Language：Japanese   Publisher：(一社)日本小児神経学会  

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Language：Japanese   Publisher：(一社)日本小児神経学会  

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Language：English   Publishing type：Research paper (scientific journal)  

In addition to genetic factors, environmental factors play a role in the pathogenesis of attention deficit/hyperactivity disorder (ADHD). This study used Lister hooded rats (LHRs) as ADHD model animals to evaluate the effects of environmental factors. Male LHR pups were kept in four rearing conditions from postnatal day 23 (4 rats in a standard cage; 12 rats in a large flat cage; and 4 or 12 rats in an enriched environment [EE]) until 9 weeks of age. EE rearing but not rearing in a large flat cage decreased the activity of LHRs in the open field test that was conducted for 7 consecutive days. In the drop test, most rats reared in an EE remained on a disk at a height, whereas most rats reared in a standard cage fell off. RNA sequencing revealed that the immediate-early gene expression in the medial prefrontal cortex of LHRs reared in an EE was reduced. cFos-expressing neurons were reduced in number in LHRs reared in an EE. These results suggest that growing in an EE improves ADHD-like behaviors and that said improvement is due to the suppression of neuronal activity in the mPFC.

DOI： 10.3390/cells11223649

PubMed

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Language：Japanese   Publisher：(一社)日本小児神経学会  

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.3390/ijns8040052

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Language：English  

Stevens-Johnson syndrome (SJS) with respiratory distress can lead to fatal outcomes. However, there are a few reports of drug-induced lung injury with diffuse alveolar damage caused by acetaminophen, the most severe type. Here, we describe a fatal case of acetaminophen-induced SJS in a child with irreversible lung lesions.

DOI： 10.1002/ccr3.6294

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Publishing type：Research paper (scientific journal)   Publisher：The Japan Epilepsy Society  

DOI： 10.3805/eands.14.71

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Publishing type：Research paper (scientific journal)   Publisher：The Japan Epilepsy Society  

DOI： 10.3805/eands.14.51

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Language：Japanese   Publisher：(一社)日本小児神経学会  

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Language：Japanese   Publisher：(一社)日本てんかん学会  

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Language：Japanese   Publisher：(一社)日本てんかん学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：日本小児感染症学会  

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Language：Japanese   Publisher：(一社)日本透析医学会  

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Language：English   Publishing type：Research paper (scientific journal)  

Appropriate animal models are necessary to determine the molecular and cellular mechanisms underlying attention-deficit/hyperactivity disorder (ADHD). This study used a battery of behavioral tests to compare Lister hooded rats (LHRs), an old outbred strain frequently used for autistic epilepsy research, with Wistar rats and spontaneously hypertensive rats (SHRs), a commonly used ADHD model. The open field, elevated plus maze, light/dark box, and drop tests demonstrated that LHRs were the most hyperactive animals and displayed the most inattentive- and impulsive-like behaviors, which are characteristics of ADHD. The radial arm maze, social interaction, and Morris water maze tests showed that LHRs did not display deficits characteristic of autism or intellectual disability. Although LHRs did not show different monoamine contents, the mRNA expression levels of various genes linked to ADHD (Cdh13, Drd5, Foxp2, Maoa, Sema6d, Slc9a9, and St3gal3) and tyrosine hydroxylase protein expression levels were lower in the prefrontal cortex of LHRs compared with that of Wistar rats or SHRs. c-Fos, synapsin I, and tau protein expression levels in the prelimbic region of the medial prefrontal cortex were also increased in LHRs compared with Wistar rats. Atomoxetine and guanfacine, commonly used non-stimulant treatments for ADHD, ameliorated ADHD-like behaviors in LHRs. These results suggest that LHRs can serve as a better ADHD model to develop novel pharmacological interventions.

DOI： 10.1016/j.neuint.2020.104857

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1111/ped.14247

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Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Spinal muscular atrophy (SMA) is an inherited neuromuscular disorder associated with spinal motor neuron loss and characterized by generalized muscle weakness. Only a few reports exist on SMA epidemiology in Japan. Additionally, nusinersen recently became available as a treatment for this condition. We estimated the prevalence of each type of SMA on Shikoku, Japan's fourth-largest major island. METHODS: We sent a questionnaire to all 131 hospitals in Shikoku that have pediatrics or neurology departments from March to September 2019, asking whether each hospital had SMA patients at that time. If so, we sent a second questionnaire to obtain more detailed information on the clinical data and treatment of each patient. RESULTS: A total of 117 hospitals (89.3%) responded to our first questionnaire, and 21 SMA patients were reported, 16 of whom had homozygous deletion of SMN1. Of the 21, nine had SMA type 1, five were type 2, five were type 3, one was type 4, and one was unidentified. The estimated prevalence for all instances of SMA and 5q-SMA was 0.56 and 0.43 per 100,000 people, respectively. Thirteen patients had received nusinersen therapy. Its outcomes varied from no obvious effects and being unable to sit to being able to sit independently. CONCLUSION: Our data showed the prevalence of SMA types 2 and 3 was relatively low on Shikoku compared with previous reports from other countries, suggesting delayed diagnosis may affect the results. Remaining motor function may be one predicting factor. Greater awareness of SMA among clinicians and patients seems necessary for more accurate epidemiological studies.

DOI： 10.1016/j.braindev.2020.05.004

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Language：Japanese   Publisher：(株)医学書院  

＜文献概要＞愛媛大学医学部附属病院は2015年4月に子どものこころセンターを開設した。本調査では,開設後2年間の初診患者の動向を明らかにすることを目的に,後方視的に調査を行った。初診患者は1〜15歳の292例(男性192例,女性100例)であった。約9割が紹介受診であり,最も多い紹介元は院外の小児科(49.7%)であった。ICD-10に基づいて行った診断では,F8が最も多く(43.6%),F4,F9と続いた。2017年3月現在の転帰は,治療継続中が150例(51.4%)で,その3/4が発達障害圏の患者であった。センター化したことで,初診患者数が増加し,低年齢化した。また,小児科からの紹介が増加し,発達障害圏を中心に患者が集約されていた。しかし,地域と当センターの連携の不十分さなどの課題も明らかとなり,今後のセンターのあり方について模索中である。

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Language：English   Publishing type：Research paper (scientific journal)  

INTRODUCTION: With improved treatments, patients with Duchenne muscular dystrophy (DMD) can survive far beyond adolescence. However, advanced-stage DMD patients are at risk of developing renal dysfunction. In this study, long-term renal function outcomes and associated risk factors in advanced stage DMD were analyzed. METHODS: Fifty-one patients were classified into three different age groups (<20, 20-29, and ≥30 years of age), and cystatin C (CysC) levels were compared among groups. RESULTS: Median serum CysC levels were 0.74 mg/L, 0.63 mg/L, and 0.76 mg/L in the age groups of <20, 20-29, and ≥30 years, respectively (P = .003). Five of the nine patients in the ≥30 years age group showed elevated serum CysC and decreased cardiac function compared with the other four in the group (P = .014). DISCUSSION: Our results indicate an association between cardiac and renal dysfunction in patients with advanced-stage DMD.

DOI： 10.1002/mus.26757

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Language：English   Publishing type：Research paper (scientific journal)  

AIM: Several studies have reported that internet addiction (IA) is more prevalent in adolescents with autism spectrum disorder (ASD). However, the characteristics of ASD adolescents with IA are unclear. The objective of this study was to investigate the prevalence of IA in ASD adolescents, and compare the characteristics between the IA and the non-IA groups in adolescents with ASD. METHODS: The study included 55 participants who were outpatients at Ehime University Hospital and Ehime Rehabilitation Center for Children in Japan, aged 10-19 years, diagnosed with ASD. Patients and their parents answered several questionnaires including the Young's Internet Addiction Test (IAT), Strengths and Difficulties Questionnaire (SDQ), Autism Spectrum Quotient (AQ), and Attention Deficit Hyperactivity Disorder Rating Scale-IV (ADHD-RS). RESULTS: Based on the total IAT score, 25 out of 55 participants were classified as having IA. Although there were no significant differences in AQ and Intelligence Quotient, the higher scores of ADHD symptoms in SDQ and ADHD-RS were observed in the IA group than the non-IA group. The IA group used portable games more often than the non-IA group. CONCLUSION: The ADHD symptoms were strongly associated with IA in ASD adolescents. More intensive prevention and intervention for IA are needed especially for the ASD adolescents with ADHD symptoms.

DOI： 10.1016/j.ridd.2019.03.002

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Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Spinal muscular atrophy (SMA) is an autosomal recessive disorder caused by homozygous mutations in the SMN1 gene. SMA has long been known to be the most common genetic cause of infant mortality. However, there have been no reports on the epidemiology of infantile SMA (types 1 and 2) based on genetic testing in Japan. In this study, we estimated the incidence of infantile SMA on Shikoku Island, which is a main island of Japan and consists of four prefectures: Ehime, Kagawa, Tokushima and Kochi. METHODS: A questionnaire was sent to 91 hospitals on Shikoku Island to investigate the number of SMA infants born from 2011 to 2015. A second questionnaire was then sent to confirm the diagnoses of SMA based on clinical and genetic features. RESULTS: Responses were received from all of the hospitals, and four patients were diagnosed with infantile SMA among 147,950 live births. We estimated the incidence of infantile SMA patients as 2.7 per 100,000 live births (95% confidence interval, 0.1-5.4). A comparison of the four prefectures indicated that the incidence of infantile SMA was significantly higher in Ehime Prefecture than in the other three prefectures; 5.6 per 100,000 live births (95% confidence interval, -0.7 to 11.9) in Ehime Prefecture and 1.1 per 100,000 live births (95% confidence interval, -1.0 to 3.1) in the other prefectures. CONCLUSION: We estimated the incidence of infantile SMA in an isolated area of Japan. For more precise determination of the incidence of infantile SMA, further studies that include neonatal screening will be needed.

DOI： 10.1016/j.braindev.2018.07.016

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Language：English  

Background: Nonocclusive mesenteric ischemia (NOMI) defines acute mesenteric ischemia without occlusion of the mesenteric arteries. The most common cause of NOMI is vasoconstriction or vasospasm of a mesenteric artery. NOMI generally affects patients >50 years of age, and few cases have been reported in children. Case Presentation: A 15-year-old boy with severe neurodevelopmental disability developed sudden-onset fever, abdominal distention, and dyspnea. Laboratory and radiological findings indicated acute intestinal obstruction and prerenal failure. He developed transient cardiopulmonary arrest and hypovolemic shock. Emergent laparotomy was performed, which revealed segmentally necrotic intestine from the jejunum to the ascending colon with pulsation of peripheral intestinal arteries, leading to a diagnosis of NOMI. The necrotic intestine was resected, and stomas were created. He was discharged on postoperative day 334 with short bowel syndrome as a complication. Conclusions: NOMI should be considered a differential diagnosis for intestinal symptoms with severe general conditions in both adults and children with underlying disease. Immediate surgical exploration is essential with NOMI to save a patient's life.

DOI： 10.1155/2019/5354074

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Language：English  

We evaluated the efficacy of linezolid treatment in 6 children with health care-associated meningitis or ventriculitis (HCAMV) caused by gram-positive cocci. All children were diagnosed and treated at the Ehime University Hospital between January 2010 and December 2017. Of these, 5 were treated with linezolid as an empirical therapy. In these 5 patients, vancomycin was initially used but was changed to linezolid because of cerebrospinal fluid (CSF) culture positivity (n = 3) and a high minimum inhibitory concentration of vancomycin (n = 2). The most common HCAMV pathogens were methicillin-resistant coagulase-negative staphylococci (n = 3). In 3 patients, vancomycin concentration was low in CSF but reached the target concentration in serum, while linezolid concentration was high in both CSF and serum. HCAMV treatment using antimicrobial agents with poor CSF penetration may increase the likelihood of therapy failure. Linezolid is more susceptible as the first-line treatment for HCAMV compared with vancomycin.

DOI： 10.1177/0009922818803399

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Language：English  

Streptococcus pyogenes (also referred to as group A streptococci, GAS) causes severe invasive diseases such as bacteremia, necrotizing fasciitis, pneumonia, osteomyelitis, septic arthritis, and toxic shock syndrome in children. However, there are only a few reports on pleural empyema caused by GAS in children. Here, we report the case of a 4-year-old boy who presented with pleural empyema due to GAS after influenza A virus infection. With intravenous antibiotic administration and continuous chest-tube drainage, followed by video-assisted thoracoscopic surgery, his condition improved. During the clinical course, cytokines induced in response to the influenza virus, especially IL-1β and IL-10, were elevated 1 week after influenza A infection, but these decreased as the symptoms improved. Reportedly, the IL-10 production increases during influenza virus-bacteria superinfection. These observations suggest that the immunological mechanisms induced by the influenza virus can play an important role in influencing the susceptibility to secondary bacterial infections, such as GAS, in children.

DOI： 10.1155/2018/4509847

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Language：English  

OBJECTIVE: Stiripentol (STP), valproate (VPA) and topiramate (TPM) are reported to have efficacy for Dravet syndrome. In this study, we sought to elucidate the mechanisms underlying the increased serum VPA concentrations following STP adjunctive therapy in patients with Dravet syndrome. METHODS: Twenty-eight patients with Dravet syndrome (age range, 1-35 years) undergoing combination therapy with VPA and STP were included in this study. We evaluated VPA and clobazam (CLB) serum concentrations before and after add-on STP. We also investigated potential factors impacting VPA metabolism during add-on STP therapy, including add-on TPM and CYP2C9 and CYP2C19 gene polymorphisms. The effect of STP on the metabolism of concomitantly administered CLB was also investigated. RESULTS: Add-on STP was significantly associated with the serum concentration-to-dose (CD) ratio of VPA. Two patients, who were concomitantly treated with TPM, developed severe anorexia and thrombocytopenia because of marked increases in serum VPA concentrations. Further analysis revealed that the rate of increase in the VPA CD ratio was positively correlated with TPM dose. In patients not receiving TPM, STP enhanced the rate of increase in the VPA CD ratio to a significantly greater extent in CYP2C19 extensive metabolizers than in CYP2C19 poor metabolizers. Add-on STP was also associated with significant increases in CLB and N-desmethyl-CLB serum concentrations. CONCLUSION: Our findings suggest that serum VPA concentrations should be carefully monitored during STP adjunctive therapy, particularly in patients receiving concomitant TPM therapy.

DOI： 10.1016/j.eplepsyres.2016.12.014

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Language：Japanese   Publisher：(一社)日本てんかん学会  

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Language：Japanese   Publisher：(一社)日本てんかん学会  

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Language：Japanese   Publisher：日本病態生理学会  

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Language：Japanese   Publisher：(株)東京医学社  

5ヵ月男児。眼球を上転し、頭部を前屈させる発作を主訴とした。epileptic spasm発作出現から2日後に紹介入院し、発作間欠期脳波にてhypsarrhythmiaを認めたが、発症までの精神運動発達は正常で、知的退行はみられなかった。頭部CT検査や血液検査で原因となる疾患は認めず、潜因性West症候群と診断して発症3日目より連日ACTH 0.0125mg/kg/日の筋肉注射を開始した結果、開始3日後より脳波の改善がみられ、開始10日後に発作は消失し、開始12日後には脳波が正常化した。ACTHは2週間連続投与した後、減量中止し、開始12日目よりゾニサミド内服を開始したが、以後も発作はみられず、5歳時点の知的予後は良好である。潜因性West症候群の治療方針として、早期発見と早期のACTH療法開始が考慮されうることが示唆された。

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Language：Japanese   Publisher：(一社)日本生理学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(一社)日本小児神経学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：日本病態生理学会  

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Language：Japanese   Publisher：(一社)日本てんかん学会  

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Language：Japanese   Publisher：(一社)日本てんかん学会  

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Language：Japanese   Publisher：(一社)日本小児神経学会  

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Language：Japanese   Publisher：(公社)日本精神神経学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(一社)日本小児神経学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(一社)日本てんかん学会  

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Language：Japanese   Publisher：(一社)日本小児神経学会  

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Language：Japanese   Publisher：(一社)日本小児救急医学会  

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Language：Japanese   Publisher：(一社)日本小児神経学会  

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Language：Japanese   Publisher：(一社)日本小児神経学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：The Japanese Society of Pediatric Hematology / Oncology  

<p>Despite the improved outcome of Ph-positive acute lymphoblastic leukemia (Ph+ ALL) with tyrosine kinase inhibitors in children, side effects and complications of treatment are still major concerns. In this report, we describe the case of a 13-year-old boy who was diagnosed as having Ph+ ALL and treated with chemotherapy including imatinib. He developed febrile neutropenia on day 14 after the re-induction therapy. An antibiotic agent was immediately administered to the patient, but septic shock and, following acute renal failure, intestinal edema and acute respiratory distress syndrome progressed. He died on day 62. Autopsy findings showed that the cause of death was multiple organ failure from septic shock. <i>E. coli </i>was detected in his blood culture. The mechanism of sepsis was considered as a bacterial translocation from his intestine. These findings show that the immediate use of antibiotics in the case of prolonged febrile neutropenia is recommended, because bacterial translocation will be induced by the imatinib-containing chemotherapy in Ph+ ALL patients.</p>

DOI： 10.11412/jspho.54.15

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(一社)日本小児救急医学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：English   Publisher：(一社)日本小児神経学会  

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Language：Japanese   Publisher：愛媛県公営企業管理局県立病院課  

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Language：Japanese   Publisher：(一社)日本小児神経学会  

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Language：Japanese   Publisher：(一社)日本小児血液・がん学会  

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Language：English   Publisher：(一社)日本小児血液・がん学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

9ヵ月男児。在胎41週5日、体重3818g、Apgar Score 9点で出生、自治体のタンデムマス・スクリーニングでは日齢5の濾紙血液検査でC16-OH高値(三頭酵素欠損症の疑い)が指摘されていた。今回、前日から38℃台の発熱を認め近医の小児科を受診、インフルエンザと診断され帰宅するも、急速に呼吸状態が悪化し心肺停止となった。以後、心肺蘇生を行ないつつ著者らの施設に救急般送されたが、約2時間後に死亡が確認された。本症例では救急般送時の検査の時点から先天性代謝異常症の疑いがあり、死後の急性期における分析では濾紙血のタンデムマス分析でC0の低値が、また血清のタンデムマス分析では長鎖アシルカルニチンの上昇などの所見が認められた。最終的には遺伝子検査によりカルニチンパルミトイルトランスフェラーゼ-II(CPT2)欠損症と確定診断された。尚、本疾患はタンデムマス・スクリーニングで発見可能であるが、診断精度などから二次対象疾患であり自治体によってはタンデムマス検査を受けても見逃している可能性がある。したがって、今後は一次対象疾患への変更ほか、精密検査では血清のアシルカルニチン分析がより重要と考えられる。

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Language：Japanese   Publisher：(一社)日本てんかん学会  

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Language：Japanese   Publisher：日本小児アレルギー学会  

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Language：Japanese   Publisher：日本小児アレルギー学会  

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Language：Japanese   Publisher：(一社)日本小児血液・がん学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(一社)日本てんかん学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：日本小児感染症学会  

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Language：Japanese   Publisher：(一社)日本てんかん学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：日本小児アレルギー学会  

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Language：Japanese   Publisher：(一社)日本小児神経学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：日本小児呼吸器学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：日本小児アレルギー学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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