Hydrocephalus is a common malformation of the central nervous system and its cause may be either congenital or acquired. The imbalance between CSF formation and absorption, obstruction of CSF pathways, impaired venous absorption, and over secretion of CSF results in excessive accumulation of the fluid in the ventricles, leading to hydrocephalus. Although ventriculo-peritoneal shunt is regarded as the main and definitive therapy for rapidly progressive hydrocephalus, shunts in newborns have a high failure rate and thus there have been a search for alternative non-invasive techniques. Acetazolamide is a carbonic anhy- drase inhibitor, which acts by reducing production of CSF in the choroid plexus. Admini- stration of acetazolamide will decrease the rate of CSF production, preventing progressive ventricular enlargement. We experienced three cases of neonatal hydrocephalus successfully treated by long-term administration of acetazolamide. Brief review and related literatures were also presented.
Absorption ; Acetazolamide* ; Carbon ; Central Nervous System ; Choroid Plexus ; Humans ; Hydrocephalus* ; Infant, Newborn ; Ventriculoperitoneal Shunt

Th17 cells (Th17) are a distinct lineage of CD4+ T cells that secrete high amounts of IL-17 under orphan nuclear receptor retinoic acid receptor-related orphan receptor gammat (RORgammat) which is a lineage-specific transcription factor. TGF-beta and inflammatory cytokines, such as IL-6, IL-21, IL-1beta, and IL-23, play central roles in the generation of Th17 cells. Th17 cells and their effector molecules, such as IL-17A, IL-17F, IL-21, IL-22, and CCL20, contribute to the progression and pathogenesis of several autoimmune and inflammatory diseases, such as rheumatoid arthritis, psoriasis, multiple sclerosis, inflammatory bowel disease and systemic lupus erythematosus. Studies of Th17 development and the effects of IL-17 signaling in autoimmune responses suggest a high potential for targeting this pathway in immune pathologies. In this review, we discuss Th17 biology in relation to autoinflammatory disorders and the various therapeutic strategies under investigation which target the IL-17-Th17 cell pathway in chronic inflammatory autoimmune disorders.
Arthritis, Rheumatoid ; Autoimmune Diseases ; Autoimmunity ; Biology ; Child ; Child, Orphaned ; Cytokines ; Humans ; Inflammatory Bowel Diseases ; Interleukin-17 ; Interleukin-23 ; Interleukin-6 ; Interleukins ; Lupus Erythematosus, Systemic ; Multiple Sclerosis ; Psoriasis ; Receptors, Interleukin-17 ; T-Lymphocytes ; Th17 Cells ; Transcription Factors ; Transforming Growth Factor beta ; Tretinoin

PURPOSE: The purpose of this study was to assess the clinical application of a bar code medication administration and blood transfusion system and to identify its effects from the aspect of patient safety and nurse satisfaction in a tertiary hospital. METHODS: The system in this study was PDA with bar code reading capability and wireless networking function. The logs created during application of the system and administration error reports were analyzed. For nurses' satisfaction with the system, data were collected from 337 nurses using the instrument developed by Otieno et al. and analyzed using descriptive statistics. RESULTS: The system application rate was 98.8%, and the main failure cases in the system application included bar code or network related factors. When the system was applied, 0.02% of errors were prevented. The nurses were satisfied with the system from the aspect of patient safety, however relatively less satisfied with the system from the aspect of work efficiency. CONCLUSION: The results of the study indicate the usefulness for patient safety of applying the bar code medication administration and blood transfusion system to clinical practice. However technological improvements including bar code and network communication are necessary to ensure higher work efficiency in nursing practice.
Automatic Data Processing ; Blood Transfusion ; Dietary Sucrose ; Information Systems ; Patient Safety ; Tertiary Care Centers

OBJECTIVE: Human embryonic stem (ES) cells have a great potential in regenerative medicine and tissue engineering. The human ES cells could be differentiated into specific cell types by treatments of growth factors and alterations of gene expressions. However, the efficacy of guided differentiation and isolation of specific cells are still low. In this study, we characterized isolated cells from differentiated human ES cells by magnetic activated cell sorting (MACS) system using specific antibodies to cell surface markers. METHODS: The undifferentiated hES cells (Miz-hESC4) were sub-cultured by mechanical isolation of colonies and embryoid bodies were spontaneously differentiated with DMEM containing 10% FBS for 2 weeks. The differentiated cells were isolated to positive and negative cells with MACS system using CD34, human epithelial antigen (HEA) and human fibroblast (HFB) antibodies, respectively. Observation of morphological changes and analysis of marker genes expression were performed during further culture of MACS isolated cells for 4 weeks. RESULTS: Morphology of the CD34 positive cells was firstly round, and then it was changed to small polygonal shape after further culture. The HEA positive cells showed large polygonal, and the HFB positive spindle shape. In RT-PCR analysis of marker genes, the CD34 and HFB positive cells expressed endodermal and mesodermal genes, and HEA positive cells expressed ectodermal genes such as NESTIN and NF68KD. The marker genes expression pattern of CD34 positive cells changed during the extension of culture time. CONCLUSION: Our results showed the possibility of successful isolation of specific cells by MACS system from undirected differentiated human ES cells. Thus, MACS system and marker antibodies for specific cell types might be useful for guided differentiation and isolation of specific cells from human ES cells.
Antibodies ; Ectoderm ; Embryoid Bodies ; Endoderm ; Fibroblasts ; Gene Expression ; Humans* ; Intercellular Signaling Peptides and Proteins ; Mesoderm ; Nestin ; Regenerative Medicine ; Tissue Engineering

OBJECTIVE: The aim of this study was to investigate whether embryonic stem (ES) cells can be established from isolated blastomeres of mouse embryos. METHODS: Blastomeres were separated from mouse (C57Bl/6J) 2- or 4-cell embryos. Isolated blastomeres or whole 4-cell embryos were co-cultured with mitosis-arrested STO feeder cells in DMEM supplemented with recombinant murine leukemia inhibitory factor and ES-qualified fetal bovine serum. After the tentative ES cell lines were maintained from isolated blastomeres or whole embryos, some of them were frozen and the others were sub-cultured continually. Characteristics of tentative ES cell lines as were evaluated for specific gene expressions with immunocytochemistry and RT-PCR. RESULTS: One ES cell line (3.0%) was established from isolated blastomere of 2-cell embryo and one cell line (4.0%) from isolated two blastomeres of 4-cell embryo. And five cell lines (16.7%) were established from whole 4-cell embryos. Both cell lines from isolated blastomere and whole embryo expressed mouse ES cells specific markers such as SSEA-1, Oct-4 and alkaline phosphatase. Marker genes of three germ layers were expressed from embryoid bodies of both cell lines. CONCLUSION: This study suggests that mouse ES cells could be established from isolated blastomeres, although the efficiency is lower than whole embryos. This animal model could be applied to establishment of autologous human ES cells from biopsied blastomeres of preimplantation embryos in human IVF-ET program.
Alkaline Phosphatase ; Animals ; Antigens, CD15 ; Blastocyst* ; Blastomeres* ; Cell Line ; Embryoid Bodies ; Embryonic Stem Cells* ; Embryonic Structures ; Feeder Cells ; Gene Expression ; Germ Layers ; Humans ; Immunohistochemistry ; Leukemia Inhibitory Factor ; Mice* ; Models, Animal

BACKGROUND: This study was performed to determine the biopsy sites that are suitable for the diagnosis of Helicobacter pylori infection and to assess the sensitivity of culture, histology, and dual-priming oligonucleotide (DPO)-based multiplex PCR. Moreover, we evaluated the usefulness of PCR for the detection of 23S rRNA mutations, which are responsible for the clarithromycin resistance of H. pylori. METHODS: From 90 patients, we obtained biopsy specimens for culture, histology, and Seeplex(R) ClaR-H. pylori PCR (Seegene Inc., Korea). Phenotypic susceptibility to clarithromycin was evaluated using the E-test (AB Biodisk, Sweden). RESULTS: H. pylori was detected in 48 of 90 patients. The positive rates of infection in the antrum and body were higher than those in the biopsies obtained from the duodenal bulb. The positive rates in histology, PCR, and culture were 46.7%, 42.2%, and 34.4%, respectively. Using histology or PCR, we identified H. pylori in 46 of the 48 patients. 23S rRNA mutations were detected in 8 patients. The clarithromycin E-test showed that all the 10 wild-type patients were susceptible. However, the results of the PCR and E-test of 3 of the 8 mutation-positive patients were discrepant. CONCLUSIONS: We observed that a combination of histology and PCR affords a high detection rate of H.pylori infection and that DPO-based PCR can be practically used for the diagnosis of H. pylori infection and the determination of clarithromycin resistance. These techniques were useful for biopsy sampling simultaneously from the antrum and body for the detection of clarithromycin resistance of multiple strain infection or heteroresistance.
Anti-Bacterial Agents/*pharmacology ; Biopsy ; Clarithromycin/*pharmacology ; Drug Resistance, Bacterial ; Genotype ; Helicobacter Infections/*diagnosis/drug therapy/pathology ; Helicobacter pylori/drug effects/genetics/*isolation & purification ; Humans ; Microbial Sensitivity Tests ; Mutation ; Polymerase Chain Reaction ; RNA, Ribosomal, 23S/genetics

OBJECTIVES: Autism is a complex neurodevelopmental spectrum disorder with a strong genetic component. Previous neurochemical and genetic studies have suggested the possible involvement of the serotonin system in autism. Tryptophan 2,3-dioxygenase(TDO2) is the rate-limiting enzyme in the catabolism of tryptophan, which is the precursor of serotonin synthesis. The aim of this study was to investigate the association between the TDO2 gene and autism spectrum disorders(ASD) in a Korean population. METHODS: The patients were diagnosed with ASD on the basis of the DSM-IV diagnostic classification outlined in the Korean version of the Autism Diagnostic Interview-Revised and Autism Diagnostic Observation Schedule. The present study included the detection of four single nucleotide polymorphisms(SNPs) in the TDO2 gene(rs2292536, rs6856558, rs6830072, rs6830800) and the family-based association analysis of the single nucleotide polymorphisms in Korean ASD trios using a transmission disequilibrium test(TDT) and haplotype analysis. The family trios of 136 probands were included in analysis. 87.5% were male and 86.0% were diagnosed with autism. The mean age of the probands was 78.5+/-35.8 months(range: 26-264 months). RESULTS: Two SNPs showed no polymorphism, and there was no significant difference in transmission in the other two SNPs. We also could not find any significant transmission in the haplotype analysis(p>.05). CONCLUSION: We could not find any significant statistical association between the transmission of SNPs in the TDO2 gene and ASD in a Korean population. This result may not support the possible involvement of the TDO2 gene in the development of ASD, and further exploration might be needed to investigate other plausible SNP sites.
Appointments and Schedules ; Autistic Disorder* ; Child ; Autism Spectrum Disorder* ; Classification ; Diagnostic and Statistical Manual of Mental Disorders ; Haplotypes ; Humans ; Male ; Metabolism ; Polymorphism, Single Nucleotide ; Serotonin ; Tryptophan

PURPOSE: Kawasaki disease(KD) is a multisystemic inflammatory vasculitis of unknown etiology, but immunological abnormalities have been documented and implicated in the pathogenesis of KD. Matrix metalloproteinases(MMPs) have proteolytic activity against connective tissue proteins, and increased activity of MMPs and a quantitative imbalance between MMP and tissue inhibitor of MMP (TIMP) can result in several pathologic conditions. MMP and TIMP may also be involved in the formation of coronary arterial lesions in KD. METHODS: Serum levels of MMP1, MMP2, MMP9, TIMP1, TIMP2, interleukin(IL)-6 and tumor necrosis factor(TNF)-alpha were measured in 27 KD patients(group I, 10 patients with normal coronary artery; group II, 17 patients with coronary arterial lesions) and 15 healthy children(group III). Blood samples from each study group were drawn before and after intravenous immunoglobulin(IVIG) therapy and in the convalescent stage. RESULTS: The MMP9 levels and MMP9/TIMP2 ratios before and after IVIG therapy were significantly higher in group II. The MMP9 levels were significantly higher before IVIG therapy, and decreased through the convalescent stage. The IL-6 and TNF-alpha levels were also significantly higher in group II than in the other groups. The serum MMP9 levels showed significantly positive correlation with the circulating leukocyte counts and IL-6 levels. CONCLUSION: The increased levels of MMP and the imbalance between MMP and TIMP increase the susceptibility to the coronary arterial lesions in KD. The cytokines including IL-6 and TNF-alpha are also important in the activation of MMP and formation of coronary arterial lesions in KD.
Connective Tissue ; Coronary Vessels ; Cytokines* ; Humans ; Immunoglobulins, Intravenous ; Interleukin-6 ; Leukocyte Count ; Matrix Metalloproteinases* ; Mucocutaneous Lymph Node Syndrome* ; Necrosis ; Tumor Necrosis Factor-alpha ; Vasculitis

Maple syrup urine disease (MSUD) is an autosomal recessive disease caused by a deficiency in subunits of the branched-chain alpha-ketoacid dehydrogenase complex (BCKDH). The disease is characterized by the accumulation of the branched-chain amino acids leucine, isoleucine, valine, alloisoleucine, and their corresponding alpha-ketoacid in blood and urine. MSUD is a heterogenous disorder, and classic, intermittent, intermediate and thiamine-responsive phenotypes have been identified. We experienced a case of Maple syrup urine disease (classic type) in a female neonate, who suffered from lethargy, poor feeding, apnea, alternating periods of hypertonicity and flaccidity, generalized convulsions, and a peculiar burned sugar smell from the body and urine. She died of respiratory failure 22 days after the birth. The brief review of the literature was made.
Acer* ; Amino Acids, Branched-Chain ; Apnea ; Burns ; Female ; Humans ; Infant, Newborn ; Isoleucine ; Lethargy ; Leucine ; Maple Syrup Urine Disease* ; Oxidoreductases ; Parturition ; Phenotype ; Respiratory Insufficiency ; Seizures ; Smell ; Valine

PURPOSE: The purpose of this study was to identify risk factors for pediatric inpatients falls. METHODS: The study was a matched case-control design. The participants were 279 patients under the age of 6 who were admitted between January 1, 2004 and December 31, 2009. Through chart reviews, 93 pediatric patients who fell and 186 ones who did not fall were paired by gender, age, diagnosis, and length of stay. Five experts evaluated the 38 fall risk factors selected by the researchers. RESULTS: In a general hospital, pediatric patients with secondary diagnosis, tests that need the patient to be moved, intravenous lines, hyperactivity, anxiolytics, sedatives and hypnotics, and general anesthetics showed significance for falls on adjusted-odds ratios. Conditional logistic regression analysis was performed to elucidate the factors that influence pediatric inpatient falls. The probability of falls increased with hyperactivity and general weakness. Patients who didn't have tests that required them to be moved and intravenous line had a higher risk of falls. CONCLUSION: These findings provide information that is relevant in developing fall risk assessment tools and prevention programs for pediatric inpatient falls.
Accidental Falls/*prevention & control ; Age Factors ; Analgesics ; Case-Control Studies ; Child ; Child, Preschool ; Female ; Hematologic Diseases/pathology ; Hospitalization ; Hospitals, General ; Humans ; Infant ; Length of Stay ; Logistic Models ; Male ; Neoplasms/pathology ; Odds Ratio ; Retrospective Studies ; Risk Factors ; Sex Factors