NELL2 gene as regulator of cell cycle in neuron differentiation / 소아과

PURPOSE: Because NELL2 expression is strictly restricted only in neurons in developing and post- differentiated neural tissues, it is thought to be involved in the neuronal differentiation during development and in the maintenance of neuronal physiology in the post-differentiated neurons. In this study, we examined whether NELL2 is involved in the regulation of cell cycle and apoptosis in the hippocampal neuroprogenitor HiB5 cells. METHODS: Effects of NELL2 on the cultured HiB5 cell numbers, DNA fragmentation, and proteins involved in the regulation of the cell cycle were measured. RESULTS: NELL2 induced a decrease in cell numbers and an increase in G1 phase arrest. Moreover, transfection of NELL2 resulted in an increase of DNA fragmentation that shows an evidence of apoptosis. Contents of proteins involved in the regulation of cell cycle were also changed by transfection of NELL2 expression vectors. CONCLUSION: This study suggests that NELL2 plays an important role in the regulation of cell cycle and apoptosis of neurons.

The Experience of the 1st Daegu-Gyeongbuk Multicenter Epilepsy Camp for Children and their Parents to improve the understanding of Epilepsy

PURPOSE:This study was undertaken to announce our experience of the 1st Daegu- Gyeongbuk Multicenter Epilepsy Camp for children and their parents to improve the understanding of epilepsy. METHODS:We held the camp on February 25th to 26th, 2006 at Gyeongju. 33 families with epileptic childlend joined the camp. At the end of the camp, we performed a survey with 37 questionnaires to obtain data concerning the parents' understanding of epilepsy and the satisfaction with the camp. Data was analyzed with SPSS 10.0 program using frequency analysis. RESULTS:Parents knew about epilepsy but for some degrel still wanted to get more information about epilepsy. Parents were satisfied with the educational programs but not with the recreation programs. CONCLUSION:This study suggests that more social approaches such as an epilepsy camp is needed to provide the families with more information fot the understanding about epilepsy.

Genetic Propensity to Neuroinflammatory CNS Diseases in Korean Children: Molecular Typing of HLA Class II

PURPOSE: The study was aimed to investigate immunogenetic peculiarities of neuroinflammatory CNS diseases in Korean children. METHODS: A total of 16 children with neuroinflammatory CNS diseases(9 males and 7 females; mean age 7.5+/-4.2 years) were consecutively recruited. Genomic typings were performed on their HLA DRB/HLA DQB genes using PCR-SSOP/SSP techniques with Gel immunoelectrophoresis. RESULTS: The frequencies of HLA-DRB1*14(38%), HLA-DRB1*15(25%), HLA-DRB3* 02(50%), HLA-DQB1*05(56%) and DQB1*06(44%) were significantly increased compared with a control group. The frequencies of HLA-DRB1*15(50%) and HLA-DQB1*06(63%) were significantly increased in children with ADEM and HLA-DRB3*0202(100%), HLA- DRB1*1302(67%), HLA-DRB3*0301(67%), and HLA-DQB1*0301(67%) in children with multiple sclerosis. HLA-DRB1*1401, HLA-DRB3*0202, and HLA-DQB1*0502 were found in children with acute necrotizing encephalopathy. CONCLUSION:HLA-DRB1*14, HLA-DRB1*15, HLA-DRB3*02, HLA-DQB1*05 and DQB1*06 may be associated with the susceptibility to neuroinflammatory CNS diseases in Korean children. The frequencies of HLA-DRB1*1501, HLA-DRB5*0101, HLA-DRB3* 0301, and HLA-DQB1*0602 in Korean children with multiple sclerosis were not as high as those in western children. However, HLA-DRB3*0202 was seen in all the children with multiple sclerosis. Our data may provide further evidence that the immunogenetic backgrounds of neuroinflammatory CNS diseases in Korean children are distinctly different from those in Westerns. However, further studies are needed.

Shock / 소아과

No abstract available.

The Comparison of Interferon-alpha Treatment by Dosages and Retreatment for Chronic Hepatitis B in Children / 대한소아소화기영양학회지

PURPOSE: We compared the therapeutic efficacy of low dose with that of standard dose of interferon (IFN) treatment and also compared the first IFN treatment with retreatment. METHODS: We have studied 51 children (age, 2~14) treated for chronic hepatitis B from March 1990 to August 1999. Twenty seven children had been treated with 3 MU/m2 (2.66+/-0.66 MU/m2) of IFN-alpha three times a week for 6 months (range, 6~12 months), whereas 24 children with 6 MU/m2 (4.45+/-0.94 MU/m2). There was no significant difference in gender, age, initial ALT and HBV DNA levels between each comparative group. RESULTS: Among the 27 children treated with 3 MU/m2 of IFN, ALT level had normalized in 11 children (41%) and anti-HBe seroconversion occurred in 9 children (33%) one year after the initiation of treatment. In comparison, among the 24 children treated with 6 MU/m2 of IFN, ALT normalized in 12 children (50%) and anti-HBe seroconversion occurred in 7 children (29%). In comparing the first treatment group to retreatment group, ALT level had normalized in 23 children (45%) and anti-HBe seroconversion occurred in 16 children (31%) among the 51 children treated with the first course of IFN treatment. In comparison, ALT normalized and anti-HBe seroconversion occurred in 3 children (25%) among the retreated 12 children. CONCLUSION: There was no significant difference in the therapeutic efficacies between 3 MU/m2 and 6 MU/m2 dose of IFN treated groups in ALT normalization and anti-HBe seroconversion. The retreatment efficacy of IFN-alpha was as effective as the first treatment.

A Clinical Profile of Peripheral Neuropathy in Korean Children

PURPOSE: To analyze the clinical features of peripheral neuropathy in Korean children. METHODS: A total of 62 children with acute flaccid paralysis, longstanding weakness of extremities, or abnormal electrophysiological studies, suggestive of peripheral neuropathy, were evaluated retrospectively from the hospital records. The subjects were recruited at the pediatric neurology and endocrine clinic, Kyungpook National University Hospital from 2000 to 2002 and they all went through neurological examination and electrophysiological studies with or without nerve biopsy. RESULTS: Thirty nine children(Male 24:Female 15; Mean age 7.6+/-4.3 years) were found to have clinical peripheral neuropathy. Inflammatory neuropathy(5 children with Guillain Barre syndrome, 1 children with chronic inflammatory demyelinating polyneuropathy, 12 children with Bell's palsy; 46%) was the most common, followed by hereditary neuropathy(4 children, 10%), Chemotherapy induced neuropathy(3 children, 8%), metachromatic leukodystrophy(2 children, 5%), trauma(2 children, 5%), diabetic neuropathy(1 children, 3%) and so on. Thirty two children had motor neuropathy(82%), six children had combined motor and sensory neuropathy(15%), two had pure sensory(5%), but nobody had autonomic neuropathy. With respect to the type of involvement, polyneuropathies constitute 59%(23 children), mononeuropathy simplex accounted for 38%(15 children), mononeuropathy multiplex was found in 3%(1 child). Based on electrophysiological studies and biopsy results, demyelinating neuropathy was seen in 22 children(56%), axonal neuropathy in 12 children(31%), combined neuropathy in 5 children(13%). Eighteen children(46%) were completely or almost completely recovered from the illness. CONCLUSION: Inflammatory neuropathy was the most common among the acquired neuropathies and hereditary motor sensory neuropathy was the most common among the genetic neuropathies. Treatable neuropathies took up 46%. Potentially preventable neuropathies accounted for 36%. Early diagnosis and early intervention may have significant impacts on the prognosis of peripheral neuropathy in children.

Clinical Significance of Auditory Brainstem Response(ABR) in Speech/Language Disorders

PURPOSE: The study aimed to evaluate the efficacy of auditory brainstem response(ABR) as a screening tool in children with speech and language disorders. METHODS: Between January 1, 1999 and December 31, 2001, 139 patients with chief complaints of speech and language delay were recruited from the pediatric neurology clinic, Kyungpook National University Hospital, Daegu, Korea. They had ABR on entry and the clinical data were then analyzed. RESULTS: Fifteen out of 139 cases(10.8%) showed abnormal findings; seven had pervasive developmental disorders, four had developmental language disorders, and four were noted to have other conditions. Among them, seven cases were noted to have conductive hearing loss and eight had sensoryneuronal hearing loss. We also evaluated the normal values in children at the ages of 18 months to seven years. The mean latency of wave I and V were 1.40+/-0.13 and 5.57+/-0.26 respectively. Interpeak latency of I-V was 4.18+/-0.24. CONCLUSION: Based on these findings, ABR has proved to be a highly sensitive and specific index of hearing impairment. It should be used as a screening tool in children with speech and language disorders.

Safety and Efficacy of Topiramate Monotherapy in Children with Recent-onset Seizures / 대한간질학회지

PURPOSE: To investigate the efficacy and safety of topiramate monotherapy in children with recent-onset seizures. METHODS: An open-label, randomized, parallel group trial compared the efficacy and tolerability of topiramate monotherapy versus oxcarbazepine monotherapy in 54 children with newly diagnosed epilepsy. RESULTS: Of the 54 patients treated with topiramate (N=25) or oxcarbazepine (N=29), 51 patients completed the full study period of 16 weeks. In topiramate group, 64% became seizure free, 20% demonstrated a greater than 50% degree in seizure frequency, and 4% got worsened in seizure frequency. In oxcarbazepine group, 59% became seizure free, 24% experienced a greater than 50% reduction in seizure frequency. The efficacy was not statistically different between the two groups. Adverse events were usually only mild to moderate and the tolerability of therapy was similar between the two groups. CONCLUSION: Topiramate monotherapy was highly effective in controlling seizures of children with newly diagnosed epilepsy (seizure free rate : 64%).

The Effectiveness of Propofol on Pediatric Cardiac Catheterization and Electrophysiologic Study

PURPOSE: Our purpose was to evaluate the effectiveness of propofol on pediatric cardiac catheterization or radiofrequency catheter ablation. METHODS: We measured the serial changes of arterial oxygen saturation, heart rate, systolic and diastolic blood pressure at 4 stages, a baseline(during normal sleep), stage 1(just after loading of propofol 2 mg/kg over 10 minutes), stage 2(10-15 minutes after propofol continuous intravenous infusion at 50 microg/kg/min), and stage 3(25-30 minutes after continuous infusion). RESULTS: Serial changes of arterial oxygen saturation were 96.4+/-7.7, 96.7+/-6.5, 97.0+/-5.2, and 96.5+/-6.1% respectively, with a significant but mild decrease between stage 2 and stage 3(P<0.05), and no significant decrease as compared with the baseline value. There were no significant changes of heart rate(102+/-22, 101+/-21, 100+/-19, 99+/-17 beats/min.), systolic blood pressure(103+/-12, 101+/-14, 103+/-17, 104+/-15 mmHg) and diastolic blood pressure(55+/-10, 52+/-9, 54+/-13, 52+/-9 mmHg). Mean time to complete recovery of consciousness was 19+/-20(+/-2SD) minutes. Oxygen supplementation was required in five cases, and oxygen saturation was sastained stable after administration of the oxygen at the rate of 2-4 L/min. CONCLUSION: During invasive pediatric cardiac procedure, intravenous propofol sedation had no significant impacts on heart rate and blood pressure but some impacts on arterial oxygen saturation which was easily corrected by oxygen administration. Propofol might be considered as a relatively safe and effective sedative drug in pediatric cardiac invasive procedures.

Asymptomatic Primary Hematuria in Children

Localized or solitary fibrous tumor (SFT) of the pleura has been classified as a type of mesothelioma, arising from the submesothelial connective tissue cells. The preoperative diagnosis of the tumor at the cytologic or histologic level is very important for the proper handling of the lesion. This preoperative diagnosis is now possible by means of the advance in the transthoracic fine needle aspiration biopsy (FNA) techniques and in the very experience of the cytopathologists. We describe FNA cytologic feature of two cases of SFT arising from the pleura. Cytologic, histologic, immunohistochemical, and electron microscopic characteristics of pleural SFT are discussed. The tumor cells of SFT are spindle or oval in shape with a variable amount of cytoplasm. They are arranged in irregular trabeculae intimately ass- ociated with capillaries. A unique cytologic feature observed in this tumor is that thick, eosinophilic, amorphous collagen bundles are scattered between tumor cells