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    • Acute Encephalopathy
    • Akihiro Hoshino
    • Akihiro Hoshino: Influence Statistics

      Akihiro Hoshino

      Akihiro Hoshino

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      Laboratory of Lymphocyte Activation and Susceptibility to EBV Infection, INSERM UMR 1163, Imagine Institute, Paris, France | Department of Developmental Medical Sciences, ...

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      Akihiro Hoshino:Expert Impact

      Concepts for whichAkihiro Hoshinohas direct influence:Acute encephalopathy,Xiap deficiency,Ipex syndrome,Ctla4 mutations,Ranbp2 mutation,Biphasic seizures,Cell transplantation,Flow cytometry.

      Akihiro Hoshino:KOL impact

      Concepts related to the work of other authors for whichfor which Akihiro Hoshino has influence:Acute encephalopathy,Inborn errors,Biphasic seizures,Immune dysregulation,Late reduced diffusion,Primary immunodeficiency,Reversible splenial lesion.

      KOL Resume for Akihiro Hoshino

      Year
      2022

      Laboratory of Lymphocyte Activation and Susceptibility to EBV Infection, INSERM UMR 1163, Imagine Institute, Paris, France

      Department of Developmental Medical Sciences, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan

      2021

      Department of Developmental Medical Sciences, School of International Health, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-0033, Japan.

      Tokyo Medical and Dental University

      2020

      Department of Esophageal Surgery, Tokyo Medical and Dental University

      2019

      Laboratory of Lymphocyte Activation and Susceptibility to EBV infection, Institut National de la Sante et de la Recherche Medicale UMR 1163.

      2018

      Department of Community Pediatrics, Perinatal and Maternal Medicine, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University.

      2017

      Department of Gastrointestinal Surgery, Tokyo Medical and Dental University, Tokyo, Japan

      2016

      Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan

      2015

      Department of Pediatrics, Graduate School of Medicine, University of Toyama, Toyama, Japan

      2014

      Department of Esophageal and General Surgery, Tokyo Medical and Dental University, Tokyo, Japan.

      2013

      Department of Pediatrics, National Rehabilitation Center for Children with Disabilities, Tokyo, Japan

      2012

      Department of Developmental Medical Sciences, Graduate School of Medicine, The University of Tokyo, Japan

      2011

      Department of Pediatrics, Metropolitan Hachioji Children’s Hospital, Tokyo, Japan

      2010

      Department of Esophago-Gastric Surgery, Tokyo Medical and Dental University, 1-5-45 Yushima, 113-8519, Bunkyo-ku, Tokyo, Japan

      2009

      Department of Neuropediatrics, Fuchu, Tokyo.

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      Sample of concepts for which Akihiro Hoshino is among the top experts in the world.
      Concept World rank
      95 0002conclusionthis study #3
      rs1850440 #3
      ranbp2 c1754c #3
      susceptibility aesd #3
      254 japanese children #3
      patient wasp #3
      stk39 rs1850440 #3
      identified stk39 #3
      stricture association #3
      wildtype ranbp2 #3
      aesd genetic #3
      aesd variant #3
      day endoscopy predicts #3
      adane interaction #3
      febrile seizures aesd #3
      esophagus superficial lesion #3
      acute hemorrhagic ranbp2 #3
      cox11 bound #3
      huge protruded lesion #3
      dental university univariate #3
      risk factor stricture #3
      aesd female humans #3
      opposite febrile #3
      rs16944 aesd #3
      rs16944 febrile #3
      resultsthe 0161–0656 #3
      blood perfusion anastomosis #3
      aesd controls #3
      gstranbp2 recombinant protein #3
      c1754c #3
      ranbp2 cultured child #3
      attenuated binding ability #3
      mucosal congestion day #3
      undergone nasal findings #3
      aesd micrornas #3
      rs1850440 stk39 #3
      ranbp2 recombinant #3
      cox11 ranbp2 #3
      rs16944 febrile seizures #3
      interaction mutated ranbp2 #3
      performed hypopharyngeal #3
      mucosal cancers patient #3
      mutated ranbp2 cox11 #3
      candidate biomarkers aesd #3
      tube congestion #3
      mutated ranbp2 #3
      studies ranbp2 #3
      ranbp2 c1754ct #3
      revealed endoscopically #3
      aesd 799 #3
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      Prominent publications by Akihiro Hoshino

      KOL-Index: 12448

      BackgroundX-linked inhibitor of apoptosis protein (XIAP) deficiency is a rare immunodeficiency that is characterized by recurrent hemophagocytic lymphohistiocytosis (HLH) and splenomegaly and sometimes associated with refractory inflammatory bowel disease (IBD). Although hematopoietic stem cell transplantation (HSCT) is the only curative therapy, the outcomes of HSCT for XIAP deficiency remain unsatisfactory compared with those for SLAM-associated protein deficiency and familial ...

      Known for Xiap Deficiency | Cell Transplantation | Hematopoietic Stem | Hsct Patients | Host Disease
      KOL-Index: 11491

      OBJECTIVE: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a childhood encephalopathy following severe febrile seizures. The pathogenesis of AESD is considered to be fever-induced seizure susceptibility and excitotoxicity, which may be caused by sodium channel dysfunction in some cases. Here we studied whether mutations in genes encoding sodium channels, SCN1A and SCN2A, predispose children to AESD.

      METHODS: We recruited 92 AESD patients in a nationwide ...

      Known for Sodium Channel | Febrile Seizures | Acute Encephalopathy | Dravet Syndrome | Scn2a Mutation
      KOL-Index: 10819

      A research committee supported by the Japanese government conducted a nationwide survey on the epidemiology of acute encephalopathy in Japan using a questionnaire. A total of 983 cases reportedly had acute encephalopathy during the past 3 years, 2007-2010. Among the pathogens of the preceding infection, influenza virus was the most common, followed by human herpesvirus-6 (HHV-6) and rotavirus. Among syndromes of acute encephalopathy, acute encephalopathy with biphasic seizures and late ...

      Known for Acute Encephalopathy | Aesd Mers | Influenza Virus | Japan Ane | Nationwide Survey
      KOL-Index: 10273

      Despite the decrease in Reye syndrome after the discontinuation of aspirin, acute encephalopathy (non-Reye syndrome type) has been continually reported in Japan. Recent studies suggested that the thermolabile phenotype of carnitine palmitoyltransferase II (CPT II) variation [F352C] was closely related to the pathomechanism of influenza-associated encephalopathy (IAE) in Japanese, causing mitochondrial ATP utilization failure during periods of high fever, resulting in brain edema. So, we ...

      Known for Acute Encephalopathy | Thermolabile Cpt | Blood Atp | Japanese Children | Preschool Encephalitis
      KOL-Index: 8797

      Ikaros/IKZF1 is an essential transcription factor expressed throughout hematopoiesis. IKZF1 is implicated in lymphocyte and myeloid differentiation and negative regulation of cell proliferation. In humans, somatic mutations in IKZF1 have been linked to the development of B cell acute lymphoblastic leukemia (ALL) in children and adults. Recently, heterozygous germline IKZF1 mutations have been identified in patients with a B cell immune deficiency mimicking common variable ...

      Known for Ikzf1 Mutations | Cells Cell | Heterozygous Germline | Dominant Negative | Incomplete Penetrance
      KOL-Index: 8088

      Most childhood cases of acute necrotizing encephalopathy (ANE) involve neither family history nor recurrence. ANE occasionally occurs, however, as a familial disorder or recurs in Caucasian patients. A mutation of RAN-binding protein 2 (RANBP2) has been discovered in more than one half of familial or recurrent ANE patients. In contrast, there has been no report of this mutation in East Asia. Here, we report the first sibling cases of typical ANE in Japan, with poor outcome. DNA analysis ...

      Known for Ranbp2 Mutation | Familial Acute | Necrotizing Encephalopathy | Caucasian Patients | Typical Ane
      KOL-Index: 8070

      Pelizaeus-Merzbacher disease (PMD; MIM#312080) is a rare X-linked recessive neurodegenerative disorder. The main cause of PMD is alterations in the proteolipid protein 1 gene (PLP1) on chromosome Xq22.2. Duplications and point mutations of PLP1 have been found in 70% and 10-25% of all patients with PMD, respectively, with a wide clinical spectrum. Since the underlining genomic abnormalities are heterogeneous in patients with PMD, clarification of the genotype-phenotype correlation is the ...

      Known for Patients Pmd | Situ Hybridization | Plp1 Duplications | Merzbacher Disease | Proteolipid Protein
      KOL-Index: 7652

      BACKGROUND: Autoimmune diseases in children are rare and can be difficult to diagnose. Autoimmune lymphoproliferative syndrome (ALPS) is a well-characterized pediatric autoimmune disease caused by mutations in genes associated with the FAS-dependent apoptosis pathway. In addition, various genetic alterations are associated with the ALPS-like phenotype.

      OBJECTIVE: The aim of the present study was to elucidate the genetic cause of the ALPS-like phenotype.

      METHODS: Candidate genes ...

      Known for Autoimmune Lymphoproliferative | Tnfaip3 A20 | Alpslike Phenotype | Negative Regulator | Syndrome Alps
      KOL-Index: 7477

      PurposePediatric inflammatory bowel disease (IBD) is a heterogeneous disorder caused by multiple factors. Although genetic and immunological analyses are required for a definitive diagnosis, no reports of a comprehensive genetic study of a Japanese population are available.MethodsIn total, 35 Japanese patients <16 years of age suffering from IBD, including 27 patients aged <6 years with very early-onset IBD, were enrolled in this multicenter study. Exome and targeted gene panel ...

      Known for Pediatric Ibd | Multicenter Study | Primary Immunodeficiency | Targeted Sequencing | Bowel Disease
      KOL-Index: 7221

      BACKGROUND: Cytotoxic T-lymphocyte antigen 4 (CTLA-4) is a negative immune regulator. Heterozygous CTLA4 germline mutations can cause a complex immune dysregulation syndrome in human subjects.

      OBJECTIVE: We sought to characterize the penetrance, clinical features, and best treatment options in 133 CTLA4 mutation carriers.

      METHODS: Genetics, clinical features, laboratory values, and outcomes of treatment options were assessed in a worldwide cohort of CTLA4 mutation carriers.

      RESULTS: We ...

      Known for Mutation Carriers | Lymphocyte Antigen | 4 Ctla4 | Clinical Manifestations | Gastric Cancer
      KOL-Index: 6805

      Hirotomo Saitsu, Noboru Mizushima, Naomichi Matsumoto and colleagues report the identification of de novo mutations in WDR45 that cause static encephalopathy of childhood with neurodegeneration in adulthood. WDR45 encodes a homolog of the yeast autophagy protein Atg18.

      Known for Novo Mutations | Static Encephalopathy | Autophagy Gene | Wdr45 Encodes | Intellectual Disability
      KOL-Index: 6767

      BACKGROUND: Ikaros, which is encoded by IKZF1, is a transcriptional factor that play a critical role in hematopoiesis. Somatic IKZF1 alterations are known to be involved in the pathogenesis of leukemia in human subjects. Recently, immunodeficiency caused by germline IKZF1 mutation has been described.

      OBJECTIVE: We sought to describe the clinical and immunologic phenotypes of Japanese patients with heterozygous IKZF1 mutations.

      METHODS: We performed whole-exome sequencing in patients from ...

      Known for Germline Ikzf1 | Bone Marrow | Autoimmune Diseases | Systemic Lupus Erythematosus | Iga Vasculitis
      KOL-Index: 6391

      Immune dysregulation, polyendocrinopathy, enteropathy, and X-linked (IPEX) syndrome is an autoimmune disorder caused by the dysfunction of FOXP3, which leads to regulatory T-(Treg) cell dysfunction and subsequently autoimmunity including type 1 diabetes mellitus (T1D). Presently, allogeneic hematopoietic stem cell transplantation (HSCT) is a potential curative therapy for IPEX syndrome, but not for T1D. Generally, after complete loss of pancreatic β-cells, HSCT cannot improve the ...

      Known for Ipex Syndrome | Diabetes Mellitus | Type 1 | Cell Transplantation | Hematopoietic Stem
      KOL-Index: 6254

      Primary immunodeficiencies (PIDs) are a heterogeneous group of inherited diseases of the immune system. The definite diagnosis of PID is ascertained by genetic analysis; however, this takes time and is costly. Flow cytometry provides a rapid and highly sensitive tool for diagnosis of PIDs. Flow cytometry can evaluate specific cell populations and subpopulations, cell surface, intracellular and intranuclear proteins, biologic effects associated with specific immune defects, and certain ...

      Known for Flow Cytometry | Diagnosis Pids | Primary Immunodeficiency | Deficiency Syndromes | Humans Immunologic

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      Laboratory of Lymphocyte Activation and Susceptibility to EBV Infection, INSERM UMR 1163, Imagine Institute, Paris, France | Department of Developmental Medical Sciences, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan | Department

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