MATSUI Akira

Faculty & Position:General Education in Graduate School  affiliate professor
Contact:matsui-a[at]slcn.ac.jp convert [at] to @
URL: 
Last Updated: May 22, 2019 at 05:08

Researcher Profile & Settings

Qualification

  • 医学博士

Research Activities

Research Areas

  • Clinical internal medicine / Pediatrics

Research Interests

    , HCV-RNA

Published Papers

  • 【小児にかかわる診療ガイドライン】 胆道閉鎖症診療ガイドライン
    安藤 久實, 猪股 裕紀洋, 岩中 督, 黒田 達夫, 仁尾 正記, 松井 陽, 吉田 雅博, 佐々木 英之
    小児外科 49(8) 783-786 Aug. 2017
  • C型肝炎ウイルス持続感染を伴う再発急性骨髄性白血病の小児に対する化学療法の経験
    木村 俊介, 平林 真介, 長谷川 大輔, 角谷 和歌子, 瀬谷 恵, 松井 俊大, 吉本 優里, 細谷 要介, 森 信好, 松井 陽, 真部 淳
    臨床血液 58(6) 619-623 Jun. 2017
    症例は初発時8歳のモンゴル人女児。モンゴルの病院にて急性骨髄性白血病(AML)と診断され,BFM-AML2004 SRに準じた治療をうけた。完全寛解が得られるも,治療薬の供給不足のため維持療法を除く4コースで中断していた。診断後7ヵ月に来日した際にC型肝炎ウイルス(HCV)感染が判明した。診断後11ヵ月に骨髄再発をきたし,その際RUNX1-RUNX1T1キメラ遺伝子が検出された。初発時の治療強度不足とHCV感染を考慮し造血細胞移植ではなく化学療法を選択した。寛解導入療法終了後に寛解を確認し,キメラmRNAも消失した。治療に伴う骨髄抑制によりHCVウイルス量は増加し,骨髄回復期に一過性の肝逸脱酵素の上昇がみられた。全治療経過中,HCVの再活性化はみられなかった。第2再発のリスクが高く,慢性肝炎は肝臓の線維化や肝硬変に進行する可能性があり,AML治療終了後早期のHCVの治療が必要と考えられた。(著者抄録)
  • Stool color card screening for early detection of biliary atresia and long-term native liver survival: a 19-year cohort study in Japan.
    Gu YH, Yokoyama K, Mizuta K, Tsuchioka T, Kudo T, Sasaki H, Nio M, Tang J, Ohkubo T, Matsui A
    The Journal of pediatrics 166(4) 897-902.e1 Apr. 2015 [Refereed]
  • Hepatocyte transplantation using a living donor reduced graft in a baby with ornithine transcarbamylase deficiency: a novel source of hepatocytes.
    Enosawa S, Horikawa R, Yamamoto A, Sakamoto S, Shigeta T, Nosaka S, Fujimoto J, Nakazawa A, Tanoue A, Nakamura K, Umezawa A, Matsubara Y, Matsui A, Kasahara M
    Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society 20(3) 391-393 Mar. 2014 [Refereed]
  • Drug development: The use of unlicensed/off-label medicines in pediatrics.
    Yamashiro Y, Martin J, Gazarian M, Kling S, Nakamura H, Matsui A, Cucchiara S, Aloi M, Wynn EL, Mulberg AE
    Journal of pediatric gastroenterology and nutrition 55(5) 506-510 Nov. 2012 [Refereed]
  • Fatal pulmonary veno-occlusive disease after chemotherapy for Burkitt's lymphoma.
    Miyata D, Fukushima T, Matsunaga M, Saito N, Kato Y, Takahashi-Igari M, Horigome H, Sumazaki R, Yamaki S, Matsui A
    Pediatrics international : official journal of the Japan Pediatric Society 53(3) 403-405 Jun. 2011 [Refereed]
  • Successful pregnancy in a female patient with congenital chloride diarrhea (CLD) and renal impairment.
    Shimizu Y, Kamoda T, Nagata M, Yoh K, Hashimoto Y, Matsui A, Yoshikawa H, Yamagata K, Koyama A
    Journal of nephrology 22(6) 809-813 Nov. 2009 [Refereed]
  • Fatal sibling cases of familial hemophagocytic lymphohistiocytosis (FHL) with MUNC13-4 mutations: case reports.
    Nakao T, Shimizu T, Fukushima T, Saito M, Okamoto M, Sugiura M, Yamamoto K, Ueda I, Imashuku S, Kobayashi C, Koike K, Tsuchida M, Sumazaki R, Matsui A
    Pediatric hematology and oncology 25(3) 171-180 Apr. 2008 [Refereed]
  • Guidelines for care of pregnant women carrying hepatitis C virus and their infants.
    Shiraki K, Ohto H, Inaba N, Fujisawa T, Tajiri H, Kanzaki S, Matsui A, Morishima T, Goto K, Kimura A, Hino S
    Pediatrics international : official journal of the Japan Pediatric Society 50(1) 138-140 Feb. 2008 [Refereed]
  • Serum resistin concentrations in growth hormone-deficient children during growth hormone replacement therapy.
    Nozue H, Kamoda T, Matsui A
    Metabolism: clinical and experimental 56(11) 1514-1517 Nov. 2007 [Refereed]
  • Effects of diet with or without exercise on leptin and anticoagulation proteins levels in obesity.
    Murakami T, Horigome H, Tanaka K, Nakata Y, Katayama Y, Matsui A
    Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis 18(5) 389-394 Jul. 2007 [Refereed]
  • Serum levels of adiponectin and IGFBP-1 in short children born small for gestational age.
    Kamoda T, Nozue H, Matsui A
    Clinical endocrinology 66(2) 290-294 Feb. 2007 [Refereed]
  • Impact of weight reduction on production of platelet-derived microparticles and fibrinolytic parameters in obesity.
    Murakami T, Horigome H, Tanaka K, Nakata Y, Ohkawara K, Katayama Y, Matsui A
    Thrombosis research 119(1) 45-53 2007 [Refereed]
  • Prolonged use of dexmedetomidine in an infant with respiratory failure following living donor liver transplantation.
    Enomoto Y, Kudo T, Saito T, Hori T, Kaneko M, Matsui A, Mizutani T
    Paediatric anaesthesia 16(12) 1285-1288 Dec. 2006 [Refereed]
  • A pediatric case of critical illness polyneuropathy: clinical and pathological findings.
    Ohto T, Iwasaki N, Ohkoshi N, Aoki T, Ichinohe M, Tanaka R, Moriyama N, Ieshima A, Kuwajima K, Matsui A
    Brain & development 27(7) 535-538 Oct. 2005 [Refereed]
  • A case of Shwachman-Diamond syndrome presenting with diabetes from early infancy.
    Kamoda T, Saito T, Kinugasa H, Iwasaki N, Sumazaki R, Mouri Y, Izumi I, Hirano T, Matsui A
    Diabetes care 28(6) 1508-1509 Jun. 2005 [Refereed]
  • Fatal eosinophilic heart disease in a child with neurofibromatosis-1 complicated by acute lymphoblastic leukemia.
    Horigome H, Sumazaki R, Iwasaki N, Imoto N, Kinugasa H, Saito M, Matsui A
    Heart and vessels 20(3) 120-122 May 2005 [Refereed]
  • Diphenylarsinic acid poisoning from chemical weapons in Kamisu, Japan.
    Ishii K, Tamaoka A, Otsuka F, Iwasaki N, Shin K, Matsui A, Endo G, Kumagai Y, Ishii T, Shoji S, Ogata T, Ishizaki M, Doi M, Shimojo N
    Annals of neurology 56(5) 741-745 Nov. 2004 [Refereed]
  • Clinical heterogeneity of neonatal intrahepatic cholestasis caused by citrin deficiency: case reports from 16 patients.
    Tazawa Y, Kobayashi K, Abukawa D, Nagata I, Maisawa S, Sumazaki R, Iizuka T, Hosoda Y, Okamoto M, Murakami J, Kaji S, Tabata A, Lu YB, Sakamoto O, Matsui A, Kanzaki S, Takada G, Saheki T, Iinuma K, Ohura T
    Molecular genetics and metabolism 83(3) 213-219 Nov. 2004 [Refereed]
  • Serum adiponectin concentrations in newborn infants in early postnatal life.
    Kamoda T, Saitoh H, Saito M, Sugiura M, Matsui A
    Pediatric research 56(5) 690-693 Nov. 2004 [Refereed]
  • Resistance to parathyroid hormone in two patients with familial intrahepatic cholestasis: possible involvement of the ATP8B1 gene in calcium regulation via parathyroid hormone.
    Nagasaka H, Yorifuji T, Kosugiyama K, Egawa H, Kawai M, Murayama K, Hasegawa M, Sumazaki R, Tsubaki J, Kikuta H, Matsui A, Tanaka K, Matsuura N, Kobayashi K
    Journal of pediatric gastroenterology and nutrition 39(4) 404-409 Oct. 2004 [Refereed]
  • A case of listerial meningitis treated with a regimen containing panipenem-betamipron.
    Hitomi S, Ohto T, Okamoto M, Nishimura Y, Iwasaki N, Matsui A
    Journal of infection and chemotherapy : official journal of the Japan Society of Chemotherapy 10(4) 242-244 Aug. 2004 [Refereed]
  • ADRB2 polymorphisms and asthma susceptibility: transmission disequilibrium test and meta-analysis.
    Migita O, Noguchi E, Jian Z, Shibasaki M, Migita T, Ichikawa K, Matsui A, Arinami T
    International archives of allergy and immunology 134(2) 150-157 Jun. 2004 [Refereed]
  • Life-threatening vocal cord paralysis in a patient with group A xeroderma pigmentosum.
    Ohto T, Iwasaki N, Okubo H, Shin K, Matsui A
    Pediatric neurology 30(3) 222-224 Mar. 2004 [Refereed]
  • Conversion of biliary system to pancreatic tissue in Hes1-deficient mice.
    Sumazaki R, Shiojiri N, Isoyama S, Masu M, Keino-Masu K, Osawa M, Nakauchi H, Kageyama R, Matsui A
    Nature genetics 36(1) 83-87 Jan. 2004 [Refereed]
  • Enhanced expression of MYCN leads to centrosome hyperamplification after DNA damage in neuroblastoma cells.
    Sugihara E, Kanai M, Matsui A, Onodera M, Schwab M, Miwa M
    Oncogene 23(4) 1005-1009 Jan. 2004 [Refereed]
  • Pulmonary veno-occlusive disease associated with partial anomalous pulmonary venous connection.
    Murakami T, Horigome H, Yamaki S, Nakao T, Hiramatsu Y, Matsui A
    Pediatrics international : official journal of the Japan Pediatric Society 45(6) 747-750 Dec. 2003 [Refereed]
  • Tricho-rhino-phalangeal syndrome type I in a Japanese boy.
    Kamoda T, Matsui A
    European journal of pediatrics 162(11) 810-811 Nov. 2003 [Refereed]
  • Ventricular fibrillation during anesthesia in association with J waves in the left precordial leads in a child with coarctation of the aorta.
    Horigome H, Shigeta O, Kuga K, Isobe T, Sakakibara Y, Yamaguchi I, Matsui A
    Journal of electrocardiology 36(4) 339-343 Oct. 2003 [Refereed]
  • Evaluation of hippocampal infolding using magnetic resonance imaging.
    Okada Y, Kato T, Iwai K, Iwasaki N, Ohto T, Matsui A
    Neuroreport 14(10) 1405-1409 Jul. 2003 [Refereed]
  • Failure to find causal mutations in the GABA(A)-receptor gamma2 subunit (GABRG2) gene in Japanese febrile seizure patients.
    Nakayama J, Hamano K, Noguchi E, Horiuchi Y, Iwasaki N, Ohta M, Nakahara S, Naoi T, Matsui A, Arinami T
    Neuroscience letters 343(2) 117-120 Jun. 2003 [Refereed]
  • Electrocardiographic changes in patients with cardiac rhabdomyomas associated with tuberous sclerosis.
    Shiono J, Horigome H, Yasui S, Miyamoto T, Takahashi-Igari M, Iwasaki N, Matsui A
    Cardiology in the young 13(3) 258-263 Jun. 2003 [Refereed]
  • Detection of repolarization abnormalities in patients with cardiomyopathy using current vector mapping technique on magnetocardiogram.
    Shiono J, Horigome H, Matsui A, Terada Y, Miyashita T, Tsukada K
    The international journal of cardiovascular imaging 19(2) 163-170 Apr. 2003 [Refereed]
  • Soluble P-selectin and thrombomodulin-protein C-Protein S pathway in cyanotic congenital heart disease with secondary erythrocytosis.
    Horigome H, Murakami T, Isobe T, Nagasawa T, Matsui A
    Thrombosis research 112(4) 223-227 2003 [Refereed]
  • A nonsense mutation of the MASS1 gene in a family with febrile and afebrile seizures.
    Nakayama J, Fu YH, Clark AM, Nakahara S, Hamano K, Iwasaki N, Matsui A, Arinami T, Ptácek LJ
    Annals of neurology 52(5) 654-657 Nov. 2002 [Refereed]
  • Intravenous prostacyclin combined with inhaled nitric oxide therapy for an infant with alveolar capillary dysplasia.
    Kinugasa H, Horigome H, Sugiura M, Saito T, Iijima T, Matsui A
    Pediatrics international : official journal of the Japan Pediatric Society 44(5) 525-527 Oct. 2002 [Refereed]
  • Failure to find evidence for association between voltage-gated sodium channel gene SCN2A variants and febrile seizures in humans.
    Nakayama J, Yamamoto N, Hamano K, Iwasaki N, Ohta M, Nakahara S, Horigome Y, Nakahara C, Noguchi E, Shiono J, Shimakura Y, Yamakawa-Kobayashi K, Matsui A, Arinami T
    Neuroscience letters 329(2) 249-251 Aug. 2002 [Refereed]
  • Association between TNFA polymorphism and the development of asthma in the Japanese population.
    Noguchi E, Yokouchi Y, Shibasaki M, Inudou M, Nakahara S, Nogami T, Kamioka M, Yamakawa-Kobayashi K, Ichikawa K, Matsui A, Arinami T
    American journal of respiratory and critical care medicine 166(1) 43-46 Jul. 2002 [Refereed]
  • Evaluation of myocardial ischemia in Kawasaki disease using an isointegral map on magnetocardiogram.
    Shiono J, Horigome H, Matsui A, Terada Y, Watanabe S, Miyashita T, Tsukada K
    Pacing and clinical electrophysiology : PACE 25(6) 915-921 Jun. 2002 [Refereed]
  • Overproduction of platelet microparticles in cyanotic congenital heart disease with polycythemia.
    Horigome H, Hiramatsu Y, Shigeta O, Nagasawa T, Matsui A
    Journal of the American College of Cardiology 39(6) 1072-1077 Mar. 2002 [Refereed]
  • Identification of a novel nonlysosomal sulphatase expressed in the floor plate, choroid plexus and cartilage.
    Ohto T, Uchida H, Yamazaki H, Keino-Masu K, Matsui A, Masu M
    Genes to cells : devoted to molecular & cellular mechanisms 7(2) 173-185 Feb. 2002 [Refereed]
  • Molecular genetics of febrile seizures.
    Iwasaki N, Nakayama J, Hamano K, Matsui A, Arinami T
    Epilepsia 43 Suppl 9 32-35 2002 [Refereed]

Conference Activities & Talks

  • 胆道閉鎖症の遺伝因子の解析 エクソーム解析による遺伝要因の探索
    右田 王介, 松井 陽, 松原 洋一, 秦 健一郎
    日本小児外科学会雑誌 Feb. 2017

Misc

  • 【移行支援-小児から成人への橋渡し-】 難治性肝胆道疾患患者の移行支援と継続的ケア
    松井 陽
    保健の科学 59(5) 304-310 May 2017
  • 胆道閉鎖症診療ガイドラインのシステマティックレビューと胆道閉鎖症全国登録データからみた胆道閉鎖症発症に関する知見
    佐々木 英之, 安藤 久實, 猪股 裕紀洋, 岩中 督, 黒田 達夫, 松井 陽, 吉田 雅博, 仁尾 正記, 胆道閉鎖症診療ガイドライン統括委員会
    日本小児外科学会雑誌 53(2) 334-334 Apr. 2017
  • 胆道閉鎖症の発症時期はいつか?小児科臨床医の視点から
    松井 陽
    日本小児外科学会雑誌 53(2) 336-336 Apr. 2017
  • 「エビデンスに基づいた胆道閉鎖症診療ガイドライン」の作成に向けて
    安藤 久實, 吉田 雅博, 仁尾 正記, 松井 陽, 岩中 督, 黒田 達夫, 猪股 裕紀洋, 胆道閉鎖症診療ガイドライン統括委員会
    日本小児外科学会雑誌 53(1) 154-154 Feb. 2017
  • 胆道閉鎖症の鑑別診断における十二指腸液(DF)採取の工夫
    工藤 豊一郎, 渕本 康史, 伊藤 玲子, 金森 豊, 松井 陽
    日本小児外科学会雑誌 53(1) 156-156 Feb. 2017
  • 乳児胆汁うっ滞性疾患の鑑別診断における十二指腸液(DF)採取の実際
    工藤 豊一郎, 渕本 康史, 金森 豊, 伊藤 玲子, 松井 陽
    日本小児栄養消化器肝臓学会雑誌 30(2) 91-92 Dec. 2016
  • 胆道閉鎖症のスクリーニング事業報告
    松井 陽
    日本小児栄養消化器肝臓学会雑誌 30(2) 99-99 Dec. 2016
  • 小児消化器病学の将来展望 多施設共同研究の推進を願って 難病指定への取り組み 先天性吸収不全症 乳児難治性下痢症
    虫明 聡太郎, 位田 忍, 牛島 高介, 柳 忠宏, 工藤 孝広, 幾瀬 圭, 新井 勝大, 土岐 彰, 米倉 竹夫, 石井 智弘, 永田 公二, 藤井 喜充, 友政 剛, 松井 陽, 玉井 浩, 田口 智章
    日本小児栄養消化器肝臓学会雑誌 30(Suppl.) 45-45 Aug. 2016
  • 厚生労働省難治性疾患政策研究事業田口班研究報告1 先天性吸収不全症の診療ガイドライン確立に向けた全国調査
    位田 忍, 虫明 聡太郎, 牛島 高介, 柳 忠宏, 工藤 孝広, 幾瀬 圭, 永田 公二, 米倉 竹夫, 石井 智弘, 土岐 彰, 新井 勝大, 友政 剛, 松井 陽, 玉井 浩, 藤井 善充, 田口 智章
    日本小児栄養消化器肝臓学会雑誌 30(Suppl.) 80-80 Aug. 2016
  • 厚生労働省難治性疾患政策研究事業田口班研究報告2 Shwachman-Diamond症候群の治療と予後
    工藤 孝広, 幾瀬 圭, 虫明 聡太郎, 位田 忍, 新井 勝大, 石井 智浩, 牛島 高介, 土岐 彰, 永田 公二, 藤井 喜充, 柳 忠宏, 米倉 竹夫, 友政 剛, 松井 陽, 玉井 浩, 田口 智章, 先天吸収不全症研究グループ
    日本小児栄養消化器肝臓学会雑誌 30(Suppl.) 80-80 Aug. 2016
  • 厚生労働省難治性疾患政策研究事業田口班研究報告3 先天性クロール下痢症の治療と予後
    柳 忠宏, 牛島 高介, 虫明 聡太郎, 位田 忍, 工藤 孝広, 幾瀬 圭, 新井 勝大, 石井 智弘, 米倉 竹夫, 土岐 彰, 永田 公二, 藤井 喜充, 友政 剛, 松井 陽, 玉井 浩, 田口 智章, 先天吸収不全症研究グループ
    日本小児栄養消化器肝臓学会雑誌 30(Suppl.) 81-81 Aug. 2016
  • 【小児慢性疾患の成人期移行の現状と問題点】 慢性消化器疾患 肝胆道疾患における成人期移行の現状と問題点 胆道閉鎖症および先天性胆道拡張症
    松井 陽
    小児科臨床 69(4) 743-748 Apr. 2016
  • 免疫の関与が疑われた小児肝障害患者におけるフローサイトメトリーを用いたリンパ球解析
    伊藤 玲子, 渡辺 信之, 内山 徹, 工藤 豊一郎, 福田 晃也, 義岡 孝子, 中澤 温子, 松井 陽, 笠原 群生, 今留 謙一
    肝臓 57(Suppl.1) A422-A422 Apr. 2016
  • 胆道閉鎖症の診断・治療の標準化に向けて 小児 胆道閉鎖症の診断について
    工藤 豊一郎, 松井 陽
    日本外科学会定期学術集会抄録集 115回 SY-9 Apr. 2015
  • Screening for Biliary Atresia : The Meaning of a Stool Colour Card Bound in a Pocket-book for the Health of Mothers and Children
    MATSUI Akira
    小児保健研究 71(6) 795-799 Nov. 2012
  • Two Pediatric Cases of Deceased Donor Split Liver Transplantation
    KAKIUCHI Toshihiko, KASAHARA Mureo, SAKAMOTO Seisuke, KANAZAWA Hiroyuki, KARAKI Chiaki, SHIGETA Takanobu, FUKUDA Akinari, NAKAGAWA Satoshi, NAKAZAWA Atsuko, MATSUI Akira
    日本小児科学会雑誌 116(6) 995-998 Jun. 2012
  • A case of Primary sclerosing Cholangitis with factous of Autoimmune Hepatitis in Childhood
    KAKIUCHI Toshihiko, ARAI Katsuhiro, KOINUMA Sachi, SHIMIZU Hirotaka, YANAGI Tadahiro, IGUCHI Umefumi, NAKAGAWA Atsuko, MATSUI Akira
    日本小児栄養消化器肝臓学会雑誌 = Japanese journal of pediatric gastroenterology, hepatology and nutrition 25(2) 63-68 Dec. 2011
  • Japanese pediatric clinical network
    栗山 猛, 松井 陽
    医学のあゆみ 238(2) 201-207 Jul. 2011
  • Diagnosis and Prediction of Fulminant Prognosis in Pediatric Acute Liver Failure : Investigations of Clinical Symptoms and Laboratory Data of 135 Cases by the Pediatric Acute Liver Failure Study Group in Japan
    MUSHIAKE Sotaro, BESSHO Kazuhiko, IDA Shinobu, SUMAZAKI Ryou, INUI Ayano, NAGATA Ikuo, MATSUI Akira
    The Journal of the Japan Pediatric Society 114(1) 64-72 Jan. 2010
  • Acute Liver Failure in Japanese Children : Findings of the Acute Liver Failure Study Group in the Japanese Pediatric Hepatology Research Association
    INUI Ayano, IDA Shinobu, SUMAZAKI Ryo, NAGATA Ikuo, MATSUI Akira, MUSHIAKE Soutaro, FUJISAWA Tomoo
    日本腹部救急医学会雑誌 = Journal of abdominal emergency medicine 29(4) 583-589 May 2009
  • Acute Liver Failure in Japanese Children : Findings of the Acute Liver Failure Study Group in the Japanese Pediatric Hepatology Research Association:: Findings of the Acute Liver Failure Study Group in the Japanese Pediatric Hepatology Research Associati
    Inui Ayano, Ida Shinobu, Sumazaki Ryo, Nagata Ikuo, Matsui Akira, Mushiake Soutaro, Fujisawa Tomoo
    Progress in Acute Abdominal Medicine 29(4) 583-589 2009
    To clarify the outcome of Japanese children with acute liver failure (severe acute liver failure and fulminant liver failure), a retrospective multicenter case study was performed from 1995 to 2005. One hundred and thirty five children were enrolled. Thirty cases were categorized into severe acute liver failure and fulminant liver failure accounted for 105 cases. The cause of acute liver failure included metabolic disease (25%), viral infection (22%), drug-related hepatotoxicity (8%), autoimmune liver disease (2%) ; and indeterminate in 43%. The survival rate has been improved from 30.7% to 69% for those who underwent liver transplantation. Even after undergoing liver transplantation, the outcome of children under one year of age was still not good. The high percentage of indeterminate cases should provide a good incentive for investigation throughout Japan.
  • The unification of a long-term follow-up system for infants detected by neonatal screening and a system for quality control and quality assurance of screening programs
    原田 正平, 加藤 忠明, 松井 陽
    ホルモンと臨床 56(12) 1235-1239 Dec. 2008
  • What can we do to prevent collapse in pediatric medical system in Japan?
    松井 陽
    Health science for children 9(1) 3-5[含 英語文要旨] Nov. 2008
  • A Case of the Infantile Tetanus
    YOSHIMATSU Shoji, HASEGAWA Makoto, KATO Keisuke, FUJIYAMA Satoshi, MUROFUSHI Wataru, SAITO Takashi, TANAKA Ryuta, MATSUI Akira
    The Journal of the Japan Pediatric Society 112(9) 1386-1389 Sep. 2008
  • Experience of Pediatric Living-Donor Liver Transplantation in National Center for Child Health and Development
    Kasahara Mureo, Fukuda Akinari, Sato Syuichi, Shigeta Takanobu, Sasaki Kazunari, Nakazato Yayoi, Tanaka Hideaki, Kuroda Tatsuo, Honna Toshiro, Matsui Akira
    Journal of the Japanese Society of Pediatric Surgeons 44(5) 679-688 Aug. 2008
    Purpose: We reviewed our initial experience of pediatric living-donor liver transplantation (LDLT) in National Center for Child Health and Development and report the results herein. Subjects and methods: We have done 48 cases of LDLT during November 2005 through February 2008 in National Center for Child Health and Development. Variables including surgical/infectious/immunological outcome were reviewed. Results: The indication for liver transplantation was biliary atresia in 41.6%, followed by metabolic liver disease (29.2%) and fulminant hepatic failure (18.8%). The mean operative time and blood loss were 5 hr 31 min, 281 ml in donors and 9 hr 46 min, 139 ml/kg in recipients. Immunosuppression consisted of tacrolimus and low-dose steroids. The graft and patient survival were 90.7%. Conclusion: Satisfactory results can be achieved in the LDLT program in National Center for Child Health and Development. Long-term observation may be necessary to collect sufficient data for the establishment of the treatment modality.
  • PD-031 Late-onset portal vein thrombosis after pediatric liver transplantation(Organ transplantation : Liver transplantation-Problems of long-term results, the 44th Annual Meeting of Japanese Society of Pediatric Surgeons)
    Hori Tetsuo
    Journal of the Japanese Society of Pediatric Surgeons 43(3) May 2007
  • PD-040 Fulminant hepatic failure in childhood in Japan(Organ transplantation : Fulminant hepatic failure in children, the 44th Annual Meeting of Japanese Society of Pediatric Surgeons)
    Inui Ayano
    Journal of the Japanese Society of Pediatric Surgeons 43(3) May 2007
  • A case of persistent hyperinsulinemic hypoglycemia of infancy treated with diazoxide and cornstarch
    IWASAKI Yoko, KAMODA Tomohiro, TAKIZAWA Fumihiko, ONO Toshiaki, OHKUBO Kumiko, MATSUI Akira
    ホルモンと臨牀 54(11) 1037-1041 Nov. 2006
  • P-64 Epilepsy of a case with juvenile Huntington's disease(The 40th Congress of the Japan Epilepsy Society)
    田中 竜太, 大戸 達之, 齊藤 貴志, 岩崎 信明, 松井 陽
    Journal of the Japan Epilepsy Society 24(3) Aug. 2006
  • Infantile liver disease: Carry-over to the adult. 3. Biliary tract disease.
    MATSUI Akira
    Kanzo 47(2) 83-85 Feb. 2006
  • Round-table discussion: "carry over" of liver disease in children to adulthood
    Fujisawa Tomoo, Kage Masayoshi, Matsui Akira, Mishiro Shunji, Shiraki Kazuo, Tanaka Koichi, Tazawa Yusaku
    Kanzo 47(2) 51-69 Feb. 2006
  • Elevated BNP is a useful predictor of cardiac dysfunction during treatment of _<98>A_3 protocol in two patients with stage 4 neuroblastoma
    ENOMOTO Yuki, FUKUSHIMA Takashi, NAKAO Tomohei, MIYATA Daiki, NISHIMURA Yoko, OKAMOTO Miho, TAKAHASHI Miho, SHIMIZU Takashi, HIRAI Misako, KANEKO Michio, MATSUI Akira
    Pediatric oncology 42(2) 244-248 Oct. 2005
    _<98>A_3プロトコールを遂行する上で心毒性は大きな制約条件となりえる.BNPは心不全のマーカーとしてだけでなく心毒性の早期評価にも有効である.我々が経験した2例において,進行神経芽腫に対する_<98>A_3プロトコールの遂行には,心筋毒性の早期評価が重要であり,心電図・心エコー検査に加えてBNPの定期的な測定と,小児循環器専門家との密接な共同診療が必須であった.
  • A Clinical Trial of a Gene Therapy to Regulate the Graft versus Host Disease after Allogeneic Hematopoietic Stem Cell Transplantation
    FUKUSHIMA Takashi, SHIMIZU Takashi, SUMAZAKI Ryo, OTSU Makoto, ONODERA Masafumi, KOIKE Kazutoshi, TSUCHIDA Masahiro, KATO Shun-ichi, NAKAUCHI Hiromitsu, OHASHI Kazuteru, SAKAMAKI Hisashi, KANEKO Shin, SUZUKAWA Kazumi, HASEGAWA Yuichi, KOJIMA Hiroshi, NAGASAWA Toshiro, MATSUI Akira
    日本小児血液学会雑誌 19(2) 101-108 Apr. 2005
  • A Clinical Trial of a Gene Therapy to Regulate the Graft versus Host Disease after Allogeneic Hematopoietic Stem Cell Transplantation
    FUKUSHIMA Takashi, SHIMIZU Takashi, SUMAZAKI Ryo, OTSU Makoto, ONODERA Masafumi, KOIKE Kazutoshi, TSUCHIDA Masahiro, KATO Shun-ichi, NAKAUCHI Hiromitsu, OHASHI Kazuteru, SAKAMAKI Hisashi, KANEKO Shin, SUZUKAWA Kazumi, HASEGAWA Yuichi, KOJIMA Hiroshi, NAGASAWA Toshiro, MATSUI Akira
    J.J.P.H. 19(2) 101-108 2005
    In allogeneic hematopoietic transplantation, donor lymphocytes have important effects on both immunological reconstitution and graft versus leukemia effect. While we can treat leukemia relapsed after a stem cell transplantation by donor lymphocyte infusion (DLI), the indication of DLI is limited by the risk of graft versus host disease (GVHD). A clinical trial was started in the 1990s in Italy and was continued in Europe, which is a suicide gene therapy for a treatment of GVHD after DLI. Herpes simplex virus thymidine kinase (HSV-TK) was used as a suicide enzyme. A human cell does not have HSV-TK, and gancyclovir has no effect on it. Gancyclovir can be activated in the cell and expresses a cytotoxic effect after HSV-TK is transduced into a genome of a human cell. In our HSV-TK DLI project, we use HSV-TK transduced donor lymphocytes for DLI, and GVHD can be controled by gancyclovir-induced elimination of the transduced donor lymphocytes. In November of 2004, we carried out the HSV-TK DLI for the first case of our clinical protocol. It should be expanded to more patients for analysis of safety, efficacy and clinical contribution of this treatment.
  • A case of hepatoblastoma complicated by Fanconi's syndrome and hemi-renal atrophy after high dose chemotherapy with autologous bone marrow transplantation
    MATSUNAGA Maki, FUKUSHIMA Takashi, SHIMIZU Takashi, NISHIMURA Yoko, MIYATA Daiki, NAKAO Tomohei, HIRAI Misako, KANEKO Michio, MATSUI Akira
    小児がん 41(4) 850-853 Dec. 2004
  • Recurrence of hepatocellular carcinoma 5 years after liver transplantation in a child with biliary atresia and hepatocellular carcinoma
    HORI Tetsuo, KANEKO Michio, IKEBUKURO Kenichi, KOMURO Hiroaki, HIRAI Misako, MATSUI Akira, IIJIMA Tatsuo, KAWASAKI Seiji
    小児がん 41(4) 854-858 Dec. 2004
  • Usefulness of molecular diagnosis, and efficacy of a regimen without cisplatin/carboplatin in an intial managemnet of advanced neuroblastoma ; a case report
    FUKUSHIMA Takashi, FUJIWARA Nobuko, NAKAJIMA Ryoko, HASUMI Hiroki, MIYAZAKI Kenji, MATOBA Kimio, HIRAI Misako, IWAKAWA Mayumi, SUMAZAKI Ryo, MATSUI Akira, KANEKO Michio
    小児がん 40(4) 578-581 Dec. 2003
  • Two cases of epipharyngeal carcinoma
    NAKAO Tomohei, FUKUSHIMA Takashi, IWASAKI Yoko, ISHIZAWA Mika, MATOBA Kimio, IWAKAWA Mayumi, KANEKO Michio, MATSUI Akira
    小児がん 40(4) 600-606 Dec. 2003
  • INVESTIGATION INTO OPTIMAL DOSE OF SUSTAINED-RELEASE THEOPHYLLINE PREPARATION (THEODUR DRY SYRUP^【○!R】) IN PEDIATRIC BRONCHIAL ASTHMA
    ICHINOHE Mika, ICHIKAWA Kunio, IMAI Hironori, MATSUI Akira
    JJACI 17(5) 530-535 Dec. 2003
    The optimal dose of a sustained-release theophylline preparation (Theodur dry syrup®) was investigated. Theodur dry syrup® was administered orally for at least one week to 150 children with bronchial asthma in ages ranging from six months to twelve years (median: 4.5 years), and serum concentrations of theophylline were measured between 3 and 5 hours after dosing. The results showed that, in children between six and eleven months of age, serum levels of theophylline were above 10μg/ml in some cases, even when the daily dose was less than 8mg/kg, and thus, the initial daily dose for children in this age group should be slightly lower. In one-year-old children, the serum concentration of theophylline did not exceed the target range of 5-10μg/ml in any patients when the daily dose was less than 11mg/kg. Therefore, the recommended initial daily dose of 8-10mg/kg to children in this age group seems appropriate. In children two years of age and older, the results suggest that it is necessary to start administration at a daily dose of 10mg/kg and then to increase the dose while monitoring theophylline concentration in the serum. The C/D ratio (serum concentration/dose) for children younger than one year of age was higher than that for children in the other age groups, and the degree of interindividual variations was also greater. Taken together, these findings suggest that it will be necessary to stratify the administration of sustained-release theophylline for different age groups.
  • A Case of Infantile Epileptic Apnea with Congenital Brain Anomaly
    OHTO Tatsuyuki, IWASAKI Nobuaki, TAKAHASHI Miho, TANAKA Ryuta, HAMANO Kenzo, MATSUI Akira
    脳と発達 35(6) 511-514 Nov. 2003
  • Accelerated idioventricular rhythm in neonates
    MURAKAMI Takashi, HORIGOME Hitoshi, SHIONO Junko, TAKAHASHI Miho, HASUMI Hiroki, SUGIURA Masatoshi, MATSUI Akira
    日本未熟児新生児学会雑誌 = Journal of Japan Society for Premature and Newborn Medicine 15(1) 99-105 Feb. 2003
  • A Case of Infantile Epileptic Apnea with Congenital Brain Anomaly
    Ohto Tatsuyuki, Iwasaki Nobuaki, Takahashi Miho, Tanaka Ryuta, Hamano Kenzo, Matsui Akira
    NO TO HATTATSU 35(6) 511-514 2003
    Two months-old girl with psychomotor retardation had aminophylline-resistant apnea attacks and was investigated by video-EEG recording. She had hypogenesis of cerebral cortex and cerebellum and complete agenesis of corpus callosum. Left hemispheric 2 Hz rhythmic δ wave burst originating from the posterior temporal area lasted about 20 seconds, and was followed by an apnea attack persisting for 30 seconds. During the apnea attack, the basic activity of EEG was suppressed. The diagnosis of epileptic apnea was made, and the attacks were controlled with valproate sodium. Reports of cases of brain anomaly presenting with epileptic apnea are rare and this interesting case provided a clue to the pathomechanism of this condition.
  • Mass-screening for biliary atresia in Sapporo city
    MIZUSHIMA Yoshikiyo, ARAI Osamu, HANAI Junji, FUJITA Kozo, HATTORI Sachiko, TACHI Mutsuko, TATEISHI Munetaka, GOTO Shiro, SASAKI Fumiaki, MATSUI Akira
    日本マス・スクリーニング学会誌 = Journal of Japanese Society for Mass-screening 12(1) 23-28 Mar. 2002
  • Mass-screening for biliary atresia in Sapporo city
    MIZUSHIMA Y.
    日本マス・スクリーニング学会誌 = Journal of Japanese Society for Mass-screening 11(2) Sep. 2001
  • Case of Down's syndrome with congenital leukemia, hydrops fetalis, and hepatic failure
    岩崎 博樹, 田中 竜太, 福島 敬, 堀米 仁志, 須磨 崎亮, 松井 陽, 浜田 洋実
    日本産婦人科・新生児血液学会誌 = The Japanese journal of obstetrical, gynecological & neonatal hematology 11(1) "S-91"-"S-92" Jun. 2001
  • Regional Variation in Bone Mineral Density and Motor Function in Children with Cerebral palsy
    SHIRAGAKI Jun, IWASAKI Nobuaki, NAKAYAMA Junko, FUJITA Kazuhiro, OHTO Tatsuyuki, MATSUI Akira
    脳と発達 33(1) 37-43 Jan. 2001
  • Regional Variation in Bone Mineral Density and Motor Function in Children with Cerebral Palsy.
    Shiragaki Jun, Iwasaki Nobuaki, Nakayama Junko, Fujita Kazuhiro, Ohto Tatsuyuki, Matsui Akira
    NO TO HATTATSU 33(1) 37-43 2001
    Bone mineral density (BMD) and motor activity of the upper extremities, were studied in 18 children with spastic cerebral palsy (CP group) and 12 age-matched normal boys (control group). The motor activity was monitored by a piezoelectric transducer. Whole body BMD as well as BMD of the head, upper limbs, ribs, spine, pelvis, and lower limbs was evaluated by dual energy x-ray absorptiometry. In the CP group, BMDs were lower compared to control group in the whole body and in all the body regions (except for the head) especially in the pelvis and lower limbs. BMDs of these regions were higher in walking patients than in bed-ridden, rolling, and crawling patients. Motor activities in the upper extremities were lower in the CP than in the control group. BMD increased with developing motor activity in the upper extremities. These results implicate diminished BMDs of the lower limbs and pelvis, which are associated with standing and walking, to the high incidence of femur fractures in CP.
  • Liver involvement in cystic fibrosi : case reports of Japanese patients with cystic fibrosis
    HASEGAWA Makoto, OSAKI Maki, SUMAZAKI Ryo, AMAGAI Yosihiro, OKAWA Haruo, MATSUI Akira
    日本小児栄養消化器病学会雑誌 = Japanese journal of pediatric gastroenterology and nutrition 14(1) 37-43 Apr. 2000
  • BILIARY ATRESIA AND THE DEVELOPING HUMAN BILIARY SYSTEM AT THE PORTA HEPATIS
    Matsui Akira
    Journal of Japan Surgical Society 100(6) 399-402 Jun. 1999
  • Renal glucosuria and membranous glomerulonephritis in chronic inflammatory demyelinating polyradiculoneuropathy: CIDP.
    KANEMOTO Katsuyoshi, NAKAHARA Chieko, SAITOH Hisako, FUKUSHIMA Takashi, KASHIWAGI Ryouichi, TAKAHASHI Miho, IWASAKI Nobuaki, OHKOSHI Norio, NAGATA Michio, KOYAMA Akio, WATANABE Teruo, MATSUI Akira, MATSUI Akira
    Jpn J Nephrol 41(5) 511-516 1999
    Glucosuria was detected in a 7-year-old boy by a routine school mass examination in . The diagnosis of renal glucosuria was made in the affiliated . The patient developed muscle weakness and gait disturbance in . Spinal fluid examination revealed a protein level of 62 mg/dl and a cell count of 4/3. Under the diagnosis of Guillain-Barre syndrome, he was treated with IV immunoglobulin and oral prednisolone. Although the therapy somewhat improved the symptoms, his muscle strength had not fully recovered at the end of the treatment. In the muscle weakness became worse;he could not go up stairs, nor stand upright on one leg. In proteinuria was detected in a school mass examination. He was referred to for a full renal study in Renal biopsy revealed global sclerosis in 16 of 19 glomeruli with extensive interstitial fibrosis and mononuclear cell infiltration. A diagnosis of membranous glomerulonephritis was established based on the findings of spikes in PASM staining, weak IgG deposition in the glomerular capillary and subepithelial deposits by electron microscopic study. Additionally, pituitary growth hormone deficiency was found by endocrinological examination. The diagnosis of CIDP was established by fbulal neuron biopsy, which revealed neuronal degeneration and profound demyelinization. The clinical course of the present case was unlike that of the few reported cases of MGN associated with CIDP described in the literature. The initial renal symptom was glucosuria, which started 5 years prior to the onset of proteinuria. Second, glomerulosclerosis was more extensive than that seen in the literature. We surmise that chronic interstitial nephritis of insidious onset was followed by MGN which developed subsequently, probably at the time of the start of proteinuria.
  • Renal glucosuria and membranous glomerulonephritis in chronic inflammatory demyelinaing polyradiculoneuropathy:CIDP
    KANEMOTO Katsuyoshi, NAKAHARA Chieko, SAITOH Hisako, HUKUSHIMA Takashi, KASHIWAGI Ryouichi, TAKAHASHI Miho, IWASAKI Nobuaki, KAMODA Tomohiro, OHKOSHI Norio, NAGATA Michio, KOYAMA Akio, WATANABE Teruo, MATSUI Akira
    The Japanese journal of nephrology = / 日本腎臓学会 [編集] 41(5) 511-516 Aug. 1998
  • Intravenous Indomethacin Therapy in Premature Infants with Patent Ductus Arteriosus
    SUWA Kiyotaka, HANMA Yoko, IGARASHI Hiroshi, MATSUI Akira, YANAGISAWA Masayoshi, MOMOI Marikoy
    日本新生児学会雑誌 31(4) 894-899 Dec. 1995
  • Radioimmunoassay of Conjugated 1β-Hydroxycholic Acid in Dried Blood Spots for Diagnosis of Congenital Biliary Atresia
    IKEGAWA Shigeo, MURAI Tsuyoshi, MATSUI Akira, TOHMA Masahiko
    Biological & pharmaceutical bulletin 17(1) 5-8 Jan. 1994
    Radioimmunological measurement of conjugated 1β-hydroxycholic acid in dried blood spots obtained from newborns at 4-5 d after birth has been carried out to investigate a diagnostic utility for congenital biliary atresia. The method allowed the determination of this bile acid, with a detection limit of 50 pg in one disc (3 mm i.d.), and showed good reproducibility with inter- and intra-assay coefficients with variations of 3.5-7.7% and 6.3-8.8%. With the analysis, a relatively high concentration of this substance was observed in the patients, as compared to normal subjects, averaging 1.63 ng/disc in the former and 0.97 ng/disc in the latter.
  • ELISA (Enzyme-Linked Immunosorbent Assay) for Glycocholic Acid in Neonatal Blood Spotted on Filter Paper
    Ikeda Hiromi, Ohkuma Hiroshi, Maeda Masako, Tsuji Akio, Matsui Akira, Kanbegawa Akira
    Ikagaku Shinpojumu 21(2) 86-91 1992
    We have developed an enzyme-linked immunosorbent assay for glycocholic acid in dried blood spotted on a filter paper.
    The linear range of this assay was 1 to 32 nmol/ml and the coefficient of variation of within and between the assay range was 2.6-8.9% and 3.4-8.2%, respectively.
    The average concentration of the glycocholic acid value in normal newborn babies was 8.25 nmol/ml, and that in babies with congenital biliary atresia (CBA) was 49.26nmol/ml (n=24).
    This method will be applicable in mass screening for CBA.
  • Truncus arteriosus with DiGeorge syndrome and tetralogy of Fallot with myelomeningocele in monozygotic twins
    Endo Hideki, Igarashi Hiroshi, Ichihashi Kou, Kuramatsu Toshihiro, Shiraishi Hirohiko, Yano Sadayuki, Matsui Akira, Yamamoto Yoshifumi, Yanagisawa Masayoshi
    Shinzo 21(11) 1305-1308 1989
    DiGeorge症候群は,第3,4鯉弓に由来する器官の発生異常により,テタニー,易感染性,心大血管系奇形,および特異な顔貌を呈する.今回,総動脈幹遺残を伴うDiGorge症候群とFallot四徴および脊髄髄膜瘤を伴った一卵性双胎を経験した.第一子は,総動脈幹遺残で低カルシウム血症性テタニーを呈し細胞性免疫の低下を示した.顔貌は正常であった.肺動脈絞拒術を施行したが,生後81日に死亡した.剖検では,胸腺・副甲状腺は認められなかった.第二子は,Fallot四徴と脊髄髄膜瘤を伴っていたが低カルシウム血症や細胞性免疫の低下は示さなかった.現在,心臓外来で経過観察中である.
    本症例は,DiGeorge症候群の発生を考える上で興味あるものと思われた.
  • Studies on seroconversion from HBeAg to anti-HBe in asymptomatic HBV carrier children.
    FUJIMOTO Takako, TAKAZAWA Yoshie, MATSUI Akira, OKANIWA Mariko, OKAMOTO Hiroaki
    Kanzo 27(1) 6-14 1986
    The natural course of seroconversion from HBeAg to anti-HBe was investigated for 0.5 to 9 years (average, 3.0 years) in 36 HBeAg positive children with serial examination of s-GPT levels, HBeAg and anti-HBe. Of 29 cases initially positive for HBeAg, 19 had remained HBeAg positive, and 10 eventually lost HBeAg, 9 of whom seroconverted to anti-HBe. In 16 (44.4%) of 36 cases, elevations of s-GPT levels were noted. Of 19 children who had remained HBeAg positive, 5 showed transient, slight elevations of s-GPT levels, whereas apparent elevations of s-GPT levels (>200 Karmen units) were noted in 8 carrier children who lost HBeAg, and after anti-HBe appeared, s-GPT levels were normalized. Seroconversion rate was approximately 5% per year in carrier children.
  • Alagille's Syndrome and Intrahepatic Bile Duct Hypolasia
    Matsui Akira, Saito Sumio, Tsuchida Yoshiaki, Honna Toshiro, Shiraki Kazuo
    Journal of the Japanese Society of Pediatric Surgeons 15(5) 769-774 Aug. 1979


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