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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. Source

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. Source

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. Source

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. Source

Kobayashi T.,Gunma University | Saji T.,Toho University | Otani T.,National Center for Child Health and Development | Takeuchi K.,Saitama University | And 18 more authors.
The Lancet | Year: 2012

Background Evidence indicates that corticosteroid therapy might be beneficial for the primary treatment of severe Kawasaki disease. We assessed whether addition of prednisolone to intravenous immunoglobulin with aspirin would reduce the incidence of coronary artery abnormalities in patients with severe Kawasaki disease. Methods We did a multicentre, prospective, randomised, open-label, blinded-endpoints trial at 74 hospitals in Japan between Sept 29, 2008, and Dec 2, 2010. Patients with severe Kawasaki disease were randomly assigned by a minimisation method to receive either intravenous immunoglobulin (2 g/kg for 24 h and aspirin 30 mg/kg per day) or intravenous immunoglobulin plus prednisolone (the same intravenous immunoglobulin regimen as the intravenous immunoglobulin group plus prednisolone 2 mg/kg per day given over 15 days after concentrations of C-reactive protein normalised). Patients and treating physicians were unmasked to group allocation. The primary endpoint was incidence of coronary artery abnormalities during the study period. Analysis was by intention to treat. This trial is registered with the University Hospital Medical Information Network clinical trials registry, number UMIN000000940. Findings We randomly assigned 125 patients to the intravenous immunoglobulin plus prednisolone group and 123 to the intravenous immunoglobulin group. Incidence of coronary artery abnormalities was significantly lower in the intravenous immunoglobulin plus prednisolone group than in the intravenous immunoglobulin group during the study period (four patients [3%] vs 28 patients [23%]; risk difference 0 20, 95% CI 0 12-0 28, p<0 0001). Serious adverse events were similar between both groups: two patients had high total cholesterol and one neutropenia in the intravenous immunoglobulin plus prednisolone group, and one had high total cholesterol and another non-occlusive thrombus in the intravenous immunoglobulin group. Interpretation Addition of prednisolone to the standard regimen of intravenous immunoglobulin improves coronary artery outcomes in patients with severe Kawasaki disease in Japan. Further study of intensified primary treatment for this disease in a mixed ethnic population is warranted. Funding Japanese Ministry of Health, Labour and Welfare. Source

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