Supporters' experiences of sensory characteristics of children with profound intellectual and multiple disabilities in after-school daycare centres: A qualitative study.

Aim: To examine how supporters working at after-school daycare centres, who are involved in the lives of children with profound intellectual and multiple disabilities in the community, pay attention to the sensory characteristics of these children and provide support.

Design: A qualitative descriptive design.

Methods: Data were collected through semi-structured interviews with 20 supporters in after-school daycare centres.

Associations Between Quality of Life, Psychosocial Well-being and Health-Related Behaviors Among Adolescents in Chinese, Japanese, Taiwanese, Thai and the Filipino Populations: A Cross-Sectional Survey.

Health-related behaviors during adolescence have lifelong impacts. However, there are unclear areas regarding the associations between health-related quality of life and demographic characteristics, as well as physical and psychosocial indicators. The aim of this study was to examine the associations between quality of life and body weight, sleep outcome, social support by age, and cohabitants, given that income, self-esteem, lifestyle, emotional, social and behavioral problems were taken into account among adolescents in East and Southeast Asia.

Treatment of infantile spasms by pediatric neurologists in Japan.

Objective: To clarify changes in clinical practice for infantile spasms, including West syndrome, in Japan over the past two decades.

Methods: We investigated common treatment strategies for infantile spasms among 157 pediatric neurologists from a designated training facility for pediatric neurology and/or a designated training facility for epilepsy in Japan. A questionnaire was used to investigate use of adrenocorticotropic hormone (ACTH) therapy including daily dose, treatment duration, and tapering off period, and preferred first to fifth-line treatment choices.

[Parents’opinions of the changes in their children’s epilepsy treatment during the transition from childhood to adulthood].

Objective: Patients with childhood-onset epilepsy often need continued epilepsy treatment into adulthood. We investigated parents’ opinions of the changes in their children’s epilepsy treatment during the transition from childhood to adulthood using questionnaires and formulated agendas to build the appropriate medical treatment system for epilepsy. Methods: We distributed questionnaires to parents of patients with epilepsy who were 12 to 18 years old.

Verification of the reliability and validity of a Japanese version of the Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-J).

Objective: A Japanese version of the Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-J) was developed using international guidelines as a QOL scale for childhood epilepsy; its reliability and validity were examined, focusing on Japanese pediatric epilepsy patients applicability.

Methods: A pilot test questionnaire survey was conducted; involving parents of pediatric epilepsy patients aged 4-15 undergoing outpatient treatment. 278 responses were obtained and analyzed.

Prognostic factors for acute encephalopathy with bright tree appearance.

Objective: To determine the prognostic factors for encephalopathy with bright tree appearance (BTA) in the acute phase through retrospective case evaluation.

Methods: We recruited 10 children with encephalopathy who presented with BTA and classified them into 2 groups. Six patients with evident regression and severe psychomotor developmental delay after encephalopathy were included in the severe group, while the remaining 4 patients with mild mental retardation were included in the mild group.

[Cyclophosphamide pulse therapy effective for childhood-onset refractory multiple sclerosis: a case report].

We report a case of a 15-year-old girl with relapsing-remitting multiple sclerosis (MS) who received cyclophosphamide pulse therapy. At the age of 5 years, she displayed symptoms such as headache and unconsciousness after varicella infection as the first episode of MS. She had been treated with methylprednisolone pulse therapy, intravenous immunoglobulin, interferon-beta1b, and azathioprine.

[Effectiveness of a modified parent training of smaller groups and shorter schedules for children with pervasive developmental disorders].

Objective: We have previously reported the modified parent training of smaller groups and shorter schedules (PTSS). In this study, we applied PTSS to the mothers of children with pervasive developmental disorder (PDD) and revealed the new evidence for its effectiveness.

Methods: The participants were 30 mothers of children with PDD aged from 4.

Development and verification of child observation sheet for 5-year-old children.

The aim of the study was to develop a newly devised child observation sheet (COS-5) as a scoring sheet, based on the Childhood Autism Rating Scale (CARS), for use in the developmental evaluation of 5-year-old children, especially focusing on children with autistic features, and to verify its validity. Seventy-six children were studied. The children were recruited among participants of the Japan Children's Cohort Study, a research program implemented by the Research Institute of Science and Technology for Society (RISTEX) from 2004 to 2009.

Ictal high-frequency oscillations on scalp EEG recordings in symptomatic West syndrome.

Objectives: High-frequency oscillations (HFOs) on intracranial electroencephalography (EEG) recordings have been reported to be useful to identify the epileptogenic zone in intractable epilepsy. We investigated whether the ictal HFOs on scalp EEG seen during spasms contributed to identification of the epileptogenic zone in symptomatic West syndrome (S-WS).

Methods: In S-WS, ictal scalp EEGs were recorded during a series of spasms.

Long-term developmental outcome in patients with West syndrome after epilepsy surgery.

It has been hypothesized that early seizure control may prevent children with intractable epileptic spasms (ES) from developmental regression and may contribute to better developmental outcome. The effectiveness of surgery for ES has been reported. We investigated long-term post-operative outcomes of seizure control and development in patients with symptomatic West syndrome (S-WS) who underwent epilepsy surgery.

SLC2A1 gene analysis of Japanese patients with glucose transporter 1 deficiency syndrome.

Glucose transporter 1 deficiency syndrome (Glut1-DS) is a congenital metabolic disorder characterized by refractory seizures with early infantile onset, developmental delay, movement disorders and acquired microcephaly. Glut1-DS is caused by heterozygous abnormalities of the SLC2A1 (Glut1) gene, whose product acts to transport glucose into the brain across the blood-brain barrier. We analyzed the SLC2A1 gene in 12 Japanese Glut1-DS patients who were diagnosed by characteristic clinical symptoms and hypoglycorrhachia as follows: all patients had infantile-onset seizures and mild to severe developmental delay, and ataxia was detected in 11 patients.

Sleep problems in physically disabled children and burden on caregivers.

Aim: The present study was implemented to investigate relationships between sleep problems in physically disabled children and sleep quality and perceived burden of caregivers.

Methods: Subjects comprised 100 caregivers of disabled children, including 96 mothers, 2 fathers and 2 grandmothers. Questionnaires included demographic data for children and caregivers, sleep problems of children, and sleep quality (Pittsburgh sleep quality index (PSQI)) and perceived burden on caregivers (Japanese version of the Zarit Burden Interview (J-ZBI)).

Effectiveness of modified parent training for mothers of children with Pervasive Developmental Disorder on parental confidence and children's behavior.

Aim: This study used parent training (PT), with modifications to smaller groups and shorter schedules (PTSS), for mothers of children with Pervasive Developmental Disorder (PDD). The usefulness of PTSS was evaluated according to the parent's confidence and child's behavior by questionnaire.

Method: PTSS was used on 14 mothers of 14 children with PDD of preschool to elementary school age, and performed in small groups of 3-4 mothers each.

A pilot study on the changes in immunity after ACTH therapy in patients with West syndrome.

Adrenocorticotropic hormone (ACTH) has been the first-line drug for the treatment of West syndrome, although the therapy has various adverse effects. ACTH depresses resistance to a variety of bacterial, viral, protozoal, and fungal agents. The timing of the various vaccinations is delayed after ACTH therapy in Japan, because the immune system is believed to be affected for approximately 6 months.

Management of and prophylaxis against status epilepticus in children with severe myoclonic epilepsy in infancy (SMEI; Dravet syndrome)--a nationwide questionnaire survey in Japan.

Unlabelled: The aim of this study was to establish strategies for prophylaxis against status epilepticus (SE) associated with high fever and for management of ongoing SE in children with severe myoclonic epilepsy in infancy (SMEI).

Methods: The investigation was performed retrospectively using a questionnaire, asking about medications, which was distributed to epilepsy specialists throughout Japan. All respondents were members of the Japan Epilepsy Society (JES) and/or the Japanese Society of Child Neurology (JSCN).

[Effect of carbamazepine on epilepsy with 1p36 deletion syndrome].

The 1p36 deletion syndrome is caused by submicroscopic deletion in the subtelomeric region of chromosome 1. Epilepsy is one of the most important features of the syndrome, in addition to the characteristic facial appearance, cardiac anomaly, dysphagia, deafness, mental retardation and growth delay. We identified three patients with this syndrome and assessed the features of complicated epilepsy.

[Clinical efficacy of shortened ACTH therapy --an individualized method for minimization of adverse effects--Part 1. The short-term outcome].

To minimize adverse effects and to get good efficacy of ACTH therapy against West syndrome, we tried a new 2-steps therapeutic protocol consisting of the shortened ACTH therapy and the additional ACTH therapy. In a prospective multi-institutional study, 20 patients with newly diagnosed West syndrome who had failed to respond to high-dose vitamin B6 and zonisamide were treated by this shortened ACTH therapy. Synthetic corticotropin (ACTH-Z 0.

[Survey of vaccination and viral infections for children with severe myoclonic epilepsy in infancy].

A study group for establishment of a proposed immunization program for neurologically high risk children (Chief, Kihei Maekawa) sponsored by the Ministry of Health, Labour and Welfare of Japan is preparing a proposal for patients with epilepsy. Severe myoclonic eplepsy in infancy (SMEI) is an intractable epilepsy which often presents with status epilepticus and triggered by hyperthermia and viral infections. In this study we investigated the history of vaccination in children with SMEI to compare the risk of vaccination with that of natural contraction of infection.

Impaired vascular endothelium-dependent relaxation in Henoch-Schönlein purpura.

Few reports have focused on vascular endothelial function in children with Henoch-Schönlein purpura (HSP). The purpose of the present study was to assess endothelial function and to follow serial changes from the acute to convalescent phases in children with HSP. Forearm flow-mediated vasodilation was evaluated in 21 patients with HSP, aged 4.

Serum KL-6 level in newborns with meconium aspiration syndrome.

Background: It has been reported that serum KL-6 increases in babies with progressing chronic lung disease (CLD). However, there have been few reports assessing KL-6 in meconium aspiration syndrome (MAS). KL-6 was measured in neonates with respiratory diseases including MAS.

Coronary diameter in normal infants, children and patients with Kawasaki disease.

Background: The coronary assessments in Kawasaki disease are mainly based on the Japanese Ministry of Health and Welfare criteria, which is simply classified according to the patient's age, over 5 years and less than 4-years-old.

Methods: We obtained normal values of coronary diameters adjusted for the body surface area and for the coronary anatomical site from 71 healthy children using 2-D echocardiography. We also studied patients with Kawasaki disease at three stages from the onset of illness: (i) 43 patients at admission; (ii) the subsequent 2-3 weeks; and (iii) 62 children followed at a clinic, for a median 2.