Clinical Manifestation of Primary Headache with Epigastric Pain or Tenderness in Children / 대한소아신경학회지

PURPOSE: Population-based studies have shown positive associations between migraine and irritable bowel syndrome, colitis and peptic ulcer and migraine prevalence was higher among patients with dysmotility-like dyspepsia or nausea/vomiting. The aim of this study was to investigate clinical manifestation between primary headache with epigastric pain or tenderness(EPT) and primary headache without EPT. METHODS: We retrospectively reviewed the medical records of 58 patients who were diagnosed primary headache[by ICHD-II(2004)] at Incheon St. Mary Hospital from January, 2006 to December, 2007. Their clinical characteristics such as age, sex, frequency & severity of headache and associated symptoms & signs were analysed. RESULTS: The rate of headache associated with EPT were 36% of migraine cases, 50% of tension-type headache cases, and 100% of unclassified headache cases. Headache with EPT were at a high rate in female. Headache with EPT were more severe than one without EPT. Regulation of behavior, diet and sleep pattern had improved severity of headache in 71% of migraine without EPT and 94% of tension-type headache without EPT, but in 12% of migraine with EPT and 18% of tension-type headache with EPT. Headache disappeared in 64% of migraine with EPT and 53% of tension-type headache by additional regular antiacid medication. CONCLUSION: Our study supports any specific correlation between headache and EPT, but further studies are needed.

An analysis of one-year experience of pediatric observation unit: The first report in Korea / 소아과

PURPOSE: While pediatric observation units (POU) have become a common practice in hospitals throughout developed countries, there has been no report about POUs in Korea so far. The aims of this study were to analyze our one-year's experience of the POU and to decide which disease entities are suitable for the POU. METHODS: All children admitted from March 2006 to February 2007 to the POU at the Department of Pediatrics in Our Lady of Mercy Hospital were included in this study. Data were collected from retrospective reviews of their medical records. RESULTS: There were a total of 1,076 POU admissions. Median age of patients was 2.4 years and median length of hospital stay 14.0 hours. The most common diagnoses were gastroenteritis (42.7%), pharyngotonsillitis (19.1%), bronchiolitis (7.8%), pneumonia (5.5%) and febrile seizure (5.2%). Overall, 7.5% of the POU patients required subsequent inpatient admissions due to hospital stays of longer than 48 hours. The disease entities that were most likely to require inpatient admission were pneumonia (17.0%), febrile seizure (12.5%) and asthma (11.5%). Diseases that allowed successful discharge from the POU were gastroenteritis (4.6%), upper respiratory tract infection (5.8%), such as otitis media and pharygnotonsillitis and seizure disorder (6.4%). Compared with the previous year when the POU was not in operation, there was a statistically significant reduction in the average length of hospital stays (from 4.69 to 3.75 days), as well as a rise in the bed turnover rate (from 78.8 to 98.2 patients/ bed). CONCLUSION: Our study shows that the POU is efficient for the management of children with certain acute illnesses. Based on this study, we suggest that the POU be used as a new modality which links between the outpatient, inpatient, and emergency departments in the field of pediatrics in Korea.

Single Nucleotide Polymorphisms of GABRG2 in Idiopathic Generalized Epilepsies(IGEs) / 대한소아신경학회지

PURPOSE: Mutations in gamma-aminobutyric acid(GABA) A receptor gamma2 subunit gene (GABRG2) were independently identified in families of generalized epilepsy with febrile seizures plus(GEFS+) and families of absence epilepsy and febrile seizures(FSs). The present study assessed the role of GABRG2 gene in idiopathic generalized epilepsies(IGEs) of Korean population. METHODS: Twenty-three IGEs and 94 healthy control subjects were selected through a collaborative study of Catholic Child Neurology Research Group. The SNP211037 of GABRG2 were screened by DHPLC. DNA fragments showing variant chromatograms were subsequently sequenced. Genotypes and allelic frequencies for GABRG2 gene polymorphism in three groups were compared. RESULTS: Genotypes and allelic frequencies of the gamma2 subunit of the GABA receptor gene(SNP211037) in both groups were not significantly different. The most common genotypes for GABRG2(SNP211037) gene in both groups were T/C heterozygote. The allele C and T frequencies for GABRG2(SNP211037) in the IGEs group were 45.7% and 54.3%, respectively and in healthy control group, 42.6% and 57.4%, respectively. The number of individuals with the GABRG2 (SNP211037)-C/C genotype in the IGEs group was greater compared with that in the healthy control group(21.7% versus 12.8%). The odds ratio for developing IGEs in individuals with the GABRG2 (SNP211037)-CC genotype was 1.65 compared with individuals with the GABRG2 (SNP211037)-T/T genotype, which was not significantly different. CONCLUSION: These data suggest that genomic variations of GABRG2 might not be one of the susceptibility factors for IGEs in the Korean population.

Clinical Features of Moyamoya Disease in Children / 대한소아신경학회지

PURPOSE: Moyamoya disease, is a chronic progressive cerebrovascular occlusive disorder that can lead to permanent neurologic deficit. The aim of this study is to analyze clinical features, radiologic findings, and prognosis of Moyamoya disease comparing with previous studies. METHODS: We retrospectively reviewed on the clinical data of 26 patients and they were analyzed in terms of age, sex, clinical features, radiologic findings, treatment and prognosis. RESULTS: The male to female ratio was 1:1.6 and the mean age was 9.3 years(male 6.1 years and female 10.9 years). There was a higher occurrence rate in 11-15 year-old group, compared to the previous studies. The most common symptoms at onset were hemiparesis and hemiplegia. Brain infarction, hemorrhage and seizure were found in five, three and two patients on brain imaging respectively. Eight patients(44%) showed reduced perfusion without structural abnormalities on brain perfusion CT. The internal carotid artery was the most common site of occlusion on conventional angiography. Indirect encephalo-duro-arterio-synangiosis was performed in 76.9% of the patients. One patient(3.85%) died and twelve patients(46.15%) showed neurologic sequelae at the time of discharge. Sequelae included hemiplegia, gaze disturbance, facial palsy, and seizures. CONCLUSION: Compared with previous studies, the age at onset was higher in our patients, with a peak incidence after the age of 10. Lower rate of severe neurologic sequelae, such as hemiparesis and hemiplegia was observed in this study. Because this study was limited by the small number of patients included, further investigations such as multi-center prospective analysis is needed.

Survival and Graft versus Host Disease in Murine MHC Mismatched Hematopoietic Stem Cell Transplantation with Co-injection of Mesenchymal Stem Cells

BACKGROUND: Intravenous injection of mesenchymal and hematopoietic stem cells (MSCs, HSCs) has the disadvantages of low delivery rate to bone marrow and sequestration of cells in the lung and liver. This study was designed to determine whether there is a relationship between the administration route and dosage of stem cells and GVHD and survival. METHODS: MSCs were retrieved from five subcultured C3H/10T1/2, cell lines from C3H/He mice. HSCs were transplanted by injecting 1 x 10(7) of bone marrow mononuclear cells and 5 x 10(6) of spleen cells from six to eight week old female C3H/He mice into six week old irradiated female BALB/c mice. The groups were divided into intravenous injection (IV) and intra-marrow (IM) injection groups. IV and IM+MSC groups consisted of mice transplanted with the same bone marrow mononuclear cells and SP, IV and IM groups, with the additional co-injection of 1 x 10(6) MSCs. RESULTS: Evaluation of all mice, in both groups, showed no difference in GVHD and survival. However, high dose injection with 1 x 10(6) MSCs led to a decreased incidence of GVHD (P<0.05) and improved survival (P<0.01) in both groups. CONCLUSION: The results of this study showed that the positive effects of MSC on GVHD and survival were primarily dependent on the number of injected cells.

Short-term follow up of thyroid function after pediatric hematopoietic stem cell transplantation / 소아과

PURPOSE: In this study, we analyzed the short term changes of thyroid function, incidence and risk factors of thyroid dysfunction soon after allogeneic hematopoietic stem cell transplantation (HSCT) in children. METHODS: We enrolled 80 pediatric patients following allogeneic HSCT, at the Catholic HSCT center between January, 2004 and February, 2006. Serum TSH (thyroid stimulating hormone), total serum thyroxine and total serum triiodothyronine levels were systematically measured in 80 patients before the HSCT, and at 1 month, 6 months and 12 months after HSCT. RESULTS: Thyroid function statistically decreased at 1 month after HSCT(P or = II) were risk factors for ETS (P=0.04, 0.01 respectively). In multivariate analysis, we could not detect an independent risk factor for ETS (P=0.19, 0.06 respectively). CONCLUSION: The present study suggests that the incidence of thyroid dysfunction is high after allogeneic HSCT. Therefore, regular monitoring of thyroid hormone levels after HSCT is required.

Hematopoietic Effects of Valproic Acid in Mouse / 대한소아혈액종양학회지

PURPOSE: Valproic acid (VPA) has been used as an anticonvulsant for a long time. Recently, there are many reports on VPA activity with regards to intracellular signal transduction, including differentiation, proliferation, and apoptosis. We experienced several hematologic toxicities during the long-term use of VPA. Therefore, we investigated whether VPA has effects on short-term or long-term hematopoiesis with respect to differing concentrations. METHODS: We obtained bone marrow mononuclear cells (BMMNC) from a 5 week old female C3H/He strain mouse. The BMMNC were cultured in semi-solid media mixed with VPA according to the concentrations of colony forming unit for granulocyte-monocytes (CFU-GM). The concentrations of VPA were used as follows: 0.01 mM, 0.1 mM, 1 mM, and 10 mM (therapeutic level: 0.07~1.1 mM). We performed long-term liquid culture under VPA to compare the frequency of long-term culture initiating cells (LTC-IC) according to various VPA levels. RESULTS: The number of CFU-GM was highest with 1 mM of VPA (45.2+/-13.5), with higher therapeutic level than control (25.7+/-11.9), in 0.01 mM of VPA (26.5+/-12.1) and in 0.1 mM of VPA (26.6+/-12.2). In 10 mM of VPA, a toxic level of VPA, was the lowest at 1.6+/-1.1 (P< 0.01). In long-term culture, the frequency of LTC-IC was increased in 0.1 mM of VPA (67.7+/-16.3%), lower therapeutic level than in control (5.5+/-10.6%). In 1 mM of VPA, the high therapeutic level decreased to 81.6+/-9.3%. With toxic levels of VPA, 10 mM, there was no hematopoiesis. CONCLUSION: The VPA might enhance short-term hematopoiesis at high therapeutic levels, while preserving LTC-IC in long-term hematopoiesis under low therapeutic concentrations. Therefore, we suggest that VPA to be used within a low therapeutic level to escape from hematopoietic suppression when using VPA as long-term medication for seizure control.