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Nakajima M.,RIKEN | Kou I.,RIKEN | Ohashi H.,Saitama Childrens Medical Center | Ikegawa S.,RIKEN
American Journal of Human Genetics | Year: 2016

Ossification of the posterior longitudinal ligament of the spine (OPLL) is a common spinal disorder that results from ectopic ossification of the posterior longitudinal ligament and causes intractable myelopathy and radiculopathy. In a previous genome-wide association study (GWAS), we found six loci associated with OPLL; however, susceptibility genes in these loci have not been identified yet. Here, we examined one of the GWAS loci and identified . RSPO2 (encoding R-spondin 2) as a susceptibility gene for OPLL. R-spondin 2 is a secreted agonist of canonical Wnt-β-catenin signaling. . RSPO2 was decreased in the early stage of chondrocyte differentiation. R-spondin 2 inhibited expression of genes encoding early chondrocyte differentiation markers by activating Wnt-β-catenin signaling. rs374810, the most significantly associated SNP in the GWAS locus in chromosomal region 8q23.1 was located in the chondrocyte promoter region of . RSPO2. A transcription factor, CCAAT-enhancer-binding protein β (C/EBPβ), specifically bound to the . RSPO2 core promoter region containing rs374810 and increased . RSPO2 expression. The risk allele of rs374810 affected the binding of the promoter with C/EBPβ and decreased the . RSPO2 transcription in vitro and in vivo. Our genetic and functional data indicate that . RSPO2 is a susceptibility gene for OPLL. © 2016 American Society of Human Genetics. Source

Muramatsu K.,Saitama Childrens Medical Center
Kyobu geka. The Japanese journal of thoracic surgery | Year: 2014

Mid aortic syndrome is uncommon acquired or congenital condition characterized by segmental narrowing of the abdominal or distal descending thoracic aorta. If left untreated, it result in life threatening complications. We described the case of 2-year-old boy admitted to our hospital for hypertension and heart failure. Diagnosis of mid aortic syndrome was made with severe stenosis in distal descending aorta. With consideration of growth, we avoided bypass grafting and implantation with prothesic graft. For severe adhesion, we performed patch aortoplasty with 0.4 mm expanded polytetrafluoroethylene (ePTFE) patch. Postoperative course was unevetful. His blood pressure and left ventricular function was normalized. He was discharged on the 20st day after the surgery. Source

Kojima H.,Saitama Childrens Medical Center
Nippon Hoshasen Gijutsu Gakkai zasshi | Year: 2011

The spiral scan with a wide detector row such as the 64-detector row computed tomography (CT) system may increase radiation exposure for infants because the irradiation range is wider than the planned range. The adaptive dose shield (ADS) prevents radiation exposure greater than the planned range. We examined the usefulness of the protection effect of the ADS for the infant inner ear CT. To confirm the protection effect of the ADS, we scanned X-ray films by using the 64-detector row CT system and measured the difference of the planned range and the irradiation range. The result of that is that when the planned range was small, the protection effect for the scan ending side was inferior to the scan starting side. And also, when the gantry rotation speed and pitch factor (PF) were high values, the protection effect was inferior to a low gantry rotation speed and low PF. There was a combination of gantry rotation speed and PF at which the protection effect decreases. Due to changes of the scanning direction and PF for the infant inner ear, the crystalline lens radiation exposure dose decreased from 11.89 mGy to 4.37 mGy. In conclusion, the ADS can reduce the radiation exposure dose of an adjacent organ. Therefore, it was thought that the ADS was a useful radiation exposure reduction function for infants in the 64-detector row CT system. Source

Nishimoto H.,Saitama Childrens Medical Center
Neurologia Medico-Chirurgica | Year: 2015

Head trauma is the leading cause of death in child abuse cases and one of the important issues in the care of abused children. Since the Child Abuse Prevention Law was enforced in 2000 in Japan, various measures have been taken to prevent child abuse over the following decade. Accordingly, medical research on abusive head trauma (AHT) has advanced, leading to significant progress in the medical diagnosis of AHT. This progress has been brought about by (1) the widespread establishment of child protection teams (CPTs) at core hospitals, (2) the progress in neuroradiological imaging and ophthalmoscopic technologies, and (3) the introduction of postmortem imaging. However, the pathological condition of patients with AHT, particularly that of the diffuse brain swelling type, still remains poorly understood. As a result, no clear treatment strategies for AHT have been developed and no treatment outcomes have been improved to date. The development of new treatment strategies for AHT and the construction of a comprehensive database that supports clinical studies are required in the future. © 2015 by The Japan Neurosurgical Society. Source

Hoshino K.,Saitama Childrens Medical Center
Clinical calcium | Year: 2012

Many epidemiological and clinical analysis have reported the relation between Mg and cardiovascular disease. Hypomagnesemia may be triggering mechanisms for ischemic heart disease, arrhythmias after open heart surgery, serious arrhythmias such as Torsades de Pointes (TdP) , and the negative feed back in congestive heart failure. Supplemental and therapeutic Mg infusion have been reported to reduce the mortality in acute myocardial infarction and having the cardioprotective effect after infarction (controversial) . It is also reported to reduce the incidence of arrhythmias after heart surgery, terminate the serious arrhythmias such as TdP, and improve the negative feed back in congestive heart failure. Magnesium metabolisms in cardiovascular disease are not necessarily clear. We expect the precise analysis of Mg actions and attractive Mg therapy in clinical literature. Source

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