Research activity information

• Dec. 2019 神戸大学小児科同門会員学術論文賞

• Pediatric Coronavirus Disease 2019 Clinical Characteristics in a Primary Emergency Medical Center: Comparative Analysis of BA.1/BA.2, BA.5/BQ.1, and XBB Periods

  Hiroshi Yamaguchi, Tomohiro Sameshima, Kazumichi Fujioka, Tomoko Horinouchi, Yoshitaka Asagai, Hiroki Ohashi, Tomoko Fujikawa, Akihiro Nishimura, Akihiro Tamura, Shingo Ishimori, Kandai Nozu, Masaaki Kugo, Akihito Ishida

  Mar. 2026, Journal of Infection and Chemotherapy, English

  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.

  Feb. 2026, BMJ Paediatrics Open, English

  Scientific journal


• Epidemiological Characterization and Clinical Utility of the Multiplex PCR FilmArray™ Gastrointestinal Panel at a Tertiary University Hospital in Japan, 2024

  Yoshitaka Asagai, Hiroshi Yamaguchi, Tomohiko Yamamura, Mari Kusuki, Kenichiro Ohnuma, Tomoko Horinouchi, Ryosuke Bo, Nobuyuki Yamamoto, Yoshihiko Yano, Hiroaki Nagase, Kandai Nozu

  Dec. 2025, Pediatrics & Neonatology, English

  Scientific journal


• The first case of Al-Raqad syndrome in Japan is associated with a homozygous DCPS exonic variant resulting in aberrant splicing

  Haruka Nozaki, Nana Sakakibara, Hiroaki Hanafusa, Yuta Inoki, Yu Tanaka, Hideaki Kitakado, Chika Ueda, China Nagano, Tomoko Horinouchi, Tomohiko Yamamura, Shingo Ishimori, Hiroshi Yamaguchi, Kandai Nozu, Naoya Morisada

  Aug. 2025, Brain and Development, English

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• 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


• 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


• 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


• Au-Kline syndrome with a novel variant in a girl presenting with heat intolerance in the summer: A case report and literature review

  Kazuki Suemune, Hiroshi Yamaguchi, Hiroaki Hanafusa, Ming Juan Ye, Kandai Nozu, Hiroaki Nagase

  Jun. 2025, Brain and Development Case Reports, English

  Scientific journal


• Description of Patterns in Reduced-Lead EEG 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

  May 2025, Pediatric Neurology, English

  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

  AbstractPurposeStatus 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.MethodThis 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.FindingFifteen 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.ConclusionOur 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, English

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• 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

  Feb. 2025, Journal of the Neurological Sciences, English

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• A Rare Case of Recurrent Disturbed Consciousness with Alternating Hemiplegia Associated with 46,XX,der(18)t(12;18)(p11.2;p11.2)

  Aoi Kawamura, Hiroshi Yamaguchi, Hiroaki Hanafusa, Tatsuhito Ito, Yuki Kyono, Tomohiro Samejima, Shizuka Oikawa, Shoichi Tokumoto, Kandai Nozu, Hiroaki Nagase

  AbstractTrisomy of the short arm of chromosome 12 (trisomy 12p) is a rare chromosomal abnormality causing dysmorphic features, congenital anomalies, intellectual disabilities, developmental delays, and seizures. Detailed information regarding the types of seizures is scarce owing to the low incidence of seizures. In contrast, 18p deletions and seizures are rare. Previous reports on trisomy 12p or monosomy 18p are limited, and little is known about epilepsy in children with trisomy 12p or monosomy 18p. Here, we report a case of 46,XX,der(18)t(12;18)(p11.2;p11.2) with repeatedly disturbed consciousness accompanied by alternating hemiplegia, corresponding to the electroencephalogram findings. G-banding of the parents showed balanced translocation of the mother. The last hospitalization occurred when the patient was 12 years old and presented with disturbed consciousness and left-sided hemiplegia. Electroencephalogram showed continuous 1 to 2 Hz slow waves in the right hemisphere and a theta burst in the left hemisphere. Based on the genes on chromosomes 12 and 18, the symptoms seemed to be related to partial trisomy 12p. Our case suggests the possibility of a novel seizure phenotype associated with trisomy 12p.

  Dec. 2024, Journal of Pediatric Neurology, English

  Scientific journal


• 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

  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.

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

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• 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


• 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


• End-stage ADPKD with a low-frequency PKD1 mosaic variant accelerated by chemoradiotherapy.

  Hiroaki Hanafusa, Hiroshi Yamaguchi, Naoya Morisada, Ming Juan Ye, Riki Matsumoto, Hiroaki Nagase, Kandai Nozu

  Autosomal dominant polycystic kidney disease (ADPKD) is commonly caused by PKD1, and mosaic PKD1 variants result in milder phenotypes. We present the case of a 32 year-old male with chronic active Epstein-Barr virus who underwent bone marrow transplantation with chemoradiotherapy at age 9. Despite a low-frequency mosaic splicing PKD1 variant, he developed severe renal cysts and end-stage renal disease in his 30 s. This case highlights how environmental factors may contribute to the genetic predisposition to ADPKD.

  Mar. 2024, Human genome variation, 11(1) (1), 17 - 17, English, International magazine

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• 意識障害と片麻痺を繰り返した46,XX,der(18)t(12;18)(p11.2;p11.2)

  川村 葵, 山口 宏, 伊藤 立人, 曽根原 晶子, 洪 聖媛, 鮫島 智大, 花房 宏昭, 老川 静香, 徳元 翔一, 坊 亮輔, 永瀬 裕朗

  (一社)日本小児神経学会, Mar. 2024, 脳と発達, 56(2) (2), 151 - 151, Japanese


• 持続する右下肢痛で発症した骨肉腫の1例

  伊藤 立人, 川村 葵, 曽根原 晶子, 西村 明紘, 洪 聖媛, 鮫島 智大, 花房 宏昭, 老川 静香, 徳元 翔一, 山口 宏, 坊 亮輔, 山本 暢之, 永瀬 裕朗

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


• 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.

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

  Scientific journal


• 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


• Treatment Options for Infantile Spasms Syndrome with SCN8A: A Case Report and Literature Review

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

  Abstract“Infantile spasms syndrome (IS),” previously known as “West syndrome (WS),” is characterized by epileptic spasms (ES), abnormal electroencephalography (EEG) patterns such as hypsarrhythmia, and developmental stagnation or regression in infancy. IS has various etiologies, including genetic abnormalities. SCN8A variants are associated with developmental and epileptic encephalopathy, characterized by developmental delay, seizures starting from infancy, and refractory epilepsy with multiple seizure types. However, previous studies have not focused on the treatment of IS caused by SCN8A variants. We report a case of a previously healthy boy who presented ES and developmental regression at 6 months of age. His EEG revealed hypsarrhythmia, leading to the diagnosis of IS. After admission, the patient was treated with hormonal therapy using intravenous methylprednisolone pulse therapy (MPT). ES and hypsarrhythmia on EEG disappeared in the early stages of MPT administration with no observed treatment complications. Furthermore, we observed no recurrence of EEG abnormalities or seizures at 17 months of age. Genetic testing revealed a novel de novo SCN8A variant (NM_001177984.2:c.2882T > G:p. M961R). The literature review confirmed that 13 patients, including our described patient, were reported to have ES owing to missense variants of SCN8A. While the previous articles do not mention intravenous MPT for ES with SCN8A, our case findings suggest that intravenous MPT therapy may be effective for short-term suppression of ES caused by the SCN8A variant in IS.

  Jan. 2024, Journal of Pediatric Epilepsy, English

  Scientific journal


• 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

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• 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


• 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


• 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

  Scientific journal


• 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


• 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


• 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


• 拡大新生児マススクリーニングで発見された脊髄性筋萎縮症の2例

  曽根原 晶子, 坊 亮輔, 池谷 紀衣子, 南部 静紀, 老川 静香, 徳元 翔一, 山口 宏, 冨岡 和美, 永瀬 裕朗, 竹島 泰弘, 飯島 一誠, 野津 寛大, 西尾 久英, 粟野 宏之

  (一社)日本小児神経学会, May 2023, 脳と発達, 55(Suppl.) (Suppl.), S319 - S319, 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


• 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


• A Case of Generalized Myasthenia Gravis Exacerbated by COVID-19

  Shingo Kanatani, Hiroshi Yamaguchi, Shizuka Oikawa, Shoichi Tokumoto, Kazumi Tomioka, Masahiro Nishiyama, Kandai Nozu, Hiroaki Nagase

  AbstractMyasthenia gravis (MG) is a rare, long-term neuromuscular disorder that can affect individuals of any age. In Japan, the Omicron variant of coronavirus disease 2019 (COVID-19) began spreading in 2022, and many cases of neurological symptoms caused by the virus have been reported. Although COVID-19 has been reported to exacerbate MG in adults, there are no reports on the effects of COVID-19 on the MG symptoms of pediatric patients. We report the case of a 6-year-old girl with a 3-year history of MG who presented to our hospital with symptom exacerbation after COVID-19 infection. Four days before admission, she developed fever with a runny nose and cough. Three days before admission, she developed severe bilateral blepharoptosis and progressive limb weakness, and 2 days before admission, she was diagnosed with COVID-19 by SARS-CoV-2 antigen test. Physical examination revealed moderate blepharoptosis and mild bilateral upper and lower limb weakness. We diagnosed her with worsening MG due to COVID-19, and she was administered 400 mg/kg intravenous immunoglobulin (IVIG) daily for 5 days with continued oral corticosteroids and tacrolimus. The patient's symptoms improved promptly after admission and, at discharge 7 days after admission, her symptoms had significantly improved. During the 1-month outpatient follow-up period, she remained stable and the anti-acetylcholine receptor (AchR) antibody level was reduced to 14.6 nmol/L (from 18.5 nmol/L on admission). Our case suggests that COVID-19 exacerbates MG in both children and adults.

  Georg Thieme Verlag {KG}, Feb. 2023, Journal of Pediatric Neurology, English

  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

  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

  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

  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


• Impact after the Change from Voluntary to Universal Oral Rotavirus Vaccination on Consecutive Emergency Department Visits for Acute Gastroenteritis among Children in Kobe City, Japan (2016-2022).

  Hiroshi Yamaguchi, Kandai Nozu, Hiroaki Hanafusa, Yoshinori Nambu, Takumi Kido, Atsushi Kondo, Akihiro Tamura, Hiroyuki Awano, Ichiro Morioka, Hiroaki Nagase, Akihito Ishida

  Rotavirus (RV) is the leading cause of acute gastroenteritis (AGE), particularly in infants. In 2006, the high efficacy of oral RV vaccines (RVVs, RotarixTM and RotaTeqTM) was demonstrated. Voluntary RVV started in Japan in 2011, and in October 2020 were launched as universal oral RVVs in Japan. However, the impact of changes from voluntary to universal RVVs has not been studied in a primary emergency medical center in Japan. We investigated changes in the number of pediatric patients with AGE after introducing universal RVVs in our center. A clinical database of consecutive patients aged <16 who presented to Kobe Children's Primary Emergency Medical Center between 1 April 2016 and 30 June 2022 was reviewed. After implementing universal RVVs, fewer children presented with RV-associated AGE (the reduction of proportion of the patients in 2022 was -61.7% (all ages), -57.9% (<1 years), -67.8% (1-<3 years), and -61.4% (3-<5 years) compared to 2019). A similar decrease in those of age who were not covered by the universal RVV was observed. There was a significant decline in the number of patients with AGE during the RV season who presented to the emergency department after implementing universal RVVs.

  Oct. 2022, Vaccines, 10(11) (11), English, International magazine

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• 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


• 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

  (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


• 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


• 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


• Serum cardiac troponin I is a candidate biomarker for cardiomyopathy in Duchenne and Becker muscular dystrophies.

  Hiroshi Yamaguchi, Hiroyuki Awano, Tetsushi Yamamoto, Yoshinori Nambu, Kazumoto Iijima

  INTRODUCTION/AIMS: Serum cardiac troponin I (cTnI), its relation to cardiomyopathy, and the contribution of the ACTN3 genotype to serum levels of cTnI in Duchenne and Becker muscular dystrophy (DMD and BMD, respectively) remain unknown. In this study we aimed to reveal the characteristics of cTnI, assess whether cTnI is a biomarker for cardiomyopathy in these dystrophinopathies, and evaluate the contribution of the ACTN3 genotype to the serum levels of cTnI in DMD patients. METHODS: Serum cTnI values obtained from 127 DMD and 47 BMD patients were analyzed retrospectively. The relationship between cTnI and echocardiography data or the ACTN3 XX genotype was assessed. RESULTS: The cTnI levels and proportion of patients with abnormal cTnI levels were significantly higher among DMD patients than BMD, especially in the second decade of life. In DMD, the cTnI level reached a maximum at 13 years, and left ventricular ejection fraction (LVEF) became abnormal approximately 1 year subsequently. In BMD, the cTnI level peaked at the age of 14 years, and LVEF became abnormal 3 years later. Decreased LVEF was observed after cTnI elevation in both populations. cTnI levels by age in DMD patients with the ACTN3 XX genotype tended to increase significantly and early. DISCUSSION: Myocardial injury indicated by cTnI elevation was more common and severe in DMD patients. cTnI elevation preceding cardiac dysfunction may represent an early phase of cardiomyopathy progression and may be a biomarker for early detection of cardiomyopathy in these dystrophinopathies. The ACTN3 XX genotype may be a risk factor for early myocardial injury.

  May 2022, Muscle & nerve, 65(5) (5), 521 - 530, English, International magazine

  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.

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

  Scientific journal

  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

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• 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


• 一次急病施設における異物・毒物誤飲診療の現状と課題

  忍頂寺 毅史, 近藤 淳, 石河 慎也, 運崎 愛, 山口 宏, 林 卓郎, 田中 亮二郎, 永瀬 裕朗, 石田 明人, 野津 寛大

  (公社)日本小児科学会, Feb. 2022, 日本小児科学会雑誌, 126(2) (2), 308 - 308, 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


• Multivariate analysis of the impact of weather and air pollution on emergency department visits for unprovoked seizure among children: A retrospective clinical observational study

  Hiroshi Yamaguchi, K, ai Nozu, Shinya Ishiko, Atsushi Kondo, Nobuyuki Yamamoto, Akihiro Tamura, Yuya Aoto, Ai Unzaki, Kazuto Ishibashi, Ichiro Morioka, Hiroaki Nagase, Akihito Ishida

  BACKGROUND: An unprovoked seizure is a seizure or a cluster of seizures occurring within 24 h in a patient older than 1 month of age without precipitating factors. Recent studies have reported that extrinsic factors, such as meteorological conditions and air pollutants, may be important in seizure occurrence. Thus, this study aimed to examine the association between the number of visits to the emergency department (ED) by children for nighttime unprovoked seizures and exposure to multi-faceted factors, such as meteorological conditions and air pollution. METHODS: We conducted a clinical observational analysis and reviewed consecutive patients younger than 16 years of age who visited the primary ED center in Kobe City, Japan, during nighttime (7:30 p.m.-7:00 a.m.) between January 1, 2011 and December 31, 2015. We investigated the effects of meteorological factors and air pollutants on the number of patients with unprovoked seizures using multivariate analysis of Poisson regression estimates. RESULTS: In total, 151,119 children visited the ED, out of which 97 patients presented with unprovoked seizures. The mean age of the patients was 4.7 years (range, 1 month to 15.3 years), and 54.6% of them were boys. The total number of patients with unprovoked seizures showed no significant changes with the seasons; however, there were dominant peaks during the fall and fewer visits during the summer. The multivariate analysis of Poisson regression estimates revealed a significant positive relationship between the number of patients presenting with unprovoked seizures and precipitation (+1 patient/87 mm; p = 0.03) and methane (+1 patient/0.14 ppm; p = 0.03) levels and a negative relationship between the number of patients presenting with unprovoked seizures and nitrogen dioxide level (-1 patient/0.02 ppm; p = 0.04). CONCLUSIONS: The present study is the first to evaluate the association between the number of children who presented to the ED with nighttime unprovoked seizures and environmental factors after controlling for confounding factors.

  Elsevier {BV}, Dec. 2021, Epilepsy & Behavior, 125, 108434 - 108434, English, International magazine

  Scientific journal


• Impact of the State of Emergency during the COVID-19 Pandemic in 2020 on Asthma Exacerbations among Children in Kobe City, Japan

  Hiroshi Yamaguchi, Kandai Nozu, Shinya Ishiko, Atsushi Kondo, Takeshi Ninchoji, China Nagano, Hiroki Takeda, Ai Unzaki, Kazuto Ishibashi, Ichiro Morioka, Hiroaki Nagase, Kazumoto Iijima, Akihito Ishida

  The coronavirus disease (COVID-19) pandemic altered environmental factors. We studied the impact of these changes on asthma exacerbation (AE) by comparing the AE-related environmental factors between COVID-19 (2020) and pre-COVID-19 (2011-2019) eras. Between 2011 and 2020, 278,465 children (<16 years old) visited our emergency department, and 7476 were diagnosed with AE. The number of patients showed spring and fall peaks in 2011-2019. Multivariate analyses showed significant positive relationships of the number of AE patients with the average temperature among all patients and 0-5-year-olds and with sulfur dioxide (SO2) levels in 2011-2019 among 0-5-year-olds. Although the spring peak in the number of patients was not observed in 2020 after declaration of a state of emergency, the fall peak was again observed after the state of emergency was lifted. No changes in average temperature were detected, but SO2 was significantly reduced following declaration of the state of emergency in 2020. Therefore, SO2 reduction might have contributed to the disappearance of the peak of AE. However, a fall peak was observed again in 2020, although SO2 levels continued to be low. These data suggest that person to person interaction seems to be associated with AE, presumably due to unknown viral infections.

  Oct. 2021, International Journal of Environmental Research and Public Health, 18(21) (21), English, International magazine

  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, K, ai 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.

  Elsevier {BV}, Oct. 2021, Brain and Development, 44(3) (3), 210 - 220, English, International magazine

  Scientific journal


• Epidemiological impact of universal varicella vaccination on consecutive emergency department visits for varicella and its economic impact among children in Kobe City, Japan

  Hiroshi Yamaguchi, K, ai Nozu, Shinya Ishiko, Hiroaki Nagase, Takeshi Ninchoji, China Nagano, Hiroki Takeda, Ai Unzaki, Kazuto Ishibashi, Ichiro Morioka, Kazumoto Iijima, Akihito Ishida

  INTRODUCTION: Previous studies reported a dramatic decline in the incidence of varicella and varicella-related deaths after implementing universal varicella vaccination (VarV). Although previous studies reported the effectiveness and economic impact of VarV, they were unknown in the emergency department (ED) setting. METHODS: To determine the effectiveness and economic impact of VarV in the ED, Kobe, Japan, we retrospectively reviewed the clinical database of consecutive patients younger than 16 years presenting to our primary ED from 2011 to 2019. RESULTS: Of the 265,191 children presenting to our ED, 3,092 patients were clinically diagnosed with varicella. The number of patients with varicella was approximately 500 annually, before introducing the universal two-dose VarV for children aged 1 to <3 years in October 2014, in the Japanese national immunization program, and decreased to approximately 200 in 2019. The number of patients with varicella younger than 1 year (ineligible for the vaccination) also decreased. Regarding the economic impact, the medical cost in our ED reduced after the introduction of VarV was JPY 4.1 million (US$ 40,049) annually. From the central data, approximately 95% of children were vaccinated after October 2014; however, a relatively large percentage of infected unvaccinated children (59.0%) presented to ED in this study. After the implementation of the universal VarV, infection was mainly observed in older children (i.e., the unvaccinated generation). CONCLUSIONS: Our data showed the effectiveness and economic impact of VarV in the ED setting. Additionally, our data suggested that the public vaccination program should include older unvaccinated children and other unvaccinated individuals.

  Elsevier {BV}, Oct. 2021, Journal of Infection and Chemotherapy, 28(1) (1), 35 - 40, English, International magazine

  Scientific journal


• Current management for foreign body and toxic agent ingestion in a paediatric primary emergency centre

  Hiroshi Yamaguchi

  OBJECTIVES: Accidental foreign body ingestion (FBI) and toxic agent ingestion (TAI) are commonly encountered among children in primary emergency settings. Early detection and appropriate medical intervention are crucial to improve outcomes. Although many reports from tertiary institutions have shown improvements in therapy, data are still lacking from primary emergency facilities. METHODS: We performed a retrospective analysis based on medical records of FBI/TAI over 4 years at the Kobe Children's Primary Emergency Medical Center. We collected patient information, including age, sex, time between FBI/TAI occurrence and centre visit, provision of first aid, symptoms, type of FBI/TAI, examinations, treatments, and outcomes. RESULTS: A total of 580 children were enrolled. The median age was 1.3 years, and patients under 2 years old accounted for 70% of total cases. Cigarettes (17.5%) were the most common ingested foreign body, followed by medicines (15.3%), detergents (8.1%), in TAI, plastics (14.1%), metal (13.4%), batteries (9.0%) in FBI, and others (22.6%). A total of 42 patients were transferred to advanced hospitals; among these, 22 patients were hospitalised but the foreign body was removed in only 3 (0.9%) patients. Transferred patients were significantly older (P<0.05) in FBI and had a higher rate of any of symptoms (P<0.05) in FBI/TAI. CONCLUSIONS: This large-scale retrospective study of accidental FBI/TAI conducted at a primary emergency facility clarified current management, including treatment at a primary facility. Very few cases of FBI/TAI were treated, even when they were transferred to an advanced treatment hospital. Unified protocols should be established, to improve the management of FBI/TAI.

  Sep. 2021, Minerva Pediatrics, English, International magazine

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Early steroid pulse therapy for children with suspected acute encephalopathy

  Yusuke Ishida, Masahiro Nishiyama, Hiroshi Yamaguchi, Kazumi Tomioka, Hiroki Takeda, Shoichi Tokumoto, Daisaku Toyoshima, Azusa Maruyama, Yusuke Seino, Kazunori Aoki, K, ai 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.

  Ovid Technologies (Wolters Kluwer Health), Jul. 2021, Medicine, 100(30) (30), e26660, English, International magazine

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• デュシェンヌ型/ベッカー型筋ジストロフィーにおける血清トロポニンIの比較と心機能との関連の検討

  山口 宏, 粟野 宏之, 山本 哲志, 松尾 雅文, 飯島 一誠

  (一社)日本小児神経学会, May 2021, 脳と発達, 53(Suppl.) (Suppl.), S259 - S259, 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


• 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


• Multivariate analysis of the impact of weather and air pollution on emergency department visits for night-time headaches among children: retrospective, clinical observational study

  Hiroshi Yamaguchi

  Objectives To examine the association between the number of visits to the emergency department (ED) by children for night-time headaches and exposure to multifaceted factors, such as meteorological conditions and air pollution. Design We conducted a clinical observational time-series analysis study. Setting We reviewed consecutive patients younger than 16 years of age at the primary ED centre in Kobe city, Japan, during the night shift (19:30–7:00 hours) between 1 January 2011 and 31 December 2019. Participants In total, 265 191 children visited the ED; 822 presented with headache during the study period. Primary outcome measures We investigated the effects of meteorological factors and air pollutants by multivariate analysis of Poisson regression estimates. A subanalysis included the relationship between the number of patients with night-time headaches and the above factors by sex. Furthermore, the effect of typhoon landing on patient visits for headache was also analysed. Headache was not classified because examinations were performed by general paediatricians (non-specialists). Results The number of patients with night-time headaches displayed distinct seasonal changes, with peaks during the summer. Multivariate analysis of Poisson regression estimates revealed a significant positive relationship between the number of patients for headache and mean temperature. Subanalysis by sex indicated a positive relationship between the number of patients with headache and mean temperature in both sexes; however, it was significant only for females. No relationship was found between the number of patients with headache and air pollution. There was no change in the number of patients for night-time headaches 3 days before and after typhoon landing. Conclusions High temperature is the main factor for visiting ED for night-time headaches among children in Kobe city. Our results suggest that preventive measures against night-time headaches may be possible by reducing time spent outside during summer.

  Apr. 2021, BMJ Open, 11(4) (4), e046520, English

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


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

  Hiroshi Yamaguchi

  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.

  Feb. 2021, Brain & development

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• 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


• 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

  Elsevier {BV}, Jan. 2021, Cytokine, 137, 155324 - 155324, English

  Scientific journal


• 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

  Elsevier {BV}, Dec. 2020, Brain and Development, English

  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, K, ai 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, zero and three splicing variants, and zero and two 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. CONCLUSIONS: 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, Dec. 2020, Pediatrics International, 63(8) (8), 918 - 922, English, International magazine

  Scientific journal


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

  Hiroshi Yamaguchi

  ObjectiveTo 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.DesignRetrospective longitudinal population-based cohort study.SettingKobe City public health center, Kobe, Japan, from 2010 to 2018.ParticipantsChildren who underwent a medical check-up at 3 years of age.MethodsInformation 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.ResultsThe 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.ConclusionsAlthough 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.

  Sep. 2020, BMJ Open, English

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• 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


• Thiamylal anaesthetic therapy for febrile refractory status epilepticus in children

  Hiroshi Yamaguchi

  May 2020, Seizure, English

  Scientific journal


• Prolonged Febrile Myoclonus in an Infant

  Hiroshi Yamaguchi

  AbstractFebrile myoclonus is considered a benign symptom of myoclonic jerks associated with fever. Although cases have been sporadically reported, it is a poorly characterized phenomenon. In this study, we reported the case of a previously healthy 20-month-old boy who developed atypically prolonged myoclonic jerks, appearing more than 100 times over a period of 3 days. Blood cultures were negative, and his electroencephalogram was normal. The patient was discharged with a diagnosis of febrile myoclonus and was followed up for several months. His brain magnetic resonance imaging and brain development were also normal. Physicians must understand the benign clinical course of febrile myoclonus in order to avoid unnecessary procedures such as lumbar puncture.

  Mar. 2020, Journal of Pediatric Epilepsy, English

  Scientific journal


• Improvement of opsoclonus after congenital cataract surgery in an infant

  Hiroshi Yamaguchi

  Jan. 2020, Pediatrics International, English

  Scientific journal


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

  Hiroshi Yamaguchi

  Jan. 2020, Journal of Infection and Chemotherapy, English

  Scientific journal


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

  Hiroshi Yamaguchi

  Jan. 2020, Journal of the Neurological Sciences, English

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Pyogenic sacroiliitis caused by pneumococcal serotype 16F in a child

  Hiroshi Yamaguchi

  Dec. 2019, Pediatrics International, English

  Scientific journal


• Detailed clinical course of fatal acute encephalopathy in children

  Hiroshi Yamaguchi

  Sep. 2019, Brain and Development, English

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Empyema necessitatis due to Pseudomonas aeruginosa in a child with cerebral palsy

  Hiroshi Yamaguchi

  Jun. 2019, Journal of Infection and Public Health, English

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Reply to the comments on “Spontaneous spinal epidural hematoma mimicking Guillain-Barre Syndrome”

  Hiroshi Yamaguchi

  Elsevier {BV}, Jun. 2019, Brain and Development, English

  Scientific journal


• Clinical time course of pediatric acute disseminated encephalomyelitis

  Hiroshi Yamaguchi

  Jun. 2019, Brain and Development, English

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


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

  Hiroshi Yamaguchi

  Apr. 2019, Pediatric Neurology, English

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Escherichia coli O157 Enterocolitis Followed by Non-diarrheagenic Escherichia coli Bacteremia

  Hiroshi Yamaguchi

  Mar. 2019, The Indian Journal of Pediatrics, English

  Scientific journal


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

  Hiroshi Yamaguchi

  Mar. 2019, Brain and Development, English

  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

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Acute focal bacterial nephritis characterized by acute encephalopathy with biphasic seizures and late reduced diffusion

  Hiroshi Yamaguchi, Hiroaki Nagase, Yusuke Ito, Natsuki Matsunoshita, Makoto Mizutani, Takeshi Matsushige, Yusuke Ishida, Daisaku Toyoshima, Masashi Kasai, Hiroshi Kurosawa, Azusa Maruyama, Kazumoto Iijima

  Elsevier {BV}, Nov. 2018, Journal of Infection and Chemotherapy, 24(11) (11), 932 - 935

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Fosphenytoin vs. continuous midazolam for pediatric febrile status epilepticus

  Hiroshi Yamaguchi

  Elsevier {BV}, Nov. 2018, Brain and Development, 40(10) (10), 884 - 890, English

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Acute encephalopathy with biphasic seizures and late reduced diffusion accompanied by Takotsubo cardiomyopathy

  Hiroshi Yamaguchi, Hiroaki Nagase, Shinobu Yoshida, Shoichi Tokumoto, Ken Hayashi, Daisaku Toyoshima, Hiroshi Kurosawa, Toshikatsu Tanaka, Azusa Maruyama, Kazumoto Iijima

  Elsevier {BV}, Oct. 2018, Brain and Development

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Infective Endocarditis Due to Streptococcus gallolyticus subsp. pasteurianus with Pulmonary Vein Obstruction

  Hiroyuki Nagao, Hiroshi Yamaguchi, Yusuke Ito, Masashi Kasai

  Springer Nature America, Inc, Sep. 2018, The Indian Journal of Pediatrics

  Scientific journal


• Non-typeable Haemophilus influenzae purulent pericarditis in a healthy child

  Koji Kanno, Hiroshi Yamaguchi, Naoko Imuta, Junichiro Nishi, Masashi Kasai

  Wiley, Sep. 2018, Pediatrics International, 60(9) (9), 886 - 887

  Scientific journal


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

  Hiroshi Yamaguchi

  Elsevier {BV}, Aug. 2018, Brain and Development, 40(7) (7), 552 - 557, English

  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

  Elsevier {BV}, Mar. 2018, Epilepsy & Behavior, 80, 280 - 284

  Scientific journal


• 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

  2018, The Journal of Pediatrics, 207, 213 - +, English

  Scientific journal


• Difficult Renal Pathological Classification in a Case of Pediatric Nephrotic Syndrome

  Hiroshi Yamaguchi, Atsutoshi Shiratori, Taku Nakagawa, Kyoko Kanda, Shigeo Hara, Norishige Yoshikawa, Ryojiro Tanaka

  Nov. 2017, Case Reports in Nephrology and Dialysis, 7(3) (3), 161 - 166, English

  Scientific journal

  Link to Kobe Univ. Repository (Kernel)


• Polymicrobial bloodstream infection in an immunocompetent child

  Akane Ono, Hiroshi Yamaguchi, Yusuke Ito, Masashi Kasai

  Wiley, Aug. 2017, Pediatrics International, 59(10) (10), 1102 - 1103

  Scientific journal


• Bilateral blepharoptosis in a juvenile

  Hiroshi Yamaguchi, Tsukasa Tanaka, Daisaku Toyoshima, Azusa Maruyama, Akihiro Ichinose, Hiroaki Nagase

  Elsevier {BV}, May 2017, Brain and Development, 39(5) (5), 452 - 454

  Scientific journal


• Clinically Mild Encephalitis/Encephalopathy With a Reversible Splenial Lesion Accompanied by Epstein-Barr Virus Hemophagocytic Lymphohistiocytosis

  Hiroshi Yamaguchi, Toshiaki Ishida, Takehito Yokoi, Tsukasa Tanaka, Azusa Maruyama, Hiroaki Nagase, Daiichiro Hasegawa, Ken-Ichi Imadome, Hiroki Takeda, Yoshiyuki Kosaka, Yoshiyuki Uetani

  Ovid Technologies (Wolters Kluwer Health), Mar. 2017, Journal of Pediatric Hematology/Oncology, 39(2) (2), e92 - e96

  Scientific journal


• Two patients with PNKP mutations presenting with microcephaly, seizure, and oculomotor apraxia

  M. Taniguchi-Ikeda, N. Morisada, H. Inagaki, Y. Ouchi, Y. Takami, M. Tachikawa, W. Satake, K. Kobayashi, S. Tsuneishi, S. Takada, H. Yamaguchi, H. Nagase, K. Nozu, N. Okamoto, H. Nishio, T. Toda, I. Morioka, H. Wada, H. Kurahashi, K. Iijima

  Wiley, 2017, Clinical Genetics, 93(4) (4), 931 - 933

  Scientific journal


• Extra-gastrointestinal anisakidosis caused by Pseudoterranova azarasi manifesting as strangulated inguinal hernia

  Akari Mitsuboshi, Hiroshi Yamaguchi, Yusuke Ito, Tetsushi Mizuno, Masaharu Tokoro, Masashi Kasai

  Elsevier {BV}, 2017, Parasitology International, 66(6) (6), 810 - 812

  Scientific journal


• Harlequin Syndrome

  Hiroshi Yamaguchi, Akiko Yokoi, Katsunori Kamimura, Yusuke Ishida, Daisaku Toyoshima, Azusa Maruyama

  Springer Nature, 2017, The Indian Journal of Pediatrics, 85(8) (8), 700 - 701

  Scientific journal


• Pediatrician role in public health: Domestic animal-related injury alerts for neonates

  Hiroshi Yamaguchi, Masashi Kasai, Eru Kozuki, Yusuke Ishida, Hiroki Takeda, Yoshiyuki Uetani

  Wiley, Sep. 2016, Pediatrics International, 58(9) (9), 954 - 955

  Scientific journal


• Decompressive craniectomy 116 h after malignant middle cerebral artery infarction

  Hiroshi Yamaguchi, Kazuki Yamamoto, Nobuyuki Akutsu, Mayumi Kusumoto, Shinsuke Kajihara, Yoshimichi Yamaguchi, Hiroki Takeda, Atsushi Kawamura, Yoshiyuki Uetani

  Wiley, Feb. 2016, Pediatrics International, 58(7) (7), 622 - 624

  Scientific journal


• Very Low Birth Weight Monochorionic Diamniotic Twins as a Risk Factor for Symptomatic Patent Ductus Arteriosus

  Hiroshi Yamaguchi, Keiko Wada, Miho Nagasawa, Takefumi Kikusui, Hitomi Sakai, Masami Mizobuchi, Seiji Yoshimoto, Hideto Nakao

  S. Karger {AG}, 2016, Neonatology, 109(3) (3), 228 - 234

  Scientific journal


• Septic Encephalopathy Characterized by Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion and Early Nonconvulsive Status Epilepticus

  Hiroshi Yamaguchi, Tsukasa Tanaka, Azusa Maruyama, Hiroaki Nagase

  Hindawi Limited, 2016, Case Reports in Neurological Medicine, 2016, 1 - 5

  Scientific journal


• Down Syndrome with Patent Ductus Venosus and Hepato-Biliary-Pancreatic Abnormalities

  Hiroshi Yamaguchi, Kiyotaka Kosugiyama, Shohei Honda, Okada Tadao, Akinobu Taketomi, Seido Iwata

  Springer Nature, Jun. 2015, The Indian Journal of Pediatrics, 83(1) (1), 78 - 80

  Scientific journal


• Streptococcus pulmonary empyema after varicella infection in a serologically immunocompetent boy

  Hiroshi Yamaguchi, Kiyoshi Nagumo, Daisuke Sasaki, Hayato Aoyagi, Hiroaki Kato, Yoshiaki Narita, Takuya Tamura, Kiyotaka Kosugiyama, Taiji Nakashima, Yoshikazu Kinugawa

  Wiley, Aug. 2014, Pediatrics International, 56(4) (4), 618 - 621

  Scientific journal


• Prolonged incubation period in neonatal Pasteurella multocida meningitis and bacteremia

  Hiroshi Yamaguchi, Takuya Tamura, Michiko Abe, Shigetoshi Ogiwara, Shuji Sai, Kiyotaka Kosugiyama, Akemi Sugihara, Kiyoshi Nagumo, Seido Iwata, Yoshikazu Kinugawa

  Wiley, 2014, Pediatrics International, 56(6) (6), e79 - e81

  Scientific journal


• Life-threatening QT prolongation in a boy with attention-deficit/hyperactivity disorder on atomoxetine

  Hiroshi Yamaguchi, Kiyoshi Nagumo, Taiji Nakashima, Yoshikazu Kinugawa, Satoru Kumaki

  Springer Nature, Nov. 2013, European Journal of Pediatrics, 173(12) (12), 1631 - 1634

  Scientific journal


• A case report of the anti-glomerular basement membrane glomerulonephritis with mesangial IgA deposition

  Hiroshi Yamaguchi, Hideki Takizawa, Yayoi Ogawa, Tamaki Takada, Izumi Yamaji, Nobuyuki Ura

  Springer Nature, Aug. 2012, CEN Case Reports, 2(1) (1), 6 - 10

  Scientific journal


• A novel coenzyme A:diacylglycerol acyltransferase 1 inhibitor stimulates lipid metabolism in muscle and lowers weight in animal models of obesity

  Toshihiro Yamamoto, Hiroshi Yamaguchi, Hiroshi Miki, Shuji Kitamura, Yoshihisa Nakada, Thomas D. Aicher, Scott A. Pratt, Koki Kato

  Elsevier {BV}, Jan. 2011, European Journal of Pharmacology, 650(2-3) (2-3), 663 - 672

  Scientific journal


• Coenzyme A: Diacylglycerol acyltransferase 1 inhibitor ameliorates obesity, liver steatosis, and lipid metabolism abnormality in KKAy mice fed high-fat or high-carbohydrate diets

  Toshihiro Yamamoto, Hiroshi Yamaguchi, Hiroshi Miki, Mitsuyuki Shimada, Yoshihisa Nakada, Masaki Ogino, Kouhei Asano, Kazuko Aoki, Norikazu Tamura, Minori Masago, Koki Kato

  Elsevier {BV}, Aug. 2010, European Journal of Pharmacology, 640(1-3) (1-3), 243 - 249

  Scientific journal


• Novel acyl coenzyme A (CoA): Diacylglycerol acyltransferase-1 inhibitors: Synthesis and biological activities of diacylethylenediamine derivatives

  Yoshihisa Nakada, Thomas D. Aicher, Yvan Le Huerou, Timothy Turner, Scott A. Pratt, Stephen S. Gonzales, Steve A. Boyd, Hiroshi Miki, Toshihiro Yamamoto, Hiroshi Yamaguchi, Koki Kato, Shuji Kitamura

  Elsevier {BV}, Apr. 2010, Bioorganic & Medicinal Chemistry, 18(7) (7), 2785 - 2795

  Scientific journal


• Social stress decreases marking behavior independently of testosterone in Mongolian gerbils

  Hiroshi Yamaguchi, Takefumi Kikusui, Yukari Takeuchi, Hiroyuki Yoshimura, Yuji Mori

  Elsevier {BV}, May 2005, Hormones and Behavior, 47(5) (5), 549 - 555

  Scientific journal

• 便中のフィルムアレイ検査が診療方針決定に有用であったエルシニア腸炎の2例

  酒井菜々花, 朝貝芳貴, 大橋浩基, 末宗和樹, 斉賀佳穂, 曽根原晶子, 鮫島智大, 花房宏昭, 老川静香, 南部静紀, 徳元翔一, 山口宏, 坊亮輔, 永瀬裕朗, 野津寛大

  2025, 日本小児科学会雑誌, 129(6) (6)


• 摂食障害患者の体重の回復と骨密度の検討

  伊藤立人, 冨岡和美, 川村葵, 曽根原晶子, 京野由紀, 鮫島智大, 花房宏昭, 老川静香, 南部静紀, 徳元翔一, 山口宏, 坊亮輔, 野津寛大, 永瀬裕朗

  2025, 日本小児科学会雑誌, 129(6) (6)


• 幅広い血縁者の解析がバリアントの評価に有用であったARXによる発達性てんかん性脳症の1男児例

  花房宏昭, 花房宏昭, 山口宏, 澤田優貴, 田中敬子, 老川静香, 坊亮輔, 中川卓, 森貞直哉, 野津寛大, 永瀬裕朗

  2025, 日本小児遺伝学会学術集会プログラム・抄録集, 47th


• Five cases of Dravet syndrome responding to low-dose fenfluramine

  上田拓耶, 西山将広, 相馬健人, 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

  2024, てんかん研究, 42(2) (2)


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

  京野由紀, 西山将広, 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

  2023, 小児保健研究(Web), 82(Suppl) (Suppl)


• 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


• 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


• 有熱性てんかん重積・急性脳症に単一遺伝子疾患は存在するのか?

  山口宏, 花房宏昭, 徳元翔一, 冨岡和美, 西山将広, 丸山あずさ, 森貞直哉, 野津寛大, 永瀬裕昭

  2022, 日本人類遺伝学会大会(CD-ROM), 67th


• 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


• 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


• 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


• 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


• 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


• 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


• 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


• 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


• 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


• 結節性硬化症の定期検査により早期発見に至った無症候性巨細胞性星細胞腫瘍(SEGA)の1例

  豊嶋 大作, 徳元 翔一, 山口 宏, 丸山 あずさ, 永瀬 裕朗

  (公社)日本小児科学会, Mar. 2019, 日本小児科学会雑誌, 123(3) (3), 629 - 629, 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


• ギランバレー症候群と鑑別に難渋した6歳女児の脊髄硬膜外血腫

  近藤 亜耶, 山口 宏, 石田 悠介, 豊嶋 大作, 丸山 あずさ, 黒澤 寛史, 河村 淳史, 田中 亮二郎, 上谷 良行

  (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


• 一過性意識消失発作を主訴に小児神経外来を受診した101例の最終診断

  豊嶋大作, 徳元翔一, 山口宏, 石田悠介, 田中司, 小川禎治, 田中敏克, 丸山あずさ, 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), 408 - 408, Japanese


• 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


• 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


• 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


• 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


• 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

• 持続脳波による急性脳症のNCSの病態と神経傷害機構の解明

  山口 宏

  日本学術振興会, 科学研究費助成事業, 若手研究, 神戸大学, 01 Apr. 2023 - 31 Mar. 2026

  急性脳症は感染を契機に発症する小児救急領域における最も重篤な中枢神経疾患の一つである。急性脳症の病態は代謝異常、全身性炎症反応、けいれん性発作による興奮毒性などが考えられているが未解明である。本研究では当研究グループ独自の救急外来から集中治療室までの連続する持続脳波（cEEG）データ、患者検体、診療情報を用いて、急性脳症に特異的な非けいれん性発作（NCS）を含む脳波異常の特徴を解析し、脳波異常と神経障害マーカーや生体ストレス反応マーカーとの関連を調べることを目的とする。令和5年度は救急外来で意識障害を呈する小児に対する詳細なcEEGの解析を行った。熱性発作（FS），急性脳症／脳炎（AE/AES），てんかん，発熱を伴うてんかんに分類し、NCSを伴う56例を抽出してそのパターンを記述した。ｃEEGパターンはACNS Standardized Critical Care EEG Terminology2021に基づいて行った。Main term1は各群で差を認めなかった。Main term2ではFSとAE/AESで律動性デルタ活動が多く，てんかんと発熱を伴うてんかんでは棘徐波が多かった。FSの約25%のみに律動性シータ活動を認め、この活動が予後良好に関連する可能性を示唆した。


• 救急外来からの連続的な脳波データを活用した非けいれん性発作の神経傷害機構の解明

  山口 宏

  日本学術振興会, 科学研究費助成事業, 若手研究, 神戸大学, 01 Apr. 2020 - 31 Mar. 2023

  今年度は、主にMATLABを用いた脳波解析手法の習得を行った。また研究対象である兵庫県立こども病院の救急外来もしくは集中治療科で記録された脳波データを神戸大学に正式に蓄積できるように手続きを行った（脳波コーパスの構築と脳波データの収集）。それにより、匿名化された臨床データと照合した脳波蓄積が確立し、より迅速に脳波解析ができる道筋を得た。初回脳波解析データとして50例の非けいれん性発作の脳波データが得られ(平均年齢：3.8歳、男女比：22:28)、現在詳細に解析中である。