S Ohga

Summary

Affiliation: Kyushu University
Country: Japan

Publications

  1. doi request reprint Protein C deficiency as the major cause of thrombophilias in childhood
    Shouichi Ohga
    Department of Perinatal and Pediatric Medicine, Kyushu University, Fukuoka, Japan
    Pediatr Int 55:267-71. 2013
  2. doi request reprint Paediatric presentation and outcome of congenital protein C deficiency in Japan
    S Ohga
    Department of Perinatal and Pediatric Medicine, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Haemophilia 19:378-84. 2013
  3. ncbi request reprint Expansion of trisomy 8 and Sweet syndrome in a prolonged course of aplastic anemia
    Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    J Pediatr Hematol Oncol 24:64-8. 2002
  4. doi request reprint Hematopoietic stem cell transplantation for familial hemophagocytic lymphohistiocytosis and Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in Japan
    Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Pediatr Blood Cancer 54:299-306. 2010
  5. ncbi request reprint Dominant expression of interleukin 10 but not interferon gamma in CD4(-)CD8(-)alphabetaT cells of autoimmune lymphoproliferative syndrome
    Shouichi Ohga
    Department of Paediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Br J Haematol 119:535-8. 2002
  6. ncbi request reprint Epstein-Barr virus load in cerebrospinal fluid of patients with chronic active Epstein-Barr virus infection
    Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Pediatr Infect Dis J 27:1027-30. 2008
  7. ncbi request reprint Immunological aspects of Epstein-Barr virus infection
    Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3 1 1 Maidashi Higashi ku, Fukuoka, 812 8582, Japan
    Crit Rev Oncol Hematol 44:203-15. 2002
  8. ncbi request reprint Treatment responses of childhood aplastic anaemia with chromosomal aberrations at diagnosis
    Shouichi Ohga
    Aplastic Anaemia Committee of the Japanese Society of Paediatric Haematology, Tokyo, Japan
    Br J Haematol 118:313-9. 2002
  9. ncbi request reprint Cytokine imbalance in hyper-IgE syndrome: reduced expression of transforming growth factor beta and interferon gamma genes in circulating activated T cells
    Shouichi Ohga
    Department of Paediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Br J Haematol 121:324-31. 2003
  10. ncbi request reprint Early-onset sarcoidosis mimicking refractory cutaneous histiocytosis
    Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Pediatr Blood Cancer 50:723-6. 2008

Collaborators

Detail Information

Publications76

  1. doi request reprint Protein C deficiency as the major cause of thrombophilias in childhood
    Shouichi Ohga
    Department of Perinatal and Pediatric Medicine, Kyushu University, Fukuoka, Japan
    Pediatr Int 55:267-71. 2013
    ..The molecular epidemiology of thrombosis in Asian children would provide a clue to establish the early intervention and optimal anticoagulant therapy in pediatric PC deficiency...
  2. doi request reprint Paediatric presentation and outcome of congenital protein C deficiency in Japan
    S Ohga
    Department of Perinatal and Pediatric Medicine, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Haemophilia 19:378-84. 2013
    ..PC nagoya was prevalent in paediatric, but not adult, patients in Japan. Early maternal screening and optimal PC therapy are required for newborns at risk of PC deficiency...
  3. ncbi request reprint Expansion of trisomy 8 and Sweet syndrome in a prolonged course of aplastic anemia
    Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    J Pediatr Hematol Oncol 24:64-8. 2002
    ..BM study showed myelodysplastic features, in which trisomy 8 occupied 74% of BM cells with additional chromosomal changes. Trisomy 8 may contribute to the late transformation of myeloid lineages in BM failure...
  4. doi request reprint Hematopoietic stem cell transplantation for familial hemophagocytic lymphohistiocytosis and Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in Japan
    Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Pediatr Blood Cancer 54:299-306. 2010
    ..Post-transplant outcomes of hemophagocytic lymphohistiocytosis (HLH) patients were analyzed in Japan where Epstein-Barr virus (EBV)-associated severe forms are problematic...
  5. ncbi request reprint Dominant expression of interleukin 10 but not interferon gamma in CD4(-)CD8(-)alphabetaT cells of autoimmune lymphoproliferative syndrome
    Shouichi Ohga
    Department of Paediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Br J Haematol 119:535-8. 2002
    ..IL-10 was exclusively expressed in DN alphabeta but not (gamma)(delta)T cells. Circulating DN alphabetaT cells may constitutively express IL-10 and contribute to the ALPS phenotype...
  6. ncbi request reprint Epstein-Barr virus load in cerebrospinal fluid of patients with chronic active Epstein-Barr virus infection
    Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Pediatr Infect Dis J 27:1027-30. 2008
    ..In 1 patient, CSF copy number exceeded the peripheral blood value. CSF EBV-load may predict the central nervous system involvement of EBVT-/NK-cells...
  7. ncbi request reprint Immunological aspects of Epstein-Barr virus infection
    Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3 1 1 Maidashi Higashi ku, Fukuoka, 812 8582, Japan
    Crit Rev Oncol Hematol 44:203-15. 2002
    ....
  8. ncbi request reprint Treatment responses of childhood aplastic anaemia with chromosomal aberrations at diagnosis
    Shouichi Ohga
    Aplastic Anaemia Committee of the Japanese Society of Paediatric Haematology, Tokyo, Japan
    Br J Haematol 118:313-9. 2002
    ..These results suggest that IST can be applied as the initial therapy for AA with cytogenetic abnormalities in the absence of completely matched donors...
  9. ncbi request reprint Cytokine imbalance in hyper-IgE syndrome: reduced expression of transforming growth factor beta and interferon gamma genes in circulating activated T cells
    Shouichi Ohga
    Department of Paediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Br J Haematol 121:324-31. 2003
    ..The reduced TGFbeta expression may involve the indigenous T-cell defects of HIES...
  10. ncbi request reprint Early-onset sarcoidosis mimicking refractory cutaneous histiocytosis
    Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Pediatr Blood Cancer 50:723-6. 2008
    ..A biopsy of nodules confirmed the diagnosis of sarcoidosis. The atypical EOS should be differentiated from histiocytosis...
  11. ncbi request reprint Dominant beta-thalassemia with hemoglobin Hradec Kralove: enhanced hemolysis in the spleen
    Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Int J Hematol 78:329-34. 2003
    ..Because of the variable augmentation of extramedullary hemolysis in dominant beta-thalassemias, genotyping is necessary for determining the clinical indication of splenectomy...
  12. ncbi request reprint Successful self-infusion of activated prothrombin complex concentrate for prophylaxis in a child with a factor VIII inhibitor
    Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Am J Hematol 82:145-9. 2007
    ..His quality of life was improved, particularly with respect to school. Our long observation proposes a well-disciplined home-based FEIBA prophylaxis in inhibitor-positive hemophiliacs...
  13. ncbi request reprint Unrelated donor cord blood transplantation for childhood severe aplastic anemia after a modified conditioning
    Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Pediatr Transplant 10:497-500. 2006
    ..Hematological recovery was favorable in complete chimerism. A major complication was only skin graft-versus-host disease (grade I). CB could be an alternate stem cell source for childhood SAA after modified preparative regimen...
  14. ncbi request reprint Quantitative monitoring of circulating Epstein-Barr virus DNA for predicting the development of posttransplantation lymphoproliferative disease
    S Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Int J Hematol 73:323-6. 2001
    ..Sequential monitoring of circulating EBV-DNA by quantitative PCR may be a useful indicator for predicting the development of posttransplantation LPD...
  15. doi request reprint Clonal origin of Epstein-Barr virus (EBV)-infected T/NK-cell subpopulations in EBV-positive T/NK-cell lymphoproliferative disorders of childhood
    Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3 1 1 Maidashi, Higashi ku, Fukuoka 812 8582, Japan
    J Clin Virol 51:31-7. 2011
    ..The clonal nature of viral infection within lymphoid subsets of patients with EBV(+)LPD of childhood is not well described...
  16. doi request reprint Thromboembolic complications in splenectomized patients with dominantly inherited beta-thalassemia
    Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Acta Haematol 120:31-5. 2008
  17. ncbi request reprint Dominant expression of interleukin-10 and transforming growth factor-beta genes in activated T-cells of chronic active Epstein-Barr virus infection
    Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3 1 1 Maidashi, Higashi ku, Fukuoka 812 8582, Japan
    J Med Virol 74:449-58. 2004
    ..The dominant expression of regulatory cytokines in T-cells may imply a viral evasion mechanism in the disease...
  18. ncbi request reprint Epstein-Barr virus (EBV) load and cytokine gene expression in activated T cells of chronic active EBV infection
    S Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812 8582, Japan
    J Infect Dis 183:1-7. 2001
    ..EBV-infected T cells may contribute to the unbalanced cytokine profiles of chronic mononucleosis...
  19. ncbi request reprint Restricted diversification of T-cells in chronic active Epstein-Barr virus infection: potential inclination to T-lymphoproliferative disease
    S Ohga
    Department of Pediatrics, Faculty of Medicine, Kyushu University, Fukuoka, Japan
    Am J Hematol 61:26-33. 1999
    ..Although the study is limited by the small number of patients, the unbalanced T-cell repertoire might contribute to the evolution of T-lymphoproliferative disease, otherwise, imply the innate defective immunity to EBV in CAEBV patients...
  20. pmc Maturational alterations of peripheral T cell subsets and cytokine gene expression in 22q11.2 deletion syndrome
    Y Kanaya
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Maidashi, Fukuoka, Japan
    Clin Exp Immunol 144:85-93. 2006
    ..2 deletion patients became less severe with age under the altered composition of minor subsets. The balanced cytokine profile in the limited T cell pool may represent a T cell homeostasis in thymic deficiency syndrome...
  21. ncbi request reprint Functional analyses of cord blood natural killer cells and T cells: a distinctive interleukin-18 response
    A Nomura
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Exp Hematol 29:1169-76. 2001
    ....
  22. pmc Increased serum levels of interferon-gamma-inducible protein 10 and monokine induced by gamma interferon in patients with haemophagocytic lymphohistiocytosis
    H Takada
    Department of Paediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Clin Exp Immunol 133:448-53. 2003
    ..These results suggest that IP-10 and MIG which are produced by activated macrophages by the stimulation of IFN-gamma, play an important role in the pathophysiology of HLH, by recruitment of activated Th1 cells into the tissues or organs...
  23. doi request reprint Genetic variation of vascular endothelial growth factor pathway does not correlate with the severity of retinopathy of prematurity
    T Kusuda
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    J Perinatol 31:246-50. 2011
    ..The aim of this study was to assess the genetic effects of the vascular endothelial growth factor (VEGF) pathway on retinopathy of prematurity (ROP)...
  24. doi request reprint Recurrent atrial fibrillation after high-dose methylprednisolone therapy in a girl with lupus-associated hemophagocytic syndrome
    K Yamamura
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Lupus 20:871-5. 2011
    ..Lupus-HPS may be an aggressive SLE subset with cardiac involvement. High-dose corticosteroid infusion controls lupus activity, but could disclose the cardiac stress in lupus-HPS patients...
  25. pmc Distinct gene expression patterns of peripheral blood cells in hyper-IgE syndrome
    T Tanaka
    Department of Paediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Clin Exp Immunol 140:524-31. 2005
    ..The distinct patterns of gene expression profiles and RGC32 expression levels will be useful for understanding the pathophysiology and for diagnosis of HIES, respectively...
  26. ncbi request reprint Functional T cell receptor delta chain gene messages in athymic nude mice
    Y Yoshikai
    Department of Immunology, Kyushu University, Fukuoka, Japan
    Eur J Immunol 18:1039-43. 1988
    ..Taken together, T cell precursors appear to proliferate and differentiate along the extrathymic pathway into TcR gamma/delta-bearing T cells in nude mice...
  27. ncbi request reprint Neonatal asphyxia and renal failure as the presentation of non-inherited protein C deficiency
    Y Matsunaga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    J Perinatol 33:239-41. 2013
    ..Renal function and PC activity have been slowly normalized until 6 months of age. He had no PROC mutation or PC-deficient parents. Selective PC deficiency may occur as an acquired cause of hypercoagulable crisis in the stressed newborn...
  28. ncbi request reprint Haptoglobin therapy for acute favism: a Japanese boy with glucose-6-phosphate dehydrogenase Guadalajara
    S Ohga
    Department of Paediatrics, Faculty of Medicine, Kyushu University, Fukuoka, Japan
    Br J Haematol 89:421-3. 1995
    ..Haptoglobin treatment might be helpful for managing the haemolytic crisis in the disease...
  29. doi request reprint NEMO mutation as a cause of familial occurrence of Behçet's disease in female patients
    H Takada
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Clin Genet 78:575-9. 2010
    ..Heterozygous NEMO mutation is a cause of familial occurrence of Behçet's disease in female patients...
  30. ncbi request reprint Deletion of self-reactive T cells in the donor-derived T cells but not in the host-derived T cells in fully allogeneic radiation chimeras. Mls-reactive T cells in allogeneic radiation chimeras
    M Ogimoto
    Department of Immunology, Kyushu University, Fukuoka, Japan
    Thymus 17:11-22. 1991
    ..These results suggested that the host-derived thymocytes may likely to escape undergoing a negative selection against donor-phenotype in the radiation bone marrow chimeras...
  31. ncbi request reprint Interleukin-18 in hemophagocytic lymphohistiocytosis
    H Takada
    Department of Pediatrics, Graduate School of Medicine Sciences, Kyushu University, Maidashi, Higashi-ku, Fukuoka 812-8582, Japan
    Leuk Lymphoma 42:21-8. 2001
    ..IL-18 may play important roles in the pathogenesis of HLH, particularly through induction of Th1 cells. In addition, IL-18 measurement may not only be useful for the diagnosis, but also for the evaluation of disease activity...
  32. ncbi request reprint Inflammatory bowel disease-like colitis in glycogen storage disease type 1b
    T Yamaguchi
    Department of Pediatric, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Inflamm Bowel Dis 7:128-32. 2001
    ..These findings suggest that IBD-like colitis is a grave complication of GSD-1b and that recurrent enteric infections due to neutrophil deficiency may contribute to the development of this bowel disease...
  33. pmc Microarray analysis of human milk cells: persistent high expression of osteopontin during the lactation period
    T Nagatomo
    Department of Paediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Clin Exp Immunol 138:47-53. 2004
    ..These results suggest that the persistently and extraordinarily high expression of OPN in human milk cells plays a potential role in the immunological development of breast-fed infants...
  34. pmc Identification of novel MUNC13-4 mutations in familial haemophagocytic lymphohistiocytosis and functional analysis of MUNC13-4-deficient cytotoxic T lymphocytes
    K Yamamoto
    Division of Molecular Population Genetics, Department of Molecular Genetics, Medical Institute of Bioregulation, and Kyushu University COE Programme on Lifestyle Related Diseases, Kyushu University, Japan
    J Med Genet 41:763-7. 2004
    ..FHL2 subtype with perforin (PRF1) mutation accounts for 30% of all FHL cases, while FHL with MUNC13-4 mutation was recently identified and designated as FHL3 subtype...
  35. ncbi request reprint Transcriptional regulation of laccase and cellulase genes during growth and fruiting of Lentinula edodes on supplemented sawdust
    S Ohga
    Department of Forest and Forest Products Science, Faculty of Agriculture, Kyushu University, Fukuoka, Japan
    FEMS Microbiol Lett 201:111-5. 2001
    ..Gene expressions for laccase and cellulase were triggered by microclimatic changes, specifically lower temperature and osmotic pressure...
  36. ncbi request reprint Augmentation of host defense against bacterial infection pretreated intraperitoneally with an alpha-glucan RBS in mice
    Y Takeda
    Department of Immunology, Medical Institute of Bioregulation, Kyushu University, Fukuoka, Japan
    Immunopharmacol Immunotoxicol 12:457-77. 1990
    ..These results suggest that adequately enhanced activities of macrophages acting as scavenger phagocytes play important roles in the enhanced resistance against bacteria in mice treated i.p. with RBS...
  37. ncbi request reprint Moyamoya syndrome with spherocytosis: effect of splenectomy on strokes
    Y Tokunaga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Pediatr Neurol 25:75-7. 2001
    ..This rare observation suggests that anemic hypoxia more greatly contributes to the progression of moyamoya syndrome than postsplenectomy thrombocytosis or reduced deformability of spherocytes...
  38. ncbi request reprint H(2)O(2)-nonproducing Streptococcus pyogenes strains: survival in stationary phase and virulence in chronic granulomatous disease
    M Saito
    Departments of Bacteriology and Pediatrics, Faculty of Medical Sciences, Kyushu University, 3 1 1 Maidashi, Higashi ku, Fukuoka 812 8582, Japan
    Microbiology 147:2469-77. 2001
    ..It is suggested that H(2)O(2)-nonproducing S. pyogenes strains are prevalent in humans and that they may be a potential threat to the health of CGD patients...
  39. ncbi request reprint Hematopoietic stem cell transplantation for Diamond-Blackfan anemia: a report from the Aplastic Anemia Committee of the Japanese Society of Pediatric Hematology
    H Mugishima
    Department of Advanced Medicine, Division of Cell Regeneration and Transplantation, Nihon University School of Medicine, Tokyo, Japan
    Pediatr Transplant 11:601-7. 2007
    ..030). No other factors were associated with engraftment and survival. These results suggest that allogeneic BMT, but not unrelated CBT, is an effective HSCT for refractory DBA...
  40. ncbi request reprint Molecular analysis of glucose phosphate isomerase deficiency associated with hereditary hemolytic anemia
    H Kanno
    Okinaka Memorial Institute for Medical Research, Tokyo, Japan
    Blood 88:2321-5. 1996
    ..The molecular characterization of human GPI variants, therefore, may provide new insights into the genotype-phenotype correlation of GPI deficiency as well as the structure-function relationship of this enzyme...
  41. ncbi request reprint Sjogren's syndrome-associated meningoencephalomyelitis: cerebrospinal fluid cytokine levels and therapeutic utility of tacrolimus
    Takayuki Hoshina
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3 1 1 Maidashi, Fukuoka 812 8582, Japan
    J Neurol Sci 267:182-6. 2008
    ..The unique cytokine profile, differing from those of infectious meningitis may be useful for predicting the central nervous system involvement in autoimmune disease...
  42. ncbi request reprint Malignant hepatic tumor occurring 10 yrs after a histocompatible sibling donor bone marrow transplantation for severe aplastic anemia
    Masataka Ishimura
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Pediatr Transplant 11:945-9. 2007
    ..Prolonged mixed chimerism and hepatitis virus infection might induce a rare oncogenesis after non-irradiated conditioning...
  43. ncbi request reprint Nationwide survey of hemophagocytic lymphohistiocytosis in Japan
    Eiichi Ishii
    Department of Pediatrics, Ehime University Graduate School of Medicine, Ehime, Japan
    Int J Hematol 86:58-65. 2007
    ..Although this nationwide survey establishes the heterogeneous characteristics of HLH, the results should be useful in planning prospective studies to identify the most effective therapy for each HLH subtype...
  44. ncbi request reprint Prognostic factors for chronic active Epstein-Barr virus infection
    Hiroshi Kimura
    Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
    J Infect Dis 187:527-33. 2003
    ..59 vs. 0.87; P<.009). Patients with CAEBV with late onset of disease, thrombocytopenia, and T cell infection had significantly poorer outcomes...
  45. ncbi request reprint Successful prolonged rituximab treatment for post-transplant lymphoproliferative disorder following living donor liver transplantation in a child
    Makoto Hayashida
    Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Higashi ku, Fukuoka, Japan
    Pediatr Transplant 11:671-5. 2007
    ..Rituximab was administered at a dose of 375 mg/m2 once a week, and the monotherapy resulted in a complete remission after 34 administrations. Based on this case, rituximab appears to be beneficial as a first-line therapy for PTLD...
  46. ncbi request reprint A new scoring system for computed tomography of the chest for assessing the clinical status of bronchopulmonary dysplasia
    Masayuki Ochiai
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    J Pediatr 152:90-5, 95.e1-3. 2008
    ..To develop a new scoring system for computed tomography (CT) of the chest for assessing the clinical status of patients with bronchopulmonary dysplasia (BPD) in comparison with a modified Edwards roentgenographic scoring system...
  47. doi request reprint Lipopolysaccharide-induced monocytic cell death for the diagnosis of mild neonatal-onset multisystem inflammatory disease
    Hidetoshi Takada
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    J Pediatr 152:885-7, 887.e1. 2008
    ..His peripheral blood monocytes died within 24 hours after lipopolysaccharide stimulation, a test that may be useful for diagnosis even in mild cases...
  48. doi request reprint Head circumference and long-term outcome in small-for-gestational age infants
    Masayuki Ochiai
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Higashi ku, Fukuoka, Japan
    J Perinat Med 36:341-7. 2008
    ..To assess risk factors for the growth and development of small-for-gestational age (SGA) infants whose birth weight was less than the 10(th) percentile...
  49. ncbi request reprint Allogeneic hematopoietic stem cell transplantation for Epstein-Barr virus-associated T/natural killer-cell lymphoproliferative disease in Japan
    Emiko Sato
    Department of Pediatrics, Osaka Medical Center and Research Institute for Maternal and Child Health, 840 Murodo cho, Izumi, Osaka, Japan
    Am J Hematol 83:721-7. 2008
    ..563 +/- 0.124. Thus, in a substantial number of cases, EBV-associated T/NK-LPD can be cured by either allogeneic conventional stem cell transplantation or reduced-intensity stem cell transplantation...
  50. ncbi request reprint Review of hemophagocytic lymphohistiocytosis (HLH) in children with focus on Japanese experiences
    Eiichi Ishii
    Department of Pediatrics, Faculty of Medicine, Saga University, 5 1 1 Nabeshima, Saga 849 8501, Japan
    Crit Rev Oncol Hematol 53:209-23. 2005
    ..In the near future, an entire pathogenesis should be clarified in order to establish appropriate therapies including immunotherapy, HSCT and gene therapy...
  51. ncbi request reprint Distinct clinical phenotype and immunoreactivity in Japanese siblings with autoimmune polyglandular syndrome type 1 (APS-1) associated with compound heterozygous novel AIRE gene mutations
    Kazuhiko Kogawa
    Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, Japan
    Clin Immunol 103:277-83. 2002
    ..The same AIRE gene mutations can thus be associated with characteristic and noncharacteristic phenotypes of APS-1, and HLA may possibly influence the phenotype of APS-1...
  52. ncbi request reprint Optic disk vasculitis associated with chronic active Epstein-Barr virus infection
    Masahiro Yamamoto
    Department of Ophthalmology, Faculty of Medicine, Kyushu University, Fukuoka, Japan
    Ophthalmologica 216:221-5. 2002
    ..To report two cases of optic disk vasculitis associated with chronic active Epstein-Barr virus infection (CAEBV)...
  53. ncbi request reprint Perforin defects of primary haemophagocytic lymphohistiocytosis in Japan
    Naohiro Suga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Br J Haematol 116:346-9. 2002
    ..The frequency of mutation was at least 20% of FHL in Japan. Flow cytometry for intracellular perforin may be useful for the screening of FHL2...
  54. ncbi request reprint Perforin gene mutations in adult-onset hemophagocytic lymphohistiocytosis
    Koji Nagafuji
    Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, Fukuoka Japan
    Haematologica 92:978-81. 2007
    ..Although primary HLH has been detected in infants and children, genetic mutation of PRF1 or other genes should be considered a differential diagnosis of HLH even in the elderly...
  55. ncbi request reprint Differential chemokine, chemokine receptor and cytokine expression in Epstein-Barr virus-associated lymphoproliferative diseases
    Koichi Ohshima
    Department of Pathology, School of Medicine, Fukuoka University, Nanakuma 7 45 1, Jonan ku, Fukuoka 814 01, Japan
    Leuk Lymphoma 44:1367-78. 2003
    ..Our study demonstrated the enormous potential of gene expression profiling for clarifying the pathogenesis of EBV-associated diseases...
  56. ncbi request reprint Genetic subtypes of familial hemophagocytic lymphohistiocytosis: correlations with clinical features and cytotoxic T lymphocyte/natural killer cell functions
    Eiichi Ishii
    Department of Pediatrics, Faculty of Medicine, Saga University, 5 1 1 Nabeshima, Saga 849 8501, Japan
    Blood 105:3442-8. 2005
    ..These results suggest an association between the type of genetic mutation in FHL cases and the magnitude of CTL cytolytic activity and age at onset...
  57. ncbi request reprint Successful unrelated cord blood transplantation for Epstein-Barr virus-associated lymphoproliferative disease with hemophagocytic syndrome
    Taikai Toubo
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Int J Hematol 80:458-62. 2004
    ..There has been no previous report of successful CBT for EBV-related LPD/lymphoma. CBT can be a curative treatment for the disease, even if no viral memory has been set in the stem cell source...
  58. ncbi request reprint Increased IL-16 levels in hemophagocytic lymphohistiocytosis
    Hidetoshi Takada
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    J Pediatr Hematol Oncol 26:567-73. 2004
    ..An increase in IL-16 mRNA expression was detected in the liver of an HLH patient. These results suggest that IL-16 plays an important role in the pathophysiology of HLH by TH1 cell recruitment and activation at organs with inflammation...
  59. ncbi request reprint Bronchiolitis obliterans organizing pneumonia as an initial manifestation in systemic lupus erythematosus
    Hidetoshi Takada
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Pediatr Pulmonol 40:257-60. 2005
    ..Although it is a rare complication among the pleuro-pulmonary manifestations in SLE, BOOP can be the first presentation, even in pediatric patients...
  60. pmc Impaired host defense against Sporothrix schenckii in mice with chronic granulomatous disease
    Hideko Kajiwara
    Department of Bacteriology, Faculty of Medical Sciences, Kyushu University, 3 1 1 Maidashi, Higashi ku, Fukuoka 812 8582, Japan
    Infect Immun 72:5073-9. 2004
    ..schenckii infection and (ii) superoxide anion and its reactive oxidative metabolites produced by neutrophils and macrophages are involved in fungistatic and fungicidal activities...
  61. ncbi request reprint A novel CIAS1mutation in a Japanese patient with chronic infantile neurological cutaneous and articular syndrome
    Hidetoshi Takada
    Department of Paediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Eur J Pediatr 164:785-6. 2005
  62. ncbi request reprint Successful umbilical cord blood transplantation for severe chronic active Epstein-Barr virus infection after the double failure of hematopoietic stem cell transplantation
    Masataka Ishimura
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Am J Hematol 80:207-12. 2005
    ..This is the first successful report of CBT for CAEBV. CB may therefore be an alternate source of stem cells for the curative treatment of CAEBV, despite the absence of EBV-specific cytotoxic T lymphocytes...
  63. ncbi request reprint Correlation between phenotypic heterogeneity and gene mutational characteristics in familial hemophagocytic lymphohistiocytosis (FHL)
    Ikuyo Ueda
    Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan
    Pediatr Blood Cancer 46:482-8. 2006
    ..Classification of familial hemophagocytic lymphohistiocytosis (FHL) into FHL2, FHL3, and other subtypes based on genetic abnormalities has recently become possible. We studied the phenotypic differences among these subtypes in Japan...
  64. ncbi request reprint CD25+CD4+ T cells in human cord blood: an immunoregulatory subset with naive phenotype and specific expression of forkhead box p3 (Foxp3) gene
    Yasushi Takahata
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Higashi ku, Fukuoka, Japan
    Exp Hematol 32:622-9. 2004
    ..To address the role of cord blood (CB) CD25+CD4+ T cells, the gene expressions and function of this subset were analyzed...
  65. ncbi request reprint A case of chronic recurrent uveitis associated with chronic granulomatous disease
    Toshie Matsuura
    Jpn J Ophthalmol 50:287-9. 2006
  66. ncbi request reprint Clinicopathological states of Epstein-Barr virus-associated T/NK-cell lymphoproliferative disorders (severe chronic active EBV infection) of children and young adults
    Keiko Suzuki
    First Department of Internal Medicine, School of Medicine, Fukuoka University, Fukuoka, Japan
    Int J Oncol 24:1165-74. 2004
    ..The EBV genotype did not differ between the groups. Our findings support an important pathogenic role for EBV-infected T/NK-cell infection, rather than the EBV state, in CAEBV and consequent EBV-associated NK/T-neoplasia...
  67. ncbi request reprint Long-term outcome of infants with twin-to-twin transfusion syndrome
    Shunji Hikino
    Perinatal and Maternity Care Unit, Kyushu University Hospital, Fukuoka, Japan
    Fetal Diagn Ther 22:68-74. 2007
    ..To clarify the long-term neurodevelopmental outcome of twin-to-twin transfusion syndrome (TTTS) survivors...
  68. ncbi request reprint Diamond-Blackfan anemia in Japan: clinical outcomes of prednisolone therapy and hematopoietic stem cell transplantation
    Shouichi Ohga
    Department of Pediatrics, Kyushu University, Fukuoka, Japan
    Int J Hematol 79:22-30. 2004
    ..Two cord blood transplants from unrelated donors failed. These findings suggest the need for developing an integral treatment strategy including selective HSCT for refractory DBA...
  69. ncbi request reprint Rheumatic fever-mimicking carditis as a first presentation of chronic active Epstein-Barr virus infection
    Taikai Toubo
    Department of Paediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Acta Paediatr 95:614-8. 2006
    ..The atypical carditis without valvitis was then complicated by coronary artery dilatations. Four months after the start of prednisolone plus antiviral/bacterial therapy, EBV+ T-cell lymphoma developed in the thigh...
  70. ncbi request reprint T-cell-depleted CD34+ cell transplantation from an HLA-mismatched donor in a low-birthweight infant with X-linked severe combined immunodeficiency
    Akihiko Nomura
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    J Pediatr Hematol Oncol 27:80-4. 2005
    ..Analysis of T-cell regeneration pathways revealed that T cells reconstituted mainly via the thymus-dependent pathway. T-cell-depleted CD34+ cell transplantation would be a safe and useful therapy for LBW infants with SCID...
  71. ncbi request reprint Moyamoya syndrome in a splenectomized patient with beta-thalassemia intermedia
    Masafumi Sanefuji
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Japan
    J Child Neurol 21:75-7. 2006
    ..Cerebral vasculopathy can be a rare but grave consequence of the thromboembolic complications in beta-thalassemia major/intermedia. (J Child Neurol 2006;21:75-77)...
  72. ncbi request reprint Prospective multicenter trial comparing repeated immunosuppressive therapy with stem-cell transplantation from an alternative donor as second-line treatment for children with severe and very severe aplastic anemia
    Yoshiyuki Kosaka
    Department of Pediatrics, Hyogo Children Hospital, Hyogo, Japan
    Blood 111:1054-9. 2008
    ..1%, SD) in the SCT group compared with 9.5% (+/- 9.0%) in the IST group (P = .001). These results suggest that SCT from an alternative donor offers a better chance of FFS than a second IST in patients not responding to an initial IST...
  73. ncbi request reprint Proposed guidelines for diagnosing chronic active Epstein-Barr virus infection
    Motohiko Okano
    Department of Pediatrics, Hokkaido University Hospital, Hokkaido University Graduate School of Medicine, Sapporo, Japan
    Am J Hematol 80:64-9. 2005
    ..Guidelines, derived mainly from the current literature and recent experiences with CAEBV in Japan, for diagnosing CAEBV are proposed to clarify this enigmatic disease...
  74. ncbi request reprint CD4-CD8- T-cell polymyositis in a patient with chronic active Epstein-Barr virus infection
    Sachiko Tsutsumi
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812 8582, Japan
    Am J Hematol 71:211-5. 2002
    ..The ominous polymyositis might denote the musculotropic invasion of EBV+gammadeltaT-cell lymphoproliferative disease as a consequence of CAEBV...
  75. ncbi request reprint Mutations of syntaxin 11 and SNAP23 genes as causes of familial hemophagocytic lymphohistiocytosis were not found in Japanese people
    Ken Yamamoto
    Department of Molecular Genetics, Medical Institute of Bioregulation, Kyushu University, Fukuoka, Japan
    J Hum Genet 50:600-3. 2005
    ..The patients had no mutations and 10% had one polymorphism (146G>A) of syntaxin 11, while no mutation of SNAP23 was observed. Our results indicate that aberrations in the SNARE system may not cause FHL in Japanese families...
  76. ncbi request reprint A four-year-old girl with cough, haemoptysis and anaemia
    Hideki Nakayama
    Department of Paediatrics, Kyushu University Hospital, Fukuoka, Japan
    Acta Paediatr 95:1707-9. 2006