Faculty Profiles - KATO, Taichi

An electrocardiographic score to predict pulmonary hypertension in children with atrial septal defect. Reviewed International coauthorship Open Access

Murni IK, Kato T, Wirawan MT, Arafuri N, Hermawan K, Hartopo AB, Anggrahini DW, Nugroho S, Noormanto N, Emoto N, Dinarti LK

BMC pediatrics Vol. 23 ( 1 ) page： 288 2023.6

Authorship：Corresponding author Language：English Publishing type：Research paper (scientific journal) Publisher：BMC Pediatrics

Background: In limited resource settings, identification of factors that predict the occurrence of pulmonary hypertension(PH) in children with atrial septal defect(ASD) is important to decide which patients should be prioritized for defect closure to prevent complication. Echocardiography and cardiac catheterization are not widely available in such settings. No scoring system has been proposed to predict PH among children with ASD. We aimed to develop a PH prediction score using electrocardiography parameters for children with ASD in Indonesia. Methods: A cross-sectional study reviewing medical record including ECG record was conducted among all children with newly diagnosed isolated ASD admitted to Dr Sardjito Hospital in Yogyakarta, Indonesia during 2016–2018. Diagnosis of ASD and PH was confirmed through echocardiography and/or cardiac catheterization. Spiegelhalter Knill-Jones approach was used to develop PH prediction score. Accuracy of prediction score was performed using a receiver operating characteristic (ROC) curve. Results: Of 144 children, 50(34.7%) had PH. Predictors of pulmonary hypertension were QRS axis ≥120°, P wave ≥ 3 mm at lead II, R without S at V1, Q wave at V1, right bundle branch block (RBBB), R wave at V1, V2 or aVR > normal limit and S wave at V6 or lead I > normal limit. ROC curve from prediction scores yielded an area under the curve (AUC) 0.908(95% CI 0.85–0.96). Using the cut-off value 3.5, this PH prediction score had sensitivity of 76%(61.8–86.9), specificity 96.8%(91.0-99.3), positive predictive value 92.7%(80.5–97.5), negative predictive value 88.4%(82.2–92.6), and positive likelihood ratio 23.8(7.7–73.3). Conclusions: A presence of PH in children with ASD can be predicted by the simple electrocardiographic score including QRS axis ≥120°, P wave ≥3 mm at lead II, R without S at V1, Q wave at V1, RBBB, R wave at V1, V2 or aVR > normal limit and S wave at V6 or lead I > normal limit. A total score ≥ 3.5 shows a moderate sensitivity and high specificity to predict PH among children with ASD.

DOI： 10.1186/s12887-023-04102-1

Familial cardiac septal defect due to a novel nine-base deletion in TBX20. Reviewed International journal

Hidenori Yamamoto, Hidehito Inagaki, Satoshi Hayano, Hiroki Kurahashi, Taichi Kato

Pediatrics international : official journal of the Japan Pediatric Society Vol. 64 ( 1 ) page： e14995 2022.1

Conception by assisted reproductive technology in infants with critical congenital heart disease in Japan Reviewed Open Access

Yoshihito Morimoto, Kiyotaka Go, Hidenori Yamamoto, Yoshie Fukasawa, Michikazu Nakai, Eiji Morihana, Kazushi Yasuda, Hiroshi Nishikawa, Naoki Ohashi, Yoshiyuki Takahashi, Taichi Kato

REPRODUCTIVE BIOMEDICINE ONLINE Vol. 44 ( 1 ) page： 163 - 170 2022.1

Authorship：Corresponding author Language：English Publishing type：Doctoral thesis Publisher：ELSEVIER SCI LTD

Research question: What is the proportion of infants born as a result of assisted reproductive technology ART across different types of neonatal critical congenital heart disease (CCHD) in a Japanese population? Design: A retrospective analysis of 418 consecutive infants with CCHD that required catheter treatment or surgery within the first 28 days of life or ductal-dependent lesions, in two paediatric centres in Japan, between January 2014 and December 2019. The proportion of ART in infants with each type of CCHD was evaluated. The proportion of ART in infants with univentricular heart defect (UVH) compared with those with biventricular heart defect (BVH) was evaluated. Results: The study group included 229 boys and 189 girls, with a gestational age of 38 +/- 2 weeks. Overall, 61 infants (14.6%) were conceived by fertility treatment with 46 (11.0%) conceived by ART. Univentricular heart defect and BVH were identified in 111 infants (26.6%) and 307 infants (73.4%), respectively. The proportion of infants conceived by ART was significantly higher in UVH (16.2%) than in BVH (9.1%) (OR 2.28, 95% CI 1.11 to 4.68, P = 0.025), regardless of maternal age and maternal history of miscarriage. Conclusions: The proportion of ART in infants with CCHD, especially UVH, was high. These findings could form the basis of a rationale for carrying out fetal echocardiography in fetuses conceived by ART.

DOI： 10.1016/j.rbmo.2021.10.005

Electrocardiographic Diagnosis of Hypertrophic Cardiomyopathy in the Pre- and Post-Diagnostic Phases in Children and Adolescents Open Access

Yoshinaga Masao, Horigome Hitoshi, Ayusawa Mamoru, Yasuda Kazushi, Kogaki Shigetoyo, Doi Shozaburo, Tateno Sigeru, Ohta Kunio, Hokosaki Tatsunori, Nishihara Eiki, Iwamoto Mari, Sumitomo Naokata, Ushinohama Hiroya, Izumida Naomi, Tauchi Nobuo, Kato Yoshiaki, Kato Taichi, Chisaka Toshiyuki, Higaki Takashi, Yoneyama Tatsuya, Abe Katsumi, Nozaki Yoshihiro, Komori Akiko, Kawai Satoru, Ninomiya Yumiko, Tanaka Yuji, Nuruki Norihito, Sonoda Masahiro, Ueno Kentaro, Hazeki Daisuke, Nomura Yuichi, Sato Seiichi, Hirono Keiichi, Hosokawa Susumu, Takechi Fumie, Ishikawa Yuichi, Hata Tadayoshi, Ichida Fukiko, Ohno Seiko, Makita Naomasa, Horie Minoru, Matsushima Shouji, Tsutsui Hiroyuki, Ogata Hiromitsu, Takahashi Hideto, Nagashima Masami

Circulation Journal Vol. 86 ( 1 ) page： 118 - 127 2021.12

Language：English Publisher：The Japanese Circulation Society

Background:The usefulness of electrocardiographic (ECG) voltage criteria for diagnosing hypertrophic cardiomyopathy (HCM) in pediatric patients is poorly defined.Methods and Results:ECGs at the 1st grade (mean [±SD] age 6.6±0.3 years) were available for 11 patients diagnosed with HCM at around the 7th grade (13.2±0.3 years). ECGs were available for another 64 patients diagnosed with HCM in the 1st (n=15), 7th (n=32), and 10th (n=17) grades. Fifty-one voltage criteria were developed by grade and sex using 62,841 ECGs from the general population. Voltage criteria were set at the 99.95th percentile (1/2,000) point based on the estimated prevalence of childhood HCM (2.9 per 100,000 [1/34,483]) to decrease false negatives. Conventional criteria were from guidelines for school-aged children in Japan. Of 11 patients before diagnosis, 2 satisfied conventional criteria in 1st grade; 5 (56%) of the remaining 9 patients fulfilled 2 voltage criteria (R wave in limb-lead I [RI]+S wave in lead V3 [SV3] and R wave in lead V3 [RV3]+SV3). Robustness analysis for sensitivity showed RV3+SV3 was superior to RI+SV3. For all patients after diagnosis, RI+SV4 was the main candidate. However, conventional criteria were more useful than voltage criteria.Conclusions:Early HCM prediction was possible using RV3+SV3 in >50% of patients in 1st grade. Voltage criteria may help diagnose prediagnostic or early HCM, and prevent tragic accidents, although further prospective studies are required.

DOI： 10.1253/circj.CJ-21-0376

Successful treatment of a novel type I interferonopathy due to a de novo PSMB9 gene mutation with a Janus kinase inhibitor. Reviewed International journal

Shinsuke Kataoka, Nozomu Kawashima, Yusuke Okuno, Hideki Muramatsu, Shunsuke Miwata, Kotaro Narita, Motoharu Hamada, Norihiro Murakami, Rieko Taniguchi, Daisuke Ichikawa, Hironobu Kitazawa, Kyogo Suzuki, Eri Nishikawa, Atsushi Narita, Nobuhiro Nishio, Hidenori Yamamoto, Yoshie Fukasawa, Taichi Kato, Hiroyuki Yamamoto, Jun Natsume, Seiji Kojima, Ichizo Nishino, Takeshi Taketani, Hidenori Ohnishi, Yoshiyuki Takahashi

The Journal of allergy and clinical immunology Vol. 148 ( 2 ) page： 639 - 644 2021.8

Language：English Publishing type：Research paper (scientific journal)

BACKGROUND: Type I interferonopathies are a recently established subgroup of autoinflammatory diseases caused by mutations in genes associated with proteasome degradation or cytoplasmic RNA- and DNA-sensing pathways. OBJECTIVE: This study aimed to unveil the molecular pathogenesis of a patient with novel type I interferonopathy, for which no known genetic mutations have been identified. METHODS: We performed the whole-exome sequencing of a 1-month-old boy with novel type I interferonopathy. We also investigated proteasome activities using patient-derived B lymphoblastoid cell lines (LCLs) and normal LCLs transduced with the mutant gene. RESULTS: Whole-exome sequencing identified a de novo proteasome 20S subunit beta 9 (PSMB9) p.G156D mutation in the patient who developed fever, a chilblain-like skin rash, myositis, and severe pulmonary hypertension due to the hyperactivation of IFN-α. Patient-derived LCLs revealed reduced proteasome activities, and exogenous transduction of mutant PSMB9 p.G156D into normal LCLs significantly suppressed proteasome activities, and the endogenous PSMB9 protein was lost along with the reduction of other immunoproteasome subunits, PSMB8 and PSMB10 proteins. He responded to the administration of a Janus kinase inhibitor, tofacitinib, and he was successfully withdrawn from venoarterial extracorporeal membranous oxygenation. At age 7 months, he received an unrelated cord blood transplantation. At 2 years posttransplantation, he no longer required tofacitinib and experienced no disease recurrence. CONCLUSIONS: We present the case of a patient with a novel type I interferonopathy caused by a de novo PSMB9 p.G156D mutation that suppressed the wild-type PSMB9 protein expression. Janus kinase inhibitor and stem cell transplantation could be curative therapies in patients with severe interferonopathies.

DOI： 10.1016/j.jaci.2021.03.010

Phosphorylated proteome analysis of a novel germline ABL1 mutation causing an autosomal dominant syndrome with ventricular septal defect. Reviewed International journal

Hidenori Yamamoto, Satoshi Hayano, Yusuke Okuno, Atsuto Onoda, Kohji Kato, Noriko Nagai, Yoshie Fukasawa, Shinji Saitoh, Yoshiyuki Takahashi, Taichi Kato

International journal of cardiology Vol. 326 page： 81 - 87 2021.3

Authorship：Last author,　Corresponding author Language：English Publishing type：Research paper (scientific journal)

BACKGROUND: A gain-of-function mutation in germline ABL1 causes a syndrome including congenital heart defects. However, the molecular mechanisms of this syndrome remain unknown. In this study, we found a novel ABL1 mutation in a Japanese family with ventricular septal defect, finger contracture, skin abnormalities and failure to thrive, and the molecular mechanisms of these phenotypes were investigated. METHODS AND RESULTS: Whole-exome sequencing on several family members revealed a novel mutation (c.1522A > C, p.I508L) in the tyrosine kinase domain of ABL1, and complete co-segregation with clinical presentations was confirmed in all members. Wild-type and mutant ABL1 were transfected into human embryonic kidney 293 cells for functional analysis. Western blotting confirmed that tyrosine phosphorylation in STAT5, a substrate of ABL1, was enhanced, and the novel mutation was proved to be a gain-of-function mutation. Since this novel mutation in ABL1 enhances tyrosine kinase activity, phosphorylated proteome analysis was used to elucidate the molecular pathology. The proteome analysis showed that phosphorylation in proteins such as UFD1, AXIN1, ATRX, which may be involved in the phenotypes, was enhanced in the mutant group. CONCLUSIONS: The onset of congenital heart defects associated with this syndrome appears to involve a mechanism caused by UFD1 common to 22q.11.2 deletion syndrome. On the other hand, AXIN1 and ATRX may be important in elucidating the mechanisms of other phenotypes, such as finger contracture and failure to thrive. Verification of these hypotheses would lead to further understanding of the pathophysiology and the development of treatment methods.

DOI： 10.1016/j.ijcard.2020.10.032

Revision of diagnostic guidelines for Kawasaki disease (6th revised edition). Reviewed International journal

Tohru Kobayashi, Mamoru Ayusawa, Hiroyuki Suzuki, Jun Abe, Shuichi Ito, Taichi Kato, Masahiro Kamada, Junko Shiono, Kenji Suda, Keiji Tsuchiya, Tsuneyuki Nakamura, Yoshikazu Nakamura, Yuichi Nomura, Hiromichi Hamada, Ryuji Fukazawa, Kenji Furuno, Hiroyuki Matsuura, Tomoyo Matsubara, Masaru Miura, Kei Takahashi

Pediatrics international : official journal of the Japan Pediatric Society Vol. 62 ( 10 ) page： 1135 - 1138 2020.10

Mesenchymal stem/stromal cells stably transduced with an inhibitor of CC chemokine ligand 2 ameliorate bronchopulmonary dysplasia and pulmonary hypertension. Reviewed International journal

Toshihiko Suzuki, Yoshiaki Sato, Hidenori Yamamoto, Taichi Kato, Yuma Kitase, Kazuto Ueda, Haruka Mimatsu, Yuichiro Sugiyama, Atsuto Onoda, Shigeki Saito, Yoshiyuki Takahashi, Takayuki Nakayama, Masahiro Hayakawa

Cytotherapy Vol. 22 ( 4 ) page： 180 - 192 2020.4

Language：English Publishing type：Research paper (scientific journal)

Perinatal bronchopulmonary dysplasia (BPD) is defined as lung injury in preterm infants caused by various factors, resulting in serious respiratory dysfunction and high mortality. The administration of mesenchymal stem/stromal cells (MSCs) to treat/prevent BPD has proven to have certain therapeutic effects. However, MSCs can only weakly regulate macrophage function, which is strongly involved in the development of BPD. 7ND-MSCs are MSCs transfected with 7ND, a truncated version of CC chemokine ligand 2 (CCL2) that promotes macrophage activation, using a lentiviral vector. In the present study, we show in a BPD rat model that 7ND-MSC administration, but not MSCs alone, ameliorated the impaired alveolarization evaluated by volume density and surface area in the lung tissue, as well as pulmonary artery remodeling and pulmonary hypertension induced by BPD. In addition, 7ND-MSCs, but not MSCs alone, reduced M1 macrophages and the messenger RNA expressions of interleukin-6 and CCL2 in the lung tissue. Thus, the present study showed the treatment effect of 7ND-MSCs in a BPD rat model, which was more effective than that of MSCs alone.

DOI： 10.1016/j.jcyt.2020.01.009

A non-selective endothelin receptor antagonist bosentan modulates kinetics of bone marrow-derived cells in ameliorating pulmonary hypertension in mice Reviewed International journal Open Access

Kato Taichi, Mitani Yoshihide, Masuya Masahiro, Maruyama Junko, Sawada Hirofumi, Ohashi Hiroyuki, Ikeyama Yukiko, Otsuki Shoichiro, Yodoya Noriko, Shinohara Tsutomu, Miyata Eri, Zhang Erquan, Katayama Naoyuki, Shimpo Hideto, Maruyama Kazuo, Komada Yoshihiro, Hirayama Masahiro

PULMONARY CIRCULATION Vol. 10 ( 2 ) page： 2045894020919355 - 2045894020919355 2020.4

Authorship：Lead author Language：English Publishing type：Research paper (scientific journal)

The aim of this study was to investigate whether a dual endothelin receptor antagonist bosentan modulates the kinetics of bone marrow-derived stem cells in inhibiting the development of pulmonary hypertension. Bone marrow chimeric mice, transplanted with enhanced green fluorescent protein (eGFP)-positive bone marrow mononuclear cells, were exposed to hypobaric hypoxia or kept in the ambient air, and were daily treated with bosentan sodium salt or saline for 21 days. After the treatment period, right ventricular pressure was measured and pulmonary vascular morphometry was conducted. Incorporation of bone marrow-derived cells was analyzed by immunohistochemistry. Gene expression and protein level in the lung tissue were evaluated by quantitative real-time PCR and western blotting, respectively. The results showed that, in hypoxic mice, right ventricular pressure and the percentage of muscularized vessel were increased and pulmonary vascular density was decreased, each of which was reversed by bosentan. Bone marrow-derived endothelial cells and macrophages in lungs were increased by hypoxia. Bosentan promoted bone marrow-derived endothelial cell incorporation but inhibited macrophage infiltration into lungs. Quantitative real-time PCR analysis revealed that interleukin 6, stromal cell-derived factor-1, and monocyte chemoattractant protein-1 were upregulated by hypoxia, in which interleukin 6 and monocyte chemoattractant protein-1 were downregulated and stromal cell-derived factor-1 was upregulated by bosentan. Protein level of endothelial nitric oxide synthase (eNOS) in the whole lung was significantly upregulated by hypoxia, which was further upregulated by bosentan. Bosentan modulated kinetics of bone marrow-derived ECs and macrophages and related gene expression in lungs in ameliorating pulmonary hypertension in mice. Altered kinetics of bone marrow-derived stem cells may be a novel mechanism of the endothelin receptor blockade in vivo and confer a new understanding of the therapeutic basis for pulmonary hypertension.

DOI： 10.1177/2045894020919355

The ALK-1/SMAD/ATOH8 axis attenuates hypoxic responses and protects against the development of pulmonary arterial hypertension. Reviewed International coauthorship International journal

Morikawa M, Mitani Y, Holmborn K, Kato T, Koinuma D, Maruyama J, Vasilaki E, Sawada H, Kobayashi M, Ozawa T, Morishita Y, Bessho Y, Maeda S, Ledin J, Aburatani H, Kageyama R, Maruyama K, Heldin CH, Miyazono K

Science signaling Vol. 12 ( 607 ) page： eaay4430 - eaay4430 2019.11

Language：English Publishing type：Research paper (scientific journal) Publisher：American Association for the Advancement of Science (AAAS)

Dysregulated bone morphogenetic protein (BMP) signaling in endothelial cells (ECs) is implicated in vascular diseases such as pulmonary arterial hypertension (PAH). Here, we showed that the transcription factor ATOH8 was a direct target of SMAD1/5 and was induced in a manner dependent on BMP but independent of Notch, another critical signaling pathway in ECs. In zebrafish and mice, inactivation of <italic>Atoh8</italic> did not cause an arteriovenous malformation–like phenotype, which may arise because of dysregulated Notch signaling. In contrast, <italic>Atoh8-</italic>deficient mice exhibited a phenotype mimicking PAH, which included increased pulmonary arterial pressure and right ventricular hypertrophy. Moreover, <italic>ATOH8</italic> expression was decreased in PAH patient lungs. We showed that in cells, ATOH8 interacted with hypoxia-inducible factor 2α (HIF-2α) and decreased its abundance, leading to reduced induction of HIF-2α target genes in response to hypoxia. Together, these findings suggest that the BMP receptor type II/ALK-1/SMAD/ATOH8 axis may attenuate hypoxic responses in ECs in the pulmonary circulation and may help prevent the development of PAH.

DOI： 10.1126/scisignal.aay4430

Investigation of novel variations of ORAI1 gene and their association with Kawasaki disease

Frequent intragenic microdeletions of elastin in familial supravalvul araortic stenosis (vol 274, pg 290, 2019) Reviewed International journal

Hayano Satoshi, Okuno Yusuke, Tsutsumi Makiko, Inagaki Hidehito, Fukasawa Yoshie, Kurahashi Hiroki, Kojima Seiji, Takahashi Yoshiyuki, Kato Taichi

INTERNATIONAL JOURNAL OF CARDIOLOGY Vol. 292 page： 283 - 283 2019.10

Thiha K., Mashimo Y., Suzuki H., Hamada H., Hata A., Hara T., Tanaka T., Ito K., Onouchi Y., Masuda H., Kobayashi T., Jibiki T., Yamazaki T., Ohkuma Y., Fujiwara M., Kobayashi T., Takeuchi H., Ouchi K., Mizuno Y., Fuse S., Fukazawa N., Saji T., Takatsuki S., Nishimura K., Hamada H., Fukazawa R., Nishumura H., Aso K., Matsubara T., Mizuno T., Iwashima S., Ayusawa M., Ikeda K., Kosuda T., Hashimoto K., Hirasawa K., Miura M., Somura J., Toba E., Hirono K., Nomura Y., Arakawa H., Ogata S., Kajino H., Kawamura S., Aoyagi H., Suzuki H., Ichinose K., Shimozono A., Kato Y., Higashikawa M., Kawamura Y., Misawa M., Nagai N., Kato T., Nagata D., Okamoto A., Suzuki H., Kishiro M., Shiono J., Higashi K., Yokoyama N., Ebata R., Onouchi Y.

Journal of Human Genetics Vol. 64 ( 6 ) page： 511 - 519 2019.6

Publisher：Journal of Human Genetics

ORAI1 encodes a calcium channel essential in the store-operated calcium entry mechanism. A previous genetic association study identified a rare in-frame insertion variant of ORAI1 conferring Kawasaki disease (KD). To deepen our understanding of the involvement of rare variants of ORAI1 in KD pathogenesis, we investigated 3812 patients with KD and 2644 healthy individuals for variations in the protein-coding region of ORAI1. By re-sequencing the study participants’ DNA, 27 variants with minor allele frequencies (MAFs) < 0.01 that had not been examined in the previous study were identified. Although no significant association with KD was observed either in single-variant analyses or in a collapsing method analysis of the 27 variants, stratification by MAFs, variant types, and predicted deleteriousness revealed that six rare, deleterious, missense variants (MAF < 0.001, CADD C-score ≥ 20) were exclusively present in KD patients, including three refractory cases (OR = ∞, P = 0.046). The six missense variants include p.Gly98Asp, which has been demonstrated to result in gain of function leading to constitutive Ca2+ entry. Conversely, five types of frameshift variants, all identified near the N terminus and assumed to disrupt ORAI1 function, showed an opposite trend of association (OR = 0.35, P = 0.24). These findings support our hypothesis that genetic variations causing the upregulation of the Ca2+/NFAT pathway confer susceptibility to KD. Our findings also provide insights into the usefulness of stratifying the variants based on their MAFs and on the direction of the effects on protein function when conducting association studies using the gene-based collapsing method.

DOI： 10.1038/s10038-019-0588-2

Guidelines for Heart Disease Screening in Schools (JCS 2016/JSPCCS 2016) - Digest Version Reviewed International journal

Sumitomo N, Baba R, Doi S, Higaki T, Horigome H, Ichida F, Ishikawa H, Iwamoto M, Izumida N, Kasamaki Y, Kuga K, Mitani Y, Musha H, Nakanishi T, Yoshinaga M, Abe K, Ayusawa M, Hokosaki T, Kato T, Kato Y, Ohta K, Sawada H, Ushinohama H, Yoshiba S, Atarashi H, Hirayama A, Horie M, Nagashima M, Niwa K, Ogawa S, Okumura K, Tsutsui H

CIRCULATION JOURNAL Vol. 82 ( 9 ) page： 2385 - 2444 2018.8

Association of Severity of Coronary Artery Aneurysms in Patients With Kawasaki Disease and Risk of Later Coronary Events Reviewed International journal Open Access

Masaru Miura, Tohru Kobayashi, Tetsuji Kaneko, Mamoru Ayusawa, Ryuji Fukazawa, Naoya Fukushima, Shigeto Fuse, Kenji Hamaoka, Keiichi Hirono, Taichi Kato, Yoshihide Mitani, Seiichi Sato, Shinya Shimoyama, Junko Shiono, Kenji Suda, Hiroshi Suzuki, Jun Maeda, Kenji Waki, Hitoshi Kato, Tsutomu Saji, Hiroyuki Yamagishi, Aya Ozeki, Masako Tomotsune, Makiko Yoshida, Yohei Akazawa, Kentaro Aso, Shouzaburoh Doi, Yoshi Fukasawa, Kenji Furuno, Yasunobu Hayabuchi, Miyuki Hayashi, Takafumi Honda, Norihisa Horita, Kazuyuki Ikeda, Masahiro Ishii, Satoru Iwashima, Masahiro Kamada, Masahide Kaneko, Hiroshi Katyama, Yoichi Kawamura, Atushi Kitagawa, Akiko Komori, Kenji Kuraishi, Hiroshi Masuda, Shinichi Matsuda, Satoshi Matsuzaki, Sayaka Mii, Tomoyuki Miyamoto, Yuji Moritou, Noriko Motoki, Kiyoshi Nagumo, Tsuneyuki Nakamura, Eiki Nishihara, Yuichi Nomura, Shohei Ogata, Hiroyuki Ohashi, Kenichi Okumura, Daisuke Omori, Tetsuya Sano, Eisuke Suganuma, Tsutomu Takahashi, Shinichi Takatsuki, Atsuhito Takeda, Masaru Terai, Manatomo Toyono, Kenichi Watanabe, Makoto Watanabe, Masaki Yamamoto, Kenichiro Yamamura

JAMA Pediatrics Vol. 172 ( 5 ) page： e180030 - e180030 2018.5

A New Z-Score Curve of the Coronary Arterial Internal Diameter Using the Lambda-Mu-Sigma Method in a Pediatric Population. Reviewed

Kobayashi T, Fuse S, Sakamoto N, Mikami M, Ogawa S, Hamaoka K, Arakaki Y, Nakamura T, Nagasawa H, Kato T, Jibiki T, Iwashima S, Yamakawa M, Ohkubo T, Shimoyama S, Aso K, Sato S, Saji T; Z Score Project Investigators.

JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Vol. 2016 ( 29 ) page： 794-801 2016.10

Hydrogen ameliorates pulmonary hypertension in rats by anti-inflammatory and anti-oxidant effects. Reviewed

Kishimoto Y, Kato T, Ito M, Azuma Y, Fukasawa Y, Ohno K, Kojima S.

JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Vol. 150 ( 3 ) page： 645-654.e3. 2015

Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease: a prospective, randomised, open, blinded-endpoint trial. Reviewed

Kobayashi T, Saji T, Otani T, Takeuchi K, Nakamura T, Arakawa H, Kato T, Hara T, Hamaoka K, Ogawa S, Miura M, Nomura Y, Fuse S, Ichida F, Seki M, Fukazawa R, Ogawa C, Furuno K, Tokunaga H, Takatsuki S, Hara S, Morikawa A, RAISE Study Group Investigators.

Vol. 379 page： 1613-1620 2012

A Netherton syndrome infant complicated with infective endocarditis. Reviewed

Tago M, Takeichi T, Suzuki Y, Yoshikawa T, Sato Y, Kato T, Go K, Sakaida T, Muro Y, Akiyama M

The Journal of dermatology Vol. 52 ( 1 ) page： e12 - e13 2025.1

Frequency of SARS-CoV-2 antecedent infection in patients with Kawasaki disease Reviewed

Go K., Kawano Y., Nagai N., Omori D., Morimoto Y., Yamamoto H., Fukasawa Y., Ito Y., Takahashi Y., Kato T.

Nagoya Journal of Medical Science Vol. 87 ( 1 ) page： 76 - 83 2025

Authorship：Last author,　Corresponding author Language：English Publishing type：Research paper (scientific journal) Publisher：Nagoya Journal of Medical Science

Since the COVID-19 pandemic, it has been found that SARS-CoV-2 antecedent infection can cause multisystem inflammatory syndrome in children. However, the frequency of SARS-CoV-2 antecedent infection in Kawasaki disease (KD) is unknown. The purpose of this study was to investigate the frequency of SARS-CoV-2 infection that preceded the onset of KD. This study is a multi-center observational study. The subjects were patients who were diagnosed with KD at 4 hospitals from April 1, 2020 to August 31, 2022. Serum SARS-CoV-2 IgM and total antibody including IgG levels were measured by the chemiluminescence immunoassay method before and after treatment for KD. A total of 55 patients participated in the study. The first antibody measurement before the initial treatment was performed on a median of 4th days of illness and the second antibody measurement was performed after the initial treatment on a median of 12th days of illness. No patient had a significant increase in SARS-CoV-2 IgM levels in two measurements. Only one patient (1.8%) had elevated total antibodies including IgG, and the patient had a history of COVID-19 6 months before the onset of KD. SARS-CoV-2 antecedent infection before the onset of KD was not observed in this study, and strong association between development of KD and SARS-CoV-2 infection was not suggested.

DOI： 10.18999/nagjms.87.1.76

Balloon angioplasty for bilateral severe peripheral pulmonary artery stenoses associated with chondrodysplasia punctata: a case report. Reviewed Open Access

Go K, Yasuda K, Kato T

Cardiology in the young Vol. 34 ( 11 ) page： 2495 - 2498 2024.11

Authorship：Last author,　Corresponding author Language：English Publishing type：Research paper (scientific journal) Publisher：Cardiology in the Young

We present a 2-month-old girl with chondrodysplasia punctata in whom bilateral peripheral pulmonary artery stenoses were successfully treated by transcatheter balloon angioplasty. The minimal diameters of the right and left pulmonary arteries of 1.3 mm and 1.1 mm, respectively, which were dilated using a 4-mm balloon catheter. Balloon angioplasty is feasible and effective for relieving peripheral pulmonary artery stenosis in patients with chondrodysplasia punctata.

DOI： 10.1017/S1047951124036229

A familial case of aortic dilatation with p.Tyr470Cys in TGFBR2 in which the phenotype included only vascular lesions. Reviewed

Yamamoto H, Tanabe A, Kato T

Cardiology in the young page： 1 - 3 2024.10

Authorship：Last author,　Corresponding author Language：English Publishing type：Research paper (scientific journal) Publisher：Cardiology in the Young

Hereditary connective tissue diseases have different risks of aortic dissection depending on the causative gene. We report a family with no extravascular phenotype and a clinical diagnosis of familial thoracic aortic aneurysm and dissection, but genetic testing confirmed p.Tyr470Cys in TGFBR2, which is typically the responsible gene for Loeys-Dietz syndrome. Validation of the clinical diagnosis by genetic testing is warranted.

DOI： 10.1017/S104795112402585X

Effects of General Anesthesia on the Results of Cardiac Catheterization in Pediatric Patients with Ventricular Septal Defect Reviewed Open Access

Go K, Kato T, Kito M, Morimoto Y, Kawai S, Yamamoto H, Fukasawa Y, Yasuda K

Congenital Heart Disease Vol. 18 ( 2 ) page： 235 - 243 2023.3

Language：English Publishing type：Research paper (scientific journal) Publisher：Congenital Heart Disease

Background: There is no gold standard sedation method for pediatric cardiac catheterization. In congenital heart diseases with intracardiac shunts, hemodynamic parameters are prone to change depending on the ventilation conditions and anesthetics, although few studies have examined these effects. The purpose of this study was to investigate the effects of two different sedation methods on the hemodynamic parameters. Methods: This study retrospectively evaluated consecutive patients with ventricular septal defect (VSD) below 1 year of age who underwent cardiac catheterization at Aichi Children’s Health and Medical Center, who were divided into age-and VSD diameter-matched general anesthesia (GA) and monitored anesthesia care (MAC) under the natural airway groups (n = 40 each), for comparison of hemodynamic parameters. Results: In the GA group, arterial blood pH and arterial partial pressure of oxygen were significantly higher (p < 0.01), whereas arterial partial pressure of carbon dioxide was significantly lower than in the MAC group (p < 0.01). Mean pulmonary artery pressure (p < 0.05) and systemic blood pressure (p < 0.01) were lower in the GA group. Pulmonary vascular resistance index (p < 0.01) and systemic vascular resistance index (p < 0.01) were also significantly lower in the GA group than the MAC group. There were no significant differences in pulmonary blood flow index, systemic blood flow index, and pulmonary/systemic blood flow ratio between the two groups. Conclusions: Cardiac catheterization under GA in VSD patients results in different hemodynamic parameters compared to that under MAC. In par-ticular, when using pulmonary artery pressure and pulmonary vascular resistance measured under GA for judg-ment regarding the surgical indications or perioperative management, consideration should be given to the fact that these parameters might be lower compared to those measured under MAC.

DOI： 10.32604/chd.2023.027590

Analysis of Coronary Arterial Aneurysm Regression in Patients With Kawasaki Disease by Aneurysm Severity: Factors Associated With Regression. Reviewed International journal Open Access

Taichi Kato, Masaru Miura, Tohru Kobayashi, Tetsuji Kaneko, Naoya Fukushima, Kenji Suda, Jun Maeda, Shinya Shimoyama, Junko Shiono, Keiichi Hirono, Kazuyuki Ikeda, Seiichi Sato, Fujito Numano, Yoshihide Mitani, Kenji Waki, Mamoru Ayusawa, Ryuji Fukazawa, Shigeto Fuse

Journal of the American Heart Association Vol. 12 ( 3 ) page： e022417 2023.2

Language：English Publishing type：Research paper (scientific journal)

Background Coronary arterial aneurysms (CAAs) associated with Kawasaki disease (KD) significantly affect prognosis. However, the clinical course of CAAs and factors associated with CAA regression have not been well analyzed. Methods and Results The cohort of the Z-Score 2nd Project Stage study, a multicenter, retrospective, cohort study involving 44 institutions in Japan including 1006 patients with KD, was examined. CAAs were classified by the z score of their internal diameter in the acute phase: small (z<5), medium (5≤z<10), and large (z≥10). The lower limit of small CAA was based on the Japanese Ministry of Health, Labour and Welfare criteria. In the right coronary artery, the CAA regression rates 10 years after diagnosis were 95.5% for small, 83.2% for medium, and 36.3% for large. In the proximal left anterior descending artery, the regression rates 10 years after diagnosis were 95.3% for small, 80.1% for medium, and 28.8% for large. Cox regression analysis showed that diagnosis under the age of 1 year and onset of KD in 2010 to 2012 for the right coronary artery and the left anterior descending artery, and female for the right coronary artery were significantly associated with a high regression rate, whereas large CAAs for the right coronary artery and the left anterior descending artery were significantly associated with a low regression rate. Conclusions The current study, the largest Japanese study of its kind, found that small aneurysm, recent onset, and diagnosis under the age of 1 year predict regression, and that even giant aneurysms could regress. These data may contribute to long-term management of coronary aneurysms. Registration URL: https://www.umin.ac.jp/ctr/; Unique identifier: UMIN000010606.

DOI： 10.1161/JAHA.121.022417

Case report: High-dose epoprostenol therapy in pediatric patients with pulmonary hypertension and developmental lung disease. Reviewed Open Access

Fukasawa Y, Yamamoto H, Ito M, Saito A, Go K, Morimoto Y, Yasuda K, Sato Y, Hayakawa M, Kato T

Frontiers in pediatrics Vol. 11 page： 1116434 2023

Authorship：Last author,　Corresponding author Language：English Publishing type：Research paper (scientific journal) Publisher：Frontiers in Pediatrics

Pulmonary hypertension (PH) with developmental lung disease is a life-threatening disease and accounts for 10%–12% of pediatric PH patients. Administration of specific pulmonary vasodilators to pediatric PH patients has brought about improvement of their long-term prognosis. Intravenous epoprostenol therapy is a gold standard therapy for severe idiopathic pulmonary arterial hypertension (IPAH), but there are few reports demonstrating the efficacy of epoprostenol for pediatric PH patients with developmental lung disease, especially when treating with high doses of epoprostenol. Two cases of pediatric PH patients with alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) and congenital diaphragmatic hernia (CDH) with bronchopulmonary dysplasia (BPD), respectively, treated with epoprostenol above 100 ng/kg/min are presented. In these two cases, severe PH was improved significantly by an aggressive increase of the epoprostenol infusion rate with administration of oral pulmonary vasodilators and appropriate respiratory management, without any significant adverse effects. High-dose epoprostenol therapy may be one of the therapeutic options in pediatric PH patients with developmental lung disease.

DOI： 10.3389/fped.2023.1116434

Using Biomarkers to Predict Unresponsiveness to Initial Immunoglobulin for Kawasaki Disease Patients Invited

Kato Taichi

Circulation Journal Vol. 86 ( 6 ) page： 984 - 985 2022.5

Tension hemothorax 1 week after pericardiocentesis associated with thrombocytopenia. Reviewed

Yamamoto H, Go K, Morimoto Y, Fukasawa Y, Kato T

Pediatrics international : official journal of the Japan Pediatric Society Vol. 64 ( 1 ) page： e15395 2022.1

Echocardiography Monitoring of Pulmonary Hypertension after Pediatric Hematopoietic Stem Cell Transplantation: Pediatric Pulmonary Arterial Hypertension and Pulmonary Veno-Occlusive Disease after Hematopoietic Stem Cell Transplantation. Reviewed International journal Open Access

Nozomu Kawashima, Yoshie Fukasawa, Eri Nishikawa, Keiko Ohta-Ogo, Hatsue Ishibashi-Ueda, Motoharu Hamada, Daisuke Ichikawa, Atsushi Narita, Yusuke Okuno, Hideki Muramatsu, Nobuhiro Nishio, Seiji Kojima, Taichi Kato, Yoshiyuki Takahashi

Transplantation and cellular therapy Vol. 27 ( 9 ) page： 786.e1 - 786.e8 2021.9

Language：English Publishing type：Research paper (scientific journal)

Pulmonary hypertension (PH) is associated with high morbidity in children undergoing hematopoietic stem cell transplantation (HSCT). However, owing to the lack of sequential echocardiography, the nature of the condition is not fully understood. This study was conducted to investigate whether routine echocardiography performed after HSCT could detect patients with PH at an earlier stage and elucidate the role of intervention using tadalafil. The study population comprised 93 consecutive children age <18 years who underwent a total of 109 HSCTs. All patients underwent routine transthoracic echocardiography during HSCT. Four children (4%) with a median age of 4 years (range, 0.7 to 6 years) were found to have PH, and their median tricuspid regurgitation peak velocity (TRV) was 4.1 m/s (range, 3.5 to 4.2 m/s). PH was diagnosed at a median of 52 days (range, 21 to 118 days) after HSCT. Three of them were diagnosed with neuroblastoma, and 1 was diagnosed with infantile leukemia. One patient developed PH after autologous HSCT, and 3 received killer immunoglobulin-like receptor ligand-mismatched cord blood. Busulfan was used for conditioning in all patients, and the proportion of patients receiving this medication was significantly higher in the PH group compared with the non-PH group (100% versus 30%; P = .011). Three of the 4 patients had a durable response (TRV ≤2.8 m/s) at a median of 46 days (range, 14 to 79 days) after starting treatment with tadalafil. No patient experienced exacerbation of PH, and treatment was completed at median of 96 days (range, 46 to 212 days). Our data suggest that routine echocardiography monitoring after HSCT should be considered in children receiving busulfan, although the precise follow-up timing needs further study. In addition, safe and effective administration of tadalafil must be ensured by close monitoring.

DOI： 10.1016/j.jtct.2021.05.017

Serum tenascin-C predicts resistance to steroid combination therapy in high-risk Kawasaki disease: a multicenter prospective cohort study. Reviewed

Yoshikane Y, Okuma Y, Miyamoto T, Hashimoto J, Fukazawa R, Kato T, Takeda A, Suda K, Matsushita T, Hiroe M, Imanaka-Yoshida K

Pediatric rheumatology online journal Vol. 19 ( 1 ) page： 82 2021.6

Language：English Publisher：Pediatric Rheumatology

Background: Tenascin-C (TN-C) is an extracellular matrix glycoprotein related to tissue inflammation. Our previous retrospective study conducted in 2016 revealed that the serum tenascin-C level was higher in patients with Kawasaki disease (KD) who were resistant to intravenous immunoglobulin (IVIG) and developed coronary artery lesions (CALs). The present study is a prospective cohort study to assess if the serum level of tenascin-C could be used as a novel biomarker to predict the risk of resistance to initial treatment for high-risk patients. Methods: A total of 380 KD patients were registered and provided serum samples for tenascin-C measurement before commencing their initial treatment. Patients who did not meet the inclusion criteria were excluded from analysis; of the 181 remaining subjects, there were 144 low-risk patients (Kobayashi score: ≤4 points) and 37 high-risk patients (Kobayashi score: ≥5 points). The initial treatments for low-risk patients and high-risk patients were conventional therapy (IVIG with aspirin) and prednisolone combination therapy, respectively. The patient clinical and laboratory data, including the serum tenascin-C level, were compared between initial treatment responders and non-responders. Results: In the low-risk patients, there was no significant difference in the median levels of serum tenascin-C between the initial therapy responders and non-responders. However, in the high-risk patients, the median serum tenascin-C level in initial therapy non-responders was significantly higher than that in initial therapy responders (175.8 ng/ml vs 117.6 ng/ml). Conclusions: Serum tenascin-C could be a biomarker for predicting the risk of high-risk patients being non-responsive to steroid combination therapy. Trial registration: This study was a prospective cohort study. It was approved by the ethics committee of each institute and performed in accordance with the Declaration of Helsinki.

DOI： 10.1186/s12969-021-00562-w

Ventricular tachycardia and chest pain due to foreign body in the pericardium caused by self-injurious behaviour Reviewed

Comprehensive pathogen detection in sera of Kawasaki disease patients by high-throughput sequencing: a retrospective exploratory study. Reviewed International journal Open Access

Yuka Torii, Kazuhiro Horiba, Satoshi Hayano, Taichi Kato, Takako Suzuki, Jun-Ichi Kawada, Yoshiyuki Takahashi, Seiji Kojima, Yusuke Okuno, Tomoo Ogi, Yoshinori Ito

BMC pediatrics Vol. 20 ( 1 ) page： 482 - 482 2020.10

Language：English Publishing type：Research paper (scientific journal)

BACKGROUND: Kawasaki disease (KD) is an idiopathic systemic vasculitis that predominantly damages coronary arteries in children. Various pathogens have been investigated as triggers for KD, but no definitive causative pathogen has been determined. As KD is diagnosed by symptoms, several days are needed for diagnosis. Therefore, at the time of diagnosis of KD, the pathogen of the trigger may already be diminished. The aim of this study was to explore comprehensive pathogens in the sera at the acute stage of KD using high-throughput sequencing (HTS). METHODS: Sera of 12 patients at an extremely early stage of KD and 12 controls were investigated. DNA and RNA sequences were read separately using HTS. Sequence data were imported into the home-brew meta-genomic analysis pipeline, PATHDET, to identify the pathogen sequences. RESULTS: No RNA virus reads were detected in any KD case except for that of equine infectious anemia, which is known as a contaminant of commercial reverse transcriptase. Concerning DNA viruses, human herpesvirus 6B (HHV-6B, two cases) and Anelloviridae (eight cases) were detected among KD cases as well as controls. Multiple bacterial reads were obtained from KD and controls. Bacteria of the genera Acinetobacter, Pseudomonas, Delfita, Roseomonas, and Rhodocyclaceae appeared to be more common in KD sera than in the controls. CONCLUSION: No single pathogen was identified in serum samples of patients at the acute phase of KD. With multiple bacteria detected in the serum samples, it is difficult to exclude the possibility of contamination; however, it is possible that these bacteria might stimulate the immune system and induce KD.

DOI： 10.1186/s12887-020-02380-7

Two-incision technique for the subcutaneous implantable cardioverter defibrillator Reviewed International journal

Nomura Yoji, Kato Taichi, Nishihara Eiki, Morishima Itsuro, Kuraishi Kenji

PEDIATRICS INTERNATIONAL Vol. 62 ( 6 ) page： 736 - 738 2020.6

A Diversified Approach for the Prevention of Pediatric Sudden Cardiovascular Death in Japan Invited International journal Open Access

Kato Taichi

CIRCULATION JOURNAL Vol. 84 ( 4 ) page： 544 - 545 2020.3

Influence of Percutaneous Occlusion of Atrial Septal Defect on Left Atrial Function Evaluated Using 2D Speckle Tracking Echocardiography Reviewed International journal

Suzuki Kazutaka, Kato Taichi, Koyama Satoshi, Shinohara Tsutomu, Inukai Sachiko, Sato Jun, Yamamoto Hidenori, Omori Daisuke, Yoshida Shuichiro, Takeda Sho, Nishikawa Hiroshi, Ohashi Naoki, Sakurai Hajime, Saitoh Shinji

International Heart Journal Vol. 61 ( 1 ) page： 83 - 88 2020.1

Language：English Publishing type：Research paper (scientific journal) Publisher：International Heart Journal Association

Percutaneous occlusion of atrial septal defect (ASD) has recently become a standard therapeutic strategy, but little is known about left atrial (LA) function thereafter. The present study aimed to determine LA function in 43 children with ASD and 13 controls based on LA strain measured by two-dimensional echocardiographic speckle tracking (2DE-ST). Among these children, 12 underwent surgery (ASD-S), 31 had device closure (ASD-D), and 13 were included as controls. LA strain was significantly decreased after ASD-D but was not significantly altered after ASD-S, indicating that percutaneous occlusion of an ASD might decrease LA function. Furthermore, the size of the ASD device negatively correlated with LA strain. These results imply that ASD occlusion devices negatively influence LA function and might be important when decided therapeutic strategies for ASD. LA strain measured by 2DE-ST should become a good indicator of LA function after ASD treatment in children.

DOI： 10.1536/ihj.19-173

Severe fetal cardiomegaly caused by multiple non-giant placental chorioangiomas Reviewed International journal

Yamamoto Hidenori, Fukasawa Yoshie, Kato Taichi

CARDIOLOGY IN THE YOUNG Vol. 29 ( 12 ) page： 1559 - 1560 2019.12

Prediction of postnatal clinical course in primary congenital dilated cardiomyopathy Reviewed International journal

Yamamoto Hidenori, Fukasawa Yoshie, Ohashi Naoki, Yokoyama Takehiko, Suzuki Kazutaka, Ota Takaya, Yasuda Kazushi, Omoya Kentaro, Takahashi Yoshiyuki, Kato Taichi

PEDIATRICS INTERNATIONAL Vol. 61 ( 12 ) page： 1196 - 1201 2019.12

Yamamoto Hidenori, Fukasawa Yoshie, Kato Taichi

CARDIOLOGY IN THE YOUNG Vol. 29 ( 9 ) page： 1217 - 1218 2019.9

Cardiovascular Abnormality in RASopathies

Kato Taichi

Pediatric Cardiology and Cardiac Surgery Vol. 35 ( 2 ) page： 132 - 134 2019.5

Successful treatment of esophageal bleeding due to rupture of major aortopulmonary collateral arteries by transcatheter arterial embolization Reviewed

Ito Takanori, Ishigami Masatoshi, Ishizu Yoji, Kuzuya Teiji, Honda Takashi, Matsushima Masaya, Kato Taichi, Hirooka Yoshiki

CLINICAL JOURNAL OF GASTROENTEROLOGY Vol. 12 ( 1 ) page： 20 - 24 2019.2

Language：English Publishing type：Research paper (scientific journal)

Major aortopulmonary collateral arteries (MAPCAs) are unique vessels associated with hypoxia induced by congenital heart disease (CHD). Although MAPCAs are essential to supply blood to the lungs, their development and proliferation can induce life-threatening complications, such as rupture into the lung. Here, we describe a rare case of esophageal bleeding from MAPCAs in a CHD patient, which was successfully treated by transcatheter arterial embolization (TAE). A 16-year-old male with CHD experienced a hematemesis and melena after the Bentall procedure to treat valvular heart disease. Emergent esophagogastroduodenoscopy revealed spurting bleeding from the middle esophageal vessels; accordingly, endoscopic variceal ligation (EVL) was performed. However, he had a hematemesis again after 2 weeks of EVL. The arterial phase of dynamic computed tomography indicated that a MAPCA associated with CHD was the origin of bleeding. Hence, TAE of this MAPCA with a mixture of n-butyl-2-cyanoacrylate and ethiodized oil was performed to prevent re-bleeding. Color Doppler mode in endoscopic ultrasonography via the esophagus revealed mosaic-like signals in MAPCAs located in the esophageal wall. This finding was consistent with tortuous MAPCAs accompanied by turbulent blood flow. When clinicians encounter CHD patients with unexpected massive esophageal bleeding, bleeding related to MAPCAs should be considered.

DOI： 10.1007/s12328-018-0895-8

Frequent intragenic microdeletions of elastin in familial supravalvular aortic stenosis

Hayano Satoshi, Okuno Yusuke, Tsutsumi Makiko, Inagaki Hidehito, Fukasawa Yoshie, Kurahashi Hiroki, Kojima Seiji, Takahashi Yoshiyuki, Kato Taichi

INTERNATIONAL JOURNAL OF CARDIOLOGY Vol. 274 page： 290 - 295 2019.1

Identification of potential pathogenic viruses in patients with acute myocarditis using next-generation sequencing Reviewed

Takeuchi Suguru, Kawada Jun-ichi, Okuno Yusuke, Horiba Kazuhiro, Suzuki Takako, Torii Yuka, Yasuda Kazushi, Numaguchi Atsushi, Kato Taichi, Takahashi Yoshiyuki, Ito Yoshinori

JOURNAL OF MEDICAL VIROLOGY Vol. 90 ( 12 ) page： 1814 - 1821 2018.12

小児循環器疾患の基礎研究から臨床への応用家族性大動脈弁上狭窄において、遺伝子内微小欠失の頻度は高い次世代シーケンサーによる7家系の解析と臨床応用への可能性 Reviewed

早野聡, 奥野友介, 堤真紀子, 稲垣秀人, 深澤佳絵, 倉橋浩樹, 小島勢二, 高橋義行, 加藤太一

日本小児循環器学会雑誌 Vol. 34 ( Suppl.1 ) page： s1 - 122 2018.7

Infliximab versus intravenous immunoglobulin for refractory Kawasaki disease: A phase 3, randomized, open-label, active-controlled, parallel-group, multicenter trial Reviewed International journal

Masaaki Mori, Takuma Hara, Masako Kikuchi, Hiroyuki Shimizu, Tomoyuki Miyamoto, Satoru Iwashima, Tatsuya Oonishi, Kunio Hashimoto, Norimoto Kobayashi, Kenji Waki, Yasuo Suzuki, Yoshikazu Otsubo, Hiroshi Yamada, Chikao Ishikawa, Taichi Kato, Shigeto Fuse

Scientific Reports Vol. 8 ( 1 ) page： 1994 2018.1

Language：English Publishing type：Research paper (scientific journal) Publisher：Nature Publishing Group

We compared the efficacy and safety of infliximab with intravenous immunoglobulin (IVIG), a standard therapy, in a phase 3 trial (NCT01596335) for Japanese patients with Kawasaki disease (KD) showing persistent fever after initial IVIG. Patients with initial IVIG-refractory KD, aged 1-10 years, received a single dose of IV infliximab 5 mg/kg or IV polyethylene glycol-treated human immunoglobulin (VGIH) 2 g/kg on day 0. Primary outcome was defervescence rate within 48 h after the start of treatment. Safety was evaluated through day 56. Overall, 31 patients were randomized (infliximab, n = 16
VGIH, n = 15)
31.3% and 60.0% patients discontinued due to worsening KD. Defervescence rate within 48 h was greater with infliximab (76.7%) than VGIH (37.0%) (p = 0.023), and defervescence was achieved earlier with infliximab (p = 0.0072). Coronary artery lesions occurred in 1 (6.3%) and 3 (20.0%) patients receiving infliximab and VGIH, respectively, up to day 21. Adverse events occurred in 15 (93.8%) and 15 (100.0%) patients in the infliximab and VGIH groups, respectively. No serious adverse events in the infliximab group and one in the VGIH group were observed. Infliximab improved the defervescence rate within 48 h and time to defervescence versus standard therapy, and was well tolerated in patients with IVIG-refractory KD.

DOI： 10.1038/s41598-017-18387-7

Successful Infant Pneumonectomy with Unilateral Pulmonary Artery Occlusion Test.

Koji Kato, Taichi Kato, Satoshi Hayano, Yoshie Fukasawa, Atsushi Numaguchi, Tetsuo Hattori, Akiko Saito, Yoshiaki Sato, Masahiro Hayakawa

International heart journal Vol. 59 ( 1 ) page： 237 - 239 2018.1

Language：English Publisher：International Heart Journal Association

The use of unilateral pulmonary artery occlusion (UPAO) test for the preoperative evaluation of pneumonectomy was reported in adult patients. On the contrary, in infants, no strategies have yet been recommended to predict hemodynamics after pneumonectomy, nor has use of the UPAO test been reported. We describe the first case of infant with abnormal pulmonary circulation in whom successful pneumonectomy was performed after preoperative evaluation using UPAO test. Right pneumonectomy was planned for an 8-month-old girl, because of decreased right pulmonary function, high risk of pneumothorax, and impaired left lung expansion due to overexpansion caused by severe left bronchial stenosis and bronchomalacia. However, she had also prolonged pulmonary hypertension and there was difficulty in accurate echocardiographic evaluation of its severity due to concomitant left pulmonary artery stenosis. Furthermore, contrast-enhanced computer tomography suggested a certain degree of right pulmonary venous flow, discordant with the result showing scarce right pulmonary flow in perfusion scintigraphy. Predicting postoperative hemodynamic changes was therefore considered difficult. To evaluate these concerns, we performed cardiac catheterization and UPAO test to simulate postoperative hemodynamics. Pulmonary arteriography showed decreased but significant right pulmonary arterial and venous flows. Measurements including pulmonary artery pressure and cardiac index showed no marked changes after occlusion. Based on UPAO test results, the operation was successfully performed and hemodynamics remained stable postoperatively. The UPAO test may be useful for infants with cardiopulmonary impairment to evaluate the tolerability of pneumonectomy.

DOI： 10.1536/ihj.16-606