No abstract available.
Spinal Muscular Atrophies of Childhood*

No abstract available.
Spinal Muscular Atrophies of Childhood*

No abstract available.
Humans*

No abstract available.
Humans*

OBJECTIVES: Attention-deficit hyperactivity disorder (ADHD) and Asperger's disorder (AD) in children are associated with attentional problems, impulsivity, hyperactivity, and difficulties with social interactions. Pharmacological treatment may alleviate symptoms of ADHD, but seldom solves difficulties with social interactions. Social skills training (SST) may assist in improving their social interactions. We examined the effects of SST on children's social competences, general behavior, and ADHD symptoms. METHODS: Thirty four children, aged 7 to 12 years, participated in the cognitive behavioral SST program once a week at the outpatient division of child-adolescent psychiatry. SST was composed of 24 sessions (ninety minutes) for 6 months. Twenty-five children were diagnosed with ADHD, and 9 children were diagnosed with AD. Parents of the children rated Korea-Child Behavior Checklist (K-CBCL), Conner's rating scales, Korean-ADHD Rating Scale (K-ARS), Social Skill Rating System (SSRS), and Matson's Social Skill Rating Scale as an evaluation of the treatment effect, before the first session and after the final session of the training. RESULTS: The ADHD group showed significantly increased scores of social and social competence of CBCL and SSRS. Further, scores of externalizing problems of CBCL, CRS, and ARS were significantly decreased. The Asperger's group showed significantly increased scores of social competence of CBCL, SSRS, and MESS. There was a significant difference of the improvement in CBCL's school and total behavior problem score, CRS between drug change group and no drug change group. CONCLUSION: The result of this study suggests that SST is effective in improving social skills for children with ADHD and AD. In addition, SST has shown its effectiveness in treating attentional problems for children with ADHD. To prove objective usefulness of SST, further studies with a more structured design and long-term duration along with a sufficient number of AD participants will be necessary.
Asperger Syndrome* ; Checklist ; Child* ; Humans ; Impulsive Behavior ; Interpersonal Relations ; Mental Competency ; Outpatients ; Parents ; Weights and Measures

The purpose of this study was to develop an information system about drugs. The Joint Application Design Method which is popular in developing information systems was used. This system will be useful for the health care personnel and nursing students with easy access to information by using computer. The user requirements for this system were collected and analyzed through a survey of 50 nurses in a hospital and 30 students at a school of nursing. Based on this analysis, the specific contents were designed. The characteristics of this system as follows. Firstly it is the fast searching function which uses not only names of drugs(generic name, functional name, commercial name), but also names of diseases. Secondly, it is the function which maintains an updated database by the way of adding, updating and deleting the data. Thirdly, this system is useful for the education. In teaching and learning process, Computer-Assisted Instruction(CAI) is considered as one of the outstanding method bacause the individual differences in learners are respected and thus educational effect in pharmacology is expected.
Access to Information ; Delivery of Health Care ; Education ; Humans ; Individuality ; Information Systems* ; Joints ; Learning ; Nursing* ; Pharmacology ; Students, Nursing

PURPOSE: The purpose of this study was to develop an e-Learning program about medication for nurses to enhance nurses' medication performance ability and to analyze learners' responses after studying with this program. METHOD: For the development of the e-Learning program, the NBISD(Network Based Instructional Systems Design) model, suggested by Jung(1999) was applied as a basic model and the instruction design theory of Gagne & Briggs(1979) and ARCS theory of Keller(1983) were applied. After the operation of this program for one month to 34 new nurses, learners' responses were analyzed. RESULT: Learners' knowledge of medication was greatly improved after this program. In addition learners' satisfaction with the overall education program, help in field applicability, ease of screen shift and exploration, and tutor activities were high and the contents were regarded suitable for e-Learning. Many things were advantageous such as easy accessibility, easy understandability with pictures and flash animation, practical cases and feedback from a tutor. Provision of a supplementary handout and improvement of a tight time schedule were pointed out as things to be improved. CONCLUSION: This e-Learning program can be used effectively for medication education for registered nurses, student nurses, and new nurses.
*Pharmaceutical Preparations ; *Internet ; Humans ; Female ; *Education, Nursing ; *Computer-Assisted Instruction ; Adult

PURPOSE: A hypomotor seizure is a useful descriptive terminology, but its clinical and ictal characteristics are not fully understood. We investigated the clinical and electrophysiological characteristics of hypomotor seizures in children. METHODS: We reviewed 394 video-taped recordings, performed between Dec. 1994 and Feb. 2003., to select patients of hypomotor seizures. Exclusion criteria were as follows; i) hypomotor seizures accompanied with other types of seizures in a single event, ii) patients without neurocognitive dysfunctions who were older than 3 years old, iii) non-convulsive status epilepticus, iv) poor video quality. A total of 329 hypomotor seizures from 42 patients (M:F=27:15) were included. The mean age of onset was 1.9 years old, the mean age at the monitoring 4.5 years old, and the mean duration of follow-ups 3.9 years. RESULTS: Thirteen patients (31%) showed generalized onset, hypomotor seizures while 29 patients (69%) showed partial onset hypomotor seizures. 35 patients (83.3%) had neurocognitive deficits. Significant abnormalities in the on brain MRIs were revealed in 23 patients (54.8 %). The concordance rates of interictal epileptiform discharges were 29.0%. In 8 patients, (hypomotor seizures were separately accompanied by other types of seizures (19.0%)). Comparing generalized seizures (group 1) with partial seizures (group 2), there were no significant differences in the clinical parameters between the two groups. In brain MRIs, group 2 tended to have focal lesions. On the analysis of ictal rhythms in group 1, diffuse spike and wave discharges were noted in 8 patients, rhythmic beta in 2 patients, semirhythmic theta in 1 patient, diffuse attenuation of the background in 1 patient, and bilateral beta in 1 patient. Among 5 patients with ictal rhythms other than diffuse spike and wave discharges, 4 patients were younger than 3 years old. On the analysis in group 2, rhythmic ictal patterns were noted in 24 patients (82.8%), seimirhythmic in 2 patients (6.9%), and irregular in 3 patients (10.3%). The distribution of ictal frequencies was as follows:alpha in 3 (10.3%), beta in 4 (13.8%), theta in 10 (34.5%), delta in 4 (13.8%), repetitive spikes or sharp waves in 5 (17.3%), and spikes and waves in 3 (10.3%). Rhythmic beta patterns tended to be localized into the posterior quadrant. CONCLUSION: Hypomotor seizures consist of generalized-onset (31%) and partial-onset (69 %). No clinical parameters can predict the ictal patterns. Various patterns in patients with generalized onset hypomotor seizures under 3 years old may suggest different mechanisms of generalized hypomotor seizures from absence seizures. Rhythmic beta patterns from the posterior quadrant may suggest the localization-specific ictal patterns.
Age of Onset ; Brain ; Child* ; Child, Preschool ; Electroencephalography ; Epilepsy, Absence ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Seizures* ; Status Epilepticus

PURPOSE: The channelopathies has been implicated in the pathogenesis of idiopathic generalized epilepsy(IGE). Recently, nonsense and missense mutations in a voltage-gated chloride channel gene(CLCN2) have been shown to be associated with IGE. Loss of CLCN2 function results in loss of sustained GABA inhibition, increasing the risk of uncontrolled firing leading to seizure activity. This study assessed the relevance of mutations in the CLCN2 gene in the Korean patients with IGE. METHODS: Twenty seven patients with IGE were recruited in the Seoul National University Boramae Hospital. PCR and direct sequencing of genomic DNA were done to analyze the complete coding region of CLCN2. 99 controls were tested for two identified polymorphisms. Genotypes and allelic frequencies were compared to controls with epilepsy patients and subgroup of IGE: 10 generalized epilepsy with febrile seizure plus(GEFSP), 9 childhood absence epilepsy(CAE) and 8 other IGE patients. RESULTS: Twelve CLCN2 polymorphisms: 3 exonic, 2 promotor and 7 intronic, were found in 22 patients(81%) and 2 polymorphisms were noble. Two polymorphisms in the exonic region with changes of amino acid, p.L15P and p.T668S and 2 polymorphisms in the promoter regions(c.1-1990T>C, c.1-693G>A) may affect on the CLCN channel function. The odds ratio for developing other IGE in patients with RS9820367-CG type was 4.2 compared to individuals with CC type. In addition, the odds ratio for developing other GEFSP in patients with RS9820367-CC type was 4.0 compared with individuals with CG type. CONCLUSION: Our findings suggest that genomic variations of CLCN2 may be implicated in the pathogenesis of IGE.
Channelopathies ; Chloride Channels ; Clinical Coding ; DNA ; Epilepsy ; Epilepsy, Generalized ; Exons ; Fires ; gamma-Aminobutyric Acid ; Genotype ; Humans ; Immunoglobulin E ; Introns ; Mutation, Missense ; Odds Ratio ; Polymerase Chain Reaction ; Seizures ; Seizures, Febrile

Monoamine oxidase inhibitors (MAOI) have been widely used as antidepressants. Recently, there has been renewed interest in MAO inhibitors. The activity-guided fractionation of extracts from Angelica keiskei Koidzumi (A. keiskei K.) led to the isolation of two prenylated chalcones, xanthoangelol and 4-hydroxyderricin and a flavonoid, cynaroside. These three isolated compounds are the major active ingredients of A. keiskei K. to inhibit the MAOs and DBH activities. Xanthoangelol is a nonselective MAO inhibitor, and a potent dopamine beta-hydroxylase (DBH) inhibitor. IC50 values of xanthoangelol to MAO-A and MAO-B were calculated to be 43.4 microM, and 43.9 microM. These values were very similar to iproniazid, which is a nonselective MAO inhibitor used as a drug against depression. The IC50 values of iproniazid were 37 microM, and 42.5 microM in our parallel examination. Moreover, IC50 value of xanthoangelol to DBH was calculated 0.52 microM. 4-Hydroxyderricin is a potent selective MAO-B inhibitor and also mildly inhibits DBH activity. The IC50 value of 4-hydroxyderricin to MAO-B was calculated to be 3.43 microM and this value was higher than that of deprenyl (0.046 microM) used as a positive control for selective MAO-B inhibitor in our test. Cynaroside is a most potent DBH inhibitor. The IC50 value of cynaroside to DBH was calculated at 0.0410 microM. Results of this study suggest that the two prenylated chalcones, xanthoangelol and 4-hydroxyderricin isolated from A. keiskei K., are expected for potent candidates for development of combined antidepressant drug. A. keiskei K. will be an excellent new bio-functional food material that has the combined antidepressant effect.
Angelica* ; Antidepressive Agents ; Chalcones ; Depression ; Dopamine beta-Hydroxylase ; Inhibitory Concentration 50 ; Iproniazid ; Monoamine Oxidase ; Monoamine Oxidase Inhibitors ; Oxidoreductases* ; Selegiline