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Sasahara A.,Gunma Childrens Medical Center
Kyobu geka. The Japanese journal of thoracic surgery | Year: 2016

We have repaired a unilateral absent pulmonary artery(UAPA) using a prosthetic graft with a ring. Case 1 was a 1-month old girl. The right pulmonary artery(RPA) was anti-anatomically reconstructed with a 4 mm ePTFE graft. In postoperative year 1, however, the graft became stenosed due to compression by the ascending aorta. We reversed the stenosis with a 6 mm stent by percutaneous transluminal angioplasty. Case 2 was 6-year old boy. His RPA was anatomically reconstructed with a ringed 5 mm ePTFE graft when he was 5-months old. In postoperative year 6, he needed the graft exchanged because of the relatively decreased pulmonary flow due to body growth. Therefore, we exchanged the graft with an anti-anatomically ringed 12 mm ePTFE graft. No graft stenosis was observed during the perioperative course. Using a prosthetic graft with a ring may be a good solution to avoid compression from the ascending aorta.

Motokawa M.,Gunma Childrens Medical Center
Kyobu geka. The Japanese journal of thoracic surgery | Year: 2016

We describe the rare case of a 1-year-old girl who had large muscular ventricular defect (VSD) nearby the moderator band. We experienced the patch closure using sandwich method. A 1-month-old girl was referred to our institution for treatment of muscular VSD. At the age of 2 month, she underwent the pulmonary artery banding to control the pulmonary high flow. After follow up, the patient have reached 70 cm tall and weighed 7 kg. One year after the "sandwich operation", cardiac catheterization revealed the tiny residual shunt. Nevertheless, the cardiac function was good and the growth was in fine fettle. Sandwich method is a useful surgical technique to close the muscular VSD without resect the right ventricular trabeculation.

Ohki Y.,Gunma University | Maruyama K.,Gunma Childrens Medical Center | Harigaya A.,Kiryu Kosei General Hospital | Kohno M.,Gunma University | Arakawa H.,Gunma University
Pediatrics International | Year: 2013

Background The aim of this study was to investigate the incidence and risk factors of peripherally inserted central venous catheter (PICC)-related complications using a multicenter case survey. Method A prospective cohort study was carried out by 19 neonatal intensive care units (NICUs) in Japan from February 2005 to March 2007. A total of 975 case records were collected. PICC-related complications including pericardial effusion/cardiac tamponade pleural effusion/ascites, catheter removal difficulties, catheter-related bloodstream infection (CR-BSI), and symptomatic catheter-related thrombosis were collected from case record forms. As for precautions during insertion, institutions were classified into three groups: those with maximum barrier precautions; standard precautions; and no specific precautions. Results PICC complications occurred in 27 cases (2.9%) among 946 PICC. The incidence was 1.6% for CR-BSI, and 0.1% for cardiac tamponade. CR-BSI rate per 1000 catheter-days was 1.1 with maximum barrier precautions at catheter insertion, 1.2 with standard precautions, and 1.8 with no specific precautions. Multiple logistic regression analysis showed that proximal placement (odds ratio [OR], 3.88; 95% confidence interval [CI]: 1.42-10.60, P = 0.008) and longer placement duration (OR, 1.35; 95%CI: 1.14-1.60, for each week, P = 0.0005) independently contributed to overall complications. Conclusion The incidence of cardiac tamponade was rare in this multicenter prospective study. Longer duration and proximal placement may be risk factors for PICC complications. In this cohort, the CR-BSI rate was low irrespective of the degree of barrier precautions at insertion. Pediatrics International © 2012 Japan Pediatric Society.

Yamada Y.,Gunma Childrens Medical Center | Cancelas J.A.,Cincinnati Childrens Hospital Medical Center | Cancelas J.A.,University of Cincinnati
International Archives of Allergy and Immunology | Year: 2010

Since the identification of the FIP1L1/PDGFRA fusion gene as a pathogenic cause of the hypereosinophilic syndrome (HES), the importance of the molecular classification of HES leading to the diagnosis of chronic eosinophilic leukemia (CEL) has been recognized. As a result, a new category, 'myeloid and lymphoid neoplasm with eosinophilia and abnormalities in PDGFRA, PDGFRB or FGFR1', has recently been added to the new WHO criteria for myeloid neoplasms. FIP1L1/PDGFRα-positive disorders are characterized by clonal hypereosinophilia, multiple organ dysfunctions due to eosinophil infiltration, systemic mastocytosis (SM) and a dramatic response to treatment with imatinib mesylate. A murine HES/CEL model by the introduction of FIP1L1/PDGFRα and IL-5 overexpression also shows SM, representing patients with FIP1L1/PDGFRα-positive HES/CEL/SM. The murine model and the in vitro development system of FIP1L1/PDGFRα-positive mast cells revealed the interaction between FIP1L1/PDGFRα, IL-5 and stem cell factor in the development of HES/CEL/SM. Current findings of FIP1L1/PDGFRα-positive HES/CEL are reviewed focusing on aberrant mast cell development leading to SM. Copyright © 2010 S. Karger AG, Basel.

Shiraishi Y.,University of Tokyo | Sato Y.,University of Tokyo | Chiba K.,University of Tokyo | Okuno Y.,University of Tokyo | And 10 more authors.
Nucleic Acids Research | Year: 2013

Recent advances in high-throughput sequencing technologies have enabled a comprehensive dissection of the cancer genome clarifying a large number of somatic mutations in a wide variety of cancer types. A number of methods have been proposed for mutation calling based on a large amount of sequencing data, which is accomplished in most cases by statistically evaluating the difference in the observed allele frequencies of possible single nucleotide variants between tumours and paired normal samples. However, an accurate detection of mutations remains a challenge under low sequencing depths or tumour contents. To overcome this problem, we propose a novel method, Empirical Bayesian mutation Calling (https://github.com/friend1ws/EBCall), for detecting somatic mutations. Unlike previous methods, the proposed method discriminates somatic mutations from sequencing errors based on an empirical Bayesian framework, where the model parameters are estimated using sequencing data from multiple non-paired normal samples. Using 13 whole-exome sequencing data with 87.5-206.3 mean sequencing depths, we demonstrate that our method not only outperforms several existing methods in the calling of mutations with moderate allele frequencies but also enables accurate calling of mutations with low allele frequencies (10%) harboured within a minor tumour subpopulation, thus allowing for the deciphering of fine substructures within a tumour specimen. © 2013 The Author(s).

Ogawa S.,University of Tokyo | Ogawa S.,Japan Science and Technology Agency | Takita J.,University of Tokyo | Sanada M.,University of Tokyo | Hayashi Y.,Gunma Childrens Medical Center
Cancer Science | Year: 2011

Neuroblastoma is one of the most common solid cancers among children. Prognosis of advanced neuroblastoma is still poor despite the recent advances in chemo/radiotherapies. In view of improving the clinical outcome of advanced neuroblastoma, it is important to identify the key molecules responsible for the pathogenesis of neuroblastoma and to develop effective drugs that target these molecules. Anaplastic lymphoma kinase (ALK) is a receptor tyrosine kinase, initially identified through the analysis of a specific translocation associated with a rare subtype of non-Hodgkin's lymphoma. Recently it was demonstrated that ALK is frequently mutated in sporadic cases with advanced neuroblastoma. Moreover, germline mutations of ALK were shown to be responsible for the majority of hereditary neuroblastoma. ALK mutants found in neuroblastoma show constitutive active kinase activity and oncogenic potentials. Inhibition of ALK in neuroblastoma cell lines carrying amplified or mutated ALK alleles results in compromised downstream signaling and cell growth, indicating potential roles of small molecule ALK inhibitors in the therapeutics of neuroblastoma carrying mutated ALK kinases. © 2011 Japanese Cancer Association.

Moriyama K.,Gunma Childrens Medical Center | Watanabe M.,Gunma Childrens Medical Center | Yamada Y.,Gunma Childrens Medical Center | Shiihara T.,Gunma Childrens Medical Center
Pediatric Neurology | Year: 2015

Introduction The ketogenic diet is a valuable therapy for patients with intractable epilepsy, but it can result in a variety of complications that sometimes limits its usefulness. Hypoproteinemia is one of the common adverse effects of this diet, although the underling mechanism is largely unknown except for the diet's reduced protein intake. Only one case of protein-losing enteropathy during the ketogenic diet has been reported. Patient Description A previously healthy 9-year-old girl experienced fever for 5 days then suddenly developed convulsive seizures that subsequently evolved to severe refractory status epilepticus. After multiple antiepileptic drugs failed to improve the patient's condition, we introduced the ketogenic diet. Although her seizures diminished, her course was complicated by hypoproteinemia. An abdominal dynamic scintigraphy and colonoscopy findings indicated protein-losing enteropathy with nonspecific mucosal inflammation. Her nutritional status deteriorated; thus, we discontinued the ketogenic diet. Her nutritional status gradually improved, whereas her seizures increased. Discussion Hypoproteinemia during the ketogenic diet is common, but the underlying etiologies are not well understood. Abdominal dynamic scintigraphy could be valuable for clarifying the etiology of hypoproteinemia during the ketogenic diet. © 2015 Elsevier Inc.

Miyamoto T.,Gunma Childrens Medical Center
Kyobu geka. The Japanese journal of thoracic surgery | Year: 2014

BACKGROUNDS: Bilateral pulmonary artery banding( BPAB), a relatively minimally invasive procedure, has begun to be undertaken as the initial operation for the treatment of hypoplastic left heart syndrome (HLHS) or HLHS variant. In the opinion of the present authors, it is practicable to avoid stepwise operations with cardiopulmonary bypass (CPB) in newborn patients by conducting an initial operation consisting of flow-adjustable bilateral pulmonary artery banding (FABPAB) combined with transcatheter pulmonary artery balloon dilatation( PABD), followed in a subsequent treatment course by transcatheter PABD, which would allow for more minimally invasive adjustment of the pulmonary artery bloodflow. This study was conducted as a retrospective evaluation of the usefulness of this therapeutic strategy.METHODS: Fourteen patients who underwent BPAB as the initial operation for HLHS/HLHS variant between April 2008 and October 2013 were included in this study. Stepwise surgical repair including BPAB was performed via a median sternotomy as the initial operation, without the use of CPB. And 7 patients underwent transcatheter PABD successfully.RESULTS: In this series, 7 patients developed a significant pulmonary artery index (PAI) after PABD (pre PABD:148.3±63.9 vs. post PABD:232.5±73.2, p<0.05). No need of the pulmonary artery reconstruction at the 2nd operation. One patient failed to survive the operation, in which difficulty in adjustment of atrioventricular valvular regurgitation was encountered after the Norwood procedure. There were 3 patients who underwent the Norwood combined BDG at the mean age of 4.6 months and the mean body weight of 5.5 kg. Fontan procedure was accomplished in 3 of the 6 patients of the survivor, while 3 patients were on standby for the Fontan procedure.CONCLUSION: We performed BPAB as the initial operation combined with PABD in the treatment of HLHS/HLHS variant, which enabled avoidance of the initial operation with cardiopulmonary bypass at the neonatal stage and developed a significant pulmonary artery index after PABD.

Kato M.,Gunma Childrens Medical Center | Yamada Y.,Gunma Childrens Medical Center | Maruyama K.,Gunma Childrens Medical Center | Hayashi Y.,Gunma Childrens Medical Center
International Archives of Allergy and Immunology | Year: 2010

Background: Little information is available on eosinophil activation and cytokine/chemokine responses in childhood asthma, thus we examined serum eosinophil cationic protein (ECP) and 27 types of cytokines/chemokines in acute exacerbation of asthma (acute asthma) and stable asthma. Methods: We determined peripheral eosinophil count, and the serum levels of ECP and 27 types of cytokines/chemokines (IL-1β, IL-1ra, IL-2, -4, -5, -6, -7, -8, -9, -10, -12, -13, -15 and -17, IFN-γ, IP-10, TNF-α, GM-CSF, G-CSF, MCP-1, MIP-1α and -1β, eotaxin, RANTES, PDGF-bb, FGF basic and VEGF) using a multiplex bead-based assay in 85 acute and 79 stable asthma patients, and 14 controls. We also examined the effects of systemic corticosteroids on these responses in acute asthma. Results: The serum levels of ECP, IL-5, -6, -8 and -10, G-CSF, MCP-1, IL-1ra and IP-10 were significantly elevated in acute compared with stable asthma. Similarly, serum levels of ECP, IL-5 and IP-10 were significantly higher in acute asthma than in controls. Furthermore, in the acute phase, elevated serum levels of ECP, IL-5, IL-6, IL-1ra and IP-10, but not IL-8, IL-10, G-CSF and MCP-1 were significantly reduced after treatments that included systemic corticosteroids. Conclusion: Eosinophil activation could be induced by acute exacerbation of childhood asthma. Copyright © 2010 S. Karger AG, Basel.

Miyamoto T.,Gunma Childrens Medical Center
Kyobu geka. The Japanese journal of thoracic surgery | Year: 2013

Surgery for common atrioventricular valve is classified into cases of groups at the univentricle repair with asplenic syndrome or cases of groups at the biventricular repair with atrial ventricular septal defect. The technique of the reoperation referred to a wide variety of surgical techniques such as commisuroplasty, valvuloplasty, annuloplasty, chordal repair, papillary muscle repair and valve replacement. This report examines the leaflet augmentation technique and supra-annular valve replacement for left atrioventricular valve after complete atrioventricular septal defect (AVSD) repair and semicircular annuloplasty with autologous pericardial strip for common atrioventricular valve after common atrioventricular valve repair. However, it is possible that numerous cases may require reoperation of valve plasty or valve replacement in the future. It is very important to follow up the subsequent history of the patients and to evaluate valve regurgitation and ventricular function while repeating cardiac catheterization, cardiac echo testing in addition to the clinical symptoms in a short period of time.

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