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CFAP43 variant in persistent respiratory symptoms after hematopoietic cell transplantation 査読あり
Nagasawa S., Nishimura T., Yamada A., Kamimura S., Ishimura M., Moritake H.
Human Genome Variation 11 ( 1 ) 41 2024年12月
記述言語:英語 掲載種別:研究論文(学術雑誌) 出版者・発行元:Human Genome Variation
We describe a case of RAS-associated autoimmune leukoproliferative disease with primary ciliary dyskinesia (PCD)-like symptoms, such as recurrent pneumonia, sinusitis, and otitis media, that occurred 7 years after hematopoietic cell transplantation. Whole-exome sequencing revealed a heterozygous CFAP43 nonsense variant. Environmental factors related to hematopoietic cell transplantation may have led to PCD symptoms in this patient with this variant. Genetic screening can help avoid subsequent complications during patient management.
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Mitochondrial dynamics as a potential therapeutic target in acute myeloid leukemia 査読あり
Kinoshita M., Saito Y., Otani K., Uehara Y., Nagasawa S., Nakagawa M., Yamada A., Kamimura S., Moritake H.
International Journal of Hematology 120 ( 5 ) 601 - 612 2024年11月
記述言語:英語 掲載種別:研究論文(学術雑誌) 出版者・発行元:International Journal of Hematology
Acute myeloid leukemia (AML) cells are highly dependent on oxidative phosphorylation and the mitochondrial dynamics regulated by fusion-related genes MFN1, MFN2, and OPA1 and fission-related genes DNM1L and MFF. An analysis of previously published gene expression datasets showed that high expression of MFF was significantly associated with poor prognosis in patients with AML. Based on this finding, we investigated the impact of mitochondrial dynamics in AML. Transduction of shRNA against fission-related genes, DNM1L and MFF, inhibited growth and increased the mitochondrial area in AML cell lines. Extracellular flux analysis showed that deletion of mitochondrial dynamic regulators reduced mitochondrial respiration without significantly affecting glycolysis, except in shDNM1L-transfected cells. Immunodeficient NOG mice transplanted with DNM1L- or MFF-knockdown AML cells survived significantly longer than controls. Treatment of AML cell lines with Mdivi-1, which inhibits the DRP1 encoded by DNM1L, inhibited cell proliferation and oxidative phosphorylation. Our results show that mitochondrial dynamics play an important role in AML, and provide novel biological insights. The inhibition of mitochondrial dynamics induces unique mitochondrial alterations, which may be explored as a potential therapeutic target in AML.
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小児血液・悪性固形腫瘍患者に対する鎖骨上アプローチを用いた腕頭静脈穿刺による中心静脈カテーテル挿入術の検討 査読あり
中目 和彦, 桝屋 隆太, 永澤 俊, 中川 緑, 山田 愛, 木下 真理子, 上村 幸代, 盛武 浩, 家入 里志, 七島 篤志
日本小児外科学会雑誌 60 ( 2 ) 158 - 165 2024年4月
記述言語:日本語 掲載種別:研究論文(学術雑誌) 出版者・発行元:特定非営利活動法人 日本小児外科学会
【目的】中心静脈カテーテル(CVC)は小児血液・悪性固形腫瘍患者の治療において使用される.近年,安全なCVC挿入法としてin-plane法を用いた超音波(US)ガイド下鎖骨上アプローチによる腕頭静脈穿刺CVC挿入術が報告されている.【方法】小児血液・悪性固形腫瘍患者を対象にout-of-plane法を用いて内頸静脈にトンネル型CVCを挿入した群(IJV群)とin-plane法を用いて腕頭静脈に挿入した群(BCV群)について患者背景,手術成績,合併症を後方視的に比較検討した.【結果】34名の患者に対し,計40回(IJV群:n=15,BCV群:n=25)のトンネル型CVCが挿入された.患者背景,術前血液凝固検査値は両群間に有意差はなかった.手術時間中央値(IQR)はIJV群:30分(27~33),BCV群:25.8分(22~27)であり,BCV群で有意に手術時間が短縮された(p=0.0026).術中合併症はIJV群で1例(6.7%)認め,BCV群では認めなかった.CVC維持管理中の合併症はIJV群:10例(66.7%),BCV群:17例(68%)であり,両群間で有意差は認めなかった.カテーテル関連血流感染はIJV群:10例(66.7%),BCV群:12例(52%)に認め,有意差はみられなかった.CVC留置期間中央値(IQR)はIJV群:273日(172~363.5),BCV群:152日(101~280)であり有意差を認めなかった.【結論】リアルタイム超音波ガイド下鎖骨上アプローチによる腕頭静脈穿刺術は小児血液・悪性固形患者に対しても安全な手技と考えられた.
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Adjunctive effects of eltrombopag on immunosuppressive therapy for childhood aplastic anemia 査読あり
Eguchi K., Ishimura M., Ohga S., Endo S., Saito S., Kamimura S., Keino D., Kato S., Azuma Y., Watanabe A., Inoue A., Higa T., Ozono S., Fujita N., Watanabe K., Takahashi Y.
International Journal of Hematology 121 ( 4 ) 533 - 542 2024年
記述言語:英語 掲載種別:研究論文(学術雑誌) 出版者・発行元:International Journal of Hematology
Eltrombopag is used with first-line immunosuppressive therapy for adult aplastic anemia, although its practical utility in childhood remains unclear. We retrospectively analyzed the outcomes of pediatric patients who received eltrombopag in Japan. Of the 27 eligible patients, 23 (85%) were previously treated, and 15 (56%) had severe or very-severe disease. Seventeen (63%) received eltrombopag with or after rabbit anti-thymocyte globulin plus cyclosporin-A. Within the first year of eltrombopag therapy, 12 patients showed a good or partial response, 15 showed no response, and 8 non-responders successfully underwent hematopoietic cell transplantation. Within the first 3 months after eltrombopag therapy, all but one of the transfusion-dependent responders became transfusion-independent. At 12 months, 6 of 12 responders were disease-free off-treatment. The one-year overall response rate was higher for severe or very-severe than non-severe cases (p = 0.006). Multivariable analysis showed that very-severe disease at the start of eltrombopag therapy was a predictor of being disease-free off-treatment (p = 0.03). No cytogenetic abnormalities developed, but myelofibrosis occurred 4 months after eltrombopag therapy in one non-responder with very-severe disease. The first 3 months’ response to adjunctive eltrombopag may guide to the safe and effective use for the cure of disease, although prospective trials are needed to determine its long-term effects.
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Chemical screening approach using single leaves identifies compounds that affect cold signaling in Arabidopsis. 査読あり
Kitawaki K, Mihara R, Kamimura S, Sato A, Ushiyama M, Ito-Inaba Y, Inaba T
Plant physiology 193 ( 1 ) 234 - 245 2023年8月
MISC 【 表示 / 非表示 】
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Nagasawa S., Yamada A., Kinoshita M., Kamimura S., Moritake H.
Pediatrics International 64 ( 1 ) e14970 2022年1月
記述言語:英語 掲載種別:速報,短報,研究ノート等(学術雑誌) 出版者・発行元:Pediatrics International
DOI: 10.1111/ped.14970
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Kinoshita M., Yamada A., Saito Y., Kamimura S., Moritake H.
Pediatrics International 64 ( 1 ) e14975 2022年1月
記述言語:英語 掲載種別:速報,短報,研究ノート等(学術雑誌) 出版者・発行元:Pediatrics International
DOI: 10.1111/ped.14975
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Prevention of cisplatin-induced hearing-loss by sodium thiosulfate in medulloblastoma
Harao T., Yamada A., Kinoshita M., Kamimura S., Moritake H.
Pediatrics International 62 ( 10 ) 1204 - 1206 2020年10月
記述言語:日本語 掲載種別:速報,短報,研究ノート等(学術雑誌) 出版者・発行元:Pediatrics International
DOI: 10.1111/ped.14271
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Noguchi M., Moritake H., Kamimura S., Sonoda M., Ishimura M., Inagaki J.
Bone Marrow Transplantation 53 ( 9 ) 1214 - 1217 2018年9月
記述言語:日本語 掲載種別:記事・総説・解説・論説等(大学・研究所紀要) 出版者・発行元:Bone Marrow Transplantation
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Yamada A., Moritake H., Kinoshita M., Sawa D., Kamimura S., Iwamoto S., Yamashita Y., Inagaki J., Takahashi T., Shimada A., Obara M., Nunoi H.
Pediatrics International 58 ( 9 ) 905 - 908 2016年9月
記述言語:日本語 掲載種別:記事・総説・解説・論説等(大学・研究所紀要) 出版者・発行元:Pediatrics International
© 2016 Japan Pediatric Society Inversion of chromosome 16 [inv(16)] has a good prognosis in acute myeloid leukemia (AML), but additional genetic aberrations influence the outcome. We herein describe the case of a 15-year-old Japanese boy with inv(16) harboring a low-allelic burden internal tandem duplication of FLT3 (FLT3-ITD) and KIT mutations. Conventional chemotherapy eradicated a clone with a low-al lelic burden FLT3-ITD mutation, although another clone with a KIT mutation occurred 17 months later. Further investigation is necessary to identify AML with inv(16) conferring poor prognosis, to facilitate appropriate treatment with additional drugs, such as dasatinib or gemtuzumab ozogamicin.
DOI: 10.1111/ped.13010
受託研究受入実績 【 表示 / 非表示 】
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ストレンジックⓇ皮下注 長期の特定使用成績調査
2016年09月 - 2025年07月
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グロウジェクトのSGA性低身長症における特定使用成績調査
2013年09月 - 2022年10月
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グロウジェクトのSGA性低身長症における特定使用成績調査
2013年09月 - 2022年03月
JCRファーマ株式会社
担当区分:研究代表者
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グロウジェクトのターナー症候群における特定使用成績調査(成人身長に関する調査)
2010年11月 - 2020年03月