Hokkaido Medical Center for Child Health and Rehabilitation

Sapporo, Japan

Hokkaido Medical Center for Child Health and Rehabilitation

Sapporo, Japan
Time filter
Source Type

Kinebuchi M.,Aichi University | Matsuura A.,Aichi University | Kiyono T.,National Cancer Center Research Institute | Nomura Y.,Aomori City Public Health Center | Kimura S.,Hokkaido Medical Center for Child Health and Rehabilitation
Scientific Reports | Year: 2016

Copper (Cu) is an indispensable metal for normal development and function of humans, especially in central nervous system (CNS). However, its redox activity requires accurate Cu transport system. ATP7A, a main Cu2+ transporting-ATPase, is necessary to efflux Cu across the plasma membrane and synthesize cuproenzymes. Menkes disease (MD) is caused by mutations in ATP7A gene. Clinically, MD is Cu deficiency syndrome and is treated with Cu-histidine injections soon after definite diagnosis. But outcome of the most remains poor. To estimate the standard therapy, Cu distribution in the treated classic MD patients is analyzed by synchrotron-generated X-ray fluorescence technique (SR-XRF), which identifies and quantifies an individual atom up to at subcellular level of resolution with wide detection area. SR-XRF analysis newly reveals that Cu exists in spinal cord parenchyma and flows out via venous and lymph systems. By systemic analysis, excess Cu is detected in the proximal tubular cells of the kidney, the mucosal epithelial cells of the intestine, and the lymph and venous systems. The current study suggests that the standard therapy supply almost enough Cu for patient tissues. But given Cu passes through the tissues to venous and lymph systems, or accumulate in the cells responsible for Cu absorption. © The Author(s) 2016.

PubMed | Osaka City General Hospital, Saiseikai Yokohamashi Tobu Hospital, Kanagawa Childrens Medical Center, Nagano Childrens Hospital and 11 more.
Type: | Journal: Journal of cardiology | Year: 2017

Persistent tachycardia in pediatric patients after congenital heart surgery further deteriorates their hemodynamic condition, and may become fatal. Therefore, immediate control of the tachycardia is mandatory in these patients. For this purpose, quick-acting, short-acting, titratable intravenous agents are required. However, there are no agents with such characteristics among the drugs approved for control of pediatric arrhythmias in Japan, and thus novel and effective medications for these patients are awaited. Landiolol, an ultrashort-acting -blocker, was approved in 2013 for tachyarrhythmias in adult patients with heart failure. However, its efficacy and safety in pediatric patients remain unclear. The aim of this prospective, multicenter, open-label phase IIb/III study is to investigate the efficacy and safety of landiolol in pediatric patients with tachyarrhythmias as well as heart failure.Eligible patients are aged 3 months and <15 years, and have tachyarrhythmia (atrial fibrillation, atrial flutter, supraventricular tachycardia) as well as heart failure. The primary endpoint of the study is 20% reduction from baseline heart rate or return to normal sinus rhythm within 2h after starting intravenous administration of landiolol. Patients will receive intravenous infusion of landiolol, starting at 1g/kg/min. The dose will be increased by 1g/kg/min every 15-20min until the tachycardia rate has decreased by >20% or tachycardia has terminated, and the dose will then be maintained or further increased depending on the patients condition. The study was started in April 2015 and will end within a few years.The study was designed and designated the HEARTFUL study in the hope of establishing a basis for control of HEART rate in inFant and child tachyarrhythmia Using Landiolol in children with heart failure.

PubMed | The Surgical Center, Hokkaido Medical Center for Child Health and Rehabilitation and Sapporo Medical University
Type: Case Reports | Journal: Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery | Year: 2016

We report the case of a 2-year-old boy who showed a huge midline mass in the brain at prenatal assessment.After birth, magnetic resonance imaging (MRI) revealed a conglomerate mass with an infolded microgyrus at the midline, which was suspected as a midline brain-in-brain malformation. MRI also showed incomplete cleavage of his frontal cortex and thalamus, consistent with lobar holoprosencephaly. The patient underwent an incisional biopsy of the mass on the second day of life. The mass consisted of normal central nervous tissue with gray and white matter, representing a heterotopic brain. The malformation was considered to be a subcortical heterotopia. With maturity, focal signal changes and decreased cerebral perfusion became clear on brain imaging, suggesting secondary glial degeneration. Coincident with these MRI abnormalities, the child developed psychomotor retardation and severe epilepsy focused on the side of the intracranial mass.

Yanase Y.,Hokkaido Medical Center for Child Health and Rehabilitation | Watanabe M.,Hokkaido Medical Center for Child Health and Rehabilitation | Ishikawa N.,Hokkaido Medical Center for Child Health and Rehabilitation | Higami T.,Sapporo Medical University
Interactive Cardiovascular and Thoracic Surgery | Year: 2012

We report a case of surgical treatment for neonatal Ebstein's anomaly with circular shunting. A prenatal diagnosis of Ebstein'sanomaly was made at 30 weeks of gestation. In addition to severe tricuspid regurgitation, severe pulmonary regurgitation was also noted, resulting in a 'circular shunt'. At 38 weeks and 2 days of gestation, the baby was delivered by Caesarean section. A two-stage palliative operation was planned. As the first palliation, the pulmonary artery was ligated 1 h after birth to eliminate the circular shunt. Lipo-prostaglandin E was administrated continuously to keep the ductus arteriosus patent. Four days after first operation, modified Starnes operation was performed as the second-stage palliation. The tricuspid valve was closed by an ePTFE patch with 3-mm fenestration. Pulmonary blood flow was supplied by a right modified Blalock-Taussig shunt with a 3.5-mm diameter ePTFE graft. The infant's postoperative haemodynamic status was stable. The patient is being followed up carefully and is scheduled to have a univentricular repair in the future. © 2012 The Author.

Inazawa N.,Sapporo Medical University | Hori T.,Sapporo Medical University | Hatakeyama N.,Sapporo Medical University | Yamamoto M.,Sapporo Medical University | And 5 more authors.
Journal of Medical Virology | Year: 2015

Viral reactivations following hematopoietic stem cell transplantation are thought to result from the breakdown of both cell-mediated and humoral immunity. As a result, many viruses could be reactivated individually or simultaneously. Using a multiplex polymerase chain reaction (PCR), we prospectively examined many kinds of viral DNAs at a time in 105 patients who underwent allogeneic hematopoietic stem cell transplantation. In total, 591 whole blood samples were collected weekly from pre- to 42 days post-transplantation and the following 13 viruses were tested; herpes simplex virus 1 (HSV-1), HSV-2, varicella-zoster virus (VZV), Epstein-Barr virus (EBV), cytomegalovirus (CMV), human herpes virus 6 (HHV-6), HHV-7, HHV-8, adenovirus, BK virus (BKV), JC virus (JCV), parvovirus B19, and hepatitis B virus (HBV). Several viral DNAs were detected in 12 patients before hematopoietic stem cell transplantation. The detection rate gradually increased after transplantation and peaked at 21 days. The most frequently detected virus was HHV-6 (n=63; 60.0%), followed by EBV (n=11; 10.5%), CMV (n=11; 10.5%), and HHV-7 (n=9; 8.6%). Adenovirus and HBV were each detected in one patient (1.0%). Detection of HHV-6 DNA was significantly more common among patients undergoing cord blood transplantation or with steroid treatment. EBV DNA tended to be more common in patients treated with anti-thymocyte globulin. Multiplex PCR was useful for detecting many viral reactivations after hematopoietic stem cell transplantation, simultaneously. Cord blood transplantation, steroid treatment, or anti-thymocyte globulin use was confirmed to be risk factors after transplantation. © 2015 Wiley Periodicals, Inc..

Kurose M.,Sapporo Medical University | Takano K.,Sapporo Medical University | Mitsuzawa H.,Hokkaido Medical Center for Child Health and Rehabilitation | Himi T.,Sapporo Medical University
International Journal of Pediatric Otorhinolaryngology | Year: 2014

Objective: Tracheo-innominate artery fistula (TIF) is a rare but life-threatening complication following tracheostomy or tracheoesophageal diversion (TED). Although successful surgical intervention for TIF has been reported, few studies have been performed in patients with severe motor and intellectual disability (SMID). Therefore, we aimed to analyze TIF in patients with SMID to clarify the clinical variables predicting the occurrence and adequate management for lifesaving of TIF. Methods: We retrospectively reviewed the records of patients with SMID undergoing surgical tracheostomy and TED between 2006 and 2012 and identified those with TIF. When TIF occurred, we obtained the clinical status and emergency management. Results: Of 70 patients who underwent tracheostomy or TED during the study period, three patients had TIFs; in one case, TIF was avoided by ligation of the innominate artery before TED. The incidence of TIF in those undergoing tracheostomy and TED was 2.3% and 7.4%, respectively. The interval between tracheostomy and TIF was 14-50 months. Conclusions: Patients with SMID may have an increased risk of TIF. Prompt diagnosis and surgical intervention to control the bleeding is the only effective management at present. © 2014 Elsevier Ireland Ltd.

PubMed | Tokyo Medical and Dental University, Hokkaido Medical Center for Child Health and Rehabilitation, Tokyo Medical University and Sapporo Medical University
Type: Journal Article | Journal: Journal of medical virology | Year: 2016

Viral reactivation following hematopoietic stem cell transplantation (HSCT) can cause various complications especially viral encephalitis. In this prospective study, we investigated the correlation of post-HSCT viral reactivation in blood with CNS dysfunction. We employed a multiplex PCR that detects 13 kinds of viruses as a first-line screening test and real-time PCR for subsequent quantitative evaluation. Five hundred ninety-one whole blood samples were collected from 105 patients from before until 42 days after HSCT. Seven patients developed CNS dysfunction such as altered consciousness. In six of the seven, the multiplex PCR test detected HHV-6 DNA in at least one sample. In contrast, DNA from other viruses, such as CMV, EBV, HHV-7, adenovirus, and HBV was never detected in any of the seven patients throughout the study period. Quantitative measurement of whole blood HHV-6 DNA levels demonstrated four of the six HHV-6 DNA loads were elevated at successive time points during the CNS dysfunction. In addition, the virus DNA peaks were temporally associated with the development of CNS dysfunction. CSF was tested in two of the four patients and high HHV-6 DNA levels comparable to those in whole blood were confirmed in both. These four patients were, thus, suspected to have developed HHV-6 encephalitis, a rate of 3.8% in the study population. Our results suggest that early diagnosis of probable HHV-6 encephalitis can be improved by confirming high HHV-6 DNA load in blood.

Honda Y.,Hokkaido Medical Center for Child Health and Rehabilitation
Kyobu geka. The Japanese journal of thoracic surgery | Year: 2012

The case was 11-month-old girl diagnosed as tetralogy of Fallot with severe pulmonary valve stenosis and suffering from severe cyanosis. A catheter study demonstrated pulmonary artery (PA) was well arborized, but severely hypoplastic in spite of previous transcatheter pulmonary valve dilatation; PA index was calculated as 69 mm 2/m 2. A central end-to-side aorta to PA shunt was created. Cyanosis was well improved, but congestive heart failure occurred after 1 month from the operation. Subsequent catheter study demonstrated pulmonary artery growth, 166 mm 2/m 2 of PA index and major aortopulmonary collateral artery (MAPCA) coil embolization was performed. Patient underwent Rastelli type definitive repair 9 month after palliation. The central end-to-side aorta to PA shunt is reported as useful measure for promoting PA size increase and definitive repair achievement in patient with pulmonary atresia, ventricular septal defect and severely hypoplastic PA. Appropriate consideration of criteria, carefull follow up and treatments are necessary.

Honda Y.,Hokkaido Medical Center for Child Health and Rehabilitation
Kyobu geka. The Japanese journal of thoracic surgery | Year: 2010

Patient is 1 month old, 3.72 kg boy, he was diagnosed as {S, L, L} double-inlet left ventricle (DILV), transposition of the great arteries, coarctation of aorta, rudimentary right ventricle. Patient was undergone Norwood procedure, with "swing-back technique"; end-to end anastomosis of ascending aorta with descending aorta, double-barreled style Damus-Kaye-Stansel (DKS), end-to side anastomosis of neoaorta to aortic arch. This technique has following advantages over other reported technique; discrepancy of great arteries were resolved without distortion ; reconstruction of the aorta without the use of patch materials: minimal length suture line to minimize the risk of bleeding; and more radical excision of ductal tissue without much dissection and mobilization of descending aorta.

Yanase Y.,Hokkaido Medical Center for Child Health and Rehabilitation
Kyobu geka. The Japanese journal of thoracic surgery | Year: 2013

We describe a baby girl with isolate absence of the right pulmonary artery. She had tachypnea just after birth. Several examinations showed absence of the right pulmonary artery and an aortopulmonary collateral artery. After follow-up, cardiac catheterization was performed the age of 14 months. It showed mild pulmonary artery hypertension. The posterior wall of right pulmonary artery was reconstructed with U-shaped in situ pulmonary artery flap and the anterior wall was reconstructed with autologous pericardium patch. Enhanced computed tomography was performed on postoperative day 9 showed occlusion of the right pulmonary artery by a thrombus. Emergency catheterization and thrombolytic therapy was performed with no success. Then, we successfully performed thrombectomy by open surgery. Cardiac catheterization performed at 6 months after the operation showed patency of the right pulmonary artery and improvement of pulmonary artery hypertension.

Loading Hokkaido Medical Center for Child Health and Rehabilitation collaborators
Loading Hokkaido Medical Center for Child Health and Rehabilitation collaborators