HAMAZAKI Takashi

写真a

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Title

Professor

Laboratory location

Abeno Campus

Degree 【 display / non-display

  • Osaka University -  Ph.D.(medicine)

Research Areas 【 display / non-display

Pediatrics

Current Career 【 display / non-display

  • Osaka City University   Graduate School of Medicine   Clinical Medicine Course   Professor  

 

Published Papers 【 display / non-display

  • Relationship Between Bedside Ketone Levels and Time to Resolution of Diabetic Ketoacidosis: A Retrospective Cohort Study

    Yuyama Yoshihiko, Kawamura Tomoyuki, Nishikawa-Nakamura Naoko, Hotta Yuko, Hashimura Kayako, Hashimoto Tomomi, Hirose Masakazu, Higashide Takashi, Hamazaki Takashi

    DIABETES THERAPY  12 ( 12 ) 3055 - 3066 2021.12  [Refereed]

     View Summary

    INTRODUCTION: There is no information on the factors that influence the time required to induce resolution of diabetic ketoacidosis (DKA). New methods are currently available for bedside measurement of serum 3-hydroxybutyrate (3HB). The aim of this study was to determine the relationship between serum 3HB and the time to DKA resolution. METHODS: We reviewed the medical records of patients with type 1 diabetes (T1D) and a history of DKA who were admitted to the Department of Pediatrics, Osaka City University Hospital, between November 2008 and October 2018. DKA resolution was defined as 3HB below 1.0 mmol/L as measured by a bedside ketone meter. RESULTS: Data of 52 T1D-DKA episodes were analyzed (median age, 8.0 years; 20 male patients; 32 female patients; new T1D diagnosis, n = 13; established diagnosis, n = 39). In all cases, correction of serum 3HB was an important aspect of T1D management. The median time to DKA resolution (defined as the time from the start of insulin infusion until the fall of 3HB level to below 1.0 mmol/L) was 11 and 10 h in new and established T1D cases, respectively. 3HB on admission and the required insulin infusion dose per body weight, but not blood pH level on admission, correlated with time to DKA resolution. There was no relationship between blood pH level and 3HB on admission. CONCLUSIONS: Our results showed that DKA resolution could be achieved within 10-11 h when DKA treatment is guided by bedside 3HB monitoring without any severe complications. Blood 3HB level is a potentially suitable marker for the severity and resolution of DKA.

    DOI PubMed

  • Factors associated with high care burden of primary caregivers of children with medical complexity after completing a discharge-support program in a recovery center

    Fuyuki Makiko, Yotani Nobuyuki, Kondo Masako, Iijima Yoshitaka, Wada Hiroshi, Takemoto Kiyoshi, Funato Masahisa, Ito Kazuya, Shintaku Haruo, Hamazaki Takashi

    BRAIN & DEVELOPMENT  43 ( 10 ) 988 - 996 2021.11  [Refereed]

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    INTRODUCTION: Recently, many seriously ill children requiring medical equipment are being recommended to transition from hospital to home care in Japan. Since 2011, our recovery center has provided a support program for the transfer process from hospital to home for ill children and their families. The purpose of this study was to evaluate the factors related to high care burden after completing the discharge-support program. METHODS: A questionnaire-based cross-sectional study was conducted on all primary caregivers whose children received the program in our center and moved from hospital to home (30 children and 29 families) from May 2011 to May 2018. Fifteen children came from the neonatal intensive care unit. The questionnaire consisted of three parts: characteristics of children and families and life after the program; the Zarit Burden Interview (ZBI); and the Positive and Negative Affect Schedule (PANAS). RESULTS: Twenty-three primary caregivers responded (79% response rate). All children received tracheostomy and 71% received home mechanical ventilation. Primary caregivers were all mothers. High ZBI score was not related to the severity and type of medical equipment. There were relationships between high ZBI score and following factors: 'unimproved relationship between patients and family members without primary caregivers' and 'additional medical equipment after discharge'. The result of PANAS showed that positive attitude was not different between those with high and low ZBI scores. CONCLUSION: It is crucial to reach out to family members without a primary caregiver. Additional medical care/equipment after the program is related to the care burden of primary caregivers.

    DOI PubMed

  • Divergent developmental trajectories in two siblings with neuropathic mucopolysaccharidosis type II (Hunter syndrome) receiving conventional and novel enzyme replacement therapies: A case report.

    Tomita K, Okamoto S, Seto T, Hamazaki T, So S, Yamamoto T, Tanizawa K, Sonoda H, Sato Y

    JIMD reports  62 ( 1 ) 9 - 14 2021.11  [Refereed]

     View Summary

    Mucopolysaccharidosis type II (MPS II; Hunter syndrome) is an X-linked recessive lysosomal storage disease caused by a mutation in the IDS gene and characterized by systemic accumulations of glycosaminoglycans. Its somatic symptoms can be relieved by enzyme replacement therapy (ERT) with idursulfase, but because the enzyme cannot cross the blood-brain-barrier (BBB), it does not address the progressive neurodegeneration and subsequent central nervous system (CNS) manifestations seen in patients with neuropathic MPS-II. However, pabinafusp alfa, a human iduronate-2-sulfatase (IDS) fused with a BBB-crossing anti-transferrin receptor antibody, has been shown to be efficacious against both the somatic and CNS symptoms of MPS II. We report two cases of MPS-II in Japanese siblings sharing the same G140V mutation in the IDS gene, who showed markedly contrasting developmental trajectories following enzyme replacement therapy (ERT). Sibling 1 was diagnosed at 2 years of age, started undergoing conventional ERT shortly afterward, and scored a developmental quotient (DQ) of 53 on the Kyoto Scale of Psychological Development (KSPD) at 4 years of age. Sibling 2 was diagnosed prenatally and received conventional ERT from the age of 1 month through 1 year and 11 months, when he switched to pabinafusp alpha. He attained a DQ of 104 at age 3 years and 11 months, along with significant declines in heparan sulfate concentrations in the cerebrospinal fluid. This marked difference in neurocognitive development highlights the importance of early initiation of ERT with a BBB-penetrating enzyme in patients with neuropathic MPS-II.

    DOI PubMed

  • Spinal Muscular Atrophy: Diagnosis, Incidence, and Newborn Screening in Japan

    Kimizu Tomokazu, Ida Shinobu, Okamoto Kentaro, Awano Hiroyuki, Niba Emma Tabe Eko, Wijaya Yogik Onky Silvana, Okazaki Shin, Shimomura Hideki, Lee Tomoko, Tominaga Koji, Nabatame Shin, Saito Toshio, Hamazaki Takashi, Sakai Norio, Saito Kayoko, Shintaku Haruo, Nozu Kandai, Takeshima Yasuhiro, Iijima Kazumoto, Nishio Hisahide, Shinohara Masakazu

    INTERNATIONAL JOURNAL OF NEONATAL SCREENING  7 ( 3 )  2021.09  [Refereed]

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    Spinal muscular atrophy (SMA) is a genetic neuromuscular disorder that causes degeneration of anterior horn cells in the human spinal cord and subsequent loss of motor neurons. The severe form of SMA is among the genetic diseases with the highest infant mortality. Although SMA has been considered incurable, newly developed drugs-nusinersen and onasemnogene abeparvovec-improve the life prognoses and motor functions of affected infants. To maximize the efficacy of these drugs, treatments should be started at the pre-symptomatic stage of SMA. Thus, newborn screening for SMA is now strongly recommended. Herein, we provide some data based on our experience of SMA diagnosis by genetic testing in Japan. A total of 515 patients suspected of having SMA or another lower motor neuron disease were tested. Among these patients, 228 were diagnosed as having SMA with survival motor neuron 1 (SMN1) deletion. We analyzed the distribution of clinical subtypes and ages at genetic testing in the SMN1-deleted patients, and estimated the SMA incidence based on data from Osaka and Hyogo prefectures, Japan. Our data showed that confirmed diagnosis by genetic testing was notably delayed, and the estimated incidence was 1 in 30,000-40,000 live births, which seemed notably lower than in other countries. These findings suggest that many diagnosis-delayed or undiagnosed cases may be present in Japan. To prevent this, newborn screening programs for SMA (SMA-NBS) need to be implemented in all Japanese prefectures. In this article, we also introduce our pilot study for SMA-NBS in Osaka Prefecture.

    DOI PubMed

  • Impact of intracerebroventricular enzyme replacement therapy in patients with neuronopathic mucopolysaccharidosis type II.

    Seo JH, Kosuga M, Hamazaki T, Shintaku H, Okuyama T

    Molecular therapy. Methods & clinical development  21   67 - 75 2021.06  [Refereed]

    DOI PubMed

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Books etc 【 display / non-display

  • Cell Therapy for Perinatal Brain Injury

    Shintaku H, Oka A, Nabetani M (Part: Contributor )

    Springer  2018

Review Papers (Misc) 【 display / non-display

  • 【新ガイドラインの理解を深める 新生児マススクリーニング】総論 アミノ酸代謝異常症

    濱崎 考史

    (株)診断と治療社 小児科診療  84 ( 2 ) 157 - 162 2021.02  [Refereed]  [Invited]

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    ●アミノ酸代謝異常症は、関係するアミノ酸の種類により病態が大きく異なる。●国内での頻度はフェニルケトン尿症・高フェニルアラニン血症が高く、メープルシロップ尿症、ホモシスチン尿症はきわめて低い。●診断方法も個々の疾患において異なることに注意が必要。●治療は、代謝できないアミノ酸を除去した特殊ミルクの使用が原則だが、特異的な補酵素を大量に補充する治療に反応する病型も存在する。(著者抄録)

  • 【小児の負荷試験2019】 代謝機能検査 テトラヒドロビオプテリン負荷試験

    濱崎 考史

    (株)東京医学社 小児内科  51 ( 4 ) 513 - 515 2019.04  [Refereed]  [Invited]

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    <Key Points>(1)BH4・1回負荷試験はPhe制限食を開始する前に負荷試験を施行できることが望ましい。(2)BH4負荷試験にはこれまでに重大な副作用は認められておらず、とくに禁忌併用薬はなく、検査前の食事制限もない。(3)負荷試験は血中Phe値の変化で判定するため、試験期間中は一定の食事内容、Phe摂取量を維持することが重要である。(著者抄録)

  • 【神経疾患とトレース・メタル-知っていますか?】 銅 Menkes病

    新宅 治夫, 保科 隆男, 濱崎 考史

    (株)中外医学社 Clinical Neuroscience  37 ( 3 ) 324 - 328 2019.03  [Refereed]  [Invited]

  • 子供たちの健やかな成長のために

    濱崎 考史

    大阪市医学会 大阪市医学会雑誌  67   21 - 26 2018.12  [Refereed]  [Invited]

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    赤ちゃんは自分では何もできない状態で生まれ、一般的な主訴があって受診する成人での診療と全くことなる医療が必要となる。この新生児期には、不可逆的な障がいを引き起こす前に診断ができる大きな可能性を秘めている。その代表的な疾患は、フェニルケトン尿症(Penylketonuria;PKU)である。日本では1977年に新生児マススクリーニングが開始となり、これまでに600人以上の患者が、重篤な精神発達遅滞を免れることができた。しかし、未だ新生児スクリーニングに加えられない疾患があり、稀少な疾患であるが故に診断が遅れ、新規治療法があるにもかかわらず、治療の機会が失われている。近年のゲノム解析技術の革新により、これまで未診断であった患者の診断が可能となってきた。2015年、日本で未診断疾患イニシアチブ(Initiative on Rare and Undiagnosed Diseases;IRUD)が立ち上がり、多くの稀少疾患専門医との連携診療体制が整いつつある。ゲノム解析技術の進歩により、個々の遺伝子の多型による健康への影響や、薬に対する反応性を予測することも可能になってきた。一方、ゲノム情報の総体としての表現型を評価することは現在も困難であり、一つのツールとして、iPS細胞を用いた疾患モデルが注目されており、ゲノム研究からの成果から推測される分子メカニズムを実証することが期待される。(著者抄録)

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Conference Activities & Talks 【 display / non-display

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Grant-in-Aid for Scientific Research 【 display / non-display

  • Study of relationship between autistic/intellectual disorder and neuronal chronic inflammation on mucopolysaccharidosis type III

    Project/Area Number : 26461550  Grant-in-Aid for Scientific Research(C) Partaker / Other

    Project Year :

    2014.04
    -
    2018.03
     

     View Summary

    Neuroglial complications, which are intellectual disability (ID) and autistic disorder, of congenital mucoporysaccharidosis (MPS) are speculated to be related with chronic inflammation in central nervous system. We have studied the pathological mechanism compared with various children with autism and ID. In order to evaluate inflammation on MPS patient we performed analysis of pteridine derivatives and cytokines, it clarified elevated some markers of inflammation. On the other hand, in order to study pathology of MPS brain, we used MPS type II model mice (IDS-KO). It was revealed that there were vacuoles in various type of cells in the brain by electron microscopy and immunostaining related autophagy. It was effective to administrate drug for suppression of autophagy in IDS-KO pathologically. Longitudinal study of MRI on MPS type II, it was shown that hematopoietic stem cell transplantation was effective for brain to some extent.

  • Modelling childhood neurotransmitter diseases using iPSCs

    Project/Area Number : 24591521  Grant-in-Aid for Scientific Research(C) Representative

    Project Year :

    2012.04
    -
    2016.03
     

    Partaker : SHINTAKU Haruo, UMEZAWA Akihiro, TOYODA Masashi

     View Summary

    Childhood neurotransmitter diseases are genetic syndromes caused by dysfunctional synapsis. Based on our national survey for these diseases, clinical phenotype and laboratory profiles have been illustrated. Conventional laboratory testing for blood and cerebrospinal fluid, however, could not explain pathogenesis of neurological symptoms. Because each of the disease is extremely rare, there is no systematic approach to develop novel treatment. Here we aimed to establish model system by using patient derived iPSCs to elucidate molecular mechanisms for this devastating neurological phenotypes. As the results, we were able to established disease-specific iPSCs and successfully differentiated into neurons. Furthermore, we examined pathogenesis of the disease at cellular and molecular level.

  • Study on the therapeutic effect of the chelating agent in Menkes disease by using microPET

    Project/Area Number : 24591523  Grant-in-Aid for Scientific Research(C) Partaker / Other

    Project Year :

    2012.04
    -
    2016.03
     

    Partaker : SHIOMI Susumu, HAMAZAKI Takashi, FUJIOKA Hiroki

     View Summary

    Menkes disease is a congenital copper metabolisc disorder, leading to both copper deficiency in brain and copper accumulation in the kidney due to copper transport disorder. Macula mice is used as Menkes disease model. Pretreatment with the lipophilic chelator disulfiram markedly increased copper uptake to the brain of macular mice. Pretreatment with the water-soluble chelator penicillamine enhanced urinary excretion and reduced copper accumulation in the kidney. Concomitant treatment with disulfiram and penicillamine retained the same effects. Combined use of disulfiram and penicillamine may improve clinical outcomes of Menkes disease. Therefore, it is possible to improve the neurologic outcome of Menkes disease, further revealed the possibility of improving the long-term prognosis of renal function.

 

Foreigner acceptance 【 display / non-display

  • Academic year : 2019

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    Number of International Students
    3