Hyogo Prefectural Kobe Childrens Hospital

Kōbe-shi, Japan

Hyogo Prefectural Kobe Childrens Hospital

Kōbe-shi, Japan
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Katsunuma S.,Kobe University | Katsunuma S.,Hyogo Prefectural Kobe Childrens Hospital
Experimental Cell Research | Year: 2017

Cells dissociated from various tissues of vertebrate embryos preferentially reaggregate with cells from the same tissue when they are mixed together. This tissue-specific recognition process in vertebrates is mainly mediated by a family of cell adhesion molecules because of their specific binding properties. Recent studies have revealed that two families of adhesion molecules, nectins and cadherins, are associated with each other, and these associations provide cells with the differential adhesive affinities required for cellular recognition and complex cellular pattern formations during development. This review provides an overview of recent findings regarding the cooperative functions of nectins and cadherins, as well as a discussion of the molecular basis underlying these functions. © 2017 Elsevier 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.

Okimoto S.,Hyogo Prefectural Kobe Childrens Hospital | Okimoto S.,Hiroshima University | Nomura K.,Hyogo Prefectural Kobe Childrens Hospital
Journal of Pediatric Ophthalmology and Strabismus | Year: 2014

Purpose: To report the clinical manifestations and therapeutic outcomes of retinoblastoma during 16 years. Methods: Forty-three eyes of 34 patients with retinoblastoma from 1996 to 2011 were retrospectively examined for eye preservation rates based on the disease stage, and the treatment outcomes between monocular and binocular cases were compared. In addition, the relationship between age at initial examination and disease stage and the difference of age at enucleation with or without preservation therapy were examined. Results: Twenty-five patients had monocular retinoblastoma and 9 patients had binocular retinoblastoma. Three patients had a family history of retinoblastoma. The mean observation period was 106.6 ± 53.0 months. Median age at initial examination was 13.5 months. There was no correlation between age at initial examination and disease stage. Preservation therapy was performed for 24 eyes. Nineteen eyes were enucleated without performing preservation therapy. There was a significant difference in age at enucleation between the preservation therapy group (finally enucleated 13 eyes, median = 30.0 months) and the non-preservation therapy group (19 eyes, median = 13.0 months). Eye preservation rates according to the International Classification of Retinoblastoma (ICRB) were as follows: 100% for group B, 100% for group C, 33% for group D, and 0% for group E. In eyes with ICRB group D, the ratio of induction with preservation therapy was 50% of monocular cases and 75% of binocular cases. Conclusions: Preservation therapy was able to significantly delay enucleation. Eye preservation therapy was performed for a higher proportion of advanced cases among binocular cases than among monocular cases. © SLACK Incorporated.

Doi Y.,Hyogo Prefectural Kobe Childrens Hospital | Kagawa T.,Hyogo Prefectural Kobe Childrens Hospital
Japanese Journal of Anesthesiology | Year: 2013

Most drugs used in practical pediatric anesthesia are off-label. Pediatric anesthesiologists and non-pediatric anesthesiologists who rarely encounter pediatric patients need to be aware of the clinical usage of these drugs based on pediatric pharmacokinetics and pharmacodynamics to ensure that children are not exposed to unnecessary risks. Clinical guidelines on anesthetic drugs have been made available on the Japanese Society of Anesthesiologists website, and anesthesiologists are encouraged to access this site at least once. Propofol is commonly used in pediatric anesthesia and sedation in several situations. However, we should always consider the possibility of propofol infusion syndrome in the case of long-term administration. Rocuronium is widely used for general anesthesia in elective surgeries, examinations, and minor procedures in pediatric patients. Rocuronium can be used for rapid sequence induction, taking into consideration the dose and duration of action. Sugammadex has recently been introduced for practice in Japan. Rocuronium and sugammadex have been used safely in pediatric patients so far, and may change the induction methods used in difficult airway patients and the management of airway emergencies. Desflurane is novel in Japan and has not been commonly used in pediatric anesthesia. Desflurane may cause very high irritability in the airway and may be used for the maintenance of anesthesia in limited situations.

Shimizu M.,Kanazawa University | Nakagishi Y.,Hyogo Prefectural Kobe Childrens Hospital | Inoue N.,Kanazawa University | Mizuta M.,Hyogo Prefectural Kobe Childrens Hospital | And 6 more authors.
Clinical Immunology | Year: 2016

To assess the role of IL-6/IL-18 in the pathogenesis of systemic juvenile idiopathic arthritis (s-JIA) and to investigate the clinical significance of serum IL-18 levels for predicting macrophage activation syndrome (MAS) development, we measured the serum IL-6/IL-18 levels in 76. s-JIA patients, including 15 with MAS, and compared them with the clinical features. We identified 2 distinct subsets on the basis of serum IL-6/IL-18 levels. The IL-18-dominant subset had more patients who developed MAS. Serum IL-18 levels during active phase in patients with MAS were significantly higher than those without MAS. The cutoff value of serum IL-18 levels for predicting MAS development was 47750. pg/ml. The patients with IL-18 dominant subset at their disease onset were significantly more likely to develop MAS after TCZ therapy started. IL-18 might have a key role in the pathogenesis of MAS. Serum IL-18 levels >. 47750. pg/ml might be useful to predict MAS development. © 2015 Elsevier Inc.

Wakita M.,Mukogawa Women's University | Fukatsu A.,Hyogo Prefectural Kobe Childrens Hospital | Amagai T.,Mukogawa Women's University
Nutrition in Clinical Practice | Year: 2011

Background: The aim of the present study was to determine whether nutrition assessment helps predict clinical outcomes (COs) in infants who have undergone cardiac surgery. Methods: Study subjects were infants, aged less than 18 months, who had undergone cardiac surgery between April 2007 and August 2008. The nutrition parameters assessed include Onodera's prognostic nutritional index (PNI), height for age, weight for height, and weight for age. COs included mortality rate during hospitalization, length of stay in intensive care unit (LOS-1), length of stay in the hospital after surgery (LOS-2), and duration of mechanical ventilation support. Method-1: the correlation between nutrition parameters and COs was examined by statistical analysis. Method-2: the cutoff point of nutrition parameters was determined using the minimum P value approach. Results: The following results were obtained: Results-1: PNI was the only nutrition parameter found to be correlated with LOS-1. Results-2: the cutoff point for PNI as a predictor of LOS-1 was 55. Conclusions: It appeared that preoperative PNI was the most influential factor on LOS-1 for infants after they underwent cardiac surgery. The PNI cutoff point 55 in infants who underwent cardiac surgery seems to be the best predictor of CO. © 2011 American Society for Parenteral and Enteral Nutrition.

Mizobuchi M.,Hyogo Prefectural Kobe Childrens Hospital | Yoshimoto S.,Hyogo Prefectural Kobe Childrens Hospital | Nakao H.,Hyogo Prefectural Kobe Childrens Hospital
Pediatrics International | Year: 2011

Background: The aim of this study was to determine the time-course effect of a single dose of hydrocortisone (HC) on arterial blood pressure in extremely low gestational age newborns (ELGAN) with refractory hypotension during the first 3 days of life. Methods: We carried out a matched case-control study of a cohort of 116 infants born between 23 and 27 weeks' gestation at a tertiary center. Twelve infants (10%) were treated with HC for refractory hypotension (HC group). HC was administered at a dose of 2 mg/kg when mean arterial pressure (MAP) was below 30 mmHg (25 mmHg for infants <25 weeks of gestational age) despite the use of inotropes and volume expanders. Changes in the MAP after the HC administration were compared with those in the infants who were not treated with HC and matched for gestational age (Control group). Results: The mean MAP before administration of HC was significantly lower in the HC group than that in the control group (24.0 ± 3.2 vs 33.3 ± 4.8 mmHg; P < 0.01). The mean MAP in the HC group increased significantly at 2 h after HC treatment, and then reached levels comparable to those in the Control group at 5 h (31.3 ± 4.0 vs 33.9 ± 4.7 mmHg; P= 0.18), and remained at normal levels until 12 h after HC treatment. Conclusion: A single dose of HC treatment induces a rapid and sustained improvement in blood pressure in ELGAN with refractory hypotension. © 2011 The Authors. Pediatrics International © 2011 Japan Pediatric Society.

Shimizu M.,Kanazawa University | Nakagishi Y.,Hyogo Prefectural Kobe Childrens Hospital | Yachie A.,Kanazawa University
Cytokine | Year: 2013

To assess the serum interleukin (IL)-6 and IL-18 levels in patients with systemic juvenile idiopathic arthritis (s-JIA) and to identify the clinical features of patient subsets with different cytokine profiles, we analyzed the serum levels of IL-6 and IL-18 in patients with s-JIA and compared them with the clinical features of s-JIA. Eighteen patients were analyzed. IL-6 and IL-18 levels were quantified in serum by enzyme-linked immunosorbent assays. Interestingly, two distinct s-JIA patient subsets based on their serum IL-6 and IL-18 levels were identified: an IL-6 dominant and an IL-18 dominant. The serum IL-6 and IL-18 levels were consistent both at relapse and at the onset of s-JIA in each subset. The IL-6-dominant subset had a significantly greater number of joints with active disease and higher serum levels of matrix metalloproteinase-3, whereas the IL-18-dominant subset was more likely to develop macrophage activation syndrome (MAS). These findings indicate that two subsets of patients with s-JIA, one which is prone for arthritis and another with prone for MAS, can be identified on the basis of their serum IL-6 and IL-18 levels. These two subsets appear to be characterized by certain distinct clinical features. © 2012 Elsevier Ltd.

Mizowaki T.,Hyogo Prefectural Kobe Childrens Hospital | Mizowaki T.,Kobe University
World neurosurgery | Year: 2014

BACKGROUND: Choroid plexus papilloma (CPP) in the third ventricle is a rare benign intracranial tumor.METHODS: We report 3 pediatric cases of CPP in the third ventricle. The lesions were totally removed by a different surgical approach in each case.RESULTS: When remarkable hydrocephalus is present, the transcortical approach is easier to perform, but may expose the patient to epilepsy and subdural effusion postoperatively. The transcallosal approach offers direct exposure of the ventricle system with minimal risk of cortical damage. The transcallosal-transforaminal approach with posterior enlargement of the foramen of Monro along the choroidal fissure provides a direct trajectory into the third ventricle through the natural cleft. The transcallosal-interforniceal approach does not depend on the size of the foramen of Monro, but it carries a risk for damage to the both fornices. The midline plane of the septum pellucidum and the forniceal columns in children are sometimes easily identifiable and separable, and in such cases the transcallosal-interforniceal approach appears to be a safe route for tumors extending to the posterior third ventricle. The interforniceal approach should be reserved for lesions that cannot be removed safely via the transforaminal approach.CONCLUSIONS: Young children have a small total blood volume and fragile cardiovascular status. Therefore, it is critical to preserve the venous system and to ligate the feeding artery before extirpation of the tumor. The surgical approach to the third ventricular CPPs should be tailored to individual children based on tumor size, location, and vascularity. Copyright © 2014 Elsevier Inc. All rights reserved.

Shimizu M.,Kanazawa University | Nakagishi Y.,Hyogo Prefectural Kobe Childrens Hospital | Kasai K.,Hyogo Prefectural Kobe Childrens Hospital | Yamasaki Y.,Kagoshima University | And 3 more authors.
Cytokine | Year: 2012

Macrophage-activation syndrome (MAS) is a potentially life-threatening complication of systemic juvenile idiopathic arthritis (s-JIA). Tocilizumab (TCZ), a humanized anti-IL-6 receptor monoclonal antibody, is an effective cytokine inhibitor for the treatment of s-JIA. We described the clinical courses of five cases of MAS during TCZ therapy and demonstrated the need for monitoring serum interleukin (IL)-18 and IL-6 concentrations. Clinical symptoms of patients with s-JIA receiving TCZ were apparently mild compared to those not receiving TCZ. Furthermore, serum CRP concentrations never increased during TCZ therapy, even in MAS. Serum IL-6 concentrations increased during s-JIA flare-up and with the complication of infection. Serum IL-18 concentrations increased persistently before the other measures of disease activity. The clinical symptoms of MAS and s-JIA could be masked during TCZ therapy; hence, monitoring serum concentrations of IL-18 and IL-6 is recommended for the evaluation of disease activity in s-JIA and to detect the complication of infection. © 2012 Elsevier Ltd.

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