掲載種別：研究論文（学術雑誌）   出版者・発行元：Wiley  

DOI： 10.1002/pbc.29699

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その他リンク： https://onlinelibrary.wiley.com/doi/full-xml/10.1002/pbc.29699

記述言語：英語  

DOI： 10.1007/s12185-021-03269-6

PubMed

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記述言語：英語  

Pediococcus spp. have been reported to cause infections in patients with underlying conditions. However, the pathogenicity of this bacteria is unclear. Herein, we describe the first case of Pediococcus acidilactici bacteremia, which occurred in a 16-year-old male with dasatinib-induced hemorrhagic colitis during maintenance therapy for leukemia and resolved without antibiotic treatment. P. acidilactici bacteremia might be self-limiting, even in immunocompromised patients receiving chemotherapy.

DOI： 10.1016/j.idcr.2022.e01384

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記述言語：英語  

DOI： 10.1002/pbc.29501

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

The characteristics of the tumor immune microenvironment remains unclear in liposarcomas, and here we aimed to determine the prognostic impact of the tumor immune microenvironment across separate liposarcomas subtypes. A total of 70 liposarcoma patients with three subtypes: myxoid liposarcoma (n = 45), dedifferentiated liposarcoma (n = 17), and pleomorphic liposarcoma (n = 8) were enrolled. The presence of tumor infiltrating lymphocytes (CD4+ , CD8+ , FOXP3+ lymphocytes) and CD163+ macrophages and expression of HLA class I and PD-L1 were assessed by immunohistochemistry in the diagnostic samples; overall survival and progression-free survival were estimated from outcome data. For infiltrating lymphocytes and macrophages, dedifferentiated liposarcoma and pleomorphic liposarcoma patients had a significantly higher number than myxoid liposarcoma patients. While myxoid liposarcoma patients with a high number of macrophages were associated with worse overall and progression-free survival, dedifferentiated liposarcoma patients with high macrophage numbers showed a trend toward favorable prognosis. Expression of HLA class I was negative in 35 of 45 (77.8%) myxoid liposarcoma tumors, whereas all dedifferentiated liposarcoma and pleomorphic liposarcoma tumors expressed HLA class I. The subset of myxoid liposarcoma patients with high HLA class I expression had significantly poor overall and progression-free survival, while dedifferentiated liposarcoma patients with high HLA class I expression tended to have favorable outcomes. Only four of 17 (23.5%) dedifferentiated liposarcomas, two of eight (25%) pleomorphic liposarcomas, and no myxoid liposarcoma tumors expressed PD-L1. Our results demonstrate the unique immune microenvironment of myxoid liposarcomas compared to other subtypes of liposarcomas, suggesting that the approach for immunotherapy in liposarcomas should be based on subtype.

DOI： 10.1007/s00262-021-02928-1

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Synovial sarcoma is a rare malignant soft-tissue tumor that is prevalent in adolescents and young adults, and poor prognosis has been reported in patients with metastatic lesions. Chimeric antigen receptor (CAR) T-cell therapy is an emerging novel therapy for solid tumors; however, its application in synovial sarcoma has not yet been explored. METHODS: A novel human epidermal growth factor receptor 2 (HER2)-targeted CAR containing scFv-FRP5, CD8α hinge and transmembrane domains as well as 4-1BB costimulatory and CD3ζ signaling domains was developed. Three synovial sarcoma cell lines that expressed the fusion transcript SS18-SSX1/2/4 were used in the study. Cytokine secretion assay, cytotoxicity assay, and real-time cell analysis experiments were conducted to confirm the function of T cells transduced with the CAR gene. RESULTS: High cell-surface expression of HER2 was observed in all the cell lines. HER2-targeted/4-1BB-costimulated CAR T cells specifically recognized the synovial sarcoma cells, secreted interferon gamma and tumor necrosis factor alpha, and exerted cytotoxic effects in these cells. CONCLUSION: To the best of our knowledge, this is the first study to indicate that HER2-targeted CAR T cells are directly effective against molecularly defined synovial sarcoma cells. Furthermore, our findings might set the basis for developing improved CAR T cell-based therapies for chemo-refractory or relapsed synovial sarcoma.

DOI： 10.1016/j.tranon.2021.101227

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記述言語：日本語   出版者・発行元：(株)北隆館  

CAR-T細胞療法は、再発・難治性の小児思春期白血病ならびに成人B細胞性リンパ腫において劇的な臨床効果を発揮し、すでに本邦においても保険診療として実施されている。急性骨髄性白血病や固形がんへの応用も強く期待されており、現在までに多くの臨床試験が実施されているものの、必ずしも十分な成果が挙がっているとは言えない。一方、小児の悪性固形腫瘍に対しては古くから臨床試験が実施され、一部の症例で治療効果が示されている。本稿では、小児の悪性腫瘍におけるCAR-T細胞療法の臨床開発の現状について述べる。(著者抄録)

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記述言語：日本語   出版者・発行元：(株)北隆館  

骨軟部腫瘍における免疫環境の研究は未開拓分野である。筆者らは肉腫の一部に自然寛解がみられることより、骨軟部腫瘍においても免疫環境が予後に影響を持つものと考え研究を続けている。これまでHLA class Iの腫瘍細胞おける発現、CD8陽性リンパ球、CD163陽性マクロファージ浸潤を解析してきたが、組織型ごとに特徴や予後に関する影響も異なっていた。今後はCAR-T細胞を用いた細胞療法等が新たな治療戦略になることを期待し研究を続けている。(著者抄録)

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

We generated dual-antigen receptor (DR) T cells from induced pluripotent stem cells (iPSCs) to mitigate tumor antigen escape. These cells were engineered to express a chimeric antigen receptor (CAR) for the antigen cell surface latent membrane protein 1 (LMP1; LMP1-CAR) and a T cell receptor directed to cell surface latent membrane protein 2 (LMP2), in association with human leucocyte antigen A24, to treat therapy-refractory Epstein-Barr virus-associated lymphomas. We introduced LMP1-CAR into iPSCs derived from LMP2-specific cytotoxic T lymphocytes (CTLs) to generate rejuvenated CTLs (rejTs) active against LMP1 and LMP2, or DRrejTs. All DRrejT-treated mice survived >100 days. Furthermore, DRrejTs rejected follow-up inocula of lymphoma cells, demonstrating that DRrejTs persisted long-term. We also demonstrated that DRrejTs targeting CD19 and LMP2 antigens exhibited a robust tumor suppressive effect and conferred a clear survival advantage. Co-operative antitumor effect and in vivo persistence, with unlimited availability of DRrejT therapy, will provide powerful and sustainable T cell immunotherapy.

DOI： 10.1016/j.ymthe.2021.10.006

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児神経外科学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Patients with Ewing's sarcoma family of tumors (ESFT) who experience relapse or progression have a poor prognosis. AIM: This study aimed to identify the prognostic and therapeutic factors affecting overall survival (OS) of patients with recurrent or refractory localized ESFT. METHODS AND RESULTS: Thirty-eight patients with localized ESFT who experienced first relapse or progression between 2000 and 2018 were retrospectively reviewed. The 5-year OS rate of the entire cohort was 48.3% (95% confidence interval, 29.9%-64.5%). Multivariate analysis of OS identified time to relapse or progression, but not stem cell transplantation (SCT), as the sole independent risk factor (hazard ratio, 35.8; P = .002). Among 31 patients who received salvage chemotherapy before local treatment, 21 received chemotherapy regimens that are not conventionally used for newly diagnosed ESFT. The objective response rate to first-line salvage chemotherapy was 55.2% in the 29 evaluable patients. Time to relapse or progression was significantly associated with response to first-line salvage chemotherapy (P = .006). CONCLUSIONS: The present study fails to demonstrate significant clinical benefit of SCT for recurrent or refractory localized ESFT. Recently established chemotherapy regimens may increase the survival rate of patients with recurrent or refractory localized ESFT while attenuating the beneficial effect of SCT.

DOI： 10.1002/cnr2.1329

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記述言語：日本語   出版者・発行元：(株)日本小児医事出版社  

症例は12歳男児。両側の手指、足趾、手掌の肢端紅痛症で発症し、99.7万/μLの血小板増多を認め、骨髄所見やJAK2変異陽性から、本態性血小板血症(essential thrombocythemia;ET)と診断した。肢端紅痛症に対し少量アスピリン投与を開始したところ、症状は速やかに消失した。アスピリン投与開始後、血小板数は140万/μLへと増加傾向を示したため、後天性フォンウィルブランド症候群の検索を行い、合併が判明した。ガイドラインではアスピリンは原則禁忌となり細胞減少療法の適応となるが、本症例では出血症状は皆無であり、年齢や殺細胞薬使用に伴う白血病転化リスクを考慮した結果、アスピリン療法単独を継続し、6年経過した現在まで安全に管理できている。小児ETは非常に稀な疾患であり、治療アルゴリズムは確立していない。小児期特有の問題を加味した診療指針が作成されることが望まれる。(著者抄録)

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2021&ichushi_jid=J00643&link_issn=&doc_id=20210427420011&doc_link_id=%2Fag1snrsd%2F2021%2F007405%2F012%2F0553-0558%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fag1snrsd%2F2021%2F007405%2F012%2F0553-0558%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

CAR-T細胞療法は,キメラ抗原受容体(chimeric antigen receptor:CAR)遺伝子により遺伝子改変した自己T細胞を用いるがん免疫療法である.CD19を標的とするCAR-T療法(tisagenlecleucel)は,再発・難治の急性リンパ性白血病において目覚ましい治療成績を示し,2017年8月に米国で,本邦でも2019年5月に保険診療として認可された.本稿では,CARの初期開発から臨床的成功に至るまでの歴史を概観する.さらにCAR-T細胞療法の共刺激シグナルによる治療成績の差異や,CAR-NK細胞療法の開発の歴史についても簡単に触れたい.(著者抄録)

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Family history is one key in diagnosing inborn errors of immunity (IEI); however, disease status is difficult to determine in deceased relatives. X-linked anhidrotic ectodermal dysplasia with immunodeficiency is one of the hyper IgM syndromes that is caused by a hypomorphic variant in the nuclear factor kappa beta essential modulator. We identified a novel IKBKG variant in a 7-month-old boy with pneumococcal rib osteomyelitis and later found that his mother has incontinentia pigmenti. Genetic analysis of preserved umbilical cords revealed the same variant in two of his deceased maternal uncles. Analysis of preserved umbilical cord tissue from deceased relatives can provide important information for diagnosing IEI in their descendants.

DOI： 10.3389/fimmu.2021.786164

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Induction deaths (ID) remain a critical issue in the treatment of pediatric patients with acute lymphoblastic leukemia (ALL). The reported rate of ID in this population is 1% or higher. We speculate that this proportion might be lower in Japan because of mandatory hospitalization during induction therapy to manage complications. We retrospectively analyzed the incidence of ID among children with ALL enrolled in 4 Japanese study groups between 1994 and 2013. Among 5620 children, 41 (0.73%) cases of ID were noted. The median age was 6.5 years; 24 children were female, and 7 had T-cell ALL. Infection was the most common cause of ID (n=22), but the incidence (0.39%) was lower than that reported in western countries. Mortality within 48 hours from the onset of infection was low, comprising 25% of infection-related deaths. The incidence of infections caused by Bacillus species was low. Only 1 patient died because of Aspergillus infection. Fatal infections mostly occurred during the third week of induction therapy. Our findings suggest that close monitoring, stringent infection control, and immediate administration of appropriate antibiotics through hospitalization might be important strategies in reducing the rate of infection-related ID in pediatric patients with ALL.

DOI： 10.1097/MPH.0000000000001926

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Rearrangements of chromosome 8q24/MYC (8q24/MYC-r), resulting from t(8;14)(q24;q32), t(2;8)(p11;q24), or t(8;22)(q24;q11), are mainly associated with Burkitt lymphoma/leukemia (BL) and rarely observed in patients with B-cell precursor acute lymphoblastic leukemia (BCP-ALL). The characteristics of BCP-ALL with 8q24/MYC-r are poorly understood. PROCEDURE: A retrospective nationwide study of data from patients with pediatric BCP-ALL with 8q24/MYC-r in Japan was conducted to clarify the clinical and biological characteristics associated with 8q24/MYC-r BCP-ALL. RESULTS: Ten patients with BCP-ALL with 8q24/MYC-r, including three with double-hit leukemia (DHL) (two with t(8;14)(q24;q32) and t(14;18)(q32;q21) and one with t(8;14) and t(3;22)(q27;q11)), were identified. Patients with BCP-ALL with 8q24/MYC-r had higher median age and uric acid and lactate dehydrogenase levels, than those without 8q24/MYC-r. All patients were initially treated with ALL-type chemotherapy; however, four, including one with DHL, were switched to BL-type chemotherapy, based on cytogenetic findings. One patient relapsed after standard-risk ALL-type chemotherapy, and two patients with DHL did not attain complete remission with chemotherapy; all three died within 11 months. The other seven patients treated with BL-type or high-risk ALL-type chemotherapy are alive without disease. CONCLUSIONS: The clinical and laboratory features of BL with IG-MYC rearrangement, displaying a BCP immunophenotype (Wagener et al. and Herbrueggen et al. termed it as pre-BLL), are similar to those of BCP-ALL with 8q24/MYC-r. Low-risk ALL-type chemotherapy may not be appropriate for them, and further studies are required to establish an adequate therapeutic strategy. Further studies of DHL to identify new treatment strategies are also needed.

DOI： 10.1002/pbc.28341

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記述言語：英語  

DOI： 10.1002/pbc.28052

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Objectives: One of the reasons as to why chimeric antigen receptors (CAR)-T cell therapy for malignancies other than CD19- or BCMA-positive tumors has yet to produce remarkable progress is the paucity of targetable antigens. NKp44 is only expressed by activated natural killer cells and detects a variety of transformed cells, while it reportedly does not react with normal tissues. The aim of this study is to develop CAR-T cell that can target multiple types of tumor cells. Methods: We created a series of novel CAR constructs in first-generation (1G) and second-generation (2G) CAR format with the extracellular immunoglobulin-like domain of NKp44 (NKp44-CAR). Results: Transduction of the best 1G construct into human primary T cells led to specific cytotoxic effects and cytokine secretion upon encountering multiple types of neoplastic cells including AML, T-ALL and childhood solid tumors. Replacement of the extracellular hinge domain of NKp44 with that of CD8α resulted in diminished CAR function. The 1G NKp44-CAR-T cells exhibited significantly better tumor control in long-term co-culture assays compared with activated NK cells, as well as with NK cells transduced with identical NKp44-CAR. T cells transduced with the best 2G-CAR construct with 4-1BB co-stimulatory domain proliferated at significantly higher levels upon single antigen exposure and showed significantly better tumor control compared with the 1G-CAR and 2G-CAR with CD28 co-stimulatory domain. Conclusions: NKp44-based CAR endows T cells with NK cell-like anti-tumor specificity. The CAR gene created in this study will be useful for the development of novel gene-modified T-cell immunotherapy.

DOI： 10.1002/cti2.1147

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記述言語：英語  

Chronic enteroviral meningoencephalitis is a well-known complication in patients with X-linked agammaglobulinemia (XLA). However, progressive neurodegenerative disorders or chronic neuroinflammatory diseases with no causative microorganisms have been recognized as rare central nervous system (CNS) complications in XLA. We herein report a family in which two of three members with XLA had developed progressive meningoencephalitis with an unknown etiology. A 15-month-old male infant presented with left-sided ptosis. Initially, the family denied any family history of inherited diseases, but later disclosed a family history of agammaglobulinemia previously diagnosed in two family members. In the early 1980s, one of the elder brothers of the index patient's mother who had been treated with intramuscular immunoglobulin [or later intravenous immunoglobulin (IVIG)] for agammaglobulinemia deceased at 10 years of age after showing progressive neurological deterioration during the last several years of his life. The index patient was diagnosed with XLA caused by Bruton tyrosine kinase deficiency (654delG; Val219Leufs*9), and chronic meningoencephalitis with an unknown infectious etiology. Magnetic resonance imaging of the brain demonstrated inflammatory changes in the basal ganglia, hypothalamus, midbrain, and pons, with multiple nodular lesions with ring enhancement, which showed impressive amelioration after the initiation of IVIG replacement therapy. Pleocytosis, which was characterized by an increase in CD4-positive and CD8-positive T cells expressing an activation marker and an elevation in inflammatory cytokines in the cerebrospinal fluid, was identified. No microorganism was identified as a cause of CNS complications. He thereafter developed brain infarction at 19 months of age and fatal status epilepticus at 5 years of age, despite regular IVIG with high trough levels and regular intraventricular immunoglobulin administration. The etiology of this rare CNS complication in XLA is currently unknown. Previous studies have suggested a possible association of IVIG, which was clearly denied in our index case because of the demonstration of his neurological disorder at presentation. In the future, extensive and unbiased molecular methods to detect causative microorganisms, as well as to investigate the possible role of autoimmunity are needed to clarify the etiology of CNS complications.

DOI： 10.3389/fped.2020.00579

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

Several novel therapeutics that employ immunological mechanisms have been introduced in recent years for the treatment of hematological malignancies. To date, very few drugs have been introduced for acute lymphoblastic leukemia (ALL). Nonetheless, three novel agents have been approved recently in the US, Europe, Australia, and Japan: blinatumomab, which kills CD19-positive leukemia cells via cytotoxic activity of the patient's autologous T cells; inotuzumab ozogamicin, which delivers the anti-cancer antibiotic calicheamicin via CD22 internalization after antibody binding; and tisagenlecleucel, which uses patient's T cells via anti-CD19 chimeric antigen receptors. Aggressive multi-agent chemotherapy followed by allogeneic hematopoietic cell transplantation has been the only curative strategy for relapsed or refractory ALL. However, treatment strategies for such patients are about to change dramatically. In this article, I review the clinical development of the new therapeutics and discuss their roles in modern therapy for ALL in children and adolescents. An approach for treatment selection has not yet been established. Therefore, it is important to understand the advantages and disadvantages of each treatment for choosing a treatment strategy for each individual.

DOI： 10.11406/rinketsu.61.673

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記述言語：日本語   出版者・発行元：長岡赤十字病院  

症例は生来健康な10歳男児であり、発熱、咳嗽、呼吸困難、胸水貯留からマイコプラズマ肺炎が疑われ前医で入院加療されていたが、胸水貯留と呼吸困難が増悪し当院紹介・入院となった。当院入院後撮影したCT検査で中縦隔下部を中心に両側胸腔と腹腔に及び、胸腹膜播種と肝臓への直接浸潤を伴い下大静脈の狭窄をきたす巨大な腫瘤が指摘された。胸水細胞診からは診断に至らず、精査加療目的に新潟大学医歯学総合病院小児科へ転院となった。その後の開胸生検の結果、横紋筋肉腫と診断された。後方視的に初診時の胸部エックス線所見を確認すると、腫瘤性病変を示唆する所見を確認することができた。あらゆる身体部位に発生しうる横紋筋肉腫は初発症状も多様であり、常に鑑別にあげ早期の発見と介入に務めるべきである。日常診療で行われることの多い胸部単純エックス線写真を適切に評価し、より適切に確定診断を行うための検査を進めていくことが肝要である。(著者抄録)

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Ploidy is a highly significant prognostic factor for pediatric acute lymphoblastic leukemia (ALL). Children with hypodiploid ALL have poor outcomes despite current intensive chemotherapy. Little has been investigated with regard to hypodiploid ALL in Japanese children. METHODS: We retrospectively collected clinical data on hypodiploid ALL cases from the registries of prospective multicenter trials conducted by the four independent clinical study groups in Japan between 1997 and 2012. RESULTS: A total of 117 ALL patients with hypodiploidy were analyzed in this study. There were 101, eight, and eight patients with 45, 44, and fewer than 44 chromosomes, respectively. The 5 year overall survival rates differed significantly: 86.0%, 87.5%, and 62.5% for patients with 45, 44, and fewer than 44 chromosomes, respectively (P = 0.037). Of the eight patients with 44 chromosomes, seven were alive, including five patients who maintained complete remission without undergoing hematopoietic stem cell transplantation (HSCT). Of the eight patients with fewer than 44 chromosomes, six were good responders to prednisolone and none had induction failure, but the relapse rate was high (5/8). No patients had central nervous system relapse. Four patients underwent HSCT after relapse, but only one survived. CONCLUSIONS: Outcomes of Japanese ALL patients with fewer than 44 chromosomes were poor, as previously reported in other countries. Although the sample size was small, patients with 44 chromosomes had better prognoses than those previously reported. Further studies including international collaboration are needed to improve outcomes for pediatric ALL patients with fewer than 44 chromosomes.

DOI： 10.1111/ped.14006

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記述言語：日本語   出版者・発行元：金原出版(株)  

＜文献概要＞CAR-T(カーティー)細胞療法とは,遺伝子改変によりキメラ抗原受容体(chimeric antigen receptor:CAR)を発現させた自己T細胞を用いる免疫細胞療法である.CARは,マウスモノクローナル抗体の高い抗原選択性と結合力を利用した人工受容体であり,HLA拘束性がなく万人に利用できる点が重要な特徴である.2017年8月,CD19を標的とするCAR-T療法が,再発・難治急性リンパ性白血病に対して,米国で保険診療として認可された.CARの機能にかかわる基礎的事項から,臨床的成功に至るまでの歴史的背景,そして今後の方向性について概説した.

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記述言語：英語   出版者・発行元：WILEY  

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記述言語：英語   出版者・発行元：WILEY  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：American Society of Hematology  

X-linked recessive ectodermal dysplasia with immunodeficiency is a rare primary immunodeficiency caused by hypomorphic mutations of the IKBKG gene encoding the nuclear factor κB essential modulator (NEMO) protein. This condition displays enormous allelic, immunological, and clinical heterogeneity, and therapeutic decisions are difficult because NEMO operates in both hematopoietic and nonhematopoietic cells. Hematopoietic stem cell transplantation (HSCT) is potentially life-saving, but the small number of case reports available suggests it has been reserved for only the most severe cases. Here, we report the health status before HSCT, transplantation outcome, and clinical follow-up for a series of 29 patients from unrelated kindreds from 11 countries. Between them, these patients carry 23 different hypomorphic IKBKG mutations. HSCT was performed from HLA-identical related donors (n = 7), HLA-matched unrelated donors (n = 12), HLA-mismatched unrelated donors (n = 8), and HLA-haploidentical related donors (n = 2). Engraftment was documented in 24 patients, and graft-versus-host disease in 13 patients. Up to 7 patients died 0.2 to 12 months after HSCT. The global survival rate after HSCT among NEMO-deficient children was 74% at a median follow-up after HSCT of 57 months (range, 4-108 months). Preexisting mycobacterial infection and colitis were associated with poor HSCT outcome. The underlying mutation does not appear to have any influence, as patients with the same mutation had different outcomes. Transplantation did not appear to cure colitis, possibly as a result of cell-intrinsic disorders of the epithelial barrier. Overall, HSCT can cure most clinical features of patients with a variety of IKBKG mutations.

DOI： 10.1182/blood-2017-03-771600

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER JAPAN KK  

Histiocytic sarcoma, a rare hematopoietic neoplasm with evidence of histiocytic differentiation, is often refractory to conventional chemotherapy and radiotherapy, and its prognosis is generally dismal. The optimal management of this malignancy has not been established. We report a case of 8-year-old girl with histiocytic sarcoma involving the left femur. The tumor rapidly responded to a combination of cladribine and high-dose cytosine arabinoside, an aggressive salvage regimen for refractory Langerhans cell histiocytosis, and became impalpable during the first cycle. The patient has remained in complete remission more than 7 years from diagnosis.

DOI： 10.1007/s12185-017-2202-8

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

ランゲルハンス細胞組織球症(LCH)は、ランゲルハンス細胞様細胞(LCH細胞)のモノクローナルな腫瘍性増殖をきたす疾患である。骨病変の頻度が最も高く、軟部組織単独病変は稀である。今回我々は、胸椎の骨融解病変、胸膜病変、および骨病変に連続しない後頸部の軟部組織病変を伴う多臓器型LCHの5歳女児例を経験した。本例は当初、後頸部皮下膿瘍および化膿性脊椎炎の疑いで抗菌薬が投与され、解熱し病変部位の腫脹が軽減消失したことから、結果的に化学療法の同意が得られず経過観察の方針となった。約9ヵ月間で2回の再燃を観察し、いずれも抗菌薬投与のみで解熱し、局所症状も軽減消失した。最終的に多剤併用化学療法を導入したところ、速やかに寛解が得られ、4年間再発なく経過良好である。本例はLCHでは稀な軟部組織病変を伴うのみならず、抗菌薬による一時的効果が観察された点で、LCHの複雑な病態を考察する上で貴重と思われる。(著者抄録)

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2017&ichushi_jid=J06030&link_issn=&doc_id=20170907100013&doc_link_id=%2Fex9syoga%2F2017%2F005402%2F013%2F0157-0160%26dl%3D0&url=http%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fex9syoga%2F2017%2F005402%2F013%2F0157-0160%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ELSEVIER SCIENCE INC  

Natural killer cells have been identified as a mediator of alloimmune reactions in allogeneic hematopoietic stem cell transplantation (HSCT). Killer immunoglobulin-like receptors (KIRs) are an important determinant of natural killer cell function. The relationship between KIR genotypes/haplotypes and clinical outcomes of allogeneic HSCT is complex and inconsistent among several reports. We assessed the clinical impact of KIR haplotype on T cell-replete allogeneic HSCTs performed in a single Japanese center for hematological malignancies (n = 106). A comparison of 2 groups, donor haplotypes A/A and B/x, revealed no significant differences in overall survival, relapse, and nonrelapse mortality. However, grade III to IV acute graft-versus-host disease (GVHD) occurred significantly more frequently in the KIR haplotype B/x group (A/A versus B/x: 4.9% versus 20.0%; P=.02). This was even more evident when HLA mismatch was present. The highest incidences of grade II to IV and grade III to IV acute GVHD were observed in patients who received allografts from HLAmismatched donors with KIR haplotype B/x. These data highlight the importance of KIR genotyping in donor matching, especially when HLA mismatch allogeneic grafting is planned. (C) 2017 American Society for Blood and Marrow Transplantation.

DOI： 10.1016/j.bbmt.2016.12.638

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記述言語：日本語   出版者・発行元：(公社)日本医師会  

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記述言語：日本語   出版者・発行元：新潟医学会  

キメラ抗原受容体(chimeric antigen receptor:CAR)は単鎖抗体を抗原結合部位とする人工受容体である。ヒトT細胞に遺伝子導入することにより、がん細胞への抗原選択的な障害活性を獲得させる。筆者らは、現在米国を中心に臨床開発が進んでいるCD19-CAR遺伝子を開発した。現在、NK細胞をエフェクターとする戦略や、固形がんに対する新規CAR開発に取り組んでいる。(著者抄録)

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2017&ichushi_jid=J00990&link_issn=&doc_id=20170524020002&doc_link_id=%2Fdg3nigta%2F2017%2F013101%2F002%2F0007-0010%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdg3nigta%2F2017%2F013101%2F002%2F0007-0010%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：英語   掲載種別：研究論文（学術雑誌）  

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記述言語：英語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER JAPAN KK  

Mature B-cell acute lymphoblastic leukemia (B-ALL) is typically associated with French-American-British (FAB)-L3 morphology and MYC gene rearrangement. However, rare cases of mature B-ALL with non-L3 morphology and MLL-AF9 fusion have been reported, and such cases are characterized by a rapid and aggressive clinical course. We here report three such cases of pediatric mature B-ALL in female patients respectively aged 15 months, 4 years, and 4 months. Bone marrow smears at diagnosis showed FAB-L1 morphology in all patients. Immunophenotypically, they were positive for cluster of differentiation (CD) 10, CD19, CD20 (or CD22), Human Leukocyte Antigen-DR, and surface immunoglobulin.. No evidence of MYC rearrangement was detected in any of the cases by fluorescent in situ hybridization (FISH) analysis. However, MLL rearrangement was detected by FISH, and MLL-AF9 fusion was confirmed by reverse transcriptase-polymerase chain reaction. All patients achieved complete remission after conventional chemotherapy and subsequently underwent hematopoietic stem cell transplantation as high-risk ALL; patient 3 for infantile ALL with MLL rearrangement and the others for ALL with MLL rearrangement and hyperleukocytosis (white blood cell count at diagnosis >50 x 10(9)/L). At the latest follow-up for each case (12-98 months post-transplantation), complete remission was maintained. Moreover, we discuss the clinical, genetic, and immunophenotypic features of this rare disease.

DOI： 10.1007/s12185-016-1971-9

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：NATURE PUBLISHING GROUP  

Recent studies revealed that a substantial proportion of patients with high-risk B-cell precursor acute lymphoblastic leukemia (BCP-ALL) harbor fusions involving tyrosine kinase and cytokine receptors, such as ABL1, PDGFRB, JAK2 and CRLF2, which are targeted by tyrosine kinase inhibitors (TKIs). In the present study, transcriptome analysis or multiplex reverse transcriptase-PCR analysis of 373 BCP-ALL patients without recurrent genetic abnormalities identified 29 patients with kinase fusions. Clinically, male predominance (male/female: 22/7), older age at onset (mean age at onset: 8.8 years) and a high white blood cell count at diagnosis (mean: 94 200/mu l) reflected the predominance of National Cancer Institute high-risk (NCI-HR) patients (NCI-standard risk/HR: 8/21). Genetic analysis identified three patients with ABL1 rearrangements, eight with PDGFRB rearrangements, two with JAK2 rearrangements, three with IgH-EPOR and one with NCOR1-LYN. Of the 14 patients with CRLF2 rearrangements, two harbored IgH-EPOR and PDGFRB rearrangements. IKZF1 deletion was present in 16 of the 22 patients. The 5-year event-free and overall survival rates were 48.6 +/- 9.7% and 73.5 +/- 8.6%, respectively. The outcome was not satisfactory without sophisticated minimal residual disease-based stratification. Furthermore, the efficacy of TKIs combined with conventional chemotherapy without allogeneic hematopoietic stem cell transplantation in this cohort should be determined.

DOI： 10.1038/bcj.2016.28

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：BIOMED CENTRAL LTD  

Background: Juvenile myelomonocytic leukemia (JMML) is a fatal, myelodysplastic/myeloproliferative neoplasm of early childhood. Patients with JMML have mutually exclusive genetic abnormalities in granulocyte-macrophage colony-stimulating factor (GM-CSF) receptor (GMR, CD116) signaling pathway. Allogeneic hematopoietic stem cell transplantation is currently the only curative treatment option for JMML; however, disease recurrence is a major cause of treatment failure. We investigated adoptive immunotherapy using GMR-targeted chimeric antigen receptor (CAR) for JMML.
Methods: We constructed a novel CAR capable of binding to GMR via its ligand, GM-CSF, and generated piggyBac transposon-based GMR CAR-modified T cells from three healthy donors and two patients with JMML. We further evaluated the anti-proliferative potential of GMR CAR T cells on leukemic CD34(+) cells from six patients with JMML (two NRAS mutations, three PTPN11 mutations, and one monosomy 7), and normal CD34(+) cells.
Results: GMR CAR T cells from healthy donors suppressed the cytokine-dependent growth of MO7e cells, but not the growth of K562 and Daudi cells. Co-culture of healthy GMR CAR T cells with CD34(+) cells of five patients with JMML at effector to target ratios of 1: 1 and 1: 4 for 2 days significantly decreased total colony growth, regardless of genetic abnormality. Furthermore, GMR CAR T cells from a non-transplanted patient and a transplanted patient inhibited the proliferation of respective JMML CD34(+) cells at onset to a degree comparable to healthy GMR CAR T cells. Seven-day co-culture of GMR CAR T cells resulted in a marked suppression of JMML CD34(+) cell proliferation, particularly CD34(+)CD38(-) cell proliferation stimulated with stem cell factor and thrombopoietin on AGM-S3 cells. Meanwhile, GMR CAR T cells exerted no effects on normal CD34(+) cell colony growth.
Conclusions: Ligand-based GMR CAR T cells may have anti-proliferative effects on stem and progenitor cells in JMML.

DOI： 10.1186/s13045-016-0256-3

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記述言語：英語   出版者・発行元：AMER SOC HEMATOLOGY  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPANDIDOS PUBL LTD  

The present study reports the first case of malignant transformation to osteosarcoma arising from osteochondroma following childhood total-body irradiation (TBI). The association between TBI and later development of osteochondroma is well-known; however, malignant degeneration arising from radiation-induced osteochondroma is rare. The current study describes the case of a 17-year-old boy with osteosarcoma arising from osteochondroma of the left distal humerus, which developed following TBI. TBI was administered as part of a conditioning regimen received prior to autologous peripheral hematopoietic stem cell transplantation (HSCT) at the age of 6 years, following an initial diagnosis of neuroblastoma at the age of 5 years. The patient subsequently underwent preoperative chemotherapy followed by wide local excision and reconstruction with an extracorporeally irradiated autograft. Postoperative chemotherapy was administered, and the patient demonstrated no clinical or radiographic evidence of recurrence after 40 months of follow-up. To the best of our knowledge, this is only the second reported case of malignant degeneration of osteochondroma following childhood TBI, and the first reported case of transformation to osteosarcoma. The current case highlights the importance of close observation for secondary malignancies in this patient population.

DOI： 10.3892/ol.2015.3257

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

悪性脳腫瘍に対する全脳脊髄照射(CSI)では頭蓋骨及び全脊椎の骨髄組織が障害されるため、CSI後の強力な化学療法では骨髄抑制が著しく遷延し、結果的に治療全体の強度が低下することが大きな問題である。そこで我々は、CSI及び強力な化学療法を要する悪性脳腫瘍に対しG-CSF単独の末梢血幹細胞(PBSC)採取を試み、化学療法に自家末梢血幹細胞移植(aPBSCT)を併用することとした。早期にCSIを開始するため、術後2週以内の採取完了を目指した。初発未治療例6例では中央値12.6×10^6/kg(3.9-21.6×10^6/kg)のCD34陽性細胞採取が可能だったが、うち2例では複数回のaPBSCTに十分な細胞数に達しなかった。再発例の2例では少なくとも1回の移植に必要な量は採取できた。これら4例ではaPBSCT併用化学療法1コース終了後に再度PBSC採取を行い十分量が確保できた(13.7-42.1×10^6/kg)。術後2週以内のPBSC採取では白血球数が増加しやすい傾向にあったが、重篤な有害事象はみられなかった。全例で予定した複数回aPBSCTを実施でき、骨髄抑制の遷延をきたすことなく規定の間隔で化学療法を完遂できた。術後2週以内のG-CSF単独PBSC採取は安全に実施できたものの少数例の経験であり、術後早期にCSIを要する悪性脳腫瘍の集学的治療における有効性に関しては更なる検討を要する。(著者抄録)

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：American Society of Hematology  

Natural killer (NK) cell survival and, hence, cytotoxicity requires cytokine support. We determined whether expression of interleukin-15 (IL-15) in a nonsecretory, membranebound form could sustain NK cell growth. We linked the human IL15 gene to that encoding CD8α transmembrane domain (mbIL15). After retroviral transduction, human NK cells expressed mbIL15 on the cell surface
IL-15 secretion was negligible. Survival of mbIL15-NK cells without interleukin-2 (IL-2) after 7-day culturewas vastly superior to that of mock-transduced NK cells (P &lt
.001, n = 15) and of NK cells expressing nonmembrane-bound IL-15 (P = .025, n = 9)
viable mbIL15-NK cells were detectable for up to 2 months. In immunodeficient mice, mbIL15-NK cells expanded without IL-2 and were detectable in all tissues examined (except brain) in much higher numbers than mock-transduced NK cells (P &lt
.001).Expansion further increasedwith IL-2. The primary mechanism of mbIL15 stimulation was autocrine
it activated IL-15 signaling and antiapoptotic signaling. NK cells expressing mbIL15 had higher cytotoxicity against leukemia, lymphoma, and solid tumor cells in vitro and against leukemia and sarcoma cells in xenograft models. Thus, mbIL15 confers independent growth to NK cells and enhances their antitumor capacity. Infusion of mbIL15-NK cells would allow NK cell therapy without the potential adverse effects of cytokine administration. © 2014 by The American Society of Hematology.

DOI： 10.1182/blood-2014-02-556837

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：AMER SOC HEMATOLOGY  

Natural killer (NK) cell survival and, hence, cytotoxicity requires cytokine support. We determined whether expression of interleukin-15 (IL-15) in a nonsecretory, membrane-bound form could sustain NK cell growth. We linked the human IL15 gene to that encoding CD8 alpha transmembrane domain (mbIL15). After retroviral transduction, human NK cells expressed mbIL15 on the cell surface; IL-15 secretion was negligible. Survival of mbIL15-NK cells without interleukin-2 (IL-2) after 7-day culture was vastly superior to that of mock-transduced NK cells (P < .001, n = 15) and of NK cells expressing nonmembrane-bound IL-15 (P = .025, n=9); viable mbIL 15-NK cells were detectable for up to 2 months. In immunodeficient mice, mbIL15-NK cells expanded without IL-2 and were detectable in all tissues examined (except brain) in much higher numbers than mock-transduced NK cells (P < .001). Expansion further increased with IL-2. The primary mechanism of mbIL15 stimulation was autocrine; it activated IL-15 signaling and antiapoptotic signaling. NK cells expressing mbIL15 had higher cytotoxicity against leukemia, lymphoma, and solid tumor cells in vitro and against leukemia and sarcoma cells in xenograft models. Thus, mbIL15 confers independent growth to NK cells and enhances their antitumor capacity. Infusion of mbIL15-NK cells would allow NK cell therapy without the potential adverse effects of cytokine administration.

DOI： 10.1182/blood-2014-02-556837

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：AMER ASSOC CANCER RESEARCH  

To expand applications for T-cell-based immunotherapy in cancer, we designed a receptor that binds the Fc portion of human immunoglobulins and delivers activation signals. The construct included the high-affinity CD16 (FCGR3A) V158 variant, CD8 alpha hinge, and transmembrane domains, along with signaling domains from CD3 zeta and 4-1BB (TNFRSF9), forming a chimeric receptor termed CD16V-BB-zeta. After retrovirus-mediated expression in human T cells, CD16V-BB-zeta bound humanized antibodies with higher affinity than a control receptor containing the more common F158 variant. Engagement of CD16V-BB-zeta provoked T-cell activation, exocytosis of lytic granules, and sustained proliferation, with a mean cell recovery after4-week coculture with Daudi lymphoma cells and rituximab of nearly 70-fold relative to input cells. In contrast, unbound antibody alone produced no effect. CD16V-BB-zeta T cells specifically killed lymphoma cells and primary chronic lymphocytic leukemia cells in combination with rituximab at a low effector target ratio, even when assayed on mesenchymal cells. Trastuzumab triggered CD16V-BB-zeta-mediated killing of HER2 (ERBB2)(+) breast and gastric cancer cells; similar results were obtained with an anti-GD2 antibody in neuroblastoma and osteosarcoma cells. Furthermore, coadministration of CD16V-BB-zeta T cells with immunotherapeutic antibodies exerted considerable antitumor activity in vivo. Signaling mediated by 4-1BB-CD3 zeta induced higher T-cell activation, proliferation, and cytotoxicity than CD3 zeta or Fc epsilon RI gamma, and the receptor was expressed effectively after mRNA electroporation without viral vectors, facilitating clinical translation. Our results offer preclinical proof of concept for CD16V-BB-zeta as a universal, next-generation chimeric receptor with the potential to augment the efficacy of antibody therapies for cancer. (C)2013 AACR.

DOI： 10.1158/0008-5472.CAN-13-1365

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：Lippincott Williams and Wilkins  

In addition to T-cell receptor signals, T lymphocytes require costimulatory signals for robust activation. Among these, those mediated by 4-1BB (CD137, TNFRSF9) are critical for tumor immunity. 4-1BB is expressed in T-cell receptor-activated lymphocytes as well as natural killer cells and other hematopoietic and nonhematopoietic cells. 4-1BB ligation induces a signaling cascade that results in cytokine production, expression of antiapoptotic molecules, and enhanced immune responses. In line with the described function of 4-1BB, its addition to CD3ζ chimeric antigen receptors (CARs) increases their capacity to provoke T-cell expansion and antitumor activity. The results of preclinical studies with 4-1BB CARs have been corroborated by encouraging results from clinical trials. Advantages and disadvantages of 4-1BB CARs versus CARs bearing other costimulatory components remain to be fully elucidated. In this review, we discuss the properties of 4-1BB, the design of 4-1BB CARs, and the function of T lymphocytes and natural killer cells expressing them. Copyright © 2014 Lippincott Williams &amp
Wilkins.

DOI： 10.1097/PPO.0000000000000028

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1038/leu.2013.82

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Secondary rhabdomyosarcoma (RMS) after treatment of osteosarcoma (OS) is rare. Reported here is the case of a metachronous RMS in the nasal cavity, developing 12 years after successful treatment of non-metastatic OS. The patient was diagnosed as having OS of the femur at 2 years of age. Chemotherapy for OS included doxorubicin (cumulative dose, 488 mg/m2). No radiotherapy was given. There was no family history suggestive of cancer predisposition syndrome. At 14 years of age, alveolar RMS was diagnosed on histopathology. PAX3-FKHR fusion transcripts were detected on reverse transcription-polymerase chain reaction. Germline TP53 mutation was not seen on standard DNA sequencing. The occurrence of secondary sarcomas, in the Children's Cancer Survivor study conducted in North America, has been associated with high cumulative doses of anthracyclines, which may also have played a role in the development of RMS in the present case. In the future, novel molecular technologies might uncover genetic cancer predisposition in patients with metachronous cancers. © 2013 The Authors.

DOI： 10.1111/ped.12070

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY-BLACKWELL  

Secondary rhabdomyosarcoma (RMS) after treatment of osteosarcoma (OS) is rare. Reported here is the case of a metachronous RMS in the nasal cavity, developing 12 years after successful treatment of non-metastatic OS. The patient was diagnosed as having OS of the femur at 2 years of age. Chemotherapy for OS included doxorubicin (cumulative dose, 488 mg/m(2)). No radiotherapy was given. There was no family history suggestive of cancer predisposition syndrome. At 14 years of age, alveolar RMS was diagnosed on histopathology. PAX3-FKHR fusion transcripts were detected on reverse transcription-polymerase chain reaction. Germline TP53 mutation was not seen on standard DNA sequencing. The occurrence of secondary sarcomas, in the Children's Cancer Survivor study conducted in North America, has been associated with high cumulative doses of anthracyclines, which may also have played a role in the development of RMS in the present case. In the future, novel molecular technologies might uncover genetic cancer predisposition in patients with metachronous cancers.

DOI： 10.1111/ped.12070

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記述言語：日本語   出版者・発行元：(株)東京医学社  

＜Key Points＞(1)MRDは他の予後因子(年齢・白血球数、染色体・遺伝子異常治療など)と独立した強力な予後因子である。(2)治療プロトコールに依存した因子であることに注意が必要。(3)治療強化のみならず治療軽減にも有用と考えられる。(4)ALL治療の理解には発展の歴史を知ることが非常に有用である。文献6の一読を勧める。(著者抄録)

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

難治性白血病・小児がんには新たな治療が必要である。その一端を担う新規戦略として、NK細胞が注目されている。本稿では、NK細胞のアロ反応性の制御、造血幹細胞移植におけるNK細胞の役割、近年欧米で実施されている同種NK細胞輸注療法について紹介し、このアプローチの実現に重要と思われるNK細胞の体外増幅法・遺伝子改変法の開発について、抗CD19キメラ型受容体(anti-CD19-BB-ζ)の開発も含めて解説する。(著者抄録)

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：AMER SOC HEMATOLOGY  

Somatic mosaicism has been described in several primary immunodeficiency diseases and causes modified phenotypes in affected patients. X-linked anhidrotic ectodermal dysplasia with immunodeficiency (XL-EDA-ID) is caused by hypomorphic mutations in the NF-kappa B essential modulator (NEMO) gene and manifests clinically in various ways. We have previously reported a case of XL-EDA-ID with somatic mosaicism caused by a duplication mutation of the NEMO gene, but the frequency of somatic mosaicism of NEMO and its clinical impact on XL-EDA-ID is not fully understood. In this study, somatic mosaicism of NEMO was evaluated in XL-EDA-ID patients in Japan. Cells expressing wild-type NEMO, most of which were derived from the T-cell lineage, were detected in 9 of 10 XL-EDA-ID patients. These data indicate that the frequency of somatic mosaicism of NEMO is high in XL-ED-ID patients and that the presence of somatic mosaicism of NEMO could have an impact on the diagnosis and treatment of XL-ED-ID patients. (Blood. 2012;119(23):5458-5466)

DOI： 10.1182/blood-2011-05-354167

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

先天性溶血性貧血の異常部位には赤血球膜、赤血球酵素、ヘモグロビンが知られている。遺伝性球状赤血球症(HS)は赤血球膜の異常に起因し、日本で最も高頻度の先天性溶血性貧血である。溶血性貧血を呈し、HSに矛盾しない背景を持つ場合では、他の稀な溶血性貧血が見逃される可能性も考えられる。Hb Koeln症はヘモグロビン異常症の一型で、稀な疾患である。今回、我々は複数の脾臓摘出者を認め、HSと考えられていた12歳男児のHb Koeln症を経験した。平均赤血球ヘモグロビン濃度(MCHC)は28.7%と低値、平時のSpO2は90%程と低値(高濃度酸素投与で92%)、HbA1cは2.0%と異常低値であった。これらの一般検査所見がHSとは矛盾したため、ヘモグロビン異常症を疑い精査を進めた。先天性溶血性貧血のような稀な疾患でも、日常的な検査の吟味が診断に重要と考えられた。(著者抄録)

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY PERIODICALS, INC  

DOI： 10.1002/pbc.23228

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記述言語：英語  

BACKGROUND: Nuclear factor-κB essential modulator (NEMO) deficiency is a developmental and immunological disorder. The genetic and phenotypic correlation has been described. METHODS: We report a unique clinical presentation and the identification of a novel missense mutation in the NEMO gene in a 3-year-old boy with bacillus Calmette-Guerin (BCG) infection. RESULTS: The patient presented with fever, cervical lymphadenopathy, and abnormal anti-Epstein-Barr virus (EBV) antibody titers, suggestive of EBV-related diseases including chronic active EBV infection, X-linked lymphoproliferative syndrome, or nasopharyngeal carcinoma. Although the biopsy specimen from a nasopharyngeal lesion was initially diagnosed as squamous cell carcinoma, this was changed to disseminated BCG infection involving the nasopharynx, multiple systemic lymph nodes, and brain. A novel mutation (designated D311E) in the NEMO gene, located in the NEMO ubiquitin-binding (NUB) domain, was identified as the underlying cause of the immunodeficiency. Impaired immune responses which are characteristic of patients with NEMO deficiency were demonstrated. The patient underwent successful unrelated bone marrow transplantation at 4.9 years of age. CONCLUSION: This study suggests the importance of the NUB domain in host defense against mycobacteria. The unique presenting features in our patient indicate that a hypomorphic NEMO mutation can be associated with atypical pathological findings of the epithelial tissues in patients with BCG infection.

DOI： 10.1007/s10875-011-9568-9

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記述言語：日本語   出版者・発行元：日本小児血液学会  

3歳男児。B前駆細胞型小児急性リンパ性白血病(ALL)と診断し、超高危険群として寛解導入療法を開始した。治療開始42週目に2回目の再寛解導入療法に続く強化療法を開始した。一時解熱したが再度発熱し、発熱から10日後に全身強直性痙攣が出現した。頭部CTで右頭頂葉に輪状造影を伴う病変を認め、脳膿瘍が疑われた。接合菌症の可能性を考慮してliposomal amphotericin B(L-AMB)を開始したところ翌日には解熱し、発症2週後のDiffusion MRIでは右頭頂葉に膿瘍腔を示唆する高信号域を認めていたが、発症2ヵ月後には消失していた。しかし、CRPは0.3〜1.0mg/dl程度で著減していたため、L-AMB継続下にmercaptopurine(6MP)、methotrexate(MTX)内服による維持療法を開始した。発症4ヵ月後もDiffusion MRIの所見は変化を認めなかったが、血清クレアチニンは0.2mg/dlから0.5mg/dlへ上昇した。そのためL-AMBを5mg/kg/dayの隔日投与へ、さらに2週間後から週2回投与へ減量したが、CRPは増加傾向とはならなかった。Diffusion MRI所見の増悪もなく、週2回投与を2週間継続後にL-AMBを中止した。その後CRPは陰性化し、投与終了後約1年、脳膿瘍の再発は認めず、維持療法継続中である。

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2011&ichushi_jid=J02258&link_issn=&doc_id=20110511060006&doc_link_id=%2Fex9syoke%2F2011%2F002502%2F006%2F0097-0101%26dl%3D0&url=http%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fex9syoke%2F2011%2F002502%2F006%2F0097-0101%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：英語   掲載種別：研究論文（学術雑誌）  

We describe a 14-year-old boy who exhibited left palatine tonsillar enlargement after 6 cycles of aggressive chemotherapy for diffuse large B-cell lymphoma of the right palatine tonsil. The cervical computed tomography scan at 4 months after completion of chemotherapy revealed enlargement of the left palatine tonsil in addition to the thymus without any clinical symptoms. The18F-fluorodeoxyglucose positron emission tomography indicated focal areas of strong18F-fluorodeoxyglucose uptake in the left palatine tonsil. Histologic examination confirmed tonsillar hyperplasia with no evidence of recurrence. Reactive tonsillar hyperplasia after chemotherapy is rarely reported. Copyright © 2011 by Lippincott Williams &amp
Wilkins.

DOI： 10.1097/MPH.0b013e3181f69205

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

We describe a 14-year-old boy who exhibited left palatine tonsillar enlargement after 6 cycles of aggressive chemotherapy for diffuse large B-cell lymphoma of the right palatine tonsil. The cervical computed tomography scan at 4 months after completion of chemotherapy revealed enlargement of the left palatine tonsil in addition to the thymus without any clinical symptoms. The F-18-fluorodeoxyglucose positron emission tomography indicated focal areas of strong F-18-fluorodeoxyglucose uptake in the left palatine tonsil. Histologic examination confirmed tonsillar hyperplasia with no evidence of recurrence. Reactive tonsillar hyperplasia after chemotherapy is rarely reported.

DOI： 10.1097/MPH.0b013e3181f69205

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY-BLACKWELL  

DOI： 10.1111/j.1442-200X.2010.03306.x

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY-BLACKWELL  

P>Using artificial receptors, it is possible to redirect the specificity of immune cells to tumour-associated antigens, which is expected to provide a useful strategy for cancer immunotherapy. Given that B-cell non-Hodgkin lymphoma (B-NHL) cells invariably express CD19 and CD38, these antigens may be suitable molecular candidates for such immunotherapy. We transduced human peripheral T cells or a T-cell line with either anti-CD19-chimeric receptor (CAR) or anti-CD38-CAR, which contained an anti-CD19 or anti-CD38 antibody-derived single-chain variable domain respectively. Retroviral transduction led to anti-CD19-CAR or anti-CD38-CAR expression in T cells with high efficiency (> 60%). The T cell line, Hut78, when transduced with anti-CD19-CAR or anti-CD38-CAR, exerted strong cytotoxicity against the B-NHL cell lines, HT and RL, and lymphoma cells isolated from patients. Interestingly, use of both CARs had an additive cytotoxic effect on HT cells in vitro. In conjunction with rituximab, human peripheral T cells expressing either anti-CD19-CAR or anti-CD38-CAR enhanced cytotoxicity against HT-luciferase cells in xenografted mice. Moreover, the synergistic tumour-suppressing activity was persistent in vivo for over 2 months. These results provide a powerful rationale for clinical testing of the combination of rituximab with autologous T cells carrying either CAR on aggressive or relapsed B-NHLs.

DOI： 10.1111/j.1365-2141.2010.08297.x

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

NK細胞の基本的事項について解説したうえで、NK細胞を利用したがん治療の可能性とその障壁に関する最近の知見を以下の項目に従って概説した。1)十分量のNK細胞の調整(NK細胞の体外増幅)。2)Killer Immunoglobulin-like receptor(KIR)リガンド不一致にかかわらずNK細胞に抵抗する腫瘍細胞。3)KIRリガンド不一致が得られないレシピエント。4)体外増幅NK細胞の問題点(輸注後のNK細胞のアポトーシス)。5)小児固形腫瘍に対するNK細胞療法。

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2010&ichushi_jid=J00638&link_issn=&doc_id=20100701070005&doc_link_id=%2Fex9syoni%2F2010%2F004702%2F007%2F0268-0274%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fex9syoni%2F2010%2F004702%2F007%2F0268-0274%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：W B SAUNDERS CO-ELSEVIER INC  

A major complication of retained testes is an occurrence of malignancy later in life. We, herein, report the case of a 2-year-old boy who presented with a huge yolk sac tumor with retroperitoneal lymph nodes metastasis that originated in a left intra-abdominal undescended testis. Computed tomography and magnetic resonance imaging showed a huge round tumor connecting to the left retroperitoneal lymph nodes with metastasis extending from the left pelvic region to the left renal hilum. The serum a-fetoprotein level was 36,528 ng/mL. The right abdominal tumor appeared to be a giant testis that had strangulated at the neck of the cord. The tumor had ruptured at the side of the left pelvic lymph node metastasis, and a yolk sac tumor was diagnosed from a histologic analysis of the resected specimens. Postoperative PEB chemotherapy was effective, and a complete surgical resection of the tumor was performed 3 months after the initial laparotomy. The pathologic findings showed fibrous tissue without any tumor cells. The patient has been doing well for 18 months after the radical operation. This case might be a coincidental association of a yolk sac tumor occurring in an undescended testis, which thus caused a delay in making an accurate diagnosis. (C) 2009 Elsevier Inc. All rights reserved.

DOI： 10.1016/j.jpedsurg.2009.08.024

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

We present a patient with Bernard-Soulier syndrome harboring a novel mutation. Flow cytometric analysis showed that the glycoprotein (GP) Ib/IX complex was absent from the platelet surface. By immunoblotting, GPIbalpha, GPIbbeta and GPIX were not detected in the platelet lysates. Glycocalicin, the soluble GPIbalpha fragment, was also absent in the plasma. Genetic analysis revealed a novel homozygous 8-base pair deletion in the GPIbalpha gene, 1136_1143delTTCACATG, which was predicted to cause a frame shift and the addition of 13 altered amino acids followed by premature termination. No mutation was found in the coding sequence of the GPIbbeta or GPIX genes. We demonstrated that the novel deletion mutation resulted in complete defectiveness of the GPIbalpha protein and null expression of the entire GPIb/IX complex, and was responsible for the Bernard-Soulier syndrome phenotype in this patient. Although the presence of a truncated GPIbalpha protein has been often documented, complete absence of the protein has been scarcely reported in Bernard-Soulier syndrome patients with a GPIbalpha mutation causing a premature stop codon. An underlying molecular mechanism to explain how the synth

DOI： 10.1097/MBC.0b013e32832b27fa

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

T-cell-mediated immunotherapy with a chimeric antigen receptor (CAR) is expected to become a powerful treatment for cancer. CD38, highly expressed in B-cell non-Hodgkin lymphoma (B-NHL) cells, is in attractive target in immunotherapy for B-NHL. We retrovirally transduced a T-cell line, Hut78, expressing little CD38, with an anti-CD38-CAR. Hut78 cells with the anti-CD38-CAR were cocultured with B-NHL cell lines hearing CD38 and also B-NHL cells from patients. Four days later most of the lymphoma cells were killed (the level of cytotoxicity was > 95%). By contrast, there was undetectable cytotoxicity against CD38-negative cell lines. Then, we introduced the anti-CD38-CAR into human peripheral T cells. However, the recovery of viable cells was very low, presumably because of all autolytic reaction caused by the association of the anti-CD38-CAR with CD38 on the cell surface. The addition of an anti-CD38 antibody increased the yield of viable transduced T cell probably by blocking the autolytic reaction. We cocultured human peripheral T cells hearing anti-CD38-CAR with B-NHL cells. The median specific cytotoxicity was greater than 90%. These cells were injected 4 times into NOD/SCID mice, which were inoculated with B-NHL cells luciferase. Luciferase activity was not detectable even 30 days after the inoculation in 5 of 6 mice injected. By contrast, it increased in all of the mice injected with the mock vector-transduced T cell. In conclusion, T cell with the anti-CD38-CAR showed powerful cytotoxicity against B-NHL cells in vitro and in vivo. These findings may provide all important Clue for improving the methodology of T-cell-mediated immunotherapy.

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

DOI： 10.1097/MBC.0b013e32832b27fa

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記述言語：日本語   出版者・発行元：(一社)日本内分泌学会  

4歳男児。患者は生後4ヵ月時にSotos症候群と診断され、今回、吸気性喘鳴を主訴に著者らの小児科へ精査入院となった。血液検査では甲状腺機能亢進が認められたが、甲状腺自己抗体はすべて陰性であった。一方、頸部超音波検査では甲状腺峡部尾側に内部不均一な低エコー腫瘤がみられ、頸部造影CTでは腫瘍による気管の圧排が確認された。また、123I甲状腺シンチグラムでは同部位にRIの集積が認められるも、遺伝子解析で変異は検出されなかった。以上より、本症例はSotos症候群に合併した機能性甲状腺結節と診断され、チアマゾール内服にて甲状腺機能を正常範囲に調整した後、腫瘍摘出術が施行された。その結果、術後6ヵ月の123I甲状腺シンチグラムでは正常甲状腺組織へのRIの取り込みがみられ、術前に認められた多動や注意力障害は甲状腺機能の正常化とともに改善した。

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY-BLACKWELL PUBLISHING, INC  

The replicative potential of human CD56(+) CD3(-) natural killer (NK) cells is unknown. We found that by exposing NK cells to the leukaemic cell line K562 genetically modified to express 4-1BB ligand and interleukin 15 (K562-mb15-41BBL), they expanded for up to 30 population doublings, achieving numbers that ranged from 1.6 x 10(5) to 1.2 x 10(11)% (median, 5.9 x 10(6)%; n = 7) of those originally seeded. However, NK cells eventually became unresponsive to stimulation and died. Their demise could be suppressed by enforcing the expression of the human telomerase reverse transcriptase gene (TERT). TERT-overexpressing NK cells continued to proliferate in response to K562-mb15-41BBL stimulation for more than 1 year of culture, while maintaining a normal karyotype and genotype. Long-lived NK cells had high cytotoxicity against myeloid and T-lineage leukaemic cells. They remained susceptible to genetic manipulation, becoming highly cytotoxic to B-lineage leukaemic cells after expression of anti-CD19 signaling receptors. Thus, human NK cells have a replicative potential similar to that of T lymphocytes and their lifespan can be significantly prolonged by an increase in TERT activity. We suggest that the methods described here should have many applications in studies of NK cell biology and NK cell-based therapies.

DOI： 10.1111/j.1365-2141.2009.07667.x

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

The replicative potential of human CD56(+) CD3(-) natural killer (NK) cells is unknown. We found that by exposing NK cells to the leukaemic cell line K562 genetically modified to express 4-1BB ligand and interleukin 15 (K562-mb15-41BBL), they expanded for up to 30 population doublings, achieving numbers that ranged from 1.6 x 10(5) to 1.2 x 10(11)% (median, 5.9 x 10(6)%; n = 7) of those originally seeded. However, NK cells eventually became unresponsive to stimulation and died. Their demise could be suppressed by enforcing the expression of the human telomerase reverse transcriptase gene (TERT). TERT-overexpressing NK cells continued to proliferate in response to K562-mb15-41BBL stimulation for more than 1 year of culture, while maintaining a normal karyotype and genotype. Long-lived NK cells had high cytotoxicity against myeloid and T-lineage leukaemic cells. They remained susceptible to genetic manipulation, becoming highly cytotoxic to B-lineage leukaemic cells after expression of anti-CD19 signaling receptors. Thus, human NK cells have a replicative potential similar to that of T lymphocytes and their lifespan can be significantly prolonged by an increase in TERT activity.

DOI： 10.1111/j.1365-2141.2009.07667.x

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Infusions of natural killer (NK) cells are an emerging tool for cancer immunotherapy. The development of clinically applicable methods to produce large numbers of fully functional NK cells is a critical step to maximize the potential of this approach. We determined the capacity of the leukemia cell line K562 modified to express a membrane-bound form of interleukin (IL)-15 and 41BB ligand (K562-mb15-41BBL) to generate human NK cells with enhanced cytotoxicity. Seven-day coculture with irradiated K562-mb15-41BBL induced a median 21.6-fold expansion of CD56(+)CD3(-) NK cells from peripheral blood (range, 5.1- to 86.6-fold; n = 50), which was considerably superior to that produced by stimulation with IL-2, IL-12, IL-15, and/or IL-21 and caused no proliferation of CD3(+) lymphocytes. Similar expansions could also be obtained from the peripheral blood of patients with acute leukemia undergoing therapy (n = 11). Comparisons of the gene expression profiles of the expanded NK cells and their unstimulated or IL-2-stimulated counterparts showed marked differences. The expanded NK cells were significantly more potent than unstimulated or IL-2-stimulated NK cells against acute myeloid leukem

DOI： 10.1158/0008-5472.CAN-08-3712

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：AMER ASSOC CANCER RESEARCH  

Infusions of natural killer (NK) cells are an emerging tool for cancer immunotherapy. The development of clinically applicable methods to produce large numbers of fully functional NK cells is a critical step to maximize the potential of this approach. We determined the capacity of the leukemia cc line K562 modified to express a membrane-bound form of interleukin (IL)-15 and 41BB ligand (K562-mb15-41BBL) to generate human NK cells with enhanced cytotoxicity. Seven-day coculture with irradiated K562-mb15-41BBL induced a median 21.6-fold expansion of CD56(+)CD3(-) NK cells from peripheral blood (range, 5.1- to 86.6-fold; n = 50), which was considerably superior to that produced by stimulation wit IL-2, IL-12, IL-15, and/or IL-21 and caused no proliferation of CD3(+) lymphocytes. Similar expansions could also be obtained from the peripheral blood of patients with acute leukemia undergoing therapy (n = 11). Comparisons of the gene expression profiles of the expanded NK cells and their unstimulated or IL-2-stimulated counterparts showed marked differences. The expanded NK cells were significantly more potent than unstimulated or IL-2-stimulated NK cells against acute myeloid leukemia cells in vitro. They could be detected for >1 month when injected into immunodeficient mice and could eradicate leukemia in murine models of acute myeloid leukemia. We therefore adapted the K562-mb15-41BBL, stimulation method to large-scale clinical-grade conditions, generating large numbers of highly cytotoxic NK cells. The results that we report here provide rationale and practical platform for clinical testing of expanded and activated NK cells for cell therapy of cancer. [Cancer Res 2009;69(9):4010-7]

DOI： 10.1158/0008-5472.CAN-08-3712

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Strategies effective for accelerating methotrexate removal in delayed methotrexate excretion have not been universally accepted. The authors report a case of a 12-year-old girl with osteosarcoma who developed acute renal failure immediately after the first administration of high-dose methotrexate. Plasma methotrexate was effectively removed with repeated charcoal hemoperfusion in addition to plasma exchange and leucovorin rescue. Charcoal hemoperfusion was most effective for reducing plasma methotrexate with approximately 50% of methotrexate being reduced during each of the procedures. No rebound increase in MTX levels was observed. The patient received further therapy with other cancer drugs and has been well for 3.5 years. © 2009 Informa UK Ltd.

DOI： 10.1080/08880010902976023

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記述言語：日本語   掲載種別：研究論文（研究会，シンポジウム資料等）  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：TAYLOR & FRANCIS INC  

Strategies effective for accelerating methotrexate removal in delayed methotrexate excretion have not been universally accepted. The authors report a case of a 12-year-old girl with osteosarcoma who developed acute renal failure immediately after the first administration of high-dose methotrexate. Plasma methotrexate was effectively removed with repeated charcoal hemoperfusion in addition to plasma exchange and leucovorin rescue. Charcoal hemoperfusion was most effective for reducing plasma methotrexate with approximately 50% of methotrexate being reduced during each of the procedures. No rebound increase in MTX levels was observed. The patient received further therapy with other cancer drugs and has been well for 3.5 years.

DOI： 10.1080/08880010902976023

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記述言語：日本語   出版者・発行元：(一社)日本内分泌学会  

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記述言語：日本語   出版者・発行元：(株)医学書院  

胞巣状構造を呈するが特異的遺伝子転座を認めない横紋筋肉腫1例を経験したので報告する.症例は1歳2ヵ月の女児で,針細胞診で横紋筋肉腫と診断し,広範切除を行った.術後組織病理学所見では,胎児型の部分と胞巣状構造が混じったパターンであった.典型的胞巣型と異なり,線維性間質に乏しく,大量の横紋筋芽細胞が混在するのが特徴であった.胞巣型に特異的なt(2;13)またはt(1;13)の転座を認めなかった.術後6年9ヵ月の現在,多発性転移を認め,再発と軽快を繰り返し,現在化学療法中である.(著者抄録)

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ELSEVIER SCIENCE INC  

Objective. There is growing interest in the use of cytokine-induced killer (CIK) cells in cancer therapy. In this study, we sought to maximize the antileukemic activity of anti-CD19 receptor-modified CIK cells against B-lineage acute lymphoblastic leukemia (ALL).
Materials and Methods. CIK cells were transduced with retroviral vectors carrying different types of anti-CD19 chimeric receptors: anti-CD19-xi, anti-CD19-DAP10, anti-CD19-4-1BB-xi, and anti-CD19-CD28-xi. A truncated form of the receptor was used as a control. Transduced CIK cells were then analyzed for their cytotoxic activity against ALL cells and for their capability to proliferate and to release cytokines after ALL encounter.
Results. CIK cells were efficiently transduced with all the anti-CD19 retroviral vectors. Anti-CD19 receptor expression conferred powerful killing activity against ALL cells. However, there were clear advantages when receptors containing the co-stimulatory molecules 4-1BB or CD28 were transduced. Such cells had significantly more potent cytotoxicity than cells expressing the anti-CD19-xi or anti-CD19-DAP10. Moreover, the presence of 4-1BB or CD28 in the receptor increased the production of interleukin (IL)-2, tumor necrosis factor (TNF)-alpha, TNF-beta, IL-5, IL-6, and IL-8 elicited by coculture with ALL cells. Notably, anti-CD19-4-1BB-xi CIK cells secreted particularly low levels of interleukin-10 and proliferated strongly after contact with ALL cells.
Conclusions. Anti-CD19 chimeric receptors delivering primary and costimulatory signals render CIK cells powerfully cytotoxic against ALL cells and induce secretion of immunostimulatory cytokines and proliferation. These results support the testing of genetically modified CIK cells in clinical trials. (c) 2007 ISEH - Society for Hematology and Stem Cells. Published by Elsevier Inc.

DOI： 10.1016/j.exphem.2007.05.018

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：MARY ANN LIEBERT INC  

Transfection of human cells with DNA in biomedical applications carries the risk of insertional mutagenesis. Transfection with mRNA avoids this problem; however, in vitro production of mRNA, based on preliminary DNA template cloning in special vectors, is a laborious and time-consuming procedure. We report an efficient vector-free method of mRNA production from polymerase chain reaction-generated DNA templates. For all cell types tested mRNA was transfected more readily than DNA, and its expression was highly uniform in cell populations. Even cell types relatively resistant to transfection with DNA could express transfected mRNA well. The level of mRNA expression could be controlled over a wide range by changing the amount of input RNA. Cells could be efficiently and simultaneously loaded with several different transcripts. To test a potential clinical application of this method, we transfected human T lymphocytes with mRNA encoding a chimeric immune receptor directed against CD19, a surface antigen widely expressed in leukemia and lymphoma. The transfected mRNA conferred powerful cytotoxicity to T cells against CD19(+) targets from the same donor. These results demonstrate that this method can be applied to generate autologous T lymphocytes directed toward malignant cells.

DOI： 10.1089/hum.2006.17.1027

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記述言語：日本語   出版者・発行元：(株)日本小児医事出版社  

先天性食道狭窄症は,出生児の1/25,000〜50,000人にしかみられない稀な疾患であり,初発症状から診断までに長い期間を要する症例が多いとされている.我々は,生後6ヵ月ごろから嘔吐を認め,1歳6ヵ月に急性増悪した1例を経験した.嘔吐はすべて,口腔内に手指を挿入することで誘発され,その症状の奇異さから心理的な要因も疑われた.しかし,食道造影やMRIによって食道の狭窄病変が発見され,先天性食道狭窄と診断された.上部消化管内視鏡で狭窄部位の口側に,さくらんぼの種を発見し,これにより症状が増悪したと考えられた.先天性食道狭窄症は小児科医にとって馴染みの薄い疾患であるが,繰り返す嘔吐の鑑別疾患として念頭におくことが重要である(著者抄録)

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2006&ichushi_jid=J00643&link_issn=&doc_id=20060803320004&doc_link_id=%2Fag1snrsd%2F2006%2F005906%2F005%2F1089-1093%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fag1snrsd%2F2006%2F005906%2F005%2F1089-1093%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：日本語   掲載種別：研究論文（学術雑誌）   出版者・発行元：(有)科学評論社  

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記述言語：日本語   掲載種別：研究論文（学術雑誌）   出版者・発行元：(有)科学評論社  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：AMER SOC HEMATOLOGY  

Natural killer (NK) cells hold promise for improving the therapeutic potential of allogeneic hematopoietic transplantation, but their effectiveness is limited by inhibitory HLA types. We sought to overcome this intrinsic resistance by transducing CD56(+)CD3(-) NK cells with chimeric receptors directed against CD19, a molecule widely expressed by malignant B cells. An abundance of NK cells for transduction was secured by culturing peripheral blood mononuclear cells with K562 cells expressing the NK-stimulatory molecules 4-1BB ligand and interleukin 15, which yielded a median greater than 1000-fold expansion of CD56(+)CD3(-) cells at 3 weeks of culture, without T-lymphocyte expansion. Expression of anti-CD19 receptors linked to CD3 xi overcame NK resistance and markedly enhanced NK-cell-mediated killing of leukemic cells. This result was significantly improved by adding the 4-1 BB costimulatory molecule to the chimeric anti-CD19-CD3 xi receptor; the cytotoxicity produced by NK cells expressing this construct uniformly exceeded that of NK cells whose signaling receptors lacked 4-1BB, even when natural cytotoxicity was apparent. Addition of 4-1 BB was also associated with increased cell activation and production of interferon gamma and granulocyte-macrophage colony-stimulating factor. Our findings indicate that enforced expression of signaling receptors by NK cells might circumvent inhibitory signals, providing a novel means to enhance the effectiveness of allogeneic stem cell transplantation.

DOI： 10.1182/blood-2004-12-4797

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：BLACKWELL PUBLISHING ASIA  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：TAYLOR & FRANCIS LTD  

To clarify the mechanism of progression and acquired drug resistance of leukemia, we searched for an overexpressed gene in drug-resistant leukemia cells and identified an approximately 5-kb transcript by using the subtraction method. The nucleotide sequence of the gene was highly homologous to those of human endogenous retrovirus (HERV) transcripts. Reverse transcriptase-polymerase chain reaction (RT-PCR) revealed that the gene was overexpressed in cells from 6 childhood acute lymphoblastic leukemia patients (60%) but not in bone marrow cells at remission. Peripheral blood mononuclear cells from normal controls (n=11) and bone marrow cells from non-leukemia patients (n=13) did not express the gene. These findings indicate that the gene may play a role in leukemogenesis and may be a novel leukemia marker. Further studies on the functional role of the gene are needed.

DOI： 10.1080/10428190412331272758

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：BLACKWELL PUBLISHING ASIA  

Background: For the purpose of studying renal side-effects induced by antineoplastic agents, the authors examined glomerular injury as well as tubular injury of patients with chemotherapy.
Methods: Thirteen patients underwent a combined total of 64 courses of chemotherapy. Urinary albumin, beta2-microglobulin (beta2-MG), N-acetyl-beta-glucosaminidase (NAG) and urinary protein were measured before and serially after chemotherapy.
Results: The values of albumin/creatinine (albumin/cre) ratio and beta2-MG/creatinine (beta2-MG/cre) ratio after chemotherapy were higher than those before chemotherapy (P < 0.01). NAG/creatinine (NAG/cre) ratio and creatinine clearance (Ccr) were not different. These were also examined before the next course of chemotherapy and were compared with those of control children. Albumin/cre ratio was significantly different (P < 0.01). beta2-MG/cre ratio and NAG/cre were not different. Furthermore, in patients with normal beta2-MG/cre, the albumin/cre ratio was significantly higher than in control children.
Conclusions: These results indicate that antineoplastic agents can not only induce tubular dysfunction but also glomerular dysfunction, which is persistent and independent of tubular dysfunction.

DOI： 10.1111/j.1442-200x.2004.01958.x

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：NATURE PUBLISHING GROUP  

Deficient expression of BLNK, an adaptor molecule crucial for normal B-cell development, is associated with increased pro-B/pre-B-cell expansion in mice. It has been proposed that BLNK deficiency is a primary cause of B-lineage acute lymphoblastic leukemia (ALL). We studied BLNK expression in the leukemic cells from 352 patients with childhood ALL (309 B-lineage; 43 T-lineage). By HG_U95Av2 Affymetrix GeneChip analysis, BLNK was expressed in 275 of 284 (96.8%) B-lineage ALL samples but in only one of 43 (2.3%) T-lineage ALL samples. Of 118 B-lineage ALL samples analyzed with the HG_U133A GeneChip, 117 (99.2%) expressed BLNK. All 30 primary B-lineage ALL samples studied by RT-PCR expressed BLNK transcripts; all 19 samples studied by Western blotting or flow cytometry expressed BLNK protein. Levels of BLNK in B-lineage ALL were as high as those of their normal counterparts; they were not related with genetic subgroups or differentiation stage. These results indicate that BLNK deficiency is a rare occurrence in childhood B-lineage ALL and is unlikely to be a common leukemogenic event as previously proposed.

DOI： 10.1038/sj.leu.2403349

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：NATURE PUBLISHING GROUP  

To develop a therapy for drug-resistant B-lineage acute lymphoblastic leukemia (ALL), we transduced T lymphocytes with anti-CD19 chimeric receptors, consisting of an anti-CD19 single-chain variable domain (reactive with most ALL cases), the hinge and transmembrane domains of CD8alpha, and the signaling domain of CD3zeta. We compared the antileukemic activity mediated by a novel receptor ('anti-CD19-BB-zeta') containing the signaling domain of 4-1BB (CD137; a crucial molecule for T-cell antitumor activity) to that of a receptor lacking costimulatory molecules. Retroviral transduction produced efficient and durable receptor expression in human T cells. Lymphocytes expressing anti-CD19-BB-zeta receptors exerted powerful and specific cytotoxicity against ALL cells, which was superior to that of lymphocytes with receptors lacking 4-1BB. Anti-CD19-BB-zeta lymphocytes were remarkably effective in cocultures with bone marrow mesenchymal cells, and against leukemic cells from patients with drug-resistant ALL: as few as 1% anti-CD19-BB-zeta-transduced T cells eliminated most ALL cells within 5 days. These cells also expanded and produced interleukin-2 in response to ALL cells at much higher rates than those of lymphocytes expressing equivalent receptors lacking 4-1BB. We conclude that anti-CD19 chimeric receptors containing 4-1BB are a powerful new tool for T-cell therapy of B-lineage ALL and other CD19(+) B-lymphoid malignancies.

DOI： 10.1038/sj.leu.2403302

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY-LISS  

A rare case of different leukemias simultaneously occurring in the bone marrow (BM) and the central nervous system (CNS) was encountered. A 4-year-old girl was diagnosed with acute lymphoblastic leukemia and achieved a complete remission with chemotherapy. Two years after diagnosis, she was found to have acute monocytic leukemia at first relapse in the BM. One month after the second course of induction therapy, lymphoblastic leukemia in the BM and monocytic leukemia in the CNS were found simultaneously. Chromosomal analysis of leukemia cells in the BM and CNS showed distinct results. The mechanism of double leukemias occurring was obscure, although a lineage switch, therapy-related, or de novo leukemia could be considered as possibilities. Double leukemias should be kept in mind when leukemias relapse in several sites. Am. J. Hematol. 75:164-167, 2004. (C) 2004 Wiley-Liss, Inc.

DOI： 10.1002/ajh.10478

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：BIOLIFE SAS  

The use of immune cells with restricted specificities for the treatment of cancer is a rapidly emerging area of clinical research. Chimeric receptors composed of the single-chain variable domain of murine antibodies and human signaling molecules are a promising tool to redirect the specificity of autologous or allogeneic immune cells. The success of this approach depends on the identification of target molecules expressed preferentially on cancer cells. Moreover, appropriate primary and secondary stimuli must be delivered to generate vigorous and durable immune responses. Since cancer cells often lack ligands for key co-stimulatory molecules, the addition of molecules such as CD28 or 4-1BB to the chimeric receptors can significantly improve their function. Studies in vitro and in animal models indicate that immune cells expressing chimeric receptors can have remarkable anti-cancer activity, while experimental and clinical data indicate that long-term persistence of adoptively transferred cells is feasible. Therefore, testing of this approach in clinical trials is warranted. We here review the principles and methodologies for designing chimeric receptors and delivering them into immune cells, as well as some of the potential complications associated with this form of cell therapy.

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：新潟大学  

To evaluate the involvement between Fasassociated phosphatase-1 (FAP-1) and drug resistance, the expression of FAP-1 in leukemic cell lines, drugresistant sublines, and leukemic cells was examined by RT-PCR. In comparison with the parental cell line (KY-821), arabinofuranosylcytosine (ara-C)-resistant subline (KY-Ra), and vincristine (VCR)-resistant subline (KY-VCR) showed an increase in FAP-1 expression and resistance to anti-CD95 antibody-induced apoptosis. Leukemic cell lines (KY-821 and Jurkat) revealed an increase in FAP-1 expression after exposure to ara-C (1×10^<-5>M) and VCR (1×10^<-7>M). Furthermore, after exposure to anticancer drugs at low dosages for five days, both KY-821 and Jurkat revealed an increase in FAP-1 expression and resistance to anti-CD95 antibody-induced apoptosis. In study with de novo leukemic cells, two cases showed an increase in FAP-1 expression after exposure to anticancer drugs, of which one revealed an increased resistance to anti-CD95 antibody-induced apoptosis. These results indicate a close involvement between CD95-mediated apoptosis and drug resistance, and suggest that anticancer drugs suppress CD95-mediated apoptosis in part by inducing FAP-1 overexpression in some leukemic cells.

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その他リンク： http://search.jamas.or.jp/link/ui/2006149677

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：BLACKWELL PUBLISHING ASIA  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：TAYLOR & FRANCIS LTD  

We report a rare case of acute lymphoblastic leukemia (ALL) who developed dyspnea, neurological disturbance with illusions, pancytopenia, phagocytosis and coagulation disturbances following bacterial tonsillitis. The values of soluble interleukin-2 receptor (slL-2R), IL-6 and IL-8 were also elevated. Her clinicolaboratory findings were similar to hemophagocytic lymphohistiocytosis (HLH), which is a cytokine disease induced by activated T cells and macrophages. Atypical HLH following bacterial tonsillitis should be kept in mind in leukemia patients.

DOI： 10.1080/1042819031000079078

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER-VERLAG  

A 1-year-old boy with hemophagocytic lymphohistiocytosis exhibited proteinuria I month after unrelated cord blood cell transplantation, which persisted without hematuria. Laboratory study showed an increase of factor VIII-related antigen and total plasminogen activator inhibitor, suggesting endothelial injury. Histological examination of autopsy materials showed increased mesangial matrices and double-contoured basement membranes, and ultrastructurally, swelling of the endothelial cells and widening of the subendothelial space with mesangial interposition. Thrombosis was not observed at any of the sites. This case may be vasculopathy distinct from thrombotic microangiopathy (TMA) or a variant form of TMA following blood stem cell transplantation (BSCT). This vasculopathy should be considered in the differential diagnosis of proteinuria in the early stages after BSCT.

DOI： 10.1007/s00467-003-1081-9

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY-BLACKWELL  

To create immortal mesenchymal cell lines, we transduced primary human bone marrow mesenchymal cells with telomerase reverse transcriptase (TERT). TERT+ mesenchymal cells continued to grow for &gt; 2 years; parallel TERT- cultures underwent senescence after 15 weeks. TERT+ mesenchymal cells did not form foci in soft agar, had a normal karyotype and could differentiate into osteoblasts and chondrocytes. Their capacity to support leukaemic lymphoblasts and normal CD34(+) haematopoietic cells was equal to or greater than that of primary cells; 42 TERT+ mesenchymal cell clones varied in their supporting capacity. Immortalized mesenchymal cells offer a promising tool for identifying molecules that regulate human haematopoiesis.

DOI： 10.1046/j.1365-2141.2003.04217.x

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

Purpose: Renal drug excretion is variously influenced by nephrotoxic drugs. This study was designed to evaluate renal function as a late renal side effects in children receiving combination chemotherapy for malignancy.
Patients and Methods: Follow-up studies of 30 newly diagnosed patients were performed a median of 12 months after completion of chemotherapy. The glomerular filtration rate (GFR) was measured using sodium thiosulfate. The following were also assessed: urinary high-molecular-weight fraction (urinary albumin/urinary creatinine ratio); para-aminohippurate (PAH) clearance; urinary low-molecular-weight fraction (urinary beta2-microglobulin/urinary creatinine ratio); and routine serum and urinary parameters.
Results: Serum and urinary electrolytes were normal in most patients. GFR was low in four patients (13%). Urinary high-molecular-weight fraction was elevated in two patients. Urinary low-molecular-weight fraction was elevated in one patient. PAH clearance was below the referenced normal value in 73% of the patients.
Conclusions: This report demonstrates decreased PAH clearance as a late renal side effect of chemotherapy and suggests disturbed function of the organic anion transport system. The unexpected high serum concentration of drugs excreted through the organic anion transport system may induce severe side effects. Elucidation of the mechanism and clinical relevance of decreased PAH clearance is warranted.

DOI： 10.1097/00043426-200303000-00006

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：TAYLOR & FRANCIS INC  

A rare case of undetermined fever and skin ulcers is reported. The patient had an 8-month history of recurrent fever destructive ulceration of the midline facial tissue, and symmetrical skin ulcer in the cheeks and the back of the hand. Pathological examination revealed that the patient had lethal midline granuloma (centrofacial malignant T-cell lymphoma), which is very rare in childhood. Centrofacial malignant T-cell lymphoma should be considered as a differential diagnosis Of unexplained fever and skin ulcer in children.

DOI： 10.1080/08880010290097422

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：BLACKWELL PUBLISHING ASIA  

DOI： 10.1046/j.1442-200X.2002.01619.x

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：TAYLOR & FRANCIS INC  

Acute suppurative thyroiditis (AST) is quite rare, even in immunocompromised patients. The authors describe 2 cases of AST during aggressive chemotherapy for acute myelogeneous leukemia (AML). They were treated with aggressive combination chemotherapy and achieved complete remission. After several courses of chemotherapy, they developed fiver and pain in the region of the thyroid gland. Laboratory tests showed hyperthyroidism and elevated levels of thyroglobulin and C-reactive protein. Ultrasonography revealed hypoechoic areas in the thyroid gland. A diagnosis of AST was made. Bacterial infections were suspected because they were success fully treated with antibiotics. After a month, the patients' thyroid function and thyroglobulin levels returned to normal without a period of transient hypothyroidism. A pyriform sinus fistula was not demonstrated. The results suggest that neutropenia and preceding cellulitis around the thyroid gland, which might be subsequent to oral mucosal damage induced by anticancer drugs, may play a role in the development of AST. AST should be considered a potential complication of aggressive chemotherapy for leukemia.

DOI： 10.1080/08880010252899415

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：BLACKWELL PUBLISHING ASIA  

Background: To better understand the mechanisms of glomerular epithelial cell (GEC) injuries in various diseases. we compared GEC excreted during chemotherapy (antineoplastic drugs) and GEC excreted in renal diseases.
Methods: For 19 patients undergoing chemotherapy (85 courses), 69 patients with IgA nephropathy and 16 patients with Henoch-Scholein purpura nephritis. the number of excreted GEC and GEC casts were counted by an immunofluorescent study. The morphological features of GEC were also studied in an immunofluorescent study combined with Hoechst stain.
Results: Glomerular epithelial cells were detected in 78% of the chemotherapy courses and in 94% of the patients with renal diseases. The GEC casts were observed in 2% of chemotherapy courses, while in renal diseases GEC casts were observed in 60% of the patients. Proteinuria ( &gt; 30 mg/dL) and hematuria were not identified in any of the chemotherapy courses. The morphology and size of GEC were more variable than that in patient,, with nephropathy. Furthermore, GEC in patients undergoing chemotherapy often showed small nuclei and fragmented nuclei, which were rarely observed in patients with nephropathy.
Conclusions: These result,, showed that the detachment of podocytes was not directly associated with proteinuria or hematuria, The findings also suggest that GEC are damaged via an apoptotic process by chemotherapy. On the contrary, GEC may be detached through a non-apoptotic process in renal diseases.

DOI： 10.1046/j.1442-200X.2001.01473.x

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

DOI： 10.1097/00043426-200101000-00020

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記述言語：日本語   出版者・発行元：(株)日本小児医事出版社  

非EBウイルス関連血球貪食症候群(HLH)8例を,化学療法を必要とした群4例と支持療法のみで軽快した群4例に分け,初期治療方針の決定に寄与した因子を回顧的に検討した.死亡したのは化学療法群の2例で,その他は治療後寛解生存中である.血算,血液凝固系,血液生化学,フェリチン,髄液検査など各臨床的因子は2群間で差がなく,発症時に利用可能な因子により2群を分別することは困難と考えられた.血清サイトカイン濃度ではインターロイキン6が化学療法群で有意に高値であったが,発症早期の治療方針の決定に有用とは考えられなかった

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2000&ichushi_jid=J00643&link_issn=&doc_id=20001102180004&doc_link_id=%2Fag1snrsd%2F2000%2F005311%2F005%2F1893-1901%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fag1snrsd%2F2000%2F005311%2F005%2F1893-1901%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：BLACKWELL SCIENCE ASIA  

DOI： 10.1046/j.1442-200x.2000.01188.x

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY-BLACKWELL  

Background: Central catecholamines: particularly dopaminergic and noradrenergic systems, have affected the appetitive behavior in patients with anorexia nervosa (AN). The purpose of this study is to distinguish the characteristics of contingent negative variation (CNV) and postimperative negative variation (PINV), which may reflect the level of catecholamine in children with AN.
Methods: Eight children with AN aged 10 to 15 years and 23 age-matched healthy children were recruited. Contingent negative variation was recorded from the frontal midline (Fz), central midline (Cz) and parietal midline (Pz) referenced to linked earlobes during 30 trials consisting of a warning stimulus and an imperative stimulus with an interstimulus interval of 2 s and an intertrial interval of 10 s. The imperative stimulus of each trial required a button press.
Results: Children with AN had a diminished amplitude of the CNV. They had a significantly more attenuated early CNV and late CNV amplitude at Ct than normal children. No significant differences were observed between AN children and normal children in the amplitude of PINV at all three electrode sites. No difference could be found between the two groups in the frequencies of normal and abnormal duration of PINV.
Conclusion: These findings suggest that early CNV may be diminished by norepinephrine deficiency and late CNV may be attenuated by dopaminergic deficiency in children with AN. Reduced CNV may represent impaired cognitive processes which reflect impaired appetitive behavior in AN children.

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：BLACKWELL SCIENCE ASIA  

Background: It is a matter of concern whether serum eosinophil cationic protein (ECP) can be considered as a disease marker in children with acute asthma being treated without corticosteroids.
Methods: Fourteen children (nine male, five female, aged 6-12 years) with acute asthmatic exacerbation, administered the appropriate drugs, with the exception of systemic or inhaled corticosteroids, were examined. They were all free from apparent asthmatic attacks during a follow-up period of 1 month. Serum ECP, eosinophil count and forced expiratory volume in 1 s (FEV1) were measured at referral, on the day of discharge, I week and 4 weeks after discharge, respectively.
Results: The ratio of ECP/eosinophil count (ECP:Eo ratio), expressed as micrograms of ECP (mu g/L)/the number of eosinophil (/mu L)x 1000, was also evaluated as a marker of eosinophil activation. Compared with the value at referral, FEV1 (% predicted) significantly increased on the day of discharge (P &lt; 0.05), 1 week after (P &lt; 0.05) and 4 weeks after discharge (P &lt; 0.05). However, serum ECP concentrations showed no significant changes during the follow-up period. Eosinophil count showed no significant changes on the day of discharge or I week after discharge, but significantly increased 4 weeks after discharge (P &lt; 0.05). In contrast, the ECP:Eo ratio significantly decreased on the day of discharge (P &lt; 0.05), 1 week after (P &lt; 0.05) and 4 weeks after discharge (P &lt; 0.05).
Conclusion: These data suggest that serum ECP is a poor disease marker in asthmatic children with acute exacerbation who receive no corticosteroid therapy, probably due to marked changes in the eosinophil count. However, the ECP:Eo ratio might be a better marker than serum ECP in such patients.

DOI： 10.1046/j.1442-200X.1999.4121045.x

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PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：BLACKWELL SCIENCE  

The intra-abdominal visceral fat to subcutaneous fat ratio (V/S ratio) has been reported to be strongly related to disorders of glucose and lipid metabolism, and hypertension. It is a matter of concern as to whether weight loss causes an improvement of the V/S ratio or not in obese children. Changes in body fat distribution during weight loss in 23 obese children were quantified by weight, bioelectrical impedance analysis (BIA) and computed tomography (CT scan of the abdomen). Twenty-three patients were divided into two groups; six were in the inpatient group and 17 were in the outpatient group. Bodyweight, body fat percentage, subcutaneous fat and visceral fat were significantly higher in the inpatient group than in the outpatient group before weight loss. Whereas the V/S ratio was almost equal between the two groups before weight loss. Bodyweight, body fat percentage, subcutaneous fat and visceral fat were found to decrease significantly during weight loss in the two groups. The V/S ratio of the outpatient group did not change after weight loss. In contrast, the V/S ratio of the inpatient group decreased significantly during weight loss. These preliminary findings suggest that a large amount of body fat and a high obesity rate are not always accompanied by a high V/S ratio in obese children. The fat pattern changes during weight loss with strict dietary therapy and therapeutic exercise. A larger sample of obese children should be studied to test this conjecture.

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記述言語：日本語   出版者・発行元：(一社)日本周産期・新生児医学会  

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記述言語：日本語   出版者・発行元：日本小児泌尿器科学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児腎臓病学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

症例は9ヵ月男児.繰り返す発熱,腋窩リンパ節腫大,全身の膿疱性皮疹を主訴に当院に紹介された.当初,前頭骨の骨融解像,臨床像,sIL-2R異常高値からランゲルハンス組織球症との鑑別を要し,皮膚生検を施行したが確定診断に至らず,腋窩リンパ節生検を施行した.生検組織からBCG菌が同定され,播種性BCG感染症と診断した.皮膚生検ではBCG菌は検出されず,BCG菌への過敏性反応による結核疹と考えられた.重症細菌感染,ウイルス感染の既往はなく,メンデル遺伝型マイコバクテリア易感染症を疑い精査した結果,IFNGR1遺伝子の既知のde-novoヘテロ変異が同定され,IFN-γR1部分欠損症と診断した.抗結核薬イソニアジド,リファンピシンの2剤による治療で症状は速やかに軽快した.18ヵ月間内服を継続し,終了後半年の現在も再燃なく経過している.ランゲルハンス組織球症として非典型的な皮疹や経過の場合にはBCG感染症を考える必要がある.(著者抄録)

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記述言語：日本語   出版者・発行元：(一社)日本小児神経外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(公社)日本整形外科学会  

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記述言語：日本語   出版者・発行元：(公社)日本整形外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本血液学会-東京事務局  

造血器腫瘍に対して免疫学的機序を持つ新規薬剤が次々と登場している。長らく急性リンパ性白血病(acute lymphoblastic leukemia,ALL)の分野への新薬導入はほとんどなかったが,近年,CD19を標的とし患者T細胞を活性化して殺細胞効果を発揮するブリナツモマブ,CD22を標的として殺細胞薬を選択的にデリバリーするイノツズマブ・オゾガマイシン,そして同じくCD19を標的とするCAR-T細胞療法チサゲンレクルユーセルが欧米豪に続いて本邦においても承認された。従来,再発難治ALLに対しては抗がん剤による再導入療法と同種造血細胞移植のみが根治を目指せる治療であったが,大きく様変わりしようとしている。本稿では,これら治療薬の臨床開発を振り返り,小児・思春期における位置づけについて考察した。現時点でこれらの治療の選択アルゴリズムは確立していないため,それぞれの利点と欠点を十分に理解しつつ,個々の症例での治療方針を決定することが重要である。(著者抄録)

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2020&ichushi_jid=J01540&link_issn=&doc_id=20200707220015&doc_link_id=130007869117&url=https%3A%2F%2Fci.nii.ac.jp%2Fnaid%2F130007869117&type=CiNii&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00003_1.gif

記述言語：英語   出版者・発行元：(一社)日本輸血・細胞治療学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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J-GLOBAL

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：WILEY  

Web of Science

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児神経外科学会  

J-GLOBAL

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記述言語：日本語   出版者・発行元：(一社)日本小児神経外科学会  

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記述言語：日本語   出版者・発行元：(株)日本小児医事出版社  

繰り返す腸重積を契機に発見され、フローサイトメトリーでλ鎖優位のlight chain restriction(LCR)を認めた虫垂リンパ過形成の6歳女児例を経験した。腹部造影CTでは最大径2.5cmの全周性虫垂腫大を認め、迅速病理診断およびスタンプ標本からも成熟B細胞リンパ腫で矛盾のない所見を得たが、免疫グロブリン遺伝子やc-myc遺伝子の再構成は認めず、最終的に永久病理標本により虫垂リンパ過形成の診断となった。追加の治療介入は行わず経過観察の方針としたが、約12ヵ月を経過し再発は認めていない。文献上、LCRを認める虫垂リンパ過形成は極めて稀で、4〜6歳の4例が報告されているのみである(すべてλ鎖優位のLCR)。本例の経験から、反復性腸重積の稀な原因として虫垂リンパ過形成があること、そして、反応性の病態ながらもLCRを示す場合があることを念頭に置く必要があることが示された。(著者抄録)

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2019&ichushi_jid=J00643&link_issn=&doc_id=20190124480010&doc_link_id=%2Fag1snrsd%2F2019%2F007202%2F010%2F0170-0174%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fag1snrsd%2F2019%2F007202%2F010%2F0170-0174%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(株)医学書院  

＜文献概要＞症例1:1歳5ヵ月,女児.頭部に脂漏性湿疹様皮疹,体幹四肢には紫斑,丘疹があり,Langerhans細胞の骨髄浸潤と,CT検査で蝶形骨の破壊像を認めた.症例2:5ヵ月,女児.下腹部や鼠径部に点状紫斑や丘疹があり,CT検査で右下顎骨の骨溶解を伴う腫瘤性病変を認めた.症例3:5歳,女児.鼠径部および大腿内側に褐色調の紅斑,丘疹があり,CT検査で第5頸椎の圧迫骨折を認めた.いずれの症例も皮疹からLangerhans細胞組織球症(Langerhans cell histiocytosis:LCH)を疑い,病理組織学的検査,電子顕微鏡検査にて多臓器型のLCHと診断した.化学療法もしくは経過観察で,皮疹は改善傾向である.LCHの皮疹は多彩であり,視診だけで診断することは困難である.LCHの皮膚病変を疑った場合は積極的に皮膚生検を行い,病型を確定する必要がある.

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2019&ichushi_jid=J01559&link_issn=&doc_id=20190201020017&doc_link_id=10.11477%2Fmf.1412205622&url=https%3A%2F%2Fdoi.org%2F10.11477%2Fmf.1412205622&type=%88%E3%8F%91.jp_%83I%81%5B%83%8B%83A%83N%83Z%83X&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00024_2.gif

記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：英語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：英語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：日本語   出版者・発行元：(株)日本小児医事出版社  

急性リンパ性白血病(ALL)は腎腫大を含め様々な臓器浸潤を来すが、腎臓に結節性病変を呈することは少ない。症例は1歳4ヵ月の男児。発熱が持続し、血液検査でLDHの異常高値を指摘され、造影CTで両側腎腫大と多発結節性病変を認めた。末梢血中に芽球の出現があり、骨髄穿刺で前駆B細胞性急性リンパ性白血病と診断した。寛解導入療法後に両側腎腫大および腎多発結節性病変は消失し完全寛解に至った。腎の多発結節性病変の鑑別として造血器腫瘍も考慮すべきである。(著者抄録)

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

平成25年に小児がん拠点病院が認定され、拠点病院と地域中隔病院との役割分担と連携効率化が求められている。新潟県では過去40年にわたり県内中核病院を中心とした小児がん診療体制を構築し、県内症例の統一的治療を行ってきた。今回、小児がん拠点病院に選出されなかったことから、地域中核病院として役割と今後の方向性を再検討すべき段階となり、外科治療を担当する立場から考察した。拠点病院の条件を満たすための課題は、先進的な臨床試験や新薬治験が行える臨床研究体制の充実、保育士、専門看護師、臨床心理士などによる高度で専門的なサポート体制、緩和ケア、義務教育年限後の学業支援、社会復帰支援などの他に、陽子線治療施設、小児がん専門病棟、ファミリーハウスなどのインフラ充実があげられる。これらは中核病院のみでは解決できない課題も多く、今後の行政への働きかけと連携が重要となる。一方、現在の進行例の外科治療は、手術リスクの評価、生検、化学療法と放射線治療後の遺残腫瘍摘出であるが、腫瘍発生部位は多岐にわたり、外科関連科との知識と技術との連携協力をもってしても、重篤な合併症を回避できない症例も多く、腫瘍完全摘出ではなく陽子線治療を併用した外科治療など、今後拠点病院との連携強化も重要である。さらに、腫瘍切除のための小児外科、整形外科、泌尿器科、脳神経外科など多領域の知識と経験を有する外科医の育成も重要と考えられる。(著者抄録)

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記述言語：日本語   出版者・発行元：(公社)日本皮膚科学会  

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記述言語：日本語   出版者・発行元：(公社)日本皮膚科学会  

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記述言語：日本語   出版者・発行元：(公社)日本整形外科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：WILEY  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：WILEY  

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記述言語：日本語   出版者・発行元：新潟医学会  

新潟県は、子ども病院を持たない県のなかで最も小児人口の多い県だとされている。本県に子ども病院はいらないのだろうか?小児科医の視点から、私見を述べた。(著者抄録)

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2017&ichushi_jid=J00990&link_issn=&doc_id=20180328050001&doc_link_id=%2Fdg3nigta%2F2017%2F013110%2F001%2F0565-0568%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdg3nigta%2F2017%2F013110%2F001%2F0565-0568%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：日本脳腫瘍病理学会  

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記述言語：日本語   出版者・発行元：新潟医学会  

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2017&ichushi_jid=J00990&link_issn=&doc_id=20170929080007&doc_link_id=%2Fdg3nigta%2F2017%2Fs13105%2F002%2F0311-0312%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdg3nigta%2F2017%2Fs13105%2F002%2F0311-0312%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：AMER SOC HEMATOLOGY  

Web of Science

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記述言語：英語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：FERRATA STORTI FOUNDATION  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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J-GLOBAL

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：AMER SOC HEMATOLOGY  

Web of Science

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記述言語：英語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児リウマチ学会  

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記述言語：日本語   出版者・発行元：(一社)日本内分泌学会  

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記述言語：英語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：英語   掲載種別：書評論文，書評，文献紹介等   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

In addition to T-cell receptor signals, T lymphocytes require costimulatory signals for robust activation. Among these, those mediated by 4-1BB (CD137, TNFRSF9) are critical for tumor immunity. 4-1BB is expressed in T-cell receptor-activated lymphocytes as well as natural killer cells and other hematopoietic and nonhematopoietic cells. 4-1BB ligation induces a signaling cascade that results in cytokine production, expression of antiapoptotic molecules, and enhanced immune responses. In line with the described function of 4-1BB, its addition to CD3 zeta chimeric antigen receptors (CARs) increases their capacity to provoke T-cell expansion and antitumor activity. The results of preclinical studies with 4-1BB CARs have been corroborated by encouraging results from clinical trials. Advantages and disadvantages of 4-1BB CARs versus CARs bearing other costimulatory components remain to be fully elucidated. In this review, we discuss the properties of 4-1BB, the design of 4-1BB CARs, and the function of T lymphocytes and natural killer cells expressing them.

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J-GLOBAL

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記述言語：日本語   出版者・発行元：(NPO)日本小児血液・がん学会・(NPO)日本小児がん看護学会・(公財)がんの子供を守る会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児血液・がん学会・(NPO)日本小児がん看護学会・(公財)がんの子供を守る会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(一社)日本口腔ケア学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児血液・がん学会・(NPO)日本小児がん看護学会・(公財)がんの子供を守る会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児血液・がん学会・(NPO)日本小児がん看護学会・(公財)がんの子供を守る会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児血液・がん学会・(NPO)日本小児がん看護学会・(公財)がんの子供を守る会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児血液・がん学会・(NPO)日本小児がん看護学会・(公財)がんの子供を守る会  

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記述言語：日本語   出版者・発行元：新潟医学会  

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2012&ichushi_jid=J00990&link_issn=&doc_id=20130222030041&doc_link_id=%2Fdg3nigta%2F2012%2Fs12610%2F034%2F0570-0570%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdg3nigta%2F2012%2Fs12610%2F034%2F0570-0570%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：日本語   出版者・発行元：(公社)日本整形外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本検査血液学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本皮膚科学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(公財)木村看護教育振興財団  

著者らが作成した「化学療法中の小児患者の食事管理基準」を、医療関係者が説明しやすく、かつ患児および保護者が利用しやすい携帯サイトのコンテンツとして開発し、その効果を、癌患児の家族会の会員40名(母親31名、父親9名)へのアンケート調査から検討した。対象を、免疫機能低下により患児に何らかの食事制限の経験がある保護者25名とそれ以外の保護者15名(対照群)に分け、検討した結果、患児に何らかの食事制限の経験がある保護者は対照群の保護者よりも、この携帯サイトを人に紹介したいと考えていることが分かった。

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：新潟医学会  

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2011&ichushi_jid=J00990&link_issn=&doc_id=20110609090004&doc_link_id=%2Fdg3nigta%2F2011%2F012502%2F004%2F0069-0074%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdg3nigta%2F2011%2F012502%2F004%2F0069-0074%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：日本語   出版者・発行元：日本小児血液学会  

2歳9ヵ月男児。患者は発熱と全身倦怠感を主訴とした。EBV関連血球貪食症候群と診断され、免疫化学療法で寛解が得られたものの、治療終了から2週間で再燃し、治療抵抗性となった。そこで、解熱療法施行後に強度を減弱した移植前処置を開始し、再燃から約4ヵ月後に1アリル不一致緊急臍帯血移植が施行された。その結果、EBV-DNAはday 5に検出感度以下となり、day 16に生着したが、short tandem repeat解析では混合キメリズムを呈していた。そこで以後、末梢血buffy coatのドナー割合は10〜19%と低値であったものの、T細胞ではドナー優位であったため静観したところ、移植4ヵ月後までに全系統でドナー優位となった。そしてDay 61にはEBV-DNAの再陽性化がみられ、day 260にはEBV特異的細胞障害性T細胞が検出され、更に15ヵ月時にはEBNA抗体の出現も認められたが、移植後22ヵ月でEBV-DNAは末梢血から消失、目下は2年間の寛解を維持している。以上、本症例の臨床的経過からも移入免疫系におけるEBVに対する免疫記憶の確立が、寛解維持に重要な役割を果たした可能性が示唆された。

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CiNii Books

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：AMER SOC HEMATOLOGY  

Web of Science

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児神経外科学会  

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記述言語：日本語   出版者・発行元：新潟医学会  

The aim of young doctor exchange program was to exchange Japanese and Russian young doctors who have just graduated and to share experiences and knowledge in both clinical and basic fields. Oxana Podkaura (from Vladivostok), Valeria Gavrikova (from Krasnoyarsk) and Stanislav Korneev (from Khabarovsk) took part in this program held in Niigata from September 4 to 18, and studied children's leukemia and its treatment, Parkinson's disease and its treatment, and surgical treatment of liver, gastric and intestine cancer, respectively. They also attended two international symposia, CHRO2009 in Niigata and 2009 Tripartite symposium (Russia-United States-Japan Symposium on Cardiovascular Disease and Cancer) in Osaka.

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記述言語：日本語   出版者・発行元：がんの子供を守る会  

14歳女児.両側頸部リンパ節腫脹,EBVCA-IgA抗体上昇を認め,前医で壊死性リンパ節炎と病理診断を受けた.ステロイドで治療されたが有痛性腫脹は持続した.転院後,咽頭ファイバーで上咽頭腫瘤が発見され,EBウイルス関連上咽頭がんと診断された.放射線化学療法を施行し,2年以上経過後も寛解を維持している.EBVCA-IgA抗体上昇を伴う頸部リンパ節腫脹の症例では,咽頭ファイバーによる積極的なスクリーニングが推奨される.

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その他リンク： https://projects.repo.nii.ac.jp/?action=repository_uri&item_id=263302

記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：日本語   出版者・発行元：(株)日本小児医事出版社  

抗HNA1b抗体による新生児同種免疫性好中球減少症の1例を経験したので報告する。日齢1の女児で赤血球増多症を疑い血液検査をしたところ好中球減少を指摘され、精査のため当科に入院した。両親ともに生来健康であったが、1歳の姉が新生児期にMRSAの皮膚感染症のため入院し治療を受けていた。本患児について、日齢20に行った検査で母親および児の血清から抗HNA1b抗体が検出され、新生児同種免疫性好中球減少症と診断した。日齢28からST合剤の予防内服を開始した。経過中に明らかな感染症は認められず、好中球数は約3ヵ月で自然に増加した。新生児期に好中球減少症を認める症例については、治療法の選択や予後を予測するうえで、本疾患を鑑別することが必要であると考えられた。(著者抄録)

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2009&ichushi_jid=J00643&link_issn=&doc_id=20090702240010&doc_link_id=%2Fag1snrsd%2F2009%2F006207%2F012%2F1673-1677%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fag1snrsd%2F2009%2F006207%2F012%2F1673-1677%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：英語   掲載種別：速報，短報，研究ノート等（学術雑誌）   出版者・発行元：WILEY-LISS  

DOI： 10.1002/pbc.21626

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記述言語：日本語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：日本語   出版者・発行元：日本小児呼吸器学会  

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記述言語：日本語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：日本語   出版者・発行元：新潟医学会  

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2008&ichushi_jid=J00990&link_issn=&doc_id=20080909080044&doc_link_id=%2Fdg3nigta%2F2008%2F012207%2F044%2F0402-0402%26dl%3D2&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdg3nigta%2F2008%2F012207%2F044%2F0402-0402%26dl%3D2&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_5.gif

記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：DUKE UNIV PRESS  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：DUKE UNIV PRESS  

Web of Science

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記述言語：日本語   出版者・発行元：日本小児泌尿器科学会  

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記述言語：日本語   出版者・発行元：(一社)日本リンパ網内系学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：AMER SOC HEMATOLOGY  

Web of Science

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：AMER SOC HEMATOLOGY  

Web of Science

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児神経外科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：AMER SOC HEMATOLOGY  

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記述言語：日本語   出版者・発行元：日本小児血液学会  

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記述言語：日本語   出版者・発行元：日本臨床血液学会  

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記述言語：英語   出版者・発行元：日本臨床血液学会  

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記述言語：日本語   出版者・発行元：がんの子供を守る会  

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その他リンク： https://projects.repo.nii.ac.jp/?action=repository_uri&item_id=261491

記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：AMER SOC HEMATOLOGY  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：AMER SOC HEMATOLOGY  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

悪性腫瘍12例(急性リンパ性白血病6例,悪性リンパ腫4例,急性骨髄性白血病,骨肉腫各1例,男児4名,女児8名・年齢中央値8歳)を対象に,全例に多剤併用化学療法を行い,1回目(平均14.5ヵ月後)と2回目(平均36.0ヵ月後)に分けて,この化学療法における晩期腎障害(尿細管分泌障害)の経時的変化について検討した.その結果,糸球体機能では2回とも全例で異常は認めなかった.近位尿細管機能ではパラアミノ馬尿酸(PAH)クリアランスは1回目で11例(92%)が正常値以下であり,2回目で10例(83%)が上昇したが,正常範囲までの上昇は11例中5例(45%)であった.遠位尿細管機能では尿比重で1例が1回目低下で2回目に正常化し,尿pHは2回とも異常は認めなかった.以上,これらのことからも化学療法3年後でも尿細管分泌機能障害が存在し,薬物の排泄遅延や相互作用による過度の血中濃度上昇に留意すべきと考えられた

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：AMER SOC HEMATOLOGY  

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記述言語：日本語   出版者・発行元：(株)日本小児医事出版社  

1歳0ヵ月女児.イレウス症状を繰り返した腸リンパ管拡張症の症例であった.検査で低アルブミン血症,低グロブリン血症,CTでは腸管壁の肥厚と内腔の狭小化,消化管造影で拇指圧痕像を認め,回腸生検で粘膜内のリンパ管拡張を認めた.低脂肪・高蛋白食による食事療法を継続していたが,感冒様症状をきっかけに嘔吐,便秘,腹痛,腹部膨満,間歇的啼泣等がみられた.絶飲食,輸液,浣腸等の保存的治療のみで諸症状は改善した.浮腫,下痢,検査上低蛋白血症等が認められたのは3歳まで2回であった.腸リンパ管拡張症が急性腹症症状を呈する可能性があることを念頭におくべきと考えられた

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2003&ichushi_jid=J00643&link_issn=&doc_id=20030829010006&doc_link_id=%2Fag1snrsd%2F2003%2F005609%2F007%2F1823-1827%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fag1snrsd%2F2003%2F005609%2F007%2F1823-1827%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：日本語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：日本語   出版者・発行元：(NPO)日本小児がん学会  

2歳女児.頭蓋骨の棘状骨反応および骨髄で幼若血液細胞の増生と稀な初発所見を呈した全身転移性神経芽腫例で造血器腫瘍を疑い頭蓋骨生検で神経芽腫と診断され,化学療法,二期的原発巣摘出術,放射線照射,2度の末梢血幹細胞移植を施行し,腫瘍マーカーの正常化および画像上の改善を認めた.その後13-シス-レチノイン酸の内服を行い現在経過観察中である

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：英語   掲載種別：速報，短報，研究ノート等（学術雑誌）  

DOI： 10.1097/00043426-200101000-00020

Scopus

PubMed

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記述言語：日本語   出版者・発行元：(株)日本小児医事出版社  

ランゲルハンス細胞組織球症(LCH)の経過中,病勢増悪時,尿中ナトリウムカリウム(Na/K)比の逆転を認めていた患児が約1年後に尿崩症を発症した.尿崩症の顕在化以前に,LCH病勢上昇時にLCHの下垂体の侵襲により潜在性の尿崩症状態を生じ,その反応としてレニン-アンギオテンシン-アルドステロン系の反応で尿細管におけるナトリウム再吸収が増し,尿中Na/K比の逆転が生じたと推測した.尿中Na/K比の逆転はMRIの所見のない状態で生じており,一つの尿崩症発症の予知マーカーであった可能性が示唆された

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記述言語：日本語   出版者・発行元：新潟医学会  

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2000&ichushi_jid=J00990&link_issn=&doc_id=20001117010020&doc_link_id=%2Fdg3nigta%2F2000%2F011409%2F020%2F0363-0363%26dl%3D2&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdg3nigta%2F2000%2F011409%2F020%2F0363-0363%26dl%3D2&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_5.gif

記述言語：日本語   出版者・発行元：日本小児血液学会  

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記述言語：英語   出版者・発行元：BLACKWELL SCIENCE ASIA  

Web of Science

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記述言語：日本語   出版者・発行元：新潟医学会  

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=1999&ichushi_jid=J00990&link_issn=&doc_id=19990817500050&doc_link_id=%2Fdg3nigta%2F1999%2F011305%2F051%2F0293-0293%26dl%3D2&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdg3nigta%2F1999%2F011305%2F051%2F0293-0293%26dl%3D2&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_5.gif

記述言語：日本語   出版者・発行元：(株)日本小児医事出版社  

症例1(11歳男)はカテコラミン心筋炎の症状が著明で,高血圧を示さなかったため診断に苦慮した.症例2(5歳男)は症例1の弟で,本邦において最年少の発症例と思われる.兄から1年後の発症であったが,高血圧を示したことと,家族歴から診断に速やかに至った.家族性褐色細胞腫は孤発例に比してその発症年齢は有意に低く,小児期発症の褐色細胞腫を経験した際には,特にその家族への検索を怠ってはならないと痛感された

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記述言語：日本語   出版者・発行元：(一社)日本血液学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：長岡赤十字病院  

29歳,タイ国人,出産は1996年6月15日,中絶は98年3月17日に行われた.この際,CDリンパ球数は妊娠中に減少したが分娩により無治療で回復した.この期間中にCD4は感冒によっても減少し,感冒の治癒後回復した.本例では3回のエピソードにおいて,CD4は500/μl以下に減少し,無治療で回復した.このことより,抗HIV療法は,CD4が500/μl以下に減少しても,それがHIVの増殖によることを確認した上で始められるべきであると考えられる

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記述言語：日本語   出版者・発行元：新潟医学会  

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=1998&ichushi_jid=J00990&link_issn=&doc_id=19990202480125&doc_link_id=%2Fdg3nigta%2F1998%2F011208%2F125%2F0510-0510%26dl%3D2&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdg3nigta%2F1998%2F011208%2F125%2F0510-0510%26dl%3D2&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_5.gif

記述言語：日本語   出版者・発行元：(株)日本小児医事出版社  

出生直後より腹部膨満,水様性下痢を認め,著明な電解質異常を伴った先天性クロール下痢症の1女児例を報告した.NaCl及びカリウム製剤内服で血清電解質は正常範囲を維持しているが,早期治療したにも関わらず体重増加不良を認める.本疾患は一生涯,下痢が続き,慢性の脱水を伴いやすく,腎障害を合併する可能性がある

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記述言語：日本語   出版者・発行元：(株)日本小児医事出版社  

症例はアプガースコア5点(1分値)の新生児仮死で出生,2生日に左半身けいれんで発症し,入院時の頭部CTで右側脳室前角近傍に境界明瞭な低吸収域が認められ,前大脳動脈穿通枝領域の脳梗塞と診断された.7生日の頭部CTで出血性梗塞が確認されたが,入院後の経過は良好であり,1歳10ヵ月の時点で片麻痺を認めず,精神運動発達は正常範囲内である.抗けいれん剤は発症早期のみ投与し,中止後現在までけいれんの再発はない

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記述言語：日本語   出版者・発行元：長岡赤十字病院  

2生日女児,アプガースコア5点の新生児仮死で出生した.2生日に左半身痙攣が発現し,入院時の頭部CTで右側脳室前角近傍に境界明瞭な低吸収域を認め,前大脳動脈穿通枝領域の脳梗塞と診断した.7生日の頭部CTで出血性梗塞を確認した.入院後の経過は良好であり,1歳10ヵ月の時点で片麻痺を認めず,精神運動発達は正常範囲内にある.抗痙攣剤は発症早期にのみ投与し,中止後現在まで痙攣の再発はない

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記述言語：日本語   出版者・発行元：新潟医学会  

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その他リンク： http://search.jamas.or.jp/link/ui/1998166790

記述言語：日本語   出版者・発行元：新潟医学会  

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その他リンク： http://search.jamas.or.jp/link/ui/1998067081

記述言語：日本語   出版者・発行元：(一社)日本小児アレルギー学会  

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記述言語：日本語   出版者・発行元：(公社)日本新生児成育医学会  

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記述言語：日本語   出版者・発行元：新潟医学会  

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=1996&ichushi_jid=J00990&link_issn=&doc_id=19961023090012&doc_link_id=%2Fdg3nigta%2F1996%2F011007%2F012%2F0296-0296%26dl%3D2&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdg3nigta%2F1996%2F011007%2F012%2F0296-0296%26dl%3D2&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_5.gif

記述言語：日本語   出版者・発行元：(一社)日本周産期・新生児医学会  

完全重複腎盂尿管に合併し,正中を越え対側にまで及ぶ巨大尿管を有した極低出生体重児の1例を経験した.本例は脂肪脊髄髄膜瘤を合併し,出生後早期にウィルソン・ミキティ症候群を発症し34日間の人工呼吸管理を必要とした.本例では全身状態の不良から,出生早期には十分な画像検査が施行できず,確定診断には時間を要した.腹部正中を越える巨大尿管は文献的にも報告がなく,本例の臨床経過と診断過程について報告した

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記述言語：日本語   出版者・発行元：新潟医学会  

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記述言語：日本語  

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記述言語：日本語   出版者・発行元：(一社)日本小児アレルギー学会  

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記述言語：日本語  

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記述言語：日本語  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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