Research activity information

• Jun. 2022 日本小児神経学会, 優秀論文賞

• Delay in benzodiazepine administration is associated with increased need for anesthetic coma therapy in children with febrile status epilepticus.

  Kento Souma, Hiroshi Yamaguchi, Takuya Ueda, Shizuka Oikawa, Yusuke Ishida, Kandai Nozu, Azusa Maruyama, Hiroaki Nagase, Masahiro Nishiyama

  BACKGROUND: Febrile status epilepticus (FSE) is associated with the development of acute encephalopathy (AE), and delays in the administration of antiseizure medications have been linked to worse outcomes. However, the effect of treatment timing on subsequent in-hospital management and short-term outcomes in pediatric patients with FSE remains inadequately elucidated. METHODS: We conducted a single-center retrospective cohort study involving children with FSE admitted to our hospital between January 2020 and April 2023. Patients who received benzodiazepines (BZDs) within 80 min of seizure onset were included. Patients were categorized into an early group (EG) (≤40 min) and a late group (LG) (41-80 min) based on the timing of initial BZD administration. Outcomes assessed included impaired consciousness at 6 h, induction of anesthetic coma therapy (ACT), development of AE, and neurological sequelae. RESULTS: A total of 93 children with FSE were analyzed. Impaired consciousness at 6 h was observed in 33 (35.4%) patients, and 16 (17.2%) required ACT. Neurological sequelae occurred in 7 (7.5%) patients, including one death (1.1%). The induction rate of ACT was significantly higher in the LG than in the EG (22.7% vs. 3.7%). Although no statistically significant differences were observed in the incidence of AE or neurological sequelae, the ACT rate increased in a time-dependent manner with delays in BZD administration. CONCLUSIONS: Delayed BZD administration may be associated with an increased likelihood of requiring intensive neurocritical care, including ACT, among children with FSE.

  Last, Aug. 2026, Epilepsy research, 227, 107898 - 107898, English, International magazine

  [Refereed]

  Scientific journal


• Association of Breakfast Habits at Age 3 With Academic Performance in Elementary School: A Retrospective Population-based Cohort Study.

  Aoi Kawamura, Masahiro Nishiyama, Hiroshi Yamaguchi, Tomoya Hirasaki, Takuya Ueda, Shizuka Oikawa, Kandai Nozu, Hiroaki Nagase

  OBJECTIVE: The long-term relationship between breakfast habits in early childhood and academic performance remains unclear. Therefore, this study aimed to investigate the association between breakfast habits at age 3 years and academic performance in the first grade of elementary school. METHODS: We conducted a retrospective analysis of a population-based cohort in Amagasaki City that followed children from birth until entry into elementary school. Academic outcomes included performance in the national language and math, as well as subdomains within each subject. RESULTS: Among 7847 children, regular breakfast consumption at age 3 years was associated with higher academic performance. Children who ate breakfast daily had higher mean scores in national language (73.12 ± 19.19 vs 66.16 ± 22.33) and math (81.09 ± 19.19 vs 73.31 ± 24.71). In multivariable regression analyses, daily breakfast consumption at age 3 years was modestly associated with higher first-grade academic performance, specifically with overall math scores (β = 2.46, 95% confidence interval [CI]: 0.07-4.85), calculation-related skills (β = 1.94, 95% CI: 0.29-3.59), and language-related skills (β = 1.74, 95% CI: 0.02-3.46). Associations with other domains were not significant. These associations remained significant after adjusting for economic status, suggesting an independent association between early childhood breakfast habits and academic performance. Children who did not eat breakfast daily were more likely to have financial difficulties and irregular lifestyles. CONCLUSION: Regular breakfast consumption during early childhood was associated with higher subsequent academic achievement. However, the observed association may reflect underlying social and environmental factors.

  Corresponding, Aug. 2026, Journal of developmental and behavioral pediatrics : JDBP, English, International magazine

  [Refereed]

  Scientific journal


• Clinical practices in prehospital rescue medication use for pediatric status epilepticus in Japan: a nationwide questionnaire survey.

  Masahiro Nishiyama, Yuki Ueda, Ryuki Matsuura, Tadashi Shiohama, Mitsuo Motobayashi, Masataka Fukuoka, Shizuka Oikawa, Sotaro Kanai, Michiko Torio, Ichiro Kuki, Hiroaki Nagase, Tomoyuki Akiyama, Kenjiro Kikuchi, Yoshihiro Maegaki

  BACKGROUND: Status epilepticus (SE) is a neurological emergency requiring rapid treatment. While prehospital management is critical, practices regarding rescue medication use and caregiver guidance, including emergency service activation, remain unclear. METHODS: We conducted a nationwide, web-based survey of pediatric neurologists between January and March 2025. One representative from each institution reported on rescue medication prescribing and caregiver instructions for prehospital seizure management. The survey assessed indications for buccal midazolam, scenario-based recommendations, and post-administration guidance. RESULTS: Of 136 responses, 134 respondents who were aware of buccal midazolam were included; 118 had prescribing experience. A history of prolonged convulsive seizures, particularly ≥30 min (95.5% high-priority), was consistently identified as a high-priority indication. Seizures lasting 5-30 min were also considered appropriate (57.5% high-priority, 38.1% low-priority), with greater variability. In contrast, frequent seizures alone were often regarded as appropriate (44.8% low-priority) but rarely high-priority (16.4%). Diagnostic categories such as epilepsy or febrile seizures themselves were not regarded as appropriate indications. Environmental factors, including distance from medical facilities, were also relevant. Similar patterns were observed in scenario-based recommendations, with consistent findings across epilepsy and febrile seizure scenarios. Caregiver instructions varied widely, particularly regarding emergency service activation. In-hospital management was generally unchanged regardless of prehospital rescue medication use. CONCLUSIONS: Prehospital rescue medication prescribing is primarily guided by seizure duration rather than diagnostic categories. Variability in caregiver instructions highlights the need for clearer guidance to optimize prehospital seizure management.

  Aug. 2026, Brain & development, 48(4) (4), 104573 - 104573, English, International magazine

  [Refereed]

  Scientific journal


• Duration and determinants of impaired consciousness after febrile status epilepticus in children.

  Tomohiro Sameshima, Hiroshi Yamaguchi, Kentaro Sano, Mitsuo Motobayashi, Mitsuru Kashiwagi, Yuka Hattori, Azusa Maruyama, Daisaku Toyoshima, Taku Nakagawa, Go Kawano, Hiroaki Nagase, Masahiro Nishiyama

  PURPOSE: While prolonged impaired consciousness is often attributed to encephalopathy or meningitis, its occurrence and associated factors in patients with febrile status epilepticus without these conditions remain unclear. This study investigated the duration and determinants of impaired consciousness following febrile status epilepticus lasting ≥ 30 min in children treated with antiseizure medications. METHODS: This retrospective multicenter study used data from the Febrile Acute Convulsion and Encephalopathy registry, a prospective multicenter consecutive case registry for acute encephalopathy and febrile convulsive status epilepticus in Japan. Children aged 6-60 months with febrile status epilepticus lasting ≥ 30 min who received antiseizure medication were analyzed. Clinical data and early laboratory results were compared between prolonged (≥4 h) and non-prolonged (<4 h) impaired consciousness groups. RESULTS: Among 227 children with febrile status epilepticus lasting ≥ 30 min, the median time to recovery of consciousness was 176 min, and 79 (34.8%) had prolonged impaired consciousness (≥4 h). The two groups did not differ in age, sex, seizure duration, body temperature, history of febrile seizures, or number of antiseizure medications. Phenobarbital use was more frequent in the prolonged group. Laboratory abnormalities, including lower pH, lower base excess, higher creatinine, higher glucose, and higher ammonia were associated with prolonged impairment. Low pH was the only independent factor. CONCLUSION: In children with febrile status epilepticus ≥ 30 min, prolonged unconsciousness (≥4 h) was associated with phenobarbital use and laboratory abnormalities, with acidosis as the sole independent predictor. Early recognition of delayed recovery may support timely clinical decision making and optimize emergency care.

  Jul. 2026, Epilepsy & behavior : E&B, 180, 111013 - 111013, English, International magazine

  [Refereed]

  Scientific journal


• A novel splice site variant in TIMM8A induced abnormal mRNA splicing resulting in Mohr-Tranebjaerg syndrome.

  Hiroaki Hanafusa, Yusuke Ishida, Ryosuke Bo, Masahiro Nishiyama, Kandai Nozu, Hiroaki Nagase, Hiroyuki Awano

  BACKGROUND: Mohr-Tranebjaerg syndrome (MTS) is an X-linked recessive neurodegenerative disorder caused by pathogenic variants in TIMM8A. Of the 39 previously reported disease-causing variants in the TIMM8A, five are splice site variants; however, none of these variants have been evaluated by transcript analysis. METHODS: We performed panel-based targeted exome analysis in a Japanese boy with sensorineural hearing loss and rapidly progressive dystonia. To assess the effect of the identified splice donor site variant, transcript analysis was performed using RNA derived from peripheral blood. RESULT: A novel hemizygous splice donor site variant in TIMM8A (NM_004085.4:c.132+5G>A) was identified. Transcript analysis revealed three aberrant transcripts: two transcripts with partial intron 1 inclusion of 606 bp or 492 bp (INS606bp and INS492bp) and one transcript with a 60 bp partial deletion of exon 1 (Δ60bp), with no detectable normal transcript. Both INS606bp and INS492bp transcripts contain premature stop codons due to the inserted intronic sequences, leading to the loss of 53 amino acids, whereas the Δ60bp transcript leads to the loss of 20 amino acids. All aberrant transcripts lacked part of the Tim10/DDP family zinc finger domain, which is essential for TIM8A function. CONCLUSION: This study provides the first transcript analysis elucidating the pathogenic mechanism of a splice donor site variant in TIMM8A. The findings suggest that splice donor site variants may share a common disease mechanism involving the production of functionally defective TIM8A protein.

  Jun. 2026, Brain & development, 48(3) (3), 104525 - 104525, English, International magazine

  [Refereed]

  Scientific journal


• Impact of the COVID‐19 Pandemic on Child Development and Caregiving: A 7‐Year Repeated Cross‐Sectional Study of 3‐Year‐Old Children in Kobe City, Japan

  Kazuki Suemune, Hiroshi Yamaguchi, Masahiro Nishiyama, Yuki Kyono, Aoi Kawamura, Shizuka Oikawa, Takumi Imai, Sae Murakami, Hiroki Mishina, Kandai Nozu, Hiroaki Nagase

  ABSTRACT Introduction Social restrictions during the coronavirus (COVID‐19) pandemic have resulted in children spending more time with their families and having fewer opportunities to attend nursery schools or therapeutic facilities. Few studies have examined the impact of the COVID‐19 pandemic on the development and parenting environments of 3‐year‐old children. In this study, we aimed to examine the impact of the COVID‐19 pandemic on fine and gross motor skills and language development, including comprehension and communication, in 3‐year‐old children. The secondary objective was to investigate their impact on caregiving environments. Methods This repeated cross‐sectional study was conducted at age 3 years using data from a longitudinal birth cohort. We analyzed data from routine 3‐year‐old health examinations conducted in Kobe City, Japan, between April 2014 and March 2021. Multivariable logistic regression models were used to assess the association between COVID‐19 birth cohorts and developmental and caregiving outcomes among 3‐year‐old children. In total, 62,192 children (3 years, 51.4% males; majority Japanese) were categorized into three birth cohorts: pre‐ (from 4/1/2014 to 3/31/2017; n = 29,421), partial‐ (from 4/1/2017 to 3/31/2020; n = 25,202), and post‐COVID‐19 (from 4/1/2020 to 3/31/2021; n = 7569). Results The prevalence of impaired gross motor skills and language comprehension was significantly higher in the post‐COVID‐19 group than in the pre‐COVID‐19 group (gross motor skill: 0.7% vs. 0.5%, OR 1.41, 95% CI 1.02–1.95, p = 0.04; language comprehension abnormalities: 3.6% vs. 2.1%, OR 1.72, 95% CI 1.49–1.99, p < 0.001). However, medical evaluations conducted by physicians showed no differences in fine motor skills. Caregiver questionnaires showed impaired verbal and communication skills, as well as reduced interactions with peers and relatives, in the post‐COVID‐19 group (limited vocabulary growth: 1.0% vs. 0.7%, OR 1.32, 95% CI 1.01–1.72, p = 0.04; inability to formulate three‐word sentences: 4.4% vs. 3.4%, OR 1.26, 95% CI 1.11–1.43, p < 0.001; inability to state the names of their playmates: 5.5% vs. 3.9%, OR 1.40, 95% CI 1.25–1.57, p = 0.003). However, no definitive conclusions regarding motor skills could be drawn from our findings. Although caregiving assistance decreased significantly in the COVID‐19 group, comparable trends were observed in the pre‐COVID‐19 group. Conclusion These findings indicate that the COVID‐19 pandemic was associated with changes in language development and social interactions, possibly reflecting decreased opportunities for social engagement outside the family.

  Wiley, Apr. 2026, Brain and Behavior, 16(4) (4), e71434, English, International magazine

  [Refereed]

  Scientific journal


• Management of pediatric status epilepticus after hospital arrival in Japan: A nationwide questionnaire survey.

  Masahiro Nishiyama, Yuki Ueda, Ryuki Matsuura, Tadashi Shiohama, Mitsuo Motobayashi, Masataka Fukuoka, Shizuka Oikawa, Sotaro Kanai, Michiko Torio, Ichiro Kuki, Hiroaki Nagase, Tomoyuki Akiyama, Kenjiro Kikuchi, Yoshihiro Maegaki

  BACKGROUND: Status epilepticus (SE) is a neurological emergency requiring rapid, stepwise treatment. In Japan, the Guidelines for the Treatment of Pediatric Status Epilepticus 2023 (GL2023) were published; however, real-world practice following their publication remains unclear. METHODS: We conducted a nationwide, cross-sectional web-based survey of pediatric neurologists between January and March 2025. One representative from each institution reported institutional practices for the in-hospital management of pediatric SE. Institutions were stratified by annual SE case volume (≥20 vs. ≤19 cases). General management strategies, antiseizure medication selection, electroencephalography (EEG) utilization, and approaches to refractory and super-refractory SE (RSE and SRSE) were analyzed. RESULTS: A total of 136 institutions responded. Most institutions reported referring to GL2023 and initiating first-line treatment within 10 min of hospital arrival. Midazolam was the most commonly used first-line drug, with widespread use of non-intravenous routes. Phenytoin or fosphenytoin remained the most commonly selected second-line drug, followed by phenobarbital. For RSE, anesthetic coma therapy with midazolam and barbiturates was used at comparable frequencies. Overall, antiseizure medication selection did not differ by institutional case volume. In contrast, institutions managing ≥20 cases annually more frequently reported having institution-specific protocols, utilizing EEG in the emergency department, administering repeat doses of benzodiazepines, and having experience with advanced therapies for SRSE. CONCLUSIONS: Pediatric SE management in Japan emphasizes rapid treatment and flexible administration routes. Institutional experience is associated with differences in EEG utilization, protocol development, and SRSE management. Further improvement will require continued evidence generation, timely treatment implementation, and reduction of institutional disparities.

  Mar. 2026, Brain & development, 48(3) (3), 104524 - 104524, English, International magazine

  [Refereed]

  Scientific journal


• Impact of the COVID-19 pandemic on infant neurodevelopmental outcomes

  Yuki Kyono, Masahiro Nishiyama, Hiroshi Yamaguchi, Aoi Kawamura, Shizuka Oikawa, Shoichi Tokumoto, Takumi Imai, Sae Murakami, Kandai Nozu, Hiroki Mishina, Hiroaki Nagase

  Objective This population-based cohort study in Kobe, Japan, investigated the impact of the COVID-19 pandemic on infant neurodevelopment by comparing children born before and during the pandemic. Design Retrospective population-based cohort study of 63 703 children born between 1 April 2014 and 31 October 2020, who underwent an 18-month health check-up. Setting Kobe, Japan. Patient Children born between April 2014 and March 2018 (pre-COVID-19 group) or April–October 2020 (during-COVID-19 group). Intervention None Main outcome measures Neurodevelopmental outcomes assessed by trained paediatricians, including language, social and behavioural indicators. Results The abnormal neurodevelopment prevalence was higher in the during-COVID-19 group (12.8%) than in the pre-COVID-19 group (10.2%) (OR, 1.30; 99% CI 1.16 to 1.46). Similarly, the rate of children without meaningful words was higher during the pandemic (7.1% vs 4.8%; OR, 1.52; 99% CI 1.31 to 1.78), indicating delayed language development. Intergroup differences in other outcomes were minimal. Conclusions The COVID-19 pandemic may have negatively influenced early neurodevelopment, particularly language acquisition. These findings suggest that infant language development is affected by social changes, such as pandemics. Further research is required to explore the underlying causes and the long-term effects.

  Corresponding, BMJ, Feb. 2026, BMJ Paediatrics Open, 10(1) (1), e003832 - e003832, English

  [Refereed]

  Scientific journal


• Life-Threatening SARS-CoV-2-Associated Encephalopathy and Multiorgan Failure in Children, Asia and Oceania, 2022-2024.

  Mariko Kasai, Hiroshi Sakuma, Motomasa Suzuki, Masahiro Nishiyama, Nanako Kawata, Jainn-Jim Lin, Kuang-Lin Lin, Velda Han, Shekeeb S Mohammad, Russell C Dale, Terrence Thomas, Kazuhiro Muramatsu, Osamu Mitani, Yoshiyuki Kobayashi, Kouhei Ishida, Yuichi Abe, Ichiro Kuki, Jun-Ichi Takanashi

  SARS-CoV-2 infections in children occasionally manifest with severe neurologic signs. We report a case series of life-threatening encephalopathy associated with SARS-CoV-2 in 25 children in Australia, Japan, Singapore, and Taiwan during February 2022-January 2024. All children had severe encephalopathy develop, characterized by rapidly progressive cerebral edema, conditions known as acute shock with encephalopathy and multiorgan failure or acute fulminant cerebral edema. Among the 25 patients, 22 (88%) eventually died; 11 (44%) children died within 24 hours of hospitalization. In addition, 18 (72%) had illness manifest with shock, and 14 (56%) had multiorgan failure develop within 6 hours of neurologic onset. Serum concentrations of cytokines/chemokines including interleukin 6 and tumor necrosis factor-α were significantly higher within 24 hours of onset than for controls. SARS-CoV-2-associated encephalopathy cases such as those described here represent an emerging neurologic crisis with high mortality rate resulting from rapidly progressive brain edema and multiorgan failure.

  Lead, Feb. 2026, Emerging infectious diseases, 32(2) (2), 169 - 179, English, International magazine

  [Refereed]

  Scientific journal


• Impact of the coronavirus disease pandemic on emergency transport times for pediatric febrile seizures: A retrospective study.

  Taisuke Matsumoto, Masahiro Nishiyama, Yusuke Ishida, Satoshi Matsui, Azusa Maruyama

  BACKGROUND: Febrile seizures are a common cause of emergency pediatric transport. Although the coronavirus disease (COVID-19) pandemic disrupted healthcare systems, including emergency services, its impact on transport times for pediatric febrile seizures remains unclear. This study aimed to evaluate how the pandemic affected transport times. METHODS: We retrospectively reviewed emergency department visits at our hospital between 2018 and 2022. Patients with febrile seizures in 2019 (before the COVID-19 pandemic) and in 2022 (during the pandemic) were included. Transport times and clinical data were compared between those years. Multivariate regression analysis was used to identify factors associated with longer transport times. RESULTS: There were 329 and 282 patients in 2019 and 2022, respectively. The median transport time increased significantly from 33 min in 2019 to 39 min in 2022 (p < 0.001). The proportion of cases exceeding 46 min increased from 10 % to 28 %, whereas those exceeding 60 min increased from 1.2 % to 7.4 %. A longer transport time was associated with seizure duration, onset year (2022), and transport from distant areas. Onset in 2022 was identified as an independent factor. Stratified analysis showed significantly longer transport times in adjacent and remote areas during the pandemic no significant difference in transport times near the hospital. CONCLUSION: Transport times for pediatric febrile seizures increased significantly during the COVID-19 pandemic, with more delays of over 60 min, especially in remote areas. These findings highlight the need to strengthen regional emergency transportation systems to reduce time to care, particularly for patients living farther from hospitals.

  Corresponding, Oct. 2025, Brain & development, 47(5) (5), 104456 - 104456, English, International magazine

  [Refereed]

  Scientific journal


• Description of Patterns in Reduced-Lead Electroencephalography Performed for Suspected Electrographic Seizures in Pediatric Patients With Altered Mental Status Using Standardized Terminology.

  Hiroshi Yamaguchi, Azusa Maruyama, Masahiro Nishiyama, Takuya Ueda, Hiroto Hongo, Shizuka Oikawa, Shoichi Tokumoto, Kazumi Tomioka, Satoshi Matsui, Takuro Hayashi, Ryojiro Tanaka, Kandai Nozu, Hiroaki Nagase

  BACKGROUND: This study aimed to analyze and describe reduced-lead electroencephalographic (EEG) data for suspected electrographic seizure (ESz) in pediatric patients presenting with altered mental status (AMS) in the emergency department (ED) according to standardized EEG terminology. The secondary aim was to compare the characteristics of these EEG patterns across febrile seizure (FS), acute encephalopathy/encephalitis (AE/AES), and epilepsy. METHODS: Epileptologists retrospectively analyzed the medical records and findings of reduced-lead EEG performed for suspected ESz in pediatric patients with AMS in ED between March 1, 2019, and February 28, 2023. Fifty-one EEG results with few artifacts were extracted; these patterns were described according to the American Clinical Neurophysiology Society's Standardized Critical Care EEG Terminology 2021. The obtained clinical diagnoses were categorized into three groups: FS, AE/AES, and epilepsy, and the characteristics of EEG patterns were compared. RESULTS: Clinical seizure types were not significantly different between FS, AE/AES, and epilepsy. In terms of EEG, there was no difference in Main term 1 (localization) among the groups. With regard to Main term 2 (morphology), patients with FS and AE/AES had commonly rhythmic delta activity, whereas patients with epilepsy had significantly more spikes and waves. ESz was observed in 25 patients; their incidence was significantly higher in the epilepsy group. CONCLUSIONS: This is the first study to describe patterns in reduced-lead EEG performed for suspected ESz in pediatric patients with AMS due to disorders commonly encountered in the ED by using standardized EEG terminology and to compare EEG patterns among these disorders.

  Aug. 2025, Pediatric neurology, 169, 21 - 30, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Systemic Primary Carnitine Deficiency Presenting With Substantia Nigra and Basal Ganglia Injury: A Case Report.

  Tomoki Saito, Kento Soma, Mai Kashisaka, Kiiko Iketani, Masaaki Matsumoto, Takuya Ueda, Masahiro Nishiyama, Azusa Maruyama, Ryosuke Nakai, Hiroshi Sakihama, Hiroshi Kurosawa, Naoya Morisada, Hironori Kobayashi, Kayo Ozaki

  Systemic primary carnitine deficiency (SPCD) is a rare congenital fatty acid metabolism disorder causing impaired β-oxidation and energy production, leading to hypoglycemia, metabolic encephalopathy, and sudden death. Early diagnosis and treatment, including L-carnitine supplementation and fasting avoidance, can improve prognosis. However, newborn screening (NBS) criteria differ by region, and standardized guidelines are lacking. This report presents a case of SPCD undetected by NBS, resulting in basal ganglia damage and dystonia due to metabolic decompensation. A 1-year-9-month-old girl with no abnormalities on NBS presented with impaired consciousness. She exhibited hypoketotic hypoglycemia, hyperammonemia, and myocardial hypertrophy. Suspecting a fatty acid metabolism disorder, L-carnitine and high-calorie infusion were initiated. Laboratory tests revealed markedly low serum total and free carnitine levels, and genetic analysis confirmed a homozygous SLC22A5 mutation. Brain MRI on day 7 revealed bilateral basal ganglia and substantia nigra abnormalities. The patient developed severe dystonia and respiratory failure, requiring ECMO management. L-DOPA was initiated on day 62, resulting in improvements in dystonia, swallowing, and motor function. By day 88, MRI showed resolution of basal ganglia abnormalities, though cerebral atrophy persisted. Basal ganglia damage is a rare but severe SPCD complication. L-DOPA may alleviate dystonia by acting on dopaminergic neurons in the substantia nigra. Early ketone measurement during emergencies is crucial for diagnosing fatty acid metabolism disorders. A standardized NBS protocol with a defined carnitine cutoff value is essential for early detection and prevention of SPCD complications.

  May 2025, JIMD reports, 66(3) (3), e70014, English, International magazine

  [Refereed]

  Scientific journal


• Hemorrhagic shock and encephalopathy syndrome with hemophagocytic lymphohistiocytosis pathology caused by cytomegalovirus infection

  Shinji Harada, Masahiro Nishiyama, Mao Mizuta, Masaki Shimizu, Azusa Maruyama, Hiroshi Kurosawa, Yasuo Nakagishi

  Corresponding, Elsevier BV, Mar. 2025, Brain and Development Case Reports, 3(1) (1), 100062 - 100062

  [Refereed]

  Scientific journal


• Epidemiology and treatment trends for acute encephalopathy under the impact of SARS-CoV-2 pandemic based on a prospective multicenter consecutive case registry.

  Shoichi Tokumoto, Masahiro Nishiyama, Hiroshi Yamaguchi, Kentaro Sano, Mitsuo Motobayashi, Mitsuru Kashiwagi, Yuka Hattori, Azusa Maruyama, Daisaku Toyoshima, Taku Nakagawa, Go Kawano, Hiroaki Nagase

  BACKGROUND: Acute encephalopathy is a severe condition predominantly affecting children with viral infections. The purpose of this study was to elucidate the epidemiology, treatment, and management of acute encephalopathy. The study also aimed to understand how the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic has affected epidemiological trends. METHODS: This retrospective study used the database of the Febrile Acute Convulsion and Encephalopathy registry, a prospective multicenter consecutive case registry for acute encephalopathy and febrile convulsive status epilepticus. Pediatric patients aged 0-18 years hospitalized and diagnosed with acute encephalopathy between January 2020 and August 2023 were included in this study. RESULTS: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) was the most common syndrome (36 cases, 27.5 %). SARS-CoV-2 was the most common pathogen (19 cases, 14.5 %), followed by influenza virus type A (15 cases, 11.5 %). Targeted temperature management was performed for 25 (69.4 %) of 36 patients with AESD; 5 (50.0 %) of 10 patients with hemorrhagic shock and encephalopathy; and only 1 (5.9 %) of 17 patients with mild encephalitis or encephalopathy with a reversible splenial lesion (MERS). High-dose corticosteroids were administered to 9 (90.0 %) of 10 patients with hemorrhagic shock and encephalopathy and 11 (30.6 %) of 36 patients with AESD. CONCLUSIONS: The primary causative pathogen of acute encephalopathy has changed to SARS-CoV-2. AESD remains the most common syndrome. Targeted temperature management is more, whereas high-dose corticosteroid therapy is less, frequently used. No specific treatment for mild encephalitis or encephalopathy with a reversible splenial lesion has been established.

  Corresponding, Feb. 2025, Journal of the neurological sciences, 469, 123377 - 123377, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Japanese guidelines for treatment of pediatric status epilepticus - 2023.

  Kenjiro Kikuchi, Ichiro Kuki, Masahiro Nishiyama, Yuki Ueda, Ryuki Matsuura, Tadashi Shiohama, Hiroaki Nagase, Tomoyuki Akiyama, Kenji Sugai, Kitami Hayashi, Kiyotaka Murakami, Hitoshi Yamamoto, Tokiko Fukuda, Mitsuru Kashiwagi, Yoshihiro Maegaki

  The updated definition of status epilepticus (SE) by the International League Against Epilepsy in 2015 included two critical time points (t1: at which the seizure should be regarded as an "abnormally prolonged seizure"; and t2: beyond which the ongoing seizure activity can pose risk of long-term consequences) to aid in diagnosis and management and highlights the importance of early treatment of SE more clearly than ever before. Although Japan has witnessed an increasing number of pre-hospital drug treatment as well as first- and second-line treatments, clinical issues have emerged regarding which drugs are appropriate. To address these clinical concerns, a revised version of the "Japanese Guidelines for the Treatment of Pediatric Status Epilepticus 2023" (GL2023) was published. For pre-hospital treatment, buccal midazolam is recommended. For in-hospital treatment, if an intravenous route is unobtainable, buccal midazolam is also recommended. If an intravenous route can be obtained, intravenous benzodiazepines such as midazolam, lorazepam, and diazepam are recommended. However, the rates of seizure cessation were reported to be the same among the three drugs, but respiratory depression was less frequent with lorazepam than with diazepam. For established SE, phenytoin/fosphenytoin and phenobarbital can be used for pediatric SE, and levetiracetam can be used in only adults in Japan. Coma therapy is recommended for refractory SE, with no recommended treatment for super-refractory SE. GL2023 lacks adequate recommendations for the treatment of nonconvulsive status epilepticus (NCSE). Although electrographic seizure and electrographic SE may lead to brain damages, it remains unclear whether treatment of NCSE improves outcomes in children. We plan to address this issue in an upcoming edition of the guideline.

  Feb. 2025, Brain & development, 47(1) (1), 104306 - 104306, English, International magazine

  [Refereed]

  Scientific journal


• Identification of Genetic Variants in Status Epilepticus Associated With Fever.

  Hiroaki Hanafusa, Hiroshi Yamaguchi, Naoya Morisada, Ming Juan Ye, Shizuka Oikawa, Shoichi Tokumoto, Masahiro Nishiyama, Kandai Nozu, Hiroaki Nagase

  PURPOSE: Status epilepticus associated with fever (SEF) is often encountered in pediatric emergency departments, and some patients develop neurological emergencies, such as acute encephalopathy (AE). Although numerous genetic variants of developmental and epileptic encephalopathy (DEE) have been reported, the frequency of these disease-associated variants of SEF is unknown. The first aim of this study was to investigate the associated genetic variants of SEF. The second aim was to compare the variations in genes between SEF and DEE. METHOD: This retrospective, clinical observational study included patients with SEF or DEE who visited Kobe University Hospital or Kobe University affiliated hospitals and provided consent for a genetic diagnosis of SEF or DEE between January 1, 2021, and December 31, 2022. FINDING: Fifteen patients with SEF and 27 patients with DEE consented to a genetic diagnosis and were included in the study. The detection rate of genetic variants was lower in patients with SEF (26.7%) than in those with DEE (63.0%), although there is no statistically significant difference (p = 0.05, Fisher's exact test). Analysis of patients with DEE revealed a wide variety of causative genes for DEE (16 different genes), whereas in SEF cases, only SCN1A variants were detected. CONCLUSION: Our study is the first to clarify the detection rates of different genetic variants in SEF. Patients with SEF may have less genetic involvement in the onset of epileptic seizures, compared to those with DEE.

  Feb. 2025, Brain and behavior, 15(2) (2), e70279, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Acute Encephalopathy Associated with Human Adenovirus Type 14 Infection in 7-Year-Old Girl, Japan.

  Shinsuke Mizuno, Yoshihiko Tanimoto, Ai Mori, Tomoaki Fuseya, Yusuke Ishida, Masahiro Nishiyama, Azusa Maruyama, Masashi Kasai

  Only 2 cases of human adenovirus type 14 (HAdV-14) have been reported in Japan since 1980. We report a 7-year-old girl with acute encephalopathy associated with HAdV-14 infection genetically similar to strains from the United States. The patient had not had contact with international travelers. HAdV-14 surveillance should be strengthened in Japan.

  Feb. 2025, Emerging infectious diseases, 31(2) (2), 377 - 379, English, International magazine

  [Refereed]

  Scientific journal


• Isoflurane Inhalation Therapy for the Management of Extremely Refractory Status Epilepticus in Case of Acute Encephalitis with Refractory, Repetitive Partial Seizures

  石田貴裕, 宮下徳久, 青木一憲, 上田拓耶, 西山将広, 丸山あずさ, 黒澤寛史

  (公社)日本小児科学会, 2025, 日本小児科学会雑誌, 129(6) (6), 781 - 787, Japanese

  [Refereed]


• Fulminant cerebellitis with Chiari malformation showing increased blood flow on arterial spin labeling imaging.

  Hajime Tanaka, Masahiro Nishiyama, Kento Soma, Takuya Ueda, Kazunori Aoki, Hiroshi Kurosawa, Azusa Maruyama

  Corresponding, 2025, Pediatrics international : official journal of the Japan Pediatric Society, 67(1) (1), e70074, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Elevated cerebrospinal fluid neuronal injury biomarkers within 24 hours of onset in infection-triggered acute encephalopathy compared to complex febrile seizures.

  Shizuka Oikawa, Hiroshi Yamaguchi, Masahiro Nishiyama, Tatsuhito Ito, Aoi Kawamura, Tomohiro Sameshima, Kento Soma, Takuya Ueda, Shoichi Tokumoto, Yusuke Ishida, Hiroshi Kurosawa, Kandai Nozu, Azusa Maruyama, Ryojiro Tanaka, Hiroaki Nagase

  OBJECTIVE: This study aimed to measure and compare cerebrospinal fluid neuronal injury biomarkers in the acute phase of complex febrile seizure (CFS) and infection-triggered acute encephalopathy (AE). Furthermore, we determined the pathogenesis of AE with biphasic seizures and late reduced diffusion (AESD). METHODS: Pediatric patients with febrile status epilepticus who visited Hyogo Prefectural Kobe Children's Hospital from November 1, 2016, to December 31, 2022, and whose cerebrospinal fluid samples were collected within 24 h of neurological symptom onset were included. Patients were classified as having CFS or infection-triggered AE according to their definitions. Patients with AE were further categorized into AESD or unclassified AE. Cerebrospinal fluid biomarkers (neuron-specific enolase, growth differentiation factor 15 [GDF-15], S100 calcium-binding protein B [S100B], glial fibrillary acidic protein, and tau protein were measured and compared among the groups. RESULTS: Total of 63 patients (45 with CFS and 18 with AE) were included. Among the AE patients, nine were classified as having AESD and nine as having unclassified AE. S100B levels were significantly higher in patients with AESD than in patients with CFS (485 pg/ml vs. 175.3 pg/ml) and were even higher in patients with AESD and neurological sequelae (702.4 pg/ml). GDF-15 levels were significantly elevated in patients with AE compared to patients with CFS (85.8 pg/ml vs. 23.6 pg/ml). CONCLUSIONS: The elevation of S100B suggests that activated astrocytes may be closely associated with the early pathology of AESD. Elevated GDF-15 levels in infection-triggered AE suggest the activation of defense mechanisms caused by stronger neurological injury.

  Nov. 2024, Journal of the neurological sciences, 466, 123238 - 123238, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Efficacy and safety of buccal midazolam for seizures outside the hospital: Real-world clinical experience.

  Takuya Ueda, Masahiro Nishiyama, Hiroshi Yamaguchi, Kento Soma, Yusuke Ishida, Azusa Maruyama, Kandai Nozu, Hiroaki Nagase

  INTRODUCTION: Buccal midazolam (buc MDL) is the first buccal mucosal delivery formulation applied for status epilepticus in Japan. Herein, we aimed to investigate the effectiveness and adverse events of buc MDL as a pre-hospital treatment for epileptic seizures in real-world clinical practice. METHODS: This study involved a retrospective review based on medical records. We included children who received buc MDL as pre-hospital treatment for epileptic seizures and were subsequently transported to the emergency department between April 2021 and November 2023. RESULTS: This study included 26 patients (136 episodes). The overall efficacy rate, which was defined as seizure cessation within 10 min after buc MDL administration with no recurrence within 30 min, was 43 %. Moreover, 70 % of the episodes did not require additional medications. None of the episodes required bag-mask ventilation or intubation following seizure cessation with buc MDL alone. The efficacy was decreased when buc MDL was administered longer than 15 min from seizure onset. Furthermore, the efficacy did not decrease as long as it was within 0.2-0.5 mg/kg, even if the dose was smaller than the appropriate dose for the specific age. CONCLUSIONS: The response rate was significantly higher in episodes where buc MDL was administered within 15 min. Additionally, there was no concern regarding respiratory depression with buc MDL alone.

  Corresponding, Nov. 2024, Brain & development, 46(10) (10), 332 - 338, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Two cases of COVID-19-related hemorrhagic shock and encephalopathy syndrome with different outcomes

  Keiichiro Toma, Kazunori Aoki, Hiroshi Kurosawa, Masahiro Nishiyama, Azusa Maruyama

  Corresponding, Elsevier BV, Sep. 2024, Brain and Development Case Reports, 2(3) (3), 100024 - 100024

  [Refereed]

  Scientific journal


• 脳保護のための小児神経集中治療

  西山 将広, 青木 一憲, 黒澤 寛史, 丸山 あずさ, 永瀬 裕朗

  (公社)日本小児科学会, Aug. 2024, 日本小児科学会雑誌, 128(8) (8), 1031 - 1044, Japanese

  [Refereed]


• Association of perinatal factors with neurodevelopmental referrals in a population-based cohort study in Japan.

  Yuki Kyono, Masahiro Nishiyama, Aoi Kawamura, Shizuka Oikawa, Shoichi Tokumoto, Hiroshi Yamaguchi, Kazumi Tomioka, Kandai Nozu, Hiroki Mishina, Hiroaki Nagase

  Although the causes of neurodevelopmental disorders remain unknown, several environmental risk factors have attracted considerable attention. We conducted a retrospective, longitudinal, population-based cohort study using data from infant health examinations of children born to mothers with pregnancies between April 1, 2014 and March 31, 2016 in Kobe City to identify the perinatal factors associated with neurodevelopmental referrals in 3-year-old children. There were 15,223 and 1283 children in the normal and referral groups, respectively. Neurodevelopmental referrals at the health checkup for 3-year-old children were significantly associated with the lack of social support during pregnancy (adjusted odds ratio [aOR] 1.99, 99% CI 1.14-3.45, p = 0.001), history of psychiatric consultation (aOR 1.56, 99% CI 1.10-2.22, p = 0.001), no social assistance post-delivery (aOR 1.49, 99% CI 1.03-2.16, p = 0.006), Edinburgh Post-natal Depression Scale (EPDS) score ≥ 9 (aOR 1.36, 99% CI 1.01-1.84, p = 0.008), infant gender (male) (aOR 2.51, 99% CI 2.05-3.06, p < 0.001), and cesarean delivery (aOR 1.39, 99% CI 1.11-1.75, p < 0.001). In conclusion, this exploratory study in the general Japanese population identified six perinatal factors associated with neurodevelopmental referrals in 3-year-old children: infant gender (male), cesarean section, maternal history of psychiatric consultation, EPDS score ≥ 9, lack of social support during pregnancy, and no social assistance post-delivery.

  Corresponding, Feb. 2024, Scientific reports, 14(1) (1), 3492 - 3492, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Clinical characteristics of SARS-CoV-2-associated encephalopathy in children: Nationwide epidemiological study.

  Mariko Kasai, Hiroshi Sakuma, Yuichi Abe, Ichiro Kuki, Yoshihiro Maegaki, Kei Murayama, Yuka Murofushi, Hiroaki Nagase, Masahiro Nishiyama, Akihisa Okumura, Yasunari Sakai, Hiroko Tada, Masashi Mizuguchi, Jun-Ichi Takanashi

  OBJECTIVE: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) sometimes triggers acute encephalopathy as a serious neurological complication in children. We previously reported the clinico-radiological findings of SARS-CoV-2-associated encephalopathy. The advent of the SARS-CoV-2 omicron variant led to a marked increase in pediatric patients with coronavirus disease 2019 (COVID-19); however, epidemiological changes with acute encephalopathy according to the emergence of SARS-CoV-2 have not yet been documented. Therefore, the present study investigated epidemiological differences in SARS-CoV-2-associated encephalopathy during the BA.1/BA.2 and BA.5 predominant periods and also between SARS-CoV-2-associated and non-SARS-CoV-2-associated encephalopathy. METHODS: We conducted a nationwide survey of SARS-CoV-2-associated encephalopathy in Japanese children between June and November 2022. We compared the present results during the BA.5 predominant period and previous findings during the BA.1/BA.2 predominant period. We also compared the clinico-radiological syndromes of encephalopathy between SARS-CoV-2-associated and non-SARS-CoV-2-associated encephalopathy. RESULTS: Although many patients with SARS-CoV-2-associated encephalopathy in the BA.5 predominant period had seizures as their initial symptoms, no significant differences were observed in the clinical features. Patients with SARS-CoV-2-associated encephalopathy had worse outcomes than those with non-SARS-CoV-2-associated encephalopathy (p-value = 0.003). Among 103 patients with SARS-CoV-2-associated encephalopathy, 14 (13.6%) had severe types of acute encephalopathy, namely, encephalopathy with acute fulminant cerebral edema (AFCE) and hemorrhagic shock and encephalopathy syndrome (HSES). Also, 28 (27.2%) patients with SARS-CoV-2-associated encephalopathy had poor outcome: severe neurological sequelae or death. Ninety-five patients (92.2%) were not vaccinated against SARS-CoV-2. CONCLUSIONS: In SARS-CoV-2-associated encephalopathy, high percentages of AFCE and HSES can result in poor outcomes.

  Jan. 2024, Journal of the neurological sciences, 457, 122867 - 122867, English, International magazine

  [Refereed]

  Scientific journal


• Response to: Is pancreatitis actually a phenotypic manifestation of Leigh syndrome?

  Kengo Nakashima, Ryosuke Bo, Hiroyuki Awano, Masahiro Nishiyama, Kazumoto Iijima

  2024, Pediatrics international : official journal of the Japan Pediatric Society, 66(1) (1), e15783, English, International magazine


• Association of early bedtime at 3 years of age with higher academic performance and better non-cognitive skills in elementary school.

  Masahiro Nishiyama, Yuki Kyono, Hiroshi Yamaguchi, Aoi Kawamura, Shizuka Oikawa, Shoichi Tokumoto, Kazumi Tomioka, Kandai Nozu, Hiroaki Nagase

  This study investigated the relationship between sleep habits in early childhood and academic performance and non-cognitive skills in the first grade. We retrospectively analyzed a longitudinal population-based cohort from birth through early childhood, up to elementary school, in Amagasaki City, Japan. The primary outcome was academic performance in the first grade. Other outcomes were self-reported non-cognitive skills. Overall, 4395 children were enrolled. Mean national language scores for children with bedtimes at 18:00-20:00, 21:00, 22:00, and ≥ 23:00 were 71.2 ± 19.7, 69.3 ± 19.4, 68.3 ± 20.1, and 62.5 ± 21.3, respectively. Multiple regression analysis identified bedtime at 3 years as a significant factor associated with academic performance. However, sleep duration was not significantly associated with academic performance. Bedtime at 3 years also affected non-cognitive skills in the first grade. Diligence decreased with a later bedtime (21:00 vs. 18:00-20:00; odds ratio [OR]: 1.98, 95% confidence interval [CI] 1.27-3.09; 22:00 vs. 18:00-20:00; OR: 2.15, 95% CI 1.37-3.38; ≥ 23:00 vs. 18:00-20:00; OR: 2.33, 95% CI 1.29-4.20). Thus, early bedtime at 3 years may be associated with a higher academic performance and better non-cognitive skills in the first grade. Optimum early-childhood sleep habits may positively impact academic future.

  Nov. 2023, Scientific reports, 13(1) (1), 20926 - 20926, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Dravet syndrome and hemorrhagic shock and encephalopathy syndrome associated with an intronic deletion of SCN1A.

  Hiroaki Hanafusa, Hiroshi Yamaguchi, Hidehito Kondo, Miwako Nagasaka, Ming Juan Ye, Shizuka Oikawa, Shoichi Tokumoto, Kazumi Tomioka, Masahiro Nishiyama, Naoya Morisada, Masafumi Matsuo, Kandai Nozu, Hiroaki Nagase

  OBJECTIVE: Hemorrhagic shock and encephalopathy syndrome (HSES) is a serious condition that requires intensive care and is associated with a high mortality rate. However, its pathogenesis remains unclear. In the present study, a genetic analysis was performed to determine the genetic background of patients with clinically suspected Dravet syndrome (DS) who developed HSES. METHODS: Whole exome sequencing was performed, followed by minigene analysis of the intron variant detected by whole exome sequencing to confirm its effect on splicing. RESULTS: Whole exome sequencing revealed a novel 21-bp deletion in intron 3 of SCN1A NM_001165963.4 (NC_000002.11:g.166073675_166073695del). This deletion was not found in the patient's parents and was proven to be de novo. Minigene analysis revealed an aberrant mRNA lacking 40 and 106 bp from the 5' end of exon 4 of SCN1A. Therefore, we diagnosed this case as DS due to the deletion in intron 3 of SCN1A. CONCLUSIONS: We report a case of DS with HSES caused by a 21-bp deletion in the intron of SCN1A that was confirmed by minigene analysis. The present case met Levin's criteria for HSES and the splicing analysis of SCN1A is an important finding. This study has important implications for understanding HSES pathogenesis.

  Jun. 2023, Brain & development, 45(6) (6), 317 - 323, English, International magazine

  [Refereed]

  Scientific journal


• Hyperkalemic periodic paralysis associated with a novel missense variant located in the inner pore of Nav1.4.

  Kazuki Segawa, Masahiro Nishiyama, Itsuki Mori, Tomoya Kubota, Masanori P Takahashi

  BACKGROUND: Hyperkalemic periodic paralysis (HyperPP) is an autosomal dominantly inherited disease characterized by episodic paralytic attacks with hyperkalemia, and is caused by mutations of the SCN4A gene encoding the skeletal muscle type voltage-gated sodium channel Nav1.4. The pathological mechanism of HyperPP was suggested to be associated with gain-of-function changes for Nav1.4 gating, some of which are defects of slow inactivation. CASE PRESENTATION & METHODS: We identified a HyperPP family consisting of the proband and his mother, who showed a novel heterozygous SCN4A variant, p.V792G, in an inner pore lesion of segment 6 in Domain II of Nav1.4. Clinical and neurophysiological evaluations were conducted for the proband and his mother. We explored the pathogenesis of the variant by whole-cell patch clamp technique using HEK293T cells expressing the mutant Nav1.4 channel. RESULTS: Functional analysis of Nav1.4 with the V792G mutation revealed a hyperpolarized shift of voltage-dependent activation and fast inactivation. Moreover, steady-state slow inactivation in V792G was impaired with larger residual currents in comparison with wild-type Nav1.4. CONCLUSION: V792G in SCN4A is a pathogenic variant associated with the HyperPP phenotype and the inner pore lesion of Nav1.4 plays a crucial role in slow inactivation.

  Lead, Apr. 2023, Brain & development, 45(4) (4), 205 - 211, English, International magazine

  [Refereed]

  Scientific journal


• Clinical and laboratory characteristics of complex febrile seizures in the acute phase: a case-series study in Japan.

  Tsukasa Tanaka, Hiroshi Yamaguchi, Yusuke Ishida, Kazumi Tomioka, Masahiro Nishiyama, Daisaku Toyoshima, Azusa Maruyama, Hiroki Takeda, Hiroshi Kurosawa, Ryojiro Tanaka, Kandai Nozu, Hiroaki Nagase

  BACKGROUND: Patients with complex febrile seizures (CFS) often display abnormal laboratory results, unexpectedly prolonged seizures, and/or altered consciousness after admission. However, no standardized values have been established for the clinical and laboratory characteristics of CFS in the acute phase, making the management of CFS challenging. This study aimed to determine the clinical and laboratory characteristics of children with CFS during the acute phase. In particular, the duration of impaired consciousness and the detailed distribution of blood test values were focused. METHODS: We retrospectively reviewed medical records of a consecutive pediatric cohort aged 6-60 months who were diagnosed with CFS and admitted to Kobe Children's Hospital between October 2002 and March 2017. During the study period, 486 seizure episodes with confirmed CFS were initially reviewed, with 317 seizure episodes included in the analysis. Detailed clinical and laboratory characteristics were summarized. RESULTS: Among 317 seizure episodes (296 children with CFS), 302 required two or fewer anticonvulsants to be terminated. In 296 episodes showing convulsive seizures, median seizure duration was 30.5 min. The median time from onset to consciousness recovery was 175 min. Impaired consciousness lasting > 6, 8, and 12 h was observed in 13.9%, 7.6%, and 1.9% patients with CFS, respectively. Additionally, the distribution of aspartate aminotransferase, lactate dehydrogenase, creatinine, and glucose were clarified with 3, 10, 50, 90, and 97 percentile values. CONCLUSION: This study detailed the clinical and laboratory findings of acute-phase CFS using the data of the largest 15-year consecutive cohort of children with CFS. These results provide important information for appropriate acute management of CFS.

  Jan. 2023, BMC neurology, 23(1) (1), 28 - 28, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Time course of serum cytokine level changes within 72 h after onset in children with acute encephalopathy and febrile seizures.

  Kazumi Tomioka, Masahiro Nishiyama, Shoichi Tokumoto, Hiroshi Yamaguchi, Kazunori Aoki, Yusuke Seino, Daisaku Toyoshima, Hiroshi Kurosawa, Hiroko Tada, Hiroshi Sakuma, Kandai Nozu, Azusa Maruyama, Ryojiro Tanaka, Kazumoto Iijima, Hiroaki Nagase

  BACKGROUND: Cytokine levels have been measured in acute encephalopathy (AE) to determine its pathology or as a diagnostic biomarker to distinguish it from febrile seizures (FS); however, the dynamics of cytokine level changes have not yet been fully captured in these two neurological manifestations. Thus, we aimed to explore the time course of serum cytokine level changes within 72 h after onset in AE and FS. METHODS: We retrospectively measured cytokine level in residual serum samples at multiple timepoints in seven children whose final diagnoses were AE or FS. RESULTS: The levels of 13 cytokines appeared to increase immediately after onset and peaked within 12-24 h after onset: interleukin (IL)-1β, IL-4 IL-5, IL-6, IL-8, IL-10, IL-17, eotaxin, fibroblast growth factor, granulocyte colony-stimulating factor, interferon gamma, interferon-inducible protein-10, and macrophage chemoattractant protein-1. There were no dynamic changes in the levels of three cytokines (IL-1 receptor agonist, macrophage inflammatory protein-1α, and platelet-derived growth factor-bb) 72 h after onset. Levels of some cytokines decreased to around control levels within 48 h after onset: IL-1β, IL-4, IL-5, IL-17, fibroblast growth factor, and interferon gamma. The levels of most cytokines appeared to be higher in AE, especially in hemorrhagic shock encephalopathy syndrome, than in FS. CONCLUSIONS: Cytokine levels in both AE and FS change dynamically, such as the levels of several cytokines increased within a few hours after onset and decreased at 12-24 h after onset. Therefore, it will be desirable to make clinical decisions regarding the administration of anti-inflammatory therapy in 24 h after onset in AE.

  Jan. 2023, BMC neurology, 23(1) (1), 7 - 7, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Anti-MOG antibody-positive meningoencephalitis without demyelinating lesions.

  Tetsuya Yamane, Takuya Ueda, Masahiro Nishiyama, Hiroto Hongo, Yusuke Ishida, Azusa Maruyama

  Corresponding, Japan Pediatric Society, 2023, Pediatrics international : official journal of the Japan Pediatric Society, 65(1) (1), e15612, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Timing of therapeutic interventions against infection-triggered encephalopathy syndrome: a scoping review of the pediatric literature.

  Hiroaki Nagase, Hiroshi Yamaguchi, Shoichi Tokumoto, Yusuke Ishida, Kazumi Tomioka, Masahiro Nishiyama, Kandai Nozu, Azusa Maruyama

  Our goal was to conduct a scoping review of the literature on the treatment of infection-triggered encephalopathy syndrome/acute encephalopathy in children, focusing on treatment targets and treatment initiation timing. We performed literature searches using PubMed for articles reporting treatments of infection-triggered encephalopathy syndrome/acute encephalopathy. We included articles describing specific treatments for acute encephalopathy with control groups. For the purpose of searching new therapies only experimentally tried in the case series, we also included case series studies without control groups in this review, if the studies contained at least two cases with clear treatment goals. Therapies were classified based on their mechanisms of action into brain protection therapy, immunotherapy, and other therapies. We operationally categorized the timing of treatment initiation as T1 (6-12 h), T2 (12-24 h), T3 (24-48 h), and T4 (>48 h) after the onset of seizures and/or impaired consciousness. Thirty articles were included in this review; no randomized control study was found. Eleven retrospective/historical cohort studies and five case-control studies included control groups with or without specific therapies or outcomes. The targeted conditions and treatment timing varied widely across studies. However, the following three points were suggested to be effective in multiple studies: (1) Careful seizure management and targeted temperature management within 12 h (T1) of onset of febrile seizure/prolonged impaired consciousness without multiple organ failure may reduce the development of acute encephalopathy with biphasic seizures and late reduced diffusion; (2) immunotherapy using corticosteroids, tocilizumab, or plasma exchange within 24 h (T1-T2) of onset of acute necrotizing encephalopathy may reduce sequelae; and (3) anakinra therapy and ketogenic diet demonstrate little evidence of neurologic sequelae reduction, but may reduce seizure frequency and allow for weaning from barbiturates, even when administered weeks (T4) after onset in children with febrile infection-related epilepsy syndrome. Although available studies have no solid evidence in the treatment of infection-triggered encephalopathy syndrome/acute encephalopathy, this scoping review lays the groundwork for future prospective clinical trials.

  2023, Frontiers in neuroscience, 17, 1150868 - 1150868, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Comparison of neurological manifestation in children with and without coronavirus 2019 experiencing seizures with fever.

  Hiroto Hongo, Masahiro Nishiyama, Takuya Ueda, Yusuke Ishida, Masashi Kasai, Ryojiro Tanaka, Hiroaki Nagase, Azusa Maruyama

  Whether neurologic symptoms due to SARS-CoV-2 differ from those of non-SARS-CoV-2 viral infection is unclear. We aimed to describe these neurological manifestations and compare the clinical characteristics and treatments in children with seizures and fever with or without COVID-19. We retrospectively analyzed data from 105 hospitalized children (<18 years) with clinical seizures and fever between September 2021 and August 2022. We compared the clinical characteristics and treatments between the COVID-19 (n = 20) and non-COVID-19 (n = 85) groups. Patients with COVID-19 were older than those without (32.5 [20-86] months vs. 20 [16-32] months, p = 0.029). Seizure type and duration and impaired consciousness duration did not differ between groups. Six and 32 patients experienced status epilepticus lasting 30 min in the COVID-19 and non-COVID-19 groups, respectively. Most treatments did not differ between groups; however, electroencephalography was used less frequently for COVID-19. Neurological sequelae occurred in one and four patients in the COVID-19 and non-COVID-19 groups, respectively. In conclusion, seizures with fever due to SARS-CoV-2 were more common in older children. Seizure characteristics and neurologic sequelae did not differ in children with and those without COVID-19. In general, electroencephalography was used less during COVID-19 for infection control measures.

  Corresponding, 2023, Epilepsy & behavior reports, 24, 100625 - 100625, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Corrigendum to: "Detailed clinical course of fatal acute encephalopathy in children" [Brain Dev. 41(8) (2019) 691-698].

  Kazumi Tomioka, Masahiro Nishiyama, Hiroaki Nagase, Yusuke Ishida, Tsukasa Tanaka, Shoichi Tokumoto, Hiroshi Yamaguchi, Daisaku Toyoshima, Azusa Maruyama, Kyoko Fujita, Kazunori Aoki, Yusuke Seino, Kandai Nozu, Noriyuki Nishimura, Hiroshi Kurosawa, Kazumoto Iijima

  Nov. 2022, Brain & development, 44(10) (10), 771 - 772, English, International magazine


• The transcription factor Hhex regulates inflammation-related genes in microglia.

  Risa Sakate, Masahiro Nishiyama, Yu Fukuda, Shiho Kitaoka, Tomoyuki Furuyashiki

  Microglia have diverse physiological and pathological functions. However, the transcriptional mechanisms remain elusive. Here we sought new transcription factors relevant to microglial functions from the microglial transcriptome of stressed mice and evaluated their roles in primary microglia. TLR2 and TLR4 agonists increased Rel, Atf3, and Cebpb and decreased Hhex in primary microglia as repeated social defeat stress. Although Hhex was not studied in microglia, TLR2 and TLR4 agonists decreased Hhex, and Hhex overexpression attenuated TLR4-increased expression of inflammation-related genes. These findings suggest that Hhex negatively regulates inflammation-related genes in microglia and that TLR2/4 activation reduces Hhex, facilitating TLR4-mediated neuroinflammation.

  Jul. 2022, Journal of pharmacological sciences, 149(3) (3), 166 - 171, English, Domestic magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Behavioral Therapy for Children with Avoidant/Restrictive Food Intake Disorder Dependent on Tube or Oral Enteral Nutrient Formula: A Feasibility Study.

  Kazumi Tomioka, Masahiro Nishiyama, Shoichi Tokumoto, Hiroshi Yamaguchi, Kandai Nozu, Hiroaki Nagase

  In children with eating disorders, nutritional status and growth may depend on enteral nutrient formula. Ultimately, its goal is to introduce or reintroduce oral feeding. Japanese research on the treatment of tube or oral formula-dependent children is scarce. This study determined the feasibility of behavioral therapy for children with avoidant/restrictive food intake disorder and dependency on the tube or oral enteral nutrient formula in Japan. Medical records of children diagnosed with this disorder, dependent on the tube or oral enteral nutrient formula and who had received behavioral therapy intervention to withdraw from the formula were retrospectively investigated. We collected their characteristics at first visit and the caloric percentage from oral food intake six months after starting the treatment. In total, four patients (age range: 2-5 years) participated in this study. The feeding routes employed before the intervention were a nasogastric tube for one patient, a gastrostomy bottom for the other patient, and oral formula for the remaining patients (i.e., two children). At the sixth month of the behavioral treatment, none of the patients needed the formula, and the caloric percentage of required nutrition from oral food intake was 100%. Our data demonstrate that this behavioral therapy is feasible for children with avoidant/restrictive food intake disorder dependent on the tube or oral formula in Japan.

  Kobe University School of Medicine, Mar. 2022, The Kobe journal of medical sciences, 67(4) (4), E155-E160 - 160, English, Domestic magazine

  [Refereed]

  Research institution

  Link to Kobe Univ. Repository (Kernel)


• Prognostic effects of treatment protocols for febrile convulsive status epilepticus in children.

  Shoichi Tokumoto, Masahiro Nishiyama, Hiroshi Yamaguchi, Kazumi Tomioka, Yusuke Ishida, Daisaku Toyoshima, Hiroshi Kurosawa, Kandai Nozu, Azusa Maruyama, Ryojiro Tanaka, Kazumoto Iijima, Hiroaki Nagase

  BACKGROUND: Febrile status epilepticus is the most common form of status epilepticus in children. No previous reports compare the effectiveness of treatment strategies using fosphenytoin (fPHT) or phenobarbital (PB) and those using anesthetics as second-line anti-seizure medication for benzodiazepine-resistant convulsive status epilepticus (CSE). We aimed to examine the outcomes of various treatment strategies for febrile convulsive status epilepticus (FCSE) in a real-world setting while comparing the effects of different treatment protocols and their presence or absence. METHODS: This was a single-center historical cohort study that was divided into three periods. Patients who presented with febrile convulsive status epilepticus for ≥60 min even after the administration of at least one anticonvulsant were included. During period I (October 2002-December 2006), treatment was performed at the discretion of the attending physician, without a protocol. During period II (January 2007-February 2013), barbiturate coma therapy (BCT) was indicated for FCSE resistant to benzodiazepines. During period III (March 2013-April 2016), BCT was indicated for FCSE resistant to fPHT or PB. RESULTS: The rate of electroencephalogram monitoring was lower in period I than period II+III (11.5% vs. 85.7%, p<0.01). Midazolam was administered by continuous infusion more often in period I than period II+III (84.6% vs. 25.0%, p<0.01), whereas fPHT was administered less often in period I than period II+III (0% vs. 27.4%, p<0.01). The rate of poor outcome, which was determined using the Pediatric Cerebral Performance Category scale, was higher in period I than period II+III (23.1% vs. 7.1%, p=0.03). The rate of poor outcome did not differ between periods II and III (4.2% vs. 11.1%, p=0.40). CONCLUSIONS: While the presence of a treatment protocol for FCSE in children may improve outcomes, a treatment protocol using fPHT or PB may not be associated with better outcomes.

  Mar. 2022, BMC neurology, 22(1) (1), 77 - 77, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Growth and differentiation factor-15 as a potential prognostic biomarker for status-epilepticus-associated-with-fever: A pilot study.

  Hiroshi Yamaguchi, Masahiro Nishiyama, Kazumi Tomioka, Hiroto Hongo, Shoichi Tokumoto, Yusuke Ishida, Daisaku Toyoshima, Hiroshi Kurosawa, Kandai Nozu, Azusa Maruyama, Ryojiro Tanaka, Hiroaki Nagase

  OBJECTIVE: Biomarkers predicting poor outcomes of status-epilepticus-associated-with-fever (SEF) at an early stage may contribute to treatment guidance. However, none have been reported thus far. We investigated the dynamics of serum growth and differentiation factor (GDF)-15 after seizure onset in patients with SEF and determined whether GDF-15 can predict poor outcomes, particularly in the first 6 h after seizure onset. METHODS: We enrolled 37 pediatric patients with SEF and eight patients with simple febrile seizures (SFS) and collected their blood samples within 24 h of seizure onset and eight febrile control patients between March 1, 2017 and September 30, 2020. All patients were aged ≤15 years. RESULTS: In the SEF group, the median post-seizure serum GDF-15 values were 1,065 (<6h), 2,720 (6-12 h), and 2,411 (12-24 h) pg/mL. The median serum GDF-15 in the first 6 h was measured in patients with SEF without a significant past medical history (n = 21) and was found to be statistically significantly higher (1,587 pg/mL) than in the febrile control (551 pg/mL) and SFS (411 pg/mL) groups. The median serum GDF-15 was statistically significantly higher in patients with SEF with sequelae (n = 5) and patients with acute encephalopathy with biphasic seizures/reduced diffusion/hemorrhagic shock and encephalopathy syndrome (n = 6) than in patients with SEF without sequelae (n = 16) (15,898 vs 756 pg/mL) and patients with prolonged FS (n = 15) (9,448 vs 796 pg/mL). CONCLUSIONS: This study demonstrates the dynamics of serum GDF-15 in patients with SEF and indicates the potential of GDF-15 as an early predictor of poor outcomes.

  Mar. 2022, Brain & development, 44(3) (3), 210 - 220, English, International magazine

  [Refereed]

  Scientific journal


• Frequent recurrence of pancreatitis in a patient with Leigh syndrome.

  Kengo Nakashima, Ryosuke Bo, Hiroyuki Awano, Masahiro Nishiyama, Kazumoto Iijima

  Jan. 2022, Pediatrics international : official journal of the Japan Pediatric Society, 64(1) (1), e15021, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Prevalence of Wilson disease based on genome databases in Japan.

  Hiroshi Yamaguchi, Hiroaki Nagase, Shoichi Tokumoto, Kazumi Tomioka, Masahiro Nishiyama, Hiroki Takeda, Takeshi Ninchoji, China Nagano, Kazumoto Iijima, Kandai Nozu

  BACKGROUND: Wilson disease (WD) is an autosomal recessive disorder caused by mutations in the ATP7B gene. In 1984, Scheinberg and Sternlieb estimated the prevalence of WD to be 1:30,000. However, recent epidemiological studies have reported increasing prevalence rates in different populations. The carrier frequency of ATP7B variants and the prevalence of WD in the Japanese population have not been reported using multiple databases. METHODS: Multiple public databases were used. First, we included mutations in the ATP7B gene that were registered in the Human Gene Mutation Database (HGMD) Professional, where 885 ATP7B variants were identified as pathogenic. Next, we investigated the allele frequencies of these 885 variants in Japanese individuals using the Human Genetic Variation Database (HGVD) and the Japanese Multi Omics Reference Panel (jMorp). RESULTS: Of the 885 variants of ATP7B, 7 and 12 missense and nonsense variants, 0 and 3 splicing variants, and 0 and 2 small deletions were found in the HGVD and in jMorp, respectively. The total allele frequencies of the ATP7B mutations were 0.011 in the HGVD and 0.014 in the jMorp. According to these data, the carrier frequencies were 0.022 (2.2%) and 0.028 (2.8%), respectively, and patient frequencies were 0.000121 (1.21/10,000 individuals) and 0.000196 (1.96/10,000 individuals), respectively. CONCLUSION: This is the first study to report the carrier frequency of ATP7B variants and the prevalence of WD in Japan using multiple databases. The calculated prevalence of WD was comparatively higher than that of previous reports, indicating previous underdiagnosis or the existence of less severe phenotypes.

  Wiley, Aug. 2021, Pediatrics international : official journal of the Japan Pediatric Society, 63(8) (8), 918 - 922, English, International magazine

  [Refereed]

  Scientific journal


• Early steroid pulse therapy for children with suspected acute encephalopathy: An observational study.

  Yusuke Ishida, Masahiro Nishiyama, Hiroshi Yamaguchi, Kazumi Tomioka, Hiroki Takeda, Shoichi Tokumoto, Daisaku Toyoshima, Azusa Maruyama, Yusuke Seino, Kazunori Aoki, Kandai Nozu, Hiroshi Kurosawa, Ryojiro Tanaka, Kazumoto Iijima, Hiroaki Nagase

  ABSTRACT: Steroid pulse therapy is widely used to treat virus-associated acute encephalopathy, especially the cytokine storm type; however, its effectiveness remains unknown. We sought to investigate the effectiveness of early steroid pulse therapy for suspected acute encephalopathy in the presence of elevated aspartate aminotransferase (AST) levels.We enrolled children admitted to Hyogo Children's Hospital between 2003 and 2017 with convulsions or impaired consciousness accompanied by fever (temperature >38°C). The inclusion criteria were: refractory status epilepticus or prolonged neurological abnormality or hemiplegia at 6 hours from onset, and AST elevation >90 IU/L within 6 hours of onset. We excluded patients with a neurological history. We compared the prognosis between the groups with or without steroid pulse therapy within 24 hours. A good prognosis was defined as a Pediatric Cerebral Performance Category Scale (PCPC) score of 1-2 at the last evaluation, within 30 months of onset. Moreover, we analyzed the relationship between prognosis and time from onset to steroid pulse therapy.Fifteen patients with acute encephalopathy and 5 patients with febrile seizures were included in this study. Thirteen patients received steroid pulse therapy within 24 hours. There was no between-group difference in the proportion with a good prognosis. There was no significant correlation between PCPC and timing of steroid pulse therapy (rs = 0.253, P = .405). Even after excluding 2 patients with brainstem lesions, no significant correlation between PCPC and steroid pulse therapy timing (rs = 0.583, P = .060) was noted. However, the prognosis tended to be better in patients who received steroid pulse therapy earlier.Steroid pulse therapy within 24 hours did not improve the prognosis in children with suspected acute encephalopathy associated with elevated AST. Still, even earlier administration of treatment could prevent the possible neurological sequelae of this condition.

  Lippincott Williams & Wilkins, Jul. 2021, Medicine, 100(30) (30), e26660, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Prediction of AESD and neurological sequelae in febrile status epilepticus.

  Masahiro Nishiyama, Yusuke Ishida, Hiroshi Yamaguchi, Shoichi Tokumoto, Kazumi Tomioka, Hiroto Hongo, Daisaku Toyoshima, Azusa Maruyama, Hiroshi Kurosawa, Ryojiro Tanaka, Kandai Nozu, Kazumoto Iijima, Hiroaki Nagase

  OBJECTIVE: The clinical prediction rule (CPR) for acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) was developed with an area under the receiver operating characteristic curve (AUC) of 0.95 - 0.96. Our objective was to verify the AESD CPR in a new cohort and compare the utilities of three CPRs of acute encephalopathy: the Tada, Yokochi, and Nagase criteria. METHODS: We reviewed the clinical data and medical charts of 580 consecutive patients (aged < 18 years) with febrile convulsive status epilepticus lasting for ≥ 30 min in 2002 - 2017 and measured the performance of the CPRs in predicting AESD and sequelae. RESULTS: The CPRs predicted AESD with an AUC of 0.84 - 0.88. The Tada criteria predicted AESD with a positive predictive value (PPV) of 0.25 and a negative predictive value (NPV) of 0.99. The Yokochi criteria predicted AESD with a PPV and NPV of 0.20 and 0.95, respectively, after 12 h. The Nagase criteria predicted AESD with a PPV and NPV of 0.14 and 1.00, respectively, after 6 h. The PPVs of the Tada, Yokochi, and Nagase criteria for sequelae were 0.28, 0.28, and 0.17, respectively; the corresponding NPVs were 0.97, 0.95, and 0.98, respectively. CONCLUSIONS: The effectiveness of the AESD CPR in a new cohort was lower than that in the derivation study. CPRs are not sufficient as diagnostic tests, but they are useful as screening tests. The Nagase criteria are the most effective for screening among the three CPRs due to their high NPV and swiftness.

  May 2021, Brain & development, 43(5) (5), 616 - 625, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Early non-convulsive seizures are associated with the development of acute encephalopathy with biphasic seizures and late reduced diffusion

  Azusa Maruyama, Shoichi Tokumoto, Hiroshi Yamaguchi, Yusuke Ishida, Tsukasa Tanaka, Kazumi Tomioka, Masahiro Nishiyama, Kyoko Fujita, Daisaku Toyoshima, Hiroaki Nagase

  INTRODUCTION: Children with either febrile seizure or acute encephalopathy exhibit seizures and/or impaired consciousness accompanied by fever of unknown etiology (SICF). Among children with SICF, we previously reported those who have refractory status epilepticus or prolonged neurological abnormalities with normal AST levels are at a high risk for the development of acute encephalopathy with biphasic seizures and late reduced diffusion (AESD), considered to be caused by excitotoxicity. Non-convulsive seizures (NCS) are common in critically ill children and cause excitotoxic neuronal injury. The aim of this study was to elucidate the prevalence of NCS in the acute phase of children at a high risk for developing AESD and the relationship between NCS in the acute phase and neurological outcomes. METHODS: We studied 137 children with SICF at a high risk for developing AESD and who underwent continuous electroencephalogram monitoring (cEEG) upon admission to a tertiary pediatric care center at Hyogo Prefectural Kobe Children's Hospital between October 2007 and August 2018. Patient characteristics and outcomes were compared between patients with NCS and without NCS. RESULTS: Of the 137 children, NCS occurred in 30 children; the first NCS were detected in cEEG at the beginning in 63.3%, during the first hour in 90%, and within 12 h in 96.7%. Neurological sequelae were more common in NCS patients (20.0%) than in non-NCS patients (1.9%; p = 0.001). Five in 30 NCS patients (16.7%) and 3 in 107 non-NCS patients (2.8%) developed AESD (p = 0.013). CONCLUSION: The occurrence of NCS is associated with subsequent neurological sequelae, especially the development of AESD.

  Elsevier BV, Apr. 2021, Brain and Development, 43(4) (4), 548 - 555, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Incidence of Kawasaki disease before and during the COVID-19 pandemic: a retrospective cohort study in Japan.

  Kyohei Iio, Kousaku Matsubara, Chisato Miyakoshi, Kunitaka Ota, Rika Yamaoka, Junji Eguchi, Osamu Matsumura, Takahiro Okutani, Ikuyo Ueda, Masahiro Nishiyama

  BACKGROUND: Epidemiological studies in Kawasaki disease (KD) have suggested infectious aetiology. During the COVID-19 pandemic, measures for mitigating SARS-CoV-2 transmission also suppress the circulation of other contagious microorganisms. The primary objective is to compare the number and incidence of KD before and during the COVID-19 pandemic in Japan, and the secondary objective is to investigate temporal association between the KD epidemiology and activities of SARS-CoV-2 and other viral and bacterial infections. METHODS: A retrospective cohort study was conducted between 2016 and 2020 in Kobe, Japan. We collected information of hospitalised KD children in Kobe. Child population was identified through the resident registry system. Activity of COVID-19 and 11 other infectious diseases was derived from a public health monitoring system. Monthly change of KD incidence was analysed using a difference-in-difference regression model. RESULTS: Throughout the study period, 1027 KD children were identified. KD had begun to decline in April 2020, coinciding with the beginning of the COVID-19 pandemic. The number of KD cases (n=66) between April and December 2020 was 40% of the average in the same period in 2016-2019 (165/year). Annual KD incidence was 315, 300, 353, 347 and 188/100 000 children aged 0-4 years in 2016-2020, respectively. The difference-in-difference value of KD incidence was significantly reduced in the fourth quarter in 2020 (-15.8, 95% CI -28.0 to -3.5), compared with that in 2016-2019. Sentinel surveillance showed a marked decrease of all infectious diseases except exanthema subitum after the beginning of the COVID-19 pandemic. There were 86 COVID-19 cases aged <10 years and no KD children associated with COVID-19. CONCLUSION: This study showed that the number and incidence of KD was dramatically reduced during the COVID-19 pandemic in Japan. This change was temporally associated with decreased activities of various infectious diseases other than COVID-19, supporting the hypothesis of infection-triggered pathogenesis in KD.

  Last, 2021, BMJ paediatrics open, 5(1) (1), e001034, English, International magazine

  [Refereed]

  Scientific journal


• Elevated cytokine, chemokine, and growth and differentiation factor-15 levels in hemorrhagic shock and encephalopathy syndrome: A retrospective observational study

  Hiroshi Yamaguchi, Masahiro Nishiyama, Shoichi Tokumoto, Yusuke Ishida, Kazumi Tomioka, Kazunori Aoki, Yusuke Seino, Daisaku Toyoshima, Hiroki Takeda, Hiroshi Kurosawa, Hiroshi Sakuma, Hiroko Tada, Kandai Nozu, Azusa Maruyama, Ryojiro Tanaka, Kazumoto Iijima, Hiroaki Nagase

  Patients with hemorrhagic shock and encephalopathy syndrome (HSES) have a high early mortality rate, which may be caused by a 'cytokine storm'. However, there is little information on how cytokines and chemokines change over time in these patients. We aimed to describe the characteristics of HSES by examining changes in serum biomarker levels over time. Six patients with HSES were included. We retrospectively evaluated their clinical course and imaging/laboratory data. We measured serum levels of multiple cytokines [interleukin 1β (IL-1β), IL-2, IL-4, IL-6, IL-10, IL-17, interferon-gamma, and tumor necrosis factor alpha], chemokines (IL-8, monocyte chemoattractant protein-1, interferon-inducible protein-10), and growth and differentiation factor (GDF)-15. The highest cytokine and chemokine levels were noted in the first 24 h, and decreased thereafter. The GDF-15 level was markedly high. Cytokine, chemokine, and GDF-15 levels were significantly higher in patients with HSES than in controls in the first 24 h, except for IL-2 and IL-4. Patients with HSES have high inflammatory cytokine and chemokine levels, a high GDF-15 level in the first 24 h, and high lactate levels. Our study provides new insights on the pathophysiology of HSES, a detailed clinical picture of patients with HSES, and potential biomarkers.

  Elsevier BV, Jan. 2021, Cytokine, 137, 155324 - 155324, English, International magazine

  [Refereed]

  Scientific journal


• Progressive length‐dependent polyneuropathy in xeroderma pigmentosum group A

  Yukio Tsuji, Takehiro Ueda, Kenji Sekiguchi, Masahiro Nishiyama, Fumio Kanda, Chikako Nishigori, Tatsushi Toda, Riki Matsumoto

  BACKGROUND: In this study, we aimed to investigate the progression of peripheral nervous system involvement in xeroderma pigmentosum group A (XP-A). METHODS: We performed nerve conduction studies in 17 genetically confirmed XP-A patients and conducted follow-ups. Of these patients we also analyzed gray matter volume (GMV) using brain MRI and assessed the severity score of clinical and skin manifestation. RESULTS: We found significant reduction in the motor and sensory nerve action potential amplitude and mild reduction in conduction velocity. These findings were predominant in sensory nerves and the lower limbs, were observed since early childhood, and gradually deteriorated with age. CONCLUSIONS: The electrophysiological characteristics of XP-A patients are consistent with length-dependent axonal polyneuropathy and there is progressive deterioration from early childhood.

  Wiley, Oct. 2020, Muscle & Nerve, 62(4) (4), 534 - 540, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Seizure prevalence in children aged up to 3 years: a longitudinal population-based cohort study in Japan

  Masahiro Nishiyama, Hiroshi Yamaguchi, Yusuke Ishida, Kazumi Tomioka, Hiroki Takeda, Noriyuki Nishimura, Kandai Nozu, Hiroki Mishina, Kazumoto Iijima, Hiroaki Nagase

  To investigate the prevalence of seizures/febrile seizures in children up to 3 years of age and examine the effects of gestational age at birth on the risk for febrile seizures. Retrospective longitudinal population-based cohort study. Kobe City public health center , Kobe, Japan, from 2010 to 2018. Children who underwent a medical check-up at 3 years of age. Information regarding seizures was collected from the parents of 96 014 children. We identified the occurrence of seizure/febrile seizure in 74 017 children, whose gestational ages at birth were noted. We conducted a multivariate analysis with the parameter, gestational age at birth, to analyse the risk of seizure. We also stratified the samples by sex and birth weight (<2500 g or not) and compared the prevalence of seizure between those with the term and late preterm births. The prevalence of seizure was 12.1% (11.8%–12.3%), 13.2% (12.2%–14.4%), 14.6% (12.4%–17.7%) and 15.7% (10.5%–22.8%) in children born at 37–41, 34–36, 28–33 and 22–27 gestational weeks, respectively. The prevalence of febrile seizures was 9.0% (8.8%–9.2%), 10.5% (9.5%–11.5%), 11.8% (9.7%–14.5%) and 11.2% (6.9%–17.7%) in children born at 37–41, 34–36, 28–33 and 22–27 gestational weeks, respectively. Male was an independent risk factor for seizures (OR: 1.15, 95% CI 1.09 to 1.20; absolute risk increase 0.014, 95% CI 0.010 to 0.019) and febrile seizures (OR: 1.21, 95% CI 1.15 to 1.28; absolute risk increase 0.016, 95% CI 0.012 to 0.020), respectively. Late preterm birth was not associated with an increased risk of seizure/febrile seizure. Although very preterm birth may increase the risk of seizure/febrile seizure, the risk associated with late preterm birth is considerably small and less than that associated with male.

  BMJ, Sep. 2020, BMJ Open, 10(9) (9), e035977 - e035977, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Thiamylal anaesthetic therapy for febrile refractory status epilepticus in children.

  Yusuke Ishida, Masahiro Nishiyama, Hiroshi Yamaguchi, Kazumi Tomioka, Tsukasa Tanaka, Hiroki Takeda, Shoichi Tokumoto, Daisaku Toyoshima, Azusa Maruyama, Yusuke Seino, Kazunori Aoki, Kandai Nozu, Noriyuki Nishimura, Hiroshi Kurosawa, Kazumoto Iijima, Hiroaki Nagase

  PURPOSE: To evaluate barbiturate anaesthetic therapy using thiamylal for febrile refractory status epilepticus (fRSE) in children. METHODS: This was a review of a prospectively-collected database between April 2012-March 2016 for fRSE cases treated with thiamylal anaesthetic therapy in a single paediatric hospital in Japan. The sample comprised 23 children (median age, 23 months) with fRSE that underwent thiamylal anaesthetic therapy for convulsive seizures lasting longer than 60 min, sustained after intravenous administration of benzodiazepine and non-benzodiazepine anticonvulsants. The intervention comprised protocol-based thiamylal anaesthetic therapy with bolus administration. We measured the dose and time required to achieve the burst suppression pattern (BSP) on electroencephalography, seizure recurrence, death, neurological sequelae, and complications. RESULTS: All patients except one reached the BSP. The thiamylal median dose until reaching the BSP was 27.5 mg/kg, and the median time from thiamylal administration to reaching the BSP was 109.5 min. There was one case of immediate treatment failure and one of withdrawal seizure, but no breakthrough seizure. No deaths occurred during treatment, and neurological sequelae occurred in four cases (17%). Vasopressors were administered in all cases. Other complications included 11 cases of pneumonia and one of enterocolitis. CONCLUSION: We revealed the time and dose required to reach the BSP with thiamylal anaesthetic therapy using bolus administration in children. Our results suggested that reaching the BSP with bolus administration requires markedly less time than without bolus administration, rarely causes seizure recurrence in paediatric fRSE, and causes haemodynamic dysfunction and infections as often as observed without bolus administration.

  May 2020, Seizure, 80, 12 - 17, English, International magazine

  [Refereed]

  Scientific journal


• 抗アセチルコリン受容体抗体価が発症時0.3nmol/Lから最大15.4nmol/Lに上昇した眼筋型重症筋無力症

  洪 聖媛, 西山 将広, 田中 司, 永井 正志, 坊 亮輔, 石田 悠介, 冨岡 和美, 粟野 宏之, 永瀬 裕朗, 飯島 一誠

  Corresponding, (株)日本小児医事出版社, Apr. 2020, 小児科臨床, 73(4) (4), 499 - 502, Japanese

  [Refereed]


• Acute rhabdomyolysis following viral infection with coxsackie A4 in a 50-day-old infant with Fukuyama congenital muscular dystrophy.

  Hiroshi Yamaguchi, Mariko Taniguchi-Ikeda, Hiroaki Nagase, Yusuke Ito, Shoichi Tokumoto, Daisaku Toyoshima, Sarantuya Enkhjargal, Masahiro Nishiyama, Hiroyuki Awano, Hiroshi Kurosawa, Masashi Kasai, Azusa Maruyama, Kazumoto Iijima

  BACKGROUND: Fukuyama congenital muscular dystrophy (FCMD), which is characterized by generalized muscle weakness, hypotonia, and motor delay during early infancy, gradually progresses with advanced age. Although acute rhabdomyolysis following infection in patients with FCMD has occasionally been reported, no studies have investigated rhabdomyolysis following viral infection in FCMD patients during early infancy. CASE REPORT: We report the case of a 50-day-old girl with no apparent symptoms of muscular dystrophy who developed severe acute rhabdomyolysis caused by viral infection, resulting in quadriplegia and respiratory failure therefore requiring mechanical ventilation. Brain magnetic resonance imaging incidentally showed the typical characteristics of FCMD, and FCMD was confirmed by genetic analysis, which revealed a 3-kb retrotransposon insertion in one allele of the fukutin gene and a deep intronic splicing variant in intron 5 in another allele. The virus etiology was confirmed to be Coxsackie A4. CONCLUSION: We report a severe case of acute rhabdomyolysis with the earliest onset of symptoms due to the Coxsackie A4 virus in a patient with FCMD. The present findings indicate that physicians should consider FCMD with viral infection a differential diagnosis if the patient presents with acute rhabdomyolysis following a fever.

  Jan. 2020, Journal of infection and chemotherapy : official journal of the Japan Society of Chemotherapy, English, International magazine

  [Refereed]


• Detailed characteristics of acute encephalopathy with biphasic seizures and late reduced diffusion: 18-year data of a single-center consecutive cohort.

  Hiroshi Yamaguchi, Masahiro Nishiyama, Shoichi Tokumoto, Yusuke Ishida, Kazumi Tomioka, Kazunori Aoki, Yusuke Seino, Daisaku Toyoshima, Hiroki Takeda, Hiroshi Kurosawa, Kandai Nozu, Azusa Maruyama, Ryojiro Tanaka, Kazumoto Iijima, Hiroaki Nagase

  OBJECTIVE: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a syndrome characterized by biphasic seizures with impaired consciousness. AESD is rare outside Asia, and consecutive cohort studies are therefore scarce. Herein, we aimed to describe the detailed characteristics of AESD, including clinical course, electroencephalogram data, laboratory data, imaging findings, treatment, and outcomes. METHODS: We reviewed the clinical database and medical charts of 43 consecutive pediatric patients (<18 years old) who developed AESD between October 1, 2002, and September 30, 2019. RESULTS: We found that AESD occurred even though patients did not develop prolonged seizures. A comparison between the two groups (first seizure duration <30 min and first seizure duration ≥30 min) revealed three main findings: first, patients with AESD who had shorter seizures had better prognosis than those with prolonged seizures; second, patients with AESD who had shorter seizures tended to have earlier occurrence of a second seizure; and third, high signal intensity on diffusion-weighted magnetic resonance imaging was observed mainly in frontal areas, not diffusely, in patients with shorter seizures, and in a broader area in patients with prolonged seizures. CONCLUSIONS: Our description of the detailed clinical picture of AESD may add new insight into its pathophysiology.

  Jan. 2020, Journal of the neurological sciences, 411, 116684 - 116684, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Fibrocartilaginous Embolism of the Spinal Cord in Children: A Case Report and Review of Literature.

  Yamaguchi H, Nagase H, Nishiyama M, Tokumoto S, Toyoshima D, Akasaka Y, Maruyama A, Iijima K

  Fibrocartilaginous embolism is assumed to be caused by fibrocartilaginous nucleus pulposus component migration through retrograde embolization to the spinal cord artery. Fibrocartilaginous embolism is currently not well recognized among pediatricians because of its rarity. We describe a previously healthy 15-year-old soccer player who, after kicking a ball, developed progressive weakness in both legs and urinary retention the next day. Magnetic resonance imaging revealed T2 hyperintensity in the anterior horn of the spinal cord at the Th12/L1 level with Schmorl node at the level of L1/2. We also review the previous literature on fibrocartilaginous embolism of the spinal cord in children (less than18 years age); a total of 25 pediatric patients, including our patient, were identified. The median age was 14 years, and 64% of the reviewed patients were female. The most common trigger event was intense exercise or sports. The neurological symptoms started within one day in most cases, and the time to symptom peak varied from a few hours to two weeks. The most common initial neurological symptoms were weakness or plegia (100%), followed by paresthesia or numbness (48%). Affected areas of the spinal cord were distributed evenly from the cervical to thoracolumbar regions. Although steroids and anticoagulants were most commonly used, the prognosis was quite poor (mild to severe sequelae with three deaths). Although fibrocartilaginous embolism is a very rare condition, physicians should be aware of the characteristics and include fibrocartilaginous embolism of the spinal cord in their differential diagnosis, especially for physically active patients.

  Oct. 2019, Pediatric neurology, 99, 3 - 6, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Detailed clinical course of fatal acute encephalopathy in children.

  Tomioka K, Nishiyama M, Nagase H, Ishida Y, Tanaka T, Tokumoto S, Yamaguchi H, Toyoshima D, Maruyama A, Fujita K, Aoki K, Seino Y, Nozu K, Nishimura N, Kurosawa H, Iijima K

  Objective: Although the mortality among previously healthy children with acute encephalopathy (AE) is approximately 5%, their detailed clinical course has not been clarified. The objective of the present study was to describe the detailed clinical course, in minutes, of fatal AE. Methods: We retrospectively reviewed the medical records of five patients (from 6 months to 14 years of age) who previously had no neurological disorders and were diagnosed with brain death due to AE between 2002 and 2018 at Kobe Children's Hospital. Results: The initial clinical symptoms were convulsion in three cases and impaired consciousness in two. The earliest noted brain imaging abnormality was 7.5 h after neurological symptom detection. Liver enzymes and creatinine levels increased at initial examination, and sodium elevated gradually. All patients met the criteria of systemic inflammatory response syndrome, disseminated intravascular coagulation, and shock within 14 h of symptom detection. High dose steroids and targeted temperature management were initiated 3.5-14 h after onset. Despite these therapies, patients were diagnosed with brain death from 16 h to 4 days after initial neurological symptoms. AE diagnoses were made between 4 h 29 min and 4 days after initial neurological symptoms and included hemorrhagic shock and encephalopathy syndromes, Reye-like syndrome, and acute necrotizing encephalopathy in two, two, and one patient(s), respectively. Conclusions: We revealed the time series' of clinical events (e.g. SIRS, shock, DIC, AE diagnosis, brain death, and treatments) and laboratory findings relative to initial neurological symptom in fatal AE. (C) 2019 Published by Elsevier B.V. on behalf of The Japanese Society of Child Neurology.

  Sep. 2019, Brain & development, 41(8) (8), 691 - 698, English

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Preliminary Effectiveness and Safety of High Frequency Oscillation in Addition to Mechanical Insufflation and Exsufflation for Intratracheal Mucus Removal in Patients With Neuromuscular Disease: Protocol for a Prospective Study.

  Awano H, Nagai M, Bo R, Murao M, Ishida Y, Tanaka T, Tomioka K, Nishiyama M, Nagase H, Iijima K

  BACKGROUND: Mechanical insufflation-exsufflation (MI-E) is necessary for noninvasive management of respiratory clearance in patients with neuromuscular disorders (NMDs). Its utility has been proven, and the technique is recommended in a number of international guidelines for the management of patients with NMDs. However, the clearance of thick secretions adhering to the tracheobronchial walls could be problematic when these patients suffer from respiratory tract infections. To improve the effectiveness of the noninvasive technique, a novel device combining MI-E with high frequency oscillation (HFO) has been developed. However, the efficacy of HFO therapy in NMDs has not been well studied. OBJECTIVE: The aim of this study was to elucidate the effect of MI-E combined with HFO for mucus removal in NMD patients. To evaluate its efficacy, changes in transcutaneous oxygen saturation (SpO2), which may predict intratracheal mucus removal, will be measured before and after use of MI-E. METHODS: This is a single-center, nonblinded, nonrandomized prospective study that will enroll 5 subjects hospitalized in Kobe University Hospital owing to respiratory tract infection. All subjects will receive MI-E therapy a few times daily and will receive HFO every other day, for 6 days. Before and after MI-E use, SpO2 will be obtained and the change in SpO2 (ΔSpO2) between MI-E with and without HFO will be calculated. For every subject, the average of ΔSpO2 with or without HFO will be obtained and the null hypothesis that there is a mean change of 0 in the SpO2 between MI-E with and without HFO will be tested using the paired t test. If the treatment with HFO is found to be statistically significantly superior to the treatment without HFO, the study will conclude that HFO addition is more efficacious than no HFO addition. RESULTS: A total of 2 subjects have already been recruited and enrolled in this study as of August 2018. CONCLUSIONS: This unique protocol will assess the efficacy of adding HFO to MI-E during the acute phase of respiratory tract infection in patients with NMDs. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/12102.

  Jun. 2019, JMIR research protocols, 8(6) (6), e12102 - e12102, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Clinical time course of pediatric acute disseminated encephalomyelitis.

  Masahiro Nishiyama, Hiroaki Nagase, Kazumi Tomioka, Tsukasa Tanaka, Hiroshi Yamaguchi, Yusuke Ishida, Daisaku Toyoshima, Kyoko Fujita, Azusa Maruyama, Kaori Sasaki, Yoshinobu Oyazato, Taku Nakagawa, Yuichi Takami, Kandai Nozu, Noriyuki Nishimura, Ichiro Nakashima, Kazumoto Iijima

  The detailed clinical time course in acute disseminated encephalomyelitis (ADEM) from initial symptoms, through exacerbation, to remission has not been widely reported. Hence, this study aimed to investigate the clinical time course of pediatric ADEM. This was a multicenter retrospective study based on registry data from medical chart reviews. The study included children who met the international consensus diagnostic criteria for ADEM. The patients comprised 18 boys and 6 girls, with a mean age of 5.5 ± 3.3 years at onset. From onset, the time until peak neurological symptoms, time until initial improvement, and time until full recovery was 3.1 ± 3.7 days, 6.0 ± 4.5 days, and 26 ± 34 days, respectively. Twenty-three (96%) patients were treated with high-dose methylprednisolone (mPSL) with a mean duration of 4.1 ± 4.0 days from onset. The condition of 15 patients (65%) improved within 3 days of high-dose mPSL initiation, whereas, that of four patients began to improve after >5 days of high-dose mPSL initiation. Only one patient (4%) did not achieve full recovery despite treatment with high-dose mPSL, intravenous immunoglobulin, and plasma exchange. This study presents the detailed clinical time course in pediatric ADEM in Japan. Progression of neurologic deficits typically lasts a few days, with initial improvement in 1 week leading to full recovery within 1 month.

  Jun. 2019, Brain & development, 41(6) (6), 531 - 537, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Nonconvulsive Seizure Detection by Reduced-Lead Electroencephalography in Children with Altered Mental Status in the Emergency Department

  Hiroshi Yamaguchi, Hiroaki Nagase, Masahiro Nishiyama, Shoichi Tokumoto, Yusuke Ishida, Kazumi Tomioka, Tsukasa Tanaka, Kyoko Fujita, Daisaku Toyoshima, Noriyuki Nishimura, Hiroshi Kurosawa, Kandai Nozu, Azusa Maruyama, Ryojiro Tanaka, Kazumoto Iijima

  Apr. 2019, JOURNAL OF PEDIATRICS, 207, 213 - +, English

  [Refereed]

  Scientific journal


• Predicting the outcomes of targeted temperature management for children with seizures and/or impaired consciousness accompanied by fever without known etiology

  Tanaka Tsukasa, Nagase Hiroaki, Yamaguchi H, Ishida Yusuke, Tomioka Kazumi, Nishiyama Masahiro, Toyoshima D, Maruyama A, Fujita K, Nozu Kandai, Nishimura Noriyuki, Kurosawa H, Tanaka R, Iijima Kazumoto

  Background: Seizures and/or impaired consciousness accompanied by fever without known etiology (SICF) is common in the pediatric emergency setting. No optimal strategy for the management of SICF in childhood currently exists. We previously demonstrated the effectiveness of targeted temperature management (TTM) against SICF with a high risk of morbidity; however, some patients with SICF develop neurological sequelae despite TTM, which necessitate additional neuroprotective treatment. The clinical characteristics of these severe cases have not been studied. Accordingly, the aim of this study was to identify the clinical characteristics of children with SICF who exhibit poor outcomes after TTM. Methods: The medical records of children admitted to Kobe Children's Hospital (Kobe, Japan) between October 2002 and September 2016 were retrospectively reviewed. Patients with SICF treated using TTM were included and divided into the satisfactory and poor outcome groups. Univariate and multivariate logistic regression analyses were used to compare clinical characteristics and laboratory findings between the two groups. Results: Of the 73 included children, 10 exhibited poor outcomes. Univariate logistic regression analysis revealed that acute circulatory failure before TTM initiation, the use of four or more types of anticonvulsants, methylprednisolone pulse therapy, and an aspartate aminotransferase (AST) level ≥73 IU/L were associated with poor outcomes. Multivariate logistic regression analysis identified an elevated AST level as a significant independent predictor of a poor outcome. Conclusions: An elevated AST level within 12 h of onset in children with SICF is an independent predictor of a poor outcome after TTM initiated within 24 h of onset.

  Mar. 2019, Brain Dev, 41(7) (7), 604 - 613, English

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Lipid and thyroid hormone levels in children with epilepsy treated with levetiracetam or carbamazepine: A prospective observational study

  Masahiro Nishiyama, Yuichi Takami, Yusuke Ishida, Kazumi Tomioka, Tsukasa Tanaka, Hiroaki Nagase, Taku Nakagawa, Shoichi Tokumoto, Hiroshi Yamaguchi, Daisaku Toyoshima, Azusa Maruyama, Kandai Nozu, Noriyuki Nishimura, Kazumoto Iijima

  Elsevier BV, Jan. 2019, Epilepsy & Behavior, 90, 15 - 19, English

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Lipid and thyroid hormone levels in children with epilepsy treated with levetiracetam or carbamazepine: A prospective observational study.

  Masahiro Nishiyama, Yuichi Takami, Yusuke Ishida, Kazumi Tomioka, Tsukasa Tanaka, Hiroaki Nagase, Taku Nakagawa, Shoichi Tokumoto, Hiroshi Yamaguchi, Daisaku Toyoshima, Azusa Maruyama, Kandai Nozu, Noriyuki Nishimura, Kazumoto Iijima

  Although previous studies have investigated the influence of antiepileptic drugs (AEDs) on lipid profiles and thyroid hormone levels, there is little evidence regarding the effects of levetiracetam (LEV). Therefore, we conducted a prospective longitudinal study to evaluate the effects of LEV and carbamazepine (CBZ) treatment on lipid profile and thyroid hormone levels in patients newly diagnosed with epilepsy. Inclusion criteria were as follows: (a) age between 4 and 15 years, (b) diagnosis of epilepsy with at least two focal seizures within a year, and (c) newly treated with LEV or CBZ monotherapy. Serum lipid profile and thyroid hormone levels were measured before and after 1 and 6 months of AED initiation. Among the 21 included patients (LEV: 13 patients, CBZ: 8 patients), all but one patient in the LEV group continued AED monotherapy during the study period. Although triglyceride (TG) levels tended to be increased in the CBZ group (baseline: 58.3 ± 22.0 mg/dl, 1 month: 63.8 ± 21.6 mg/dl, 6 months: 92.3 ± 63.6 mg/dl, p = 0.22, analyses of variance (ANOVA)), there were no significant changes in total cholesterol (TC), TG levels, high-density lipoprotein cholesterol (HDL-C), or low-density lipoprotein cholesterol (LDL-C) in either group. Serum free thyroxine (fT4) levels were significantly decreased in the CBZ group (baseline: 1.15 ± 0.06 ng/dl, 1 month: 1.00 ± 0.16 ng/dl, 6 months: 0.98 ± 0.14 ng/dl, p = 0.03, ANOVA). In contrast, there were no significant changes in fT4 or thyroid-stimulating hormone (TSH) levels in the LEV group. The results of the present study suggest that LEV monotherapy does not affect lipid profile or thyroid function while CBZ monotherapy may cause thyroid dysfunction.

  Jan. 2019, Epilepsy & behavior : E&B, 90, 15 - 19, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Fosphenytoin vs. continuous midazolam for pediatric febrile status epilepticus

  Nishiyama Masahiro, Nagase Hiroaki, Tomioka Kazumi, Tanaka Tsukasa, Yamaguchi Hiroshi, Ishida Yusuke, Toyoshima Daisaku, Fujita Kyoko, Maruyama Azusa, Kurosawa Hiroshi, Uetani Yoshiyuki, Nozu Kandai, Taniguchi-Ikeda Mariko, Morioka Ichiro, Takada Satoshi, Iijima Kazumoto

  Nov. 2018, Brain & Development, 40(10) (10), 884‐890, English

  [Refereed]

  Scientific journal


• Fosphenytoin vs. continuous midazolam for pediatric febrile status epilepticus.

  Masahiro Nishiyama, Hiroaki Nagase, Kazumi Tomioka, Tsukasa Tanaka, Hiroshi Yamaguchi, Yusuke Ishida, Daisaku Toyoshima, Kyoko Fujita, Azusa Maruyama, Hiroshi Kurosawa, Yoshiyuki Uetani, Kandai Nozu, Mariko Taniguchi-Ikeda, Ichiro Morioka, Satoshi Takada, Kazumoto Iijima

  BACKGROUND: Fosphenytoin (fPHT) and continuous intravenous midazolam (cMDL) had commonly been used as second-line treatments for pediatric status epilepticus (SE) in Japan. However, there is no comparative study of these two treatments. METHODS: We included consecutive children who 1) were admitted to Kobe Children's Hospital because of convulsion with fever and 2) were treated with either fPHT or cMDL as second-line treatment for convulsive SE lasting for longer than 30 min. We compared, between the fPHT and cMDL groups, the proportion of barbiturate coma therapy (BCT), incomplete recovery of consciousness, mechanical ventilation, and inotropic agents. RESULTS: The proportion of BCT was not significantly different between the two groups (48.7% [20/41] in fPHT and 35.3% [29/82] in cMDL, p = 0.17). The prevalence of incomplete recovery of consciousness, mechanical ventilation, and inotropic agents was not different between the two groups. After excluding 49 patients treated with BCT, incomplete recovery of consciousness 6 h and 12 h after onset was more frequent in the cMDL group than in the fPHT group (71.7% vs. 33.3%, p < 0.01; 56.6% vs. 14.2%, p < 0.01; respectively). Mechanical ventilation was more frequent in the cMDL group than in the fPHT group (32.0% vs. 4.7%, p = 0.01). CONCLUSIONS: Our results suggest that 1) the efficacy of fPHT and cMDL is similar, although cMDL may prevent the need for BCT compared with fPHT, and 2) fPHT is relatively safe as a second-line treatment for pediatric SE in patients who do not require BCT.

  Nov. 2018, Brain & development, 40(10) (10), 884 - 890, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• A群色素性乾皮症における神経伝導検査所見の経時的変化

  辻 佑木生, 上田 健博, 関口 兼司, 西山 将広, 苅田 典生, 錦織 千佳子

  (一社)日本臨床神経生理学会, Oct. 2018, 臨床神経生理学, 46(5) (5), 467 - 467, Japanese

  [Refereed]


• Early risk factors for mortality in children with seizure and/or impaired consciousness accompanied by fever without known etiology.

  Kazumi Tomioka, Hiroaki Nagase, Tsukasa Tanaka, Masahiro Nishiyama, Hiroshi Yamaguchi, Yusuke Ishida, Daisaku Toyoshima, Azusa Maruyama, Kyoko Fujita, Mariko Taniguchi-Ikeda, Kandai Nozu, Ichiro Morioka, Noriyuki Nishimura, Hiroshi Kurosawa, Yoshiyuki Uetani, Kazumoto Iijima

  BACKGROUND: Children who present with seizure and/or impaired consciousness accompanied by fever without known etiology (SICF) may be diagnosed with either acute encephalopathy (AE) or febrile seizure (FS). Although approximately 5% of AE cases are fatal, it is difficult to identify fatal cases among children with SICF, which are often critical by the time of diagnosis. Thus, early prediction of outcomes for children with SICF, prior to diagnosis, may help to reduce mortality associated with AE. The aim of the present study was to identify clinical and laboratory risk factors for mortality acquired within 6 h of onset among children with SICF. METHODS: We retrospectively reviewed the medical records of children who had been admitted to Kobe Children's Hospital (Kobe, Japan) with SICF between October 2002 and September 2015. We compared clinical and laboratory characteristics acquired within 6 h of onset and outcomes between survivors and non-survivors using univariate and multivariate analyses. RESULTS: The survivor and non-survivor groups included 659 and nine patients, respectively. All patients in the non-survivor group received a final diagnosis of AE. Univariate analysis revealed significant differences between the groups with regard to seizure duration and the following laboratory parameters: aspartate transaminase (AST), alanine aminotransferase, lactate dehydrogenase, sodium, and lactate. The multivariate analysis identified AST as a significant independent factor associated with mortality. CONCLUSIONS: Elevation of AST within 6 h of onset is independently correlated with mortality in children with SICF. Our result may elucidate earlier intervention for patients with high risk of mortality.

  Aug. 2018, Brain & development, 40(7) (7), 552 - 557, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Congenital Cytomegalovirus Infection in Children with Autism Spectrum Disorder: Systematic Review and Meta-Analysis.

  Kaori Maeyama, Kazumi Tomioka, Hiroaki Nagase, Mieko Yoshioka, Yasuko Takagi, Takeshi Kato, Masami Mizobuchi, Shinji Kitayama, Satoshi Takada, Masashi Nagai, Nana Sakakibara, Masahiro Nishiyama, Mariko Taniguchi-Ikeda, Ichiro Morioka, Kazumoto Iijima, Noriyuki Nishimura

  Association of congenital cytomegalovirus (CMV) infection with autism spectral disorder (ASD) has been suggested since 1980s. Despite the observed association, its role as a risk factor for ASD remains to be defined. In the present review, we systematically evaluated the available evidence associating congenital CMV infection with ASD using PubMed, Web of Science, Cochrane Library, and Embase databases. Any studies on children with CMV infection and ASD were evaluated for eligibility and three observational studies were included in meta-analysis. Although a high prevalence of congenital CMV infection in ASD cases (OR 11.31, 95% CI 3.07-41.66) was indicated, too few events (0-2 events) in all included studies imposed serious limitations. There is urgent need for further studies to clarify this issue.

  May 2018, Journal of autism and developmental disorders, 48(5) (5), 1483 - 1491, English, International magazine

  [Refereed]

  Scientific journal


• Clinical features predicting group A streptococcal pharyngitis in a Japanese paediatric primary emergency medical centre.

  Masahiro Nishiyama, Ichiro Morioka, Mariko Taniguchi-Ikeda, Takeshi Mori, Kazumi Tomioka, Keita Nakanishi, Junya Fujimura, Noriyuki Nishimura, Kandai Nozu, Hiroaki Nagase, Kazuto Ishibashi, Akihito Ishida, Kazumoto Iijima

  Objectives To identify clinical features that predict Group A streptococcal (GAS) pharyngitis in a Japanese paediatric primary emergency medical centre. Methods The prevalence of GAS pharyngitis according to age and body temperature (BT) was calculated among 3098 paediatric patients with pharyngitis. The numbers of GAS-positive and -negative patients for each clinical parameter, and each point increase in the McIsaac score were compared and likelihood ratios (LRs) were calculated. Results The prevalence of GAS pharyngitis was extremely low in patients aged < 1 (1.2%) and 1 year (3.9%). The GAS-positive rate was significantly higher in patients with a BT < 38.0°C compared with ≥ 38.0°C (30.0% vs. 19.8%). A BT ≥ 38.0°C was not a predictive finding for GAS pharyngitis (positive LR: 0.82). Rash was the most useful individual predictor, and a McIsaac score of 4 or 5 increased the probability; however, the positive LRs were 1.74 and 1.30, respectively. Conclusions The prevalence of GAS pharyngitis is extremely low in patients aged < 1 and 1 year, and a BT ≥ 38.0°C is not a predictive symptom. Although a rash and McIsaac score of 4 or 5 are associated with an increased probability, they cannot be used to confirm GAS infection.

  Lead, May 2018, The Journal of international medical research, 46(5) (5), 1791 - 1800, English, International magazine

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Diurnal occurrence of complex febrile seizure and their severity in pediatric patients needing hospitalization.

  Hiroshi Yamaguchi, Hiroaki Nagase, Yusuke Ishida, Daisaku Toyoshima, Azusa Maruyama, Kazumi Tomioka, Tsukasa Tanaka, Masahiro Nishiyama, Kyoko Fujita, Taniguchi-Ikeda Mariko, Kandai Nozu, Ichiro Morioka, Noriyuki Nishimura, Hiroshi Kurosawa, Satoshi Takada, Yoshiyuki Uetani, Kazumoto Iijima

  Several studies describing the diurnal occurrence of febrile seizures have reported greater seizure frequency early or late in the evening relative to midnight or early morning. However, no articles have reported on the diurnal occurrence of complex febrile seizure. Moreover, no studies have addressed the relationship between seizure severity and diurnal occurrence. We retrospectively evaluated complex febrile seizures in 462 children needing hospitalization, and investigated the relationship between severity and diurnal occurrence according to four categorized time periods (morning, afternoon, evening, and night). Our study showed that complex febrile seizures occurred most often in the evening, peaking around 18:00 (18:00-18:59), and least often at night (02:00-02:59). In addition, the frequency with which patients developed status epilepticus or needed anticonvulsant treatments was also lower during the night. However, the seizure duration and the proportion of the patients who needed anticonvulsant treatment were the same among the four time periods. Furthermore, we compared three subclasses (repeated episodes of convulsions, focal seizures, and prolonged seizures (≧15min)), two of the complex features (focal seizures and prolonged seizures), and all complex features among the four time periods. However, they were the same among the four time periods. Taken together, our data indicate that although the severity of seizures was stable over a 24-hour period, the occurrence of seizures in our cohort of pediatric patients with complex febrile seizures requiring hospitalization was highest in the evening and lowest at night.

  Mar. 2018, Epilepsy & behavior : E&B, 80, 280 - 284, English, International magazine

  [Refereed]

  Scientific journal


• Diurnal occurrence of complex febrile seizure and their severity in pediatric patients needing hospitalization.

  Hiroshi Yamaguchi, Hiroaki Nagase, Yusuke Ishida, Daisaku Toyoshima, Azusa Maruyama, Kazumi Tomioka, Tsukasa Tanaka, Masahiro Nishiyama, Kyoko Fujita, Taniguchi-Ikeda Mariko, Kandai Nozu, Ichiro Morioka, Noriyuki Nishimura, Hiroshi Kurosawa, Satoshi Takada, Yoshiyuki Uetani, Kazumoto Iijima

  Several studies describing the diurnal occurrence of febrile seizures have reported greater seizure frequency early or late in the evening relative to midnight or early morning. However, no articles have reported on the diurnal occurrence of complex febrile seizure. Moreover, no studies have addressed the relationship between seizure severity and diurnal occurrence. We retrospectively evaluated complex febrile seizures in 462 children needing hospitalization, and investigated the relationship between severity and diurnal occurrence according to four categorized time periods (morning, afternoon, evening, and night). Our study showed that complex febrile seizures occurred most often in the evening, peaking around 18:00 (18:00-18:59), and least often at night (02:00-02:59). In addition, the frequency with which patients developed status epilepticus or needed anticonvulsant treatments was also lower during the night. However, the seizure duration and the proportion of the patients who needed anticonvulsant treatment were the same among the four time periods. Furthermore, we compared three subclasses (repeated episodes of convulsions, focal seizures, and prolonged seizures (≧15min)), two of the complex features (focal seizures and prolonged seizures), and all complex features among the four time periods. However, they were the same among the four time periods. Taken together, our data indicate that although the severity of seizures was stable over a 24-hour period, the occurrence of seizures in our cohort of pediatric patients with complex febrile seizures requiring hospitalization was highest in the evening and lowest at night.

  Mar. 2018, Epilepsy & behavior : E&B, 80, 280 - 284, English, International magazine

  [Refereed]

  Scientific journal


• 複雑型熱性けいれんの好発時間帯とその重症度(Diurnal Occurrence of Complex Febrile Seizure and its Severity in Children)

  山口 宏, 永瀬 裕朗, 石田 悠介, 豊嶋 大作, 丸山 あずさ, 富岡 和美, 田中 司, 西山 将広, 藤田 杏子, 黒澤 寛史, 高田 哲, 上谷 良行, 飯島 一誠

  (公社)日本小児科学会, Feb. 2018, 日本小児科学会雑誌, 122(2) (2), 313 - 313, English

  [Refereed]

  Scientific journal


• Renal insufficiency mimicking glutaric acidemia type 1 on newborn screening

  Masaaki Matsumoto, Hiroyuki Awano, Ryosuke Bo, Masashi Nagai, Kazumi Tomioka, Masahiro Nishiyama, Takeshi Ninchouji, Hiroaki Nagase, Mariko Yagi, Ichiro Morioka, Yuki Hasegawa, Yasuhiro Takeshima, Kazumoto Iijima

  Blackwell Publishing, Jan. 2018, Pediatrics International, 60(1) (1), 67 - 69, English

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Renal insufficiency mimicking glutaric acidemia type 1 on newborn screening

  Masaaki Matsumoto, Hiroyuki Awano, Ryosuke Bo, Masashi Nagai, Kazumi Tomioka, Masahiro Nishiyama, Takeshi Ninchouji, Hiroaki Nagase, Mariko Yagi, Ichiro Morioka, Yuki Hasegawa, Yasuhiro Takeshima, Kazumoto Iijima

  Blackwell Publishing, Jan. 2018, Pediatrics International, 60(1) (1), 67 - 69, English

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Clinical prediction rule for neurological sequelae due to acute encephalopathy: a medical community-based validation study in Harima, Japan

  SasakiK, Nagase Hiroaki, MaruyamaA, FujitaK, Nishiyama Masahiro, TanakaT, NukinaS, TakumiT, TakenakaK, OyazatoY, NishiyamaA, KawataT, SaekiK, TakamiY, Satake-InoueE, Iijima Kazumoto, Morioka Ichiro, UetaniY

  Objectives This study aimed to verify the screening performance of our clinical prediction rule for neurological sequelae due to acute encephalopathy (NSAE-CPR), which previously identified the following three variables as predictive of poor outcomes: (1) refractory status epilepticus; (2) consciousness disturbance and/or hemiplegia at 6 hours from onset and (3) aspartate aminotransferase >90 IU/L within 6 hours of onset. Design Medical community-based multicentre retrospective cohort study. Setting Six regional hospitals in Harima and one tertiary centre in Kobe, Japan, from 2008 to 2012. Participants We enrolled a total of 1612 patients aged <16 years who met the diagnostic criteria for an initial diagnosis of complex febrile seizure. Patients with a history of neurological disease and those included in the derivation cohort were excluded. Primary outcome measures Univariate and multivariate analyses were performed to determine the association between each of the three predictor variables and poor AE outcome (Pediatric Cerebral Performance Category score ≥2). Receiver operating characteristic curve (ROC) analysis was also performed to assess the screening performance of the NSAE-CPR. Results The ROC analysis identified at least one of the three predictive variables as an optimal cut-off point, with an area under the curve of 0.915 (95% CI 0.825 to 1.000). The sensitivity, specificity, positive and negative predictive values, positive and negative likelihood ratios and Matthews correlation coefficient were 0.867, 0.954, 0.149, 0.999, 18.704, 0.140 and 0.349, respectively. Conclusions Our findings indicate that the NSAE-CPR can be used for the screening and identification of patients with poor outcomes due to acute encephalopathy within 6 hours of onset.

  BMJ Publishing Group, Nov. 2017, BMJ Open, 7(11) (11), e016675 - e016675, English

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Clinical features in very early-onset demyelinating disease with anti-MOG antibody

  Masahiro Nishiyama, Hiroaki Nagase, Masaaki Matsumoto, Kazumi Tomioka, Hiroyuki Awano, Tsukasa Tanaka, Daisaku Toyoshima, Kyoko Fujita, Azusa Maruyama, Yoshinobu Oyazato, Keisuke Saeki, Kazuhiro Shiraishi, Satoshi Takada, Kimihiko Kaneko, Toshiyuki Takahashi, Ichiro Nakashima, Kazumoto Iijima

  Oct. 2017, BRAIN & DEVELOPMENT, 39(9) (9), 756 - 762, English

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Quantitative analysis of brain atrophy in patients with xeroderma pigmentosum group A carrying the founder mutation in Japan

  Takehiro Ueda, Fumio Kanda, Masahiro Nishiyama, Chikako Nishigori, Tatsushi Toda

  Oct. 2017, JOURNAL OF THE NEUROLOGICAL SCIENCES, 381, 103 - 106, English

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Clinical features in very early-onset demyelinating disease with anti-MOG antibody

  Masahiro Nishiyama, Hiroaki Nagase, Masaaki Matsumoto, Kazumi Tomioka, Hiroyuki Awano, Tsukasa Tanaka, Daisaku Toyoshima, Kyoko Fujita, Azusa Maruyama, Yoshinobu Oyazato, Keisuke Saeki, Kazuhiro Shiraishi, Satoshi Takada, Kimihiko Kaneko, Toshiyuki Takahashi, Ichiro Nakashima, Kazumoto Iijima

  Oct. 2017, BRAIN & DEVELOPMENT, 39(9) (9), 756 - 762, English

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• 有熱性けいれん重積症例におけるAESDおよび急性脳症鑑別のためのAESD prediction scoreの有用性の検証

  西山 将広, 永瀬 裕朗, 石田 悠介, 田中 司, 藤田 杏子, 豊嶋 大作, 丸山 あずさ, 冨岡 和美, 黒澤 寛史, 竹田 洋樹, 上谷 良行, 高田 哲, 飯島 一誠

  (一社)日本小児神経学会, May 2017, 脳と発達, 49(Suppl.) (Suppl.), S299 - S299, Japanese

  [Refereed]


• Targeted temperature managementを導入した急性脳症疑い症例における早期予後因子の検討

  田中 司, 石田 悠介, 冨岡 和美, 西山 将広, 藤田 杏子, 豊嶋 大作, 丸山 あずさ, 永瀬 裕朗, 黒澤 寛史, 竹田 洋樹, 上谷 良行, 飯島 一誠

  (一社)日本小児神経学会, May 2017, 脳と発達, 49(Suppl.) (Suppl.), S300 - S300, Japanese

  [Refereed]


• 発症6時間以内に得られる急性脳症死亡予測因子の検討

  冨岡 和美, 永瀬 裕朗, 石田 悠介, 田中 司, 西山 将広, 藤田 杏子, 豊嶋 大作, 丸山 あずさ, 黒澤 寛史, 竹田 洋樹, 上谷 良行, 高田 哲, 飯島 一誠

  (一社)日本小児神経学会, May 2017, 脳と発達, 49(Suppl.) (Suppl.), S301 - S301, Japanese

  [Refereed]


• 小児難治てんかん重積状態に対するチアミラールを用いた全身麻酔療法

  石田 悠介, 冨岡 和美, 田中 司, 西山 将広, 藤田 杏子, 豊嶋 大作, 丸山 あずさ, 永瀬 裕朗, 黒澤 寛史, 竹田 洋樹, 上谷 良行, 飯島 一誠

  (一社)日本小児神経学会, May 2017, 脳と発達, 49(Suppl.) (Suppl.), S346 - S346, Japanese

  [Refereed]


• 超低出生体重児のSGA性低身長症に対する成長ホルモン治療の検討

  松本 真明, 粟野 宏之, 永井 正志, 坊 亮輔, 冨岡 和美, 前山 花織, 西山 将広, 永瀬 裕朗, 森岡 一朗, 飯島 一誠

  (公社)日本小児科学会, Feb. 2017, 日本小児科学会雑誌, 121(2) (2), 374 - 374, Japanese


• Changes in the numbers of patients with acute gastroenteritis after voluntary introduction of the rotavirus vaccine in a Japanese children's primary emergency medical center

  Ichiro Morioka, Naohiro Kamiyoshi, Masahiro Nishiyama, Tomohiko Yamamura, Shogo Minamikawa, Sota Iwatani, Hiroaki Nagase, Kandai Nozu, Noriyuki Nishimura, Mariko Taniguchi-Ikeda, Kazuto Ishibashi, Akihito Ishida, Kazumoto Iijima

  2017, ENVIRONMENTAL HEALTH AND PREVENTIVE MEDICINE, 22(1) (1), 15 - 15, English

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Changes in the numbers of patients with acute gastroenteritis after voluntary introduction of the rotavirus vaccine in a Japanese children's primary emergency medical center

  Ichiro Morioka, Naohiro Kamiyoshi, Masahiro Nishiyama, Tomohiko Yamamura, Shogo Minamikawa, Sota Iwatani, Hiroaki Nagase, Kandai Nozu, Noriyuki Nishimura, Mariko Taniguchi-Ikeda, Kazuto Ishibashi, Akihito Ishida, Kazumoto Iijima

  BioMed Central Ltd., 2017, Environmental Health and Preventive Medicine, 22(1) (1), 22:15 (WEB ONLY), English

  [Refereed]

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• A pediatric patient with interstitial pneumonia due to enterovirus D68

  Masaaki Matsumoto, Hiroyuki Awano, Miki Ogi, Kazumi Tomioka, Ai Unzaki, Masahiro Nishiyama, Daisaku Toyoshima, Mariko Taniguchi-Ikeda, Akihito Ishida, Hiroaki Nagase, Ichiro Morioka, Kazumoto Iijima

  Elsevier B.V., Oct. 2016, Journal of Infection and Chemotherapy, 22(10) (10), 712 - 715, English

  [Refereed]

  Scientific journal


• A pediatric patient with interstitial pneumonia due to enterovirus D68

  Masaaki Matsumoto, Hiroyuki Awano, Miki Ogi, Kazumi Tomioka, Ai Unzaki, Masahiro Nishiyama, Daisaku Toyoshima, Mariko Taniguchi-Ikeda, Akihito Ishida, Hiroaki Nagase, Ichiro Morioka, Kazumoto Iijima

  Elsevier B.V., Oct. 2016, Journal of Infection and Chemotherapy, 22(10) (10), 712 - 715, English

  [Refereed]

  Scientific journal


• Short and long-term outcomes in children with suspected acute encephalopathy

  Masahiro Nishiyama, Hiroaki Nagase, Tsukasa Tanaka, Kyoko Fujita, Mayumi Kusumoto, Shinsuke Kajihara, Yoshimichi Yamaguchi, Azusa Maruyama, Hiroki Takeda, Yoshiyuki Uetani, Kazumi Tomioka, Daisaku Toyoshima, Mariko Taniguchi-Ikeda, Ichiro Morioka, Satoshi Takada, Kazumoto Iijima

  Elsevier BV, Sep. 2016, Brain and Development, 38(8) (8), 731 - 737, English

  [Refereed]

  Scientific journal


• Short and long-term outcomes in children with suspected acute encephalopathy

  Masahiro Nishiyama, Hiroaki Nagase, Tsukasa Tanaka, Kyoko Fujita, Mayumi Kusumoto, Shinsuke Kajihara, Yoshimichi Yamaguchi, Azusa Maruyama, Hiroki Takeda, Yoshiyuki Uetani, Kazumi Tomioka, Daisaku Toyoshima, Mariko Taniguchi-Ikeda, Ichiro Morioka, Satoshi Takada, Kazumoto Iijima

  Sep. 2016, BRAIN & DEVELOPMENT, 38(8) (8), 731 - 737, English

  [Refereed]

  Scientific journal


• 異なる経過をたどった腸管出血性大腸菌による溶血性尿毒症症候群の姉妹例

  中西啓太, Minamikawa Shogo, 山村智彦, 神吉直宙, 西山将広, 忍頂寺毅史, 豊嶋大作, Nozu Kandai, 濱平陽史, Iijima Kazumoto

  東京 : 日本小児腎不全学会, Jul. 2016, 日本小児腎不全学会雑誌, 36, 175 - 178, Japanese

  [Refereed]


• Clinical factors associated with prehospital exacerbation of anaphylaxis in children

  NinchojiT, Iwatani Souta, Nishiyama Masahiro, KamiyoshiN, NakagawaT, Taniguchi-IkedaM, MorisadaN, IshibashiK, Iijima Kazumoto, IshidaA, Morioka Ichiro

  Jun. 2016, MINERVA PEDIATR, English

  [Refereed]

  Scientific journal


• ジストロフィン遺伝子イントロン64のスプライス供与部位の変異(c.9361+1G>A)により発症したDuchenne型筋ジストロフィーの一例

  粟野 宏之, 松本 真明, 冨岡 和美, 運崎 愛, 西山 将広, 李 知子, 永瀬 裕明, 池田 真理子, 八木 麻理子, 竹島 泰弘, 松尾 雅文, 飯島 一誠

  (一社)日本小児神経学会, May 2016, 脳と発達, 48(Suppl.) (Suppl.), S259 - S259, Japanese


• Next-generation sequencing discloses a nonsense mutation in the dystrophin gene from long preserved dried umbilical cord and low-level somatic mosaicism in the proband mother

  Mariko Taniguchi-Ikeda, Yasuhiro Takeshima, Tomoko Lee, Masahiro Nishiyama, Hiroyuki Awano, Mariko Yagi, Ai Unzaki, Kandai Nozu, Hisahide Nishio, Masafumi Matsuo, Hiroki Kurahashi, Tatsushi Toda, Ichiro Morioka, Kazumoto Iijima

  Nature Publishing Group, Apr. 2016, Journal of Human Genetics, 61(4) (4), 351 - 355, English

  [Refereed]

  Scientific journal


• Current Situation of Treatment for Anaphylaxis in a Japanese Pediatric Emergency Center

  NinchojiT, Iwatani Souta, Nishiyama Masahiro, KamiyoshiN, Taniguchi-IkedaM, MorisadaN, IshibashiK, Iijima Kazumoto, IshidaA, Morioka Ichiro

  Apr. 2016, Pediatr Emerg Care, English

  [Refereed]

  Scientific journal


• Rituximab treatment for relapsed opsoclonus-myoclonus syndrome

  Daisaku Toyoshima, Naoya Morisada, Yuichi Takami, Hiroyuki Kidokoro, Masahiro Nishiyama, Taku Nakagawa, Takeshi Ninchoji, Kandai Nozu, Yasuhiro Takeshima, Satoshi Takada, Hisahide Nishio, Kazumoto Iijima

  Mar. 2016, BRAIN & DEVELOPMENT, 38(3) (3), 346 - 349, English

  [Refereed]

  Scientific journal


• A Novel PIGN Mutation and Prenatal Diagnosis of Inherited Glycosylphosphatidylinositol Deficiency

  Taku Nakagawa, Mariko Taniguchi-Ikeda, Yoshiko Murakami, Shota Nakamura, Daisuke Motooka, Tomomi Emoto, Wataru Satake, Masahiro Nishiyama, Daisaku Toyoshima, Naoya Morisada, Satoshi Takada, Shinya Tairaku, Nobuhiko Okamoto, Ichiro Morioka, Hiroki Kurahashi, Tatsushi Toda, Taroh Kinoshita, Kazumoto Iijima

  Jan. 2016, AMERICAN JOURNAL OF MEDICAL GENETICS PART A, 170(1) (1), 183 - 188, English

  [Refereed]

  Scientific journal


• A woman with beta-propeller protein-associated neurodegeneration identified by the WDR45 mutation presenting as Rett-like syndrome in childhood

  Naoya Morisada, Syuichi Tsuneishi, Kazuhiro Taguchi, Ryuzaburo Yagi, Masahiro Nishiyama, Daisaku Toyoshima, Taku Nakagawa, Yasuhiro Takeshima, Satoshi Takada, Kazumoto Iijima

  Japanese Society of Child Neurology, 2016, No To Hattatsu, 48(3) (3), 209 - 212, Japanese

  [Refereed]


• Somatic mosaicism of a CDKL5 mutation identified by next-generation sequencing

  Takeshi Kato, Naoya Morisada, Hiroaki Nagase, Masahiro Nishiyama, Daisaku Toyoshima, Taku Nakagawa, Azusa Maruyama, Xue Jun Fu, Kandai Nozu, Hiroko Wada, Satoshi Takada, Kazumoto Iijima

  Elsevier, Oct. 2015, Brain and Development, 37(9) (9), 911 - 915, English

  [Refereed]

  Scientific journal


• Somatic mosaicism of a CDKL5 mutation identified by next-generation sequencing

  Takeshi Kato, Naoya Morisada, Hiroaki Nagase, Masahiro Nishiyama, Daisaku Toyoshima, Taku Nakagawa, Azusa Maruyama, Xue Jun Fu, Kandai Nozu, Hiroko Wada, Satoshi Takada, Kazumoto Iijima

  Oct. 2015, BRAIN & DEVELOPMENT, 37(9) (9), 911 - 915, English

  [Refereed]

  Scientific journal


• Demographics and outcomes of patients with pediatric febrile convulsive status epilepticus.

  Nishiyama M, Nagase H, Tanaka T, Fujita K, Maruyama A, Toyoshima D, Nakagawa T, Taniguchi-Ikeda M, Morioka I, Morisada N, Takada S, Iijima K

  May 2015, Pediatric neurology, 52(5) (5), 499 - 503, English

  [Refereed]

  Doctoral thesis


• Targeted temperature management of acute encephalopathy without AST elevation

  Masahiro Nishiyama, Tsukasa Tanaka, Kyoko Fujita, Azusa Maruyama, Hiroaki Nagase

  Lead, Mar. 2015, BRAIN & DEVELOPMENT, 37(3) (3), 328 - 333, English

  [Refereed]

  Scientific journal


• Two cases of traumatic head injury mimicking acute encephalopathy with biphasic seizures and late reduced diffusion

  Masahiro Nishiyama, Kyoko Fujita, Azusa Maruyama, Hiroaki Nagase

  Lead, Nov. 2014, BRAIN & DEVELOPMENT, 36(10) (10), 928 - 931, English

  [Refereed]

  Scientific journal


• Nonconvulsive Seizures in Children with Prolonged Febrile Seizures

  丸山あずさ, Nishiyama Masahiro, 藤田杏子, 永瀬裕朗

  日本小児科学会, Oct. 2014, 日本小児科学会雑誌, 118(10) (10), 1475 - 1480, Japanese

  [Refereed]


• Midazolam Fails to Prevent Neurological Damage in Children With Convulsive Refractory Febrile Status Epilepticus

  Hiroald Nagase, Masahiro Nishiyama, Taku Nakagawa, Kyoko Fujita, Yohsuke Saji, Azusa Maruyama

  Jul. 2014, PEDIATRIC NEUROLOGY, 51(1) (1), 78 - 84, English

  [Refereed]

  Scientific journal


• A Case of Acute Encephalopathy with Secondary Carnitine Deficiency after Taking Pivalate-conjugated Antibiotics for 3 Days

  Nishiyama Masahiro, 田中司, 藤田杏子, 丸山あずさ, 永瀬裕朗, 竹田洋樹, 上谷良行, 重松陽介

  Lead, 日本小児科学会, May 2014, 日本小児科学会雑誌, 118(5) (5), 812 - 818, Japanese

  [Refereed]


• Application of an automated epileptic seizure detection system in a pediatric intensive care unit setting

  丸山あずさ, Nishiyama Masahiro, 藤田杏子, 永瀬裕朗

  日本小児医事出版社, Sep. 2013, 小児科臨床, 66(9) (9), 1887 - 1891, Japanese

  [Refereed]


• 全身性けいれんと意識障害で発症した中枢神経系ループスの6歳例

  Nishiyama Masahiro, 濱平陽史, 植村加奈子, 二階堂量子, 山本寛子, 岸田真, 北山路子, 山本暢之, 上田陽子, 伴紘文, 高見勇一, 柄川剛, 高橋宏暢, 岸本芽子, 五百蔵智明, 久呉真章

  6歳男児。持続する発熱、前腕・頬部の紅斑、肝機能異常を主訴とした。入院時、リンパ球減少を認めた。入院6時間後、全身性強直間代痙攣が出現し、気管挿管下に高容量チオペンタールを投与し、2時間後にようやく痙攣が頓挫した。チオペンタール中止後も意識障害は4日間遷延した。頭部CTで明らかな異常を認めなかったが、脳症または中枢神経系ループスを疑い、入院1日目からマンニトールとアシクロビルの静脈投与を開始し、3日目からステロイドパルス療法を開始したところ意識障害は改善した。同日抗核抗体2560倍、抗ds-DNA抗体105.6IU/mLを認め、小児全身性エリトマトーデスの診断基準12項目中6項目を満たしており、診断が確定した。入院12日目から13日目にメチルプレドニゾロン・パルス療法、13日目からプレドニゾロンとミゾリビンを開始した。入院19日目に施行した腎生検より、ループス腎炎のI型に分類した。以降、神経学的後遺症を認めず、入院43日目に退院した。発症から1年半が経過し、プレドニゾロン6mg/日まで減量したが、症状の再燃はなかった。

  (公社)日本小児科学会, Mar. 2011, 日本小児科学会雑誌, 115(3) (3), 611 - 615, Japanese

  [Refereed]


• Acute myeloid leukemia with t(16;21)(q24;q22) in a child

  NOGUCHI Yoriko, NAKAMACHI Yuji, OYABU Chinami, KIKUMA Tomoe, HASEGAWA Daiichiro, YAMASHITA Tatsuya, NISHIYAMA Masahiro, KOSAKA Yoshiyuki, KAWANO Seiji

  The t(16;21)(q24;q22), a rare chromosomal translocation observed mostly in therapy-related acute myelogenous leukemia (AML), produces a RUNX1-CBFA2T3 fusion gene. Here we report a de novo AML case of 1-year-old girl with t(16;21)(q24;q22). In this case, we demonstrated the RUNX1-CBFA2T3 fusion gene and established quantitative RT-PCR for detecting minimal residual disease.

  The Japanese Society of Hematology, 2011, Rinsho Ketsueki, 52(12) (12), 1893 - 1895, Japanese

  [Refereed]


• 全身性痙攣で発症しステロイドパルス療法が奏功したCNSループスの1例

  Nishiyama Masahiro, 濱平陽史, 植村加奈子, 二階堂量子, 山本寛子, 岸田真, 北山路子, 山本暢之, 上田陽子, 伴紘文, 高見勇一, 柄川剛, 高橋宏暢, 岸本芽子, 五百蔵智明, 久呉真章

  6歳男児。患者は持続する発熱、紅斑、肝機能障害を主訴とした。当初、意識は清明で、額、頬、上肢等の紅斑、リンパ球減少が認められた。しかし、入院6時間後に突然の全身痙攣と意識障害が出現し、ミダゾラム、フェニトインの静脈内投与が施行されるも、痙攣は持続し、高容量チオペンタールを気管挿管下に投与されたが、頓挫した。一方、チオペンタールは入院2日目に終了したが、JCS 3桁の意識障害は4日間遷延した。更に入院3日目からCNSループスまたは脳症が疑われ、3日間メチルプレドニゾロン・パルス療法を施行したところ、次第に意識レベルが改善した。以上、これらの臨床経過に加え、抗核抗体2560倍、抗ds-DNA抗体105.6IU/mL等から、本症例は全身性エリトマトーデスと診断され、メチルプレドニゾロン・パルス療法の2クール目を施行し、あわせてプレドニゾロン、ミゾリビンを開始した結果、経過は良好で、患者は神経学的後遺症を残さず退院となった。尚、6歳以下で初診時に痙攣と意識障害を来したCNSループスの報告は、検索した限り認められなかった。

  姫路赤十字病院図書学術委員会, Jul. 2010, 姫路赤十字病院誌, 34, 13 - 18, Japanese

  [Refereed]

  Research institution


• 当院における髄膜炎起炎菌と抗生剤感受性に関する検討

  山本暢之, 岸田真, 北山路子, Nishiyama Masahiro, 李知子, 住谷珠子, 伴紘文, 高見勇一, 柄川剛, 高橋宏暢, 岸本芽子, 濱平陽史, 五百蔵智明, 久呉真章

  過去5年間に細菌性髄膜炎と診断され、治療を行った児38症例の、その入院時における髄液培養の結果を基に、起炎菌と抗生剤の感受性について後方視的に検討した。1)起炎菌が判明したのは28例で、インフルエンザ菌20例、肺炎球菌4例、大腸菌3例、B群溶血性連鎖球菌1例であった。2)インフルエンザ菌・大腸菌に対してはCTX・CTRX・MEPMが良好な感受性を示した。3)PAPM/BPについては試験をしたほぼすべての株において耐性を示した。肺炎球菌についてもPAPM/BPは耐性であるものが多く、CTX・MEPMが比較的良好な感受性を示した。VCMは全例で感受性を示した。3)これらの結果を踏まえ、初期に使用する抗生剤はPAPM/BPからMEPMに変更された。但し、今後も時期をおいて感受性を検討する必要があると考えられた。

  姫路赤十字病院図書学術委員会, Jun. 2009, 姫路赤十字病院誌, 33, 11 - 14, Japanese

  [Refereed]

  Research institution


• 短期間に腸重積症を反復した嚢胞型消化管重複症の1例

  大片祐一, 畠山理, Nishiyama Masahiro, 高見勇一, 濱平陽史, 五百蔵智明, 久呉真章, 綿貫裕, 藤澤真義

  4ヵ月男児。患者は嘔吐、下痢(粘血便)を主訴とした。腹部超音波では上行結腸にtarget signが認められ、腸重積症と考えられた。注腸整復を行って嘔吐と血便はいったん消失したが、入院3日目以降、短期間のうちに頻回の再発を繰返した。回腸回腸結腸型の所見で、初回、再発時には明らかな器質的病変は指摘できなかったが、再々発時cystic lesionを認めた。入院8日目に試験開腹術を施行し、腸間膜側から対側にかけて漿膜面に突出しない、可動性良好なφ20mm大の腫瘤が確認され、これが腸重積の先進部となっていた。そこで、腫瘤を含む回腸4cmを切除し、病理組織学的所見では病変内腔は貯留液で満たされており、この嚢胞病変は小腸の筋層間に存在して筋層を共有しており、内腔はすべて胃粘膜上皮で覆われていた。以上より、本症例は嚢胞型消化管重複症と診断され、術後は経過良好であった。尚、内腔に突出する形態をとった消化管重複症は稀で、検索した限り我が国では本症例を含め3例の報告を認めるのみである。

  姫路赤十字病院図書学術委員会, Jun. 2009, 姫路赤十字病院誌, 33, 30 - 34, Japanese

  [Refereed]

  Research institution

• 被膜下巨大血腫を来し、側彎による固有腎の偏位がリスクと考えられた1例

  石森 真吾, 佐藤 郷介, 平崎 智也, 木村 由香, 青山 周平, 山本 朝日, 上田 拓耶, 猪野木 雄太, 花房 宏昭, 堀之内 智子, 山村 智彦, 榊原 菜々, 長野 智那, 西山 将広, 梅野 晃弘, 佐々木 康二, 野津 寛大

  日本小児泌尿器科学会, Jul. 2026, 日本小児泌尿器科学会雑誌, 35(2) (2), 225 - 225, Japanese


• 自律神経障害が遷延したギランバレー症候群の1歳男児例

  田中 敦, 西山 将広, 鮫島 智大, 伊藤 立人, 相馬 健人, 石田 悠介, 丸山 あずさ

  (公社)日本小児科学会, May 2026, 日本小児科学会雑誌, 130(5) (5), 763 - 764, Japanese


• 偏食・摂食の問題を主訴に高次医療機関を受診した乳幼児の臨床的特徴

  冨岡 和美, 永瀬 裕朗, 伊藤 立人, 相馬 健人, 鮫島 智大, 石田 悠介, 西山 将広, 丸山 あずさ, 末宗 和樹, 上田 拓耶, 川村 葵, 老川 静香, 徳元 翔一, 山口 宏

  (公社)日本小児科学会, May 2026, 日本小児科学会雑誌, 130(5) (5), 764 - 764, Japanese


• 多様化する選択肢をどう活かすか 熱性けいれん・てんかん重積状態の管理

  西山 将広

  (公社)日本小児科学会, May 2026, 日本小児科学会雑誌, 130(5) (5), 765 - 765, Japanese


• 視神経炎の自覚症状がなく片麻痺・構音障害を主訴に来院したAQP4抗体陽性NMOSDの男児例

  末宗 和樹, 國澤 久路, 伊藤 立人, 冨岡 和美, 石田 悠介, 西山 将広, 丸山 あずさ

  (一社)日本小児神経学会, May 2026, 脳と発達, 58(3) (3), 246 - 246, Japanese


• 無治療で軽快した皮質性脳炎に続発して視神経炎を呈した抗MOG抗体関連疾患の1例

  相馬 健人, 西山 将広, 伊藤 立人, 石田 悠介, 永瀬 裕朗, 丸山 あずさ

  (一社)日本小児神経学会, May 2026, 脳と発達, 58(3) (3), 246 - 246, Japanese


• Current status and future directions following the publication of the guidelines for treatment of pediatric status epilepticus 2023

  Nishiyama Masahiro, Shiohama Tadashi

  The Japanese Society of Child Neurology, 01 May 2026, NO TO HATTATSU, 58(3) (3), 213 - 216, Japanese


• 【小児神経学入門-小児科医必須の神経症候学と治療論】治療 てんかん重積状態の治療

  西山 将広

  (株)東京医学社, Apr. 2026, 小児内科, 58(4) (4), 511 - 516, Japanese


• 小児急性脳症の最新情報と課題 急性脳症治療の最適化:FACEレジストリからの新たな知見

  西山 将広

  (公社)日本小児科学会, Feb. 2026, 日本小児科学会雑誌, 130(2) (2), 176 - 176, Japanese


• 神経救急の進歩 小児てんかん重積状態・けいれん重積状態の治療法に関するアンケート調査 病院前治療の実態

  西山 将広, 植田 佑樹, 塩濱 直, 松浦 隆樹, 本林 光雄, 福岡 正隆, 老川 静香, 金井 創太郎, 鳥尾 倫子, 菊池 健二郎, 九鬼 一郎, 永瀬 裕朗, 秋山 倫之, 前垣 義弘

  (一社)日本てんかん学会, Sep. 2025, てんかん研究, 43(2) (2), 330 - 330, Japanese


• 当院PICUにおける持続脳波モニタリングの実施状況と臨床的意義に関する検討

  末宗 和樹, 西山 将広, 國澤 久路, 伊藤 立人, 石田 悠介, 青木 一憲, 椎間 優子, 黒澤 寛史, 丸山 あずさ

  (一社)日本てんかん学会, Sep. 2025, てんかん研究, 43(2) (2), 493 - 493, Japanese


• 小児てんかん重積状態・けいれん重積状態の治療法に関するアンケート調査 病院到着後の治療実態

  西山 将広, 植田 佑樹, 塩濱 直, 松浦 隆樹, 本林 光雄, 福岡 正隆, 老川 静香, 金井 創太郎, 鳥尾 倫子, 菊池 健二郎, 九鬼 一郎, 永瀬 裕朗, 秋山 倫之, 前垣 義弘

  (一社)日本てんかん学会, Sep. 2025, てんかん研究, 43(2) (2), 496 - 496, Japanese


• 1年以上生存した18トリソミー児に合併したてんかんの臨床的特徴

  石田 悠介, 西山 将広, 國澤 久路, 末宗 和樹, 伊藤 立人, 岩谷 壮太, 芳本 誠司, 丸山 あずさ

  (一社)日本てんかん学会, Sep. 2025, てんかん研究, 43(2) (2), 504 - 504, Japanese


• 循環不全を伴う難治性てんかん重積状態に対しケタミン持続静注が有効であった可逆性後頭葉白質脳症の1例

  鮫島 智大, 西山 将広, 伊藤 立人, 相馬 健人, 石田 悠介, 丸山 あずさ

  (一社)日本小児神経学会, Sep. 2025, 脳と発達, 57(5) (5), 382 - 382, Japanese


• ACTH療法後の早期再発にvigabatrinが有効であった滑脳症の1例

  相馬 健人, 西山 将広, 伊藤 立人, 鮫島 智大, 石田 悠介, 丸山 あずさ

  (一社)日本小児神経学会, Sep. 2025, 脳と発達, 57(5) (5), 383 - 383, Japanese


• 当院PICUにおける持続脳波モニタリングの実施状況と臨床的意義に関する検討

  末宗 和樹, 西山 将広, 國澤 久路, 伊藤 立人, 石田 悠介, 青木 一憲, 椎間 優子, 黒澤 寛史, 丸山 あずさ

  (一社)日本てんかん学会, Sep. 2025, てんかん研究, 43(2) (2), 493 - 493, Japanese


• 小児てんかん重積状態・けいれん重積状態の治療法に関するアンケート調査 病院到着後の治療実態

  西山 将広, 植田 佑樹, 塩濱 直, 松浦 隆樹, 本林 光雄, 福岡 正隆, 老川 静香, 金井 創太郎, 鳥尾 倫子, 菊池 健二郎, 九鬼 一郎, 永瀬 裕朗, 秋山 倫之, 前垣 義弘

  (一社)日本てんかん学会, Sep. 2025, てんかん研究, 43(2) (2), 496 - 496, Japanese


• 小児急性脳症の到達点と将来展望 急性脳症の治療最適化を目指して FACEレジストリからわかること

  西山 将広, 永瀬 裕朗

  (一社)日本小児神経学会, Jun. 2025, 脳と発達, 57(Suppl.) (Suppl.), s133 - s133, Japanese


• 小児急性脳症の到達点と将来展望 新型コロナウイルス感染症に伴う劇症脳浮腫型脳症と出血性ショック脳症症候群のアジア・オセアニア地域における25症例の臨床像

  葛西 真梨子, 佐久間 啓, 鈴木 基正, 河田 奈々子, 西山 将広, 高梨 潤一

  (一社)日本小児神経学会, Jun. 2025, 脳と発達, 57(Suppl.) (Suppl.), s134 - s134, Japanese


• てんかん重積状態治療の最前線

  西山 将広

  (一社)日本小児神経学会, Jun. 2025, 脳と発達, 57(Suppl.) (Suppl.), s179 - s179, Japanese


• 原因による治療戦略 熱性と無熱性,基礎疾患への配慮

  老川 静香, 西山 将広

  (一社)日本小児神経学会, Jun. 2025, 脳と発達, 57(Suppl.) (Suppl.), s181 - s181, Japanese


• 小児てんかんに対するラコサミド単剤療法における血中濃度と有効性との関係

  伊藤 立人, 西山 将広, 相馬 健人, 石田 悠介, 丸山 あずさ

  (一社)日本小児神経学会, Jun. 2025, 脳と発達, 57(Suppl.) (Suppl.), s238 - s238, Japanese


• 小児てんかんにおけるペランパネル開始後3ヵ月時点での有効性と有害事象

  相馬 健人, 西山 将広, 伊藤 立人, 石田 悠介, 丸山 あずさ

  (一社)日本小児神経学会, Jun. 2025, 脳と発達, 57(Suppl.) (Suppl.), s239 - s239, Japanese


• 熱性てんかん重積状態に続発する意識障害遷延の臨床像

  鮫島 智大, 西山 将広, 徳元 翔一, 山口 宏, 高梨 潤一, 本林 光雄, 柏木 充, 服部 有香, 丸山 あずさ, 豊嶋 大作, 中川 卓, 河野 剛, 永瀬 裕朗

  (一社)日本小児神経学会, Jun. 2025, 脳と発達, 57(Suppl.) (Suppl.), s256 - s256, Japanese


• 18トリソミー症候群における年齢別てんかん有病率の検討

  石田 悠介, 西山 将広, 伊藤 立人, 相馬 健人, 鮫島 智大, 岩谷 壮太, 芳本 誠司, 丸山 あずさ

  (一社)日本小児神経学会, Jun. 2025, 脳と発達, 57(Suppl.) (Suppl.), s267 - s267, Japanese


• 当院での抗MOG抗体関連疾患19例の後方視的検討

  上田 拓耶, 西山 将広, 相馬 健人, 石田 悠介, 徳元 翔一, 山口 宏, 野津 寛大, 丸山 あずさ, 永瀬 裕朗

  (一社)日本小児神経学会, Jun. 2025, 脳と発達, 57(Suppl.) (Suppl.), s269 - s269, Japanese


• 二相性臨床経過と遅発性拡散能低下を呈する乳児頭部外傷(TBIRD)における二相性発作の発症時期の検討

  川村 葵, 西山 将広, 伊藤 立人, 相馬 健人, 石田 悠介, 丸山 あずさ, 青木 一憲, 椎間 優子, 黒澤 寛史, 阿久津 宣行, 小山 淳二, 永瀬 裕朗

  (一社)日本小児神経学会, Jun. 2025, 脳と発達, 57(Suppl.) (Suppl.), s302 - s302, Japanese


• 発熱を伴う30分以上のけいれん性てんかん重積状態における転帰不良関連因子 連続症例多施設コホートでの検証

  老川 静香, 西山 将広, 徳元 翔一, 山口 宏, 高梨 潤一, 本林 光雄, 柏木 充, 服部 有香, 丸山 あずさ, 豊嶋 大作, 中川 卓, 河野 剛, 永瀬 裕朗

  (一社)日本小児神経学会, Jun. 2025, 脳と発達, 57(Suppl.) (Suppl.), s303 - s303, Japanese


• ケトン食療法を含む集学的治療で発作を軽減できた難治頻回部分発作重積型急性脳炎の1例

  奥田 太郎, 岡西 徹, 土江 宏和, 丸山 あずさ, 西山 将広, 松井 亮仁, 細田 龍生, 熊崎 健介, 太田 健人, 吉野 豪, 岡田 健太朗, 荒井 勇人, 中村 裕子, 前垣 義弘

  (一社)日本小児神経学会, Jun. 2025, 脳と発達, 57(Suppl.) (Suppl.), s303 - s303, Japanese


• Changes in electroencephalogram findings in a newborn with hemimegalencephaly

  Takahashi Eri, Kobayashi Takao, Nishimaki Ryo, Iwatani Sota, Soma Kento, Nishiyama Masahiro, Maruyama Azusa, Yoshimoto Seiji

  Hemimegalencephaly is a congenital brain malformation often accompanied by symptomatic epilepsy. In patients with refractory epilepsy onset during the neonatal period, psychomotor developmental delay tends to progress. We here report a newborn case who presented with seizure on day 3 of life and was diagnosed as left hemimegalencephaly via head magnetic resonance imaging on day 7 of life. Despite treatment with multiple antiepileptic drugs, including phenobarbital, levetiracetam, and lacosamide, clinical seizures and epileptic discharges on electroencephalogram（EEG）persisted. Slow wave activity in the background EEG of the unaffected hemisphere also progressed. To control the refractory epilepsy, a hemispherectomy was performed at 5 months of age. Following this, the frequency of seizure decreased, and the background EEG activity of the unaffected hemisphere normalized with single-drug therapy using levetiracetam. Her psychomotor developmental resumed. This report details the changes in clinical symptoms and EEG findings from the neonatal to the infancy period. We emphasize the importance of not only early diagnosis in the perinatal period but also subsequent epilepsy treatment in collaboration with neurologists and neurosurgeons in managing hemimegalencephaly.

  Japan Society of Perinatal and Neonatal Medicine, May 2025, Journal of Japan Society of Perinatal and Neonatal Medicine, 61(1) (1), 152 - 157, Japanese


• 【これからのガイドラインの読み方,使い方】ガイドラインのつくり エビデンスレベルが表すこと

  西山 将広

  (株)診断と治療社, Apr. 2025, 小児科診療, 88(春増刊) (春増刊), 25 - 31, Japanese


• 研究者の最新動向 小児摂食障害における脳波周波数解析を用いた脳機能評価

  冨岡 和美, 上田 拓耶, 西山 将広, 永瀬 裕朗

  (株)北隆館, Apr. 2025, Precision Medicine, 8(4) (4), 326 - 329, Japanese


• 【外来診療・診断のピットフォール】代表的な疾患のピットフォール 中枢神経系 川崎病だと思ったら細菌性髄膜炎

  西山 将広

  (株)東京医学社, Apr. 2025, 小児内科, 57(4) (4), 448 - 450, Japanese


• Arterial spin labeling imaging(ASL)で血流亢進を示したChiari奇形合併の劇症型小脳炎

  田中 元, 西山 将広, 伊藤 立人, 相馬 健人, 上田 拓耶, 石田 悠介, 丸山 あずさ

  (一社)日本小児神経学会, Mar. 2025, 脳と発達, 57(2) (2), 148 - 148, Japanese


• 黒質および淡蒼球が障害された後天性ジストニアに対してL-dopaが有効であった1例

  相馬 健人, 西山 将広, 上田 拓耶, 石田 悠介, 西藤 知城, 尾崎 佳代, 黒澤 寛史, 丸山 あずさ

  (一社)日本小児神経学会, Nov. 2024, 脳と発達, 56(6) (6), 460 - 460, Japanese


• 有熱性けいれん小児患者に対する救急外来での脳波モニタリング開始時期と神経学的転帰との関連

  丸山 あずさ, 相馬 健人, 上田 拓耶, 石田 悠介, 西山 将広, 永瀬 裕朗

  (一社)日本てんかん学会, Sep. 2024, てんかん研究, 42(2) (2), 481 - 481, Japanese


• 小児病院における抗てんかん薬処方の2016年から2023年までの経年変化

  西山 将広, 相馬 健人, 上田 拓耶, 石田 悠介, 丸山 あずさ

  (一社)日本てんかん学会, Sep. 2024, てんかん研究, 42(2) (2), 516 - 516, Japanese


• 低用量のフェンフルラミンで効果が得られたDravet症候群の5例

  上田 拓耶, 西山 将広, 相馬 健人, 石田 悠介, 徳元 翔一, 山口 宏, 野津 寛大, 永瀬 裕朗, 丸山 あずさ

  (一社)日本てんかん学会, Sep. 2024, てんかん研究, 42(2) (2), 519 - 519, Japanese


• 前方視的多施設症例集積により明らかにする小児有熱性けいれん重積に対する管理の実際

  徳元 翔一, 西山 将広, 柏木 充, 河野 剛, 高梨 潤一, 中川 卓, 豊嶋 大作, 服部 有香, 丸山 あずさ, 本林 光雄

  (一社)日本てんかん学会, Sep. 2024, てんかん研究, 42(2) (2), 531 - 531, Japanese


• 脳保護のための小児神経集中治療

  西山 将広, 青木 一憲, 黒澤 寛史, 丸山 あずさ, 永瀬 裕朗

  (公社)日本小児科学会, Aug. 2024, 日本小児科学会雑誌, 128(8) (8), 1031 - 1044, Japanese


• 脳保護のための小児神経集中治療 脳傷害はこうやって科学する! 小児神経科の頭の中

  西山 将広

  (一社)日本小児神経学会, May 2024, 脳と発達, 56(Suppl.) (Suppl.), S113 - S113, Japanese


• 前向き多施設レジストリにより明らかにする急性脳症の特徴

  徳元 翔一, 西山 将広, 柏木 充, 河野 剛, 高梨 潤一, 中川 卓, 豊嶋 大作, 服部 有香, 丸山 あずさ, 本林 光雄, 永瀬 裕朗

  (一社)日本小児神経学会, May 2024, 脳と発達, 56(Suppl.) (Suppl.), S200 - S200, Japanese


• 小児蘇生後症例の神経学的瞳孔指数による転帰の予測

  川本 昌平, 青木 一憲, 潮見 祐樹, 上田 拓耶, 西山 将広, 丸山 あずさ, 黒澤 寛史

  (一社)日本小児神経学会, May 2024, 脳と発達, 56(Suppl.) (Suppl.), S204 - S204, Japanese


• 3歳時の朝食習慣と小学1年生での学力との関連 尼崎市の人口ベースコホート研究

  川村 葵, 西山 将広, 伊藤 立人, 京野 由紀, 鮫島 智大, 花房 宏昭, 老川 静香, 徳元 翔一, 山口 宏, 野津 寛大, 永瀬 裕朗

  (一社)日本小児神経学会, May 2024, 脳と発達, 56(Suppl.) (Suppl.), S206 - S206, Japanese


• 急性脳症患者における治療介入前後の血清GDF-15の経時的推移

  山口 宏, 伊藤 立人, 相馬 健人, 川村 葵, 鮫島 智大, 京野 由紀, 上田 拓耶, 老川 静香, 徳元 翔一, 石田 悠介, 西山 将広, 丸山 あずさ, 野津 寛大, 永瀬 裕朗

  (一社)日本小児神経学会, May 2024, 脳と発達, 56(Suppl.) (Suppl.), S225 - S225, Japanese


• 有熱性てんかん重積状態における髄液神経傷害バイオマーカーの検討

  老川 静香, 山口 宏, 伊藤 立人, 相馬 建人, 川村 葵, 上田 拓郎, 鮫島 智大, 京野 由紀, 徳元 翔一, 石田 悠介, 西山 将広, 丸山 あずさ, 野津 寛大, 永瀬 裕朗

  (一社)日本小児神経学会, May 2024, 脳と発達, 56(Suppl.) (Suppl.), S225 - S225, Japanese


• 有熱性けいれん重積に対する初期治療までの時間と入院後治療・転帰の関係

  相馬 健人, 西山 将広, 上田 拓耶, 石田 悠介, 永瀬 裕朗, 丸山 あずさ

  (一社)日本小児神経学会, May 2024, 脳と発達, 56(Suppl.) (Suppl.), S229 - S229, Japanese


• Dravet症候群におけるてんかん重積発作後または発作群発後の再発予防としてのレベチラセタムとホスフェニトインの有効性

  上田 拓耶, 西山 将広, 相馬 健人, 石田 悠介, 丸山 あずさ, 永瀬 裕朗

  (一社)日本小児神経学会, May 2024, 脳と発達, 56(Suppl.) (Suppl.), S231 - S231, Japanese


• 機能性神経学的症状症と診断された3例で認めた類似する脳血流異常と髄液サイトカイン所見

  田中 司, 青砥 悠哉, 百々 菜月, 二野 菜々子, 徳田 央士, 山本 寛子, 松野下 夏樹, 竹中 佳奈栄, 富永 健太, 忍頂寺 毅史, 川崎 圭一郎, 西山 将広, 永瀬 裕朗, 高橋 幸利

  (一社)日本小児神経学会, May 2024, 脳と発達, 56(Suppl.) (Suppl.), S298 - S298, Japanese


• 1歳6ヵ月児の神経学的発達の検討 新型コロナウイルス感染症流行前後での変化

  京野 由紀, 西山 将広, 永瀬 裕朗

  (一社)日本小児精神神経学会, Apr. 2024, 小児の精神と神経, 64(1) (1), 82 - 83, Japanese


• 3歳時の就寝時刻が遅いと小学1年生時の勤勉性や思いやりが低下する 尼崎市コホート研究

  西山 将広, 京野 由紀, 川村 葵, 老川 静香, 徳元 翔一, 山口 宏, 冨岡 和美, 野津 寛大, 永瀬 裕朗

  (公社)日本小児科学会, Apr. 2024, 日本小児科学会雑誌, 128(4) (4), 625 - 625, Japanese


• 薬剤抵抗性てんかんへの移行が懸念された左片側巨脳症の新生児例

  高橋 英里, 小林 孝生, 西牧 良, 岩谷 壮太, 原 将輝, 田中 敦, 西内 徳子, 宇保 早希子, 後藤 弘樹, 中山 栗太, 生田 寿彦, 武岡 恵美子, 松井 紗智子, 玉置 祥子, 三村 仁美, 西山 将広, 丸山 あずさ, 芳本 誠司

  (公社)日本小児科学会, Apr. 2024, 日本小児科学会雑誌, 128(4) (4), 629 - 629, Japanese


• MRIのarterial spin-labelingで体重減少時と回復期で脳血流を評価した神経性やせ症の13歳女子

  上田 拓耶, 西山 将広, 相馬 健人, 石田 悠介, 小川 裕子, 青木 一憲, 黒澤 寛史, 丸山 あずさ

  (一社)日本小児神経学会, Mar. 2024, 脳と発達, 56(2) (2), 152 - 152, Japanese


• 3歳児の睡眠習慣と小学1年生の学力および非認知能力との関連

  西山 将広, 野津 寛大, 永瀬 裕朗

  (公社)日本小児科学会, Feb. 2024, 日本小児科学会雑誌, 128(2) (2), 188 - 188, Japanese


• 体重減少時と回復期にArterial Spin-Labelingで脳血流と脳波を評価した神経性やせ症の1例

  上田 拓耶, 西山 将広, 相馬 健人, 石田 悠介, 小川 裕子, 青木 一憲, 黒澤 寛史, 丸山 あずさ

  (公社)日本小児科学会, Feb. 2024, 日本小児科学会雑誌, 128(2) (2), 219 - 219, Japanese


• 前向き多施設レジストリによる急性脳症の病型および治療の実態調査

  西山 将広, 徳元 翔一, 山口 宏, 高梨 潤一, 本林 光雄, 柏木 充, 服部 有香, 丸山 あずさ, 豊嶋 大作, 中川 卓, 河野 剛, 永瀬 裕朗

  (公社)日本小児科学会, Feb. 2024, 日本小児科学会雑誌, 128(2) (2), 253 - 253, Japanese


• 高IL-6血症を伴う後期早産児にみられた脳室周囲の高度な白質障害

  小林 孝生, 岩谷 壮太, 萩元 慎二, 西山 将広, 丸山 あずさ, 芳本 誠司

  (公社)日本小児科学会, Feb. 2024, 日本小児科学会雑誌, 128(2) (2), 355 - 355, Japanese


• Autoimmune glial fibrillary acidic protein astrocytopathy in children diagnosed by characteristic imaging findings

  Ueda Takuya, Hongo Hiroto, Ishida Yusuke, Nishiyama Masahiro, Toyoshima Daisaku, Yamaguchi Yoshimichi, Norimoto Syuhei, Kimura Akio, Maruyama Azusa

  Recently, encephalitis associated with antibodies to glial fibrillary acidic protein (GFAP), which presents in the cytoskeleton of astrocytes, has been proposed as a new disease. Although it presents with characteristic imaging findings of subacute meningoencephalitis and meningoencephalomyelitis, most patients are adult. In recent years, pediatric patients have been reported from foreign countries ; however, only one case has been reported from Japan. Our patient was a 5-year 8-month-old boy. He had no previous medical history and presented with prolonged fever, meningeal irritation, and altered consciousness. Despite an elevated cerebrospinal fluid cell count, infection was ruled out. Inflammatory demyelinating disease was suspected and the patient was started on steroid pulse therapy. The disturbance of consciousness improved after the start of treatment. Diffusion-weighted magnetic resonance imaging showed high spotty and linear signals in the periventricular white matter and corpus callosum bilaterally ; additionally, positive spinal fluid anti-GFAP antibodies led to the diagnosis of GFAP astrocytopathy. Furthermore, serum anti-myelin oligodendrocyte glycoprotein antibody and serum anti-aquaporin 4 antibody were negative. The patient underwent a total of three courses of steroid pulses and was discharged from the hospital without sequelae. Prednisolone was tapered off over a period of approximately 8 months without any recurrence. Autoimmune GFAP astrocytopathy might not be rare in Japan, it may remain undiagnosed. Therefore, it should be suspected in children with steroid-responsive meningoencephalitis or meningoencephalomyelitis, or in patients of central inflammatory diseases with characteristic imaging findings.

  The Japanese Society of Child Neurology, Nov. 2023, NO TO HATTATSU, 55(6) (6), 438 - 442, Japanese


• 1歳6ヵ月児の神経学的発達の検討 新型コロナウイルス感染症流行前後での変化

  京野 由紀, 西山 将広, 永瀬 裕朗

  (一社)日本小児精神神経学会, Nov. 2023, 日本小児精神神経学会プログラム・抄録集, 130回, 77 - 77, Japanese


• 【エキスパートが教える 小児の薬物治療】疾患別 E.神経筋疾患 急性脳症

  西山 将広, 永瀬 裕朗

  (株)東京医学社, Nov. 2023, 小児内科, 55(増刊) (増刊), 508 - 512, Japanese


• 意識障害を呈した小児に対する救急外来簡易脳波の原因疾患別特徴

  山口 宏, 上田 拓耶, 老川 静香, 徳元 翔一, 西山 将広, 丸山 あずさ, 野津 寛大, 永瀬 裕朗

  (一社)日本てんかん学会, Sep. 2023, てんかん研究, 41(2) (2), 326 - 326, Japanese


• Dravet症候群に合併した急性脳症の4例

  上田 拓耶, 西山 将広, 相馬 健人, 石田 悠介, 丸山 あずさ, 永瀬 裕朗

  (一社)日本てんかん学会, Sep. 2023, てんかん研究, 41(2) (2), 349 - 349, Japanese


• 有熱性けいれん発作をきたした小児における発症早期の非けいれん性発作

  丸山 あずさ, 上田 拓耶, 石田 悠介, 山口 宏, 西山 将広, 永瀬 裕朗

  (一社)日本てんかん学会, Sep. 2023, てんかん研究, 41(2) (2), 421 - 421, Japanese


• 発達性てんかん性脳症に対してACTH療法が有効であったが再発したCabezas型X連鎖性知的障害の一例

  石田 悠介, 相馬 健人, 上田 拓耶, 西山 将広, 森貞 直哉, 丸山 あずさ

  (一社)日本てんかん学会, Sep. 2023, てんかん研究, 41(2) (2), 476 - 476, Japanese


• 【小児の集中治療の実践-躬行実践-】重症疾患の管理 神経疾患とその管理 急性脳症を中心に

  西山 将広

  (株)東京医学社, Aug. 2023, 小児内科, 55(8) (8), 1356 - 1360, Japanese


• サイトカインストーム型の急性脳症をきたした小児COVID-19の2症例

  當間 圭一郎, 伊藤 由作, 青木 一憲, 黒澤 寛史, 上田 拓耶, 本郷 裕斗, 西山 将広, 丸山 あずさ

  (一社)日本集中治療医学会, Jun. 2023, 日本集中治療医学会雑誌, 30(Suppl.1) (Suppl.1), S850 - S850, Japanese


• 新型コロナウイルス感染症(COVID-19)の流行が1歳6ヵ月児の発達に及ぼす影響

  京野 由紀, 西山 将広, 老川 静香, 徳元 翔一, 山口 宏, 冨岡 和美, 三品 浩基, 野津 寛大, 永瀬 裕朗

  (公社)日本小児保健協会, May 2023, 小児保健研究, 82(講演集) (講演集), 138 - 138, Japanese


• 我が国における出血性ショック脳症症候群(HSES)の病態と治療

  西山 将広, 徳元 翔一, 山口 宏, 冨岡 和美, 石田 悠介, 丸山 あずさ, 永瀬 裕朗

  (一社)日本小児神経学会, May 2023, 脳と発達, 55(Suppl.) (Suppl.), S212 - S212, Japanese


• 3歳時の就寝時刻または睡眠時間と小学1年生の学力との関連 尼崎市の人口ベースの研究

  西山 将広, 京野 由紀, 川村 葵, 老川 静香, 徳元 翔一, 山口 宏, 冨岡 和美, 野津 寛大, 永瀬 裕朗

  (一社)日本小児神経学会, May 2023, 脳と発達, 55(Suppl.) (Suppl.), S284 - S284, Japanese


• 小児てんかん重積状態に対する病院前治療としてのミダゾラム頬粘膜投与製剤の安全性

  上田 拓耶, 西山 将広, 本郷 裕斗, 石田 悠介, 丸山 あずさ, 永瀬 裕朗

  (一社)日本小児神経学会, May 2023, 脳と発達, 55(Suppl.) (Suppl.), S287 - S287, Japanese


• 出血性ショック脳症症候群をきたしたDravet症候群にSCN1Aのイントロンに新規の21塩基欠失を認めminigene解析を実施した1例

  花房 宏昭, 山口 宏, 老川 静香, 徳元 翔一, 冨岡 和美, 西山 将広, 長坂 美和子, 近藤 秀仁, 森貞 直哉, 松尾 雅文, 野津 寛大, 永瀬 裕朗

  (一社)日本小児神経学会, May 2023, 脳と発達, 55(Suppl.) (Suppl.), S302 - S302, Japanese


• 新型コロナウイルス感染症(COVID-19)により有熱性けいれんを認めた患者の臨床的特徴

  本郷 裕斗, 西山 将広, 上田 拓耶, 石田 悠介, 笠井 正志, 田中 亮二郎, 永瀬 裕朗, 丸山 あずさ

  (一社)日本小児神経学会, May 2023, 脳と発達, 55(Suppl.) (Suppl.), S310 - S310, Japanese


• 前向き多施設レジストリによる有熱性けいれん性発作後の意識障害持続時間と転帰との関連

  徳元 翔一, 西山 将広, 柏木 充, 高梨 潤一, 高見 勇一, 豊嶋 大作, 服部 有香, 丸山 あずさ, 本林 光雄, 永瀬 裕朗

  (一社)日本小児神経学会, May 2023, 脳と発達, 55(Suppl.) (Suppl.), S315 - S315, Japanese


• 発熱を伴う30分以上のけいれん性てんかん重積状態における転帰不良関連因子 前向き多施設コホートでの検証

  老川 静香, 徳元 翔一, 山口 宏, 冨岡 和美, 西山 将広, 柏木 充, 高梨 潤一, 高見 勇一, 豊嶋 大作, 服部 有香, 丸山 あずさ, 本林 光雄, 永瀬 裕朗

  (一社)日本小児神経学会, May 2023, 脳と発達, 55(Suppl.) (Suppl.), S316 - S316, Japanese


• 有熱性てんかん重積・急性脳症に対する疾患関連遺伝子の探索

  山口 宏, 花房 宏昭, 老川 静香, 徳元 翔一, 冨岡 和美, 西山 将広, 森貞 直哉, 野津 寛大, 永瀬 裕朗

  (一社)日本小児神経学会, May 2023, 脳と発達, 55(Suppl.) (Suppl.), S318 - S318, Japanese


• サイトメガロウイルス初感染によりHSESおよびHLH病態を呈した乳児例

  原田 晋二, 西山 将広, 水田 麻雄, 上田 拓耶, 本郷 裕斗, 石田 悠介, 中岸 保夫, 黒澤 寛史, 丸山 あずさ

  (一社)日本小児神経学会, May 2023, 脳と発達, 55(Suppl.) (Suppl.), S325 - S325, Japanese


• COVID-19感染症を契機に急性増悪した全身型重症筋無力症の女児例

  金谷 真吾, 岩本 宗矩, 山口 宏, 南部 静紀, 徳元 翔一, 坊 亮輔, 冨岡 和美, 西山 将広, 粟野 宏之, 野津 寛大, 永瀬 裕朗

  (公社)日本小児科学会, Apr. 2023, 日本小児科学会雑誌, 127(4) (4), 627 - 627, Japanese


• 当院での脳症関連遺伝子パネルを用いた疾患関連遺伝子の同定の試み

  山口 宏, 花房 宏昭, 徳元 翔一, 冨岡 和美, 西山 将広, 森貞 直哉, 野津 寛大, 永瀬 裕朗

  (公社)日本小児科学会, Apr. 2023, 日本小児科学会雑誌, 127(4) (4), 628 - 628, Japanese


• 脱髄病変を伴わず髄膜の造影効果のみを認めた抗MOG抗体陽性の髄膜脳炎の4歳児

  山根 徹也, 上田 拓耶, 西山 将広, 本郷 裕斗, 石田 悠介, 丸山 あずさ

  (公社)日本小児科学会, Feb. 2023, 日本小児科学会雑誌, 127(2) (2), 267 - 267, Japanese


• Nav1.4ポアドメインの新規ミスセンス変異による高カリウム性周期性四肢麻痺家系の病態解析

  瀬川 和樹, 西山 将広, 森 樹貴, 久保田 智哉, 高橋 正紀

  (一社)日本臨床神経生理学会, Oct. 2022, 臨床神経生理学, 50(5) (5), 407 - 407, Japanese


• Nav1.4ポアドメインの新規ミスセンス変異による高カリウム性周期性四肢麻痺家系の病態解析

  瀬川 和樹, 西山 将広, 森 樹貴, 久保田 智哉, 高橋 正紀

  (一社)日本臨床神経生理学会, Oct. 2022, 臨床神経生理学, 50(5) (5), 407 - 407, Japanese


• てんかん重積状態・急性脳症における疾患原因遺伝子の同定の試み

  山口 宏, 花房 宏昭, 徳元 翔一, 冨岡 和美, 西山 将広, 森貞 直哉, 野津 寛大, 永瀬 裕朗

  (一社)日本てんかん学会, Aug. 2022, てんかん研究, 40(2) (2), 410 - 410, Japanese


• 小児てんかん重積状態に対するミダゾラム頬粘膜投与製剤の投与量

  上田 拓郎, 西山 将広, 本郷 裕斗, 石田 悠介, 丸山 あずさ

  (一社)日本てんかん学会, Aug. 2022, てんかん研究, 40(2) (2), 417 - 417, Japanese


• 発熱を伴う30分以上のけいれん性てんかん重積状態における転帰不良の予測

  西山 将広, 丸山 あずさ, 高梨 潤一, 本林 光雄, 服部 有香, 豊嶋 大作, 高見 勇一, 徳元 翔一, 永瀬 裕朗

  (一社)日本てんかん学会, Aug. 2022, てんかん研究, 40(2) (2), 457 - 457, Japanese


• 3歳児の神経発達予後と周産期における母体のメンタルヘルスとの関連

  京野 由紀, 西山 将広, 徳元 翔一, 山口 宏, 冨岡 和美, 三品 浩基, 野津 寛大, 永瀬 裕朗

  収集根拠 : オンライン資料収集制度 資料形態 : テキストデータ コレクション : 国立国会図書館デジタルコレクション > 電子書籍・電子雑誌 > 学術機関 > 学協会

  (公社)日本小児保健協会, May 2022, 小児保健研究, 81(講演集) (講演集), 152 - 152, Japanese


• 小児けいれん重積治療ガイドライン2017の改訂の要点 第1選択薬(ベンゾジアゼピン系薬剤)を考える

  西山 将広

  (一社)日本小児神経学会, May 2022, 脳と発達, 54(Suppl.) (Suppl.), S154 - S154, Japanese


• オナセムノゲンアベパルボベク治療後に、ヌシネルセン治療を追加した脊髄性筋萎縮症1型の1例

  南部 静紀, 坊 亮輔, 徳元 翔一, 山口 宏, 冨岡 和美, 西山 将広, 永瀬 裕朗, 西尾 久英, 野津 寛大, 粟野 宏之

  (一社)日本小児神経学会, May 2022, 脳と発達, 54(Suppl.) (Suppl.), S210 - S210, Japanese


• 小児てんかん重積に対するミダゾラム頬粘膜投与製剤の有効性と安全性 単一施設29機会の経験から

  上田 拓耶, 本郷 裕斗, 石田 悠介, 西山 将広, 豊嶋 大作, 丸山 あずさ, 田中 亮二郎, 永瀬 裕朗

  (一社)日本小児神経学会, May 2022, 脳と発達, 54(Suppl.) (Suppl.), S223 - S223, Japanese


• ホスフェニトインによる有熱性けいれん重積後の発作再発予防の有効性

  本郷 裕斗, 上田 拓耶, 石田 悠介, 西山 将広, 豊嶋 大作, 丸山 あずさ, 田中 亮二郎, 永瀬 裕朗

  (一社)日本小児神経学会, May 2022, 脳と発達, 54(Suppl.) (Suppl.), S223 - S223, Japanese


• 熱性けいれんにおける発症後早期のサイトカイン動態

  徳元 翔一, 西山 将広, 上田 拓耶, 本郷 裕斗, 山口 宏, 石田 悠介, 冨岡 和美, 豊嶋 大作, 丸山 あずさ, 野津 寛大, 石田 明人, 永瀬 裕朗

  (一社)日本小児神経学会, May 2022, 脳と発達, 54(Suppl.) (Suppl.), S251 - S251, Japanese


• 有熱性てんかん重積予後予測バイオマーカーとしてのgrowth and differentiation factor-15

  山口 宏, 西山 将広, 上田 拓耶, 本郷 裕斗, 徳元 翔一, 石田 悠介, 冨岡 和美, 豊嶋 大作, 中川 卓, 高見 勇一, 黒澤 寛史, 丸山 あずさ, 野津 寛大, 永瀬 裕朗

  (一社)日本小児神経学会, May 2022, 脳と発達, 54(Suppl.) (Suppl.), S252 - S252, Japanese


• 前向き多施設レジストリより算出した6時間以上の意識障害遷延例における急性脳症と転帰不良の発生頻度

  西山 将広, 高梨 潤一, 本林 光雄, 服部 有香, 丸山 あずさ, 親里 嘉展, 高見 勇一, 永瀬 裕朗

  (一社)日本小児神経学会, May 2022, 脳と発達, 54(Suppl.) (Suppl.), S253 - S253, Japanese


• 神戸市東部療育センター診療所における発達障害に合併した摂食障害のまとめ

  冨岡 和美, 永瀬 裕朗, 徳元 翔一, 山口 宏, 西山 将広, 岡田 由香, 野津 寛大

  (一社)日本小児神経学会, May 2022, 脳と発達, 54(Suppl.) (Suppl.), S317 - S317, Japanese


• 初回発症時にMASを合併した若年性特発性若年性関節炎の1例

  吉本 啓修, 洪 聖媛, 古林 真佐美, 南部 静紀, 徳元 翔一, 山口 宏, 坊 亮輔, 冨岡 和美, 西山 将広, 粟野 宏之, 永瀬 裕朗, 飯島 一誠

  (公社)日本小児科学会, Mar. 2022, 日本小児科学会雑誌, 126(3) (3), 545 - 545, Japanese


• 過去5年間の小児摂食障害に対する家族療法の経験

  冨岡 和美, 永瀬 裕朗, 洪 聖媛, 南部 静紀, 徳元 翔一, 山口 宏, 坊 亮輔, 西山 将広, 粟野 宏之

  (公社)日本小児科学会, Mar. 2022, 日本小児科学会雑誌, 126(3) (3), 551 - 551, Japanese


• 小児科医の意識調査 大事にする価値観は?兵庫県で次に取り組むべき課題は?

  西山 将広, 山本 暢之, 堀之内 智子, 藤岡 一路, 永瀬 裕朗, 野津 寛大

  (公社)日本小児科学会, Mar. 2022, 日本小児科学会雑誌, 126(3) (3), 557 - 557, Japanese


• COVID-19蔓延前後の川崎病の発生頻度 神戸市population-based study,2016-2020

  飯尾 享平, 夏木 茜, 中尾 聡宏, 金 伽耶, 内藤 昭嘉, 川崎 悠, 磯目 賢一, 堀 雅之, 岩田 あや, 山口 善道, 平海 良美, 松原 康策, 宮越 千智, 太田 國隆, 山岡 利佳, 江口 純治, 松村 治, 奥谷 貴弘, 上田 育代, 西山 将広

  (公社)日本小児科学会, Feb. 2022, 日本小児科学会雑誌, 126(2) (2), 324 - 324, Japanese


• 幼児期の生活習慣と小学1年生の学力との関連 3歳時点での就寝時刻が遅いと学力が低下する

  西山 将広, 徳元 翔一, 山口 宏, 冨岡 和美, 野津 寛大, 永瀬 裕朗

  (公社)日本小児科学会, Feb. 2022, 日本小児科学会雑誌, 126(2) (2), 369 - 369, Japanese


• 学力および非認知能力に影響を与える乳幼児期因子の解明

  西山 将広

  [東京] : 小児医学研究振興財団, 2022, 公益財団法人小児医学研究振興財団研究報告書, 27 - 29, Japanese


• 熱性けいれんと発熱患者におけるサイトカインの比較

  徳元 翔一, 山口 宏, 冨岡 和美, 西山 将広, 飯島 一誠, 永瀬 裕朗

  (一社)日本小児神経学会, May 2021, 脳と発達, 53(Suppl.) (Suppl.), S267 - S267, Japanese


• 有熱性けいれん重積症例における意識障害遷延時間毎のAESD発症リスク

  石田 悠介, 西山 将広, 本郷 裕斗, 徳元 翔一, 山口 宏, 冨岡 和美, 豊嶋 大作, 丸山 あずさ, 永瀬 裕朗

  (一社)日本小児神経学会, May 2021, 脳と発達, 53(Suppl.) (Suppl.), S268 - S268, Japanese


• 急性脳症・有熱性けいれん重積の前向き多施設レジストリより算出した有熱性けいれん重積に占めるAESD発症頻度

  西山 将広, 本林 光雄, 福山 哲広, 服部 有香, 丸山 あずさ, 親里 嘉展, 高見 勇一, 永瀬 裕朗

  (一社)日本小児神経学会, May 2021, 脳と発達, 53(Suppl.) (Suppl.), S268 - S268, Japanese


• Growth and differentiation factor 15 出血性ショック脳症症候群と他の有熱性けいれん性疾患の早期鑑別マーカーとしての検討

  山口 宏, 西山 将広, 本郷 裕斗, 徳元 翔一, 石田 悠介, 冨岡 和美, 中川 卓, 高見 勇一, 豊嶋 大作, 丸山 あずさ, 飯島 一誠, 永瀬 裕朗

  (一社)日本小児神経学会, May 2021, 脳と発達, 53(Suppl.) (Suppl.), S274 - S274, Japanese


• 3歳児健診における発達通過状況とけいれん既往との関連

  西山 将広, 京野 由紀, 徳元 翔一, 山口 宏, 冨岡 和美, 藤岡 一路, 三品 浩基, 飯島 一誠, 永瀬 裕朗

  (公社)日本小児科学会, Feb. 2021, 日本小児科学会雑誌, 125(2) (2), 273 - 273, Japanese


• A SMA1 infant who received Zolgensma therapy at the age of 50 days

  南部静紀, 粟野宏之, 洪聖媛, 徳元翔一, 山口宏, 坊亮輔, 冨岡和美, 西山将広, 篠原正和, 永瀬裕朗, 西尾久英, 飯島一誠

  (公社)日本小児科学会, 2021, 日本小児科学会雑誌, 125(2) (2), 291 - 291, Japanese


• 小児けいれん重積治療ガイドライン改訂に向けて 難治性てんかん重積の治療 ミダゾラムまたはバルビツレートによる麻酔療法

  西山 将広, 永瀬 裕朗, 丸山 あずさ

  (一社)日本小児神経学会, Aug. 2020, 脳と発達, 52(Suppl.) (Suppl.), S161 - S161, Japanese


• 有熱性けいれんを主訴に入院した小児におけるけいれん時間毎のAESD発症頻度と予後 単一施設の連続症例コホートでの検討

  徳元 翔一, 豊嶋 大作, 丸山 あずさ, 山口 宏, 石田 悠介, 富岡 和美, 西山 将広, 飯島 一誠, 永瀬 裕朗

  (一社)日本小児神経学会, Aug. 2020, 脳と発達, 52(Suppl.) (Suppl.), S232 - S232, Japanese


• 急性脳症の予測基準の有用性の検証(第2報) 後遺症またはAESD予測のために開発された基準の比較

  西山 将広, 石田 悠介, 徳元 翔一, 冨岡 和美, 山口 宏, 豊嶋 大作, 丸山 あずさ, 飯島 一誠, 永瀬 裕朗

  (一社)日本小児神経学会, Aug. 2020, 脳と発達, 52(Suppl.) (Suppl.), S263 - S263, Japanese


• 急性脳症の予測基準の有用性の検証(第1報) AESD予測のために開発された基準の別コホートにおける再検証

  石田 悠介, 西山 将広, 徳元 翔一, 山口 宏, 冨岡 和美, 豊嶋 大作, 丸山 あずさ, 飯島 一誠, 永瀬 裕朗

  (一社)日本小児神経学会, Aug. 2020, 脳と発達, 52(Suppl.) (Suppl.), S264 - S264, Japanese


• けいれん重積型(二相性)急性脳症の初回けいれん時間による臨床像の比較検討

  山口 宏, 西山 将広, 徳元 翔一, 石田 悠介, 冨岡 和美, 豊嶋 大作, 丸山 あずさ, 飯島 一誠, 永瀬 裕朗

  (一社)日本小児神経学会, Aug. 2020, 脳と発達, 52(Suppl.) (Suppl.), S265 - S265, Japanese


• 有熱時けいれん/意識障害の発症6時間以内のサイトカインによる予後予測 血清IL-1RA,IL-10の可能性

  冨岡 和美, 西山 将広, 山口 宏, 石田 悠介, 徳元 翔一, 豊嶋 大作, 丸山 あずさ, 服部 有香, 親里 嘉展, 中川 卓, 高見 勇一, 多田 弘子, 佐久間 啓, 飯島 一誠, 永瀬 裕朗

  (一社)日本小児神経学会, Aug. 2020, 脳と発達, 52(Suppl.) (Suppl.), S266 - S266, Japanese


• 発熱に伴うけいれん・意識障害小児患者の長期予後

  豊嶋 大作, 徳元 翔一, 山口 宏, 石田 悠介, 冨岡 和美, 西山 将広, 永瀬 裕朗, 丸山 あずさ

  (一社)日本小児神経学会, Aug. 2020, 脳と発達, 52(Suppl.) (Suppl.), S266 - S266, Japanese


• 神経筋疾患患者に対する排痰補助装置のオシレーション機能が及ぼす気道分泌物除去効果に関する検討

  粟野 宏之, 永井 正志, 坊 亮輔, 石田 悠介, 冨岡 和美, 西山 将広, 竹田 洋樹, 永瀬 裕朗, 飯島 一誠

  (一社)日本小児神経学会, Aug. 2020, 脳と発達, 52(Suppl.) (Suppl.), S300 - S300, Japanese


• 詳細な問診から診断に至った線維軟骨塞栓症の一例

  山口宏, 山口宏, 徳元翔一, 徳元翔一, 石田悠介, 石田悠介, 冨岡和美, 西山将広, 豊嶋大作, 丸山あずさ, 永瀬裕朗

  (公社)日本小児科学会, 2020, 日本小児科学会雑誌, 124(2) (2), 398 - 398, Japanese


• 神戸市における熱性けいれんの発症頻度:後期早産であることのリスクの検討

  西山将広, 山口宏, 石田悠介, 冨岡和美, 三品浩基, 飯島一誠, 永瀬裕朗

  (公社)日本小児科学会, 2020, 日本小児科学会雑誌, 124(2) (2), 288 - 288, Japanese


• 胃腸炎罹患後に低血糖発作をおこした中鎖アシルCoA脱水素酵素(MCAD)欠損症の1例

  坊亮輔, 粟野宏之, 永井正志, 中西啓太, 石田悠介, 冨岡和美, 村尾真理子, 田中司, 西山将広, 奥谷貴弘, 永瀬裕朗, 飯島一誠

  (公社)日本小児科学会, 2020, 日本小児科学会雑誌, 124(6) (6), 1036 - 1037, Japanese


• 治療抵抗性の眼筋型重症筋無力症に対してステロイドパルス療法を行った2歳女児例

  洪聖媛, 田中司, 西山将広, 永井正志, 坊亮輔, 石田悠介, 冨岡和美, 村尾真理子, 粟野宏之, 永瀬裕朗, 飯島一誠

  2020, 日本小児科学会雑誌, 124(6) (6)


• 新規STXBP1変異を認め大田原症候群と診断したSTXBP1脳症

  山口 宏, 永瀬 裕朗, 西山 将広, 徳元 翔一, 豊嶋 大作, 丸山 あずさ, 森貞 直哉, 飯島 一誠

  (一社)日本てんかん学会, Sep. 2019, てんかん研究, 37(2) (2), 637 - 637, Japanese


• 小児難治てんかん重積状態における急性期の血中サイトカイン推移

  冨岡 和美, 西山 将広, 山口 宏, 石田 悠介, 徳元 翔一, 豊嶋 大作, 丸山 あずさ, 永瀬 裕朗, 飯島 一誠

  (一社)日本てんかん学会, Sep. 2019, てんかん研究, 37(2) (2), 675 - 675, Japanese


• 在胎週数毎に層別化した熱性けいれんの発生頻度 population-based study

  西山 将広, 永瀬 裕朗, 山口 宏, 石田 悠介, 冨岡 和美, 三品 浩基, 飯島 一誠

  (一社)日本てんかん学会, Sep. 2019, てんかん研究, 37(2) (2), 705 - 705, Japanese


• 小児けいれん重積に対する脳波モニタリング下ミダゾラム昏睡療法の有効性と安全性

  石田 悠介, 冨岡 和美, 田中 司, 西山 将広, 永瀬 裕朗, 徳元 翔一, 山口 宏, 豊嶋 大作, 丸山 あずさ, 黒澤 寛史, 飯島 一誠

  (一社)日本小児神経学会, May 2019, 脳と発達, 51(Suppl.) (Suppl.), S239 - S239, Japanese


• 有熱性難治性けいれん重積の治療プロトコル変遷による短期的予後の検討

  徳元 翔一, 山口 宏, 豊嶋 大作, 丸山 あずさ, 石田 悠介, 富岡 和美, 田中 司, 西山 将広, 永瀬 裕朗, 飯島 一誠

  (一社)日本小児神経学会, May 2019, 脳と発達, 51(Suppl.) (Suppl.), S239 - S239, Japanese


• 出血性ショック脳症症候群(HSES)7症例の詳細な臨床経過の検討

  山口 宏, 徳元 翔一, 西山 将広, 豊嶋 大作, 永瀬 裕朗, 丸山 あずさ, 飯島 一誠

  (一社)日本小児神経学会, May 2019, 脳と発達, 51(Suppl.) (Suppl.), S267 - S267, Japanese


• 時間単位で評価した急性脳症のサイトカイン動態解析(第1報)

  冨岡 和美, 西山 将広, 永瀬 裕朗, 石田 悠介, 田中 司, 徳元 翔一, 山口 宏, 豊嶋 大作, 丸山 あずさ, 黒澤 寛史, 多田 弘子, 佐久間 啓, 飯島 一誠

  (一社)日本小児神経学会, May 2019, 脳と発達, 51(Suppl.) (Suppl.), S267 - S267, Japanese


• 時間単位で評価した急性脳症のサイトカイン動態解析(第2報)

  西山 将広, 冨岡 和美, 永瀬 裕朗, 石田 悠介, 田中 司, 徳元 翔一, 山口 宏, 豊嶋 大作, 丸山 あずさ, 起塚 庸, 親里 嘉展, 高見 勇一, 多田 弘子, 佐久間 啓, 飯島 一誠

  (一社)日本小児神経学会, May 2019, 脳と発達, 51(Suppl.) (Suppl.), S268 - S268, Japanese


• 1歳以降にヌシネルセン治療を開始した脊髄性筋萎縮症1型の3例

  永井 正志, 粟野 宏之, 坊 亮輔, 村尾 真理子, 石田 悠介, 冨岡 和美, 田中 司, 西山 将広, 永瀬 裕朗, 西尾 久英, 飯島 一誠

  (一社)日本小児神経学会, May 2019, 脳と発達, 51(Suppl.) (Suppl.), S288 - S288, Japanese


• 小児けいれん重積に対する脳波モニタリング下ミダゾラム昏睡療法の有効性と安全性

  石田 悠介, 冨岡 和美, 田中 司, 西山 将広, 永瀬 裕朗, 徳元 翔一, 山口 宏, 豊嶋 大作, 丸山 あずさ, 黒澤 寛史, 飯島 一誠

  (一社)日本小児神経学会, May 2019, 脳と発達, 51(Suppl.) (Suppl.), S239 - S239, Japanese


• 有熱性難治性けいれん重積の治療プロトコル変遷による短期的予後の検討

  徳元 翔一, 山口 宏, 豊嶋 大作, 丸山 あずさ, 石田 悠介, 富岡 和美, 田中 司, 西山 将広, 永瀬 裕朗, 飯島 一誠

  (一社)日本小児神経学会, May 2019, 脳と発達, 51(Suppl.) (Suppl.), S239 - S239, Japanese


• 出血性ショック脳症症候群(HSES)7症例の詳細な臨床経過の検討

  山口 宏, 徳元 翔一, 西山 将広, 豊嶋 大作, 永瀬 裕朗, 丸山 あずさ, 飯島 一誠

  (一社)日本小児神経学会, May 2019, 脳と発達, 51(Suppl.) (Suppl.), S267 - S267, Japanese


• 時間単位で評価した急性脳症のサイトカイン動態解析(第1報)

  冨岡 和美, 西山 将広, 永瀬 裕朗, 石田 悠介, 田中 司, 徳元 翔一, 山口 宏, 豊嶋 大作, 丸山 あずさ, 黒澤 寛史, 多田 弘子, 佐久間 啓, 飯島 一誠

  (一社)日本小児神経学会, May 2019, 脳と発達, 51(Suppl.) (Suppl.), S267 - S267, Japanese


• 時間単位で評価した急性脳症のサイトカイン動態解析(第2報)

  西山 将広, 冨岡 和美, 永瀬 裕朗, 石田 悠介, 田中 司, 徳元 翔一, 山口 宏, 豊嶋 大作, 丸山 あずさ, 起塚 庸, 親里 嘉展, 高見 勇一, 多田 弘子, 佐久間 啓, 飯島 一誠

  (一社)日本小児神経学会, May 2019, 脳と発達, 51(Suppl.) (Suppl.), S268 - S268, Japanese


• 1歳以降にヌシネルセン治療を開始した脊髄性筋萎縮症1型の3例

  永井 正志, 粟野 宏之, 坊 亮輔, 村尾 真理子, 石田 悠介, 冨岡 和美, 田中 司, 西山 将広, 永瀬 裕朗, 西尾 久英, 飯島 一誠

  (一社)日本小児神経学会, May 2019, 脳と発達, 51(Suppl.) (Suppl.), S288 - S288, Japanese


• 酵素活性・負荷試験で異常を認めなかった糖原病IXa型の2歳男児例

  坊 亮輔, 粟野 宏之, 元生 和宏, 永井 正志, 松本 真明, 冨岡 和美, 前山 花織, 田中 司, 西山 将広, 中尻 智史, 西山 敦史, 永瀬 裕朗, 飯島 一誠

  (公社)日本小児科学会, Mar. 2019, 日本小児科学会雑誌, 123(3) (3), 615 - 615, Japanese


• 1型糖尿病患者に対するグルカゴンの処方実態

  松本 真明, 粟野 宏之, 廣田 勇士, 永井 正志, 坊 亮輔, 冨岡 和美, 前山 花織, 田中 司, 西山 将広, 永瀬 裕朗, 小川 渉, 飯島 一誠

  (公社)日本小児科学会, Mar. 2019, 日本小児科学会雑誌, 123(3) (3), 616 - 616, Japanese


• 急性散在性脳脊髄炎の髄液所見の検討

  西山 将広, 永井 正志, 坊 亮輔, 冨岡 和美, 前山 花織, 田中 司, 粟野 宏之, 永瀬 裕朗, 佐々木 香織, 親里 嘉展, 中川 卓, 高見 勇一, 山口 宏, 石田 悠介, 豊嶋 大作, 丸山 あずさ, 飯島 一誠

  (公社)日本小児科学会, Mar. 2019, 日本小児科学会雑誌, 123(3) (3), 620 - 620, Japanese


• 新生児マススクリーニングを契機に発見された先天性乳糖不耐症の新生児例

  坊 亮輔, 粟野 宏之, 永井 正志, 松本 真明, 冨岡 和美, 田中 司, 西山 将広, 永瀬 裕朗, 飯島 一誠

  (公社)日本小児科学会, Mar. 2019, 日本小児科学会雑誌, 123(3) (3), 623 - 623, Japanese


• バセドウ病を合併した若年ミオクロニーてんかんの1例

  三上 華奈, 永井 正志, 石田 悠介, 冨岡 和美, 村尾 真理子, 田中 司, 坊 亮輔, 西山 将広, 粟野 宏之, 永瀬 裕朗, 竹中 佳奈栄, 飯島 一誠

  (公社)日本小児科学会, Mar. 2019, 日本小児科学会雑誌, 123(3) (3), 630 - 630, Japanese


• 幼児期からヌシネルセン治療を開始した脊髄性筋萎縮症3型の2例の経過

  永井正志, 粟野宏之, 坊亮輔, 村尾真理子, 石田悠介, 冨岡和美, 田中司, 西山将広, 永瀬裕朗, 西尾久英, 飯島一誠

  (公社)日本小児科学会, Feb. 2019, 日本小児科学会雑誌, 123(2) (2), 366 - 366, Japanese


• Isolation and Characterization of Human Umbilical Cord-derived Mesenchymal Stem Cells from Preterm and Term Infants.

  Sota Iwatani, Makiko Yoshida, Keiji Yamana, Daisuke Kurokawa, Jumpei Kuroda, Khin Kyae Mon Thwin, Suguru Uemura, Satoru Takafuji, Nanako Nino, Tsubasa Koda, Masami Mizobuchi, Masahiro Nishiyama, Kazumichi Fujioka, Hiroaki Nagase, Ichiro Morioka, Kazumoto Iijima, Noriyuki Nishimura

  Mesenchymal stem cells (MSCs) have considerable therapeutic potential and attract increasing interest in the biomedical field. MSCs are originally isolated and characterized from bone marrow (BM), then acquired from tissues including adipose tissue, synovium, skin, dental pulp, and fetal appendages such as placenta, umbilical cord blood (UCB), and umbilical cord (UC). MSCs are a heterogeneous cell population with the capacity for (1) adherence to plastic in standard culture conditions, (2) surface marker expression of CD73+/CD90+/CD105+/CD45-/CD34-/CD14-/CD19-/HLA-DR- phenotypes, and (3) trilineage differentiation into adipocytes, osteocytes, and chondrocytes, as currently defined by the International Society for Cellular Therapy (ISCT). Although BM is the most widely used source of MSCs, the invasive nature of BM aspiration ethically limits its accessibility. Proliferation and differentiation capacity of MSCs obtained from BM generally decline with the age of the donor. In contrast, fetal MSCs obtained from UC have advantages such as vigorous proliferation and differentiation capacity. There is no ethical concern for UC sampling, as it is typically regarded as medical waste. Human UC starts to develop with continuing growth of the amniotic cavity at 4-8 weeks of gestation and keeps growing until reaching 50-60 cm in length, and it can be isolated during the whole newborn delivery period. To gain insight into the pathophysiology of intractable diseases, we have used UC-derived MSCs (UC-MSCs) from infants delivered at various gestational ages. In this protocol, we describe the isolation and characterization of UC-MSCs from fetuses/infants at 19-40 weeks of gestation.

  26 Jan. 2019, Journal of visualized experiments : JoVE, 143(143) (143), e58806, English, International magazine

  [Refereed]

  Introduction scientific journal


• 有熱性けいれん重積状態の小児における非けいれん性発作

  丸山あずさ, 徳元翔一, 徳元翔一, 山口宏, 山口宏, 石田悠介, 石田悠介, 田中司, 田中司, 西山将広, 豊嶋大作, 永瀬裕朗

  (一社)日本てんかん学会, 12 Sep. 2018, てんかん研究, 36(2) (2), 482 - 482, Japanese


• 小児におけるカルバマゼピンとレベチラセタムによる血清脂質値および甲状腺ホルモンの変化

  西山将広, 高見勇一, 石田悠介, 冨岡和美, 田中司, 永瀬裕朗, 中川卓, 徳元翔一, 徳元翔一, 山口宏, 山口宏, 豊嶋大作, 丸山あずさ, 飯島一誠

  (一社)日本てんかん学会, 12 Sep. 2018, てんかん研究, 36(2) (2), 464 - 464, Japanese


• 7歳時に診断した若年/成人型ガラクトシアリドーシスの臨床経過

  坊亮輔, 粟野宏之, 永井正志, 冨岡和美, 田中司, 西山将広, 永瀬裕朗, 成田綾, 飯島一誠

  日本先天代謝異常学会, Sep. 2018, 日本先天代謝異常学会雑誌, 34, 201 - 201, Japanese


• けいれんの第一選択薬はジアゼパム? それともミダゾラム?

  西山 将広, 永瀬 裕朗, 丸山 あずさ

  (一社)日本小児神経学会, May 2018, 脳と発達, 50(Suppl.) (Suppl.), S202 - S202, Japanese


• サイトカインストームによる急性脳症が予測される小児に対するステロイドパルス療法の有効性

  石田 悠介, 山口 宏, 冨岡 和美, 田中 司, 西山 将広, 藤田 杏子, 豊嶋 大作, 丸山 あずさ, 永瀬 裕朗, 黒澤 寛史, 上谷 良行, 高田 哲, 飯島 一誠

  (一社)日本小児神経学会, May 2018, 脳と発達, 50(Suppl.) (Suppl.), S325 - S325, Japanese


• 急性散在性脳脊髄炎24例における発症から軽快までの経時的な臨床経過の特徴

  西山 将広, 永瀬 裕朗, 冨岡 和美, 田中 司, 佐々木 香織, 親里 嘉展, 中川 卓, 高見 勇一, 山口 宏, 石田 悠介, 豊嶋 大作, 丸山 あずさ, 高田 哲, 飯島 一誠

  (一社)日本小児神経学会, May 2018, 脳と発達, 50(Suppl.) (Suppl.), S327 - S327, Japanese


• 小児救急外来における非けいれん性発作を示す小児患者の臨床的特徴

  山口 宏, 石田 悠介, 冨岡 和美, 田中 司, 西山 将広, 豊嶋 大作, 丸山 あずさ, 永瀬 裕朗, 高田 哲, 上谷 良行, 飯島 一誠

  (一社)日本小児神経学会, May 2018, 脳と発達, 50(Suppl.) (Suppl.), S333 - S333, Japanese


• 神戸大学における小児科と麻酔科の連携による脊髄性筋萎縮症のヌシネルセン治療

  永井 正志, 粟野 宏之, 松本 真明, 坊 亮輔, 冨岡 和美, 田中 司, 西山 将広, 前山 花織, 永瀬 裕朗, 小幡 典彦, 溝渕 知司, 西尾 久英, 飯島 一誠

  (一社)日本小児神経学会, May 2018, 脳と発達, 50(Suppl.) (Suppl.), S352 - S352, Japanese


• 複雑型熱性けいれんの急性期の臨床経過の特徴

  田中 司, 永瀬 裕朗, 冨岡 和美, 西山 将広, 山口 宏, 石田 悠介, 豊嶋 大作, 丸山 あずさ, 黒澤 寛史, 藤田 杏子, 上谷 良行, 高田 哲, 飯島 一誠

  (一社)日本小児神経学会, May 2018, 脳と発達, 50(Suppl.) (Suppl.), S431 - S431, Japanese


• 発熱に伴う難治性てんかん重積状態に対するバルビツレート昏睡療法の最適な鎮静深度に関する多施設共同研究

  冨岡 和美, 永瀬 裕朗, 田中 司, 西山 将広, 起塚 庸, 高見 勇一, 山口 宏, 石田 悠介, 豊嶋 大作, 丸山 あずさ, 黒澤 寛史, 藤田 杏子, 上谷 良行, 高田 哲, 飯島 一誠

  (一社)日本小児神経学会, May 2018, 脳と発達, 50(Suppl.) (Suppl.), S432 - S432, Japanese


• カーボカウントが1型糖尿病の血糖管理に有用であった7歳男児例

  松本真明, 粟野宏之, 坊亮輔, 冨岡和美, 前山花織, 西山将広, 永瀬裕朗, 飯島一誠

  01 Apr. 2018, 日本小児科学会雑誌, 122(4) (4), 809, Japanese


• 先天性甲状腺機能低下症(CH)のフォロー中に偽性副甲状腺機能低下症と診断された1例

  坊亮輔, 粟野宏之, 松本真明, 永井正志, 冨岡和美, 田中司, 西山将広, 前山花織, 永瀬裕朗, 飯島一誠

  01 Apr. 2018, 日本小児科学会雑誌, 122(4) (4), 816, Japanese


• 不明熱の精査中にRadiologically Isolated Syndromeが疑われた1例

  元生和宏, 冨岡和美, 松本真明, 永井正志, 坊亮輔, 前山花織, 田中司, 西山将広, 粟野宏之, 永瀬裕朗, 飯島一誠

  01 Apr. 2018, 日本小児科学会雑誌, 122(4) (4), 820, Japanese


• 乳幼児感覚プロファイルを用いた発達障害児の感覚特性に関する検討

  前山花織, 高木康子, 吉岡三惠子, 加藤威, 溝渕雅巳, 北山真次, 高田哲, 松本真明, 永井正志, 坊亮輔, 冨岡和美, 西山将広, 粟野宏之, 永瀬裕朗, 飯島一誠, 西村範行

  01 Apr. 2018, 日本小児科学会雑誌, 122(4) (4), 808‐809, Japanese


• 2歳時に早期診断が出来た脊髄性筋萎縮症3a型の女児例

  永井正志, 粟野宏之, 松本真明, 坊亮輔, 冨岡和美, 田中司, 西山将広, 前山花織, 永瀬裕朗, 西尾久英, 飯島一誠

  01 Feb. 2018, 日本小児科学会雑誌, 122(2) (2), 460, Japanese


• A case of paroxysmal kinesigenic dyskinesia diagnosed early after initial visit

  Eguchi Junji, Matumoto Kazunori, Mitsuda Yoshihiro, Yasujima Hidehiro, Tanaka ukiko, Nishiyama Masahiro

  Paroxysmal kinesigenic dyskinesia is a rare disorder, characterized by recurrent and brief attacks of involuntary movements such as choreoathetosis and dystonia, triggered by sudden movements. A 12-year-old boy presented with paroxysmal episodes,which occurred mainly on the left upper limb. His involuntary movements consisted of chorea, athetosis, and dystonia, but were not accompanied by impaired consciousness, and spontaneously disappeared within 1 minute. In the intermittent phase of the attack, we observed no physical abnormalities and altered laboratory findings. Symptoms disappeared promptly after starting carbamazepine therapy. This disease responds well to medication. Therefore, it is important to have a good understanding of this disease, to avoid misdiagnosing it as a psychogenic reaction or tic.

  Kobe City Hospital Organization, 2018, KOBE CITY HOSPITAL BULLETIN, 57, 7 - 11, Japanese


• 偶発的に発見された成長ホルモン産生下垂体腺腫の11歳男児例

  永井正志, 粟野宏之, 松本真明, 坊亮輔, 石田悠介, 冨岡和美, 田中司, 村尾真理子, 西山将広, 谷口理章, 飯島一誠

  2018, 日本小児内分泌学会学術集会プログラム・抄録集, 52nd


• 発作性運動誘発性ジスキネジアを呈した偽性副甲状腺機能低下症の一例

  坊亮輔, 粟野宏之, 西山将広, 永井正志, 冨岡和美, 田中司, 永瀬裕朗, 飯島一誠

  2018, 日本小児内分泌学会学術集会プログラム・抄録集, 52nd, 228, Japanese


• カルニチンのみで良好な経過をたどる慢性進行型メチルマロン酸血症同胞例

  坊亮輔, 粟野宏之, 永井正志, 松本真明, 富岡和美, 前山花織, 田中司, 西山将広, 永瀬裕朗, 飯島一誠

  12 Sep. 2017, 日本先天代謝異常学会雑誌, 33, 202, Japanese


• 乳幼児期における発達障害児の感覚特性についての検討 自閉症および知的障害特性との関連

  前山 花織, 高木 康子, 吉岡 三惠子, 加藤 威, 溝渕 雅巳, 北山 真次, 高田 哲, 坊 亮輔, 冨岡 和美, 西山 将広, 粟野 宏之, 永瀬 裕朗, 飯島 一誠, 西村 範行

  (一社)日本小児神経学会, May 2017, 脳と発達, 49(Suppl.) (Suppl.), S360 - S360, Japanese


• ビタミンB12反応性メチルマロン酸血症の1例

  永井正志, 粟野宏之, 松本真明, 坊亮輔, 冨岡和美, 前山花織, 西山将広, 西山敦史, 永瀬裕朗, 長谷川有紀, 飯島一誠

  01 May 2017, 日本小児科学会雑誌, 121(5) (5), 908‐909, Japanese


• 点頭てんかん再発例に対しケトン食療法とACTH反復投与を行った1例

  本郷裕斗, 永瀬裕朗, 永井正志, 松本真明, 冨岡和美, 前山花織, 西山将広, 粟野宏之, 飯島一誠

  01 May 2017, 日本小児科学会雑誌, 121(5) (5), 912, Japanese


• 間質性肺炎を合併したエンテロウイルスD68型感染症の1例

  松本真明, 粟野宏之, 冨岡和美, 運崎愛, 西山将広, 豊嶋大作, 永瀬裕朗, 森貞直哉, 飯島一誠

  01 May 2017, 日本小児科学会雑誌, 121(5) (5), 902‐903, Japanese


• 超低出生体重児のSGA性低身長症に対する成長ホルモン治療の検討

  松本真明, 粟野宏之, 永井正志, 坊亮輔, 冨岡和美, 前山花織, 西山将広, 永瀬裕朗, 森岡一朗, 飯島一誠

  (公社)日本小児科学会, 01 Feb. 2017, 日本小児科学会雑誌, 121(2) (2), 374 - 374, Japanese


• 近位筋優位の筋力低下から筋疾患が疑われたシャルコー・マリー・トゥース病2型

  永井正志, 粟野宏之, 松本真明, 坊亮輔, 冨岡和美, 前山花織, 西山将広, 港敏則, 永瀬裕朗, 飯島一誠

  01 Feb. 2017, 日本小児科学会雑誌, 121(2) (2), 380, Japanese


• Digenicな変異で発症した,致死性不整脈と心筋緻密化障害を認めた兄弟例

  長坂美和子, 長坂美和子, 池田真理子, 池田真理子, 西山将広, 粟野宏之, 永瀬裕朗, 松本真明, 冨岡和美, 森岡一朗, 倉橋浩樹, 飯島一誠

  01 Feb. 2017, 日本小児科学会雑誌, 121(2) (2), 261, Japanese


• CGMが診断・治療に有用であったダンピング症候群の乳児例

  松本真明, 粟野宏之, 福岡秀規, 永井正志, 坊亮輔, 冨岡和美, 前山花織, 田中司, 西山将広, 永瀬裕明, 飯島一誠

  2017, 日本内分泌学会雑誌, 93(2) (2)


• 幼児期からの糖・脂質代謝の経過をフォローしえたPTRF遺伝子変異による全身性脂肪萎縮症の一例

  松本真明, 粟野宏之, 廣田勇士, 永井正志, 坊亮輔, 冨岡和美, 前山花織, 西山将広, 李知子, 永瀬裕明, 八木麻理子, 竹島泰弘, 小川渉, 飯島一誠

  2017, 糖尿病(Web), 60(Suppl) (Suppl), S.379(J‐STAGE), Japanese


• ビタミンB12反応性を認めた軽症メチルマロニルCoAムターゼ欠損症の一例

  永井正志, 粟野宏之, 松本真明, 坊亮輔, 冨岡和美, 西山将広, 西山敦史, 永瀬裕朗, 長谷川有紀, 飯島一誠

  30 Sep. 2016, 日本先天代謝異常学会雑誌, 32, 191, Japanese


• AST上昇を認めない重症熱性けいれん小児における非けいれん性発作―長期予後の検討

  丸山あずさ, 石田悠介, 田中司, 西山将広, 豊嶋大作, 永瀬裕朗

  02 Sep. 2016, てんかん研究, 34(2) (2), 460, Japanese


• 間質性肺炎を発症したエンテロウイルスD68感染症の1例

  松本真明, 粟野宏之, 荻美貴, 冨岡和美, 運崎愛, 西山将広, 豊嶋大作, 池田真理子, 石田明人, 永瀬裕朗, 森岡一朗, 飯島一誠

  14 Jun. 2016, 日本小児救急医学会雑誌, 15(2) (2), 245, Japanese


• 小児のてんかん重積状態におけるセカンドライン治療の有効性と意識抑制作用の検討

  西山 将広, 永瀬 裕朗, 石田 悠介, 田中 司, 藤田 杏子, 豊嶋 大作, 丸山 あずさ, 松本 真明, 冨岡 和美, 運崎 愛, 粟野 宏之, 竹田 洋樹, 上谷 良行, 高田 哲, 飯島 一誠

  (一社)日本小児神経学会, May 2016, 脳と発達, 48(Suppl.) (Suppl.), S275 - S275, Japanese


• 急性脳症による死亡例の検討

  冨岡 和美, 永瀬 裕朗, 石田 悠介, 田中 司, 西山 将広, 藤田 杏子, 豊嶋 大作, 丸山 あずさ, 竹田 洋樹, 上谷 良行, 松本 真明, 運崎 愛, 粟野 宏之, 高田 哲, 飯島 一誠

  (一社)日本小児神経学会, May 2016, 脳と発達, 48(Suppl.) (Suppl.), S393 - S393, Japanese


• L‐カルニチン内服治療を行った3‐メチルクロトニルCoAカルボキシラーゼ欠損症の一例

  松本真明, 粟野宏之, 冨岡和美, 運崎愛, 西山将広, 豊嶋大作, 八木麻理子, 長谷川有紀, 竹島泰弘, 飯島一誠

  (公社)日本小児科学会, 01 Feb. 2016, 日本小児科学会雑誌, 120(2) (2), 480 - 480, Japanese


• タンデムマス・スクリーニングでグルタル酸血症1型が疑われた低形成腎の1例

  松本 真明, 粟野 宏之, 運崎 愛, 西山 将広, 豊嶋 大作, 忍頂寺 毅史, 森貞 直哉, 中川 卓, 上村 裕保, 八木 麻理子, 飯島 一誠

  (公社)日本小児科学会, Jan. 2016, 日本小児科学会雑誌, 120(1) (1), 85 - 85, Japanese


• 当院における自己免疫性神経疾患に対するリツキシマブの使用経験

  冨岡和美, 西山将広, 松本真明, 運崎愛, 豊嶋大作, 粟野宏之, 中川卓, 森貞直哉, 高田哲, 飯島一誠

  2016, 日本小児科学会雑誌, 120(2) (2)


• 3‐メチルクロトニル‐CoAカルボキシラーゼ欠損症の2例

  松本真明, 粟野宏之, 冨岡和美, 運崎愛, 西山将広, 加藤神奈, 豊嶋大作, 西山敦史, 八木麻理子, 竹島泰弘, 飯島一誠

  (公社)日本小児科学会, 01 Jan. 2016, 日本小児科学会雑誌, 120(1) (1), 91 - 91, Japanese


• 重篤な経過を呈した,両親由来の異なる心筋障害遺伝子の変異を受け継いだ兄弟例

  長坂美和子, 長坂美和子, 池田真理子, 池田真理子, 稲垣秀人, 大内雄矢, 西山将広, 粟野宏之, 永瀬裕朗, 森岡一朗, 戸田達史, 戸田達史, 倉橋浩樹, 飯島一誠

  2016, 日本小児遺伝学会学術集会プログラム・抄録集, 39th, 52, Japanese


• 発作時脳波により診断したinfantile spasms without hypsarrhythmiaの2例

  冨岡和美, 松本真明, 運崎愛, Nishiyama Masahiro, 加藤神奈, 豊嶋大作, 粟野宏之, 池田真理子, 森貞直哉, 高田哲, 飯島一誠

  Sep. 2015, てんかん研究, 33(2) (2), 569, Japanese

  Meeting report


• TruSight Oneシークエンスパネルで原因遺伝子を同定した神経発達疾患の3例

  森貞直哉, 豊嶋大作, 西山将広, 永瀬裕朗, 丸山あずさ, 中川卓, 和田博子, 高田哲, 西尾久英, 飯島一誠

  Jul. 2015, 日本先天異常学会学術集会プログラム・抄録集, 55th, 95, Japanese


• 幼児期に発症した多発性硬化症の4例

  Nishiyama Masahiro, 永瀬裕朗, 田中司, 丸山あずさ, 豊嶋大作, 中川卓, 森貞直哉, 親里嘉展, 佐伯啓介, 高田哲, 飯島一誠

  May 2015, 脳と発達, 47(Suppl.) (Suppl.), S226, Japanese

  Meeting report


• タンデムマスクリーニングでグルタル酸血症1型が疑われた低形成腎の1例

  松本真明, 粟野宏之, 冨岡和美, 運崎愛, 加藤神奈, 西山将広, 豊嶋大作, 八木麻理子, 長谷川有紀, 飯島一誠

  2015, 日本先天代謝異常学会雑誌, 31


• 当施設における先天性筋強直性ジストロフィー症例の周産期合併症

  松本真明, 粟野宏之, 冨岡和美, 運崎愛, 加藤神奈, 西山将広, 豊嶋大作, 黒川大輔, 長坂美和子, 香田翼, 池田真理子, 森岡一朗, 山田秀人, 飯島一誠

  2015, 日本人類遺伝学会大会プログラム・抄録集, 60th


• 編集委員会への手紙 西山将広、他「ピボキシル基含有抗菌薬3日間投与によるカルニチン欠乏が関与した急性脳症の1例」(日本小児科学会雑誌118巻5号812～818,2014年について)

  今高城治, 有阪治, Nishiyama Masahiro

  Sep. 2014, 日本小児科学会雑誌, 118(9号) (9号), 1429 - 1430, Japanese

  Report scientific journal


• 急性脳症治療を目指した一般人口研究のための予備的研究

  佐々木香織, 永瀬裕朗, 丸山あずさ, 藤田杏子, 西山将広, 田中司, 上谷良行, 貫名貞之, 宅見徹, 親里嘉展, 西山敦史, 河田知子, 佐伯啓介, 佐竹恵理子, 高見勇一

  01 Jun. 2014, 日本小児科学会雑誌, 118(6) (6), 969, Japanese


• 神経学的基礎疾患を有する症例における急性脳症での神経学的後遺症の予測

  西山将広, 田中司, 藤田杏子, 丸山あずさ, 永瀬裕朗

  01 Feb. 2014, 日本小児科学会雑誌, 118(2) (2), 232, Japanese


• Induced Hypothermia/Normothermia with General Anesthesia Is Related to Better Outcomes in Children with Acute Encephalopathy Caused by Excitotoxicity

  Masahiro Nishiyama, Kyoko Fujita, Azusa Maruyama, Hiroaki Nagase

  Feb. 2013, NEUROLOGY, 80, English

  Summary international conference


• 急性脳症における神経学的短期および長期予後の比較

  西山将広, 藤田杏子, 丸山あずさ, 永瀬裕朗, 竹田洋樹, 上谷良行

  01 Feb. 2013, 日本小児科学会雑誌, 117(2) (2), 349, Japanese


• 慢性肺疾患増悪期におけるサーファクタント(STA)洗浄の有効性

  岸田 祐介, 猪俣 慶, 坂井 仁美, 溝渕 雅巳, 芳本 誠司, 中尾 秀人, 西山 将広, 田中 聡, 岩谷 壮太, 浅野 貴大

  20 Oct. 2011, 日本肺サーファクタント・界面医学会雑誌 = Journal of Japanese Medical Society for Lung Surfactant and Biological Interface, 42, 37 - 38, Japanese


• 再発又は治療抵抗性進行神経芽腫に対する13-cis retinoic acidの意義

  山下 達也, 長谷川 大一郎, 西山 将広, 田中 愛子, 石田 敏章, 竹田 洋樹, 川崎 圭一郎, 横井 暁子, 吉田 牧子, 赤坂 好宣, 小阪 嘉之

  (NPO)日本小児がん学会, Dec. 2010, 小児がん, 47(プログラム・総会号) (プログラム・総会号), 245 - 245, Japanese


• HLA1座不一致同胞からの骨髄移植を行ったbilineal leukemiaの1例

  奥野 啓介, 竹田 洋樹, 田中 愛子, 西山 将広, 山下 達也, 石田 敏章, 長谷川 大一郎, 川崎 圭一郎, 小阪 嘉之

  (NPO)日本小児がん学会, Dec. 2010, 小児がん, 47(プログラム・総会号) (プログラム・総会号), 431 - 431, Japanese


• 一児が臍帯ヘルニアであった一絨毛膜二羊膜性双胎の2例

  植村 加奈子, 二階堂 量子, 山本 寛子, 岸田 真, 山本 暢之, 西山 将広, 北山 路子, 上田 陽子, 伴 紘文, 高見 勇一, 柄川 剛, 高橋 宏暢, 岸本 芽子, 濱平 陽史, 五百蔵 智明, 久呉 真章

  (公社)日本小児科学会, Dec. 2010, 日本小児科学会雑誌, 114(12) (12), 1939 - 1939, Japanese


• 精神症状を主訴とした14歳女児のBasedow病の1例

  二階堂 量子, 山本 寛子, 植村 加奈子, 山本 暢之, 西山 将広, 北山 路子, 岸田 真, 上田 陽子, 伴 紘文, 高見 勇一, 柄川 剛, 高橋 宏暢, 岸本 芽子, 濱平 陽史, 五百蔵 智明, 久呉 真章, 丸山 準

  (公社)日本小児科学会, Dec. 2010, 日本小児科学会雑誌, 114(12) (12), 1941 - 1941, Japanese


• 生来健康な児に発症したサイトメガロウイルス肝炎の1例

  山本 寛子, 植村 加奈子, 二階堂 量子, 岸田 真, 北山 路子, 西山 将広, 山本 暢之, 上田 陽子, 伴 紘文, 高見 勇一, 柄川 剛, 高橋 宏暢, 濱平 陽史, 五百蔵 智明, 久呉 真章

  (公社)日本小児科学会, Dec. 2010, 日本小児科学会雑誌, 114(12) (12), 1942 - 1942, Japanese


• 化学療法中に新たに骨転移が出現したstage4S乳児神経芽腫の1例

  西山 将広, 長谷川 大一郎, 山下 達也, 田中 愛子, 石田 敏章, 竹田 洋樹, 川崎 圭一郎, 横井 暁子, 吉田 牧子, 赤坂 好宣, 副島 俊典, 高橋 宏暢, 小阪 嘉之

  (NPO)日本小児がん学会, Dec. 2010, 小児がん, 47(プログラム・総会号) (プログラム・総会号), 370 - 370, Japanese


• 2回のTA-TMAに対して初回時には血漿交換、再燃時にはトロンボモジュリン製剤が奏功した小児Ph1 ALLの1例

  石田 敏章, 西山 将広, 山下 達也, 田中 愛子, 奥野 啓介, 竹田 洋樹, 長谷川 大一郎, 田中 亮二郎, 川崎 圭一郎, 小阪 嘉之

  (NPO)日本小児がん学会, Dec. 2010, 小児がん, 47(プログラム・総会号) (プログラム・総会号), 404 - 404, Japanese


• Basedow病の診断時に僧帽弁逸脱を認めた2歳女児の1例

  岸田 真, 山本 暢之, 西山 将広, 北山 路子, 李 知子, 住谷 珠子, 伴 紘文, 高見 勇一, 柄川 剛, 高橋 宏暢, 岸本 芽子, 濱平 陽史, 五百蔵 智明, 久呉 真章

  (公社)日本小児科学会, Dec. 2009, 日本小児科学会雑誌, 113(12) (12), 1894 - 1894, Japanese


• 当院における胃食道逆流症例の検討

  山本 暢之, 植村 加奈子, 二階堂 量子, 山本 寛子, 北山 路子, 岸田 真, 西山 将広, 李 知子, 伴 紘文, 高見 勇一, 柄川 剛, 高橋 宏暢, 岸本 芽子, 濱平 陽史, 五百蔵 智明, 久呉 真章, 畠山 理, 大片 祐一

  (公社)日本小児科学会, Dec. 2009, 日本小児科学会雑誌, 113(12) (12), 1896 - 1896, Japanese


• 小児疾患の身近な漢方治療 インフルエンザ・感冒性嘔吐下痢症を中心に

  岸本 芽子, 北山 路子, 岸田 真, 山本 暢之, 西山 将広, 李 知子, 伴 紘文, 住谷 珠子, 高見 勇一, 柄川 剛, 高橋 宏暢, 濱平 陽史, 五百蔵 智明, 久呉 真章

  姫路赤十字病院図書学術委員会, Jun. 2009, 姫路赤十字病院誌, 33, 136 - 136, Japanese


• 低血糖と意識障害をきっかけに診断されたプロピオン酸血症の1例

  西山 将広, 鄭 聡柄, 岸田 真, 北山 路子, 山本 暢之, 李 知子, 住谷 珠子, 伴 紘文, 高見 勇一, 柄川 剛, 高橋 宏暢, 濱平 陽史, 五百蔵 智明, 久呉 真章

  (公社)日本小児科学会, Mar. 2009, 日本小児科学会雑誌, 113(3) (3), 604 - 604, Japanese


• 新生児におけるくも膜下出血と初期嘔吐の臨床像の比較

  岸田 真, 山本 暢之, 西山 将広, 北山 路子, 李 知子, 住谷 珠子, 伴 紘文, 高見 勇一, 柄川 剛, 高橋 宏暢, 岸本 芽子, 濱平 陽史, 五百蔵 智明, 久呉 真章

  (公社)日本小児科学会, Mar. 2009, 日本小児科学会雑誌, 113(3) (3), 604 - 604, Japanese


• 発熱を主訴に当院に入院した新生児74例の検討

  北山 路子, 岸田 真, 西山 将広, 山本 暢之, 李 知子, 住谷 珠子, 伴 紘文, 高見 勇一, 柄川 剛, 高橋 宏暢, 岸本 芽子, 濱平 陽史, 五百蔵 智明, 久呉 真章

  (公社)日本小児科学会, Mar. 2009, 日本小児科学会雑誌, 113(3) (3), 605 - 605, Japanese


• 当科における腹腔鏡下鼠径ヘルニア手術(LPEC法)の導入

  畠山 理, 大片 祐一, 岸田 真, 北山 路子, 西山 将広, 山本 暢之, 李 知子, 住谷 珠子, 伴 紘文, 高見 勇一, 柄川 剛, 高橋 宏暢, 岸本 芽子, 濱平 陽史, 五百蔵 智明, 久呉 真章

  (公社)日本小児科学会, Mar. 2009, 日本小児科学会雑誌, 113(3) (3), 605 - 606, Japanese


• 当院における髄膜炎起炎菌と抗生剤感受性に関する検討

  山本 暢之, 岸田 真, 北山 路子, 西山 将広, 李 知子, 住谷 珠子, 伴 紘文, 高見 勇一, 柄川 剛, 高橋 宏暢, 岸本 芽子, 濱平 陽史, 五百蔵 智明, 久呉 真章

  (公社)日本小児科学会, Mar. 2009, 日本小児科学会雑誌, 113(3) (3), 608 - 608, Japanese


• 抗SS-B抗体陽性であったエルシニア感染症の1例

  西山 将広, 岸田 真, 北山 路子, 山本 暢之, 李 知子, 住谷 珠子, 伴 紘文, 高見 勇一, 柄川 剛, 高橋 宏暢, 岸本 芽子, 濱平 陽史, 五百蔵 智明, 久呉 真章

  (公社)日本小児科学会, Mar. 2009, 日本小児科学会雑誌, 113(3) (3), 608 - 608, Japanese


• 当院での川崎病におけるIVIG療法不応症例の検討

  山本 暢之, 岸田 真, 北山 路子, 西山 将広, 李 知子, 住谷 珠子, 伴 紘文, 高見 勇一, 鄭 聡柄, 柄川 剛, 高橋 宏暢, 濱平 陽史, 五百蔵 智明, 久呉 真章

  日本赤十字社医学会, Sep. 2008, 日赤医学, 60(1) (1), 241 - 241, Japanese


• シクロフォスファミドが有効であった小児難治性ネフローゼ症候群の2例

  浜平 陽史, 岸田 真, 北山 路子, 西山 将広, 山本 暢之, 伴 紘文, 住谷 珠子, 高見 勇一, 柄川 剛, 鄭 聡柄, 高橋 宏暢, 五百蔵 智明, 久呉 真章, 吉川 徳茂

  日本赤十字社医学会, Sep. 2008, 日赤医学, 60(1) (1), 242 - 242, Japanese

• 小児てんかん重積状態 初期対応と原因検索実践ガイド

  西山 将広

  東京 : 日本医事新報社, Aug. 2026, Japanese


• 特集 小児神経学入門─小児科医必須の神経症候学と治療論 治療 てんかん重積状態の治療

  西山 将広

  東京医学社, Apr. 2026

  DOI


• 特集 小児診療ガイドラインのダイジェスト解説 & プログレス2025 神経 11 てんかん重積状態･けいれん重積状態

  菊池 健二郎, 西山 将広

  金原出版, Dec. 2025

  DOI


• 特集 これからのガイドラインの読み方,使い方 Ⅰ.ガイドラインのつくり エビデンスレベルが表すこと

  西山 将広

  診断と治療社, Apr. 2025

  DOI


• 特集 外来診療･診断のピットフォール 各論:代表的な疾患のピットフォール 中枢神経系 川崎病だと思ったら細菌性髄膜炎

  西山 将広

  東京医学社, Apr. 2025

  DOI


• 【エキスパートが教える 小児の薬物治療】疾患別 E.神経筋疾患 急性脳症

  西山 将広, 永瀬 裕朗

  (株)東京医学社, Nov. 2023, Japanese


• 特集 小児の集中治療の実践-躬行実践- 各論 重症疾患の管理 神経疾患とその管理-急性脳症を中心に

  西山 将広

  東京医学社, Aug. 2023

  DOI


• エキスパートが教える 小児の薬物療法 神経筋疾患 急性脳症

  西山将広, 永瀬裕朗

  東京医学社, 2023


• 日常診療に活かす 診療ガイドライン UP-TO-DATE 2024-2025 小児けいれん重積

  西山将広, 永瀬裕朗

  メディカルレビュー社, 2023


• 小児の集中治療の実践-躬行実践-重症疾患の管理 神経疾患とその管理-急性脳症を中心に

  西山将広

  中山書店, 2023


• 小児科ベストプラクティス「小児急性脳症」 熱性けいれん重積と急性脳症の鑑別

  西山将広

  中山書店, 2022


• 最新ガイドライン準拠 小児科診断・治療指針 改訂第2版 / けいれん重積、意識障害

  Nishiyama Masahiro, 永瀬裕朗

  Others, 中山書店, 2017, Japanese

  Textbook

• 我が国における出血性ショック脳症症候群(HSES)の病態と治療

  西山 将広, 徳元 翔一, 山口 宏, 冨岡 和美, 石田 悠介, 丸山 あずさ, 永瀬 裕朗

  脳と発達, May 2023, Japanese, (一社)日本小児神経学会

  Nominated symposium


• 出血性ショック脳症症候群をきたしたDravet症候群にSCN1Aのイントロンに新規の21塩基欠失を認めminigene解析を実施した1例

  花房 宏昭, 山口 宏, 老川 静香, 徳元 翔一, 冨岡 和美, 西山 将広, 長坂 美和子, 近藤 秀仁, 森貞 直哉, 松尾 雅文, 野津 寛大, 永瀬 裕朗

  脳と発達, May 2023, Japanese, (一社)日本小児神経学会


• 当院での脳症関連遺伝子パネルを用いた疾患関連遺伝子の同定の試み

  山口 宏, 花房 宏昭, 徳元 翔一, 冨岡 和美, 西山 将広, 森貞 直哉, 野津 寛大, 永瀬 裕朗

  日本小児科学会雑誌, Apr. 2023, Japanese, (公社)日本小児科学会


• COVID-19感染症を契機に急性増悪した全身型重症筋無力症の女児例

  金谷 真吾, 岩本 宗矩, 山口 宏, 南部 静紀, 徳元 翔一, 坊 亮輔, 冨岡 和美, 西山 将広, 粟野 宏之, 野津 寛大, 永瀬 裕朗

  日本小児科学会雑誌, Apr. 2023, Japanese, (公社)日本小児科学会


• てんかん重積状態・急性脳症における疾患原因遺伝子の同定の試み

  山口 宏, 花房 宏昭, 徳元 翔一, 冨岡 和美, 西山 将広, 森貞 直哉, 野津 寛大, 永瀬 裕朗

  てんかん研究, Aug. 2022, Japanese, (一社)日本てんかん学会


• 発熱を伴う30分以上のけいれん性てんかん重積状態における転帰不良の予測

  西山 将広, 丸山 あずさ, 高梨 潤一, 本林 光雄, 服部 有香, 豊嶋 大作, 高見 勇一, 徳元 翔一, 永瀬 裕朗

  てんかん研究, Aug. 2022, Japanese, (一社)日本てんかん学会


• 3歳児の神経発達予後と周産期における母体のメンタルヘルスとの関連

  京野 由紀, 西山 将広, 徳元 翔一, 山口 宏, 冨岡 和美, 三品 浩基, 野津 寛大, 永瀬 裕朗

  小児保健研究, May 2022, Japanese, (公社)日本小児保健協会


• 小児てんかん重積に対するミダゾラム頬粘膜投与製剤の有効性と安全性 単一施設29機会の経験から

  上田 拓耶, 本郷 裕斗, 石田 悠介, 西山 将広, 豊嶋 大作, 丸山 あずさ, 田中 亮二郎, 永瀬 裕朗

  脳と発達, May 2022, Japanese, (一社)日本小児神経学会


• ホスフェニトインによる有熱性けいれん重積後の発作再発予防の有効性

  本郷 裕斗, 上田 拓耶, 石田 悠介, 西山 将広, 豊嶋 大作, 丸山 あずさ, 田中 亮二郎, 永瀬 裕朗

  脳と発達, May 2022, Japanese, (一社)日本小児神経学会


• 熱性けいれんにおける発症後早期のサイトカイン動態

  徳元 翔一, 西山 将広, 上田 拓耶, 本郷 裕斗, 山口 宏, 石田 悠介, 冨岡 和美, 豊嶋 大作, 丸山 あずさ, 野津 寛大, 石田 明人, 永瀬 裕朗

  脳と発達, May 2022, Japanese, (一社)日本小児神経学会


• 有熱性てんかん重積予後予測バイオマーカーとしてのgrowth and differentiation factor-15

  山口 宏, 西山 将広, 上田 拓耶, 本郷 裕斗, 徳元 翔一, 石田 悠介, 冨岡 和美, 豊嶋 大作, 中川 卓, 高見 勇一, 黒澤 寛史, 丸山 あずさ, 野津 寛大, 永瀬 裕朗

  脳と発達, May 2022, Japanese, (一社)日本小児神経学会


• 前向き多施設レジストリより算出した6時間以上の意識障害遷延例における急性脳症と転帰不良の発生頻度

  西山 将広, 高梨 潤一, 本林 光雄, 服部 有香, 丸山 あずさ, 親里 嘉展, 高見 勇一, 永瀬 裕朗

  脳と発達, May 2022, Japanese, (一社)日本小児神経学会


• 神戸市東部療育センター診療所における発達障害に合併した摂食障害のまとめ

  冨岡 和美, 永瀬 裕朗, 徳元 翔一, 山口 宏, 西山 将広, 岡田 由香, 野津 寛大

  脳と発達, May 2022, Japanese, (一社)日本小児神経学会


• オナセムノゲンアベパルボベク治療後に、ヌシネルセン治療を追加した脊髄性筋萎縮症1型の1例

  南部 静紀, 坊 亮輔, 徳元 翔一, 山口 宏, 冨岡 和美, 西山 将広, 永瀬 裕朗, 西尾 久英, 野津 寛大, 粟野 宏之

  脳と発達, May 2022, Japanese, (一社)日本小児神経学会


• 小児科医の意識調査 大事にする価値観は?兵庫県で次に取り組むべき課題は?

  西山 将広, 山本 暢之, 堀之内 智子, 藤岡 一路, 永瀬 裕朗, 野津 寛大

  日本小児科学会雑誌, Mar. 2022, Japanese, (公社)日本小児科学会


• 初回発症時にMASを合併した若年性特発性若年性関節炎の1例

  吉本 啓修, 洪 聖媛, 古林 真佐美, 南部 静紀, 徳元 翔一, 山口 宏, 坊 亮輔, 冨岡 和美, 西山 将広, 粟野 宏之, 永瀬 裕朗, 飯島 一誠

  日本小児科学会雑誌, Mar. 2022, Japanese, (公社)日本小児科学会


• 過去5年間の小児摂食障害に対する家族療法の経験

  冨岡 和美, 永瀬 裕朗, 洪 聖媛, 南部 静紀, 徳元 翔一, 山口 宏, 坊 亮輔, 西山 将広, 粟野 宏之

  日本小児科学会雑誌, Mar. 2022, Japanese, (公社)日本小児科学会


• 幼児期の生活習慣と小学1年生の学力との関連 3歳時点での就寝時刻が遅いと学力が低下する

  西山 将広, 徳元 翔一, 山口 宏, 冨岡 和美, 野津 寛大, 永瀬 裕朗

  日本小児科学会雑誌, Feb. 2022, Japanese, (公社)日本小児科学会


• 熱性けいれんと発熱患者におけるサイトカインの比較

  徳元 翔一, 山口 宏, 冨岡 和美, 西山 将広, 飯島 一誠, 永瀬 裕朗

  脳と発達, May 2021, Japanese, (一社)日本小児神経学会


• 有熱性けいれん重積症例における意識障害遷延時間毎のAESD発症リスク

  石田 悠介, 西山 将広, 本郷 裕斗, 徳元 翔一, 山口 宏, 冨岡 和美, 豊嶋 大作, 丸山 あずさ, 永瀬 裕朗

  脳と発達, May 2021, Japanese, (一社)日本小児神経学会


• 急性脳症・有熱性けいれん重積の前向き多施設レジストリより算出した有熱性けいれん重積に占めるAESD発症頻度

  西山 将広, 本林 光雄, 福山 哲広, 服部 有香, 丸山 あずさ, 親里 嘉展, 高見 勇一, 永瀬 裕朗

  脳と発達, May 2021, Japanese, (一社)日本小児神経学会


• Growth and differentiation factor 15 出血性ショック脳症症候群と他の有熱性けいれん性疾患の早期鑑別マーカーとしての検討

  山口 宏, 西山 将広, 本郷 裕斗, 徳元 翔一, 石田 悠介, 冨岡 和美, 中川 卓, 高見 勇一, 豊嶋 大作, 丸山 あずさ, 飯島 一誠, 永瀬 裕朗

  脳と発達, May 2021, Japanese, (一社)日本小児神経学会


• 3歳児健診における発達通過状況とけいれん既往との関連

  西山 将広, 京野 由紀, 徳元 翔一, 山口 宏, 冨岡 和美, 藤岡 一路, 三品 浩基, 飯島 一誠, 永瀬 裕朗

  日本小児科学会雑誌, Feb. 2021, Japanese, (公社)日本小児科学会


• 小児けいれん重積治療ガイドライン改訂に向けて 難治性てんかん重積の治療 ミダゾラムまたはバルビツレートによる麻酔療法

  西山 将広, 永瀬 裕朗, 丸山 あずさ

  脳と発達, Aug. 2020, Japanese, (一社)日本小児神経学会

  Nominated symposium


• 有熱性けいれんを主訴に入院した小児におけるけいれん時間毎のAESD発症頻度と予後 単一施設の連続症例コホートでの検討

  徳元 翔一, 豊嶋 大作, 丸山 あずさ, 山口 宏, 石田 悠介, 富岡 和美, 西山 将広, 飯島 一誠, 永瀬 裕朗

  脳と発達, Aug. 2020, Japanese, (一社)日本小児神経学会


• 急性脳症の予測基準の有用性の検証(第2報) 後遺症またはAESD予測のために開発された基準の比較

  西山 将広, 石田 悠介, 徳元 翔一, 冨岡 和美, 山口 宏, 豊嶋 大作, 丸山 あずさ, 飯島 一誠, 永瀬 裕朗

  脳と発達, Aug. 2020, Japanese, (一社)日本小児神経学会


• 急性脳症の予測基準の有用性の検証(第1報) AESD予測のために開発された基準の別コホートにおける再検証

  石田 悠介, 西山 将広, 徳元 翔一, 山口 宏, 冨岡 和美, 豊嶋 大作, 丸山 あずさ, 飯島 一誠, 永瀬 裕朗

  脳と発達, Aug. 2020, Japanese, (一社)日本小児神経学会


• けいれん重積型(二相性)急性脳症の初回けいれん時間による臨床像の比較検討

  山口 宏, 西山 将広, 徳元 翔一, 石田 悠介, 冨岡 和美, 豊嶋 大作, 丸山 あずさ, 飯島 一誠, 永瀬 裕朗

  脳と発達, Aug. 2020, Japanese, (一社)日本小児神経学会


• 有熱時けいれん/意識障害の発症6時間以内のサイトカインによる予後予測 血清IL-1RA,IL-10の可能性

  冨岡 和美, 西山 将広, 山口 宏, 石田 悠介, 徳元 翔一, 豊嶋 大作, 丸山 あずさ, 服部 有香, 親里 嘉展, 中川 卓, 高見 勇一, 多田 弘子, 佐久間 啓, 飯島 一誠, 永瀬 裕朗

  脳と発達, Aug. 2020, Japanese, (一社)日本小児神経学会


• 発熱に伴うけいれん・意識障害小児患者の長期予後

  豊嶋 大作, 徳元 翔一, 山口 宏, 石田 悠介, 冨岡 和美, 西山 将広, 永瀬 裕朗, 丸山 あずさ

  脳と発達, Aug. 2020, Japanese, (一社)日本小児神経学会


• 神経筋疾患患者に対する排痰補助装置のオシレーション機能が及ぼす気道分泌物除去効果に関する検討

  粟野 宏之, 永井 正志, 坊 亮輔, 石田 悠介, 冨岡 和美, 西山 将広, 竹田 洋樹, 永瀬 裕朗, 飯島 一誠

  脳と発達, Aug. 2020, Japanese, (一社)日本小児神経学会


• ７歳時に診断した若年/成人型ガラクトシアリドーシスの臨床経過

  BOU RYOUSUKE, AWANO HIROYUKI, NAGAI MASASHI, TOMIOKA KAZUMI, TANAKA TSUKASA, NISHIYAMA MASAHIRO, NAGASE HIROAKI, 成田 綾, IIJIMA KAZUMOTO

  第60回日本先天代謝異常学会総会, Nov. 2018, Japanese, 岐阜市, Domestic conference

  Oral presentation


• A群色素性乾皮症における神経伝導検査所見の経時的変化

  辻 佑木生, 上田 健博, 関口 兼司, 西山 将広, 苅田 典生, 錦織 千佳子

  臨床神経生理学, Oct. 2018, Japanese, (一社)日本臨床神経生理学会


• 有熱性けいれん重積状態の小児における非けいれん性発作

  丸山 あずさ, 徳元 翔一, 山口 宏, ISHIDA YUSUKE, TANAKA TSUKASA, NISHIYAMA MASAHIRO, 豊嶋 大作, NAGASE HIROAKI

  第52回日本てんかん学会学術集会, Oct. 2018, Japanese, 横浜市, Domestic conference

  Oral presentation


• 発作性運動誘発性ジスキネジアを呈した偽性副甲状腺機能低下症の一例

  BOU RYOUSUKE, AWANO HIROYUKI, NISHIYAMA MASAHIRO, NAGAI MASASHI, TOMIOKA KAZUMI, TANAKA TSUKASA, NAGASE HIROAKI, IIJIMA KAZUMOTO

  第52回日本小児内分泌学会学術集会, Oct. 2018, Japanese, 東京都, Domestic conference

  Oral presentation


• 小児におけるカルバマゼピンとレベチラセタムによる血清脂質および甲状腺ホルモンの変化

  NISHIYAMA MASAHIRO, 高見 勇一, ISHIDA YUSUKE, TOMIOKA KAZUMI, TANAKA TSUKASA, NAGASE HIROAKI, 中川 卓, 徳元 翔一, 山口 宏, 豊嶋 大作, 丸山 あずさ, IIJIMA KAZUMOTO

  第52回日本てんかん学会学術集会, Oct. 2018, Japanese, 横浜市, Domestic conference

  Oral presentation


• 急性硬膜下血腫を発症し、脳動静脈瘻が指摘されたMELASの1例

  ISHIDA YUSUKE, NAGAI MASASHI, BOU RYOUSUKE, TOMIOKA KAZUMI, 村尾 真理子, TANAKA TSUKASA, NISHIYAMA MASAHIRO, AWANO HIROYUKI, NAGASE HIROAKI, IIJIMA KAZUMOTO, 井村 隼, 今堀 太一郎, KOHMURA EIJI

  第64回日本小児神経学会近畿地方会, Oct. 2018, Japanese, 神戸市, Domestic conference

  Oral presentation


• バセドウ病を合併した若年ミオクロニーてんかんの13歳女児

  NAGAI MASASHI, BOU RYOUSUKE, ISHIDA YUSUKE, TOMIOKA KAZUMI, TANAKA TSUKASA, NISHIYAMA MASAHIRO, 村尾 真理子, NAGASE HIROAKI, AWANO HIROYUKI, IIJIMA KAZUMOTO

  第64回日本小児神経学会近畿地方会, Oct. 2018, Japanese, 神戸市, Domestic conference

  Oral presentation


• バセドウ病を合併した若年ミオクロニーてんかんの一例

  三上 華奈, NAGAI MASASHI, ISHIDA YUSUKE, TOMIOKA KAZUMI, 村尾 真理子, TANAKA TSUKASA, BOU RYOUSUKE, NISHIYAMA MASAHIRO, AWANO HIROYUKI, NAGASE HIROAKI, 竹中 佳奈栄, IIJIMA KAZUMOTO

  第275回日本小児科学会近畿地方会, Sep. 2018, Japanese, 姫路市, Domestic conference

  Oral presentation


• 複雑型熱性けいれんの急性期の臨床経過の特徴

  TANAKA TSUKASA, NAGASE HIROAKI, TOMIOKA KAZUMI, NISHIYAMA MASAHIRO, 山口 宏, 石田 悠介, 豊嶋 大作, 丸山 あずさ, 黒澤 寛史, 藤田 杏子, 上谷 良行, 高田 哲, IIJIMA KAZUMOTO

  第60回日本小児神経学会学術集会, Jun. 2018, Japanese, 千葉市, Domestic conference

  Oral presentation


• 発熱に伴う難治性けいれん重積状態に対するバルビツレート昏睡療法の最適な鎮静深度に関する多施設共同研究

  TOMIOKA KAZUMI, NAGASE HIROAKI, TANAKA TSUKASA, NISHIYAMA MASAHIRO, 起塚 庸, 高見 勇一, 山口 宏, 石田 悠介, 豊嶋 大作, 丸山 あずさ, 黒澤 寛史, 藤田 杏子, 上谷 良行, 高田 哲, IIJIMA KAZUMOTO

  第60回日本小児神経学会学術集会, Jun. 2018, Japanese, 千葉市, Domestic conference

  Oral presentation


• 神戸大学における小児科と麻酔科の連携による脊髄性筋萎縮症のヌシネルセン治療

  NAGAI MASASHI, AWANO HIROYUKI, 松本 真明, BOU RYOUSUKE, TOMIOKA KAZUMI, TANAKA TSUKASA, NISHIYAMA MASAHIRO, MAEYAMA KAORI, NAGASE HIROAKI, 小幡 典彦, MIZOBUCHI SATOSHI, 西尾 久英, IIJIMA KAZUMOTO

  第60回日本小児神経学会学術集会, Jun. 2018, Japanese, 千葉市, Domestic conference

  Oral presentation


• 小児救急外来における非けいれん性発作を示す小児患者の臨床的特徴

  山口 宏, 石田 悠介, TOMIOKA KAZUMI, TANAKA TSUKASA, NISHIYAMA MASAHIRO, 豊嶋 大作, 丸山 あずさ, NAGASE HIROAKI, 高田 哲, 上谷 良行, IIJIMA KAZUMOTO

  第60回日本小児神経学会学術集会, Jun. 2018, Japanese, 千葉市, Domestic conference

  Oral presentation


• 急性散在性脳脊髄炎24例における発症から軽快までの経時的な臨床経過の特徴

  NISHIYAMA MASAHIRO, NAGASE HIROAKI, TOMIOKA KAZUMI, TANAKA TSUKASA, 佐々木 香織, 親里 嘉展, 中川 卓, 高見 勇一, 山口 宏, 石田 悠介, 豊嶋 大作, 丸山 あずさ, 高田 哲, IIJIMA KAZUMOTO

  第60回日本小児神経学会学術集会, Jun. 2018, Japanese, 千葉市, Domestic conference

  Oral presentation


• サイトカインストームによる急性脳症が予測される小児に対するステロイドパルス療法の有効性

  石田 悠介, 山口 宏, TOMIOKA KAZUMI, TANAKA TSUKASA, NISHIYAMA MASAHIRO, 藤田 杏子, 豊嶋 大作, 丸山 あずさ, NAGASE HIROAKI, 黒澤 寛史, 上谷 良行, 高田 哲, IIJIMA KAZUMOTO

  第60回日本小児神経学会学術集会, Jun. 2018, Japanese, 千葉市, Domestic conference

  Oral presentation


• けいれんの第一選択薬はジアゼパム?それともミダゾラム?

  NISHIYAMA MASAHIRO, NAGASE HIROAKI, 丸山 あずさ

  第60回日本小児神経学会学術集会, Jun. 2018, Japanese, 千葉市, Domestic conference

  [Invited]

  Nominated symposium


• When does SMN shortage become evident in spinal muscular atrophy patients?

  Noriyuki Nishimura, Sota Iwatani, Keiji Yamana, Masashi Nagai, Ryousuke Bou, Hiroyuki Awano, Kaori Maeyama, Masahiro Nishiyama, Hiroaki Nagase, Masami Mizobuchi, Kazumichi Fujioka, Ichiro Morioka, Kazumoto Iijima, Hisahide Nishio

  第60回日本小児神経学会学術集会, Jun. 2018, English, 千葉市, Domestic conference

  Oral presentation


• Is congenital cytomegalovirus infection associated with autism spectrum disorder?

  Noriyuki Nishimura, Kaori Maeyama, Kazumi Tomioka, Hiroaki Nagase, Mieko Yoshioka, Yasuko Takagi, Takeshi Kato, Masami Mizobuchi, Shinji Kitayama, Satoshi Takada, Masashi Nagai, Nana Sakakibara, Masahiro Nishiyama, Ichiro Morioka, Kazumoto Iijima

  第60回日本小児神経学会学術集会, Jun. 2018, English, 千葉市, Domestic conference

  Oral presentation


• 新生児マススクリーニングを契機に発見された先天性乳糖不耐症の新生児例

  BOU RYOUSUKE, AWANO HIROYUKI, NAGAI MASASHI, 松本 真明, TOMIOKA KAZUMI, TANAKA TSUKASA, NISHIYAMA MASAHIRO, NAGASE HIROAKI, IIJIMA KAZUMOTO

  第274回日本小児科学会兵庫県地方会, May 2018, Japanese, 神戸市, Domestic conference

  Oral presentation


• 急性散在性脳脊髄炎の髄液所見の検討

  NISHIYAMA MASAHIRO, NAGAI MASASHI, BOU RYOUSUKE, TOMIOKA KAZUMI, MAEYAMA KAORI, TANAKA TSUKASA, AWANO HIROYUKI, NAGASE HIROAKI, 佐々木 香織, 親里 嘉展, 中川 卓, 高見 勇一, 山口 宏, 石田 悠介, 豊嶋 大作, 丸山 あずさ, IIJIMA KAZUMOTO

  第274回日本小児科学会兵庫県地方会, May 2018, Japanese, 神戸市, Domestic conference

  Oral presentation


• 複雑型熱性けいれんの好発時間帯とその重症度

  山口 宏, NAGASE HIROAKI, 石田 悠介, 豊嶋 大作, 丸山 あずさ, TOMIOKA KAZUMI, TANAKA TSUKASA, NISHIYAMA MASAHIRO, 藤田 杏子, 黒澤 寛史, 高田 哲, 上谷 良行, IIJIMA KAZUMOTO

  第121回日本小児科学会学術集会, Apr. 2018, Japanese, 福岡市, Domestic conference

  Oral presentation


• 2歳時に早期診断が出来た脊髄性筋萎縮症3a型の女児例

  NAGAI MASASHI, AWANO HIROYUKI, 松本 真明, BOU RYOUSUKE, TOMIOKA KAZUMI, TANAKA TSUKASA, NISHIYAMA MASAHIRO, MAEYAMA KAORI, NAGASE HIROAKI, 西尾 久英, IIJIMA KAZUMOTO

  第121回日本小児科学会学術集会, Apr. 2018, Japanese, 福岡市, Domestic conference

  Poster presentation


• 複雑型熱性けいれんの好発時間帯とその重症度(Diurnal Occurrence of Complex Febrile Seizure and its Severity in Children)

  山口 宏, 永瀬 裕朗, 石田 悠介, 豊嶋 大作, 丸山 あずさ, 富岡 和美, 田中 司, 西山 将広, 藤田 杏子, 黒澤 寛史, 高田 哲, 上谷 良行, 飯島 一誠

  日本小児科学会雑誌, Feb. 2018, English, (公社)日本小児科学会


• CGMが診断・治療に有用であったダンピング症候群の乳児例

  松本 真明, Hiroyuki Awano, Fukuoka Hidenori, Fujita Kaori, Nagase Hiroaki, Nishiyama Masahiro, Iijima Kazumoto

  第27回臨床内分泌代謝Update, Nov. 2017, Japanese, 日本内分泌学会, 神戸, Domestic conference

  Poster presentation


• 症候性PKDを呈した偽性副甲状腺機能低下症の8歳女児例

  Hiroyuki Awano, Nishiyama Masahiro, 松本真明, Fujita Kaori, Nagase Hiroaki, Takada Satoshi, Iijima Kazumoto

  日本小児神経学会近畿地方会, Oct. 2017, Japanese, 日本小児神経学会, 大阪, Domestic conference

  Oral presentation


• カルニチンのみで良好な経過をたどる慢性進行型メチルマロン酸血症同胞例

  Hiroyuki Awano, 松本真明, Fujita Kaori, Nishiyama Masahiro, Nagase Hiroaki, Iijima Kazumoto

  第59回日本先天代謝異常学会, Oct. 2017, Japanese, 日本先天代謝異常学会, 埼玉, Domestic conference

  Poster presentation


• 不明熱の精査中にRadiologically Isolated Syndromeが疑われた1例

  元生和宏, 松本真明, Fujita Kaori, Nishiyama Masahiro, Hiroyuki Awano, Nagase Hiroaki, Iijima Kazumoto

  第272回日本小児科学会兵庫県地方会, Sep. 2017, Japanese, 日本小児科学会, 姫路, Domestic conference

  Oral presentation


• 新生児期に高TSH血症を示した偽性副甲状腺機能低下症の乳児期BMI

  Hiroyuki Awano, 松本真明, Nishiyama Masahiro, Fujita Kaori, Nagase Hiroaki, Iijima Kazumoto

  第51回日本小児内分泌学会, Sep. 2017, Japanese, 日本小児内分泌学会, 大阪, Domestic conference

  Poster presentation


• Brain atrophy and clinical severity in patients with xeroderma pigmentosum group A

  Ueda Takehiro, Fumio Kanda, Nishiyama Masahiro, Nishigori Chikako, Toda Tatsushi

  XXIII World Congress of Neurology (WCN2017), Sep. 2017, English, World Federation of Neurology, Kyoto, Japan, International conference

  Poster presentation


• 有熱性けいれん重積症例におけるAESDおよび急性脳症鑑別のためのAESD prediction scoreの有用性の検証

  Nishiyama Masahiro, Nagase Hiroaki, 石田悠介, 藤田杏子, 豊嶋大作, 丸山あずさ, 黒澤寛史, 竹田洋樹, 上谷良行, Takada Satoshi, Iijima Kazumoto

  第59回日本小児神経学会学術集会, Jun. 2017, Japanese, 日本小児神経学会, 大阪, Domestic conference

  Oral presentation


• 乳幼児期における発達障害児の感覚特性についての検討～自閉症および知的障害特性との関連～

  Fujita Kaori, 高木康子, 吉岡三惠子, 加藤威, 溝渕雅巳, 北山真次, Takada Satoshi, Nishiyama Masahiro, Hiroyuki Awano, Nagase Hiroaki, Iijima Kazumoto, Nishimura Noriyuki

  第59回日本小児神経学会学術集会, Jun. 2017, Japanese, 日本小児神経学会, 大阪, Domestic conference

  Oral presentation


• 神戸こども初期急病センターにおける水痘・おたふく風邪での受診状況－水痘ワクチン定期接種後の変化も踏まえて－

  石橋和人, 森本麻紀, 福田徹哉, 尾崎美恵, 安井美佳, 児玉真美, 片山以登, 木村誠, 石田明人, 神吉直宙, Minamikawa Shogo, Yamamura Tomohiko, Nishiyama Masahiro, Ikeda Mariko, Morioka Ichiro

  第31回日本小児救急医学会, Jun. 2017, Japanese, 日本小児救急医学会, 東京, Domestic conference

  Oral presentation


• 小児難治てんかん重積状態に対するチアミラールを用いた全身麻酔療法

  石田悠介, Nishiyama Masahiro, 藤田杏子, 豊嶋大作, 丸山あずさ, Nagase Hiroaki, 黒澤寛史, 竹田洋樹, 上谷良行, Iijima Kazumoto

  第59回日本小児神経学会学術集会, Jun. 2017, Japanese, 日本小児神経学会, 大阪, Domestic conference

  Oral presentation


• 三次救急医療施設における脳炎脳症の治療戦略-治療内容と治療開始時期の選択-

  Nishiyama Masahiro, Nagase Hiroaki, 丸山あずさ

  第59回日本小児神経学会学術集会, Jun. 2017, Japanese, 日本小児神経学会, 大阪, Domestic conference

  [Invited]

  Nominated symposium


• Targeted temperature managementを導入した急性脳症疑い症例における早期予後因子の検討

  石田悠介, Nishiyama Masahiro, 藤田杏子, 豊嶋大作, 丸山あずさ, Nagase Hiroaki, 黒澤寛史, 竹田洋樹, 上谷良行, Iijima Kazumoto

  第59回日本小児神経学会学術集会, Jun. 2017, Japanese, 日本小児神経学会, 大阪, Domestic conference

  Oral presentation


• 乳幼児期における発達障害児の感覚特性についての検討 自閉症および知的障害特性との関連

  前山 花織, 高木 康子, 吉岡 三惠子, 加藤 威, 溝渕 雅巳, 北山 真次, 高田 哲, 坊 亮輔, 冨岡 和美, 西山 将広, 粟野 宏之, 永瀬 裕朗, 飯島 一誠, 西村 範行

  脳と発達, May 2017, Japanese, (一社)日本小児神経学会


• 有熱性けいれん重積症例におけるAESDおよび急性脳症鑑別のためのAESD prediction scoreの有用性の検証

  西山 将広, 永瀬 裕朗, 石田 悠介, 田中 司, 藤田 杏子, 豊嶋 大作, 丸山 あずさ, 冨岡 和美, 黒澤 寛史, 竹田 洋樹, 上谷 良行, 高田 哲, 飯島 一誠

  脳と発達, May 2017, Japanese, (一社)日本小児神経学会


• Targeted temperature managementを導入した急性脳症疑い症例における早期予後因子の検討

  田中 司, 石田 悠介, 冨岡 和美, 西山 将広, 藤田 杏子, 豊嶋 大作, 丸山 あずさ, 永瀬 裕朗, 黒澤 寛史, 竹田 洋樹, 上谷 良行, 飯島 一誠

  脳と発達, May 2017, Japanese, (一社)日本小児神経学会


• 発症6時間以内に得られる急性脳症死亡予測因子の検討

  冨岡 和美, 永瀬 裕朗, 石田 悠介, 田中 司, 西山 将広, 藤田 杏子, 豊嶋 大作, 丸山 あずさ, 黒澤 寛史, 竹田 洋樹, 上谷 良行, 高田 哲, 飯島 一誠

  脳と発達, May 2017, Japanese, (一社)日本小児神経学会


• 小児難治てんかん重積状態に対するチアミラールを用いた全身麻酔療法

  石田 悠介, 冨岡 和美, 田中 司, 西山 将広, 藤田 杏子, 豊嶋 大作, 丸山 あずさ, 永瀬 裕朗, 黒澤 寛史, 竹田 洋樹, 上谷 良行, 飯島 一誠

  脳と発達, May 2017, Japanese, (一社)日本小児神経学会


• 幼児期からの糖・脂質代謝の経過をフォローしえたPTRF遺伝子変異による全身性脂肪萎縮症の一例（ポスター発表）

  松本真明, Hiroyuki Awano, Hirota Yushi, Fujita Kaori, Nishiyama Masahiro, 李 知子, Nagase Hiroaki, 八木麻理子, 竹島 泰弘, Ogawa Wataru, Iijima Kazumoto

  第60回日本糖尿病学会年次学術集会, May 2017, Japanese, 日本糖尿病学会, 名古屋, Domestic conference

  Poster presentation


• 幼児期からの糖・脂質代謝の経過をフォローしえたPTRF 遺伝子変異による全身性脂肪萎縮症の一例

  松本 真明, Hiroyuki Awano, Hirota Yushi, Fujita Kaori, Nishiyama Masahiro, 李 知子, Nagase Hiroaki, 八木 麻理子, 竹島 泰弘, Ogawa Wataru, Iijima Kazumoto

  第60回日本糖尿病学会年次学術集会, May 2017, Japanese, 日本糖尿病学会, 名古屋, Domestic conference

  Oral presentation


• 不明熱を契機にRadiologically Isolated Syndromeを呈した1例

  Nishiyama Masahiro, 松本真明, Fujita Kaori, Hiroyuki Awano, Nagase Hiroaki, Takada Satoshi, Iijima Kazumoto

  第61回 日本小児神経学会近畿地方会, May 2017, Japanese, 日本小児神経学会, 大阪, Domestic conference

  Oral presentation


• 乳幼児感覚プロファイルを用いた発達障害児の感覚特性に関する検討

  Fujita Kaori, 高木康子, 吉岡三惠子, 加藤威, 溝渕雅巳, 北山真次, Takada Satoshi, 松本真明, Nishiyama Masahiro, Hiroyuki Awano, Nagase Hiroaki, Iijima Kazumoto, Nishimura Noriyuki

  270回 日本小児科学会兵庫県地方会, May 2017, Japanese, 日本小児科学会, 尼崎, Domestic conference

  Oral presentation


• 超低出生体重児のSGA性低身長症に対する成長ホルモン治療の検討

  松本真明, Hiroyuki Awano, Fujita Kaori, Nishiyama Masahiro, Nagase Hiroaki, Morioka Ichiro, Iijima Kazumoto

  第120回 日本小児科学会学術集会, May 2017, Japanese, 日本小児科学会, 東京, Domestic conference

  Poster presentation


• 早産児の臍帯由来間葉系幹細胞の増殖におけるWNTシグナル経路の役割

  岩谷壮太, Shono Akemi, Yamana Keiji, Khin Kyae Mon Thwin, 黒川大輔, Nishida Kosuke, Nishiyama Masahiro, 生田寿彦, 吉田牧子, 溝渕雅巳, Morioka Ichiro, Iijima Kazumoto, Nishimura Noriyuki

  第16回日本再生医療学会総会, May 2017, Japanese, 日本再生医療学会, 仙台, Domestic conference

  Oral presentation


• 先天性甲状腺機能低下症（CH）のフォロー中に偽性副甲状腺機能低下症と診断された1例

  Hiroyuki Awano, 松本真明, Nishiyama Masahiro, Fujita Kaori, Nagase Hiroaki, Iijima Kazumoto

  日本小児科学会兵庫県地方会, May 2017, Japanese, 日本小児科学会, 神戸, Domestic conference

  Oral presentation


• 迅速検査を行った咽頭炎患児におけるA群溶連菌感染症の陽性率とその陽性予測因子

  Nishiyama Masahiro, Yamamura Tomohiko, Minamikawa Shogo, Ikeda Mariko, 安達正時, 石橋和人, Iijima Kazumoto, 石田明人, Morioka Ichiro

  第120回 日本小児科学会学術集会, May 2017, Japanese, 日本小児科学会, 東京, Domestic conference

  Oral presentation


• 近位筋優位の筋力低下から筋疾患が疑われたシャルコー・マリー・トゥース病2型

  Hiroyuki Awano, 松本真明, Fujita Kaori, Nishiyama Masahiro, 港敏朗, Nagase Hiroaki, Iijima Kazumoto

  第120回 日本小児科学会学術集会, May 2017, Japanese, 日本小児科学会, 東京, Domestic conference

  Poster presentation


• ブレオマイシン誘発肺障害モデルラットにおける臍帯由来間葉系幹細胞の効果

  Yamana Keiji, 岩谷壮太, 黒川大輔, Khin Kyae Mon Thwin, Nishida Kosuke, Nishiyama Masahiro, Shono Akemi, 生田寿彦, 吉田牧子, 溝渕雅巳, Morioka Ichiro, Iijima Kazumoto, Nishimura Noriyuki

  第16回日本再生医療学会総会, May 2017, Japanese, 日本再生医療学会, 仙台, Domestic conference

  Oral presentation


• カーボカウントが1型糖尿病の血糖管理に有用であった7歳男児例

  松本 真明, Hiroyuki Awano, Fujita Kaori, Nishiyama Masahiro, Nagase Hiroaki, Iijima Kazumoto

  270回 日本小児科学会兵庫県地方会, May 2017, Japanese, 日本小児科学会, 尼崎, Domestic conference

  Oral presentation


• Digenicな変異で発症した、致死性不整脈と心筋緻密化障害を認めた兄弟例

  長坂 美和子, Ikeda Mariko, Nishiyama Masahiro, Hiroyuki Awano, Nagase Hiroaki, Morioka Ichiro, 倉橋浩樹, Iijima Kazumoto

  第120回 日本小児科学会学術集会, May 2017, Japanese, 日本小児科学会, 東京, Domestic conference

  Poster presentation


• Comparison of the efficacy and complications of fosphenytoin versus continuous midazolam in children with febrile status epilepticus

  Nishiyama Masahiro, Nagase Hiroaki, Ishida Y, Tanaka T, Fujita Kaori, Toyoshima D, Maruyama A, Matsumoto M, Nagai M, Tomioka K, Bo R, Fujita Kaori, Hiroyuki Awano, Takeda H, Uetani Y, Takada Satoshi, Iijima Kazumoto

  14th Asian and Oceanian Congress of Child Neurology, May 2017, English, Fukuoka, Japan, International conference

  Oral presentation


• Case series of fatal acute encephalopathy

  Tomioka K, Nagase Hiroaki, Ishida Y, Tanaka T, Nishiyama Masahiro, Fujita Kaori, Toyoshima D, Maruyama A, Matsumoto M, Nagai M, Boh R, Hiroyuki Awano, Takeda H, Uetani Y, Takeda S, Iijima Kazumoto

  14th Asian and Oceanian Congress of Child Neurology, May 2017, English, Fukuoka, Japan, International conference

  Poster presentation


• 幼児期からの糖・脂質代謝の経過をフォローしえたPTRF遺伝子変異による全身性脂肪萎縮症の一例

  松本 真明, 粟野 宏之, 廣田 勇士, 永井 正志, 坊 亮輔, 冨岡 和美, 前山 花織, 西山 将広, 李 知子, 永瀬 裕明, 八木 麻理子, 竹島 泰弘, 小川 渉, 飯島 一誠

  糖尿病, Apr. 2017, Japanese, (一社)日本糖尿病学会


• Efficacy and Complications of Fosphenytoin Versus Continuous Midazolam in Children with Febrile Status Epilepticus

  NISHIYAMA Masahiro, NAGASE Hiroaki, ISHIDA Yusuke, TANAKA Tsukasa, FUJITA Kyoko, TOYOSHIMA Daisaku, MARUYAMA Azusa, MATSUMOTO Masaaki, TOMIOKA Kazumi, BO Ryosuke, MAEYAMA Kaori, AWANO Hiroyuki, TAKEDA Hiroki, UETANI Yoshiyuki, TAKADA Satoshi, IIJIMA Kazumoto

  Brain & Development, Apr. 2017, English


• Description of Clinical Course and Predictive Score in Fatal Acute Encephalopathy

  TOMIOKA Kazumi, NAGASE Hiroaki, NAGASE Hiroaki, ISHIDA Yusuke, TANAKA Tsukasa, NISHIYAMA Masahiro, NISHIYAMA Masahiro, FUJITA Kyoko, TOYOSHIMA Daisaku, MARUYAMA Azusa, MATSUMOTO Masaaki, NAGAI Masashi, BOH Ryosuke, MAEYAMA Kaori, AWANO Hiroyuki, TAKEDA Hiroki, UETANI Yoshiyuki

  Brain & Development, Apr. 2017, English


• 重篤な経過を呈した、両親由来の異なる心筋障害遺伝子の変異を受け継いだ兄弟例

  長坂 美和子, Ikeda Mariko, 稲垣 秀人, 大内 雄矢, Nishiyama Masahiro, Hiroyuki Awano, Nagase Hiroaki, Morioka Ichiro, Toda Tatsushi, 倉橋 浩樹, Iijima Kazumoto

  日本小児遺伝学会, Dec. 2016, Japanese, 日本小児遺伝学会, 東京, Domestic conference

  Oral presentation


• Changes In Patients With Acute Gastroenteritis After Voluntary Introduction Of Rotavirus Vaccine In A Japanese Children’s Primary Emergency Medical Center

  Kamiyoshi N, Iwatani Souta, Nishiyama Masahiro, Yamamura Tomohiko, Minamikawa Shogo, Ikeda Mariko, Ishibashi K, Iijima Kazumoto, Ishida A, Morioka Ichiro

  12nd Congress for Asian Society for Pediatric Research 2016, Nov. 2016, English, Congress for Asian Society for Pediatric Research, Bangkok, Thailand, International conference

  Poster presentation


• A pediatric patient with interstitial pneumonia due to enterovirus D68.

  Matsumoto M, Hiroyuki Awano, Ogi M, Tomioka K, Unzaki A, Nishiyama Masahiro, Toyoshima D, Ikeda Mariko, Ishida A, Nagase Hiroaki, Morioka Ichiro, Iijima Kazumoto

  12nd Congress for Asian Society for Pediatric Research 2016, Nov. 2016, English, Congress for Asian Society for Pediatric Research, Bangkok, Thailand, International conference

  Poster presentation


• ビタミンB12反応性を認めた軽症メチルマロニルCoAムターゼ欠損症の一例

  永井正志, Hiroyuki Awano, 松本真明, 坊亮輔, 冨岡和美, Nishiyama Masahiro, 西山敦史, Nagase Hiroaki, 長谷川有紀, Iijima Kazumoto

  第58回先天代謝異常学会, Oct. 2016, Japanese, 日本先天代謝異常学会, 東京, Domestic conference

  Poster presentation


• AST 上昇を認めない重症熱性けいれん小児における非けいれん性発作―長期予後の検討

  丸山あずさ, 石田悠介, 田中司, Nishiyama Masahiro, 豊嶋大作, Nagase Hiroaki

  第50回日本てんかん学会学術集会, Oct. 2016, Japanese, 日本てんかん学会, 静岡, Domestic conference

  Oral presentation


• 3歳児より筋力低下を呈したシャルコリー・マリー・トゥース病2型の一例

  永井正志, Hiroyuki Awano, 松本真明, 坊 亮輔, 冨岡和美, Fujita Kaori, Nishiyama Masahiro, Nagase Hiroaki, Iijima Kazumoto

  第60回日本小児神経学会近畿地方会, Oct. 2016, Japanese, 日本小児神経学会, 大阪, Domestic conference

  Oral presentation


• 点頭てんかん再発例に対しケトン食療法とACTH反復投与を行った一例

  本郷 裕斗, Nagase Hiroaki, 永井 正志, 松本 真明, 冨岡 和美, Fujita Kaori, Nishiyama Masahiro, Hiroyuki Awano, Iijima Kazumoto

  第 269 回 日本小児科学会兵庫県地方会, Sep. 2016, Japanese, 日本小児科学会兵庫県地方会, 姫路, Domestic conference

  Oral presentation


• Interferon beta-1a therapy for multiple sclerosis diagnosed in early childhood

  Nishiyama Masahiro, Nagase Hiroaki, Matsumoto M, Tomioka K, Hiroyuki Awano, Tanaka T, Toyoshima D, Maruyama A, Oyazato Y, Saeki K, Shiraishi K, Takada Satoshi, Iijima Kazumoto

  Congress of the European Committee for Treatment and Research in Multiple Sclerosis, Sep. 2016, English, EUROPEAN COMMITTEE FOR TREATMENT AND RESEARCH IN MULTIPLE SCLEROSIS, London, UK, International conference

  Poster presentation


• 神戸こども初期急病センターにおけるインフルエンザ流行の影響

  石橋和人, 石田明人, 木村誠, 片山以登, 児玉真美, 安井美佳, 尾崎美恵, 福田徹哉, 森本麻紀, Morioka Ichiro, Ikeda Mariko, 神吉直宙, Iwatani Souta, Nishiyama Masahiro

  第30回日本小児救急医学会学術集会, Jul. 2016, Japanese, 日本小児救急医学, 仙台, Domestic conference

  Oral presentation


• 間質性肺炎を発症したエンテロウイルスD68感染症の一例

  松本真明, Hiroyuki Awano, 荻 美貴, 冨岡和美, 運﨑 愛, Nishiyama Masahiro, 豊嶋大作, Ikeda Mariko, 石田明子, Nagase Hiroaki, Morioka Ichiro, Iijima Kazumoto

  第30回日本小児救急医学会学術集会, Jul. 2016, Japanese, 日本小児救急医学, 仙台, Domestic conference

  Poster presentation


• 一次小児救急施設の二歳以下の感染性胃腸炎の受診児と重症児数：ウイルス流行時期での比較

  神吉直宙, Nishiyama Masahiro, Iwatani Souta, Ikeda Mariko, 石橋和人, Iijima Kazumoto, 石田明人, Morioka Ichiro

  第30回日本小児救急医学会学術集会, Jul. 2016, Japanese, 日本小児救急医学, 仙台, Domestic conference

  Poster presentation


• 一次小児救急施設の二歳以下の感染性胃腸炎の受診児と重症児数：2011～2014年の変遷

  神吉直宙, Nishiyama Masahiro, Iwatani Souta, Ikeda Mariko, 石橋和人, Iijima Kazumoto, 石田明人, Morioka Ichiro

  第30回日本小児救急医学会学術集会, Jul. 2016, Japanese, 日本小児救急医学, 仙台, Domestic conference

  Oral presentation


• Efficacy and CNS depression of second–line treatment in children with febrile status epilepticus

  Nishiyama Masahiro, Nagase Hiroaki, Ishida Y, Tanaka T, Fujita Kaori, Toyoshima D, Maruyama A, Matsumoto M, Tomioka K, Unzaki A, Hiroyuki Awano, Takeda H, Uetani Y, Takada Satoshi, Iijima Kazumoto

  18th Annual Meeting of Infantile Seizure Society, Jul. 2016, English, Infantile Seizure Society, 東京, International conference

  Oral presentation


• Case series of fatal acute encephalopathy

  Tomioka K, Nagase Hiroaki, Ishida Y, Tanaka T, Nishiyama Masahiro, Fujita Kaori, Toyoshima D, Maruyama A, Matsumoto M, Unzaki A, Hiroyuki Awano, Takeda H, Uetani Y, Takada Satoshi, Iijima Kazumoto

  18th Annual Meeting of Infantile Seizure Society, Jul. 2016, English, International Symposium on Acute Encephalopathy in Infancy and Its Related Disorders, 東京, International conference

  Poster presentation


• 急性脳症治療における全身麻酔療法導入時のチアミラール投与の検討

  石田悠介, 冨岡和美, 田中 司, Nishiyama Masahiro, 藤田杏子, 豊嶋大作, 丸山あずさ, Nagase Hiroaki, 竹田洋樹, 上谷良行

  第58回日本小児神経学会学術集会, Jun. 2016, Japanese, 日本小児神経学会, 東京, Domestic conference

  Oral presentation


• 急性脳症による死亡例の検討

  冨岡和美, Nagase Hiroaki, 石田悠介, 田中司, Nishiyama Masahiro, 藤田杏子, 豊嶋大作, 丸山あずさ, 竹田洋樹, 上谷良行, 松本真明, 運崎愛, Hiroyuki Awano, Takada Satoshi, Iijima Kazumoto

  第58回日本小児神経学会学術集会, Jun. 2016, Japanese, 日本小児神経学会, 東京, Domestic conference

  Oral presentation


• ジストロフィン遺伝子イントロン64のスプライス供与部位の変異（c.9361+1 G>A）により発症したDuchenne型筋ジストロフィーの一例

  Hiroyuki Awano, 松本真明, 冨岡和美, 運﨑 愛, Nishiyama Masahiro, 李 知子, Nagase Hiroaki, Ikeda Mariko, 八木麻理子, 竹島泰弘, 松尾雅文, Iijima Kazumoto

  第58回日本小児神経学会学術集会, Jun. 2016, Japanese, 日本小児神経学会, 東京, Domestic conference

  Oral presentation


• 小児のてんかん重積状態におけるセカンドライン治療の有効性と意識抑制作用の検討

  西山 将広, 永瀬 裕朗, 石田 悠介, 田中 司, 藤田 杏子, 豊嶋 大作, 丸山 あずさ, 松本 真明, 冨岡 和美, 運崎 愛, 粟野 宏之, 竹田 洋樹, 上谷 良行, 高田 哲, 飯島 一誠

  脳と発達, May 2016, Japanese, (一社)日本小児神経学会


• 急性脳症による死亡例の検討

  冨岡 和美, 永瀬 裕朗, 石田 悠介, 田中 司, 西山 将広, 藤田 杏子, 豊嶋 大作, 丸山 あずさ, 竹田 洋樹, 上谷 良行, 松本 真明, 運崎 愛, 粟野 宏之, 高田 哲, 飯島 一誠

  脳と発達, May 2016, Japanese, (一社)日本小児神経学会


• ジストロフィン遺伝子イントロン64のスプライス供与部位の変異(c.9361+1G>A)により発症したDuchenne型筋ジストロフィーの一例

  粟野 宏之, 松本 真明, 冨岡 和美, 運崎 愛, 西山 将広, 李 知子, 永瀬 裕明, 池田 真理子, 八木 麻理子, 竹島 泰弘, 松尾 雅文, 飯島 一誠

  脳と発達, May 2016, Japanese, (一社)日本小児神経学会


• 当院における自己免疫性神経疾患に対するリツキシマブの使用経験

  冨岡和美, Nishiyama Masahiro, 松本真明, 運﨑 愛, 豊嶋大作, Hiroyuki Awano, 中川 卓, 森貞直哉, Takada Satoshi, Iijima Kazumoto

  第119回日本小児科学会学術集会, May 2016, Japanese, 日本小児科学会, 札幌, Domestic conference

  Oral presentation


• ビタミンB12反応性メチルマロン酸血症の一例

  永井正志, Hiroyuki Awano, 松本真明, 坊亮輔, 冨岡和美, Fujita Kaori, Nishiyama Masahiro, 西山敦史, Nagase Hiroaki, 長谷川有紀, Iijima Kazumoto

  第268回日本小児科学会兵庫県地方会, May 2016, Japanese, 日本小児科学会兵庫県地方会, 神戸, Domestic conference

  Oral presentation


• L‐カルニチン内服治療を行った3‐メチルクロトニルCoAカルボキシラーゼ欠損症の一例

  松本真明, Hiroyuki Awano, 冨岡和美, 運﨑 愛, Nishiyama Masahiro, 豊嶋大作, 八木麻理子, 長谷川有紀, 竹島泰弘, Iijima Kazumoto

  第119回日本小児科学会学術集会, May 2016, Japanese, 日本小児科学会, 札幌, Domestic conference

  Poster presentation


• 臍帯由来間葉系幹細胞における児の在胎週数による転写制御解析

  Iwatani Souta, 黒川大輔, Nishiyama Masahiro, 庄野朱美, 吉田牧子, 溝渕雅巳, Morioka Ichiro, Iijima Kazumoto, Nishimura Noriyuki

  第15回日本再生医療学会, Mar. 2016, Japanese, 日本再生医療学会, 大阪, Domestic conference

  Oral presentation


• Current Situation of Treatment for Anaphylaxis in a Japanese Pediatric Emergency Center.

  Ninchoji Takeshi, Sota Iwatani, Nishiyama Masahiro, Kamiyoshi Naohiro, Ikeda Mariko, Morisada Naoya, Kazuto Ishibashi, Iijima Kazumoto, Akihito Ishida, Morioka Ichiro

  the 5th Global Congress for Consensus in Pediatrics & Child Health, Mar. 2016, English, CIP, Xi’an, 中国, International conference

  Oral presentation


• 間質性肺炎を合併したエンテロウイルスD68型感染症の一例

  松本真明, Hiroyuki Awano, 冨岡和美, 運崎愛, Nishiyama Masahiro, Toyoshima Daisaku, 永瀬裕朗, Morisada Naoya, Iijima Kazumoto

  第267回日本小児科学会兵庫県地方会, Feb. 2016, Japanese, 日本小児科学会兵庫県地方会, 西宮, Domestic conference

  Oral presentation


• タンデムマス・スクリーニングでグルタル酸血症1型が疑われた低形成腎の1例

  松本 真明, 粟野 宏之, 運崎 愛, 西山 将広, 豊嶋 大作, 忍頂寺 毅史, 森貞 直哉, 中川 卓, 上村 裕保, 八木 麻理子, 飯島 一誠

  日本小児科学会雑誌, Jan. 2016, Japanese, (公社)日本小児科学会


• 異なる経過をたどった腸管出血性大腸菌による溶血性尿毒症症候群の姉妹例

  中西啓太, Ninchoji Takeshi, 南川将吾, 山村智彦, Kamiyoshi Naohiro, Nishiyama Masahiro, Toyoshima Daisaku, Nozu Kandai, 濱平陽史, Iijima Kazumoto

  第37回日本小児腎不全学会学術集会, Nov. 2015, Japanese, 日本小児腎不全学会, 石川, Domestic conference

  Oral presentation


• 発作時脳波により診断したInfantile spasms without hypsarrhythmiaの2例

  冨岡和美, 松本真明, 運崎 愛, Nishiyama Masahiro, 加藤神奈, Toyoshima Daisaku, Hiroyuki Awano, Ikeda Mariko, Morisada Naoya, Takada Satoshi, Iijima Kazumoto

  第49回日本てんかん学会総会, Oct. 2015, Japanese, 日本小児感染症学会, 長崎, Domestic conference

  Poster presentation


• 次子の出生前診断を契機に確定診断に至った先天性GPI欠損症の孤発例

  運崎 愛, Ikeda Mariko, 中川 卓, Nishiyama Masahiro, Toyoshima Daisaku, Morisada Naoya, 岡本伸彦, 倉橋浩樹, 村上良子, Morioka Ichiro, Toda Tatsushi, Iijima Kazumoto

  日本人類遺伝学会 第60回大会, Oct. 2015, Japanese, 日本人類遺伝学会, 東京, Domestic conference

  Poster presentation


• 重度の神経学的後遺症を残した腸管出血性大腸菌による溶血性尿毒症症候群の1 女児例

  中西啓太, 南川将吾, 山村智彦, Kamiyoshi Naohiro, Nishiyama Masahiro, Ninchoji Takeshi, Toyoshima Daisaku, Nozu Kandai, 濱平陽史, Iijima Kazumoto

  第266 回 日本小児科学会兵庫県地方会, Sep. 2015, Japanese, 日本小児科学会兵庫県地方会, 姫路, Domestic conference

  Oral presentation


• 3 -メチルクロトニル- CoAカルボキシラーゼ欠損症の2例

  松本真明, Hiroyuki Awano, 冨岡和美, 運﨑 愛, Nishiyama Masahiro, 加藤神奈, Toyoshima Daisaku, 西山敦史, 八木麻理子, 竹島泰弘, Iijima Kazumoto

  第266 回 日本小児科学会兵庫県地方会, Sep. 2015, Japanese, 日本小児科学会兵庫県地方会, 姫路, Domestic conference

  Oral presentation


• 発作時脳波により診断したinfantile spasms without hypsarrhythmiaの1例

  冨岡和美, 松本真明, 運崎 愛, Nishiyama Masahiro, 加藤神奈, Toyoshima Daisaku, Hiroyuki Awano, Ikeda Mariko, Morisada Naoya, Takada Satoshi, Iijima Kazumoto

  第11回日本てんかん学会近畿地方会, Jul. 2015, Japanese, 日本てんかん学会, 大阪, Domestic conference

  Oral presentation


• TruSight One シークエンスパネルで原因遺伝子を同定した神経発達疾患の3例

  Morisada Naoya, Toyoshima Daisaku, Nishiyama Masahiro, 永瀬裕朗, 丸山あずさ, 中川 卓, 和田博子, Takada Satoshi, Nishio Hisahide, Iijima Kazumoto

  第38回日本小児遺伝学会学術集会, Jul. 2015, Japanese, 日本小児遺伝学会, 横浜, Domestic conference

  Oral presentation


• 幼児期に発症した多発性硬化症の4例

  Nishiyama Masahiro, 永瀬裕朗, 田中 司, 丸山あずさ, Toyoshima Daisaku, 中川 卓, Morisada Naoya, 親里嘉展, 佐伯啓介, Takada Satoshi, Iijima Kazumoto

  第57回日本小児神経学会学術集会, May 2015, Japanese, 日本小児神経学会, 大阪, Domestic conference

  Oral presentation


• タンデムマス・スクリーニングでグルタル酸血症1 型が疑われた低形成腎の1 例

  松本真明, Hiroyuki Awano, 運﨑 愛, Nishiyama Masahiro, Toyoshima Daisaku, Ninchoji Takeshi, Morisada Naoya, 中川 卓, 上村裕保, 八木麻理子, Iijima Kazumoto

  第265 回 日本小児科学会兵庫県地方会, May 2015, Japanese, 日本小児科学会兵庫県地方会, 神戸, Domestic conference

  Oral presentation


• The first case of Japanese limb-girdle muscular dystrophy 2I with a novel FKRP mutation.

  Hiroyuki Awano, Nishiyama Masahiro, Tomoko Lee, Mariko Yagi, Yasuhiro Takeshima, Kanako Goto, Satomi Mitsuhashi, Ichizo Nishino, Masafumi Matsuo, Iijima Kazumoto

  第57回日本小児神経学会学術集会, May 2015, English, 日本小児神経学会, 大阪, Domestic conference

  Oral presentation


• Short and long-term outcome in children with acute encephalopathy.

  Nishiyama Masahiro, Nagase Hiroaki, Tanaka T, Fujita Kaori, Maruyama A, Toyoshima Daisaku, Nakagawa Taku, Morisada Naoya, Takada Satoshi, Iijima Kazumoto

  Asian and Oceania Congress of Child Neurology（AOCCN）2015, May 2015, English, ASEAN Neurological Association, 台湾, 台湾, International conference

  Poster presentation


• Efficacy and limitations of everolimus for 5 tuberous sclerosis complex patients with refractory epilepsies.

  Toyoshima Daisaku, Nishiyama Masahiro, Morisada Naoya, Nakagawa Taku, Adachi M, Takada Satoshi, Iijima Kazumoto

  Asian and Oceania Congress of Child Neurology（AOCCN）2015, May 2015, English, ASEAN Neurological Association, 台湾, 台湾, International conference

  Poster presentation


• Demographics and outcomes of pediatric febrile convulsive status epilepticus.

  Nishiyama Masahiro, Hiroaki Nagase, Tsukasa Tanaka, Kyoko Fujita, Azusa Maruyama, Toyoshima Daisaku, Nakagawa Taku, Ikeda Mariko, Morioka Ichiro, Morisada Naoya, Takada Satoshi, Iijima Kazumoto

  American Epilepsy Society 68th Annual Meeting, Dec. 2014, English, AES, Seattle, USA, International conference

  Poster presentation


• てんかん発作にビタミンB6製剤が著効した先天性GPI欠損症の一男児例

  Nakagawa Taku, Nishiyama Masahiro, Toyoshima Daisaku, Morisada Naoya, Ikeda Mariko, 岡本 伸彦, 村上 良子, Takada Satoshi, Iijima Kazumoto

  第48回日本てんかん学会学術集会, Oct. 2014, Japanese, 日本てんかん学会, 東京, Domestic conference

  Poster presentation

• 日本臨床神経生理学会


• 日本小児精神神経学会


• American Epilepsy Society


• American Academy of Neurology


• Japan Epilepsy Society


• Japanese Society of Child Neurology


• Japan Pediatric Society

• 長時間脳波データ解析とプロテオミクスによる包括的な急性脳症の病態解明

  西山 将広

  日本学術振興会, 科学研究費助成事業, 基盤研究(C), 神戸大学, Apr. 2022 - Mar. 2026


• 学力および非認知能力に影響を与える乳幼児期因子の解明

  公益財団法人小児医学研究振興財団, Apr. 2022 - Mar. 2023, Principal investigator


• 痙攣重積型（二相性）急性脳症の発病予測と後遺症軽減のためのレジストリ構築

  西山将広

  公益財団法人難病医学研究財団, 令和2年度医学研究奨励助成事業, Apr. 2021 - Mar. 2023, Principal investigator


• 臨床データベースと患者検体を活用した経時的かつ包括的解析による急性脳症のバイオマーカーの開発

  西山将広

  公益財団法人 川野小児医学奨学財団, 令和2年度 第31回若手枠研究助成金, Jun. 2020 - Mar. 2021, Principal investigator


• 臨床データベースと患者検体を活用したサイトカインストーム型急性脳症の病態解明

  西山 将広

  科学研究費補助金／若手研究, Apr. 2018 - Mar. 2021, Principal investigator

  Competitive research funding


• てんかん重積状態の脳傷害に対する間葉系幹細胞を用いた新規治療法の開発

  西山 将広

  学術研究助成基金助成金／若手研究(B), Apr. 2015 - Mar. 2018, Principal investigator

  Competitive research funding