Elucidation of the mechanism of human antibody production by identifying causative genes for primary antibody deficiency
Project/Area Number |
18K07814
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Research Category |
Grant-in-Aid for Scientific Research (C)
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Allocation Type | Multi-year Fund |
Section | 一般 |
Review Section |
Basic Section 52050:Embryonic medicine and pediatrics-related
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Research Institution | Tokyo Medical and Dental University |
Principal Investigator |
IMAI Kohsuke 東京医科歯科大学, 大学院医歯学総合研究科, 寄附講座准教授 (90332626)
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Project Period (FY) |
2018-04-01 – 2021-03-31
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Project Status |
Completed (Fiscal Year 2020)
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Budget Amount *help |
¥4,420,000 (Direct Cost: ¥3,400,000、Indirect Cost: ¥1,020,000)
Fiscal Year 2020: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2019: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2018: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
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Keywords | 抗体産生 / 免疫記憶 / 原発性免疫不全症 / 形質細胞 / B細胞 / DNAメチル化 / 遺伝子再構成 / エクソーム解析 / 抗体産生不全症 / 免疫グロブリン / メモリーB細胞 / APRIL / 分類不能型免疫不全症 / 免疫グロブリンクラススイッチ / 樹状細胞 |
Outline of Final Research Achievements |
Common variable immunodeficiency (CVID) is a disease in which antibody production is not possible due to a single gene abnormality. In this study, through the analysis of novel CVID patient due to complete APRIL deficiency, we found that APRIL is responsible for lifelong immunological memory by maintaining memory B cells and plasma cells, and proposed a new CVID subclassification method using serum BAFF and APRIL. In addition, through the analysis of patients with PNKP deficiency and DNMT3B mutated ICF syndrome, which were found by whole exome sequencing, we studied the mechanisms by which each molecule is involved in B cell receptor gene rearrangement and memory B cell differentiation and maintenance.
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Academic Significance and Societal Importance of the Research Achievements |
APRIL欠損症の発見と病態の解明から、抗体産生へのAPRILの役割がわかり、組換えAPRILの臨床応用への可能性が示唆された。抗体産生不全症約200例について、記憶B細胞、形質細胞、IgG/A/M/E、血漿APRIL, BAFFを用いた分類を行った。XLA患者では全例BAFF高値・APRIL正常範囲であり、その他の抗体産生不全症患者、自己免疫疾患患者を、BAFF and/or APRIL高値と正常の4群にわけることが可能であった。また、抗体産生不全症を中心とした175例でのExome解析の結果、約1/3は既知遺伝子異常が同定されたが、残り2/3は原因不明であり、今後の研究の進展が望まれる。
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Report
(4 results)
Research Products
(193 results)
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[Journal Article] Correction to: Clinical and Immunological Heterogeneity in Japanese Patients with Gain-of-Function Variants in STAT32021
Author(s)
Tanita K, Sakura F, Nambu R, Tsumura M, Imanaka Y, Ohnishi H, Kato Z, Pan J, Hoshino A, Suzuki K, Yasutomi M, Umetsu S, Okada C, Takagi M, Imai K, Ohara O, Muise AM, Okada S, Morio T, Kanegane H.
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Pages: 791-794
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[Journal Article] A case of autoimmune enteropathy with CTLA4 haploinsufficiency2021
Author(s)
Miyazaki H, Hoshi N, Kohashi M, Tokunaga E, Ku Y, Takenaka H, Ooi M, Yamamoto N, Uemura S, Nishimura N, Iijima K, Jimbo K, Okano T, Hoshino A, Imai K, Kanegane H, Kobayashi I, Kodama Y.
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Intest Res
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Pages: 144-149
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[Journal Article] Liver Abscess in Chronic Granulomatous Disease-Two Decades of Experience from a Tertiary Care Centre in North-West India2021
Author(s)
Pilania RK, Rawat A, Vignesh P, Guleria S, Jindal AK, Das G, Suri D, Gupta A, Gupta K, Chan KW, Lau YL, Imai K, Singh S.
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Journal Title
J Clin Immunol
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Issue: 3
Pages: 552-564
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[Journal Article] Hemophagocytic Lymphohistiocytosis in Children with Chronic Granulomatous Disease-Single-Center Experience from North India.2021
Author(s)
Vignesh P, Loganathan SK, Sudhakar M, Chaudhary H, Rawat A, Sharma M, Shekar A, Vaiphei K, Kumar N, Sachdeva MUS, Jindal AK, Suri D, Gupta A, Ray P, Imai K, Ohara O, Nonoyama S, Lau YL and Singh S.
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J Allergy Clin Immunol Pract,
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[Journal Article] Inborn errors of type I IFN immunity in patients with life-threatening COVID-192020
Author(s)
Zhang Q, Bastard P, Liu Z, Le Pen J, Moncada-Velez M, Chen J, Ogishi M, Sabli IKD, Hodeib S, Korol C, Rosain J, Bilguvar K, Ye J, Bolze A, Bigio B, Yang R, Arias AA, Zhou Q, Zhang Y, Onodi F, Korniotis S, Karpf L, Philippot Q, Chbihi M, Bonnet-Madin L, Dorgham K, Smith N, Schneider WM, Razooky BS, Hoffmann HH, Michaili
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Science
Volume: 370
Issue: 6515
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[Journal Article] Autoantibodies against type I IFNs in patients with life-threatening COVID-192020
Author(s)
Bastard P, Rosen LB, Zhang Q, Michailidis E, Hoffmann HH, Zhang Y, Dorgham K, Philippot Q, Rosain J, Bテゥziat V, Manry J, Shaw E, Haljasmテ、gi L, Peterson P, Lorenzo L, Bizien L, Trouillet-Assant S, Dobbs K, de Jesus AA, Belot A, Kallaste A, Catherinot E, Tandjaoui-Lambiotte Y, Le Pen J, Kerner G, Bigio B, Seeleuthner Y,
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Journal Title
Science
Volume: 370
Issue: 6515
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[Journal Article] DNA ligase IV deficiency identified by chance following vaccine-derived rubella virus infection.2020
Author(s)
Matsumoto K, Hoshino A, Nishimura A, Kato T, Mori Y, Shimomura M, Naito C, Watanabe K, Hamazaki M, Mitsuiki N, Takagi M, Imai K, Nonoyama S, Kanegane H, Morio T.
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Journal Title
J Clin Immunol.
Volume: 40
Issue: 8
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[Journal Article] Current Perspectives and Unmet Needs of Primary Immunodeficiency Care in Asia Pacific2020
Author(s)
Leung D, Chua GT, Mondragon AV, Zhong Y, Nguyen-Ngoc-Quynh L, Imai K, Vignesh P, Suratannon N, Mao H, Lee WI, Kim YJ, Chan GCF, Liew WK, Huong LTM, Kanegane H, Muktiarti D, Zhao X, Santos-Ocampo FJ, Latiff AHA, Seger R, Ochs HD, Singh S, Lee PP, Lau YL.
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Front Immunol
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[Journal Article] Hematopoietic Cell Transplantation for Chronic Granulomatous Disease in Japan2020
Author(s)
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Front Immunol
Volume: 11
Pages: 1617-1617
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[Journal Article] Immunophenotyping of A20 haploinsufficiency by multicolor flow cytometry2020
Author(s)
Kadowaki, T. Ohnishi, H. Kawamoto, N. Kadowaki, S. Hori, T. Nishimura, K. Kobayashi, C. Shigemura, T. Ogata, S. Inoue, Y. Hiejima, E. Izawa, K. Matsubayashi, T. Matsumoto, K. Imai, K. Nishikomori, R. Ito, S. Kanegane, H. Fukao, T.
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Clinical Immunology
Volume: 216
Pages: 108441-108441
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[Journal Article] Pathogenic mutations identified by a multimodality approach in 117 Japanese Fanconi anemia patients.2020
Author(s)
Mori M, Hira A, Yoshida K, Muramatsu H, Okuno Y, Shiraishi Y, Anmae M, Yasuda J, Tadaka S, Kinoshita K, Osumi T, Noguchi Y, Adachi S, Kobayashi R, Kawabata H, Imai K, Morio T, Tamura K, Takaori-Kondo A, Yamamoto M, Miyano S, Kojima S, Ito E, Ogawa S, Matsuo K, Yabe H, Yabe M, Takata M.
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Journal Title
Haematologica
Volume: 105
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Pages: 1166-116
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[Journal Article] High-throughput analysis revealed the unique immunoglobulin gene rearrangements in plasmacytoma-like post-transplant lymphoproliferative disorder2020
Author(s)
Hoshino A, Nishimura A, Naruto T, Okano T, Matsumoto K, Okamoto K, Shintaku H, Tokoro S, Okamoto H, Wada T, Takagi M, Imai K, Kanegane H, Morio T
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Journal Title
Br J Haematol
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[Journal Article] Ruxolitinib treatment of a patient with steroid-dependent severe autoimmunity due to STAT1 gain-of-function mutation2020
Author(s)
Moriya K, Suzuki T, Uchida N, Nakano T, Katayama S, Irie M, Rikiishi T, Niizuma H, Okada S, Imai K, Sasahara Y, Kure S
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Journal Title
Int J Hematol
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[Journal Article] Impact of graft-versus-host disease on the clinical outcome of allogeneic hematopoietic stem cell transplantation for non-malignant diseases2020
Author(s)
Umeda K, Imai K, Yanagimachi M, Yabe H, Kobayashi M, Takahashi Y, Kajiwara M, Yoshida N, Cho Y, Inoue M, Hashii Y, Atsuta Y, Morio T
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Journal Title
Int J Hematol
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Issue: 6
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[Journal Article] Pathogenic mutations identified by a multimodality approach in 117 Japanese Fanconi anemia patients.2019
Author(s)
Mori M, Hira A, Yoshida K, Muramatsu H, Okuno Y, Shiraishi Y, Anmae M, Yasuda J, Tadaka S, Kinoshita K, Osumi T, Noguchi Y, Adachi S, Kobayashi R, Kawabata H, Imai K, Morio T, Tamura K, Takaori-Kondo A, Yamamoto M, Miyano S, Kojima S, Ito E, Ogawa S, Matsuo K, Yabe H, Yabe M, Takata M.
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Journal Title
Haematologica.
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Issue: 10
Pages: 1962-1973
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[Journal Article] Long-Term Evaluation of Low-Dose Betamethasone for Ataxia Telangiectasia2019
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Hasegawa S, Kumada S, Tanuma N, Tsuji-Hosokawa A, Kashimada A, Mizuno T, Moriyama K, Sugawara Y, Shirai I, Miyata Y, Nishida H, Mashimo H, Hasegawa T, Hosokawa T, Hisakawa H, Uematsu M, Fujine A, Miyata R, Sakuma H, Kashimada K, Imai K, Morio T, Hayashi M, Mizutani S, Takagi M
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Journal Title
Pediatric Neurology
Volume: 100
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[Journal Article] Long-term outcome and chimerism in patients with Wiskott-Aldrich syndrome treated by hematopoietic cell transplantation: a retrospective nationwide survey2019
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Iguchi A, Cho Y, Yabe H, Kato S, Kato K, Hara J, Koh K, Takita J, Ishihara T, Inoue M, Imai K, Nakayama H, Hashii Y, Morimoto A, Atsuta Y, Morio T
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Int J Hematol
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[Journal Article] A synonymous splice site mutation in IL2RG gene causes late-onset combined immunodeficiency2019
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Yamashita M, Wakatsuki R, Kato T, Okano T, Yamanishi S, Mayumi N, Tanaka M, Ogura Y, Kanegane H, Nonoyama S, Imai K, Morio T
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[Journal Article] Hematopoietic cell transplantation for asymptomatic X-linked lymphoproliferative syndrome type 12018
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[Journal Article] Long-Term Efficacy and Safety of Hizentra in Patients with Primary Immunodeficiency in Japan, Europe, and the United States: a Review of 7 Phase 3 Trials2018
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Jolles S, Rojavin MA, Lawo JP, Nelson R Jr, Wasserman RL, Borte M, Tortorici MA, Imai K, Kanegane H
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[Journal Article] Clinical and Immunological Characterization of ICF Syndrome in Japan2018
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J Clin Immunol
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[Journal Article] B-lymphoblastic lymphoma with TCF3-PBX1 fusion gene2018
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Haematologica
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[Journal Article] Comprehensive molecular diagnosis of Epstein-Barr virus-associated lymphoproliferative diseases using next-generation sequencing2018
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Ono S, Nakayama M, Kanegane H, Hoshino A, Shimodera S, Shibata H, Fujino H, Fujino T, Yunomae Y, Okano T, Yamashita M, Yasumi T, Izawa K, Takagi M, Imai K, Zhang K, Marsh R, Picard C, Latour S, Ohara O, et al.
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Int J Hematol
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[Journal Article] Hematopoietic stem cell transplantation for pulmonary alveolar proteinosis associated with primary immunodeficiency disease2018
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Tanaka-Kubota M, Shinozaki K, Miyamoto S, Yanagimachi M, Okano T, Mitsuiki N, Ueki M, Yamada M, Imai K, Takagi M, Agematsu K, Kanegane H, Morio T
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Int J Hematol
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[Journal Article] Phenotype, penetrance, and treatment of 133 cytotoxic T-lymphocyte antigen 4-insufficient subjects2018
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[Journal Article] Dysregulation of Epstein-Barr virus infection in hypomorphic ZAP70 mutation2018
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[Journal Article] Haploinsufficiency of A20 causes autoinflammatory and autoimmune disorders.2018
Author(s)
Kadowaki T, Ohnishi H, Kawamoto N, Hori T, Nishimura K, Kobayashi C, Shigemura T, Ogata S, Inoue Y, Kawai T, Hiejima E, Takagi M, Imai K, Nishikomori R, Ito S, Heike T, Ohara O, Morio T, Fukao T, Kanegane H.
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Volume: 141
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[Presentation] 移植後6ヶ月以降にドナーキメリズムの回復を認めたDNAリガーゼIV欠損症2021
Author(s)
友政弾, 満生紀子, 磯田健志 森下あおい, 山下基, 友田昂宏, 岡野翼, 遠藤明史, 神谷尚宏, 梶原道子, 金兼弘和, 高木正稔, 今井耕輔, 森尾友宏
Organizer
日本免疫不全・自己炎症学会 第4回総会・学術集会
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[Presentation] 原発性線毛機能不全症同胞例に対する高張食塩水吸入療法の有用性2020
Author(s)
江口詩織, 磯田健志, 金田明也, 井上健斗, 星野顕宏, 滝島茂, 柳町昌克, 高木正稔, 金兼弘和, 今井耕輔, 竹内万彦, 森尾友宏
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第123回日本小児科学会学術集会
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[Presentation] ALLの移植後再発に対し分子標的薬により寛解を得,PT-CY法で再移植した一例2020
Author(s)
森下あおい, 山下基, 磯田健志, 友田昂宏, 岡野翼, 神谷尚宏, 遠藤明史, 柳町昌克, 今井耕輔, 金兼弘和, 高木正稔, 森尾友宏
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第123回日本小児科学会学術集会
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[Presentation] 神経症状・免疫不全を合併したPNKP欠損症姉妹例の病態解析2020
Author(s)
渡部眞仁, 谷田けい, 岡野翼, 葉姿?, 重野美湖, 春日彩季, 時政定雄, 濱崎考史, 新宅治夫, 高木正稔, 今井耕輔, 森尾友宏
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第123回日本小児科学会学術集会
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[Presentation] 高張食塩水吸入療法が奏効した原発性線毛機能不全症の兄弟例2020
Author(s)
江口詩織, 磯田健志, 金田朋也, 井上健斗, 星野顕宏, 滝島茂, 柳町昌克, 高木正稔, 金兼弘和, 今井耕輔, 竹内万彦, 森尾友宏
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第123回日本小児科学会学術集会
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[Presentation] 重症感染症を繰り返すも病識が欠如し管理に難渋している高IgE症候群の一例2020
Author(s)
江口詩織, 磯田健志, 金田朋也, 井上健斗, 星野顕宏, 滝島茂, 柳町昌克, 高木正稔, 金兼弘和, 今井耕輔, 竹内万彦, 森尾友宏
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第123回日本小児科学会学術集会
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[Presentation] ろ紙血プロテオームを用いた原発性免疫不全症における新生児スクリーニングの可能性2020
Author(s)
柴田洋史, 八角高裕, 前田由可子, 宮本尚幸, 伊佐真彦, 本田吉孝, 田中孝之, 井澤和司, 川島祐介, 大西秀典, 石村匡崇, 今井耕輔, 小野寺雅史, 小原收, 滝田順子
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第123回日本小児科学会学術集会
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[Presentation] Hematopoietic stem cell transplantation for inborn errors of immunity in Japan: overview of a nationwide retrospective analysis2020
Author(s)
Miyamoto S, Yanagimachi M, Umeda K, Iguchi A, Sasahara Y, Takada H, Yamada M, Ishimura M, Koike T, Yasui M, Takahashi Y, Kawaguchi H, Kajiwara M, Inoue M, Kato K, Hashii Y, Atsuta Y, Yabe H, Imai K , Morio T.
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46th Annual Meeting of the European Society for Blood and Marrow Transplantation (EBMT 2020 Virtual)
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[Presentation] Hematopoietic stem cell transplantation for severe combined immunodeficiency in Japan: a nationwide retrospective analysis2020
Author(s)
Satoshi Miyamoto, Masakatsu Yanagimachi, Katsutsugu Umeda, Akihiro Iguchi, Yoji Sasahara, Hidetoshi Takada, Yoshiyuki Takahashi, Michiko Kajiwara, Masami Inoue, Koji Kato, Yoshiko Hashii, Yoshiko Atsuta, Hiromasa Yabe, Kohsuke Imai, Tomohiro Morio
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46th Annual Meeting of the European Society for Blood and Marrow Transplantation (EBMT 2020 Virtual)
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[Presentation] CARD9 DEFICIENCY IN A PATIENT WITH INVASIVE INFECTION DUE TO EXOPHIALA DERMATITIDIS AND THE ASYMPTOMATIC SIBLINGS2020
Author(s)
Y. Imanaka, M. Taniguchi, T. Doi, M. Shimomura, M. Tsumura, S. Karakawa, K. Imai, T. Morio, A. Puel, J.-L. Casanova, O. Ohara, K. Kamei, M. Kobayashi, S. Okada
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19th Biennial Meeting of The European Society of immunodeficiencies (ESID 2020)
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[Presentation] CLINICAL, IMMUNOLOGICAL, AND MOLECULAR FEATURES OF SEVERE COMBINED IMMUNE DEFICIENCY: A REPORT OF 87 PATIENTS FROM 3 TERTIARY CARE CENTRES IN INDIA2020
Author(s)
P. Vignesh, A. Rawat, A. Singh, R. Kumrah, A. Gummadi, A. Kaur, S. Bhattad, A. Mehta, D. Suri, A. Jinda, D. Leung, S. Ramprakash, R. Cp, K.W. Chan, Y. Ogura, K. Imai, O. Ohara, S. Nonoyama, M. Hershfield, Y. Lau, S. Singh
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19th Biennial Meeting of The European Society of immunodeficiencies (ESID 2020)
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[Presentation] PATIENTS WITH THE GAIN-OF-FUNCTION MUTATIONS IN STAT3 PRESENT WITH A VARIETY OF AUTOIMMUNE DISEASES2020
Author(s)
K. Tanita, F. Sakura, M. Tsumura, H. Ohnishi, A. Hoshino, K. Suzuki, S. Okada, R. Nambu, S. Umetsu, K. Imai, T. Morio, H. Kanegane
Organizer
19th Biennial Meeting of The European Society of immunodeficiencies (ESID 2020)
Related Report
Int'l Joint Research
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[Presentation] 当科で経験したXIAP欠損症関連炎症性腸疾患について2020
Author(s)
小野真太郎, 金兼弘和, 柳町昌克, 足洗美穂, 田中真理, 廣木遥, 井上健斗, 星野顕宏, 神谷尚宏, 満生紀子, 磯田健志, 高木正稔, 今井耕輔, 森尾友宏
Organizer
第47回日本小児栄養消化器肝臓学会
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[Presentation] 経口ロタウイルスワクチン株が検出された重症複合免疫不全症2020
Author(s)
谷田けい,河村吉紀,友田昴宏,井上健斗,井口晶裕,山田雅文,吉田太郎,村松秀城,多田憲正,石村匡崇,大賀正一,今井耕輔,森尾友宏,吉川哲史,金兼弘和
Organizer
第52回日本小児感染症学会 総会・学術集会
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[Presentation] 造血細胞移植によるXIAP欠損症関連炎症性腸疾患の治療2020
Author(s)
Shintaro Ono, Takahiro Kamiya, Masakatsu Yanagimachi, Toshihiko Imamura, Yoji Sasahara, Junichi Sugita, Kohsuke Imai, Tomohiro Morio, Motohiro Kato, Hirokazu Kanegane
Organizer
第62回日本小児血液・がん学会学術集会
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[Presentation] PTCyハプロ移植症例の6例の後方視的検討2020
Author(s)
Akifumi Endo, Motoi Yamashita, Takahiro Kamiya, Noriko Mitsuiki, Takeshi Isoda, Kohsuke Imai, Masatoshi Takagi, Michiko Kajiwara, Hirokazu Kanegane, Tomohiro Morio
Organizer
第62回日本小児血液・がん学会学術集会
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[Presentation] An intractable case of hyper IgE syndrome.2020
Author(s)
Tomoda T, Okano T, Inoue K, Yamashita M, Kamiya T, Isoda T, Yanagimachi, M Kanegane H, Takagi M, Morio T, Imai K.
Organizer
3rd Scientific Congress of Asia Pacific Society for Immunodeficiencies
Related Report
Int'l Joint Research / Invited
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[Presentation] Second transplantation rescued the graft failure after hematopoietic stem cell transplantation for a case of activated PI3K-delta syndrome type 22020
Author(s)
Tomoda T, Okano T, Inoue K, Yamashita M, Kamiya T, Isoda T, Yanagimachi, M Kanegane H, Takagi M, Morio T, Imai K.
Organizer
3rd Scientific Congress of Asia Pacific Society for Immunodeficiencies.
Related Report
Int'l Joint Research / Invited
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[Presentation] 非悪性疾患を対象とした移植後シクロフォスファミドと抗胸腺グロブリンを併用したGVHD予防法によるHLA不一致血縁者間造血幹細胞移植の多施設共同前方視的臨床試験: Haplo-NM.2020
Author(s)
加藤元博, 大隅朋生, 富澤大輔, 内山徹, 佐古、あゆみ, 井上永介, 瀧本哲也, 石川尊士, 河合利尚, 竹内一郎, 山田全毅, 出口隆生, 今井耕輔, 湯坐有希, 新井勝大, 今留謙一, 金兼弘和, 小野寺雅史, 松本公一
Organizer
第3回日本免疫不全・自己炎症学会総会・学術集会
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[Presentation] Cancer development in a Korean family with CTLA-4 haploinsufficiency2020
Author(s)
Yoonsun Yoon, Ji-man Kang, Junsik Choi, Kangmo Ahn, Keon Hee Yoo, Eun-Suk Kang, Kyoung-Mee Kim, Jung Eun Lee, Geum-Youn gawk, Kihyun Kim, Kosuke Imai, Hirokazu Kanegane, Yae-jean Kim.
Organizer
第3回日本免疫不全・自己炎症学会総会・学術集会
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[Presentation] Plasmacytoma-Like Post-Transplant Lymphoproliferative Disorder with Multiple Autoantibodies in a XIAP Deficient Patient2019
Author(s)
Hoshino A, Okano T, Matsumoto K, Nishimura A, Shintaku H, Tokoro S, Okamoto H, Wada T, Mitsuiki N, Kajiwara M, Takagi M, Imai K, Kanegane H, Morio T
Organizer
19th Biennial Meeting of the European Society of Immunodeficiencies
Related Report
Int'l Joint Research
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[Presentation] The First Intronic Mutations Which Caused Autosomal Recessive Complete STAT1 Deficiency2019
Author(s)
Sakata S, Okada S, Matsubayashi T, Tamaura M, Tsumura M, Okano T, Imai K, Bustamante J, Morio T, Casanova JL, Kobayashi M.
Organizer
19th Biennial Meeting of the European Society of Immunodeficiencies
Related Report
Int'l Joint Research
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[Presentation] 免疫不全患者に対するマルチプレックスPCR法を用いた呼吸器感染症早期診断の臨床的有用性の検討2019
Author(s)
遠藤明史,今井耕輔,井上健斗,岡野翼,清水則夫,寺田尚美,森下あおい,友田昴宏,山下基,柳田昌克,磯田健志,神谷尚宏,髙木正稔,金兼弘和,森尾友宏
Organizer
第51回日本小児・感染症学会
Related Report
Invited
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[Presentation] GATA2異常症に伴うMDSに対するAzacitidineの有効性2019
Author(s)
Issei Nunoda, Takahiro Tmoda, Takahiro Kmiya, Tsubasa Okano, Maho Hatano, Yuri Yoshimoto, Hideki Muramatu, Aoi Morishita, Kento Inoue, Motoi Yamashita, Akifumi Endo, Masakatsu Yanagimachi, Takeshi Isoda, Hirokazu Kanegane, Masatoshi Takagi, Kohsuke Imai, Tomohiro Morio
Organizer
第61回日本小児血液・がん学会学術集会
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[Presentation] 急性骨髄性白血病からT細胞型急性リンパ性白血病へclonal evolutionを起こしたMLL-AF9陽性乳児白血病の一例2019
Author(s)
Takahiro Tomoda, Akira Nishimura, Rintaro Ono, Takeshi Isoda, Maiko Inoue, Takahiro Kamiya, Masakatsu Yanagimachi, Kohsuke Imai, Hirokazu Kanegane, Daisuke Hasegawa, Tomohiro Morio, Atsushi Manabe, Masatoshi Takagi
Organizer
第61回日本小児血液・がん学会学術集会
Related Report
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[Presentation] 非血縁者間骨髄移植後早期にバンコマイシン自然耐性腸球菌性髄膜炎に罹患した活性型P13Kδ症候群2型の1例2019
Author(s)
Maho Hatano, Takahiro Tomoda, Tsubasa Okano, Issei Nunoda, Aoi Morishita, Kento Inoue, Motoi Yamashita, Akifumi Endo, Takahiro Kamiya, Takeshi Isoda, Masakatsu Yanagimachi, Masatoshi Takagi, Hirokazu Kanegane, Keisuke Okuno, Kohsuke Imai, Tomohiro Morio
Organizer
第61回日本小児血液・がん学会学術集会
Related Report
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[Presentation] Wiskott-Aldrich症候群における血小板輸血不応に対する各種治療薬の効果と安全性の検討2019
Author(s)
松本和明, 西村聡, 井上真依子, 井上健斗, 廣木遥, 小野真太郎, 岡野翼, 田中真理, 足洗美穂, 宮本智史, 宮脇零士, 山岸千佳, 手束真理, 大川哲平, 星野顕宏, 遠藤明史, 神谷尚宏, 青木由貴, 満生紀子, 小野敏明, 柳町昌克, 磯田健志, 富澤大輔, 長澤正之, 梶原道子, 高木正稔, 金兼弘和, 今井耕輔, 森尾友宏
Organizer
第61回日本小児血液・がん学会学術集会
Related Report
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[Presentation] B前駆細胞性急性リンパ性白血病の移植後再発に対して、GVHDの治療を行いながらBlinatumomabを使用した一例2019
Author(s)
森下あおい, 磯田健志, 山下基, 神谷尚宏, 岡野翼, 友田昂宏, 遠藤明史, 柳町昌克, 今井耕輔, 金兼弘和, 高木正稔, 森尾友宏
Organizer
第61回日本小児血液・がん学会学術集会
Related Report
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[Presentation] 骨髄移植後のIgG2選択的低値時に発症した侵襲性肺炎球菌感染症2019
Author(s)
坂川由里歌, 友田昂宏, 岡野翼, 森下あおい, 井上健斗, 山下基, 遠藤明史, 磯田健志, 神谷尚宏, 柳町昌克, 高木正稔, 金兼弘和, 今井耕輔, 竹下健一, 石和田稔彦, 森尾友宏
Organizer
第61回日本小児血液・がん学会学術集会
Related Report
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[Presentation] 免疫不全患者に対するマルチプレックスPCR法を用いた呼吸器感染症早期診断の臨床的有用性の検討2019
Author(s)
遠藤明史,今井耕輔,井上健斗,岡野翼,清水則夫,寺田尚美,森下あおい,友田昴宏,山下基,柳田昌克,磯田健志,神谷尚宏,髙木正稔,金兼弘和,森尾友宏
Organizer
第61回日本小児血液・がん学会学術集会
Related Report
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[Presentation] B前駆細胞性急性リンパ性白血病の移植後再発に対して、Blinatumomab、ドナーリンパ球輸注、Inotuzumab ozogamicinを使用した後、post-transplant cyclophosphamideを用いて、HLA半合致移植を行った一例2019
Author(s)
森下あおい,山下基,磯田健志,友田昴宏,岡野翼,神谷尚宏,柳町昌克,今井耕輔,金兼弘和,高木正稔,森尾友宏
Organizer
第25回小児血液・SCT研究会
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[Presentation] GVHD治療中再発の難治性ALLに対するブリナツモマブ、イノツズマブオゾガマイシンの使用経験2019
Author(s)
山下基,磯田健志,森下あおい,神谷尚宏,岡野翼,友田昴宏,遠藤明史,柳町昌克,今井耕輔,金兼弘和,高木正稔,森尾友宏
Organizer
第11回日本血液疾患免疫療法学会学術集会
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[Presentation] 腸管気腫を来した血液腫瘍性疾患・原発性免疫不全症の5例2019
Author(s)
西村聡,足洗美穂,神谷尚宏,山本俊亮,山本薫,友田昴宏,井上真依子,井上健斗,宮本智史,小野林太郎,平林真介,磯田健志,柳町昌克,細谷要介,長谷川大輔,今井耕輔,高木正稔,金兼弘和,真部淳,森尾友宏
Organizer
第122回日本小児科学会
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[Presentation] 本邦におけるICF症候群の臨床的および免疫学的検討2019
Author(s)
釜江智佳子,今井耕輔,加藤環,岡野翼,本間健一,中川紀子,野口恵美子,久保田健夫,森尾友宏,金兼弘和,野々山恵章
Organizer
第122回日本小児科学会
Related Report
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[Presentation] 造血細胞移植後に生じた腸管気腫症に対して間欠的酸素投与が有効であった3例2019
Author(s)
足洗美穂, 西村聡, 神谷尚宏, 山本俊亮, 小野林太郎, 友田昴宏, 山本薫, 井上健斗, 井上真依子, 廣木遥, 宮本智史, 星野顕宏, 梅原直, 平林真介, 岡本健太郎, 柳町昌克, 磯田健志, 細谷要介, 長谷川大輔, 今井耕輔, 高木正稔, 金兼弘和, 森尾友宏, 真部淳
Organizer
第41回日本造血細胞移植学会総会
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[Presentation] 慢性肉芽腫症に対する造血細胞移植成績に及ぼす遺伝子型の影響(JSHCTとPIDJ データ解析より)2019
Author(s)
柳町昌克,布井博幸,水上智之,西村豊樹,佐々木康二,清谷知賀子,康勝好,井口晶裕,田淵健,井上雅美,加藤剛二,今井耕輔,矢部普正,小林正夫,森尾友宏
Organizer
第41回日本造血細胞移植学会総会
Related Report
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[Presentation] 非腫瘍性疾患に対する同種造血幹細胞移植においてGVHD が治療成績に与える影響2019
Author(s)
梅田雄嗣,今井耕輔,柳町昌克,矢部普正,川口浩史,高橋義行,梶原道子,井上雅美,橋井佳子,熱田由子,森尾友宏
Organizer
第41回日本造血細胞移植学会総会
Related Report
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[Presentation] 高IgE血症を伴う難治性下痢から診断し造血細胞移植で根治したIPEX症候群2019
Author(s)
矢田裕太郎,江口克秀,石村匡崇,幸伏寛和,長谷川一太,白石 暁,古賀大介,西 眞範,今 井耕輔,金兼弘和,大賀正一
Organizer
第2回 日本免疫不全・自己炎症学会総会・学術集会
Related Report
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[Presentation] 原発性免疫不全症に対するフルダラビン・ブスルファンとフルダラビン・メルファランの強度減弱前処置による造血細胞移植2019
Author(s)
西村聡, 青木由貴, 石渡泰芳, 井上真依子, 市村卓也, 上山潤一, 松本和明, 井上健斗, 廣木遥, 小野真太郎, 岡野翼, 田中真理, 足洗美穂, 宮本智史, 宮脇零士, 山岸千佳, 手束真理, 大川哲平, 星野顕宏,今井耕輔、他
Organizer
第2回 日本免疫不全・自己炎症学会総会・学術集会
Related Report
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[Presentation] 成人期に達し遅発型複合免疫不全症に移行したNFKB1異常症に対する同種造血幹細胞移植2019
Author(s)
奥廣有喜,山崎夏維,谷村一輝,比良洸太,仁谷千賀,岡田恵子,藤崎弘之,岡本圭祐,岡 田 賢,重水大智,今井耕輔,森尾友宏,原 純一
Organizer
第2回 日本免疫不全・自己炎症学会総会・学術集会
Related Report
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[Presentation] 新たに診断した残存活性を有する遺伝性葉酸吸収不全症例と既報3症例との比較検討2019
Author(s)
戸澤雄介,Abdrabou Shimaa,野川奈津子,西内津雄,石田敏章,佐野秀樹,鈴木雄一,小林良二,小原收,成戸卓也,今井耕輔,小林邦彦,有賀正,山田雅文
Organizer
第2回 日本免疫不全・自己炎症学会総会・学術集会
Related Report
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[Presentation] Whole exome sequence analysis using the known and candidate genes for primary immunodeficiency diseases2018
Author(s)
Okamoto K, Shigemizu D, Okano T, Yeh TW, Takashima T, Yamashita M, Ono S, Mitsuiki N, Takagi M, Mori M, Kanegane H, Tsunoda T, Imai K, Morio T
Organizer
The 2nd APSID Scientific Congress
Related Report
Int'l Joint Research
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[Presentation] 早産児等における原発性免疫不全症新生児スクリーニング法の検討2018
Author(s)
勝屋恭子,伊藤一之,藤田華子,宮原宏幸,早田茉莉,佐藤千穂,箕面崎至宏,奥起久子,滝敦子,森丘千夏子,今井耕輔
Organizer
第63回日本新生児成育医学会・学術集会
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[Presentation] Hematopoietic stem cell transplantation for XIAP deficiency with chromosomally integrated human herpesvirus 62018
Author(s)
Inoue k, Kamiya T, Miura H, Inoue M, Yanagimachi M, Park MJ, Imai K, Takagi M, Yoshikawa T, Kanegane H, Morio T
Organizer
第50回日本小児感染症学会学術集会
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[Presentation] 同じ遺伝子変異で異なる臨床病型を認めたhypomorphic RAG1 mutationsによる複合型免疫不全症の兄弟例2018
Author(s)
上島洋二, 磯部勉, 小倉友美, 谷田けい, 寺田尚美, 古市美穂子, 佐藤智, 菅沼栄介, 康勝好, 高木正稔, 今井耕輔, 野々山恵章, 森尾友宏, 金兼弘和
Organizer
第46回日本臨床免疫学会総会
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