St Lukes International Hospital
St Lukes International Hospital
Niimi Y.,St Lukes International Hospital
Pediatrics International | Year: 2017
Intracranial arteriovenous shunts (ICAVS) in young children are characterized by frequent high-flow fistulas. In association with high-flow fistulas and the physiological condition of the developing brain and heart, each ICAVS type tends to present at a certain age with unique symptoms. Vein of Galen aneurysmal malformation (VGAM) and dural sinus malformation with arteriovenous (AV) shunt tend to present in the neonate with high output cardiac failure. In infancy, VGAM, pial arteriovenous fistula (AVF) and infantile dural AVF (DAVF) tend to present with hydrodynamic disorder such as macrocephaly, ventriculomegaly, prominent facial veins, and developmental delay. Pial AVF, AV malformation, and infantile DAVF can present with focal neurological signs such as seizure or hemorrhage at older ages. Endovascular treatment is currently the first choice of treatment for most pediatric ICAVS. The treatment goal should be defined on a patient-by-patient basis, according to the unique physiological condition of the child. © 2016 Japan Pediatric Society
Kimura S.,St Lukes International Hospital
[Rinsho ketsueki] The Japanese journal of clinical hematology | Year: 2016
Thiopurine S-methyltransferase (TPMT) and nucleoside diphosphate-linked moiety X-type motif 15 (NUDT15) variants are considered to be genes responsible for severe myelotoxicity induced by 6-mercaptopurine (6MP). We report a 4-year-old girl with acute lymphoblastic leukemia, who developed the complication of severe 6MP-induced myelotoxicity due to homozygous NUDT15 variant alleles. In early consolidation therapy containing 6MP, her course was complicated by severe neutropenia (Grade 4) and chemotherapy had to be discontinued for 33 days. The 6MP dose was subsequently adjusted based on the white blood cell count. The ratios of the prescribed 6MP dose over the protocol dose in early consolidation, central nervous system (CNS) prophylaxis, re-induction, interim maintenance and maintenance therapy were 63%, 27%, 4%, 26% and 7%, respectively. Suspension of therapy was required during early consolidation, CNS prophylaxis and interim maintenance therapy. We investigated candidate genes for 6MP-associated myelotoxicity and found homozygous NUDT15 variant alleles and a heterozygous inosine triphosphate pyrophosphatase (ITPA) variant allele. In patients with homozygous NUDT15 variants, drastic reduction (less than 10%) of the 6MP dose from the protocol dose might be required not only during maintenance therapy, but also during other treatment courses containing 6MP. Screening of candidate genes at diagnosis is recommended in order to avoid serious adverse events.
Zanjani K.S.,Tehran University of Medical Sciences |
Niwa K.,St Lukes International Hospital
Journal of Cardiology | Year: 2013
Longer survival after corrective surgery for congenital heart diseases has rendered late complications more important. One of these complications is aortic dilatation which may occur in patients with repaired or unrepaired disease and can progress to aneurysm, dissection, and rupture. This aortic dilatation in various congenital heart diseases does not simply mean anatomical dilatation of the aortic root, but it closely relates to the aortic pathophysiological abnormality, aortic regurgitation, and aortic and ventricular dysfunction; therefore, we can recognize this complex lesion as a new concept: " aortopathy" The pathophysiology of this disease is complex and only partially understood.In this review, we first discuss history, pathophysiology, and clinical features of aortic dilatation and aortopathy of congenital heart disease. Then we provide a review of the evaluation and management of this disease. © 2012 Japanese College of Cardiology.
Ishida Y.,St Lukes International Hospital
International journal of hematology | Year: 2011
Social outcomes and quality of life (QOL) of childhood cancer survivors (CCSs) remain unknown in Japan. We investigated these outcomes in young adult CCSs compared to those of their siblings in Japan, and analyzed the association between social outcome and SF-36 health survey subscale scores. Between 2007 and 2009, we performed a cross-sectional survey using self-rating questionnaires. We estimated social outcomes and health-related QOL by performing the SF-36 in each group: CCSs with or without stem cell transplantation (SCT)/radiotherapy (RT) and their siblings. Adjusted odds ratios for outcomes of interest were estimated using logistic regression analysis. Questionnaires from 185 CCSs and 72 CCS's siblings were analyzed. There were no differences in educational attainment or annual income. The SF-36 subscale scores of CCSs with SCT and RT were significantly lower than those of siblings in physical functioning (PF) (p < 0.001 and 0.003, respectively) and general health (GH) (both p = 0.001). Lower PF scores correlated with recurrence (p = 0.041) and late effects (p = 0.010), and poor GH scores with late effects (p = 0.006). The CCSs had made efforts to attain educational/vocational goals; however, a significant proportion of CCSs who had experienced late effects remain at increased risk of experiencing diminished QOL.
Tamaki H.,St Lukes International Hospital
Modern rheumatology / the Japan Rheumatism Association | Year: 2010
We report a case of refractory adult-onset Still's disease (AOSD) successfully controlled with anakinra, an interleukin-1 (IL-1) receptor antagonist. The patient was a 23-year-old Japanese woman with AOSD who could not be induced into remission despite of two courses of pulsed methylprednisolone followed by high-dose glucocorticoid administration in conjunction with high-dose intravenously administered gamma-globulin and methotrexate. To the best of our knowledge, this is the first case report in Japan of AOSD remission induced with anakinra.
Yamauchi H.,St Lukes International Hospital |
Ueno N.T.,University of Texas M. D. Anderson Cancer Center
Cancer | Year: 2010
Despite the introduction of multimodality treatment approaches, the prognosis of inflammatory breast cancer (IBC) is poor. Recent developments in molecular targeted therapy may be effective against IBC. The authors report the results of a literature review. Trastuzumab and lapatinib, which target human epidermal growth factor receptor 2 (HER-2), have demonstrated benefit in clinical trials for HER-2-positive breast cancers. WNT1-inducible signaling pathway protein 3, Ras homolog gene family member C guanosine triphosphatase, epidermal growth factor receptor (EGFR), and p27kip1 also have been studied as potential targets in IBC. Molecular targets in vasculolymphatic processes (angiogenesis, lymphangiogenesis, and vasculogenesis) have demonstrated greater potential in IBC than in non-IBC. Although loss of E-cadherin is a hallmark of epithelial-to-mesenchymal transition and may correlate with the promotion of metastasis, paradoxically, E-cadherin is overexpressed in IBC through an unknown mechanism. On the basis of dissecting the molecular mechanism of the aggressiveness of IBC, the authors currently are investigating whether EGFR may aid in developing innovative targeted therapies. © 2010 American Cancer Society.
Ohkoshi K.,St Lukes International Hospital |
Yamaguchi T.,St Lukes International Hospital
American Journal of Ophthalmology | Year: 2010
Purpose: To assess the efficacy and safety of subthreshold micropulse diode laser photocoagulation for diabetic macular edema (ME). Design: Prospective, nonrandomized interventional case series. Methods: setting: Institutional. patients: Thirty-six consecutive diabetic patients (43 eyes) with clinically significant ME and a central macular thickness (CMT) <600 μm by optical coherence tomography. observation procedures: Subthreshold micropulse diode laser photocoagulation was done with a 15% duty cycle (0.2 to 0.3 sec; 200 μm) at 50% to 90% of the burn threshold energy. The treated area was monitored on color images for 12 months. main outcome measures: CMT, best-corrected visual acuity (BCVA), and total macular volume at 3 months. Results: After 3 months, there was a significant reduction of CMT (P = .05, paired t test), but the changes of BCVA and macular volume were not significant. The preoperative CMT, BCVA (logarithm of the minimal angle of resolution; logMAR), and macular volume were 341.8 ± 119.0 μm, 0.12 ± 0.20, and 8.763 ± 1.605 mm3 respectively, vs 300.7 ± 124.1 μm, 0.12 ± 0.21, and 8.636 ± 1.408 mm3 at 3 months. CMT decreased significantly from 1 month (P = .015, Friedman test). Visual acuity was improved or maintained within 0.2 logMAR for 12 months in 94.7% of the patients. No obvious laser scars were detected in any patient. Conclusions: In patients with moderate diabetic ME, subthreshold micropulse diode laser photocoagulation controls ME and maintains visual acuity with minimal retinal damage. These findings confirm the efficacy of this method for Japanese patients. © 2010 Elsevier Inc. All rights reserved.
Kojima Y.,St Lukes International Hospital |
Tsunoda H.,St Lukes International Hospital
Breast Cancer | Year: 2011
Background: Triple-negative breast cancer is characterized as a cancer with a high malignancy potential and a poor prognosis. Therefore, early detection of this subtype of breast cancer is vital. In this paper, we describe the mammography and ultrasound findings of triple-negative breast cancer in a large population and investigate the specific features of this subtype. Methods: From January 2007 to April 2010, mammography and ultrasound findings of 88 patients with triplenegative breast cancer were retrospectively reviewed. In this cohort, 52 patients underwent neoadjuvant chemotherapy. We compared the pathological chemotherapy effects and radiological findings among these patients. Mammograms were reviewed according to the Japanese mammography guideline. Ultrasound findings were classified as masses, low echoic area, distortions, and calcifications. Noted features included shapes, patterns of internal echoes, posterior echoes, vascularity, and elasticity scores. Results: On mammography, triple-negative breast cancers frequently presented with a mass (62.4%). Masses with microlobulated margins were the most frequent (39.6%), indistinct (32.0%) and circumscribed (20.8%) were commonly observed, but spiculated margins were rare (4.7%). On ultrasound, cancers were more likely to present as a mass (92.5%), and less likely to show attenuating posterior echoes (8.8%). Of the 40 cases obtained via elasticity imaging, 35 (87.5%) lesions were scored as 4 or 5. There were no significant differences in the mammography or ultrasound findings between the chemotherapy effects. Conclusion: Mammography and ultrasound imaging together revealed that the morphological features of triplenegative breast cancer include a lobulated mass, with less attenuating posterior echoes, some vascularity, and low elasticity. © The Japanese Breast Cancer Society 2010.
Niwa K.,St Lukes International Hospital
Annals of Pediatric Cardiology | Year: 2014
Recently, further expansion of patients with congenital heart disease (CHD) and adults with CHD (ACHD) and evolution of specialized care facilities for pediatric cardiology and ACHD can be anticipated in the Asia-Pacific countries. These patients have a lot of problems to be solved now and in next decade, and they include various medical issues including issues specific to oriental populations, psychosocial issues, establishment of CHD care facilities and so on. We now have two established societies namely, Asia-Pacific Pediatric Cardiology Society (APPCS) and Asia Pacific Society for Adult Congenital Heart Disease (APSACHD). Through these Societies, we should solve these problems by promoting international collaborative studies and support projects to facilitate the rapid progress that is desperately needed in the field in the Asia Pacific Region.
Niwa K.,St Lukes International Hospital
Korean Circulation Journal | Year: 2013
Bicuspid aortic valve and/or coarctation of the aorta are consistently associated with ascending aortic and para-coarctation medial abnormalities. Medial abnormalities in the ascending aorta are prevalent in other types of patients with a variety of forms congenital heart disease (CHD), such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, tetralogy of Fallot. These abnormalities encompass a wide age range, and may predispose to dilatation, aneurysm, and rupture that necessitates aortic valve and root surgery. This dilatation can develop in CHD patients without stenotic region. These CHDs exhibit ongoing dilatation of the aortic root and reduced aortic elasticity and increased aortic stiffness that may relate to intrinsic properties of the aortic root The concept of aortic dilatation is shifting a paradigm of aortic dilatation, as so called post stenotic dilatation, to primary intrinsic aortopahy. These aortic dilatation and increased stiffness can induce aortic aneurysm, rupture of the aorta and aortic regurgitation, but also provoke left ventricular hypertrophy, reduced coronary artery flow and left ventricular failure. We can recognize this association of aortic pathophysiological abnormality, aortic dilation and aorto-left ventricular interaction as a new clinical entity: "aortopathy". Copyright © 2013 The Korean Society of Cardiology.