatic or only slightly symptomatic. MMPH tends to progress slowly and needs no treatment. We herein describe two cases of MMPH with its characteristic radiological features and clinical manifestations of TSC. Case 1: a 20-year-old female with definitive TSC in infancy. Chest CT at the age of 18 revealed multiple nodular opacities and ground-glass attenuations in a scattered and random distribution in the bilateral lungs. Case 2: a 44-year-old female with probable TSC at 36 years of age. Chest CT at the age of 43 showed random areas of small ground-glass attenuations, predominantly in the upper lung fields. Case 1 and Case 2 have had no respiratory symptoms or radiographic changes in the recent two years and four years, respectively. Although pathological examinations of the lung were not performed because consent for surgical lung biospies was unobtainable, we considered that these pulmonary manifestations were most likely MMPH with TSC because of these characteristic radiographical findings of multiple nodular opacities and ground-glass attenuations of 10 mm or less in size and their scattered distribution, and because there have been no abnormal laboratory data or changes in their chest radiological findings for years. Neither patient is under treatment for pulmonary lesions. 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結節性硬化症に伴う多巣性微小結節性肺細胞過形成を呈したと考えられる2症例
https://uoeh-u.repo.nii.ac.jp/records/770
https://uoeh-u.repo.nii.ac.jp/records/7709ff00d93-567b-44e5-a629-9ba80ddd4f41
名前 / ファイル | ライセンス | アクション |
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S00039202.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 | 2020-08-03 | |||||
タイトル | ||||||
タイトル | 結節性硬化症に伴う多巣性微小結節性肺細胞過形成を呈したと考えられる2症例 | |||||
タイトル | ||||||
言語 | en | |||||
タイトル | Two Cases of Tuberous Sclerosis Complex Suggestive of Complicating Multifocal Micronodular Pneumocyte Hyperplasia: A Case Report | |||||
言語 | ||||||
言語 | eng | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | 多巣性微小結節性肺細胞過形成 | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | 結節性硬化症 | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | リンパ脈管症 | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | 肺癌 | |||||
キーワード | ||||||
言語 | en | |||||
主題Scheme | Other | |||||
主題 | multifocal micronodular pneumocyte hyperplasia | |||||
キーワード | ||||||
言語 | en | |||||
主題Scheme | Other | |||||
主題 | tuberous sclerosis complex | |||||
キーワード | ||||||
言語 | en | |||||
主題Scheme | Other | |||||
主題 | lymphangioleiomyomatosis | |||||
キーワード | ||||||
言語 | en | |||||
主題Scheme | Other | |||||
主題 | lung cancer | |||||
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 | |||||
記事区分 | ||||||
値 | Case Report(s) | |||||
著者 |
西田, 千夏
× 西田, 千夏× 矢寺, 和博× 城戸, 貴志× 野口, 真吾× 赤田, 憲太朗× 花香, 未奈子× 山﨑, 啓× 星野, 鉄兵× 下野, 昌幸× 石本, 裕士× 坂本, 憲穂× 迎, 寛× Nishida, Chinatsu× Yatera, Kazuhiro× Kido, Takashi× Noguchi, Shingo× Akata, Kentaro× Hanaka, Minako× Yamasaki, Kei× Hoshino, Teppei× Shimono, Masayuki× Ishimoto, Hiroshi× Sakamoto, Noriho× Mukae, Hiroshi |
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抄録 | ||||||
内容記述タイプ | Abstract | |||||
内容記述 | Multifocal micronodular pneumocyte hyperplasia (MMPH) is pathologically characterized by multifocal nodular hyperplasia of type Ⅱ pneumocyte-like cells. MMPH is usually complicated with tuberous sclerosis complex (TSC). MMPH patients tend to be asymptomatic or only slightly symptomatic. MMPH tends to progress slowly and needs no treatment. We herein describe two cases of MMPH with its characteristic radiological features and clinical manifestations of TSC. Case 1: a 20-year-old female with definitive TSC in infancy. Chest CT at the age of 18 revealed multiple nodular opacities and ground-glass attenuations in a scattered and random distribution in the bilateral lungs. Case 2: a 44-year-old female with probable TSC at 36 years of age. Chest CT at the age of 43 showed random areas of small ground-glass attenuations, predominantly in the upper lung fields. Case 1 and Case 2 have had no respiratory symptoms or radiographic changes in the recent two years and four years, respectively. Although pathological examinations of the lung were not performed because consent for surgical lung biospies was unobtainable, we considered that these pulmonary manifestations were most likely MMPH with TSC because of these characteristic radiographical findings of multiple nodular opacities and ground-glass attenuations of 10 mm or less in size and their scattered distribution, and because there have been no abnormal laboratory data or changes in their chest radiological findings for years. Neither patient is under treatment for pulmonary lesions. Although MMPH is a rare disease, multiple nodules and ground-glass attenuations on lung imaging findings should be considered as pulmonary manifestations in patients with TSC. | |||||
抄録 | ||||||
内容記述タイプ | Abstract | |||||
内容記述 | 多巣性微小結節性肺細胞過形成(multifocal micronodular pneumocyte hyperplasia(MMPH))は,Ⅱ型肺胞上皮様細胞の増殖からなる多巣性結節性病変で,結節性硬化症(tuberous sclerosis complex( TSC))に併発する疾患である.自覚症状はあっても軽微であり,進行は緩徐で治療を要しないとされている.われわれは,TSCに併発した,特徴的な画像所見を持つMMPH と考えられる2症例を経験したので報告する.症例1:幼児期にTSCと診断された20歳女性.18歳時の胸部CT で両肺に散在する結節影やスリガラス影を指摘された.症例2:36歳時にTSCと診断された44歳女性.43歳時の胸部CT で上葉優位にランダム分布の小さなスリガラス影を認めた.症例1は2年間,症例2は4年間に渡り,呼吸器症状も画像変化も認めない.肺生検の同意が得られず,病理学的な診断ができていないが,血液検査で有意な所見はなく,10 mm以下の結節影やスリガラス影が散在する特徴的画像所見や経年的に不変である経過などから,TSCに伴うMMPHと考えた.2症例とも肺病変に対する治療は行っていない.MMPHは稀な疾患であるが,TSC患者において両肺に多発する結節影やスリガラス影が認められた際にはMMPHである可能性を考慮すべきである. | |||||
書誌情報 |
産業医科大学雑誌 en : Journal of UOEH 巻 39, 号 2, p. 133-141, 発行日 2017-06-01 |
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出版者 | ||||||
出版者 | 産業医科大学(産業医科大学学会) | |||||
ISSN | ||||||
収録物識別子タイプ | ISSN | |||||
収録物識別子 | ISSN 0387-821X(PRINT), ISSN 2187-2864(ONLINE) | |||||
関連サイト | ||||||
識別子タイプ | URI | |||||
関連識別子 | https://www.jstage.jst.go.jp/browse/juoeh/39/2/_contents/-char/ja | |||||
関連名称 | Journal of UOEH | |||||
関連サイト | ||||||
識別子タイプ | URI | |||||
関連識別子 | https://www.uoeh-u.ac.jp/kouza/journal/i_2017-2j.html | |||||
関連名称 | 産業医科大学雑誌 | |||||
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 |