著者

Megumi Fukuyama Minoru Horie Koichi Kato Hisaaki Aoki Shuhei Fujita Yoko Yoshida Hisanori Sakazaki Takako Toda Michihiko Ueno Gaku Izumi Nobuo Momoi Jun Muneuchi Takeru Makiyama Yoshihisa Nakagawa Seiko Ohno

出版者

The Japanese Circulation Society

雑誌

Circulation Journal (ISSN:13469843)

巻号頁・発行日

pp.CJ-23-0195, (Released:2023-06-28)

参考文献数

38

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Background: Cardiac calmodulinopathy, characterized by a life-threatening arrhythmia and sudden death in the young, is extremely rare and caused by genes encoding calmodulin, namely calmodulin 1 (CALM1), CALM2, and CALM3.Methods and Results: We screened 195 symptomatic children (age 0–12 years) who were suspected of inherited arrhythmias for 48 candidate genes, using a next-generation sequencer. Ten probands were identified as carrying variants in any of CALM1–3 (5%; median age 5 years), who were initially diagnosed with long QT syndrome (LQTS; n=5), catecholaminergic polymorphic ventricular tachycardia (CPVT; n=3), and overlap syndrome (n=2). Two probands harbored a CALM1 variant and 8 probands harbored 6 CALM2 variants. There were 4 clinical phenotypes: (1) documented lethal arrhythmic events (LAEs): 4 carriers of N98S in CALM1 or CALM2; (2) suspected LAEs: CALM2 p.D96G and D132G carriers experienced syncope and transient cardiopulmonary arrest under emotional stimulation; (3) critical cardiac complication: CALM2 p.D96V and p.E141K carriers showed severe cardiac dysfunction with QTc prolongation; and (4) neurological and developmental disorders: 2 carriers of CALM2 p.E46K showed cardiac phenotypes of CPVT. Beta-blocker therapy was effective in all cases except cardiac dysfunction, especially in combination with flecainide (CPVT-like phenotype) and mexiletine (LQTS-like).Conclusions: Calmodulinopathy patients presented severe cardiac features, and their onset of LAEs was earlier in life, requiring diagnosis and treatment at the earliest age possible.

著者

Sakyo Hirai Hirotaka Sato Toshihiro Yamamura Koichi Kato Mariko Ishikawa Hirotaka Sagawa Jiro Aoyama Shoko Fujii Kyohei Fujita Toshinari Arai Kazutaka Sumita

出版者

The Japanese Society for Neuroendovascular Therapy

雑誌

Journal of Neuroendovascular Therapy (ISSN:18824072)

巻号頁・発行日

pp.oa.2022-0026, (Released:2022-07-30)

参考文献数

29

被引用文献数

1

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Objective: CT perfusion (CTP) provides various hemodynamic parameters. However, it is unclear which CTP parameters are useful in predicting clinical outcome in patients with acute ischemic stroke (AIS).Methods: Between February 2019 and June 2021, patients with anterior circulation large-vessel occlusion who achieved successful recanalization within 8 hours after stroke onset were included. The relative CTP parameter values analyzed by the reformulated singular value decomposition (SVD) method in the affected middle cerebral artery territories compared to those in the unaffected side were calculated. In addition, the ischemic core volume (ICV) was evaluated using a Bayesian Vitrea. The final infarct volume (FIV) was assessed by 24-hour MRI. The correlation between these CTP-derived values and clinical outcome was assessed.Results: Forty-two patients were analyzed. Among the CTP-related parameters, the ICV, relative cerebral blood volume (rCBV), and relative mean transit time (rMTT) showed a strong correlation with the FIV (ρ = 0.74, p <0.0001; ρ = −0.67, p <0.0001; and ρ = −0.66, p <0.0001, respectively). In multivariate analysis, rCBV, rMTT, and ICV were significantly associated with good functional outcome, which was defined as a modified Rankin Scale score ≤2 (OR, 6.87 [95% CI, 1.20–39.30], p = 0.0303; OR, 11.27 [95% CI, 0.97–130.94], p = 0.0269; and OR, 36.22 [95% CI, 2.78–471.18], p = 0.0061, respectively).Conclusions: Among the CTP parameters analyzed by the SVD deconvolution algorithms, rCBV and rMTT could be useful imaging predictors of response to recanalization in patients with AIS, and the performances of these variables were similar to that of the ICV calculated by the Bayesian Vitrea.

著者

Masao Yoshinaga Yumiko Ninomiya Yuji Tanaka Megumi Fukuyama Koichi Kato Seiko Ohno Minoru Horie Hiromitsu Ogata

出版者

The Japanese Circulation Society

雑誌

Circulation Journal (ISSN:13469843)

巻号頁・発行日

pp.CJ-23-0409, (Released:2023-12-01)

参考文献数

32

被引用文献数

1

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Background: This study was performed to clarify the clinical findings of pediatric patients diagnosed with long QT syndrome (LQTS) through electrocardiographic screening programs and to predict their outcome using Holter electrocardiographic approaches.Methods and Results: This retrospective study included pediatric patients with a Schwartz score of ≥3.5 who visited the National Hospital Organization Kagoshima Medical Center between April 2005 and March 2019. Resting 12-lead and Holter electrocardiograms were recorded at every visit. The maximum resting QTc and maximum Holter QTc values among all recordings were used for statistical analyses. To test the prognostic value of QTc for the appearance of cardiac events after the first hospital visit, receiver operating characteristic curves were used to calculate the area under the curve (AUC). Among 207 patients, 181 (87%) were diagnosed through screening programs. The prevalence of cardiac events after the first hospital visit was 4% (8/207). Among QTc at diagnosis, maximum resting QTc, and maximum Holter QTc, only maximum Holter QTc value was a predictor (P=0.02) of cardiac events after the hospital visit in multivariate regression analysis. The AUC of the maximum Holter QTc was significantly superior to that of maximum resting QTc.Conclusions: The maximum Holter QTc value can be used to predict the appearance of symptoms in pediatric patients with LQTS.

著者

Meng GUO Hiromasa MORIYAMA Yoshihiro HARADA Hideaki NISHIMURA Manabu KODAMA Shuichiro HIRAI Masami KADONAGA Koichi KATO Kazuyoshi FUSHINOBU

出版者

The Imaging Society of Japan

雑誌

日本画像学会誌 (ISSN:13444425)

巻号頁・発行日

vol.60, no.6, pp.580-591, 2021-12-10 (Released:2021-12-11)

参考文献数

20

被引用文献数

1

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Theoretical models to integrate pores size distribution and to analyze permeation of a droplet into coated paper are proposed. Droplet permeation into porous media plays an important role in the image formation of inkjet printing. In this study, pores size distribution of porous media of different coated papers was investigated using nitrogen adsorption-desorption method. Based on the pores size distribution, theoretical model for estimating permeation amount into coated paper of pico-scale droplet was proposed, with the consideration of Lucas-Washburn equation. In order to verify the theoretical model, experiments of droplet observation with pure water and several propylene glycol (PG) aqueous solutions were conducted. For some paper samples, results of theoretical calculation and experiments generally agree well. However, there are cases where the theoretical predictions show discrepancy. Possible reasons are considered to be deviation of pores size distribution. Moreover, the accuracy of estimated evaporation volume by Hu and Larson's Equation, and the complex shape of the pores in coated layer also contribute to the errors. The effect of these factors still needs to be further explored.

著者

Tomoki Nakayoshi Koichi Kato Eiji Kurimoto Akifumi Oda

出版者

The Pharmaceutical Society of Japan

雑誌

Biological and Pharmaceutical Bulletin (ISSN:09186158)

巻号頁・発行日

vol.44, no.7, pp.967-975, 2021-07-01 (Released:2021-07-01)

参考文献数

39

被引用文献数

1

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Isomerized aspartic acid (Asp) residues have previously been identified in various aging tissues, and are suspected to contribute to age-related diseases. Asp-residue isomerization occurs nonenzymatically under physiological conditions, resulting in the formation of three types of isomerized Asp (i.e., L-isoAsp, D-Asp, and D-isoAsp) residues. Asp-residue isomerization often accelerates protein aggregation and insolubilization, making structural biology analyses difficult. Recently, Sakaue et al. reported the synthesis of a ribonuclease A (RNase A) in which Asp121 was artificially replaced with different isomerized Asp residues, and experimentally demonstrated that the enzymatic activities of these artificial mutants were completely lost. However, their structural features have not yet been elucidated. In the present study, the three-dimensional (3D) structures of these artificial-mutant RNases A were predicted using molecular dynamics (MD) simulations. The 3D structures of wild-type and artificial-mutant RNases A were converged by 3000-ns MD simulations. Our computational data show that the structures of the active site and the formation frequencies of the appropriate catalytic dyad structures in the artificial-mutant RNases A were quite different from wild-type RNase A. These computational findings may provide an explanation for the experimental data which show that artificial-mutant RNases A lack enzymatic activity. Herein, MD simulations have been used to evaluate the influences of isomerized Asp residues on the 3D structures of proteins.