Tokyo Metropolitan Childrens Medical Center

Tokyo, Japan

Tokyo Metropolitan Childrens Medical Center

Tokyo, Japan

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Nakanishi K.,Wakayama Medical University | Iijima K.,Kobe University | Ishikura K.,Tokyo Metropolitan Childrens Medical Center | Hataya H.,Tokyo Metropolitan Childrens Medical Center | And 4 more authors.
Clinical Journal of the American Society of Nephrology | Year: 2013

Background and objectives Early identification of frequently relapsing children with idiopathic nephritic syndrome is desirable. Design, setting, participants, & measurements The relapse status and clinical data of patients previously registered (January of 1993 to December of 2001) in a multicenter prospective study of the International Study of Kidney Disease in Children regimen were analyzed for risk of frequent relapsers over a 2-year follow-up period. Results Of 166 children with nephrotic syndrome (113 boys and 53 girls; median age=5.1 years), 145 (87.3%, median age=5.5 years) children were steroid-sensitive, and 21 (12.7%, median age=2.9 years) children were steroid-resistant. Of 145 children with steroid-sensitive nephrotic syndrome, 32 (22.1%, median age=4.2 years) children experienced frequent relapses over 2 years. The time to initial response was significantly longer (10 versus 7 days, P,0.001, log-rank test) in the 32 frequent relapsers than in the 106 non-frequent relapsers. The time from start of initial treatment to first relapse was significantly shorter (2.6 versus 6.1 months, P,0.001, log-rank test) in the 32 frequent relapsers than in the 57 infrequent relapsers. In a Cox regression model, the time to initial response >9 days and the duration from start of initial treatment to first relapse,6 months were significant predictors of frequent relapses (unadjusted and adjusted). Conclusions Initial remission time >9 days and first relapse within 6 months were associated with frequent relapses. These findings may also be useful also in selecting potential frequent relapsers for clinical trials. © 2013 by the American Society of Nephrology.


Hasegawa K.,Brigham and Women's Hospital | Hasegawa K.,Massachusetts General Hospital | Shigemitsu K.,Osaka Saiseikai Senri Hospital | Hagiwara Y.,Tokyo Metropolitan Childrens Medical Center | And 4 more authors.
Annals of Emergency Medicine | Year: 2012

Study objective: Although repeated intubation attempts are believed to contribute to patient morbidity, only limited data characterize the association between the number of emergency department (ED) laryngoscopic attempts and adverse events. We seek to determine whether multiple ED intubation attempts are associated with an increased risk of adverse events. Methods: We conducted an analysis of a multicenter prospective registry of 11 Japanese EDs between April 2010 and September 2011. All patients undergoing emergency intubation with direct laryngoscopy as the initial device were included. The primary exposure was multiple intubation attempts, defined as intubation efforts requiring greater than or equal to 3 laryngoscopies. The primary outcome measure was the occurrence of intubation-related adverse events in the ED, including cardiac arrest, dysrhythmia, hypotension, hypoxemia, unrecognized esophageal intubation, regurgitation, airway trauma, dental or lip trauma, and mainstem bronchus intubation. Results: Of 2,616 patients, 280 (11%) required greater than or equal to 3 intubation attempts. Compared with patients requiring 2 or fewer intubation attempts, patients undergoing multiple attempts exhibited a higher adverse event rate (35% versus 9%). After adjusting for age, sex, principal indication, method, medication, and operator characteristics, intubations requiring multiple attempts were associated with an increased odds of adverse events (odds ratio 4.5; 95% confidence interval 3.4 to 6.1). Conclusion: In this large Japanese multicenter study of ED patients undergoing intubation, we found that multiple intubation attempts were independently associated with increased adverse events. Copyright © 2012 by the American College of Emergency Physicians.


Yamamoto S.,Tokyo Metropolitan Childrens Medical Center
Japanese Journal of Anesthesiology | Year: 2012

Atrial septal defect (ASD) is one of the commonest cardiac anomalies in children. Surgical repair leads to excellent results with very low morbidity and mortality, but percutaneous transcatheter closure with a septal occluder is gradually becoming an alternative method, as it is minimally invasive. Anesthesia with intubation is necessary for this procedure, because transesophageal echocardiography is essential in the evaluation of ASD and placement of the occlusion device for ASD. During the procedure, minor complications including device embolizations and arrhythmia need attention. Balloon test occlusion of atrial septal defects may identify patients with left ventricular restrictive physiology before ASD closure. Intravenous anticongestive conditioning medication might be useful for preventing congestive heart failure after interventional closure of an ASD in the patient with a restrictive left ventricle.


Ishimaru S.,Tokyo Metropolitan Childrens Medical Center
Leukemia | Year: 2016

In the treatment of childhood acute lymphoblastic leukemia (ALL), excess shortening of maintenance therapy resulted in high relapse rate, as shown by our previous trial, TCCSG L92-13, in which maintenance therapy was terminated at 1 year from initiation of treatment. In this study, we aimed to confirm the long-term outcome of L92-13, and to identify who can or cannot be cured by shorter duration of maintenance therapy. To obtain sentinel cytogenetics information that had been missed before, we performed genetic analysis with genomic microarray and target intron-capture sequencing from diagnostic bone marrow smear. Disease-free survival (DFS) at 10 years from the end of therapy was 66.0±2.8%. Females (n=138) had better DFS (74.6±3.7%) than males (n=142, 57.5±4.2%, P=0.002). Patients with TCF3-PBX1 (n=11) and ETV6-RUNX1 (n=16) had excellent DFS (90.9±8.7% and 93.8±6.1%, respectively), whereas high hyperdiploidy (n=23) was the most unfavorable subgroup, with 56.6±10.3% of DFS. Short duration of therapy can cure more than half of pediatric ALL, especially females, TCF3-PBX1 and ETV6-RUNX1. Our retrospective observations suggest a gender/karyotype inhomogeneity on the impact of brief therapy.Leukemia advance online publication, 25 October 2016; doi:10.1038/leu.2016.274. © 2016 Macmillan Publishers Limited, part of Springer Nature.


Nukui Y.,University of Tokyo | Hatakeyama S.,University of Tokyo | Okamoto K.,University of Tokyo | Yamamoto T.,University of Tokyo | And 5 more authors.
Journal of Antimicrobial Chemotherapy | Year: 2013

Objectives: Thrombocytopenia is sometimes observed during linezolid therapy. Here, we aimed to investigate the factors affecting linezolid-induced thrombocytopenia. Methods: A prospective observational study was performed between October 2009 and February 2011; 30 patients were included. Plasma linezolid trough concentrations were measured on days 3, 7 and 14 after initial drug administration. Platelet counts and haemoglobin levels were also monitored. Results: Thrombocytopenia occurred in 17 patients (56.7%). Median linezolid trough concentrations on day 3 were significantly higher in patients with renal impairment (creatinine clearance, 60 mL/min) than in patients without renal impairment (14.7 versus 4.8 mg/L; P,0.0001). Median linezolid trough concentrations on day 3 in patients who developed thrombocytopenia were also significantly higher than those in patients who did not (13.4 versus 4.3 mg/L, P,0.0001). Development of thrombocytopenia occurred significantly more frequently in patients with linezolid trough concentration.7.5 mg/L (OR, 90.0; P,0.0001) and renal impairment (OR, 39.0; P 1/40.0002). The Kaplan-Meier plot showed that the median time from the initiation of therapy to development of thrombocytopenia was 11 days. Conclusions: Patients with renal impairment are more likely to have a high plasma linezolid concentration. In addition, a high plasma linezolid concentration and renal impairment significantly affected the development of linezolid-induced thrombocytopenia. Further studies are required to evaluate whether therapeutic drug monitoring- guided dosage adjustment of linezolid decreases the adverse effects while maintaining treatment efficacy in patients with renal dysfunction. © The Author 2013. Published by Oxford University Press on behalf of the British Society for Antimicrobial Chemotherapy. All rights reserved.


Superti-Furga A.,University of Lausanne | Spranger J.,University Hospital Freiburg | Nishimura G.,Tokyo Metropolitan Childrens Medical Center
American Journal of Medical Genetics, Part C: Seminars in Medical Genetics | Year: 2012

The so-called "enchondromatoses" are skeletal disorders defined by the presence of ectopic cartilaginous tissue within bone tissue. The clinical and radiographic features of the different enchondromatoses are distinct, and grouping them does not reflect a common pathogenesis but simply a similar radiographic appearance and thus the need for a differential diagnosis. Recent advances in the understanding of their molecular and cellular bases confirm the heterogeneous nature of the different enchondromatoses. Some, like Ollier disease, Maffucci disease, metaphyseal chondromatosis with hydroxyglutaric aciduria, and metachondromatosis are produced by a dysregulation of chondrocyte proliferation, while others (such as spondyloenchondrodysplasia or dysspondyloenchondromatosis) are caused by defects in structure or metabolism of cartilage or bone matrix. In other forms (e.g., the dominantly inherited genochondromatoses), the basic defect remains to be determined. The classification, proposed by Spranger and associates in 1978 and tentatively revised twice, was based on the radiographic appearance, the anatomic sites involved, and the mode of inheritance. The new classification proposed here integrates the molecular genetic advances and delineates phenotypic families based on the molecular defects. Reference radiographs are provided to help in the diagnosis of the well-defined forms. In spite of advances, many cases remain difficult to diagnose and classify, implying that more variants remain to be defined at both the clinical and molecular levels. © 2012 Wiley Periodicals, Inc.


Shido H.,Tokyo Metropolitan Childrens Medical Center | Tamada I.,Tokyo Metropolitan Childrens Medical Center
Pediatric Dermatology | Year: 2015

We often encounter injuries caused by pencils or colored pencils in toddlers and young children, but subsequent segmental tumor formation is rare. Here we report the case of a 4-year-old boy who had been stabbed under his left eyebrow with a red pencil. Colored pencil-core granuloma, a foreign body granuloma arising from an injury with a colored pencil, was diagnosed on the basis of intraoperative and histopathologic findings. Because of the severe tissue damage that the colorant causes, this type of tumor grows rapidly within a few days and may be accompanied by resorption of the skull if it occurs on the head or face. © 2015 Wiley Periodicals, Inc.


Iijima K.,Kobe University | Sako D.M.,National Health Research Institute | Nozu K.,Kobe University | Mori R.,National Health Research Institute | And 13 more authors.
The Lancet | Year: 2014

Background Rituximab could be an effective treatment for childhood-onset, complicated, frequently relapsing nephrotic syndrome (FRNS) and steroid-dependent nephrotic syndrome (SDNS). We investigated the efficacy and safety of rituximab in patients with high disease activity. Methods We did a multicentre, double-blind, randomised, placebo-controlled trial at nine centres in Japan. We screened patients aged 2 years or older experiencing a relapse of FRNS or SDNS, which had originally been diagnosed as nephrotic syndrome when aged 1-18 years. Patients with complicated FRNS or SDNS who met all other criteria were eligible for inclusion after remission of the relapse at screening. We used a computer-generated sequence to randomly assign patients (1:1) to receive rituximab (375 mg/m2) or placebo once weekly for 4 weeks, with age, institution, treatment history, and the intervals between the previous three relapses as adjustment factors. Patients, guardians, caregivers, physicians, and individuals assessing outcomes were masked to assignments. All patients received standard steroid treatment for the relapse at screening and stopped taking immunosuppressive agents by 169 days after randomisation. Patients were followed up for 1 year. The primary endpoint was the relapse-free period. Safety endpoints were frequency and severity of adverse events. Patients who received their assigned intervention were included in analyses. This trial is registered with the University Hospital Medical Information Network linical trials registry, number UMIN000001405. Findings Patients were centrally registered between Nov 13, 2008, and May 19, 2010. Of 52 patients who underwent randomisation, 48 received the assigned intervention (24 were given rituximab and 24 placebo). The median relapsefree period was signifi cantly longer in the rituximab group (267 days, 95% CI 223-374) than in the placebo group (101 days, 70-155; hazard ratio: 0 27, 0 14-0 53; p<0 0001). Ten patients (42%) in the rituximab group and six (25%) in the placebo group had at least one serious adverse event (p=0 36). Interpretation Rituximab is an eff ective and safe treatment for childhood-onset, complicated FRNS and SDNS.


Hasegawa K.,Harvard University | Chiba T.,Obama Municipal Hospital | Hagiwara Y.,Tokyo Metropolitan Childrens Medical Center | Watase H.,Oregon Health And Science University | And 4 more authors.
Journal of Allergy and Clinical Immunology: In Practice | Year: 2013

Background: Little is known about the quality of acute asthma care in emergency departments (EDs) outside of North America. Objective: We evaluated concordance of acute asthma management in Japanese EDs with recommendations in the 2007 National Institutes of Health asthma guidelines and investigated whether guideline concordance was associated with risk of hospital admission. Methods: We conducted a multicenter chart review study in 23 EDs across Japan. We identified ED patients aged 18 to 54 years with acute asthma between 2009 and 2011. Concordance with evidence-based guideline recommendations was evaluated by using item-by-item quality measures and composite concordance scores both at patient and ED levels. These scores ranged from 0 to 100. Results: Among 1380 patients, the median age was 35 years and 11% were hospitalized. Overall guideline concordance score was suboptimal both at the patient level (mean ± SD, 72 ± 14) and ED level (mean ± SD, 72 ± 6). Specifically, asthma care at the patient level was suboptimal in several areas: inhaled anticholinergics in ED (2%), systemic corticosteroid in ED (56%) and at discharge (36%), and peak flow assessment (9%). Amultivariable model that adjusted for severity at presentation and several ED characteristics showed that higher guideline concordance was associated with significantly lower risk of hospital admission (odds ratio, 0.70 per 10-unit increase in composite score; 95% CI, 0.62-0.79 per 10-unit increase in composite score). Conclusion: The management of acute asthma in Japanese EDs is suboptimal. Greater concordance with guideline-recommended management might reduce unnecessary hospitalizations. Knowledge translation initiatives are warranted to increase adherence with best practice in acute asthma management. © 2013 American Academy of Allergy, Asthma & Immunology.


Yuza Y.,Tokyo Metropolitan Childrens Medical Center
Japanese Journal of Cancer and Chemotherapy | Year: 2014

Because of recent advances in cancer treatments, about 70% of childhood cancer patients can be cured and reach adulthood. Childhood cancer survivors (CCS) are estimated to account for one out of 1,000 adults currently in their 20s. In the continuing care of CCS, two aspects of long-term follow-up (LTFU) are necessary different from that of adulthood cancer survivors. As CCS receive cancer treatment, including chemotherapy and radiotherapy, during their growth phase, one aspect of LTFU is monitoring for late side effects on their physiological and psychosocial growth. The other aspect is transition medicine to encourage adolescent and young adult (AYA)-CCS into adult-oriented medicine to receive age-appropriate clinical care. Because there are differences between child-oriented medicine and adult-oriented medicine, AYA-CCS often refuse to visit adult-oriented clinics or avoid follow-ups. Therefore, the establishment of a comprehensive LTFU system is necessary, and transition medicine began being introduced in the 1990s. In Japan, the importance of these aspects has just begun to be recognized and the system is still not fully established. In this paper, we discuss these LTFU topics together with current situations in Japan.

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