Sendai-shi, Japan
Sendai-shi, Japan

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PubMed | Tokyo Medical and Dental University, Fukushima Medical University, Ibaraki Childrens Hospital, Childrens Cancer Center and 10 more.
Type: | Journal: Pediatric blood & cancer | Year: 2017

In the tyrosine kinase inhibitor (TKI) era, outcomes after salvage therapy for relapsed or refractory Philadelphia chromosome-positive (Ph+) acute lymphoblastic leukemia (ALL) remain unclear.The clinical courses of 19 patients with relapse (n = 13) or induction failure (n = 6) in the Japanese Pediatric Leukemia/Lymphoma Study Group Ph+ ALL04 study were retrospectively reviewed.Fifteen male and four female patients had a median age of 8 (range 4-15) years at relapse or induction failure. Patients received imatinib in combination with hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone (hyper-CVAD) and methotrexate and cytarabine (MTX/Ara-C) (n = 9), imatinib in combination with other chemotherapy (n = 5), chemotherapy without imatinib (n = 2), imatinib alone (n = 2), or no additional chemotherapy (n = 1). Two patients underwent hematopoietic stem cell transplantation (HSCT) without achieving complete remission (CR) and died of leukemia. The remaining 17 patients achieved CR with salvage therapies and underwent HSCT whilst in CR: 10 patients remain alive in CR, five died of transplantation-related complications, and two died of relapse. In six of seven patients with available data on minimal residual disease (MRD), imatinib in combination with the first course of hyper-CVAD was more effective in achieving a favorable MRD response compared with the Ph+ ALL04 induction regimen.This study suggested that cross-resistance to imatinib failed to develop after conventional chemotherapy. Imatinib in combination with chemotherapy including hyper-CVAD+MTX/Ara-C was effective and safe for relapsed or refractory Ph+ ALL patients who received frontline therapy without imatinib.

Yokota S.,Yokohama City University | Imagawa T.,Yokohama City University | Mori M.,Yokohama City University | Miyamae T.,Yokohama City University | And 8 more authors.
Journal of Rheumatology | Year: 2014

Objective. To assess the longterm safety and effectiveness of tocilizumab (TCZ) in systemic-onset juvenile idiopathic arthritis (sJIA). Methods. The longterm extension phase of 2 pivotal studies (phase II with 11 patients and phase III with 56 patients) in patients with active sJIA was analyzed. Patients received open-label TCZ (8 mg/kg, every 2 weeks) without concomitant use of disease-modifying antirheumatic drugs. Results. In total, 67 patients were enrolled. All patients received corticosteroid at baseline. Median duration of exposure to TCZ was 3.4 years. Nine patients withdrew from the study [4 because of adverse events (AE), 4 because of the development of anti-TCZ antibodies, and 1 because of inadequate response]. Rates of AE and serious AE were 803.7/100 patient-years (PY) and 34.7/100 PY, respectively. The most common serious AE were infections (13.2/100 PY). No cases of malignancy or death were reported. Two serious infusion reactions were reported in patients testing negative for anti-TCZ antibodies. One definite macrophage activation syndrome (MAS) case and 1 potential MAS case were identified. American College of Rheumatology (ACR) response rates attained early in the TCZ treatment period were maintained throughout the study: at Week 168, JIA ACR 30, 50, 70, 90, and 100 response rates were 80.3%, 80.3%, 75.4%, 60.7%, and 18.0%, respectively. In total, 22 of 67 patients (32.8%) completely discontinued corticosteroids without flare. Conclusion. TCZ has demonstrated durability of effectiveness in the longterm treatment of children with sJIA and has shown good tolerability and a low discontinuation rate associated with AE, development of anti-TCZ antibodies, or inadequate response. ( NCT00144599 and NCT00144612). Copyright © 2014. All rights reserved.

Miyazaki O.,National Center for Child Health and Development | Sawai H.,Hyogo College of Medicine | Murotsuki J.,Miyagi Childrens Hospital | Murotsuki J.,Tohoku University | And 3 more authors.
Pediatric Radiology | Year: 2014

Background: Recently, computed tomography (CT) has been used to diagnose fetal skeletal dysplasia. However, no surveys have been conducted to determine the radiation exposure dose and the diagnostic reference level (DRL). Objective: To collect CT dose index volume (CTDIvol) and dose length product (DLP) data from domestic hospitals implementing fetal skeletal 3-D CT and to establish DRLs for Japan. Materials and methods: Scan data of 125 cases of 20 protocols from 16 hospitals were analyzed. The minimum, first-quartile, median, third-quartile and maximum values of CTDIvol and DLP were determined. The time-dependent change in radiation dose setting in hospitals with three or more cases with scans was also examined. Results: The minimum, first-quartile, median, third-quartile and maximum CTDIvol values were 2.1, 3.7, 7.7, 11.3 and 23.1 mGy, respectively, and these values for DLP were 69.0, 122.3, 276.8, 382.6 and 1025.6 mGy•cm, respectively. Six of the 12 institutions reduced the dose setting during the implementation period. Conclusions: The DRLs of CTDIvol and DLP for fetal CT were 11.3 mGy and 382.6 mGy•cm, respectively. Institutions implementing fetal CT should use these established DRLs as the standard and make an effort to reduce radiation exposure by voluntarily decreasing the dose. © 2014 Springer-Verlag.

Hayashi T.,Miyagi Childrens Hospital | Shirane R.,Miyagi Childrens Hospital | Fujimura M.,Tohoku University | Tominaga T.,Tohoku University
Journal of Neurosurgery: Pediatrics | Year: 2010

Object. Young patients with moyamoya disease frequently exhibit extensive cerebral infarction at the time of initial presentation, and even in the early postoperative period. To investigate clinical characteristics in the early postoperative period, the authors prospectively analyzed findings of MR imaging, MR angiography, and SPECT before and after surgery. The authors focused in particular on how postoperative neurological deterioration occurred. Methods. Between August 2005 and June 2009, 22 patients younger than 18 years of age with moyamoya disease were treated at Miyagi Children's Hospital. The mean patient age (± SD) was 8.58 ± 4.55 years (range 2-17 years). Superficial temporal artery-middle cerebral artery bypass and indirect bypass of encephalosynangiosis between the brain surface and the temporal muscle, galea, and dura mater were performed in 35 hemispheres. Magnetic resonance imaging and MR angiography were performed before surgery, at 7 days postoperatively, and 3-6 months after surgery. A 123I-isopropyl iodoamphetamine SPECT scan was also obtained pre- and postoperatively. Results. During the postoperative period, neurological deterioration was observed after 15 operations (10 cases of motor paresis, 1 of aphasia, and 4 of sensory disturbance) in 13 patients. All symptoms had resolved by the time of discharge, except in 2 patients who suffered cerebral infarction. All patients exhibited disappearance (94.3%) or reduction (5.7%) of transient ischemic attacks (TIAs) during the follow-up period. Perioperative studies revealed 2 different types of radiological findings, focal uptake decrease on SPECT indicative of cerebral ischemia due to dynamic change in cerebral hemodynamics caused by bypass flow, the so-called watershed shift, and perioperative edematous lesions on MR imaging due to cerebral hyperperfusion. The frequent occurrence of preoperative TIAs was significantly associated with watershed shift, whereas preoperative MR imaging findings and preoperative SPECT findings were not. Age at operation was the only factor significantly associated with postoperative hyperperfusion. Conclusions. In young patients, moyamoya disease exhibits rapid progression, resulting in poor clinical outcome. The risk of postoperative neurological deterioration in very young moyamoya patients with frequent TIAs should be noted. The findings in this study showed that direct bypass is not completely safe in patients with moyamoya disease because it causes dynamic change in postoperative cerebral hemodynamics.

Kitazawa H.,Miyagi Childrens Hospital | Kure S.,Tohoku University
Clinical Medicine Insights: Circulatory, Respiratory and Pulmonary Medicine | Year: 2015

Interstitial lung disease (ILD) in childhood is a heterogeneous group of rare pulmonary conditions presenting chronic respiratory disorders. Many clinical features of ILD still remain unclear, making the treatment strategies mainly investigative. Guidelines may provide physicians with an over-view on the diagnosis and therapeutic directions. However, the criteria used in different clinical studies for the classification and diagnosis of ILDs are not always the same, making the development of guidelines difficult. Advances in genetic testing have thrown light on some etiologies of ILD, which were formerly classified as ILDs of unknown origins. The need of genetic testing for unexplained ILD is growing, and new classification criteria based on the etiology should be adopted to better understand the disease. The purpose of this review is to give an overview of the clinical and genetic aspects of ILD in children. © the authors, publisher and licensee Libertas Academica Limited.

Hayashi T.,Miyagi Childrens Hospital | Shirane R.,Miyagi Childrens Hospital | Yokosawa M.,Miyagi Childrens Hospital | Kimiwada T.,Miyagi Childrens Hospital | Tominaga T.,Tohoku University
Journal of Neurosurgery: Pediatrics | Year: 2010

Object. The rate of infection following shunt procedures is unacceptably high. The authors have hypothesized that the key to reducing the shunt infection rate is in reducing bacteria in the operating field and wound. This hypothesis has been tested in a prospective nonrandomized controlled manner. Methods. Data obtained in all patients undergoing shunt insertions or revisions for hydrocephalus performed between October 1, 2003, and June 12, 2009, were reviewed. Starting in August 2006, we began routinely irrigating the operating field and wound with saline solution from a syringe. Prior to this, we had not used any irrigation techniques, providing an adequate control group (Group A) for the effect of the irrigation technique. Prior to November 2007, we used saline containing amikacin for irrigation (Group B). After that date, we used saline only for irrigation (Group C). Results. A total of 150 shunt procedures were performed in 79 girls and 71 boys during the study period. The mean age of all patients was 44.0 ± 59.1 months. Groups A, B, and C comprised 61, 40, and 49 shunt procedures, respectively. There was no statistical difference in age among the 3 groups. Nine infections occurred within 90 days in the postoperative period. The overall infection rate was 6.0%. Eight infections occurred before introducing the irrigation procedure (infection rate 13.1%). One infection was noted after introducing irrigation (Group B [0.0%] + Group C [2.0%]; combined B and C infection rate = 1.1%). There was a statistical difference in the infection rate between Group A and Groups B and C combined (p = 0.003), Groups A and B (p = 0.021), and Groups A and C (p = 0.035). In contrast, no statistical difference was observed between Groups B and C (p > 0.99). Six of the 9 infections were due to staphylococcal species. Conclusions. An irrigation technique used to reduce bacteria in the operating field and wound is effective for preventing shunt infection. Irrigation alone, and not antibiotics, contributed to the prophylaxis of shunt infection.

Matsuo S.,Miyagi Childrens Hospital | Takahashi G.,Miyagi Childrens Hospital | Konishi A.,Miyagi Childrens Hospital | Sai S.,Miyagi Childrens Hospital
Pediatric Cardiology | Year: 2013

We investigated the optimal treatment for refractory chylothorax after pediatric cardiovascular surgery. We retrospectively reviewed the cases of 15 consecutive patients who developed chylothorax after congenital heart surgery performed between December 2004 and November 2010. Among the 15 patients (12 male and 3 female; median age 13.9 months) who developed postoperative chylothorax, 10 recovered with conservative therapy, such as a low-fat diet, medium chain triglyceride-enriched diet, or total parenteral nutrition. Of the remaining 5 patients who underwent surgical treatment followed by conventional therapy, 4 showed improvement, and 1 died from cardiac failure. Surgical treatment was performed at a median of 19 days after diagnosis of chylothorax. Average drainage output of thoracocentesis for the first 5 days before thoracic duct ligation was 33.1 ml/kg/day. Duration of chylous fluid drainage was significantly longer in surgical patients than in patients who recovered with conservative therapy (p < 0.01). Surgical patients tended to be younger with lower body weight. Significant risk factors for surgical intervention were age <4 months, body weight <4 kg, and duration of drainage >10 days. In cases of refractory postoperative chylothorax, surgical therapy such as thoracic duct ligation should be considered when discharge from the drainage tube is >30 ml/kg/day or chylothorax is not improved within 10 days. © 2012 Springer Science+Business Media New York.

Kato M.,Yamagata University | Saitsu H.,Yokohama City University | Murakami Y.,Osaka University | Kikuchi K.,Saitama Childrens Medical Center | And 14 more authors.
Neurology | Year: 2014

Objective: To investigate the clinical spectrum caused by mutations in PIGA at Xp22.2, which is involved in the biosynthesis of the glycosylphosphatidylinositol (GPI) anchor, among patients with early-onset epileptic encephalopathies (EOEEs). Methods: Whole-exome sequencing was performed as a comprehensive genetic analysis for a cohort of 172 patients with EOEEs including early myoclonic encephalopathy, Ohtahara syndrome, and West syndrome, and PIGA mutations were carefully investigated. Results: We identified 4 PIGA mutations in probands showing early myoclonic encephalopathy, West syndrome, or unclassified EOEE. Flow cytometry of blood granulocytes from patients demonstrated reduced expression of GPI-anchored proteins. Expression of GPI-anchored proteins in PIGA-deficient JY5 cells was only partially or hardly restored by transient expression of PIGA mutants with a weak TATA box promoter, indicating a variable loss of PIGA activity. The pheno-typic consequences of PIGA mutations can be classified into 2 types, severe and less severe, which correlate with the degree of PIGA activity reduction caused by the mutations. Severe forms involved myoclonus and asymmetrical suppression bursts on EEG, multiple anomalies with a dysmorphic face, and delayed myelination with restricted diffusion patterns in specific areas. The less severe form presented with intellectual disability and treatable seizures without facial dysmorphism. Conclusions: Our study confirmed that PIGA mutations are one genetic cause of EOEE, suggesting that GPI-anchor deficiencies may be an underlying cause of EOEE. © 2014 American Academy of Neurology.

Takemoto J.,Miyagi Childrens Hospital | Yamazaki Y.,Kanagawa Childrens Medical Center | Sakai K.,Miyagi Childrens Hospital
International Journal of Urology | Year: 2011

Bladder hemangioma is a rare benign tumor. While partial cystectomy remains an effective treatment option for large bladder hemangioma, the combined method of neodymium:yttrium aluminium garnet and holmium:yttrium aluminium garnet laser irradiation is an effective and less invasive treatment option. We report a case of large bladder hemangioma in an otherwise healthy 4-year-old boy, successfully treated with serial endoscopic yttrium aluminium garnet laser irradiation. There was no recurrence, and normal bladder function was preserved. Serial yttrium aluminium garnet laser irradiation is thus a useful, less invasive method for cases of large tumors. © 2011 The Japanese Urological Association.

Kunishima S.,National Hospital Organization Nagoya Medical Center | Nishimura S.,University of Tokyo | Nishimura S.,Jichi Medical University | Suzuki H.,Nippon Medical School | And 2 more authors.
European Journal of Haematology | Year: 2014

This report describes a family with TUBB1-associated macrothrombocytopenia diagnosed based on abnormal platelet β1-tubulin distribution. A circumferential marginal microtubule band was undetectable, whereas microtubules were frayed and disorganized in every platelet from the affected individuals. Patients were heterozygous for novel TUBB1 p.F260S that locates at the α- and β-tubulin intradimer interface. Mutant β1-tubulin was not incorporated into microtubules with endogenous α-tubulin, and α-tubulin expression was decreased in transfected Chinese hamster ovary cells. Transduction of mutant β1-tubulin into mouse fetal liver-derived megakaryocytes demonstrated no incorporation of mutant β1-tubulin into microtubules with endogenous α-tubulin and diminished proplatelet formation, leading to the production of fewer, but larger, proplatelet tips. Furthermore, mutant β1-tubulin was not associated with endogenous α-tubulin in the proplatelets. Deficient functional microtubules might lead to defective proplatelet formation and abnormal protrusion-like platelet release, resulting in congenital macrothrombocytopenia. © 2013 John Wiley & Sons A/S.

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