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医学部 附属病院 総合周産期母子医療センター |
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助教 |
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論文 【 表示 ／ 非表示 】
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Hirono K, Hata Y, Ichimata S, Nishida N, Imamura T, Asano Y, Kuramoto Y, Tsuboi K, Takarada S, Okabe M, Nakaoka H, Ibuki K, Ozawa S, Muneuchi J, Yasuda K, Urayama K, Oka H, Miyamoto T, Baba K, Kato A, Saiki H, Kuwabara N, Harada M, Baba S, Morikawa M, Iwasaki H, Hirata Y, Ito Y, Sakaguchi H, Urata S, Toda K, Kittaka E, Okada S, Hasebe Y, Hoshino S, Fujii T, Mitsushita N, Nii M, Ogino K, Fujino M, Yoshida Y, Fukuda Y, Iwashima S, Takigiku K, Sakata Y, Inuzuka R, Maeda J, Hayabuchi Y, Fujioka T, Namiki H, Fujita S, Nishida K, Kuraoka A, Kan N, Kido S, Watanabe K, Ichida F
Scientific reports 14 ( 1 ) 30469 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：Scientific Reports
Dilated cardiomyopathy (DCM) is a progressive myocardial disorder characterized by impaired cardiac contraction and ventricular dilation. However, some patients with DCM improve when experiencing left ventricular reverse remodeling (LVRR). Currently, the detailed association between genotypes and clinical outcomes, including LVRR, particularly among children, remains uncertain. Pediatric patients with DCM from multiple Japanese institutions recorded between 2014 and 2023 were enrolled. We identified their DCM-related genes and explored the association between gene variants and clinical outcomes, including LVRR. We included 123 pediatric patients (62 males; median age: 8 [1–51] months) and found 50 pathogenic variants in 45 (35.0%) of them. The most identified gene was MYH7 (14.0%), followed by RYR2 (12.0%) and TPM1 (8.0%). LVRR was achieved in 47.5% of these patients. The left ventricular ejection fraction remained unchanged (31.4% to 39.8%, P = 0.1913) in patients with sarcomere gene variants and in those with non-sarcomere gene variants (33.4% to 47.8%, P = 0.0522) but significantly increased in those without gene variants (33.6% to 54.1%, P < 0.0001). LVRR was not uniform across functional gene groups. Hence, an individualized gene-guided prediction approach may be adopted for children with DCM.
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Isolated Blind-Ended Major Aortic Pulmonary Collateral Artery With an Aneurysm in an Infant With Trisomy 21. 査読あり
Yonaga R, Kodama Y, Takamura K, Harada M, Moritake H
Cureus 16 ( 10 ) e72078 2024年10月
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Determination of Genotype and Phenotypes in Pediatric Patients With Biventricular Noncompaction 査読あり
Hirono K., Hata Y., Imamura T., Tsuboi K., Takarada S., Okabe M., Nakaoka H., Ibuki K., Ozawa S., Ichimata S., Nishida N., Iwasaki H., Urata S., Okada S., Hiratsuji T., Sakaguchi H., Takigiku K., Nakazawa M., Nishihara E., Harada M., Matsuo O., Yasuda K., Yoshida Y., Namiki H., Yasuda K., Ifuku T., Urayama K., Oka H., Ogino K., Kato A., Kan N., Seki S., Seki M., Odanaka Y., Iwashima S., Yoshida S., Miyata T., Miyamoto T., Watanabe K., Kuwabara N., Inuzuka R., Takahashi Y., Sakazaki H., Muneuchi J., Kogaki S., Numano F., Kido S., Nii M., Hoshino S., Ishida H., Maeda J., Hayabuchi Y., Otsubo Y., Ikeda K., Tsukano S., Watanabe M., Momoi N., Fujii T., Fujioka T., Fujino M., Uchiyama H., Baba S., Horigome H., Honda T., Suzuki K., Ichida F.
Journal of the American Heart Association 13 ( 21 ) 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：Journal of the American Heart Association
BACKGROUND: Left ventricular noncompaction (LVNC) is a hereditary type of cardiomyopathy characterized by prominent tra-beculations. Detailed characteristics of biventricular noncompaction (BiVNC) remain unknown. This study aimed to elucidate the clinical characteristics and genetic landscape of BiVNC.METHODS AND RESULTS: We recruited children with left ventricular noncompaction from Japanese multi-institutional centers from 2013 to 2021. Left ventricular noncompaction was classified as BiVNC, congenital heart disease, arrhythmia, dilated car-diomyopathy, or normal function. In these patients, cardiomyopathy-associated genes were screened. A total of 234 patients (127 male; mean age, 4 months [range, 0–6.6 years]) were enrolled in this study, of whom 25 had BiVNC; 55, normal function; 84, dilated cardiomyopathy; 38, congenital heart disease; and 32, arrhythmia. BiVNC was diagnosed during the perinatal period in 10 patients, in whom the prevalence was higher than that in other patients. A total of 14 patients in the group with BiVNC had congenital heart disease, but not necessarily right heart lesions. Left ventricular dyskinesis was frequently observed in the lateral wall (24%) and apex (28%). Eleven pathogenic variants were found in 11 patients with BiVNC (44.0%). The group with BiVNC had a higher ratio of mitochondrial and developmental gene variants than the other groups. Among all groups, the group with BiVNC had the worst survival rate (P=0.0009). CONCLUSIONS: Pediatric patients with BiVNC had a high rate of ventricular dyskinesis and poor outcome. A comprehensive and careful screening for disease-causing genes and phenotype may help identify specific patients with left ventricular noncom-paction and mortality-related cardiac phenotypes.
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Ebihara Shusei, Kodama Yoshihiko, Takamura Kazunari, Harada Masako, Moritake Hiroshi
Journal of Pediatric Cardiology and Cardiac Surgery advpub ( 0 ) 82 - 86 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：特定非営利活動法人 日本小児循環器学会
Symptoms of coronavirus infectious disease 2019 (COVID-19) are usually mild in young patients. Some children, however, present with a significant degree of complications which may be associated with an excessive reaction by the immune system. Herein, we report an analysis of cytokine and chemokine in an 11-year-old girl diagnosed with left ventricular dysfunction and cardiac tamponade complicated with COVID-19. She recovered without complications after intravenous immunoglobulin, dexamethasone, remdesivir, and surgical pericardial drainage. Cytokine concentrations had markedly increased in the pericardial fluid specimen; especially for interleukin-6 being disproportionate to its serum concentration. Cytokine storm originating from the pericardial cavity was considered an underlying mechanism of her condition.
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宮崎県における免疫グロブリン療法不応川崎病に対する 血漿交換療法の現状 査読あり
山本 絵里子
宮崎医会誌 47 ( 1 ) 36 - 40 2023年3月
記述言語：日本語 掲載種別：研究論文（学術雑誌）
MISC 【 表示 ／ 非表示 】
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(, 10.32388/74e1v5) 査読あり
Chida-Nagai A., Masaki N., Maeda K., Sasaki K., Sato H., Muneuchi J., Ochiai Y., Murayama H., Tahara M., Shiono A., Shinozuka A., Kono F., Machida D., Toyooka S., Sugimoto S., Nakamura K., Akagi S., Kondo M., Kasahara S., Kotani Y., Koizumi J., Oda K., Harada M., Nakajima D., Murata A., Nagata H., Yatsunami K., Kobayashi T., Matsunaga Y., Inoue T., Yamagishi H., Nakagawa N., Ohtani K., Yamamoto M., Ito Y., Hokosaki T., Kuwahara Y., Masutani S., Nomura K., Wada T., Sawada H., Abiko M., Takahashi T., Ishikawa Y., Okada S., Naitoh A., Toda T., Ando T., Masuzawa A., Hoshino S., Kawada M., Nomura Y., Ueno K., Ohashi N., Tachibana T., Cao Y., Ueda H., Yanagi S., Koide M., Mitsushita N., Higashi K., Minosaki Y., Hayashi T., Okamoto T., Kuraishi K., Ehara E., Ishida H., Horigome H., Murakami T., Takei K., Ishii T., Harada G., Hirata Y., Maeda J., Tatebe S., Ota C., Hayabuchi Y., Sakazaki H., Sasaki T., Hirono K., Suzuki S., Yasuda M., Takeda A., Sawada M., Miyaji K., Kitagawa A., Nakai Y., Kakimoto N., Agematsu K., Manabe A., Saiki Y.
Frontiers in Cardiovascular Medicine 11 1369831 2024年
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：Frontiers in Cardiovascular Medicine
In the published article, an author name was incorrectly written as Madoka Sawai. The correct spelling is Madoka Sawada. The authors apologize for this error and state that this does not change the scientific conclusions of the article in any way. The original article has been updated.
受託研究受入実績 【 表示 ／ 非表示 】
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ウプトラビ錠0.2mg・0.4mg特定使用成績調査（長期使用に関する調査）
2018年06月 - 2023年09月
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レバチオ特定使用成績調査（小児を対象とした長期使用に関する調査）
2018年02月 - 2022年08月
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ヴォリブリス錠2.5mg使用成績調査
2015年10月 - 2020年01月