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6. Low risk of treatment resistance in Down syndrome with Kawasaki disease

Author

Takatsuki, Shinichi, Ogata, Shohei, Ishii, Masahiro, Yokozawa, Masato, Ono, Masae, Fujiwara, Masako, Ida, Hiroyuki, Motomura, Hideki, Moriuchi, Hiroyuki, Taketazu, Mio, Kawamura, Yoichi, Kawano, Tatsuya, Izumi, Tatsuro, Shiono, Junko, Tsuchiya, Shiro, Tsuchiya, Keiji, Goushi, Terufumi, Ichida, Fukiko, and Saji, Tsutomu

Published
2017
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16. Revision of diagnostic guidelines for Kawasaki disease (6th revised edition)

Author

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

Published
2020
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22. Prostaglandin E-major urinary metabolite as a noninvasive surrogate marker for infantile necrotizing enterocolitis

Author

Konishi, Ken-ichiro, primary, Yoshida, Mariko, additional, Nakao, Atsushi, additional, Tsuchiya, Keiji, additional, Tsurisawa, Chisa, additional, Ichiki, Kunihiko, additional, Takeda, Tomohiro, additional, Hisaeda, Yoshiya, additional, Hirota, Atsushi, additional, Amagata, Shusuke, additional, Odashima, Takayuki, additional, Yokomori, Kinji, additional, Ishida, Kazuo, additional, Matsuura, Masaaki, additional, Ito, Satoru, additional, Fujiwara, Mutsunori, additional, and Nakahara, Saori, additional

Published
2019
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23. Abstract O.04: Kawasaki Disease With Down Syndrome:low Risk For Ivig Resistance And Coronary Artery Abnormalities

Author

Takatsuki, Shinichi, primary, Yokozawa, Masato, additional, Ono, Masae, additional, Fujiwara, Masako, additional, Ida, Horoyuki, additional, Motomura, Hideki, additional, Moriuchi, Horoyuki, additional, Taketazu, Mio, additional, Oki, Junichi, additional, Nonoyama, Shigeaki, additional, Kawano, Tatsuya, additional, Ihara, Kenji, additional, Kido, Sachiko, additional, Shiono, Junko, additional, Tsuchiya, Shiro, additional, Tsuchiya, Keiji, additional, Goushi, Teruhumi, additional, Ogata, Shuhei, additional, Ishii, Masahiro, additional, Ichida, Fukiko, additional, and Saji, Tsutomu, additional

Published
2015
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30. External Validation of a Risk Score to Predict Intravenous Immunoglobulin Resistance in Patients With Kawasaki Disease

Author

Seki, Mitsuru, primary, Kobayashi, Tohru, additional, Kobayashi, Tomio, additional, Morikawa, Akihiro, additional, Otani, Tetsuya, additional, Takeuchi, Kazuo, additional, Ayusawa, Mamoru, additional, Tsuchiya, Keiji, additional, Yasuda, Kenji, additional, Suzuki, Takahiro, additional, Shimoyama, Shinya, additional, Ikeda, Kentaro, additional, Ishii, Yoichiro, additional, and Arakawa, Hirokazu, additional

Published
2011
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44. Abstract 9906: Early Pulmonary Artery Banding Improves the Long-term Survival in Trisomy 18 Infants: A Receiver Operating Characteristic Curve Analysis

Author

Okamura, Kenichi, Suzuki, Toshihiko, Fujioka, Tao, Nakao, Atsushi, Tsuchiya, Keiji, and Kobayashi, Jotaro

Abstract

Introduction:Trisomy 18 (T18) is a genetic diagnosis often associated with cardiac malformations. Several recent studies have reported that cardiac interventions improve the survival of T18 infants; however, none have described the optimal age for performing pulmonary artery banding (PAB).Purpose:We investigated the cut-off age for performing PAB, as we have empirically observed that T18 infants who undergo PAB beyond 30 days of life have a relatively poor prognosis.Methods:We reviewed the detailed clinical information of T18 patients at our institute between July 2004 and December 2018. The area under the receiver operating characteristic (ROC) curve (AUC) was used to identify the optimal cut-off age for PAB to predict the one- and five-year survival, and the long-term survival was assessed with the Kaplan-Meier method.Results:A total of 110 infants were diagnosed with T18 at our hospital, and 51 of them underwent PAB, not achieving intracardiac repair. The PAB cases included 34 cases of PAB with patent ductus arteriosus closure, 8 with isolated PAB, 7 with bilateral PAB, and 2 with coarctectomy. The median age at PAB was 29 days old (range, 6-190 days). The age at PAB was younger in alive patients than in dead patients at 1 and 5 years old (P=0.093 and 0.107, respectively). The ROC analysis showed that the optimal cut-off age for PAB was 33 days old for the 5-year survival (sensitivity 80% and specificity 80%, AUC: 0.740) and 42 days old for the 1-year survival (sensitivity 75% and specificity 56%, AUC: 0.593). The 5-year survival rates were 19.8% and 66.5% in PAB patients >33 and ?33 days old, respectively (Log-rank, P=0.046).Conclusions:This is the first study to analyze the optimal age of PAB using the ROC method. Our findings suggest that the optimal age of PAB in T18 infants was ?33 days old if possible, or 42 days old at the latest to improve the long-term survival.

Published
2019
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45. Abstract 12723: Impact of Cardiac Intervention on Long-term Survival and Home Discharge in Trisomy 18 Patients: A Propensity Score-matched Analysis

Author

Okamura, Kenichi, Suzuki, Toshihiko, Fujioka, Tao, Nakao, Atsushi, Tsuchiya, Keiji, and Kobayashi, Jotaro

Abstract

Introduction:Trisomy 18 (T18) is a genetic diagnosis that is often associated with cardiac malformations. Limited literature is available on the impact of congenital heart surgery (CHS) on the prognosis of trisomy 18 patients. None have investigated it using a propensity score matched analysis.Purpose:We aimed to investigate the impact of CHS on long-term survival and the home discharge rate.Methods:We reviewed the detailed clinical information of T18 patients treated at our institute between July 2004 and December 2018. The patients were divided into 2 groups depending on whether they underwent CHS or not (non-CHS). CHS included all types of cardiac surgery (e.g., pulmonary artery banding, patent ductus arteriosus closure, coarctectomy, or intracardiac repair). The long-term survival and home discharge rates were compared by propensity score matching (1:1 matching, caliper: 0.2).Results:A total of 110 infants were diagnosed with T18. Among them, the survival of the CHS group (n=73, 66.3%) was longer than that of the non-CHS group (n=37, 33.6%) (hazard ratio [HR]:0.41, 95% confidence interval [CI]: 0.24-0.69; P<0.001). The home discharge rate of the CHS group was better than that of the non-CHS group (79.5%, 46.9%, respectively; P=0.002). After propensity score matching (C-statistic = 0.773), the CHS group (n=19) survived longer than the non-CHS group (n=19) (HR: 0.40, 95% CI: 0.17-0.99, P=0.047). During follow-up, the CHS group had higher rates of 1-, 3-, 5-year survival than the non-CHS group (68.4% vs. 32.6%, 46.3% vs. ?11.5%, 39.7% vs. ?11.5). The home discharge rate in the CHS group tended to be better than that in the non-CHS group (84.2%, 57.1%, respectively; P=0.183).Conclusions:This is the first study to investigate survival and the rate of home discharge according to CHS in T18 patients by a propensity score matched analysis. Our findings suggest that CHS prolongs long-term survival and that CHS may increase the chance of home discharge in T18 patients.

Published
2019
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48. Neonatal repair of right interrupted aortic arch with cerebro-myocardial perfusion technique.

Author

Takeuchi, Koh, Masuzawa, Akihiro, Kobayashi, Jotaro, and Tsuchiya, Keiji

Abstract

Right interrupted aortic arch and descending aorta is exceedingly rare and most likely cause respiratory presentation, since patent ductus arteriosus (PDA) courses over the right mainstem bronchus. We report a case of successful neonatal biventricular repair of a right interrupted aortic arch (type B), with an aberrant right subclavian artery ventricular septal defect (VSD) in a 2.7 kg term neonate with DiGeorge syndrome. Patient presented in severe respiratory distress and acidosis at one day old. Two-dimensional (2D) echocardiography revealed aortic arch interruption beyond the common carotid arteries with large perimembranous outlet VSD. Aortic annulus diameter was 4.8 mm and there was no left ventricle (LV) outflow tract obstruction. Three-dimensional (3D) CT-scan confirmed these findings and identified a right-sided ductal arch that continued over the right mainstem bronchus into a right-sided descending aorta and aberrant right subclavian artery. Brachiocephalic perfusion and ductal perfusion was employed for cooling during cardiopulmonary bypass. Under deep hypothermia (27 °C rectal temperature), selective cerebro-myocardial perfusion was used for successful aortic arch repair without sacrificing the aberrant right subclavian artery. A direct tension-free anastomosis was attained. Her postoperative course was uneventful and her respiratory symptoms disappeared postoperatively. Early surgical correction is mandatory for these patients with unique anatomy and presentation.

Published
2011
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49. [Diagnosis of incomplete Kawasaki disease].

Author

Tsuchiya K, Imada Y, Aso S, and Sonobe T

Subjects
Child, Preschool, Diagnosis, Differential, Humans, Infant, Mucocutaneous Lymph Node Syndrome classification, Mucocutaneous Lymph Node Syndrome diagnosis
Abstract

We defined incomplete Kawasaki disease (KD) as those having less than 5 principal symptoms of KD in this article. Japanese Nationwide KD surveys revealed that the prevalence of coronary artery lesion in incomplete KD is almost the same as that of complete KD. Thus, incomplete KD should not be equated with mild KD. However, there is no royal way to make the correct diagnosis of incomplete KD. We have to remind that incomplete KD is not uncommon (around 15%) and is not mild KD when we work-up patients with few principal symptoms of KD. We have to pay attention also to other significant symptoms or findings of the guideline, especially to the skin changes at the site of BCG inoculation.

Published
2008

50. Utility of coronary MR angiography in children with Kawasaki disease.

Author

Takemura A, Suzuki A, Inaba R, Sonobe T, Tsuchiya K, Omuro M, and Korenaga T

Subjects
Child, Child, Preschool, Coronary Artery Disease etiology, Female, Humans, Infant, Male, Mucocutaneous Lymph Node Syndrome complications, Prognosis, Reproducibility of Results, Retrospective Studies, Sensitivity and Specificity, Coronary Angiography methods, Coronary Artery Disease diagnosis, Image Enhancement methods, Image Interpretation, Computer-Assisted methods, Magnetic Resonance Angiography methods, Mucocutaneous Lymph Node Syndrome diagnosis
Abstract

Objective: Although coronary arterial lesions due to Kawasaki disease (KD) should be evaluated as early as possible after the acute phase, conventional X-ray coronary angiography poses high risks for young children with the disease. The use of noninvasive MR coronary angiography is desirable, although it is difficult to produce clear images in young children. We developed a method to improve the quality of MR coronary angiography in young children. MR coronary angiography with vector electrocardiogram gating, real-time navigator-echo, 3D, steady-state free precession was performed in 35 children with KD. Many parameters (i.e., field of view, acquisition delay, turbo-field echo-factor, navigator window, and resolution) were optimized for each patient., Conclusion: Optimization resulted in the acquisition of high-resolution and highsignal images of the coronary arteries. It remarkably improved not only the quality of the images, but also the detection rate of coronary artery segments. MR coronary angiography is a useful method for evaluating coronary aneurysms from the early stages of KD, even in infants and small children.

Published
2007
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