ABSTRACT OF POSTER PRESENTATION (APRIL 17, SATURDAY)

ABSTRACT OF POSTER PRESENTATION(APRIL 17, SATURDAY)P2-001 Allergy/Immunology/Rheumatology P2-002 Allergy/Immunology/RheumatologyUNIQUE ACTIVATION STATUS OFPERIPHERAL BLOOD MONONUCLEARMOLECULAR DIAGNOSIS OF SEVERE COMBINEDIMMUNODEFICIENCY –IDENTIFICATION OF IL2RG,CELLS AT ACUTE PHASE OF KAWASAKI JAK3, IL7R, DCLRE1C AND RAG2 MUTATIONS IN ADISEASECOHORT OF CHINESE AND SOUTHEAST ASIANCHILDRENKazuyuki Ikeda 1 , Kenichiro Yamaguchi 2 , TamamiTanaka 3 , Yumi Mizuno 4 , Atsushi Hijikata 5 , OsamuOhara 6 , Hidetoshi Takada 7 , Koichi Kusuhara 8 , ToshiroHara 9Department of Pediatrics, Graduate School of Medical Sciences 1 ,Department of Pediatrics, Graduate School of Medical Sciences, KyushuUniversity 2, 3 , Department of Pediatrics, Fukuoka Children’s Hospital andMedical Center for Infectious Disease 4 , Laboratory for Immunogenomics,RIKEN Research Center for Allergy and Immunology 5,6,7,9 , Department ofPediatrics, University of Occupational and Environmental Medicine 8OBJECTIVE: Although Kawasaki disease (KD) ischaracterized by a marked activation of the immunesystem with elevations of serum proinflammatorycytokines and chemokines at acute phase, the majorsources for these chemical mediators remain controversial.The aim of this study was to analyze the activation statusof peripheral blood mononuclear cells (PBMNCs) inacute-phase KD patients.METHODS: We examined the proportion of CD69 + cellsin αβT cells, γδT cells, NK cells, and B cells by flowcytometry, and performed microarray analysis of PBMNCsisolated from KD patients and controls. Furthermore weperformed pathway analysis using microarray data. Toconfirm the microarray data, we determined the geneexpression levels of cytokines by quantitative RT-PCR. Wealso analyzed intracellular cytokines in PBMNC at acuteand convalescent phases of KD by flow cytometry.RESULTS: The proportions of CD69 + cells in both NKcells and γδT cells at acute-phase of KD were significantlyhigher than those at convalescent-phase of KD. Microarrayanalysis revealed that 5 genes such as NAIP, IPAF,S100A9, FCGR1A, and GCA were up-regulated inacute-phase KD and the pathways involved in acute phaseof KD were closely related to innate immune system. Therelative expression levels of damage-associated molecularpattern molecule (DAMP) (S100A9 and S100A12) genes inPBMNCs at acute-phase KD were significantly higher thanthose at convalescent-phase KD, while those of TNFA,IL1B and IL6 genes were not significantly differentbetween KD patients and healthy controls. Intracellularproduction of TNF-α, IL-10 and IFN-γ in PBMNCs wasnot observed in KD patients.CONCLUSION: The present data have indicated thatPBMNC showed a unique activation status with highexpression of DAMP genes but low expression ofproinflammatory cytokine genes, and that the innateimmune system appears to play a role in the pathogenesisand pathophysiology of KD.[Keywords]Kawasaki disease, peripheral blood mononuclear cells(PBMNCs), innate immunity, acquired immunity, cytokinesPamela P.W. Lee1, Koon-Wing Chan1, Tong-Xin Chen2,Li-Ping Jiang3, Xiao-Chuan Wang4, Hua-Song Zeng5,Xiang-Yuan Chen5, Bee-Wah Lee6, Lynette Shek6,Woei-Kang Liew7, Anselm C.W. Lee8, Hsin-Hui Yu9,Zarina Abdul Latiff 10, Marco H.K. Ho1, Tsz-Leung Lee1,Yu-Lung Lau11 Department of Paediatrics and Adolescent Medicine, LKS Faculty ofMedicine,The University of Hong Kong; 2 Department of Pediatrics, XinhuaHospital,Shanghai Institute for Pediatric Research, Shanghai Jiao TongUniversitySchool of Medicine, China; 3 Children’s Hospital of ChongqingMedicalUniversity, Chongqing, China; 4 Children's Hospital of FudanUniversity,Shanghai, China; 5Department of Allergy, Immunology andRheumatology,Guangzhou Children’s Hospital, Guangdong, China; 6Department of Paediatrics, National University of Singapore; 7 Department ofPaediatric Medicine, KK Children’s Hospital, Singapore; 8 Children’sHaematology & Cancer Centre, Parkway Medical Centre, Singapore; 9Department of Pediatrics, National Taiwan University Hospital, Taiwan; 10Department of Paediatrics, Faculty of Medicine, Universiti KebangsaanMalaysia Medical Centre, Malaysia.OBJECTIVES: Severe combined immunodeficiencies (SCID) is a group of rareinherited disorders with profound defects in T-cell and B-cell immunity. Atpresent, over ten SCID genes were identified, accounting for T-B+SCID (IL2RG,JAK3, IL7R, CD45, CORO1A and CD3d/CD3e/CD3z) and T-B-SCID (ADA,PNP, RAG1, RAG2, LIG4, DCLRE1C, Cernunnos/XLF, DNA-PKcs and AK2).IL2RG mutations in X-linked SCID, the commonest form, were reported inmainland China and Taiwan, but other types were not characterized in Chineseand Southeast Asians. From 2005-2009, our unit developed molecular diagnosticsfor IL2RG, JAK3, IL7R, RAG1, RAG2, DCLRE1C, AK2 and ZAP70.Twenty-seven referrals for genetic diagnosis of SCID were received. This studyaims to review the clinical features and genetic diagnoses of these patients.METHODS: Genetic studies were performed by candidate gene approach, basedon patient’s gender, immune-phenotype and inheritance pattern. Mutations wereidentified by direct sequencing of the coding regions and splice sites of respectivegenes.MAIN RESULTS: Our cohort included Chinese (n=24), Malay (n=1), Korean-American (n=1) and Arabic (n=1) patients. The median age of initiatingimmunological investigation was 4 months. These infants suffered from majorinfections including regional (n=5) or disseminated BCG disease (n=3),disseminated CMV disease (n=1), candidemia (n=4), pulmonary aspergillosis(n=1), and bacteremia (n=4). Fourteen boys had T-B+NK-SCID, thirteen ofwhom had IL2RG mutations including 4 missense, 2 nonsense, 3 frameshift and 4splice-junction mutations. Two girls had T-B+NK+ phenotype, and one of themhad compound heterozygous IL7R mutations. Three patients had T-B+NKphenotype,and one of them had homozygous JAK3 splice-junction mutation. Sixpatients had Omenn syndrome or T-B-NK+ phenotype; 2 of whom hadcompound heterozygous mutations in RAG2 and DCLRE1C respectively.Reticular dysgenesis was suspected in a newborn baby presenting with severesepsis, pancytopenia and T-B-NK- phenotype, but AK2 mutation was not found.A girl had severe CD8 lymphopenia, but ZAP70 mutation was not identified.Fifteen patients died while five were lost to follow-up. Four patients hadsuccessful match-unrelated hematopoietic stem cell transplantation (HSCT). Apatient with T-B+NK-SCID received 1-antigen mismatch haploidenticaltransplant but unfortunately died of complications on D+59.CONCLUSION: Disseminated BCG and fungal infections should be recognizedas clinical indicators of underlying immunodeficiency in infants, and detailedimmunological studies are warranted. SCID is a heterogeneous genetic disorder.Identification of genotype facilitates diagnostic confirmation and geneticcounseling. Moreover, HSCT protocols can be tailored to specificSCID-genotype for better outcomes. Early diagnosis, timely referral and securingresources for HSCT are urgently needed to improve prognosis of these patients.[Keywords] severe combined immunodeficiency, SCID, molecular diagnosis, genetics,Chinese, Asian255

P2-003 Allergy/Immunology/Rheumatology P2-004 Allergy/Immunology/RheumatologyHIGH MOBILITY GROUP BOX 1 (HMGB1)AND MACROPHAGE MIGRATIONINHIBITORY FACTOR (MIF) IN KAWASAKIDISEASETHE EFFECT OF FK506 ONSUPERANTIGEN-INDUCED STEROIDRESISTANCE OF CUTANEOUSLYMPHOCYTE-ASSOCIATED ANTIGEN CD4 TCELLSTakayuki Hoshina 1 , Koichi Kusuhara 2 , KazuyukiIkeda 3 , Mitsumasa Saito 4 , Toshiro Hara 5Pediatrics, Graduate School of Medical Sciences, Kyushu University 1 ,Department of Pediatrics, University of Occupational and EnvironmentalMedicine 2 , Department of Pediatrics, Graduate School of MedicalSciences 3 , Pediatrics, Kyushu university 4 , Department of Pediatrics,Graduate School of Medical Sciences, Kyushu University, Japan 5Liang-Shiou Ou, Jing-Long HuangDivision of Allergy, Asthma and Rheumatology, Department of Pediatrics, ChangGung Memorial Hospital, and Chang Gung University, Taoyuan, Taiwan.OBJECTIVE: To investigate whether twoproinflammatory cytokines, high mobility group box1 (HMGB1) and macrophage migration inhibitoryfactor (MIF) are involved in the development ofKawasaki Disease (KD).METHODS: Twenty-seven patients with KD wereincluded in this study. Eleven patients with sepsisand 28 healthy children served as controls. Serumlevels of HMGB1 and MIF were measured bycorresponding enzyme-linked immunosorbent assaykits, respectively. Real-time polymerase chainreaction was used to quantify the expression levels ofgenes encoding receptor for advanced glycation endproducts (RAGE), an HMGB1 receptor and CD74,an MIF receptor in peripheral blood mononuclearcells.RESULTS: Serum levels of HMGB1 and MIF inKD patients were the highest in the early acute phaseand gradually decreased after defervescence. SerumHMGB1 and MIF levels in KD patients weresignificantly higher than those in controls (HMGB1,P< 0.001; MIF, P< 0.01). The expression levels ofthe RAGE gene and CD74 gene in KD patients weresignificantly higher than those in controls (RAGE,P< 0.001; CD74, P< 0.01).CONCLUSIONS: These data suggest that HMGB1and MIF play an important role in immune responsesin KD patients.[Keywords]Kawasaki disease, High mobility group box-1,Macrophage migration inhibitory factor, Receptor for advancedglycation end products, CD74OBJECTIVE: Atopic dermatitis is a chronicinflammatory skin disease involving colonization bysuperantigen secreting Staphylococcus aureus.Superantigens are thought to play an important role ininducing inflammatory responses. This study is todetermine the effect of FK506 on superantigen-inducedsteroid resistance of cutaneous lymphocyte-associatedantigen CD4 T cells.METHODS: CD4 + CLA + T cells were isolated bymagnetic microbeads and cell proliferation wasmeasured by [ 3 H]-thymidine incorporation with differentstimulators and suppressors. Flow cytometry for surfacemarkers were determined.MAIN RESULTS: The proliferation of purifiedCD4 + CLA + or CD4 + CLA - T cells with anti-CD3stimulation with presence the APCs were sensitive to theinhibition of dexamethasone (DEX). The inhibition ratesof 10 -6 M DEX were 78.4±20.1% for CD4 + CLA + cellsand 70.1 ± 19.4% CD4 + CLA - T cells. The proliferationof purified CD4 + CLA + or CD4 + CLA - T cells withstaphylococcal enterotoxin B (SEB), stimulation withpresence the APCs were insensitive to the inhibition ofDEX, and the inhibition rate of 10 -6 M DEX were39.6±33.0% and 37.6±43.3% for CD4 + CLA + andCD4 + CLA - T cells. Tacrolimus (FK506), however, washighly effective at inhibiting SEB-induced T cellactivation. SEB-induced CD4 + CLA + and CD4 + CLA - Tcells proliferation both were significantly suppressed byFK506 in the presence APCs. The inhibition rates of 10 -6M FK506 were 90.8 ± 20.0% and 88.5 ± 9.8% forCD4 + CLA + and CD4 + CLA - T cells.CONCLUSION: Superantigen can induce steroidresistance on CD4 + CLA + and CD4 + CLA - T cells withpresence of APCs. However, the suppressive function ofFK506 might revere superantigen- induced steroidresistance.256

P2-005 Allergy/Immunology/Rheumatology P2-006 Allergy/Immunology/RheumatologyANALYSIS OF CLINICAL AND MOLECULARCHARACTERISTICS OF WISKOTT-ALDRICHCLINICAL AND IMMUNE EFFECTS OFETANERCEPT USED IN JIASYNDROME IN 24 PATIENTS FROM 23UNRELATED CHINESE FAMILIESXiaodong Zhao, Zhi-yong Zhang, Hui-qin Xiao ,Li-pingJiang , Yu Zhou, Qin Zhao, Jie Yu, Wei Liu, Xi-qiangYangDivision of Nephrology and Immunology, Children's Hospital of ChongqingMedical University, Chongqing ChinaCaifeng LI, Xiao-Hu HE, Xiao-Hua TAN, Feng-Dan YUBei Jing children’s hospital affiliated to Capital Medical University, ChinaThe clinical data of 24 children with Wiskott-Aldrichsyndrome (WAS) from 23 unrelated Chinese familieswere reviewed in the present study. WAS protein(WASP) expression in peripheral blood mononuclearcells (PBMCs) was examined by flow cytometry;WASP gene was amplified by PCR and directlysequenced to analyze mutations of the WASP gene inpatients and their female relatives. Flow cytometryanalysis of 21 cases showed that 18 cases wereWASP-negative and three had partially WASPexpression. WASP gene analysis revealed mutationsin 23 patients, including 5 missense mutations, 4nonsense mutations, 4 deletion mutations, 3 insertionmutations, 6 splice site mutations and one complexmutation, among which, 20 unique mutations weredetected, including 7 novel mutations (168 C>A,747-748 del T, 793-797del C, 1185 ins C, Dup1251-1267,1277 insA and 1266 C> G; 1267-1269delC). Five WAS children underwent stem celltransplantation. After two months of transplantation,WASP expression was restored to normal in all fivecases whereas one patient died ofcytomegalovirus-induced interstitial lung disease.WASP gene analysis can make a definite diagnosis ofWAS and identify mutation carriers, beneficial fortimely treatment and genetic counseling for childrenwith WAS.[Keywords]Wiskott-Aldrich syndrome; WAS protein; WAS proteingene; clinical phenotype; molecular diagnosisOBJECTS: To discover the Clinical and immune effectsof Etanercept used in JIA.METHOD: During the year of 2006.10~2009.9, 103severe patients of JIA were selected for this study.Before the treatment of Etanercept(0.4mg/kg a dose, 2doses of a week by subcutaneous injection), MTX andprednisone had been regularly used. The course ofEtanercept is 3 months at least. Study contents: (1)Clinical effects of Etanercept: we recorded andanalyzed the level of ACR Pedi (30, 50, 70),inflammation index (ESR, CRP, HGB, WBC, SF) ,bonedestruction and drug side effects, separately on thefollowing periods: before the treatment of Etanerceptand 2 weeks, 1 month, 3 months, 6 months, 12 months,24 months after its use.(2) Immune effects of Etanercept:before the use of Etanercept and 3 months after thetreatment, serum level of TNF-α、IL-1β、IFN-γ and IL-6were detected. SPSS11.5 was used for data statisticalanalysis.RESORTS: All of the 103 patients, 56 cases were male,the other 47 cases were female. The age were between3~14 years,average age was 8-year-old. There were 35cases of JAS, 51 cases of PolyJRA and 17 cases ofSoJRA (5 cases of MAS among SoJRA patients). (1)Clinical effects of Etanercept: After the use ofEtanercept, all the patients felt better, and the ACR Pediwere improved significantly. Inflammation index of mostpatients were decreased significantly (P

P2-007 Allergy/Immunology/Rheumatology P2-008 Allergy/Immunology/RheumatologyCHANGE IN PLAMSA LEVEL OF SOLUBLEE- SELECTIN AND+A561C POLYMORPHISMSIN CHILDREN WITH KAWASAKI DISEASEZENG Hua-song, CHEN gang, YU ming-hua, et alGuangzhou children's Hospital, Guangzhou, ChinaOBJECTIVE: To investigate the correlationbetween E-selectin. E-selectin A561C polymorphismand kawasaki disease (KD).METHODS: The plamsa level of E-selectin wasdetermined by ELISA. PCR-RFLP method was usedto determine +A561C locus mutation polymorphismsin E-selectin gene The gene polymorphisms wereconfirmed by sequenceing.RESULT: ES levels in the acute phase group(193±29 ng/ ml), subacute phase group (150±48 ng/ml) were significantly higher than those in thecontrol group( P < 0.01). The peak level of ES(193±29 ng/ ml) appeared in the acute phase. PlasmaES levels of CAL group were significantly higherthan those of NCAL group in the acute phase.(226±36vs158±31 ng/ ml) (P < 0.01).E-selectin gene+A561Cpolymorphism was exiting in children, thefrequency of E-selectin AA genotype was the highestamong E-selectin genotypes (92.5%), ACgenotype(7.5%), and these gene polymorphisms hadno difference between male and female (P>0.05).The allele frequencies of +A561C site of E-selectingene have no significant difference between childrenwith KD and normal controls, also between CAL andNCAL .There were no significant differences in thelevels of ES among different genotypes.CONCLUSION: E-selectin may potentially be apredictor of CAL in children with KD. AlthoughE-selectin levels were significantly elevated in theacute stage of KD, +A561C polymorphism do notconfer a relevant role in the susceptibility or CALdevelopment of KD.[Keywords]E-selectin;Kwasaki disease;Single NucleotidePolymorphismsPRODUCTION OF INTERLEUKIN-4,INTERLEUKIN-10, AND INTERFERON-Γ FROMCD4+ T CELLS AND CD8+ T CELLS INPATIENTS WITH ASTHMAJong Seo Yoon, Su Jung Kim, Eugene Kim, Hyun Hee Kim,Jin Tack Kim, Jin Han Kang, Joon Sung LeePediatrics, Kangnam St. Mary’s Hospital, The Catholic University of Korea,KoreaPURPOSE: T cells play an important role in thepathophysiology of asthma and control the activity ofother inflammatory cells in the airway by the release ofcytokines. It has been thought that CD4+ T cells aremostly involved in the exacerbation of asthma, andCD8+ T cells are involved in the suppression of airwayhyperresponsiveness and inflammation. However, recentstudies have reported that CD8+ T cells produce type 2cytokines and cause inflammatory response in theairway in asthma patients. In this study, house dust miteandvirus-stimulated cytokine production in T cells wasexamined.METHODS: Whole blood samples were obtained fromhealthy control and asthma patients, and peripheralblood mononuclear cells (PBMC) were separated. ThePBMC were exposed to Dermatophagoides farinae (derf1), respiratory syncytial virus, rhinovirus, andadenovirus; the number of CD4+ and CD8+ Tcells—which produce interleukin-4, interleukin-10, andinterferon-γ—were determined using flow cytometry.RESULTS: When stimulated with der f1, the productionof interleukin-4 by CD4+ T cells and CD8+ T cells washigher in asthma patients than in healthy controls. Whenexposed to der f1 or viruses, the production ofinterferon-γ by CD8+ T cells was higher in healthycontrols than in asthma patients. Production ofinterleukin-10 was not found in both groups.Conclusion: It is possible that CD8+ T cells are involvedin airway inflammation with an increase in interluekin-4production and decrease in interferon-γ production inasthma patients.258

P2-009 Allergy/Immunology/Rheumatology P2-010 Allergy/Immunology/RheumatologyCLINICAL FEATURES AND PERFORIN A91VGENE ANALYSIS IN SO-JIA CHILDRENWITH MACROPHAGE ACTIVATIONTHE IMMUNOLOGICAL ALTERATION ANDGENE MUTATIONS IN COMBINEDIMMUNODEFICIENCYSYNDROMEZeng Huasong 1,2 *,Chen Xiangyuan 2 *,XiongXiaoyian 2 *,Wei Yandan 2 *, Luo Xiaoping 1§1 Pediatric Department, Tongji Hospital, Tongji Medical College, HuazhongUniversity of Science and Technology, Wuhan, Hubei,China; 2 Departmentof Allergy,Immunology and Rheumatology, Guangzhou Children’sHospital ,Guangzhou Woman and Children’s Medical Center,GuangzhouMedical College,Guangzhou ,Guangdong,China; Supported Grants werefunded by the Science and Technology Commission Research Grants ofGuangzhou City Governments(2003Z2-E0181, 2005Z1-E0104), theScience and Technology Commission Research Grant of GuangdongGovernment(2003B30503), the Science and Technology CommissionResearch Grants of National Ministry of Personnel(200499),PRCBACKGROUND: Macrophage activation syndrome(MAS) is a severe, potentially life-threateningsyndrome.Here we aim to review the clinical featuresincluding precipitating events, clinical features, treatment,outcome and perforin A91V gene analysis in systemiconset juvenile idiopathic arthritis (SoJIA) children withMAS.METHODS: Retrospective review of cases of MAS froma collected database of fourteen children with SoJIA from2003 to 2008. Gene-specific polymerase chain reaction(PCR) primers were used to analyze the perforin A91Vgene polymorphism.RESULTS: Fourteen patients(nine boys) were consideredto have evidence of MAS, with age ranged from 4 monthsto 12 years.The primary diagnosis was systemic onsetjuvenile idiopathic arthritis.No medication was identifiedas trigger. Eleven had infections prior to MAS, specificinfectious agents were identified in four. High fever, newonset hepatosplenomegaly, lymphadenopathy, liverdysfunction, abnormal lipid metabolism andhemophagocytosis were common clinical features.Twocases were with acute respiratory distress syndrome(ARDS), multiple organ failure (MOF) in three and threedied. The perforin A91V (NCBI: SNP rs35947132) variantgene was detected in seven systemic onset juvenileidiopathic arthritis compolicated with MAS cases, but nomutation were found..Glucocorticoid, intravenousimmunoglobulin, immunoimpressive therapy wereeffective and HP (Plasmapheresis) used in one serious casewas also effective.CONCLUSIONS: MAS is a rare and potentially fatalcomplication of childhood rheumatoid diseases, especiallysystemic onset juvenile idiopathic arthritis. Most of ourpatients were male, and most cases were preceded byinfection. Bone marrow studies support the diagnosis.MOF may be a poor prognostic sign.Aggressive earlytherapy is essential.[Keywords] perforin, macrophage, activation, syndrome,juvenileidiopathic arthritisYing-Ying Jin 1 , Rui-Ming Cao 2 , Tong-Xin Chen 1,21 Department of Pediatrics, Xinhua Hospital, Shanghai Jiao Tong UniversitySchool of Medicine, Shanghai, China; 2 Department of Immunology/Oncology,Shanghai Institute for Pediatric Research, Shanghai Jiao Tong University Schoolof Medicine, Shanghai, ChinaOBJECTIVE: The aim of this study was to investigate theimmunological features and characteristic of gene mutation inChinese patients with combined immunodeficiency to furtherensure the early diagnosis and therapy for children suffering fromcombined immunodeficiency.METHODS: Case histories were analyzed repeatedly to graspimportant characteristics of the diseases. Distribution oflymphocyte subsets from peripheral blood and the level of CD40Lexpressing on activated CD3+CD8- T lymphocyte surface wereexamined by flow cytometry. Amplify and identify exons fromgenes encoding CD40L and IL-2Rγc by PCR and agarose gelelectrophoresis, and then followed by gene sequencing.MAIN RESULTS: Among 124 patients with primaryimmunodeficiency diseases, 14 (11.29%) were diagnosed ascombined immunodeficiency. Four of them were X-linked HyperIgM Syndrome (XHIGM), among which three has gene diagnosis:two deletion mutation, one insertion mutation. All the four patientswhose clinical manifestations included recurrent dental ulcer,persisitent neutropenia and arthritis had obviously decreased serumIgG and IgA level, increased but one within normal range serumIgM level. All of them showed normal distribution of lymphocytesubsets, and obviously decreased CD40L expression level. Tenpatients were diagnosis as severe combined immunodeficiency(SCID), eight belonged to X-linked kind (XSCID) including fourgene diagnosis: two missense mutation, one deletion mutation andone deletion mutation combined with insertion mutation. The restbelonged to none X-linked type. The ten patients whose clinicalmanifestations included recurrent upper respiratory infection andpneumonia, thrush and other kinds of fungous infections andGVHD had obviously all kinds of serum immunoglobulin levelsdecreased. All XSCID patients showed obviously decreasedpropotion of T and NK cells but increased B cells. One noneXSCID patient showed decreased propotion of T cells and B cellsbut increased NK cells while the other one had decreasedpropotion of all kinds of lymphocyte subsets.CONCLUSIONS: For male patient suspected with combinedimmunodeficiency primary diagnosis could be made on the basisof early recurrent infection involved respiratory and alimentarysystem, with no satisfactory response to antibiotic, within orwithout the same cases occurred in maternal family history,combined with certain changes in all kinds of serumimmunoglobulin level and absolute lymphocyte counts. Thensequent secreening methods of XHIGM and SCID will be needed,including analyzing the distribution of lymphocyte subsets fromperipheral blood and the level of CD40L expressing on activatedCD3+CD8- T lymphocyte surface by flow cytometry. Finaldiagnosis could be conducted by gene sequencing.[Keywords]immunological alteration; gene mutations; combinedimmunodeficiency259

P2-011 Allergy/Immunology/Rheumatology P2-012 Allergy/Immunology/RheumatologyGENETIC DIAGNOSIS FOR PRIMARYIMMUNODEFICIENCIES: A SINGLE CENTEREXPERIENCE IN CHINATong-Xin Chen, 1,2 Xi Wang 2 , Rui-Ming Cao 2 ,Ying-Ying Jin 1 , Hui Zhang 2 , Juan-Juan Wang 2 ,Chun-Mei Yao 2 , Yu-Lung Lau 31 Department of Pediatrics, Xinhua Hospital, Shanghai Jiao TongUniversity School of Medicine, Shanghai, China; 2 Department ofImmunology/Oncology, Shanghai Institute for Pediatric Research, ShanghaiJiao Tong University School of Medicine, Shanghai, China; 3 Department ofPaediatrics and Adolescent Medicine, LKS Faculty of Medicine, TheUniversity of Hong Kong, HKSAR, ChinaMAPPING OF HUMAN IGE AND IGG1-4ANTIBODY-BINDING EPITOPES IN CYN D 1,THE MAJOR ALLERGEN OF BERMUDA GRASSPOLLENHan-Chih Yuan 1,2 , Ho-Jen Peng 1,2 , Keh-Gong Wu 1Department of Pediatrics 1 , Medical Research and Education 2 , Taipei VeteransGeneral Hospital, TaiwanOBJECTIVE: Our study aims to review the clinicalfeatures and genetic diagnoses of 218 patients withprimary immunodeficiencies refered to our center from2004-2009.METHODS: The genomic DNA of patients and theirfamily members were detected by PCR-directedsequencing of corresponding pathogenic genes of thesePID patients based on their clinical manifestations andimmunological evaluations.MAIN RESULTS: Our cohort included 201 males and 17females from 20 provinces in China. Most of themcharacterized by recurrent respiratory infections, diarrheaand skin infections, 29 of them were found withautoimmnune disease(JRA 13, ALPS 2, SLE 1, colitis 9,neutropenia 6) and 4 with tumor. Fourteen genessequencing had been done including BTK, CD40L, AID,TACI, μheavy chain, IL-2RG, PNP, WASP, IL-12RB1,SH2D1A, Fas, FasL, CYBB, CD18 and 52 types mutationsdefined with 34 family members confirmed to be carriers.Forty-eight males were found to have BTK mutationsamong 54 patients suspected to be XLA including 14missense, 3 multiple, 5 deletion and 6 splice-junctionmutations. Four males had CD40L mutation including 3deletion and 1 insertion. Four males had IL-2RG mutationincluding 1 missense, 1 multiple and 2 splice-junctionmutations. Eight males had WASP mutations including 5deletion, 2 missense, 1 duplication and 1 splice-junctionmutations. Two males had SH2D1A mutations amongwhich 1 with nonsense and 1 with multiple mutations.IL-12RB1 mutation (compound heterozygous missensemutation) was found in a patient with BCG disease. CYBBgene mutation was found in a patient with disseminatedBCG but his mother was normal and spontaneous mutationmay be the cause. CD18 mutation (missense) was found ina male and his father and mother defined to be carriers.CONCLUSIONS: Most patients in out cohort were malewith higher mortality for those as delayed diagnosis andsevere infections. Genetic diagnosis can provide earlydiagnosis and therapy for children suffering from PID.Finding carrier are necessary for avoiding birth of PIDpatients.[Keywords] genetic diagnosis; PIDOBJECTIVES: Bermuda grass pollen (BGP) is animportant aeroallergen worldwide which induces allergicrhinitis and asthma. The positive rate of skin tests to thecrude BGP extract was about 27% in asthmatic childrenin Taiwan. Cyn d 1 is the major allergen of BGP. Its IgEand IgG1-4 antibody-binding epitopes werecharacterized for the first time in the present study.METHODS: Synthetic peptides (10-mers; 5overlapping residues) spanning the entire length of Cynd 1 were used to map specific IgE and IgG1-4antibody-binding epitopes in the sera of BGP allergicchildren. For further mapping of exact epitopes, 9overlapping peptides were further employed. Eachoverlapping peptide was stepwise different from theprevious peptide by one residue, upstream ordownstream. The essential amino acids for specific IgEand IgG1-4 antibody binding within each epitopes wereexamined by single amino acid substitution with alanine.Epitopes of BGP isoforms were also included tocompare their differences among IgG 1-4 and IgEantibody binding.MAIN RESULTS: Based on the results of peptidebinding in the sera of 22 patients, three IgE bindingepitopes (amino acids 101-110, 161-170 and 171-180)and three IgG epitopes (amino acids 71-80, 146-155 and191-200) were selected for further epitope mapping ofCyn d 1. Three IgE-binding epitopes were positioned atamino acids 101-110, 159-167 and 172-181, while threeIgG-binding epitopes were positioned at amino acids70-79, 144-153 and 192-200. The epitopes for IgG1,IgG2, IgG3, and IgG4 were identical. Critical aminoacids for IgE- and IgG-binding epitopes were alsocharacterized. In addition, both epitopes for twoisoforms of Cyn d 1 (1 for IgG and 1 for IgE) had strongantibody-binding capacity.CONCLUSIONS: This is the first study to identify theepitopes of IgE and IgG subclasses in Cyn d 1. It will behelpful for future development of safer immunotherapyand better diagnostic kits.[Keywords]Cyn d 1, antibody-binding epitope, synthetic peptide, dotimmunoblotting260

P2-013 Allergy/Immunology/Rheumatology P2-014 Allergy/Immunology/RheumatologyEXPLORING THE RELATIONSHIPBETWEEN GASTRO SCOPE OFHENOCH–SCHONLEIN PURPURA ANDEFFECT OF PGI 2 ANALOGUE ON TH1- ANDTH2-RELATED CHEMOKINES IN MONOCYTESVIA EPIGENETICSHELICOBACTER PYLORI INFECTION INCHILDRENZeng HuaSong, LiFengAllergy,Immunology and Rheumatology, Guangzhou Children'sHospital,Guangzhou Woem and Children's Medical Center, ChinaOBJECTIVE: To study the relationship ofGastroscope of Henoch–Schonlein Purpura andHelicobacter pylori infection.METHODS: 87 cases of Henoch–Schonlein Purpurawho hospitalized from Oct. 2006 to Oct. 2008. Allcarry on the electric gastro scope check and detectHelicobacter pylori.RESULTS: The endoscopic findings includecongestion, edema, splotch hemorrhage, erosion etal. The gastro scope show that the commonestpositions involved were duodenum descendent andbulb. The infection rate in sever group with HP wassignificantly higher than those of not serious group.CONCLUSIONS: Helicobacter pylori infectionmay be one of the causes in Henoch–SchonleinPurpura, and it was in correlation with degree ofmucosa disease.[Keywords]Henoch–Schonlein Purpura; Gastro scope;Helicobacter PyloriChang-Hung Kuo, M.D. 1 , San-Nan Yang, M.D., Ph.D. 1,2,3,4 ,Yu-Te Chu, M.D. 1 , Wei-Li Wang, M.D. 1 , Shau-Ku Huang,Ph.D. 5 ,Yuh-Jyh Jong M.D., Ph.D. 1,2,3,4 and Chih-HsingHung, M.D., Ph.D. 1,2,3,41 Department of Pediatrics, Kaohsiung Medical University Hospital, KaohsiungMedical University, Kaohsiung, Taiwan; 2 Center of Excellence for EnvironmentalMedicine, Kaohsiung Medical University, Kaohsiung, Taiwan, 3 Department ofPediatrics, Faculty of Medicine, College of Medicine, Kaohsiung MedicalUniversity, Kaohsiung, Taiwan; 4 Graduate Institute of Medicine, KaohsiungMedical University, Kaohsiung, Taiwan; 5 Johns Hopkins Asthma and AllergyCenter, Johns Hopkins University School of Medicine, Baltimore, MD 21224OBJECTIVES: Chemokines play important roles inasthma. Prostaglandin I 2 (PGI 2 ) analogue is regarded as apotential treatment for asthma. The effects of PGI 2analogues on expression of Th1- and Th2- relatedchemokines in monocytes is unknown.MATERIAL AND METHODS: The human monocyteswere pretreated with iloprost and treprostinil, two PGI 2analogues, before stimulation with lipopolysaccharide(LPS). Th1-related chemokine interferon-γ-inducibleprotein-10 (IP-10/CXCL10) and Th2-related chemokinemacrophage-derived chemokine (MDC/CCL22) weremeasured by ELISA. Intracellular signaling wasinvestigated by western blot and chromatinimmunoprecipitation.RESULTS: PGI 2 analogues enhanced MDC, butsuppressed IP-10, expression induced by LPS inmonocytes. These effects were reversed by an Iprostanoid receptor antagonist, CAY10449. Forskolin, acAMP activator, conferred similar effects. PGI 2analogue-enhanced LPS-induced MDC expression wasreduced by nuclear factor (NF)-κB inhibitor, BAY117085, and mitogen-activated protein kinase(MAPK)-p38 inhibitor, SB203580. PGI 2 analoguesenhanced LPS-induced pp65 and pp38, but suppressedpERK expression. Iloprost enhanced H3 acetylation atMDC promoter area, and suppressed H3K4, H3K36 andH3K79 trimethylation at IP-10 promoter area inLPS-stimulated monocytes.CONCLUSION: PGI 2 analogues suppressedLPS-induced IP-10 expression via IP-receptor-cAMP,ERK-MAPK pathways and histone trimethylation, butenhanced MDC expression via IP-receptor-cAMP,p65-NFκB, p38-MAPK pathways and histoneacetylation. These effects should be considered for PGI 2analogues as treatment for asthma.[Keywords] prostaglandin I 2 , epigenetics, interferon-γ-inducibleprotein-10, macrophage-derived chemokine, monocytes261

P2-015 Allergy/Immunology/Rheumatology P2-016 Allergy/Immunology/RheumatologyROLE OF HLA-DRB1 ALLELICPOLYMORPHISM IN JUVENILE IDIOPATHICARTHRITIS IN CHINESE PATIENTSZENG HUA SONG**,WU JUAN***Department of Allergy, Immunology and Rheumatology, Guangzhouchildren’s hospital, Guangzhou, China, 510120A PILOT STUDY EVALUATING THETHERAPEUTIC EFFECTS AND SAFETY OF ANORALLY ADMINISTERED EXTRACT OFDENDROBIUM HUOSHANENSE FOR SEVERERECALCITRANT ATOPIC DERMATITIS INCHILDRENOBJECTIVE: To investigate the predisposing allelesof HLA - DRB1 genes in Juvenile idiopathic arthritis.METHODS: Polymerase chain reaction - specificsequence primers (PCR - SSP) method was used totype HLA -DRB1 subregion in 94 cases of patientswith Juvenile idiopathic arthritis and matched controlsubjects of Han nationality in Guangdong province.RESULTS: The frequency of HLA-DRB1*08(Pc:0.0049、OR:2.20) allele in patients with Juvenileidiopathic arthritis was significantly higher than thatin controls, and the frequency of HLA-DRB1*12(Pc:0.0488、OR:0.60)allele was significantly lowerthan that in controls. According to the diffenrent JIAisoforms, We found that in systemic JIA subset(Pc:0.0130、OR:2.37)and polyarthritis JIA subset(Pc:0.0222、OR:2.73),the allete HLA-DRB1*08were expressed most commonly,In systemic JIAsubset(Pc:0.0293、OR:0.39)and oligoarthritis subset(Pc:0.0352、OR:0.15),the allete HLA-DRB1*15was expressed lower frenquent than the control,whatever, in polyarthritis JIA the alleteHLA-DRB1*13(Pc:0.0214、OR:2.74) was expressedhigher frenquent than the control.CONCLUSION: HLA-DRB1*08 may be thesusceptible alleles of Juvenile idiopathic arthritis inHan Guangdong Chinese, HLA-DRB1*12 may bethe protective alleles of Juvenile idiopathic arthritisin Han Guangdong Chinese. The HLA-DRB1*08was the susceptible alleles in systemic JIA andpolyarthrits.HLA-DRB1*13 was also the susceptiblealleles in systemic JIA, HLA-DRB1*15 was theprotective alleles in systemic JIA and oligoarthritis.[Keywords]Juvenile arthritis idiopathic; HLA - DRB1; PCR - SSP;isoformTsung-Han Li, Chun-Jen Chen, Chiao-Wei Lo, Chun-MingChen, Hao-I Cheng, Keh-Gong WuDepartment of Pediatrics, Taipei Veterans General Hospital and NationalYang-Ming University, Taipei, Taiwan.BACKGROUND: Atopic dermatitis (AD) is a commoninflammatory skin disorder for which few safe andeffective systemic treatments are available.OBJECTIVES: To test the safety and effectiveness ofextract of Dendrobium huoshanense for the treatment ofsevere recalcitrant AD in children in a pilot, case seriesstudy.PATIENTS: Twenty-seven patients with AD who hadnot responded to topical therapy were enrolled.METHODS: Patients were treated with crude extractderived from Dendrobium huoshanense (1000 mg pertablet) twice daily (total 2000 mg per day) (body weight

P2-017 Allergy/Immunology/Rheumatology P2-018 Allergy/Immunology/RheumatologyTHE ASSOCIATION OF SKIN TESTREACTIVITY TO COW`S MILK PROTEINJUNVENILE SCLERODERMA-EXPERIENCE INONE INSTITUTIONAND/OR SOY PROTEIN WITH THESEVERITY OF ATOPIC DERMATITIS AMONGINFANTS AGED 1-6 MONTHS OLDMelanie Yagdulas GavinoPediatrics, De La Salle University Health Sciences Institute, PhilippinesOBJECTIVE: To determine the association of skin testreactivity to Cow’s Milk Protein (CMP) and/or Soy Protein(SP) with Atopic Dermatitis (AD) in infants aged 1 to 6months old.STUDY DESIGN: Hybrid Design, specifically aFollow-up Prevalence Study.SETTING: Cavite, Philippines.PARTICIPANTS: Infants aged 1 to 6 months olddiagnosed with AD using the Hanifin and Rajka criteria.OUTCOME MEASURE: Severity of atopic dermatitisusing Scoring Atopic Dermatitis.METHODS: All infants who fulfilled the diagnosticcriteria of AD underwent allergy skin prick test (SPT) toCMP, SP, a positive and a negative control. Severity of ADwas assessed using the SCORAD index. Statistical analysiswas performed using SPSSPC+ and Epi-Info softwares.Chi-square was used to determine if there was statisticallysignificant association between skin test reactivity to CMPand/or SP and the severity of atopic dermatitis, familyhistory of atopy and feeding. T-test was used todetermine the association between skin test reactivity andMean SCORAD. Spearman`s rank correlation coefficientwas computed to determine the correlation of severity ofatopic dermatitis with the number of positive skin pricktest results.RESULTS: There were a total of 54 participants. Mostwere males with a mean age of 3 months. More than 2/3was with mild to moderate atopic dermatitis (77.8%).Majority were positive to skin prick test (66.7%). Theproportion of skin test reactivity to CMP and SP amonginfants with AD was at 48.2% and 50%, respectively.There was a significant association between the skin testreactivity to CMP and/or SP and the severity of AD.Statistical analysis showed that there was a significantassociation between skin test reactivity and family historyof AD. There was no association between skin testreactivity and whether the patient is on pure milk formulaor mixed feeding. A positive direct relationship betweenseverity of AD and number of skin test reactivity was alsonoted.CONCLUSION: Although AD is viewed by many as aminor dermatologic problem, the stress that it poses to thefamily and economy cannot be ignored. The resultsshowed a higher proportion of positive skin test reactivityto CMP and SP among AD infants as compared tointernational studies. There was a significant associationbetween skin test reactivity to CMP and/or SP and severityof AD. This study supports the advocacy of the AAPregarding the importance of breastfeeding.[Keywords]atopic dermatitis, skin test reactivity, Scoring AtopicDermatitis (SCORAD)Chia-Yi Lo, Shyh-Dar Shyur, Szu-Hung Chu, Li-HsinHuang, Yu-Hsuan Kao, Wei-Te Lei, Chieh-Han Cheng,Kuo-Hsi Lee, Chen-Kuan Chen, Ling-ChunLiuDepartment of Pediatrics, Mackay Memorial Hospital, Taipei, TaiwanOBJECTIVE: Scleroderma is a chronic connective tissuedisease characterized by sclerodermatous skin changes andwidespread abnormalities of the viscera, which are rare inpediatric age group. In this study, we retrospectivelyreviewed 22 pediatric patients with systemic and localizedscleroderma.MATERIALS AND METHODS: Twenty-two patientsdiagnosed as systemic and localized scleroderma wereenrolled in our study, from March 1993 to September 2009in Department of Pediatrics, Mackay Memorial Hospital,Taipei, Taiwan. The diagnosis is made based on the criteriaof American College of Rheumatology and clinicalmanifestations of hard skin involvement. Data extractedfrom the records includes gender, age at onset, age atdiagnosis, clinical manifestations, laboratory data, familyhistory, trauma history, treatment, and the outcome.RESULTS: Three patients had systemic scleroderma.Nineteen patients had localized scleroderma. The ratio ofLS and SSc is 6.3:1. There were 15 females and 7 males(female to male ratio 2.1:1). The mean age at diagnosis was8.90±3.41 years. The mean age at onset was 6.23±2.98years. Antinuclear antibodies were positive in 15 at titers of40X to 10240X; 2 had a nucleolar pattern, 7 had a speckledpattern, 4 had a homogenous pattern, and 2 had aspeckled-to- homogeneous pattern. Tests for anti-SCL70antibodies were positive in only 1 patient with systemicscleroderma. Tests for anti-double-stranded-DNA were allnegative. Serum levels of rheumatoid factor ranged from

P2-019 Allergy/Immunology/Rheumatology P2-020 Allergy/Immunology/RheumatologySCREENING OF THE BRUTON TYROSINEKINASE (BTK) GENE MUTATIONS IN 16CHINESE PATIENTS WITH CONGENITALAGAMMAGLOBULINEMIAIDENTIFICATION OF CYBB GENE MUTATIONSIN 11 CHINESE BOYS WITH X-LINKED CGDAND NCF1 GENE MUTATION IN A GIRL WITHAUTOSOMAL RECESSIVE CGDZhao Xiaodong, Zhang Zhi-yong, Jiang Li-ping, WangMo,Yu Jie, An Yu-fei, Yang Xi-qiangDivision of Nephrology and Immunology, Children's Hospital of ChongqingMedical University, Chongqing ChinaX-linked agammaglobulinemia (XLA) is a humoralprimary immunodeficiency in which affectedpatients have very low levels of peripheral B cellsand a profound deficiency of all immunoglobulinisotypes. Mutations in the gene encoding forBruton’s tyrosine kinase (Btk) are responsible formost of the agammaglobulinemia. In this study weinvestigated 16 male patients with XLA-compatiblephenotype from 16 unrelated Chinese families.Eleven different mutations were identified in 12patients from 12 unrelated families and no mutationof BTK gene was found in four patients. Fourmutations had been reported previously includingone gross deletion (del 722-2041), one missensepoint mutation (1764G>T) and two nonsensemutation (194C>A, 895 C>T) .Seven novel mutationwere identified (504delG, 537delC, 1081T>C,1637G>A, 1878T>C, IVS9+2T>C, IVS15-2A>G).Five of the eleven mutations were located in thekinase domain, three were pleckstrin homologyfunctional area, two were Src homology 3 regionsand one was Src homology 2 regions respectively.The results of this study further support the notionthat molecular genetic testing represents animportant tool for definitive and early diagnosis ofXLA and may allow accurate carrier detection andprenatal diagnosis. Those patients without mutationidentified in the BTK gene may require a furthergenetic analysis on autosomal resseive bases.[Keywords]X-linked agammaglobulinemia (XLA); Bruton’styrosine kinase (BTK); Molecular diagnosis; Mutation analysisLi-Ping Jiang, Shu-Juan Li, Wei Liu, Xiao-Dong Zhao,Xi-Qiang YangChildren's Hospital of Chongqing Medical University, Chongqing 400014, ChinaOBJECTIVES: Molecular identification and clinicalcharacterization of genetic mutations in 11 Chinese boyswith X-linked chronic granulomatous disease (XCGD)and a girl with autosomal recessive (AR) -CGD.METHODS: Genomic DNA or cDNA from 11 male and1 female patients with CGD and their family members,based on clinical history, clinical examination, andspecific granulocyte function tests were amplified bypolymerase chain reaction (PCR) for sequences of theCYBB gene or NCF1 gene, encoding gp91-phox orp47-phox, respectively. The Genomic DNA from fetusamniotic fluid cells, whose mother was a carrier ofmutant CYBB gene, was amplified CYBB gene.Mutations in the resulting PCR products were identifiedby DNA sequencing.RESULTS: Sequence analysis revealed 11 kinds ofCYBB gene mutations were found in XCGD patients,including four deletion mutations, three splicing errors,two nonsense mutations, a missense mutation and aninsertion mutation. Four of these were novel mutations,including deletion mutations (1341delT), splicing errors(IVS10 +2 dup T), missense mutation (591T>C) andinsertion mutation (497-498ins115bp). The femalepatient with AR-CGD had a homozygous deletionmutation (73-74delGT) in NCF1 gene, which had beenreported, but was the first case in mainland china.Except for the mother of the patient with a nonsensemutation, other mothers were all carriers. The fetus hadnormal CYBB gene.CONCLUSIONS: We identified eleven mutations andfour novel mutations in CYBB gene in 11 XCGDpatients, and the first reported the AR-CGD withhomozygous the NCF1 gene deletion mutation in aChinese girl. Genetic diagnosis has provided valuableevidences for identification of CGD patients andcarriers. Early diagnosis and proper treatment cansignificantly improve the survival status and quality ofCGD patients.[Keywords]CYBB, NCF1, Mutation, CGD264

P2-021 Allergy/Immunology/Rheumatology P2-022 Allergy/Immunology/RheumatologyTHE ASSOCIATION STUDY OF ACE GENEPOLYMORPHISM AND LOBAR PNEUMONIAIN TAIWANChan Sheng Liu, Guan Yi Lue, Min Sho Ku, Hai LunSun, Ko Huang LuePediatrics, Chung Shan Medical University Hospital,TaiwanBACKGROUND: Pneumonia occurs in all age groups butis more common in children. Studies have shown thatangiotensin-converting enzyme (ACE) inhibitors maylower the risk of developing pneumonia by inducing thecough reflex through inhibition of the degradation of theprotussive peptides bradykinin and substance p. Aninsertion/deletion polymorphism of the ACE gene has beenshown to be associated with serum ACE levels. The DDgenotype was associated with the highest plasma levels,and II genotype with the lowest levels. Recent studies havesuggested the involvement of the angiotensin-convertingenzyme (ACE) insertion/deletion (I/D) polymorphism inthe susceptibility to and severity of community-acquiredpneumonia (CAP) in Asian populations. One study showedthat the ACE D allele is an independent risk factor for(fatal) pneumonia in an Asian population. However, otherstudies showed that ACE I/D polymorphism is notassociated with risk and outcome of CAP in the Dutchwhite population. From previous studies, we found that thethe role of ACE gene I/D polymorphism in pneumoniaremain to be established. We hypothesized that the ACEI/D polymorphism might be associated with lobarpneumonia formation in children if they have lunginfection, because children with the DD genotype have alower cough reflex. The objective of our study was todetermine whether this polymorphism is associated withlobar pneumonia formation in Taiwanese children.METHOD: 40 unrelated children (age 6-14 y/o) withlobar pneumonia were recruited from our pediatric wards.102 age matched children without pneumonia history wererecruited from 3 elementary schools as control groups.History taking and physical examination were arranged inboth groups. General lab data and blood culture wereexamined in lobar pneumonia group. Restriction fragmentlength polymorphism were used to characterize ACEgenotype.RESULT: Compare the incidence of genotypes DD, ID, IIbetween lobar pneumonia group and control group, wefound that the frequency of different genotypes betweenlobar pneumonia group and control group was notstatistically different (p = 0.857, Odds ratio = 1.289, 95%confidence interval was -0.8582~ 2.6182).CONCLUSION: From the result of our study, we foundthat there is no relationship between lobar pneumoniaformation and ACE gene polymorphism in Taiwanchildren. The association of ACE gene polymorphism andlobar pneumonia formation were not reported by otherstudy, so we first report the finding.[Keywords] lobar pneumonia, ACE Gene Polymorphism, childrenEFFECT OF PROCATEROL ON TH2-RELATEDCHEMOKINES PRODUCTION IN HUMANMONOCYTE AND BRONCHIAL EPITHELIALCELLSChing-Hua Huang, MD 1 ,Yu-Te Chu, MD 3,4 ,Chang-HungKuo, MD 3 ,Wei-Li Wang, MD 3 ,Min-Sheng Lee,MD 3 ,Chih-Hsing Hung, MD, PhD 2,3,4*1 Department of Pediatrics, Yuan’s General Hospital, 2 Department of Pediatrics,Faculty of Pediatrics, College of Medicine, Kaohsiung Medical University,3 Department of Pediatrics, Kaohsiung Medical University Hospital, KaohsiungMedical University, 4 Graduate Institute of Medicine, Kaohsiung MedicalUniversity, TaiwanOBJECTIVE: β2-adrenoceptor agonists are widely used asbronchodilators in the treatment of asthma and may haveanti-inflammatory activity. Chemokines play a central role in thepathogenesis of airway inflammation in asthma. However, theeffects of β2-adrenoceptor agonists on chemokines are uncertain.We try to identify the effect of procaterol, a shortactingβ2-adrenoceptor agonist, on Th2 and Th1-relatedchemokines in monocytes and bronchial epithelial cells as well asthe modulation mechanisms.METHODS: We investigated whether procaterol could suppresslipopolysaccharide (LPS)-induced Th2-related(macrophage-derived chemokine (MDC)/CCL22 and I-309/CCL1)and Th1-related chemokines (monokine induced by IFN-gamma(Mig)/CXCL9 and interferon-inducible protein 10(IP-10)/CXCL10) production of THP-1 cells and human primarymonocytes. The effect of procaterol on thymus- andactivation-regulated chemokine (TARC)/CCL17 in a humanbronchial epithelial cell line (BEAS-2B) was also evaluated.NF-κB and MAPK inhibitors were used to verify the intracellularpathways. Propanolol and ICI-118551 were used as antagonist ofprocaterol. Etazolate, a phosphodiesterase 4 inhibitor, was used toverifyβ2-adrenoceptor-cAMP pathway. Besides, chromatinimmunopricipitation assay was performed to detect the histonemodification on TARC promotor region.RESULTS: MDC and I-309 production of both THP-1 cells andprimary human monocytes and TARC expression of BEAS-2Bcells were significantly inhibited by procaterol. In contrast,procaterol did not significantly suppress Mig and IP-10 expressionby THP-1 cells. MDC production in monocytes is associated withNF-κB and MAPK signaling pathways, especially p38- and JNKMAPKs. Inhibition of MDC production by procaterol was reversedby propanolol and ICI-118551. The etazolate could block theexpression of MDC by THP-1 cells and TARC of BEAS-2B cells.The CHIP assay revealed the trimethylation at H3K4 at the TARCpromoter region was decreased by procaterol in BEAS-2B cells.CONCLUSIONS: Procaterol at physiologic concentrations couldinhibit Th2-related chemokines in human monocytes and bronchialepithelial cells. The effect might be mediated through NF-κB, p38and JNK MAPK pathways. Meanwhile, theβ2-adrenoceptor-cAMP pathway might be also involved. Mostimportantly, procaterol decreased H3K4 trimethylation at TARCpromoter region in bronchial epithelial cells.[Keywords] procaterol; Th2-related chemokines; monocytes; bronchialepithelial cells; epigenetic regulation.265

P2-023 Allergy/Immunology/Rheumatology P2-024 Allergy/Immunology/RheumatologySODIUM SULFITE AFFECTS AIRWAYFUNCTION IN MITE-ALLERGENROLE OF TH17 AND IL-6 IN THE ACUTE PHASEOF KAWASAKI DISEASESENSITIZED BALB/C MICEMing-Chin Tsai 1 , Lin-Shien Fu 2Department of Pediatrics, Taichung Veterans General Hospital, The TaiwanPediatric Association 1 , Department of Pediatrics, Taichung VeteransGeneral Hospital, Taiwan 2Li-Ping Jiang, Wen-Ting Shen, Wei Liu, Xi-Qiang YangChildren's Hospital of Chongqing Medical University, Chongqing 400014, ChinaOBJECTIVE: Sodium sulfite (Na 2 SO 3 ), one of themain air pollutants, is associated with manyrespiratory diseases, such as bronchial asthma. Manystudies have shown that sodium sulfite may play animportant role in airway inflammation, respiratoryepithelial damage and bronchialhyperresponsiveness. The goal of our study was toinvestigate the influence of sodium sulfite on airwayfunction in mite-allergen sensitized murine model.METHODS: BALB/c mice were separated into (1)control: no mite sensitization or any treatment; (2)sodium sulfite intranasal (sIN) group: sodium sulfitewas given intranasally for 22 days (days 1-22); (3)mite intranasal (mIN) group: mite allergen wasadministered intranasally for 8 days (days 15-22); (4)sodium sulfite and mite intranasal (sIN+mIN) group:sodium sulfite (days 1-22) and mite allergen (days15-22) were both administered intranasally. The micein experimental groups were subcutaneouslyimmunized twice with mite allergen on Day 1 andDay 8. The murine airway responsiveness to aerosolmethacholine (Mch) at increasing concentrations of 0(baseline), 6.25 and 12.5 mg/ml was assessed on day22 with unrestrained whole-body plethysmography.MAIN RESULTS: As compared with the controlgroup, there were marked increases in enhancedpause (Penh) to inhaled methacholine at aconcentration of 12.5 mg/ml in the sIN group, andmild increases in Penh to inhaled methacholine at aconcentration of 12.5 mg/ml in the mIN group. In thesIN+mIN group, the change of Penh value at anyinhaled methacholine concentration demonstratedinconclusive data.CONCLUSION: Intranasal exposure ofmite-allergen sensitized mice to sodium sulfite ormite allergen can induce airway bronchoconstrictionin response to aerosolized methacholine. However, inour study, the combined effects of sodium sulfite andmite allergen exposure on mice airway functionremain indefinite. Further larger sample size will berequired to determine the interaction between sodiumsulfite and mite allergen on airway responsiveness inmice.OBJECTIVE: To investigate the role of Th17 and IL-6in the acute phase of Kawasaki disease.METHODS: Forty-three patients with Kawasakidisease (KD) in the acute phase and forty-oneage-matched healthy children were studied. Theproportion of Th17 cells and CD4 + CD25 + FOXP3 +regulatory T cells (Treg) in peripheral bloodmononuclear cells (PBMCs) were analyzed with flowcytometry. Real-time -PCR was performed to evaluatethe mRNA expression of interleukin-23 (IL-23) andretinoid-related orphan receptor γt (RORγt) in peripheralblood. The level of plasma IL-6 was assayed byenzyme-linked immunosorbent assay (ELISA).RESULTS:The proportion of Th17 and expression ofRORγt mRNA from patients with KD in the acute phasewere (1.285±0.625%) and (M:5.62, Q:7.16),respectively, which were significantly increased ascompared with the controls [(0.584±0.407%) and(M:1.79, Q:2.55), respectively] (P

P2-025 Allergy/Immunology/Rheumatology P2-026 Allergy/Immunology/RheumatologyFOOD HYPERSENSITIVITY IN PRIMARYSCHOOL CHILDREN IN TAIWAN:RELATIONSHIP WITH ASTHMAYu-Mao Su 1 , Kong-Sang Wan 1,2 , Wen-Hsiang Chiu 2,31Department of Pediatrics, Taipei City Hospital, Renai Branch, Taiwan.2School of Medicine, National Yang-Ming University, Taipei, Taiwan.3 Department of Health, Taipei City Government, TaiwanTWO CASES OF IMMUNE DYSREGULATION,POLYENDOCRINOPATHY, ENTEROPATHY,X-LINKED SYNDROME (IPEX) WITH NOVELFOXP3 MUTATIONYunfei An, Xiaodong Zhao, Feng Xu, Xiqiang YangDivision of Nephrology and Immunology, Children's Hospital of ChongqingMedical University, Chongqing ChinaBACKGROUND: According to our previouscommon allergens study of Taipei City schoolchildren in 2008, milk and egg white hypersensitivitywere persistently high in six to eight-year-oldprimary school children. A review of the literaturealso suggested that food allergen intolerance wasclosely related to inhaled allergen upregulation. Astudy of food allergens in the regular diet of childrenis critically needed.METHODS: One thousand and ten six toeight-year-old primary school students were selectedfor evaluation of common food allergen sensitivityby using a Phadia ImmunoCAP system, PhadiatopInfant and RAST. A telephone interview to eachchild to ascertain their allergy history was performed.RESULTS: The Phadiatop mixed allergens testpositive sequence was milk, egg, meat mix, fruit mixand nut mix. The results of RAST showed that maleswere more sensitive than females to food allergens.The major food allergen sequence was: scallop,abalone, lobster, pork, casein, alfalactalbumin, andgarlic, in which the severity was mostly over class 2.The positive rate of fruit allergen sequence includingcherry, strawberry, pear, lemon, plum and mango washigher in males, but conversely, the sensitivity tofruit allergens including banana, kiwi, pineapple,avocado, and papaya, was higher in females. Theindividual specie of cereals, nuts and vegetablespositive rate was less than 1% in all participants.According to the telephone interview, among thefood hypersensitive children, who has doctordiagnosed atopic history that included asthma 21.6%,allergic rhinitis 65.4%, atopic dermatitis 16.7%, andurticaria 1.7% respectively.CONCLUSION: Food allergen hypersensitivitymay be an important marker of inhaledallergen-induced respiratory allergy in laterchildhood, and food allergen avoidance may be agood preventive method which should be included inasthma control programs.OBJECTIVE: To investigate variation of FOXP3 andits expression in male children presented with severe andearly-onset enteropathy, rash, with or withoutinsulin-dependent diabetes (IDDM).METHODS: Five male children presented with severeand early-onset enteropathy, rash, with or withoutinsulin-dependent diabetes were subjected to detectionof FOXP3 expression on the PBMCs by flow cytometryand FOXP3 gene analysis. All 11 coding exons andintron/exon boundaries of FOXP3 gene were amplifiedby polymerase chain reaction (PCR) and sequenceanalysis was performed directly on the bulk PCRproducts in forward and reversely. The candidatemutation site was compared with that of 100 healthycontrols to exclude polymorphism. Flow cytometry wasused to determine FOXP3 expression on CD4 + CD25 + Tcells and the frequency of Tregs in CD4 + T cells.RESULTS: Molecular analysis of FOXP3 in the 5subjects revealed 1 insertion and 1 missense mutations.P1 harboured a novel frame shift insertion in exon11(g.13098-13099 ins A, c.1080-1081 ins A,p.N361KfsX1), which leaded to a premature stop codonwithin the FKH domain and abortion ofCD4 + CD25 + FOXP3 + regulatory T cells. P2 carried amissense mutation in exon11 (g.13128 G>A, c.1110G>A, p. Met370Ile), in which frequency of regulatory Tcells was slightly increased.CONCLUSION: Two novel mutation of FOXP3 whichcauses IPEX phenotype were identified accordingimmunologic screening and gene Sequencing. Infantswith early-onset of IDDM and persistent diahrrea shouldbe suspected IPEX, FOXP3 gene analysis but not itsprotein expression will be the reliable diagnostics forIPEX.[Keywords] Immune dysregulation, polyendocrinopathy, enteropathy,X-linked syndrome; Regulatory T cells; FOXP3; Mutation; Primaryimmunodeficiency;[Keywords]food hypersensitivity, asthma, allergy, children267

P2-027 Allergy/Immunology/Rheumatology P2-028 Allergy/Immunology/RheumatologyTWO NOVEL LYST GENE MUTATIONS IN ACHINESE GIRL WITH CHEDIAK HIGASHISYNDROMEWei Liu, Zheng Liu, Xiao-Dong Zhao, Xi-Qiang Yang,Li-Ping JiangChildren's Hospital of Chongqing Medical University, Chongqing 400014,ChinaRANDOMIZED, OPEN LABEL AND CROSSOVER TRIAL COMPARING XYZAL AND XYZALWITH LACTOBACILLUS JOHNSONII EM1 FORTREATING PERENNIAL ALLERGIC RHINITISIN CHILDREN AGED 7-12 YEARSChen-Yen Liu, Ching-Hui Chan, Min-Sho Ku, Pen-fenLiao, Yan-Hu Wang, Hai-Lun Sun, Ko-Huang Lue,Division of Allergy, Asthma and Rheumatology, Department of Pediatrics, ChungShan Medical University Hospital, TaiwanBACKGROUND AND OBJECTIVE:Chediak-Higashi syndrome (CHS) is a rareautosomal recessive disorder (incidence around 1 in10 6 births), characterized by complex immunologicdefects, reduced pigmentation, a mild bleedingtendency, with late neurologic dysfunction andpresence of giant granules in many different celltypes. Most patients also undergo an acceleratedphase of lymphohistiocytosis and die at an early ageunless they receive an allogeneic hematopoietic stemcell transplant (SCT). CHS has always been reportedassociated with mutations of LYST. The study wasfirst time conducted to analyze clinical features andto identify the mutation types in a Chinese girl withCHS.METHODS: Genomic DNA from the patient, basedon clinical history, clinical examination, wereamplified by PCR and sequencing analysis for LYSTgene mutations.RESULTS: A girl, 5 years old, has identifiedcharacters of CHS such as hypopigmentation of theskin, eyes and hair, recurrent infections from born,decrease of PLT and WBC and Hb, peripheralneuropathy, increased large eosinophilic,peroxidase-positive inclusion bodies in bone marrowcells. PCR amplification and sequence analysis ofLYST gene revealed compound heterozygous for twonovel LYST gene mutations, both of which arepredicted to result in truncated proteins. The twomutations are nonsense mutation (c.3113C>T,CAG>TAG, Q980X) in exon 6 and (c.6788A>T,AAA>TAA, K2205X) in exon23.CONCLUSIONS: Chediak Higashi syndromewould be suspected by clinical manifestations, andbe identified by gene analysis. The confirmation ofdiagnoses could contribute to the reasonablemanagement for such patients. It was the firstChediak Higashi syndrome case identified by LYSTgene analysis in China. We had found two novelLYTS gene nonsense mutations in the Chinese CHSgirl.[Keywords] Chediak-Higashi syndrome; CHS1 gene; LYSTmutation; immune deficiencyOBJECTIVE: The purpose of this study was to comparethe clinical efficacy between Xyzal and Xyzal withLactobacillus johnsonii EM1 for treating perennial allergicrhinitis in children aged 7-12 years.METHODS: Fifty-six children aged 7 to 12 years fulfilledthe criteria for the study and were with moderate to severeperennial allergic rhinitis for at least one year. All subjectswere randomized to two arms two cross over treatmentregimen, whereby each was administered Xyzal or Xyzalwith Lactobacillus johnsonii EM1 for 12 weeks then viceversa for a further 12 weeks. The effects of the tworegimens were compared with the PediatricRhinoconjunctivitis Quality of Life Questionnaire(PRQLQ) and the total symptom score (TSS) of diary card.Nasal peak expiratory flow rate (nPEFR), FVC, FEV1 andlaboratory examinations including IgE level, eosinophiliccationic protein, blood eosinophil counts, eosinophilpercentage in a nasal smear IL4, IL10, interferon-γ andTGF-β.RESULTS: After the first 12 weeks of treatment, bothXyzal (group 1) and Xyzal with Lactobacillus johnsoniiEM1 (group 2) improved progressively TSS compared withthe run-in period in both groups (P

P2-029 Allergy/Immunology/Rheumatology P2-030 Allergy/Immunology/RheumatologyINFLUENCES OF ANTIBIOTICSADMINISTRATION ON NEONATAL RAT OFINTESTINAL FLORA AND IMMUNE SYSTEMNATURAL KILLER CELLS FROM SUBJECTSWITH TYPE 1 DIABETES HAVE DEFECTS INNKG2D-DEPENDENT FUNCTIONDEVELOPMENTWU Qing-bin, WANG Ai-liDivision of Gastroenterology, Children’s Hospital, Soochow University,Suzhou 215003, ChinaOBJECTIVE: To investigate the influence of antibiotics administration onneonatal rat model of intestinal flora and immunity system development.METHODS: 50 neonate Sprague-Dawley rats (7 day olds, weighing 15~21g, male and female) were divided into Contract group(A),Antibiotic-treated group (B), Probiotics group(C), Probiotics interventiongroup(D) and saline -treated group (E) that were 10 rats in each group atrandom in experiment. Rats in group B were received a daily dose of 100mg/kg body weight of either a commercial form of Cefaclor(made by thePH 7.4 Sodium Chlorid,0.2ml per rat) by intragastric gavage ; group C weregiven Bifidobacterium longum (0.5 a hundred million viable organism Perml ) by intragastric gavage ,0.2ml per rat ; group D were given Cefaclorfirstly(0.2ml), 2 hours later given Bifidobacterium longum(0.2ml), totle0.4ml per rat; group E were given the equal Sodium Chloride solution(0.2ml per rat). The model-groups were administrated by above mentionsonce per day for 2 weeks. Group A were sacrificed at the beginning of theexperiment without treatment. Eight rats ,selected from each group atrandom, were sacrificed respectively at the end of the experiment .Theintestinal flora in Caecum luminal contents of the rats were counted byFecal smears assay; The CD4+,CD8+T cells of terminal ileum tissue weredetected by immunohisto- chemistry assay.RESULTS: The fecal smears assay display the amount of total intestinalbacterial in group A were less, but G+ bacillus took up absolutely dominant,G- bacillus relatively less, and slight G+ coccus and G- coccus could befound in group A, as a typical bacteria distribution of Neonatal rat.Compared with group A , group C、D or E, the proportion of G+ bacillus inthe intestinal flora in group B decreased obviously, but the proportion of G-bacillus and G+ coccus in group B increased obviously, there werestatistically significant difference between the groups(P

P2-031 Allergy/Immunology/Rheumatology P2-032 Allergy/Immunology/RheumatologyASSOCIATION STUDY BETWEEN B- ANDT-LYMPHOCYTE ATTENUATOR GENE ANDTYPE 1 DIABETES MELLITUS ORSYSTEMIC LUPUS ERYTHEMATOSUS INTHE DIFFERENT PRESENTATIONS OF ASTHMAAND ASTHMA-LIKE SYMPTOMS BETWEENFOREIGN AND NATIVE MOTEHR AMONGPRESCHOOL CHILDREN IN TAIWANTHE JAPANESE POPULATION1 Mika Makimura, 1 Kenji Ihara, 1 Terumichi Matsuo,2 Hitoshi Kohno, 1 Toshiro Hara1) Department of Pediatrics, Graduate School of Medical Sciences, KyushuUniversity,; 2) Department of Endocrinology and Metabolism, FukuokaChildren’s Hospital and Medical Center for Infectious Diseases, JapanKuo-Wei Yeh 1,2 , Shun-An Wu 1 , Liang-Shiou Ou 1,2 ,Jing-Long Huang 1,21.Department of Pediatrics, Chang Gung Children’s Hospital, Taoyuan,Taiwan; 2.Community Medicine Research Center, Chang Gung Memorialhospital at Keelung, Keelung, TaiwanBACKGROUND & AIMS: B- and T-lymphocyteattenuator (BTLA) is a member of co-inhibitoryreceptor in the CD28 family and functions as anegative regulation of T cell activation. BTLA alsointeracts with herpesvirus-entry mediator whichbelongs to a tumor-necrosis factor receptorsuperfamily, suggesting a unique crosstalk betweenthese superfamilies. Our study is to elucidatewhether the BTLA gene is a new susceptibility genein the CD28 family for the development of T1Dand/or SLE in the Japanese population.METHODS: We selected three polymorphic sites inthe BTLA gene, and then alleles, genotypes orestimated haplotype frequencies of these sites werecompared between T1D or SLE patients andcontrols.RESULTS: None of them showed any significantdifference.CONCLUSIONS: The contribution of the BTLAgene to development of T1D and SLE in Japanesepopulation was not shown, therefore, furtherevaluation in other ethnic groups with larger studypopulation for other autoimmune diseases may showsome different results, and disclose the genetic rolesof the BTLA gene. (164 wards)[Keywords]BTLA; T1D; SLE; autoimmune diseasePURPOSE: The traditional asthma prevalence survey wasbased on International Study of Asthma and Allergies inChildhood (ISAAC) questionnaire which focused on age of 6-7and 13-14. However, asthma-like symptoms usuallycommenced in preschool children, and it was hard to makedefinite diagnosis of asthma among them. To marry newimmigrants increased in recent years in Taiwan. It was reportedthat one out of eight newborns was born by foreign mothers. Itis worth to determine the prevalence rate of asthma orasthma-like symptoms and analyze the different presentationsamong preschool children with native or foreign mothers.MATERIAL AND METHOD: Children aged 3–6 years wererecruited through the kindergartens for this survey in Keelungcity, Taiwan. The questionnaire used was based on the ISAACphase III core and environmental questionnaires and includedquestions on asthma, rhinoconjunctivitis, and eczema, alongwith questions to elicit common and early presentations ofasthma, as well as other demographic and environmental data.The questionnaires delivered and completed by parents.RESULTS: There were 2395 questionnaires delivered toparents with children in 50 kindergartens. There were 2170questionnaires returned and the return rate was 90.6% and80.3% of returned questionnaires were completed by nativemothers. It was 9.9% of the preschool children withphysician-diagnosed asthma generally. However, it was 20.4%of them experienced asthma-like symptoms in kindergarten. Itwas 10.1% of children with native mothers hadphysician-diagnosed asthma and 9.6% of children with foreignmothers. Both group had no significant difference (p=0.765).However, as for presentations of wheeze ever, it was 18.2% inchildren with native mothers, and 11.3% of foreign mothersrespectively (p=0.001). Children with native mothers also hadexperienced cough more than 3 weeks more frequently(21.0%) comparing children with foreign mothers (12.0%,p

P2-033 Allergy/Immunology/Rheumatology P2-034 Allergy/Immunology/RheumatologyUTILIZATION OF CHITIN-INDUCED IFN-ΓFOR DIAGNOSING ALLERGIC ASTHMAChia-Rui Shena,#, Li-Cheng Chenb,#, Chia-LiangKaoa, Che-Wei Changa, Yi-Ju Changa and Chao-LinLiuc# Medical Biotechnology and Laboratory Science, Chang Gung University,TaiwanInflammation in allergic asthma is generated andactivated by endogenous cytokines including IL-4and IL-5 produced by Th2-type lymphocytes.Several reports showed that development ofTh1/cell-mediated immune response by chitinsignificantly down-regulated Th2 responses byeliciting IFN-γproduction in experimental inducedallergic asthma models. Administration of chitinsuccessfully suppressed allergen-inducedimmunopathological responses. Here we furtherinvestigate the potential mechanisms and found thatspleen cells isolated from the sensitized animalswith/out chitin-treatment showed much less abundantamounts of IFN-γ production in response to thestimulation of chitin, although there were lessamounts of Th2 cytokine with OVA-stimulation inchitin treated animal. It led us to hypothesize thatless IFN-γ production of immune cells in response tochitin in allergic subjects. Therefore, we nextexamined IFN-γ production of peripheral bloodmononuclear cells (PBMC) elicited by chitin inhealthy individuals and patients with allergic asthma.It appeared that PBMC obtained from asthmapatients secret less IFN-γthan that from healthyindividuals. Less expression of T-bet,IFN-γ-associated transcription factor, was also foundin chitin-stimulated cells in those allergic patients.Furthermore, such low response to chitin was shownto associate with the elevation of mite-specific serumIgE, but appeared little or no correlation with totalIgE or other allergens. It provides some basis toestablish a potential platform utilizing chitin tocontribute allergic asthma diagnosis.[Keywords] asthma, chitin, IFN-γ, T-bet, mite, PBMCETIOLOGIC AND PATHOGENETICPARTICULARITIES OF THEBRONHOOBSTRUKTIVE SYNDROME ININFANTPrikhodchenko Nelly, Shumatova T.A.Vladivostok state medical university, Russian FedrationTo date the place of antimicrobial and immunomodulatetherapy in treatment of the recidivatingbronhoobstructive syndrome (BOS) in children is notdetermined. To study of the infection role(cytomegalovirus infection −CMV, herpes simplexinfection - HSV, mycoplasma infection - Mp, Chlamydiainfection (Chlamydia pneumoniae − Cp, Chlamydiatrachomatis − Ct) and immune changes degree wasstudied of immune status and blood serum of 18 infantwith recidivating BOS and frequent acute respiratoryinfections. It was found cellular immunity abnormalitiesin all observed children: reduction of the number CD3+,CD4+, reduction of CD4+/CD8+, reduction ofproliferative activities, disturbance of the expressionsreceptor to IL-2 and apoptosis intensification. It wasdiscovered deflections in humoral immunity (increaseCD20+, increase of IgM level and CIK level).Immunoglobulin G in diagnostic subtitle to Mp wasdiscovered in 27,7%, Cp in 44,4%, Ct in 11,1%, HSV in22,2%, CMV in 55,5% patient with BOS. It wasestablished the associations of the selected agents in themajority of patient (83,3%), most often we met theassociation HSV with CMV (38,8%), Cp and CMV(33,3%). Thus the disturbance of the celluar and humoralimmunity played important role in the pathogenesis ofrecidivating bronhoobstructive syndrome in children. Atthe same time the leading etiological agents were anassociations of HSV+CMV, Cp+CMV. It was caused ofimmunity abnormalities. So the inclusion ofantibacterial, antiviral and immunomodulate preparationin the modern protocols of the treatment recidivatingbronhoobstructive syndrome is necessary.[Keywords]bronhoobstruction, immunity, infection, infant271

P2-035 Allergy/Immunology/Rheumatology P2-036 Allergy/Immunology/RheumatologyASSOCIATION OF HIGH RISK ASTHMAWITH SEVERITY OF ALLERGIC RHINITISKuo-Hsi Lee, Shyh-Dar Shyur, Szu-Hung Chu, Li-HsinHuang, Yu-Hsuan Kao, Chieh-Han Cheng, Wei-Te Lei,Chia-Yi LoDepartment of Pediatrics, Mackay Memorial Hospital, Taipei, TaiwanINTRODUCTION: In Taipei city, approximately 50% of thefirst grade elementary school children had allergic rhinitis andmore than 20% had asthma were reported in recent years. Tochildren with both atopic asthma and allergic rhinitis, we try tofind out if the severity of allergic rhinitis had any predictivevalue for children with high risk asthma, who had increasedrisk for visiting emergency department and hospitalization formanagement of acute exacerbation of asthma. Children withatopic asthma and allergic rhinitis were enrolled. This studyaimed to explore the relationship between high risk asthmaand various parameters of allergic rhinitis, including nasaltotal symptom scores (NTSS), individual nasal symptomscores, total IgE, total eosinophil count and specific IgEconcentrations ( cat、dog、D.pteronyssinus、D. farinae、cock、milk、egg、fish) in atopic children with both asthma andallergic rhinitis.MATERIALS AND METHODS: 299 patient aged 5-18years-old (average age: 8.4 years-old) whom diagnosed withatopic asthma and allergic rhinitis were enrolled. We definedthe high risk asthma is that these patients were admitted orgiven emergency medical treatment because of asthma.According the above definition, the patients were distributedto two groups: (Low risk asthma group: no any emergencymedical treatment or admission history for asthma; High riskasthma group: emergency medical treatment or admissionhistory for asthma). Allergic rhinitis symptom score used nasaltotal symptom scores (NTSS) ranging 0–12 were obtainedfrom patients or their families by using four-point scales forsneezing, itching, rhinorrhea and for congestion (0: none; 1:mild; 2: moderate; 3: severe). We compared the differentparameters of allergic rhinitis, including NTSS, individualnasal symptom scores, total IgE, total eosinophil count andspecific IgE concentrations ( cat、dog、D.pteronyssinus、D.farinae、cock、milk、egg、fish) between these two groups ofasthma with low and high risks.RESULTS: Total 299 children diagnosed with Atopic asthmaand allergic rhinitis were enrolled into our study. 191 patientswere in low risk asthma group. 108 patients were in high riskasthma group. We found that high risk asthma group hadhigher specific IgE concentration of D. pteronyssinus andindividual nasal symptom scores for rhinorrhea which werestatistics significantly than low risk asthma group (p value

P2-037 Allergy/Immunology/Rheumatology P2-038 Allergy/Immunology/RheumatologyTHE INHIBITORY EFFECTS ANDMECHANISMS OF FORMOTEROL ANDSALMETEROL ON RANTES IN BRONCHIALTHE CLINICAL ANALYSIS OF 16 GASES OFKAWASAKI DISEASE IN INFANTS THREEMONTHS OF AGE OR YOUNGEREPITHELIAL CELLSYu-Te Chu , Chih-Hsing HungThe pediatric department, Kaohsiung Medical University Hospital, TaiwanNan Zhou, Ying BaoXian Children’s Hospital, Shaanxi 710003, China.OBJECTIVE: RANTES (CCL5) is a potentchemoattractant for eosinophil recruitment.Formoterol and salmeterol are two inhaled longacting β 2 adrenoceptor agonists (LABAs), widelyused for the local treatment of asthma. There were afew papers talking about the inhibitory effect ofLABAs in RANTES produced by the bronchialepithelial cells. We used BEAS-2B cells to elucidatethe effect and mechanism of LABAs on RANTESexpression from human bronchial epithelial cells.METHODS: BEAS-2B cells were stimulated bypoly I:C 10mcg/mL with or without 2h pretreatmentof formoterol or salmeterol. ICI 118551, a specificβ2 adrenergic receptor antagonist, was used to studywhether the inhibition was through this receptor.Forskolin was used as a cyclic AMP activator toverify the importance of cAMP activation. Theprotein expression of RANTES was measured byELISA assay. MAPK inhibitors was used to verifythe intracellular pathway that activated by poly I:C.The western blot was used to access the exactpathway of the inhibitory effect from LABAs.MAIN RESULTS: Formoterol and salmeterol(10-7-10-9 M) significantly down-regulated polyI:C− induced RANTES protein in BEAS-2B cells.ICI 118,551 reversed their suppression of RANTESproduction. Forskolin suppressed the RANTESproduction. By MAPK inhibitor assay and westernblot, the JNK-cJUN pathway is the main pathwayinvolving RANTES production.CONCLUSIONS: Formoterol and salmeterol, twoinhaled long-acting β2 agonists, down-regulated polyI:C- induced RANTES expression in BEAS-2B cellsat physiologic doses. The effect was mediated via theβ2 adrenoceptor, cAMP, and JNK-cJUN pathway.Our study implied that the LABAs down-regulatedthe amount of RANTES from bronchial epithelialcells and provided lesser affinity for eosinophils,which exert additional anti-inflammatory effects inthe treatment of asthma.OBJEETIVE: To summarize clinical features ofkawasakj disease (KD) of young infants in infants ≤3months of age.METHODS: The clinical data of 16 infants aged 1 to 3months suffering from KD were retrospectively analyzedfrom January 2004 to May 2009.RESULTS: There were 12 male and 4 female infants inthis study. The frequency of the 5 principal clinicalfeatures was as follows: changes in lips and oral cavity,75.0%; bilateral bulbar conjunctival injection, 62.5%;polymorphous exanthema, 56.3%; cervicallymphadenopathy, 25.0%; and changes in extremities,50.0%. Two infants fulfilled criteria for KD includingfever which persists for 5 or more days with at least 4 ofthe principal clinical criteria.CONCLUSION: Infants ≤3 months of age with KDusually presented with incomplete clinical features. Theabnormality of coronary artery is the only gold index forfinal diagnosis of incomplete KD.[Keywords]Kawasaki disease, coronary artery[Keywords]RANTES, Poly I:C, TLR3, Bronchial epithelium, Longacting beta II agonist273

P2-039 Allergy/Immunology/Rheumatology P2-040 Allergy/Immunology/RheumatologyHOUSE DUST MITE AND CHILDHOODASTHMA AMONG ASIAN CHILDRENCheong Wooi CheahPaediatrics, International Medical University, MalaysiaBACKGROUND: Asthma is the most common chronicdisease among children and its prevalence is increasing byan astounding fifty percent every decade. The Phase Oneof International Study of Asthma and Allergies inChildhood (ISAAC) found that there is a marked variationin the prevalence of asthma symptoms between differentcountries. This variation can be up to 15-fold and is likelydue to environmental factors. House dust mite (HDM) isthe most potent indoor environmental allergen. To date,there are only a few studies on the sensitization profiles ofHDM in childhood asthma in South East Asia.OBJECTIVES: To determine the profiles of asthmaticchildren who are sensitized to the HDM,Dermatophagoides pteronyssinus (DP) andDermatophagoides farinae (DF). To determine associationof HDM sensitization to the control and severity ofchildhood asthma. To test the viability of using saliva inimmunoassay in associating the levels of Immunoglobulin(Ig)E in saliva with the severity and control of asthmaticpatients.METHODOLOGY: The subjects were unselectedchildren attending asthma follow-up clinic or admitted inthe ward in a tertiary hospital. They were deemed, onclinical grounds, to be suffering from asthma. Skin pricktest was performed on all the participants. Their saliva wascollected. A questionnaire regarding the control andseverity of asthmatic symptoms of the participant wereasked. The control and severity of asthma were assessed inaccordance to the Global Initiative for Asthma (GINA)guideline 2008. The saliva collected was selectedrandomly and the IgE specific to both DP and DF wasquantified by Enzyme Linked Immunosorbent assay(ELISA). Non-asthmatic children attending the generalclinic were recruited as the control group for comparison.RESULTS: Patients with poorer control have asignificantly higher percentage of DP and DF sensitizationas compared to patients with better control. There is nosignificant association between DP sensitization and theseverity of asthma. The mean IgE titre in subjects withcontrolled asthma is lower than that of subjects with partlycontrolled and uncontrolled asthma. The mean IgE titre insubjects with moderate and severe asthma is lower thanthat of subjects with mild asthma. However, the differenceis not statistically significant.CONCLUSION: There is subtle association between mitesensitivity and control (persistence) and severity ofchildhood asthma. IgE titre in expectorated (spitted) salivais not a good indicator for the severity and control ofasthma.[Keywords]House dust mite, Dermatophagoides pteronyssinus,Dermatophagoides farinae, childhood asthma, control, severityTHAPSIGARGIN AND FLAVIN ADENINEDINUCLEOTIDE EX VIVO TREATMENTRESCUES TRAFFICKING-DEFECTIVEGP91PHOX IN CHRONIC GRANULOMATOUSDISEASE LEUKOCYTESHuang Ya-Fang 1 , Liu Si-Yen 2 ,Yang Pei-Wen 3 , YenChia-Liang 4 , Shieh Chi-Chang 5Department of Microbiology and Immunology, National Cheng Kung University 1 ,Institute of Basic Medicine, National Cheng-Kung University Medical College 2 ,Institute of Microbiology and Immunology, National Cheng-Kung UniversityMedical College 3 , Institute of Basic Medicine, National Cheng-Kung UniversityMedical College 4 , Institute of Microbiology and Immunology; Department ofPediatrics, National Cheng-Kung University Medical College, Taiwan 5Mutations in leukocyte NADPH oxidase genes lead todefective respiratory burst in leukocytes and causechronic granulomatous diseases (CGD) in humans. Themost common form of CGD is caused by mutations inthe membrane-bound oxidase component gp91phoxwhich is encoded by the CYBB gene on theX-chromosome. We previously reported a patient withCYBB mutation (H338Y) which prevents theintracellular trafficking and expression of gp91phox onleukocytes. The capacity of the leukocytes to producereactive oxygen species (ROS) was rescued by treatmentwith thapsigargin and flavin adenine dinucleotide (FAD).The increase in ROS production was not due to theincrease of cytoplasmic calcium induced by thapsigarginbecause the treatment of calcium ionophore did not havethe same effect. Protein and cellular analyses onleukocytes and cells transfected with GFP-taggedgp91phox mutant showed that treated cells expressedmore Endo-H resistant gp91phox protein on cell surfaceand are more effective in killing bacteria. Thapsigarginand FAD-treated CGD leukocytes had enhanced activityin protecting mice from staphylococcus-inducedperitoneal abscess formation in a mouse model of CGD.These results indicate that thapsigargin-FAD ex vivotreatment is effective in rescuing the ROS-producingactivity of leukocytes in selected CGD patients.[Keywords]cell therapy, immunodeficiency, reactive oxygen species,chronic granulomatous disease, NADPH oxidase, calcium pumpinhibitor274

P2-041 Allergy/Immunology/Rheumatology P2-042 Allergy/Immunology/RheumatologyTHE ASSOCIATION OF SKIN TESTREACTIVITY TO COW`S MILK PROTEINSCHOOL CHILDREN HEALTH ANDENVIRONMENT IN DEVELOPING COUNTRYAND/OR SOY PROTEIN WITH THESEVERITY OF ATOPIC DERMATITIS AMONG1 Shambhu Dutta Joshi, 2 R. P. BhandariINFANTS AGED 1-6 MONTHS OLDPrimary Health Center, Malakheti, NEPAL, 2 Community Health andEnvironmental Society Nepal, NepalMelanie Yagdulas GavinoPediatrics, De La Salle University Health Sciences Institute, PhilippinesOBJECTIVE: To determine the association of skin testreactivity to Cow’s Milk Protein (CMP) and/or Soy Protein(SP) with Atopic Dermatitis (AD) in infants aged 1 to 6months old.STUDY DESIGN: Hybrid Design, specifically a Follow-upPrevalence Study.SETTING: Cavite, Philippines.PARTICIPANTS: Infants aged 1 to 6 months old diagnosedwith AD using the Hanifin and Rajka criteriaOUTCOME MEASURE: Severity of atopic dermatitis usingScoring Atopic Dermatitis.METHODS: All infants who fulfilled the diagnostic criteriaof AD underwent allergy skin prick test (SPT) to CMP, SP, apositive and a negative control. Severity of AD was assessedusing the SCORAD index. Statistical analysis was performedusing SPSSPC+ and Epi-Info softwares. Chi-square was usedto determine if there was statistically significant associationbetween skin test reactivity to CMP and/or SP and the severityof atopic dermatitis, family history of atopy and feeding.T-test was used to determine the association between skin testreactivity and Mean SCORAD. Spearman`s rank correlationcoefficient was computed to determine the correlation ofseverity of atopic dermatitis with the number of positive skinprick test results.RESULTS: There were a total of 54 participants. Most weremales with a mean age of 3 months. More than 2/3 was withmild to moderate atopic dermatitis (77.8%). Majority werepositive to skin prick test (66.7%). The proportion of skin testreactivity to CMP and SP among infants with AD was at48.2% and 50%, respectively. There was a significantassociation between the skin test reactivity to CMP and/or SPand the severity of AD. Statistical analysis showed that therewas a significant association between skin test reactivity andfamily history of AD. There was no association between skintest reactivity and whether the patient is on pure milk formulaor mixed feeding. A positive direct relationship betweenseverity of AD and number of skin test reactivity was alsonoted.CONCLUSION: Although AD is viewed by many as a minordermatologic problem, the stress that it poses to the family andeconomy cannot be ignored. The results showed a higherproportion of positive skin test reactivity to CMP and SPamong AD infants as compared to international studies. Therewas a significant association between skin test reactivity toCMP and/or SP and severity of AD. This study supports theadvocacy of the PPS regarding the importance ofbreastfeeding.[Keywords] atopic dermatitis, skin test reactivity, Scoring AtopicDermatitis (SCORAD)BACKGROUND: Developing country like Nepal haspoor health status in school students.OBJECTIVE: To know and evaluate the indoorenvironmental condition of government andprivate-schools.METHODS: A cross sectional studied of representativesamples of 35 schools of selected region of Nepalincluding government and private schools, from Oct2007-2009 September. Onsite observation and healthcheck up & interview with students and teachers weredone. Specific scores was given in each criteria. The datawere analysed and edited in EPI info program.RESULTS: The results shows that 89% of governmentschool and 45%of private-schools have poorenvironmental condition and 69% of governmentschools students are suffering from environmental-healthproblem while only 22%of private school student aresuffering from some kind of diseases.Government-school do not have the standard classroom,adequate sports facility, safe drinking water, light andventilation in comparison with private schools.CONCLUSIONS: We conclude that the main causes arepoor socio-economic status, illiteracy of parents,negligence, hard housework for children, diseases,malnutrition, incomplete immunisation and lack ofhealth education. The poor environment conditionincludes crowed students in a classroom, poorventilation, shortage of clean drinking water, untidyclothes of students, poor nutrition and lack of greeneryin the school area, school near by road, air pollution andlack of environmental awareness among teachers andparents. The government schools have limited budget,resources with compared to private schools and most ofthe lower and lower middle class family children arestudying in government schools which covers nation82% of total students.RECOMMENDATION: The government shouldallocate the special budget to the government schoolsand it should be utilized from the available resourcessuch as good ventilation, limited student in a class,awareness among teachers and parents. Last but not theleast, this type of programs are helpful to prevent fromenvironmental health hazards also.[Keywords]Children, allergy,Health,Environment,Developing country275

P2-043 Allergy/Immunology/Rheumatology P2-044 Neuropsychiatry (Neurology)FOOD ALLERGEN SPECIFIC IGEANTIBODIES IN PRIMARYSCHOOLCHILDRENSEQUENTIAL CHANGE IN REGIONALCEREBRAL BLOOD FLOW ABNORMALITY INPATIENTS WITH KAWASAKI DISEASEHsin-Lin Wu 1 , Kong-Sang Wan 2 , Tzee-Chung Wu 3 ,Keh-Gong Wu 3 , Winnei Yang 4 , Ting-Fang Chiu 1 ,BetauHwang 1,5Taipei City Hospital Zhongxiao Branch 1 , Taipei City Hospital RenaiBranch 2 , Taipei Veterans General Hospital 3 ,Taipei City Hospital YangMingBranch 4 , Taipei City Hospital Zhongxiao Branch and National Yang MingUniversity, Taiwan 5Toshiyuki Hikita 1 , Tatsuro Kaminaga 2 , Suguru Wakita 1 ,Yasushi Fujii 1 , Yukishige Yanagawa 1Pediatrics, Teikyo University sch of Med 1 , Radiology, Teikyo University School ofMedicine, Japan 2OBJECTIVES: To evaluate the types of food allergens andits relationship with aeroallergens in primary schoolchildren inTaipei City , the total IgE by using Phadiatop infant andspecific IgE against Dermatophagoides pteronyssinus ,Dermatophagoides farinae, Blomia tropicalis,Germancockroach, egg white, casein, alpha-lactoalbumin,beta-lactoglobulin, shrimp, kiwi, & mango were detected inthe sera of 88 primary schoolchildren who had clinicalsuspicion of food allergy and, aged ranged from 6 to 12 yearsold.METHODS: The 3-5 ml of serum was tested byradioabsorbent method using Pharymacia ImmunoCAP-250(Phadia Taiwan Inc.). Serum levels of total IgE andallergen-specific IgE were determined by fluorescence enzymeimmuno-assay (FEIA) using the ImmunoCAP-system (Phadia,Uppsala, Sweden). The ImmunoCAP is an in vitro-specificIgE test that uses a three-dimensional cellulose solid allergenphase.MAIN RESULTS: Among the 88 children with suspicioushistory of food allergy, 7 (8.0 %) children have IgE level lessthan 0.35 PAu/L indicating negative for allergy, other 32(36.4%) children have only positive specific IgE antibodiesagainst aeroallergens including Dermatophagoidespteronyssinus in 32(100 %), Dermatophagoides farinae in 31(96.9%), Blomia tropicalis in 28 (87.5%), and cockroach in 11(34.4%). The remaining 49 (55.9 %) have positive specific IgEantibodies against various foods. Two of them are pure foodsallergy including positive specific IgE antibodies against eggwhite in 2, against milk, casein, and alpha-lactoalbumin ineach 1. Forty-seven (53.4%) children have positive specificIgE antibodies against both aeroallergens and various foods.The positive foods allergens are egg white in 26 (53.1 %),shrimp in 23 (46.9 %), milk in 15 (30.6 %), casein in 10 (20.4%), alpha lactoalbumin in 9 (18.4 %), beta lactoglobulin in 4(8.2 %), kiwi in 4 (8.2 %) and mango in 4 (8.2%). Of the 79children with positive specific IgE antibodies againstaeroallergens, 47 (59.5%) have positive evidence of specificIgE antibodies against foods allergens.CONCLUSION: The primary schoolchildren with clinicalsuspicion of food allergies, 55.9 % have positive sera specificIgE antibodies against various tested foods in this study. Themost common allergens are egg white, shrimp and milk. Mostof children (95.9 %) with positive specific IgE antibodiesagainst foods allergens are combined with positive specificIgE against aeroallergens. On the contrary, only 59.5 % ofchildren with positive of specific IgE antibodies againstaeroallergens have the evidence of positive specific IgEantibodies against foods allergens.BACKGROUND: We reported localized cerebralhypoperfusion in 8 patients with Kawasaki’s disease (KD)using brain single-photon emission computed tomography(SPECT) at the 4th Congress of the Asian Society for PediatricResearch. However, we did not discuss whether thishypoperfusion was transient or not, and detected it only in itsacute phase. Sequential changes during such hypoperfusionshave not been discussed by previous studies. In this study,follow-up brain SPECT was performed in these 8 KD patientsand in new KD patients.OBJECTIVE: This study aimed to estimate the sequentialchange in localized cerebral hypoperfusion in KD patients overa period time.METHODS: This study included 22 KD patients admitted toTeikyo University Hospital and was approved by the TeikyoUniversity Ethics Committee. KD was diagnosed on the basisof standard diagnostic criteria and all subjects presented inacute phase of KD. All patients underwent brain SPECT fromday 6 to day 30 from the onset of KD, after an informedconsent was obtained from their parents. Ten of 22 patientsunderwent follow-up SPECT from 1 to 12 months after firstexamination. Six of 10 patients underwent further follow-upSPECT from 12 to 36 months after first examination.The patients were injected with 190–550 MBq of 99m Tchexamethylpropylene amine oxime under anesthesia. Dataacquisition was done using a two-headed gamma camera after5 min of administering the injection. Reconstructed transverseimages were obtained and examined by one board-certifiedradiologist.MAIN RESULTS: Localized cerebral hypo perfusion wasobserved in 18 of 22 patients in the initial SPECT and detectedin 7 patients in the second SPECT (from 1 to 11 months). Itwas resolved in 3 patients in the second SPECT (from 1 to 14months). Six of these 7 patients underwent further follow-upsafter the second SPECT. Localized cerebral hypoperfusionwas not detected in the third to sixth SPECT examinations(from 4 to 36 months) in 2 of these 6 patients. Localizedcerebral hypoperfusion persisted in 5 of 10 patients throughoutthe study period.CONCLUSION: Localized cerebral hypoperfusion wasdetected between 1 and 24 months after the onset of KD, anddisappeared in half of the patients during the study period(from 4 to 36 months after onset).[Keywords] Kawasaki disease, localized cerebral hypoperfusion,single-photon emission computed tomography, 99mTc-hexamethylpropylene amine oxime[Keywords]Allergen ,Food Allergy, Specific IgE276

P2-045 Neuropsychiatry (Neurology) P2-046 Neuropsychiatry (Neurology)ANTIBIOTIC CEFTRIAXONE ATTENUATESHYPOXIC-ISCHEMIC BRAIN INJURY INNEONATAL RATPLASMA CALCITONIN GENE RELATEDPEPTIDE LEVELS PREDICT PREVENTIVETHERAPY IN PEDIATRIC MIGRAINEPei Chun Lai 1,2 , Yen Ta Huang 1,3,4 ,Chia Chen Wu 5 , PenJung Wang 2 and Ted H. Chiu 1,6,*1 Institute of Pharmacology and Toxicology, 6 Department of Pharmacology,Tzu Chi University, Hualien 970, Taiwan; 2 Department of Pediatrics,3 Department of Emergency Medicine, 4 Division of Surgical Critical CareUnit, 5 Department of Research, Buddhist Tzu Chi General Hospital,Hualien 970, Taiwan (*Correspondence: Ted H. Chiu, Ph.D.)PiChuan Fan 1,3 , Ping-Hung Kuo 2 , Lih-Chu Chiou 3,41 Departments of Pediatrics, and 2 Internal Medicine, National Taiwan UniversityHospital, Taipei, Taiwan; 3 Graduate Institute of Pharmacology, College ofMedicine and 4 Neurobiology and Cognitive Center, National Taiwan University,Taipei, TaiwanOBJECT: Perinatal brain injury is the leading causeof subsequent neurological disability in both termand preterm baby. Hypoxic-ischemic encephalopathy(HIE) is predominantly involved in the multiplecauses of perinatal insult. HIE is associated withcerebral palsy, learning disability, and epilepsy. Thepathophysiology of HIE includes energy failure,glutamate excitotoxicity, reactive oxygen species andsubsequent inflammatory reaction. Excitatory aminoacid transporter 2 (EAAT 2) is a major glutamatetransporter in the brain, which is expressed mainly inastrocytes. However, in premature infant, EAAT 2 isexpressed in pre-oligodendrocytes instead ofastrocytes. The HIE induced excessive glutamaterelease which is not reuptakes by immatureastrocytes, may induce neuronal andpre-oligodentrocyte damage.METHODS: In this study, we used a neonatal ratmodel of HIE by unilateral ligation of carotid arteryand subsequent exposure to 8% oxygen for 2 hoursin postnatal day (PN) 7 rats. Neonatal rats were dailyinjected with cephalosporin antibiotic, ceftriaxone(50, 100, 200mg/kg, i.p.), 48 hours prior toexperimental HIE. Brain tissues were evaluated inPN14 included Nissl stain, immunohistochemistrystain for myelin basic protein, and TUNEL assay.Besides, neonatal rats were sacrificed in PN 7 afterceftriaxone or vehicle treatment. Western blot andimmunofluorescent stain were conducted.MAIN RESULTS: Our results showed thatpre-treatment with ceftriaxone 200mg/kgsignificantly reduced the brain infarct volume, braininjury scores, apoptotic cells in hippocampus andrestored the myelination in PN14 rats. Theimmunofluorescent study demonstrated thatceftriaxone pre-treatment induced the expression ofEAAT2 in immature astrocytes.CONCLUSION: These results suggest thatpretreatment of infants at risk for HIE withceftriaxone may provide a potential therapy for braininjury resulted from HIE.The approach to treating pediatric patients with migraineis mostly empirical. Rare clinical parameters aresuggestive for choosing an optimal anti-migraine drug.Calcitonin gene-related peptide (CGRP), a neuropeptidevasodilator released from trigeminal nerve terminal, hasbeen known to be involved in the pathogenesis ofmigraine. To investigate the role of plasma CGRP in thepharmacotherapy of pediatric migraine, we measured theplasma CGRP levels in 88 blood samples collectedduring (35%) or between (65%) attacks from 68migraineurs (40F, 59%), aged 4-18 yr (mean 11.4 yr)from Apr. 2004 to Oct. 2009. These patients receivedmonotherapy (84%) or combined therapy (16%) formore than 2 weeks. Responders were defined to havemore than 50% headache reduction, in terms of severityor frequency. Plasma CGRP concentrations wereassessed by ELISA. Sixty-one (91%) patients requiredacute treatment and 47 (69%) needed preventive therapy.Plasma CGRP levels in the patients requiring preventivetherapy were higher than those not (p=0.031), whereasno significant difference were noted between those withand without acute treatment. The response rate to theacute treatment with naproxen (15/27; 55%) was higherthan sumatriptan (6/14; 43%) and acetaminophen (20/48;42%). The preventives from high to low response ratesare valproate (4/5; 80%), topiramate (14/20; 70%),flunarizine (16/29; 55%), gabapentin (7/14; 54%),propranolol (4/8; 50%) and cyproheptadine (4/12; 33%).Plasma CGRP levels were significantly elevated in thepatients responsive to topiramate compared withnon-responders, but the differences were not significantin the other preventive therapies. Plasma CGRP ispredictive for the patients requiring preventive therapyand having better response to topiramate.[Keywords]CGRP, migraine, childhood, treatment[Keywords] hypoxic-ischemic brain injury, neuroprotection,ceftriaxone277

P2-047 Neuropsychiatry (Neurology) P2-048 Neuropsychiatry (Neurology)FEEDING AND SWALLOWING PROBLEMS,GASTROINTESTINAL DYSFUNCTION, ANDUNUSUAL ADVERSE EVENTS POST H1N1VACCINATIONNUTRITIONAL STATUS IN PATIENTS WITHDUCHENNE MUSCULAR DYSTROPHYYen-Shan Chen, M.D. 1 , Hsiang-Hung Shih, M.D. 1,2 ,Yuh-Jyh Jong M.D., Ph.D. 1,3,4Departments of 1 Pediatrics and 3 Laboratory medicine,Kaohsiung MedicalUniversity Hospital, Kaohsiung, Taiwan; 2 Department of Pediatrics,Kaohsiung Municipal Hsiao-Kang Hospital, Kaohsiung, Taiwan; 4 GraduateInstitute of Medicine, College of Medicine, Kaohsiung Medicine University,Kaohsiung, TaiwanOBJECTIVE: Duchenne muscular dystrophy (DMD) is aprogressive X-linked myopathy arising from the completeabsence of functional dystrophin at the myofiber plasmamembrane and also results from mutations in the dystrophingene. The progression of muscle degeneration affect motorfunctions, may compromise feeding and swallowing,gastrointestinal dysfunction, and nutritional status. We conducta questionnaire survey for addressing the prevalence of andpredictive factors for feeding and swallowing difficulties,gastrointestinal dysfunction, and malnutrition in DMDpatients.METHODS: From January 2009 to November 2009, adedicated questionnaire survey was conducted by a medicalcenter in Kaohsiung, Taiwan, and 77 biopsied and/orgenetically confirmed DMD patients participated. Thequestionnaire includes demographic data, current ambulatorystatus (walker, sitter, non-sitter), feeding and swallowingdifficulties (pre-oral phase, oral phase, pharyngeal phase,esophageal phase), gastrointestinal and respiratorydysfunctions. Malnutrition is defined as body weight Z score

P2-049 Neuropsychiatry (Neurology) P2-050 Neuropsychiatry (Neurology)STRUCTURED SENSORY STIMULATIONIMPROVES GLASGO COMA SCALE SCOREHEMORRHAGIC CEREBROVASCULARDISEASES (HCVD) IN CHILDRENIN COMATOSE CHILDREN HOSPITALIZEDWITH PYOGENIC MENINGITIS INBANGLADESH1* Dr. M Abid Hossain Mollah, MBBS, FCPS, Dip-MedEdu (Lon),FRCP (Edin),FACP (USA); 2 Dr. Md A Hai,MBBS, MD (Dhaka, Bangladesh); 3# Dr. Kazi SelimAnwar, MD (USSR), Res Dip (Japan), M. Phi (England)1 Professor, Department of Paediatrics, Dhaka Medical College Hospital(Currently Prof. of Peadiatrics, Mymensingh Medical College Hospital,Bangladesh); 2 Post-Graduate Research Fellow, Dhaka Medical CollegeHospital (currently waiting for an academic position by the Ministry ofHealth, Govt. of Bangladesh); 3# Public Health Expert & Microbiologist,Inst. Public Health, Dhaka (Currently joined a short-term consultancy atthe Ministry of Health, Saudi Arabia on Govt. lien), & Ex-Gen. Secretary,DOSHER, Bangladesh* Presenting author, # For all sorts of correspondencePURPOSE: Coma is a clinical state when patients lackawareness to external/internal environment/stimuli, whichoccurs due to various causes including non-traumatic braininjury like pyogenic meningitis (PM). Of several measurestaken towards recovering children from coma, repeatedstructured sensory stimulation (SSS) remains effective.Since it is not studied in Bangladesh yet, we determinedhow much SSS-therapy helps improving GlasgowComa-Scale (GCS-score) in comatose children with PM.METHODS: This single-blinded randomized control trialwas conducted among 60 children aged

P2-051 Neuropsychiatry (Neurology) P2-052 Neuropsychiatry (Neurology)PREVALENCE OF DEVELOPMENTALDISABILITY CHILDREN IN NORTHEAST OFTAIWANHui-Ju Chen 1 , Nan-Chang Chiu 2,3 , Hung-Chi Lue 1 ,Lo-Lin Tseng 11 Department of Pediatrics, Saint Mary’s Hospital Luodong, Taiwan,2 Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan,3 Mackay Medicine,Nursing and Management College, Taipei, TaiwanBACKGROUND: Since early identification of children withdevelopmental disability and early intervention can improve theoutcome, we conducted a developmental screen program to detectthe developmental delay children and estimate the prevalence ofdevelopmental disability children in northeast of Taiwan.PATIENT & METHODS: We enrolled children between 4months and 6 years of age from July 2008 to December 2009.Taipei City Developmental Screening Checklist for Preschoolers,version 2(Taipei Ⅱ) was used as the screening tool. Parents orcaregivers completed the screening checklist while waiting forchild health examination or vaccination at local clinics in I-Lan orin Saint Mary’s Hospital Luodong. The screening results wereexplained immediately by the visited pediatrist. The results of thescreen were divided into three groups: (1) normal (no failureitem), (2) follow-up (one failure item), and (3) suspecteddevelopmental delay (more than two failure items). In thesuspected developmental delay group, the children were referredto the pediatric neurologist for further neurological examinationand performing Denver Developmental Screen Test (DDST). Inthe follow-up group, the children were rechecked 2 to 6 monthslater by the screening checklist (Taipei II) again; if the failureitems were still more than one, the children then were referred tothe pediatric neurologist, too. The mother country, income andeducation level of the parents were also analyzed.RESULTS: Among the 3151 screened children, 2744 (87.1%)passed the screen checklist, 240 (7.6%) failed to pass one item inthe first screen and need further follow up, and 167 (5.3%) weresuspected developmental delay. Of the follow-up children,34(1.1%) children were screened as suspected developmentaldelay later. Further neurological examination and DDSTperformed in 173 patients revealed normal in 11 children (0.35%),borderline developmental delay in 41 children (1.30%); and in 120children (3.8%) significant developmental delay were found andthey were referred to early intervention program. The diagnosis ofthe 161 developmental delay children included speech andlanguage delay/disorders (n=96, 3.04%), , autistic spectrumdisorder (n=12, 0.38%), mental retardation (n=6, 0.19%), globaldevelopmental delay (n=21, 0.67%), attention deficit hyperactivitydisorder (n=26, 0.82%), motor delay (n=28, 0.89%), infantilecerebral palsy (n=11, 0.35%), syndromic disorder/congenitalanomaly (n=8, 0.25%), hearing impairment (n=2, 0.07%), etc.Low parental income, low parental education level and aboriginewere associated with their children’s developmental delay.CONCLUSIONS: The prevalence of developmental disability inour study is 6.4%. The most common disability disorder is speechand language delay/disorders follows by attention deficithyperactivity disorder. Environmental factors including aboriginefamily, parental education level and income are associated withtheir children’s screening result.[Keywords]developmental disability, developmental disorder,screeningVAGUS NERVE STIMULATION TREATMENTFOR CHILDHOOD REFRCCTORY EPILEPSY INCHANG GUNG CHILDREN MEDICAL CENTEROF TAIWANChuan-Yu Wang, MD, PhD 1 *, Chen-Nen Chang, MD 2 *,Hsu-Tung Lee, MD 3 * and Dah-Chin Yan, MD 4 *1 Taipei division of Pediatric Neurology , Chang Gung Children Hospital , Linko,Taiwan; 2 Department of Neurosurgery, Chang Gung Memorial Hospital, Linko,Taiwan; 3 Department of Neurosurgery, Taichung Veterans General Hospital,Taiwan; 4 Taipei division of Pediatric Immunology , Chang Gung ChildrenHospital ,Linko, TaiwanPURPOSE: Vagus Nerve Stimulation (VNS) isapproved by FDA in 1997 for adjunctive therapy formedical intractable epileptic patients. In Taiwan, the firstModel 102 VNS was implanted since August,2007(almost adult patients) under informed consent andIRB certification. To investigate VNS effect forPediatric' patient, we collected intractable epilepticchildren for this study.METHODS: 7 refractory epileptic patients aging from2~17 Y/O were enrolled into the study and the data of 3months before VNS implantation, including seizuredairy and quality of life variables, and the patients werefollowed for 1 year. Patients’ classification of epilepsyincluded 2 Lennox-Gastaut syndrome, Autism withlocalized epilepsy, Progressive Myoclonic Epilepsy,Rasmussen syndrome, Severe migration partial seizuresin infancy, Cryptogenic parietal lobe epilepsy. Theseizure reduction rate, EEG, BAEP, SPECT, brain MRIand intelligence test were done before and afterperformance of VNS for 1/2~1 year.RESULTS: The seizure reduction rate was more than50% in all patients, and EEG showed mild ameliorationof spikes foci frequency (5/7), and interictal SPECTshowed elevation of perfusion over basal ganglia ordiffuse cortical areas in most patients (4/7) after VNSimplantation. Life quality quotient test and Depressionquotient test were both improved, but IQ test improvedjust a little without significant change. BAEP and brainMRI were not changed.CONCLUSION: (1) Seizure reduction rates weremore than 50% after implantation for 1 year in thesecases; (2) Quality of life, such as: alertness, mood andlanguage function improved prominently; (3) EEG,SPECT were improved with mild degree; (4) Partialseizure is eliminated prominently in seizure reduction byVNS therapy, and focal discharges were diminishedeasily than generalized one. (5) The relationship betweenVNS performance and parameter setting will be stillunder investigation.[Keywords] vagus nerve stimulation, parameter setting, seizurereduction, quality of life improvement280

P2-053 Neuropsychiatry (Neurology) P2-054 Neuropsychiatry (Neurology)CASPASE3 EXPRESSION AND APOPTOSIS INRAT HIPPOCAMPUS FOLLOWINGELECTRICAL KINDLED SEIZURESCHEN Xuqin, WANG Zhedong, BAO Guanshui, et alNeurological Department of Soochow University Children’s Hospital,SuZhou, Jiangsu, China 215003CHANGES OF SERUM FERRITIN AND BLOODLEAD LEVELS IN CHILDREN WITHATTENTION DIFICIT HYPERACTIVITYDISORDERHui- Ming YANG, Ji-hua CUIWest China Second University Hospital, Sichuan University, Chengdu 610041,ChinaOBJECTIVE: To study the Caspase3 expressionchange and its relationship with cells apoptosis in thehippocampus of rats after electrical kindled seizures.METHODS: Bipolar electrodes were implanted inBLA in rats, through which the rats received chronicelectrical stimuli for kindling to make amygdalaelectrical kindled epilepsy model. Caspase3mRNAwas detected with semi-quantitative RT-PCR. TheCaspase3 protein was detected withimmunohistochemical staining, meanwhile cellsapoptosis was also detected with TdT-mediateddUTP-biotin nick end labeling (TUNEL).RESULTS: 24 hours after epileptic seizures, both ofthe expression of Caspase3mRNA and Caspase3protein were increased markedly (p

P2-055 Neuropsychiatry (Neurology) P2-056 Neuropsychiatry (Neurology)THE RELATIONSHIP OF THE RISK FACTORAND THE PROGNOSIS OF AGENESIS OFINFLUENZA B ENCEPHALITIS WITH UNCALHERNIATION: A CASE REPORTCORPUS CALLOSUMYun-Hsuan Yang , Huei-Shyong Wang, Kuang-Lin Lin,Po-Cheng Hung, Min-Liang Chou, Meng-Ying Hsieh,Reyinl LienDepartment of Pediatric, Chang Gung Memorial Hospital, Lin-Ko, TaiwanOBJECTIVES: Agenesis of the corpus callosum (ACC) consistsof a heterogeneous group of disorders that vary in expression fromsevere intellectual and neurologic abnormalities to theasymptomatic and normally intelligent individual. When agenesisof the corpus callosum is an isolated phenomenon, the patient maybe normal, whereas individuals with neurologic symptoms,including mental retardation, microcephaly, hemiparesis, diplegia,and seizures, have associated brain anomalies due to cellmigration defects, such as heterotopias, microgyria, andpachygyria in addition to the absence of the corpus callosum.Prognosis of ACC depends on the extent and severity ofmalformations. ACC does not cause death in the majority ofchildren. Mental retardation does not worsen. Although manychildren with the disorder have average intelligence and leadnormal lives, neuropsychological testing reveals subtle differencesin higher cortical function compared to individuals of the sameage and education without ACC. Our aim is to study therelationship of the risk factor and the prognosis of ACC.METHODS: We retrospectively reviewed the patients diagnosedof ACC in Chang Gung Children’s Hospital. The case number was49 and the following duration was from 1999 April to 2009 atpresent. Clinical histories were reviewed including birth history,admission course, genetic analysis, image study and outpatientfollow up. Risk factors evaluated including age, sex, birthcondition (preterm/term, NSD/CS, AS), ACCpattern(isolated/combined with other malformations), time ofdiagnosis, EEG pattern, congenital anomaly and maternalcondition. We use Glasgow Outcome Scale to evaluate theprognosis of the patient.MAIN RESULTS: There were 24 male and 25 female patientsand average age was 7-year-6-month old (11-month-old to11-year-1-month old). 27 patients were fullterm and 9 wherepreterm babies when birth (13 patients had no birth data). 5patients were diagnosed by prenatal sonography and 44 patientswere diagnosed after birth by brain echo, brain MRI or brain CT.30 patient were isolated ACC and other 19 patients were ACCwith other malformations. Only one patient was partial ACC and15 patients were complete ACC confirmed by brain MRI or brainCT. 33 patients received EEG study and 7 patients were normalwhereas other 26 patients had cortical dysfunction or epileptiformdischarge. 19 patients had congenital anomaly. We evaluated 32patients by Glasgow Outcome Scale and 16 patients were higherthan score 3 whereas 16 patients were lower than score 4. Wefound that Apgar score and congenital anomaly had relationshipwith prognosis of ACC (95% CI 1.205-13.283 P=0.010; 95% CI0.014-0.499, P=0.005).CONCLUSION: Although prognosis of agenesis of corpuscallosum depends on the extent and severity of malformations, wefound that Apgar score and congenital anomaly also havecorrelation with prognosis. However, the further clinicalpresentation and treatment effect still need to be studied in thefuture.Zheng-Nan Chin 1 , Yu-Tzu Chang 1 , I-Ching Chou 1 *,Sung-Hsi Wei 3 , Huang-Tsung Kuo 2 and Chang-Hai Tsai 11 Division of Pediatric Neurology, Children’s Medical Center, China MedicalUniversity Hospital, Taichung, Taiwan; 2 Division of Development and BehaviorPediatrics, China Medical University Hospital, Taichung, Taiwan; 3 TaiwanCenters for Disease Control, TaiwanOBJECTIVE: Influenza A and B viruses primarily cause aself-limited upper respiratory tract infection. Complicationsfrom influenza A or B virus infection include pneumonia,myositis, myocarditis, and encephalitis. Although encephalitishas been associated with both influenza A and B virusinfection, this complication appears to be less frequent withtype B infections. We reported a case of influenza Bencephalitis associated (IBAE) who was characterized by rapidonset of neurologic sign and progressed to death, and severebrain edema with uncal herniation.METHODS: A 9 years old Taiwanese female presented withfever, dizziness, myalgia, decreased in appetite and cough for 2days. Six hours prior to admission, high fever up to 40.5℃,conscious disturbance, eye upward gaze, and flaccid withoutcyanosis were noted. Four hours later, generalized seizureattack again for 5 minutes. Physical examination found flaccidand comatose girl with bilateral pupil dilatation and withabsence of light reflex. Mild retinal hemorrhage andpapilledema was found by funduscopy.MAIN RESULTS: Cerebrospinal fluid analysis revealed nopleocytosis but elevated protein. Brain CT, performed 11 hoursafter seizure attack, revealed diffused brain edema with uncalherniation. This image finding was rarely reported in previousmedical literature. Patient expired 29 hours after admissionbecause of multi-organ failure.CONCLUSION: In our review, majority of the influenza Bassociated encephalitis (IBAE) cases fully recovered butminority of them suffered from neurologic sequela or evendeath. The image findings (CT or MRI) were consisted withvarious characteristics, which included normal findings,normal findings in the acute phase followed by mild brainatrophy, diffuse cortical necrosis, symmetric thalamic lesions,and diffused brain edema in acute phase followed by normalfindings. In Taiwan, an unpublished data from Centers ofDisease Control, Taiwan reported 8 patients had evidence ofinfluenza B infection, included our case. Two patients hadcommon characteristics of brain edema but only our patienthad uncal herniation. Influenza B associated encephalitis(IBAE) has very low worldwide prevalence. The manifestationof rapid deterioration brain edema to uncal herniation is evenrarer. Clinical physician should be aware of this fatalcomplication.[Keywords] Influenza B, encephalitis, brain edema[Keywords]Agenesis of corpus callosum, risk factor, prognosis282

P2-057 Neuropsychiatry (Neurology) P2-058 Neuropsychiatry (Neurology)DEVELOPMENTAL REGULATION OFACETYLATION STATUSES OF HISTONEPROTEINS IN NEURAL PROGENITORCELLS OF MURINE CEREBRAL WALLTHYROXINE POST-TREATMENT PROTECTPERIVENTRICULAR WHITE MATTER INJURYFROM HYPOXIA-ISCHEMIA INSULTS INIMMATURE RAT PUPSTakayuki Mitsuhashi, Takao TakahashiDepartment of Pediatrics, School of Medicine, Keio University, JapanOBJECTIVE: For any given population of proliferativeprogenitors during histogenesis, progenitor cells have afixed number of cell division cycles which is predefined bya sequence of activation of number of genes. The exampleof such rule of thumb is neocortical histogenesis inmammals, where the ultimate structure is orderlyorganized in six-layer pattern. Neuron production occurs ina strictly defined region that is within the ventricular zonesurrounding lateral ventricles of the embryonic forebrain:number of cell divisions of neural progenitor cells (NPCs)is critically regulated, for example, 11 and 28 cycles inmice and in rhesus monkeys, respectively. In this study, weexamined the epigenetic regulation of a cascade of criticalevents within a specified population of NPCs wheredevelopmental-stage-dependent gene expression profile isexpected to serve as the key modulator. Especially, we putfocus upon acetylation statuses of histone proteins in NPCsbecause acetylation of lysine residues of histone proteins isconsidered to be a critical epigenetic mechanism forvarious cell cycle regulatory gene expressions.METHODS: Embryonic day (E) 10, 12, 14, and 16 mouseembryonic forebrains were isolated from wild type miceand dorsomedial cerebral wall were dissected underdissecting microscope. Tissues were homogenizedmanually and mechanically by pellet pestle. Proteins wereseparated by SDS-PAGE and blotted to supportednitrocellulose membranes. Immunoblot analyses wereconducted by using anti-acetylated histone antibodies,which recognize lysine residue-specific acetylated histoneproteins.RESULTS: Acetylation statuses of histone H3 lysine9/27and histone H4 lysine 8 residues were altered in NPCsduring the course of neuronogenesis. The acetylationstatuses of other residues including lysine 14 and 23 inhistone H3 and lysine 5 and 16 in histone H4 were notsignificantly changed as neuronogenesis proceed.CONCLUSION: Lysine residue-specific acetylationstatuses of histone proteins in NPCs appear to bedevelopmentally regulated during the course ofneuronogenesis. We consider that this mechanism mightprovide a pivotal clue for investigating epigeneticregulation of cell division number of NPCs in mammals.The observations suggest that a balance between functionsof histone acetyl transferase (HAT) complex, whichacetylates lysine residues of histone proteins, and histonedeacetylase (HDAC) family of proteins, which removeacetyl group from lysine residues, is critical for theprogression of cell division of the NPCs.[Keywords] development, cerebral cortex, epigenetics, cell cyclePI-LIEN HUNG 1 , YING-CHAO CHANG 1 , DOM-GENETU 2 , CHAO-CHING HUANG 3 , HSIU-MEI HUANG 41.Department of Pediatrics, Chang Gung Memorial Hospital- KaohsiungMedical Center, Chang Gung University College of Medicine, Kaohsiung,Taiwan; 2.Department of Nuclear Medicine ,Chia-Yi Christian Hospital,Chia-Yi, Taiwan; 3.Department of Pediatrics , National Cheng Kung UniversityCollege of Medicine, Tainan,Taiwan; 4.Department of Ophthalmology, ChangGung Memorial Hospital- Kaohsiung Medical Center, Chang Gung UniversityCollege of Medicine, Kaohsiung, TaiwanOBJECTIVE: Periventricular white matter injury is the leadingcause of cerebral palsy in premature infants for which no treatmentwas available. Myelin disruption, and depletion of oligodendrocyteprogenitors (pre-OLs) is central to the pathogenesis of PVL.Thyroid hormone is required for early brain development,especially for the maturation of immature oligodendrocytes. Wetested the hypothesis that exogenous thyroxine (T4) post-treatmentcan protect periventricular white matter injury and preserved thedepletion of pre-OLs in immature rat brains.METHODS: Periventricular white matter injury was establishedby unilateral common carotid artery ligation followed by 6.5%oxygen hypoxia for 1 hour in P7 rat pups. The pups were thendivided into four group: naïve, vehicle, T4 200μg/Kg (T4-2), T4 1mg/Kg (T4-10) at P7, P9, P11 with the first regimen injectedintraperitoneally immediately after hypoxia-ischemia. Functionalrecovery was assessed by neurobehavior tests, including gaitanalysis, climbing incline angles and beam walking at P11 andP21. Immunohistochemical staining for myelin basic protein(MBP), pre-OLs markers (O1, O4) and reactive astrogliosismarkers (GFAP, ED1) were examined at P11. To investigate thestatus of cellular proliferation and apoptosis, bromodeoxyuridine(BrdU) staining and terminal deoxynucleotidyl transferase -mediated deoxyuridine triphosphate nick-end labeling (TUNEL)stain were used, respectively, at P11.MAIN RESULTS: Compared with the vehicle-treated PVL group,T4-10-treated pups had significantly both motor score, includinggait analysis and climbing incline angles at P11. At P21, T4-10also performed better beam walking task than the vehicle-treatedPVL group. At neuropathologic levels, HE & FJC stain revealedno significant differences in neuron damage between groups. T4post-treatment increased ipsilateral periventricular myelination, asdemonstrated by MBP staining.There was also increased O4, O1 immunostaining in T4-10-treatedgroup as compared with the vehicle-treated PVL group. Astrocyteactivation, as demonstrated by GFAP staining, was decreased inT4-10 treated group. ED1 immunoreactivity showed strong andscatter in the deep cortical layer and periventricular white mattertin the vehicle and T4-2 treated groups while it seemed lighter andmore concentrated in periventricular white matter in T4-10 treatedgroup. Thyroxine post-treatment attenuated reactive astrogliosis inPVL. Both T4-2 and T4-10 reduced proliferation of pre-OLs inperiventricular white matter by BrdU staining. However,significantly decreased periventricular white matter apoptosis byTUNEL staining was only noted in T4-10-treated group.CONCLUSION: The present study suggests that moderate doseof thyroxine post-treatment can protect periventricular whitematter injury from hypoxia-ischemia insult in developing brain atboth behavior level and pathologic levels.283

P2-059 Neuropsychiatry (Neurology) P2-060 Neuropsychiatry (Neurology)DIETARY RESTRICTION REDUCED BRAINDAMAGE CAUSED BY NEONATALHYPOXIC-ISCHEMIA THROUGH INSULININTRAVENOUS VALPROIC ACID FORCONTROL OF STATUS EPILEPTICUS INCHILDREN: A META ANALYSISSIGNALING PATHWAYYi-Fang Tu 1,2 , Pei-Jung Lu 1 , Chao-Ching Huang 1,31 Graduate Institute of Clinical Medicine, National Cheng Kung UniversityCollege of Medicine, Tainan, Taiwan;Departments of 2 Emergency Medicineand 3 Pediatrics, National Cheng Kung University College of Medicine andHospital, Tainan, TaiwanOBJECTIVE: Neuroplasticity altered by perinatalprogramming may aggravate or protect againstneonatal hypoxic-ischemia (HI) brain injury. Weexamined whether metabolic plasticity induced bydietary restriction (DR) during the early lactationperiod provided neuroprotection against HI inneonatal rats through insulin signaling pathway.METHODS: Sprague-Dawley pups were groupedfrom postnatal day (P) 1: normal birth litter size (NL)rats (12pups/dam) and DR rats (18 pups/dam). OnP7, pups were subjected to HI induced by modifiedRice-Vannucci model. Outcomes and signalingpathways were investigated. In vitro study, theoxygen glucose deprivation (OGD) model inhippocampal neuronal cells H19-7 was established tofurther prove the causal signaling pathways.MAIN RESULTS: DR rats significantly reducedbody weight and brown and white adipose tissue onP7. After subjecting to HI on P7, post-HI DR ratsreduced TUNEL-positive cells andapoptosis-associated proteins (caspases and Poly(ADP-ribose) polymerase). The adult post-HI DRrats behaved significantly better learning ability andhad less brain-volume loss than adult post-HI NLrats. The level of insulin receptor substrate-1 (IRS-1)in insulin signaling pathway was noted to bepreserved in post-HI DR rats and degraded inpost-HI NL rats. It leaded to activate PI3K/Aktpathway in post-HI DR rats. Over-expression andknockdown of IRS-1 in H19-7 caused increased anddecreased cell viability after OGD, respectively.Knockdown of IRS-1 further reduced the activationof PI3K/Akt and increased the level of cleavedcaspase 3.CONCLUSION: Metabolic plasticity induced byDR during the early lactation period providesneuroprotection against neonatal HI brain injurythrough insulin signaling pathway.[Keywords] Dietary Restriction, Neonatal Hypoxic-ischemiaEncephalopathy, Insulin Receptor Substrate-1Pamela T. Roxas, M.D.De La Salle University Health Sciences Institute, Dasmarinas Cavite, PhilippinesOBJECTIVE: The primary outcome is to establish theefficacy and safety of intravenous valproic acid incontrolling status epilepticus in children aged 3 monthsto 18 years old.METHODS: A search was done in Medline, Pubmed,Cochrane, EMBASE, CENTRAL using the Mesh terms"status epilepticus", " prolonged seizures", " intravenousvalproic acid", "valproic acid", "intravenous sodiumvalproate", "sodium valproate" and "children" for trialspublished from 1990-2009. Cross reference withbibiliogaphies, search for unpublished and local trials wasdone. Randomized controlled trials on the use ofintravenous valproic acid versus another anti epilepticdrug for control of status epilepticus in children ages 3months to 18 years old were included. Data wereextracted independently and quality of included trialswere assessed independently using standard forms. Datawas analyzed using Review Manager 5.MAIN RESULTS: A total of 3 randomized controlledtrials and population of 208 subjects were included.Intravenous valproic acid is 76.63% effective incontrolling status epilepticus in children. (OR= 0.49,95% CI 0.23 to 1.04, p=0.06) Loading dose of20-40mg/kg and infusion rates of 2-5 mg/kg/min wasused. Its safety is well tolerated with a statisticallysignificant freedom from adverse events of respiratorydepression (OR=0.10, 95% CI 0.02 to 0.04) andhypotension (OR= 0.06, 95% CI 0.01 to 0.32).However, elevation in liver enzymes was thrice morelikely in treated patients (0R 3.08, 95% CI 0.71 to13.29). Breakthrough seizures for 6 hours from initiatonof treatment was 5% less likely for the valproic acidtreated children. (OR 0.95, 95% CI 0.41 to 2.16)Neurologic outcome and mortality did not differ betweenthe two groups.CONCLUSION: Intravenous valproic acid is effectivefor control of status epilepticus in children and may be abetter alternative for those at high risk for adverse eventswith the first line drugs and for refractory cases.[Keywords]status epilepticus, children, valproic acid, sodiumvalrproate284

P2-061 Neuropsychiatry (Neurology) P2-062 Neuropsychiatry (Neurology)PRENATAL STRESS IN RATS CAUSELONG-TERM SPATIAL DEFICIT: EFFECT OFPOSTWEANING ENRICHED ENVIRONMENTTHE NEUROCOGNITIVE CHARACTERISTICS OFSCHOOL-AGED EPILEPTIC CHILDREN WITHPRECEDING FEBRILE SEIZURESYing-Chao Chang a , Nai-Wen Guo b , Chao-ChingHuang c*a Department of Pediatrics, Chang Gung Memorial Hospital-KaohsiungMedical Center, Chang Gung University College of Medicine, Kaohsiung,Taiwan; b Institute of Behavioral Medicine, National Cheng Kung UniversityCollege of Medicine, Tainan, Taiwan; c Institute of Clinical Medicine andDepartment of Pediatrics, National Cheng Kung University College ofMedicine, Tainan, TaiwanLi-Tung HuangDepartment of Pediatrics, Chang Gung Memorial Hospital-Kaohsiung MedicalCenter, Chang Gung University College of Medicine, Kaohsiung, TaiwanBACKGROUND: Febrile seizures (FS) are the mostcommon seizure disorder in childhood. Long-termfollow-up studies have shown that children with FS havecomparable neurocognitive development compared to thecontrols. However, children with FS are at a four- tofivefold increased risk for subsequent epilepsy. No studieshave characterized the neurocognitive profiles of thosechildren with FS and subsequent epilepsy.METHODS: In a hospital-based prospective study, aneurocognitive profile, including WISC III, comprehensivenon-verbal attention test, comprehensive non-verbalmemory test, Guo’s verbal memory test, Wisconsin cardsorting test, Peabody picture vocabulary test, test ofvisuo-perceptual skills, were compared in children withepilepsy and preceding FS (FSE group, n=110), theirhealthy siblings controls (sibling group, n=70), andchildren with FS only (FS group, n=94).RESULTS: There were no significant group differences ingender or socioeconomic status. The mean age at the timeof the first FS and FS recurrence were similar for FSE andFS groups, but the children in FSE group had more familyhistory of epilepsy than the FS group. The age of the firstunprovoked seizures onset was 65.4 months. There werehigh incidence of intractable epilepsy (29.4%), statusepilepticus (24.8%), and remote symptomatic epilepsy(37.8 %) in the FSE group. Fifteen percent of FSE childrenhad benign epilepsy syndrome, including generalizedepilepsy and FS plus, rolandic epilepsy and absence,whereas 5.6 % had refractory temporal lobe epilepsy. TheFSE group children obtained statistically significantlyworse scores than the siblings and FS groups in all theneurocognitive domains, including attention, intelligence,language, working memory, executive function, andvisuospatial function. There were no significant differencesbetween the FS group and siblings groups in most of theneurocognitive domains. The neurocognitive differencesbetween FSE and the control groups could not be ascribedto any of the FS or epilepsy characteristics, but to theunderlying diseases and the intractability of seizures.CONCLUSION: Significant neurocognitivedisadvantages were found in the children with FS andsubsequent epilepsy. It is important to follow thesehigh-risk children for further early intervention.OBJECTIVES: To evaluate the effects of prenatal stresson hippocampal synaptic plasticity in offspring and thevalue of postnatal enriched environment.METHODS: Pregnant dams were left undisturbed orrestrained 6 h per day from days 14 to 21. Control andprenatal stressed offspring rats were subjected to anormal rearing environment or an enriched environmenton postnatal days 22-120. Adult offspring (n=6-10, eachgroup) performed Morris water maze at ~4 months ofage. After completion of maze test, rats (n=5-6, eachgroup) were subjected to western blot analyses forN-methyl-D-aspartate receptor subunits (NR) andsynaptophysin, and quantitative PCR for β-integrin andtissue-plasminigen activator (t-PA).MAIN RESULTS: Prenatal stress offspring showeddecreased NR2B, β-integrin, t-PA, and synaptophysin.Enriched environment treatment on postnatal days22-120 counteracted the spatial deficit and increasedhippocampal NR1 expression.CONCLUSION: The results of this study indicateprenatal stress in rats cause long-term spatial deficit andaltered hippocampal signaling transduction. Postnatalenriched environment treatment has differential benefitsin terms of spatial learning and signaling transduction.[Keywords]Prenatal stress; spatial learning; NMDA receptor;synapse; enriched environment;[Keywords] febrile seizures, epilepsy, neurocognition285

P2-063 Neuropsychiatry (Neurology) P2-064 Neuropsychiatry (Neurology)PREVALENCE OF PRESUMPTIVE READINGDISORDER AMONG GRADE TWOSTUDENTS IN PUBLIC SCHOOLS OFCLINICAL ANALYSIS OF PATIENTSSUSPECTED OF HAVING NOVEL INFLUENZA A(H1N1) ENCEPHALOPATHYDISTRICT 1 DASMARIÑAS, CAVITE ADESCRIPTIVE CROSS SECTIONAL STUDYJasmin Morales MarasiganPediatrics, DLSU HIS, PhilippinesHiroshi IDEGUCHI 1 , Yukiko IHARA 1 , Yuko TOMONOU 1 ,Takahito INOUE 1 , Sawa YASUMOTO 1 , Shinichi HIROSE 1Institution of First Author 1 , Institution of Second Author 2 , Institution of ThirdAuthor 3; Department of Pediatrics, Faculty of Medicine, Fukuoka University,Fukuoka, JapanBACKGROUND: Reading disorders are present inapproximately 75% of children and adolescents withlearning disorders. Prevalence studies find ratesranging between 2 to 8 percent. Students who havelearning problems in other academic areas mostcommonly experience difficulties with reading aswell. The child may become frustrated by thedifficulty in learning to read and other problems canarise which can jeopardize the child’s success in theschool and in the future. Moreover, readingdifficulties that are not intervened at by 8 year of agetend to persist.OBJECTIVE: To determine the prevalence ofpresumptive reading disorder among grade twostudents in public schools of District 1 Dasmarinas,Cavite.DESIGN: Cross-sectional descriptive study.SUBJECTS: Grade two students from 15 publicschools of District 1 Dasmarinas, Cavite.METHODOLOGY: Stratified random samplingwas used and twenty grade two students wererandomly selected from each 15 Public schools tocollate 300 sample size. The PediatricNeuropsychology Screening Examination on SingleWord Reading was used to screen the students forpresumptive reading disorder.OUTCOME MEASURES: Students who screenedhaving presumptive reading disorder.RESULTS: The prevalence of presumptive readingdisorder was 8.3% with a mean age of 7.84 + 1.248years, found higher among females at 56%.Associated difficulties in written language were alsoidentified.CONCLUSION: Presumptive reading disorder is ascommon among grade two students in public schoolsof Dasmarinas, Cavite as elsewhere. Hence, earlyscreening for all elementary students for readingdisorder is recommended.OBJECTIVES: Stating concisely why the study wasconducted. To analyze the clinical and neurologicalsymptoms and histories of patients who were suspectedof having novel influenza A (H1N1) encephalopathy.METHODS: An explanation of the study design andexperimental methods used From August to November2009, 19 children were admitted to Fukuoka UniversityHospital with suspected encephalopathy due to H1N1infection. The diagnosis of H1N1 infection was made bya rapid influenza test. Here, we investigate their clinicalsymptoms, previous histories, and final diagnoses.MAIN RESULTS: A concise summary of the majorfindings of the experiment or study. The 19 childrenranged from 3 to 12 years of age (average 8 years); 10were boys, 9 girls. Their clinical symptoms showedconsciousness disturbances and hallucinations (6),convulsive seizures and consciousness disturbances (5),convulsive seizures (3), consciousness disturbances (2),convulsive seizures and hallucinations (1),consciousness disturbances, hallucinations, and ataxia(1), hallucinations (1). Previous histories showed febrileconvulsion (6), bronchial asthma (2), acuteencephalopathy (1), and epilepsy (1). Before admission,oseltamivir was given to 8 of the patients, rimantadine to6, and 5 received nothing. We treated withmethylprednisolone pulse therapy 3 children who, onadmission, had high amplitude slowed wave EEGs. In allcases, consciousness disturbances improved the day afteradmission. Our final diagnosis included complex febrileseizures (8), febrile delirium (7), acute cerebellar ataxiawith febrile delirium (1), influenza encephalopathy (1),epilepsy (1), and moyamoya disease (1).CONCLUSION: Summary of the overall findings andthe importance of the study. Patients with a history offebrile seizures are at risk for neurological complicationson H1N1 infection.[Keywords] Arial font, Bold, size 10,)novel influenza[Keywords]reading disorder, reading disabilities, dyslexia,primary school students286

P2-065 Neuropsychiatry (Neurology) P2-066 Neuropsychiatry (Neurology)SECONDARY MALIGNANT NEOPLASMSA STUDY OF ASSOCIATION BETWEENFOLLOWING THE TREATMENT OF PRIMARY EPILEPSY ATTACK AND SLEEPING CYCLE INBRAIN TUMORS IN CHILDRENCHILDRENChung-Hao Wang 1* , Kai-Ping Chang 1 , Ting-Rong Hsu 1 ,Tzu-Ying Yang 1 , Tai-Tong Wong 2 , Feng-Chi Chang 3 ,Donald Ming-Tak Ho 41 Department of Pediatrics, 2 Division of Pediatric Neurosurgery,Department of Neurology, 3 Department of Radiology, 4 Department ofPathology and Laboratory Medicine, Taipei Veterans General Hospital,Taipei, Taiwan, R.O.C.Rong Luo, Tang Xiao Cai, Lan Jie zouPeadatric neurology, West China Second University Hospital, SichuanUniversity, ChinaOBJECTIVE: Brain tumors are the most common solidtumors in children. Secondary malignant neoplasm is a knowncomplication following radiotherapy and chemotherapy. Thisstudy analyzed the secondary malignant neoplasms that havedeveloped as a result of the treatment of primary brain tumorsin children.METHODS: We retrospectively analyzed 615 pediatricpatients with primary central nervous system tumors who wereevaluated and treated at our hospital between January 1, 2000and December 31, 2009. The primary treatment strategy wasgross total removal by surgery whenever possible.Histopathologic examination was done according to the WorldHealth Organization classification. There were 378 patientswho received adjuvant radiotherapy with or withoutchemotherapy or chemotherapy alone. Any newly developedneoplasm that was distinct from the initial tumor byhistopathology was considered to be a secondary neoplasm.The anaplastic transformation from low-grade gliomas tohigh-grade gliomas was excluded. Patients who had adiagnosis of neurofibromatosis type 1 and type 2 were alsoexcluded because of their known predisposition to developsecondary neoplasms.MAIN RESULTS: There were six patients (three males andthree females) developed secondary malignant neoplasms aftertreatment of primary brain tumors with surgery, radiotherapy,with or without conventional intravenous chemotherapy. Themean age of the patients at the time of the initial diagnosis was8.3 years (ranged 5-13 years). The overall incidence rate was1.6% (6/378). The histopathologic diagnosis of primary braintumors were atypical teratoid/rhabdoid tumor (n=2), germ celltumor (n=2), ependymoma (n=1), and medulloblastoma (n=1).The histopathologic diagnosis of secondary malignantneoplasms were malignant spindle cell tumor (n=1),osteosarcoma (n=1), rhabdomyosarcoma (n=2), atypicalteratoid/rhabdoid tumor (n=1), and acute lymphoid leukemia(n=1). The median latency time to the development of thesecondary malignant neoplasms was 3.4 years (ranged 1.8-6.5years). Four patients died with a mean survival period 1.4years (ranged 0.8-2.5 years) after secondary malignantneoplasms diagnosed. Two patients still survive now with amean following period 1.3 years after secondary malignantneoplasms diagnosed.CONCLUSION: A wide spectrum of secondary malignantneoplasms was found after the treatment of primary braintumors in children. Most commonly secondary malignantneoplasms occurred in the central nervous system, followed byhematological malignancy. The prognosis was poor of thesecondary malignant neoplasms.OBJECTIVE: To explore whether sleep cycle affect theseizure of epilepsy as epileptiform discharge that couldbe most easily detected during non-rapid eye movementsleep.METHOD: Sleep cycles of 95 cases with total 504seizure attacks were analyzed by video-EEG.RESULTS: 75 percent of seizures occurred duringwaking and 25 percent of seizures occurred duringsleeping. Among the seizures during sleeping, 85.7%appeared during the second stage of NREM, thirty-sevenpercent during the first stage, 12.7 percent during thethird and the fourth stage, 0.02 percent during REM. Thefrontal epilepsy and temporal epilepsy and Rolandicepilepsy were prone to occur during sleeping, theoccurrence rates were 40.6% 、 33.3% 、 38.7%respectively.CONCLUSIONS: NREM can induce the seizure attack inchildren confirmed epilepsy, the type of focal epilepsywere more easily induced during NREM than others.[Keywords]Epilepsy Attack, Sleeping Cycle, Children[Keywords] Secondary Malignant Neoplasm, Brain Tumor287

P2-067 Neuropsychiatry (Neurology) P2-068 Neuropsychiatry (Neurology)ANTI–N-METHYL-D-ASPARTATERECEPTOR ENCEPHALITIS –TWO CASESREPORTFangHsuan Tu 1 , SongChei Huang 1 , YingChao Chang 1 ,ChaoChing Huang 2 , JiinHaur Chuang 31. Department of Pediatrics, Chang Gung Memorial Hospital-KaohsiungMedical center, and Chang Gung University College of Medicine,Kaohsiung, Taiwan; 2. Institutes of Clinical Medicine and Department ofPediatrics, National Cheng Kung University College of Medicine, Taiwan3. Department of Pediatric Surgery, Chang Gung MemorialHospital-Kaohsiung Medical center, and Chang Gung University College ofMedicine, Kaohsiung, TaiwanBACKGROUND: Anti-N-methyl-D-aspartate receptor(NMDAR) encephalitis is a recently described raredisorder with a well defined set of clinical features, such aschanges of mood, behavior, and personality, resemblingacute psychosis. Without early treatment, the patientsusually progress to seizures, decreased level ofconsciousness, dyskinesias, autonomic instability,hypoventilation, and sometimes to mortality. Sixty percentof young female patients with anti-NMDAR encephalitishave ovary tumor, mainly teratoma. Removal of tumorwith immunotherapy or plasmapheresis is the treatment ofchoice. To our knowledge, there was no such casesreported in Taiwan.METHODS: We report 2 female adolescents withcharacteristic clinical manifestation of anti-NMDARencephalitis.RESULT: Case 1, a 17-year-old female, initially presentedwith progressive agitation for 3 weeks followed bydeteriorated consciousness level, orofacial dyskinesias,flailing of four extremities and hypoventilation. All bloodtests, including haemogram, biochemistry, virology,immunity, and toxicology were negative. The CSF studyrevealed pleocytosis with lymphocyte predominant andnormal sugar and protein level. Brain and pelvic MRI werenormal. EEG showed diffuse cortical dysfunction. Therewas mildly elevated anti-NMDAR antibody in serum andstrongly positive in her CSF. She got prominentimprovement after twice intravenous immunoglobulintreatment. She is completely normal now, 9 months afterthe disease onset. Case 2, a 14-year-old female, presentedwith behavior change for 2 weeks followed by progressivelethargy. Then she had orofacial dyskinesias and emotionlability. All blood test was negative. Her CSF study wasnegative, without pleocytosis. Brain MRI and EEG werenegative. However, bilateral ovary tumor was noted byabdominal CT. She received operation and hadimprovement after methylprednisolone pulse therapy.CONCLUSION: Anti-NMDAR encephalitis should beone of the differential diagnosis in female adolescentspresenting with acute psychosis and deterioratedneurologic symptoms, especially orofacial dyskinesias.Ovary tumor should be surveyed in these patients, becauseearly removal of tumor may shorten the course of disease.[Keywords]anti-NMDAR encephalitis, acute psychosis, orofacialdyskinesia, teratomaCOMPARISON OF TRAIT EMOTIONALINTELLIGENCE OF CHILDREN IN CONFLICTWITH THE LAW AND NON-HIGH RISKADOLESCENTSKristyn A. Yatco, MDDe La Salle University Health Sciences Institute, Dasmariñas,Cavite, PhilippinesOBJECTIVE: To compare the trait emotionalintelligence of Children in Conflict with the Law (CICL)and non-high risk adolescents aged 14-18 years old.METHODS: A matched case control study done inDSWD Centers and a public high school of Dasmarinas,Cavite which included 38 CICL and 38 Non-high riskadolescents. Assessment of Trait Emotional Intelligence(EI) was facilitated using the Trait EmotionalIntelligence Questionnaire – Adolescent Short Form.Questionnaire on socio-demographic data was alsoincluded. The Mean EI scores were measured andcomparison using Mc Nemar’s paired t-test wasanalyzed. Outcome measures comprise thesocio-demographic data of the study population andemotional intelligence scores which includes four factorsand facets under each factor measured in trait EI.RESULTS: The mean age of the study population is 16.4years old with a male-to-female ratio of 6:1. The studyshowed a statistically significant difference among thetwo groups in terms of educational attainment of thesubjects, educational attainment of mothers, history ofachievement and truancy of both groups. No statisticaldifference was seen on parental custody, occupationalstatus of parents and history of violence. Comparisonof trait EI of the two groups was statistically significantrevealing a lower trait EI mean score in the CICL group.The factors which had statistical significance arewell-being and sociability and the facets identified to becontributory are optimism, happiness, impulsiveness andemotion management.CONCLUSION: The trait EI of CICL is lower comparedto Non-high risk adolescents. It is necessary to focusdiversion and intervention programs on the significantfacets mentioned to help in the rehabilitation of thesechildren.[Keywords]Children in Conflict with the Law (CICL), JuvenileDelinquency, Trait Emotional Intelligence (EI), TEIQue-ASF288

P2-069 Neuropsychiatry (Neurology) P2-070 Neuropsychiatry (Neurology)SCHOOL PERFORMANCE OF CHILDRENDIAGNOSED WITH SEIZURE DISORDERAGES 6-19 YEARS OLD AT DE LA SALLEHEMISPHERECTOMY IN CHILDREN OFREFRACTORY EPILEPSY: FOUR CASES INVGHTPEUNIVERSITY MEDICAL CENTEROUT-PATIENT DEPARTMENT FROM THEYEAR 2006-2008BETHZAIDA RACHEL S. FABIAN, M.D.Pediatrics, De La Salle University Health Sciences Institute,PhilippinesTzu-Ying,Yang 1 , Kai-Ping Chang 1 , Chung-Hao Wang 1 ,Ting Rong Hsu 1 , Tai-Tong Wong 2Department of Pediactrics, Taiwan, Taipei Veterans General Hospital 1 , Divisionof Pediatric Neurosurgery, Neurological Institute, Taipei Veterans GeneralHospital, Taipei 2OBJECTIVES: To determine the schoolperformance of children diagnosed with seizuredisorder ages 6-19 years old at DLSHSI-MC OPDfrom year 2006-2008.DESIGN: Descriptive Study.SETTING: This study was conducted in De La SalleUniversity Medical Center, Dasmariñas, Cavite,PhilippinesSUBJECT SELECTION: Pediatric patients ages6-19 years old diagnosed with primary seizuredisorder consulting the OPD clinic of DLSUMC whoare currently enrolled in a regular school.OUTCOME MEASURES: The data obtained wereanalyzed using CDC-WHO package EPI-INFO.Using the ordinal scale of level of measurement, datawas presented in frequency distributions, rates andratios, as indicators.RESULTS: There were a total of 66 subjects with anoverall male-to-female ratio of 1:1.1. Malescomprise 47% and females 53% of the subjects.Results showed that school performance in generalare average in most of the patients. The mean GPA ofthe subjects is 1.7 and the mean conduct grade is 1.6.62 out of the 66 subjects have no involvement inextracurricular activities.CONCLUSION: Patients with seizure disorder atDLSUMC-OPD have an average grade in academicsand conduct. Absenteeism is not a problem. Most ofthe subjects are not involved in any extracurricularactivity. Usual activities are concentrated inside thehome.[Keywords]School performance seizure disorderOBJECTIVE: Hemispherectomy is resective procedureto remove epileptogenic regions involving onehemisphere. It is generally performed in patients withsevere, refractory epilepsy with epileptogenic focusrestricted to on hemisphere. In this retrospective study,we try to evaluate the seizure control and outcomefollowing the operation.METHODS: We retrospectively reviewed the medicalrecords of pediatric patients receiving hemispherectomyin Taipei Veterans General Hospital between 2002 and2008. The characteristics including pre- andpost-operative seizure pattern, frequency, antiepilepticagents, EEG, and brain MRI result were described.RESULTS: There are four cases, two boys and twogirls, in this study and two are Rasmussen syndrome,one is Sturge-Weber syndrome and the other is diffusecortical dysplasia in one hemisphere. All hadhemiparesis contralateral to the brain lesion andrefractory seizure with median 2.5 types antiepilepticdrugs (range from 2 to 4) before surgery. One of themreceived hemispherectomy at 6 months of age, one at 2years old of age and the other two at 4 years of age.Regarding to the surgical outcome, three of four patientshave reached the ILAE pediatric scale class I, which isseizure free. However, the patient with diffuse corticaldysplasia has persistent refractory seizure after thesurgery. For this patient, the follow-upelectroencephalography revealed epileptogenic foci onthe unresected hemisphere which was not discoveredbefore the surgery.CONCLUSION: Hemispherectomy is worthwhile forselected cases with refractory seizures due to Rasmussensyndrome or Sturge-Weber syndrome. For patient withdiffuse cortical dysplasia in one hemisphere, theprognosis after the hemispherectomy is guarded.289

P2-071 Neuropsychiatry (Neurology) P2-072 Neuropsychiatry (Neurology)AN EXPERIENCE OF BRAIN DEATHDETERMINATION IN CHILDREN YOUNGERTHAN FIVE YEARS OLD IN TAIWANWen-Cheng Chang, Jao-Shwann LiangDepartment of Pediatrics, Far Eastern Memorial Hospital, TaiwanBACKGROUND: The experience of brain deathdetermination in children younger than 5 years old inTaiwan is limited. We present 3 cases with severebrainstem dysfunction who received protocol fordetermination of brain death and ancillary tests andreview the literature.METHOD: We collected 3 cases with severebrainstem dysfunction of different causes between2006 and 2007. Case 1 was a 3-year-9-month old boywho suffered from viral sepsis with the initialpresentation of drowsy consciousness, shock andseizures. Loss of all brain stem reflexes developedwithin 2 days. Case 2 was a 2-year-5-month old girldiagnosed as herpetic encephalitis and hypoxicischemic encephalopathy. The initial presentationwas sudden loss of consciousness and cyanosis athome. Loss of all brain stem reflexes was noted onarrival at emergent department. Case 3 was a1-year-10-month old boy who suffered from hypoxicischemic encephalopathy after foreign bodyaspiration. Absent of brainstem reflex was noted 2days after the event. Magnetic resonance imaging(MRI) showed diffuse ischemic change and edema ofthe periventricular white matter, midbrain and pons.Before clinical tests for brainstem reflex, includingapnea test, we observed for 83 days in case 1, 13days in both case 2 and case 3. Silentelectroencephalography (EEG) records weredocumented in every case and positron emissiontomography (PET) showed no cerebral flow in case2.RESULT: Case 1 showed no cranial nerve reflex.However, gasping was noted at the end of apnea test.He expired 14 days after the apnea test when hisfamily withdrew supportive care from hospital. Case2 and 3 passed 2 sets of clinical tests of brainstemreflex and apnea test separated 24 hours apart. Case2 became a donor 4 days after the 2nd apnea test.Unexpectedly, case 3 developed irregular movementof tongue and face 30 days after 2nd apnea test. Heexpired 145 days due to septic shock.CONCLUSION: Absence of brainstem reflux andabsolute apnea in apnea test, coupled with silentEEG records do not guarantee of permanent loss ofall brainstem function. Determination of brain deathshould be cautious in young children.[Keywords]BRAIN DEATH, CHILDRENPROTEOMIC ANALYSIS OF ASTROCYTICSECRETION THAT REGULATESNEUROGENESIS USING QUANTITATIVEAMINE-SPECIFIC ISOBARIC TAGGINGHu Yan a,1, Wenhao Zhou a,1, Liming Wei b, Fan Zhong b,Yi Yang a,*a Children’s Hospital of Fudan University, 399 Wanyuan Road, Shanghai201102, China; b Institutes of Biomedical Sciences, Fudan University, 138Yixueyuan Roda, Shanghai 200032, ChinaAstrocytes are essential components of neurogenicniches that affect neurogenesis through membraneassociation and/or the release of soluble factors. Toidentify factors released from astrocytes that couldregulate neural stem cell differentiation andproliferation, we used mild oxygen–glucose deprivation(OGD) to inhibit the secretory capacity of astrocytes.Using the Transwell co-culture system, we found thatOGD-treated astrocytes could not promote neural stemcell differentiation and proliferation. Next, isobarictagging for the relative and absolute quantitation(iTRAQ) proteomics techniques was performed toidentify the proteins in the supernatants of astrocytes(with or without OGD). Through a multi-step analysisand gene ontology classification, 130 extracellularproteins were identified, most of which were involved inneuronal development, the inflammatory response,extracellular matrix composition and supportivefunctions. Of these proteins, 44 had never been reportedto be produced by astrocytes. Using ProteinPilotsoftware analysis, we found that 60 extracellular proteinswere significantly altered (27 upregulated and 33downregulated) in the supernatant of OGD-treatedastrocytes. Among these proteins,7 have been reported tobe able to regulate neurogenesis, while others may havethe potential to regulate neurogenesis. This studyprofiles the major proteins released by astrocytes, whichplay important roles in the modulation of neurogenesis.290

P2-073 Neuropsychiatry (Neurology) P2-074 NephrologyPOSSIBLE LINK BETWEEN HYPOXIAINDUCIBLE FACTOR-1α AND BREASTCANCER RESISTANT PROTEIN IN HUMANENDOTOXEMIA AGGRAVATES KIDNEY INJURYIN DEVELOPING RATS WITH BILE DUCTLIGATIONBREAST CANCER CELL LINESFu-Ming Wang, Hueng-Chuen Fan, Shin-Nan Cheng 2 ,Tai-Ping Fan 11 Department of Pediatric, Tri-Service General Hospital, Taipei, TaiwanJia-Fu Hung, Li-Tung Huang, Chih-Cheng Chen,Jiunn-Ming Sheen, You-Lin TainDepartment of Pediatrics, Chang Gung Memorial Hospital-Kaohsiung MedicalCenter, Chang Gung University, TaiwanHypoxia is known to confer resistance to some drugsleading to failure of chemotherapy, probably due to theup-regulation of drug resistant proteins, such as breastcancer resistance protein (BCRP), via hypoxia induciblefactor-1α (HIF-1α) (Krishnamurthy et al., 2004). HIF-1αhas been regarded as a master regulator that activatespathways regulating physiological responses to variouslevels of hypoxia. In cancer cells, HIF-1α supports theiradaptation to hypoxia, e.g. production of vascularendothelial growth factor (VEGF), thereby enhancingtumour growth and progression (Semenza, 2002). Here weexamine how hypoxia affects the responsiveness of cancercells to drug treatment. BCRP is overexpressed inmitoxantrone (MX)-resistant MCF-7/MX cells. ByRT-PCR, we found lower mRNA levels of HIF-1α andVEGF in these cells, but higher mRNA levels of negativeregulators of HIF-1α including phosphatase and tensinhomolog deleted on chromosome 10 (PTEN) and vonHippel-Lindau (VHL) when compared with MX-sensitiveMCF-7/S cells. Kinetic studies on the effect of hypoxia(1% O 2 ) on HIF-1α mRNA level in MCF-7/S cellsrevealed an increase in response to hypoxia and reached apeak at 3h, but subsequently declined at 18h. The BCRPmRNA level steadily increased within the first 3h, and thendeclined after 9h. In MCF-7/MX cells, the initial lowHIF-1α mRNA level peaked at 3h and then declined to thebasal level at 18h. The BCRP mRNA remained constantduring hypoxia, but dropped to an undetectable level by18h. MTT assay showed that 18h hypoxia renderedMCF-7/S cells resistant to the cytotoxic effects of MX, butdid not affect the chemosensitivity of MCF-7/MX cells tothe drug. The results of Western blot suggest a BCRP-likeprotein to be responsible for the increased chemoresistanceof MCF-7/S cells to MX under 18h hypoxia. Since theregulation of HIF-1α has been linked to the generation ofreactive oxygen species (ROS) (Chandel et al., 1998), theeffects of hypoxia on the production of ROS in both celllines were investigated. By FACScan, a biphasic effect ofhypoxia on ROS production was evident in MCF-7/S cells.In contrast, there was no obvious change of ROSproduction in MCF-7/MX cells, except at 18 h where itslevel doubled. The data presented here do not confirm therelationship between HIF-1α and BCRP as proposed byKrishnamurthy (2004). Significantly, these data provide aplatform for comparative studies on chemosensitive andchemoresistant cancer cells, and their modification byoxygen tension and chemotherapeutics. It is hoped thatfurther studies of this kind would lead to the developmentof novel drugs for circumventing clinical drug resistance.BACKGROUND: Bile-duct ligation (BDL) is a commonlyused model of extrahepatic cholestasis. We recently founddeveloping rat had BDL-induced kidney injury viainduction of asymmetric dimethylarginine (ADMA) andoxidative stress. In contrast to BDL, endotoxin (e.g.,lipopolysaccharide) induced intrahepatic cholestasis.Because patients with chronic liver disease develop kidneyinjury is usually precipitant with bacterial infection, weintended to elucidate whether LPS exacerbates kidneyinjury in developing rats with BDL and whether ADMA andoxidative stress are involved.METHODS: Four groups of young male Sprague-Dawlyrats (17±1 days old) were conducted: diet control sham(DC, N=6), rats received BDL (BDL, N=9), rats withintra-peritoneal LPS injection (1mg/kg) 3 hours beforesacrifice (LPS, N=8), and rats received BDL and LPS(BDL+LPS, N=14). All rats were sacrificed 2 weeks aftersurgery.RESULTS: The mortality rate for the DC group, BDLgroup, LPS group, and BDL+LPS group were 0% (0/6),33%(3/9), 25%(2/8), and 50%(7/14), respectively. All BDLrats had higher plasma creatinine, AST, ALT, direct andtotal bilirubin levels than those in DC and LPS group. LPSexacerbates kidney and liver injury as demonstrated byhigher Cr, AST, and ALT levels in BDL+LPS vs. BDLgroup (Creatinine 0.59±0.01 vs 0.48±0.03 mg/dL, P

P2-075 Nephrology P2-076 NephrologyCOMPARISON OF NPHS2 POLYMORPHISMSAND THEIR ASSOCIATIONS WITHEND-STAGE RENAL DISEASE IN CHINESEAND MALAY CHILDRENCLINICAL COURSE AND OUTCOME OFIDIOPATHIC NEPHROTIC SYNDROME INMALAYSIAN CHILDREN: A SINGLE CENTERSTUDYJL Ng 1 , ID Liu 2 , KH Ng 1 , CK Heng 1 , M Than 2 , EL Ng 1 ,SS Chong 1 , WS Yeo 2 , HK Yap 11 Department of Pediatrics, Yong Loo Lin School of Medicine, NationalUniversity of Singapore, Singapore; 2 Shaw-NKF Children’s Kidney Centre,University Children’s Medical Institute, National University Health System,SingaporeINTRODUCTION: NPHS2 mutations have been reportedin familial and sporadic nephrotic syndrome, but mostreports have originated from Caucasian populations.Singapore is a country with a multi-ethnic population, anda 16-year retrospective review of renal biopsy data at ourcentre showed a striking increased prevalence of focal andsegmental glomerulosclerosis (FSGS) among Malaypatients compared to Chinese patients, and a greatertendency toward end-stage renal disease (ESRD) in Malaypatients. Hence, this study aimed to investigate theassociation of NPHS2 polymorphisms with nephroticsyndrome and end-stage renal disease in a bi-ethnic studypopulation of Chinese and Malay children in Singapore.METHOD: Direct sequencing of the 5’ promoter and all 8exons of NPHS2 was performed on genomic DNA from 95Chinese and 22 Malay patients with primary sporadicnephrotic syndrome (age of diagnosis ranging from 1-20years) and 75 Chinese and 49 Malay normal controls.RESULTS: There was a greater prevalence of FSGS andend-stage renal disease in Malay patients vs. Chinesepatients (FSGS: 45.5% vs. 16.8%, p=0.008; ESRD: 31.8%vs. 11.5%, p=0.04, respectively). Nine NPHS2 genevariants were identified – there were four single nucleotidepolymorphisms (SNPs) located in the 5’ promoter region(-670C/T, -213G/A, -116C/T and -51G/T); four exonicSNPs (288C/T, 685C/A, 954T/C and 1038A/G); also apreviously reported 871C/T missense mutation (R291W)was found in two Chinese patients who progressed toend-stage renal disease. The SNP genotype frequencieswere consistent with Hardy-Weinberg expectations. Therewas no significant difference in SNP allele frequencybetween patients and controls, nor between Chinese andMalays. In Chinese patients, the missense mutation 871C/Twas found to be significantly associated with progressionto end-stage renal disease as detailed above (p=0.02),while in Malay patients, homozygosity for the C allele atpromoter region position -670 was significantly associatedwith progression to end-stage renal disease (OR: 16.7,95%CI 1.36-204.0) (p=0.03).CONCLUSION: FSGS has a higher prevalence in Malaypatients in Singapore, as compared to Chinese patients, andan increased proportion of Malay patients progress toend-stage renal disease. A NPHS2 promoter SNP -670CCmay be associated with progression to end-stage renaldisease in Malay patients. Functional studies are neededto validate this hypothesis and elucidate possibledisease-causing mechanisms.Ong Sik Yong, Tan Pek Yong, Lynster Liaw Chiew TungDepartment of Paediatrics, Penang Hospital, Jalan Residensi, 10990 Penang,MalaysiaBACKGROUND: Most children with idiopathic nephroticsyndrome (INS) respond to corticosteroids but 20% areresistant to therapy. Of those who are steroid-sensitive,cyclophosphamide or ciclosporin is sometimes required. Up to70% of patients with focal segmental glomerulosclerosis(FSGS) on renal biopsy have primary steroid resistance. Theoutcome for steroid resistant patients is poor, with a 50% riskof progression to end stage renal disease (ESRD). Theobjectives of this study were to investigate the response andoutcome of children with INS to immunosuppressive therapies,describe the histology of children who underwent renal biopsy,determine the proportion of children who progressed to ESRDand the factors predicting this risk of progression.METHOD: Retrospective analysis of clinic records of all INSchildren aged 6 months to 18 years who were followed up inthe paediatric nephrology clinic of Penang Hospital fromJanuary 1987 to October 2009. The course of INS wasdetermined for those who were steroid-sensitive andsteroid-resistant. The renal histology of those who werebiopsied was described. The outcome and risk factors forprogression to ESRD of both groups were analyzed.RESULTS: The clinic records of 206 children with INScomprising 145 (70.4%) boys were analyzed. The median ageat diagnosis was 41.5 months, the median follow up period was62.5 months. 169 (82%) children were steroid-sensitive atinitial diagnosis. 65 (31.5%) patients received oralcyclophosphamide, 28 (13.6%) received ciclosporin and 17received both. The remission rate post-cyclophosphamide was29.2% and post-ciclosporin 42.3%. Of the 74 children whowere biopsied, 31 (41.9%) had primary steroid resistance.FSGS (78.4% of all those biopsied) was the main histologicaldiagnosis and accounted for 8 patients progressing to ESRD.The only significant risk factor for progression to ESRD wassteroid resistance. None of the 19 (9.2%) patients with chronickidney disease at last follow-up were steroid-sensitive. Elevenwere on permanent dialysis. There were a total of 7 deaths,accounting for an overall mortality of 3.4%; with 4 (36.4%)occurring at dialysis.CONCLUSION: The majority of children with INS weresteroid-sensitive but 18% had primary steroid resistance.Patients who received ciclosporin had better remission ratesthan those on oral cyclophosphamide. Renal survival was100% in patients who were steroid-sensitive. FSGS was themain histological diagnosis in steroid-resistant children andaugurs a poor prognosis.[Keywords]idiopathic nephrotic syndrome[Keywords]NPHS2, podocin, nephrotic syndrome292

P2-079 Nephrology P2-080 NephrologyPREDICTIVE FACTORS OF RENALSCARRING IN INFANTS AND CHILDRENWITH URINARY TRACT INFECTIONFlorencea Permatasari, MD 1De La Salle University Medical Center, PhilippinesANALYSIS OF UNDERLYING DISEASES OFCHRONIC RENAL FAILURE WITHUNAVAILABLE CAUSE BASED ON 254CASES—DATING BACK TO THE CHILDHOODJing Lu, Zheng Wang, Hua ChunDepartment of Pediatrics, West China 2nd University Hospital, SichuanUniversity,Chengdu,Sichuan,China,610041OBJECTIVES: To evaluate if there aredemographic, clinical, laboratory and other factorsthat can predict renal scarring in infants and childrenwith urinary tract infection (UTI).METHODS: Medical records of children with UTIwere retrieved. Demographic data, clinicalmanifestations, history of UTI, urine culture, voidingcystourethrogram (VCUG) and DMSA renal scanresults were tabulated and analyzed.MAIN RESULTS: Majority of the subjects werechildren aged below 1 year old with male:femaleratio of 1:1.6. Most common clinical manifestationwas fever and E coli is the most common isolatedetiologic organism. Seventy children underwentVCUG, 64.3% of them had reflux with predominanceof males aged less than 1 year old. Out of 86children, 47 patients (54.7%) had positive DMSAresults. Laterality relationship between VCUG andDMSA renal scan findings were tested using Kappastatistics which showed moderate agreement and thisis statistically significant. Demographic and clinicalfactors were not statistically significant fordevelopment of renal scar except for vesicoureteralreflux (VUR).CONCLUSION: There are no demographic andclinical factors that can predict renal scar formation.Presence of VUR as well as the degree of its severityis associated with renal scar formation.[Keywords] Renal Scar, DMSA Renal Scintigraphy, VesicoureteralReflux,UTI, Recurrent UTIOBJECTIVE: Our aim is to reveal the underlyingdiseases of CRF patients with unavailable cause so thatwe may provide some evidence for the prevention andintervention of CRF.METHODS: 1.700 cases in hospital were investigatedwith questionnaire: 1Hemodialysis center of WestChina Hospital:493. 2Sichuan People's Hospital:175;3Great Wall of Kidney Disease Hospital:32. 2.Patient’sbasic data, past history, family disease history, habits,health condition in the childhood and so on were allincluded in this questionaire.3.All data was statisticallyanalyzed by χ 2 test and logistic regression.RESULTS: There were 254 cases with unavailablecause in the total 700 CRF (36.3%). Cases whose agewas between 18 and 60 years old made the topproportion (198/254, 77.95%), and only 3 cases (3/254,1.6%) whose age were under 18 years. Male appears tobe a risk factor to develop CRF, as male patients(159/254, 62.6%) were much more than female (95/254,37.4%). The 254 patients were divided into fourcategories according to the job types:Manual-predominant, mental-predominant and other(including jobless and unwilling to disclose one’s job),there were 87(34.25%), 59(23.23%), 25(9.84%)respectively. Frequent respiratory tract infection occursin 22 cases during their childhood (22/254,8.66%), and 3patient had the abnormality of urine routine test before18 years, of course not including those who didn’t havetheir urine tested in their childhood. Besides, CRFpatient seemed to be more vulnerable to allergies fromtheir childhood (38/254, 14.96%). Smoking is obvious inCRF patients (43/254, 16.93%) before their diagnosis.The 254 cases has 26 drinking patients (10.24%).Logistic regression revealed that: age, drinking, toxicexposure, vulnerable to allergies, family history withhigher rates of kidney and other diseases were proved tobe the independent risk factors of chronic renal failure.CONCLUSION: Age, male, toxic exposure, smoking,drinking, abnormal urine routine test during childhood,vulnerable to allergies and family history with higherrates of kidney and other diseases may be the possibleunderlying diseases of chronic renal failure withunavailable cause.[Keywords] chronic renal failure,unavailable cause294

P2-081 Nephrology P2-082 NephrologyOUTCOME AND RISK FACTORS FORMORTALITY IN PEDIATRIC PERITONEALDIALYSISESTIMATED GLOMERULAR FILTRATIONRATE USING UEMURA FORMULA IS VERIFIEDBY INULIN CLEARANCELing Yu Yang 1 , Jei Wen Chang 2 , Hsin Ln Tsai 3 ,Hsin-Hui Wang 4Pediatric Department, Veterans General Hospital 1 , Department ofPediatrics, Taipei Veterans General Hospital, Taiwan 2 , Division of PediatricSurgery, Department of Surgery, Taipei Veterans General Hospital 3 ,Department of Pediatrics, Section of Nephrology; Department of Pediatrics,Faculty of Medicine, Taipei Veterans General Hospital; NationalYang-Ming University, Taiwan 4BACKGROUND: The mortality rate among childrenrequiring renal replacement therapy is marked higher thanthose among children without end stage renal disease(ESRD). Some factors such as hypoalbuminemia, highperitoneal transport rate, age, malnutrition, cardiovasculardisease and recurrent peritonitis appear to be associatedwith lower survival in adult peritoneal dialysis patients.Data regarding risk factors of mortality in children withcontinuous ambulatory peritoneal dialysis (CAPD) islimited. The aims of the study were to analyze the clinicalcharacteristics of patients and investigate if routinely usedlaboratory and clinical variables are independent riskfactors for mortality in children on CAPD.METHODS: We performed a retrospective chart analysisof pediatric ESRD patients on CAPD between January1997 and September 2008. 29 patients undergoing CAPDfor more than 3 months were enrolled. An analysis wasperformed on clinical and biochemical variables forsurvivors and non-survivors to identify potential riskfactors for mortality.RESULTS: The mean age was 12.18±4.57 years. Duringthe follow- up period, eight patients were transferred tohemodialysis and 13 patients received deceased donorrenal transplantation. At the end of the study, five patientshad died. The actuarial survival rate at 2 and 5 years was96.55% and 91.19%, respectively. The major complicationduring therapy was peritonitis (one episode 57.79 patientmonths). In the univariate analysis, younger age atinitiation of dialysis, the presence of comorbid disease,higher peritoneal transport rate, increased protein lossesthrough peritoneal dialysis, high total daily protein loss,hypoalbuminemia, and hypophosphatemia, were variablesassociated with mortality in pediatric CAPD patients.However, in the multivariate analysis, only low serumalbumin (b=-2.089, p=0.006, Hazard ratio=8.06; 95% CI=0.028, 0.546) was independently associated withmortality.CONCLUSION: Mortality was low in our pediatricpatients receiving CAPD. Hypoalbuminemia showed asignificant association with death in CAPD patients.[Keywords] Hypoalbuminemia, Mortality, Outcome, Peritonealdialysis, Risk factorTakuhito Nagai 1 , Osamu Uemura 1 , Satoshi Yamakawa 1 ,Yoshiko Hibi 1Department of Nephrology Aichi Children's Health and Medical Center, Japan 1OBJECTIVES: Glomerular filtration rate (GFR) is thekey factor used in diagnosing chronic kidney disease(CKD). The golden standard for the measurement ofGFR is considered to be inulin clearance, but physiciansusually substitute creatinine clearance for GFR and usethis value as the estimated GFR (e-GFR). Severalformulas have been proposed to calculate e-GFR usingbody height, serum creatinine level, or serum cystatin Clevel. Recently, Uemura reported a simple formula thatuses body height to calculate e-GFR. In this study, wesought to verify this formula using the inulin clearancemethod.METHODS: In this study, 38 children with stages 1–3CKD were enrolled. The patients included 28 boys (age,1.05–12.55 years; median, 7.30 years) and 10 girls (age,3.25–12.38 years; median, 8.80 years). The Uemuraformula (e-GFR UMR) represented as “body height (m)× 0.3/s-Cre (mg/dl) × 100” and inulin clearanceperformed at the same time is statistically analyzed.MAIN RESULTS: The median 2-hours creatinineclearance rate in the study population was 104.73ml/min/1.73m2 (range, 28.30–353.95). e-GFR UMR andinulin clearance were analyzed using Spearman’scorrelation test. For the study population as a whole, theP value was 1.64 × 10-08 and R2 was 0.55. For thefemale subjects, the P value was 0.0029 and R2 was0.76, and for the male subjects, the values were 1.94 ×10-06 and 0.50, respectively. All the patients showed astrong correlation between inulin clearance and theUemura formula.CONCLUSION: The e-GFR UMR analysis was verysimple, easy to calculate, and showed a good correlationto inulin clearance in children of ages 1–12. We proposethat Uemura formula is clinically valuable and reliablefor the estimation of GFR.[Keywords] GFR, Inulin clearance, Child.295

P2-083 Nephrology P2-084 NephrologyTRANSPLANTS OF METANEPHRONMESENCHYMAL CELL CONTRIBUTE TOFUNCTIONAL REPAIR INTHE TYPE 2 ANGIOTENSIN II RECEPTOR GENEPOLYMORPHISMS IN PRIMARYVESICOURETERAL REFLUX IN CHILDRENADRIAMYCIN-INDUCEDGLOMERULOPATHY IN RATJiao Yu-qing, Yi Zhu-wen, He Xiao-jie, Liu Xi-hong, HeQing-nan, Dang Xi-qiang, Wu Xiao-chuan, HuangDan-lin, Mo Shuang-hongDivision of Pediatric Nephrology, Department of Pediatrics, The SecondXiangya Hospital of Central South University, Changsha, Hunan 410011,P.R.ChinaYee-Hsuan Chiou 1 , Tzu-Hui Wang 1,2 , Lin-Yu Wang 2 ,Yuan-Yow Chiou 3 , and Shiao-Ping Huang 21 Department of Pediatrics, Kaohsiung Veterans General Hospital; 2 Departmentof Medical Technology, Fooyin University; 3 Department of Pediatrics, NationalCheng Kung University Hospital, Kaohsiung, TaiwanOBJECTIVE: To detect the functional repair oftransplants of metanephron mesenchymal cell (MMC) inadriamycin (ADR)-induced glomerulopathy in rat.METHODS: 80 Sprague-Dawley female rats weighting200 - 220 g were randomly divided into three groups: ADRglomerulopathy (ADR group, n=40): Rats were subjectedto ADR via the tail vein over a period of 3 weeks. A singledose of 0.25 mg/100 g body weight was used. ADRglomerulopathy followed by MMCs transplantation(ADR-MMCs group, n=40): 8 weeks after the second ADRadministration, animals were transplanted with MMCs.Approximately, 5-7×10 6 MMCs were used per rat. Control(n=10). All rats were scarified 16 weeks after the secondADR injection. Parameters including 24h urinary proteinexcretion (UP), serum total protein(TP), album(ALB),blood urea nitrogen(BUN), creatinine(CR), totalcholesterol(TCH), renal histology, and collagen IVexpression in renal were detected in this study. Moreover,matrix metalloproteinases 2 (MMP-2) and matrixmetalloproteinases 9 (MMP-9) expression in the renal werealso detected with immunohistochemistry, and quantityanalysis of protein and gene was further demonstrated withWestern blot and RT-PCR analysis, respectively.RESULTS: Administering two injections of ADR to ratsresulted in chronic ADR nephrosis, which displayed severeproteinuria, tubulointerstitial injury andglomerulosclerosis. Compared with ADR group, increasedsurvival rate was detected in MMCS-ADR group. Also,decreased collagen IV and MMP-2 expression, andincreased MMP-9 expression were detected in renal inMMCS-ADR group. Collagen IV expression was positivecorrelation with MMP-2, and negative correlation withMMP-9. There were no significant difference betweenADR and MMCS-ADR group in levels of UP, TP, ALB,TCH, BUN, Scr, tubulointerstitial injury score andglomerulosclerosis degree.CONCLUSION: Transplants of MMC contribute todecrease the fibrosis in adriamycin-inducedglomerulopathy in rat, and the signaling pathways ofMMPs appear to be involved in these processes.[Keywords] Chronic kidney disease; Cell therapy; Adriamycin;Metanephron mesenchyma; matrix metalloproteinases 2; matrixmetalloproteinases 9OBJECTIVES: Primary vesicoureteral reflux (VUR) is acommon pediatric disease that may lead to refluxnephropathy and end-stage renal disease. Currentknowledge indicates that the evolution of VUR is not equalin all patients. It suggested the influence of various factorsincluding genetics. The aim of this study is to investigatethe association of the AT2R gene polymorphisms with thedevelopment and severity of primary VUR in children.METHOD: We studied the AT2R gene polymorphismsT-151C, A190G, C2310T, A2827G, C3123A, and C3203Gfor association with development of primary VUR anddisease severity in 111 VUR children and 60 healthycontrols. Sixty of the 111 VUR patients had low-grade VUR(grade I-III) and 51 had high-grade VUR (grade IV and V).To analyze the polymorphisms, the SNP genotyping assaywas performed. The genotypic frequency and allelefrequency were analyzed to detect the correlation betweenthe patients with mild, severe VUR, and healthy control.RESULT: First, presence of VUR in females weredemonstrated to be associated with gene polymorphisms inT-151C (p

P2-085 Nephrology P2-086 NephrologyGENETIC AND HISTOLOGIC ANALYSIS OF AKINDRED WITH AUTOSOMAL RECESSIVEALPORT SYNDROMETHE CELLULAR MECHANISM OFCARDIOTOXICITY INDUCED BY PERITONEALDIALYSIS RELATED PERITONITISKenichiro Miura, Takashi Sekine, Kazuhiro Takahashi,Atsuhiro Yanagisawa, Yoshiyuki Namai, Kandai Nozu,Masafumi Oka, Kazumoto Iijima, Ichiro Naito,TAKASHI IGARASHIDepartment of Pediatrics, Tokyo University Graduate School of Medicine,JapanBACKGROUND: Autosomal recessive Alport syndrome(ARAS) is caused by mutations of either genes encodingthe alpha 3 or the alpha 4 chains of type IV collagen. It ischaracterized by the loss of alpha 3, alpha 4, and alpha 5chains in the glomerular basement membrane (GBM) butthe remaining of alpha 5, and alpha 6 chains in theBowman’s capsule. We describe here a girl with ARAS inwhich a renal specimen was analyzed using antibodiesagainst alpha 1 through alpha 6 chains of type IV collagen.Genetic analyses of the family members revealedcompound heterozygous mutations in COL4A3 genes ofthe patient and her brother.PATIENT: Hematuria was noted in a 3-year-old girl by thenational urinary screening test. Proteinuria also developedand the urinary protein-creatinine ratio was 1.5-4.0 g/gCrat the age of 6. Ophthalmologic and otolaryngologicexamination revealed no abnormalities. Her father and herpaternal grandmother, but not her mother, had hematuria.Her younger brother also had hematuria and proteinuria.The light microscopic examination of this patient’s renalspecimen at the age of 6 revealed minor glomerularabnormalities. Immunofluorescence studies forimmunoglobulins and complements were all negative.Electron microscopy showed that there was thinning, butnot splitting and lamellation, of the GBM.Immunofluorescence studies for type IV collagen revealedattenuated staining of the GBM for alpha 4 and alpha 5chains, and preserved staining of the Bowmann’s capsulefor alpha 5 and alpha 6 chains, which was compatible witha diagnosis of ARAS. Immunoreactivity of the GBM foralpha 3 chain was also attenuated, but partially preserved,which was not typical of ARAS. Genetic analyses of thepatient, her parents, and her younger brother wereperformed. The patient and her brother had twoheterozygous mutations in the COL4A3 gene: c.1354G>A(G452R) in exon 22 and c.3822_3823insG in exon 43. Theformer mutation was identified in the father’s gene and thelatter in the mother’s gene.DISCUSSION: Although compound heterozygousmutations in the COL4A3 gene were identified in thepresent patient, immunofluorescence studies showed thatthere was partially preserved staining of the GBM for typeIV collagen alpha 3 chain. Incomplete formation of type IVcollagen alpha 3 chains due to missense mutation in oneallele might be a possible explanation for this observation.[Keywords] autosomal recessive Alport syndrome, type IVcollagen, immunofluorescenceHsin-Hui Wang, 1,2 Ping-Chun Li, 3 Ya-Ling Chiou,4 Tzong-Yann Lee, 5,6 and Ching-Yuang Lin, 4,7,81 Department of Pediatrics, Section of Nephrology, Taipei Veterans GeneralHospital; 2 Department of Pediatrics, Faculty of Medicine, National Yang-MingUniversity, Taipei; 3 Department of Surgery, Division of CardiovascularSurgery,China Medical University and Hospital; 4 Institute of Immunology andMicrobiology, National Yang-Ming University, Taipei; 5 Department of InternalMedicine, Section of Nephrology, En Chu Kong hospital; 6 Department ofIntegrated Diagnostics & Therapeutics, National Taiwan University Hospital,7 College of Medicine, China Medical University; 8 Division of PediatricNephrology, China Medical University Hospital, TaiwanOBJECTIVE: Peritoneal dialysis (PD) is the predominant dialyticmodality for the pediatric patients with end stage renal disease.Peritonitis is the most serious complication and the leading cause ofmortality in patients undergoing PD. In most patients withperitonitis-related mortality, the immediate cause of death was acardiovascular event. The purpose of this study was to examine thecellular mechanism for the clinical observation that PD-relatedperitonitis is accompanied by a high incidence of cardiac mortality.METHODS: The turbid PD effluent (PDE) prior to antibiotictreatment were collected in 8 culture positive peritonitis episodes fromPD patients. The microorganisms were gram-positive bacteria in 5episodes and gram-negative bacteria in 3 episodes. Cultured humancardiomyocytes were treated with PDE during peritonitis, and theeffects of PDE on cultured cardiomyocytes in terms of viability,apoptosis and the expression of several apoptosis-related genes wereassessed. Cell viability was evaluated by MTT assay. Apoptosis wasevaluated by flow cytometry, TUNEL staining with confocalmicroscopy, and Comet assay. The expression of genes was determinedby quantitative real-time RT-PCR.MAIN RESULTS: PDE treatment induced human cardiomyocytedeath in a dose- and time-dependent manner. When cardiomyocyteswere pre-exposed to 12.5, 18, or 25 mg/ml PDE for 24 h, cellviabilities were 70.6±5.7%, 58.7±9.7%, and 41.6±7.8%, respectively(P

P2-087 Nephrology P2-088 NephrologyPROTECTIVE EFFECTS OF ADIPONECTINAGAINST RENAL ISCHEMIA-REPERFUSIONINJURY VIA PROSTACYCLIN -PPARΑ- HEMEOXYGENASE-1 SIGNALING PATHWAYVESICOURETERAL REFLUX (VUR) TREATEDWITH DEXTRANOMER/HYALURONIC ACID(DEFLUX) IN CHILDREN: EXPERIENCE IN ASOUTHERN TAIWAN MEDICAL CENTERChing-Feng Cheng 1, 2 Wei-Shiung Lian 2 , Heng Lin 31 Department of Pediatrics and Medical Research, Tzu Chi General Hospitaland Tzu Chi University, Hualien, Taiwan; 2 Institute of Biomedical Sciences,Academia Sinica, Taipei, Taiwan; 3 Graduate Institute of Pharmacology &Toxicology, Tzu Chi University, Hualien, TaiwanAdiponectin (APN), a circulating adipose-derivedhormone that regulates inflammation and energymetabolism, has beneficial effects on the cardio- andcerebro-vascular disorders. Serum APN levels arelower in patients with coronary artery disease andtype II diabetes, while hyper-adiponectinemia isfound in patients with chronic kidney disease.However, the precise role and molecular mechanismof APN in acute reno-vascular disease is not clear.Results of the present study show that the serumconcentration of APN decreased after ischemia/reperfusion (I/R) injury in mice. In addition,I/R-induced renal dysfunction (elevated serumcreatinine and urea levels), inflammation (number ofinfiltrating neutrophils, myeloperoxidase activity,induction of IL-6 and P-selectin) and apoptoticresponses (apoptotic cell number and caspase-3activation) were attenuated in APN-treated comparedto control mice. Molecular and biochemical analysisrevealed that APN up-regulates heme oxygenase-1(HO-1) via peroxisome-proliferatoractivated-receptor-α(PPARα) dependent pathwaywhich is mediated through the enhancement ofCOX-2 and 6-keto PGF1α expression. Chromatinimmune-precipitation assay demonstrated that APNincreases the binding activity of PPARα to the PPREregion of HO-1 promoter. Furthermore, APN inducedHO-1 expression only in wild type but not in PPARαgene deleted mice. This provides in vivo evidencethat APN mediated HO-1 expression depends onPPAR signaling pathway. In conclusion, our resultsprovide a novel APN mediated prostacyclin-PPARα-HO-1 signaling pathway that mediates its protectiveeffects on renal I/R injury.[Keywords] adiponectin, renal ischemic repurfusion injury, HO-1Chih-Chong Lin 1 , Sin-Yi Lee 2 , Jia-Fu Hong 1 ,You-Lin Tain 11 Division of Pediatric Nephrology and 2 Pediatric Surgery, Chang GungMemorial Hospital-Kaohsiung Medical Center, Chang Gung University, TaiwanBACKGROUND: Vesicoureteral reflux (VUR) is acommon genitourinary anomaly in children. Managementof VUR is crucial because reflux nephropathy accounts for~20 % of pediatric chronic kidney disease. Endoscopicinjection of dextranomer/hyaluronic acid (Deflux) at theureterovesical junction is an established alternative tolong-term antibiotic prophylaxis and ureteralreimplantation. We intended to survey the feasibility ofDeflux injection as a suitable option for VUR in ourhospital.MATERIALS AND METHODS: We retrospectivelyreviewed the records of children with documented primaryVUR between 2005 and 2009. Deflux injection wasperformed if VUR graded 2 or above and grade 1 VUR wasexcluded. Injections of Deflux on the right and left sideswere regarded as independent events. Success of Defluxinjection was defined by complete resolution of VUR onpost-operative voiding cystourethrogram (VCUG). Seconddose of Deflux was suggested if follow-up VCUG graded ≥2 three months later. The first 33 Deflux injections on 19ureters were selected as the learning curve (L) group andthe injections thereafter were classified as mature (M)group. The success rate of the L and M groups werecompared. Success rate of high grade (≥ 4) and low grade(2 or 3) groups were also analyzed.RESULTS: A total of 87 children with documented VURreceived Deflux injection and underwent post-operativeVCUG. The median age at entry was 29 months (7months-15 years); 46% of the patients were boys and 31%were high grade. A total of 168 Deflux injections wereperformed on 131 ureters. The success rate of the procedurewas 26% (5/19) for the first injection and 73% (14/19) forthe second injection in the L group. In the M group, thesuccess rate was increased to 68% (76/112) for the firstinjection and 79% (88/112) for the second injection.Especially, the success rates in the high grade group were ashigh as 74% (22/31) and 80% (25/31) following the firstand second injection, respectively.CONCLUSIONS: Our observations demonstrate thatDeflux injection is a suitable treatment option for VUR.The success rate can be increased with a second dose ofDeflux injection. Once the operator can overcome thelearning curve, Deflux injection should be considered as analternative to ureteral reimplantation for VUR.[Keywords] VUR, Deflux, success rate298

P2-089 Nephrology P2-090 NephrologySHORT TIME FOR FULL DOSE OF STEROIDPLUS HIGH-DOSE ALTERNATE DAYPREDNISONE IN TREATMENT OFCHILDHOOD NEPHROTIC SYNDROMETHE UTILIZATION OF THE THREE-MEDIADIPSLIDE CULTURE TEST TO DIAGNOSEURINARY TRACT INFECTION IN PEDIATRICPATIENTS IN CARDINAL SANTOS MEDICALCENTERHui ZhangPediatrics, West China Second University Hospital, ChinaOBJECTIVE: There are conflicting evidences regardinglongtime full dose of steroid in routine therapy ofchildhood nephrotic syndrome, which could be associatedwith the development of steroid resistance. Thereby weproposed a new steroid regimen in treatment of childhoodnephrotic syndrome.METHODS: We administered a questionnaire to 323children who have been diagnosed as nephrotic syndromeand received regular steroid therapy in West China SecondUniversity Hospital. 236 cases were followed up after 6months since the first questionnaire survey. All the patientswere clasified to two groups according to clinicalmanagement. Group A received new steroid regimen(dexamethasone pulse followed by two-week oral full doseof prednisone and high-dose alternate day prednisone);Group B received the routine moderate-long coursetherapy of prednisone recommended widely in China. Thetherapeutic effects and adverse effects of two groups wereobserved and compared.RESULTS: 1. It was much more rapidly for urinaryprotein to disappear in group A than that in group B (P

P2-091 Nephrology P2-092 GastroenterologyNPHS2 VARIATION IN CHINESE CHILDRENWITH LATE STEROID-RESISTANTNEPHROTIC SYNDROMEMINIMAL INVASIVE SURGERY IN INFANTSWITH UPPER GASTROINTESTINALOBSTRUCTIONLi YU, Zhi-hong HAOPediatrics, Guangzhou First Municipal People’s Hospital, ChinaYu-Ting Lin 1 , Pi-Feng Chang 1 , Yun Chen 2 , Yu-Cheng Lin 1 ,Shu-Jen Yeh 1Department of Pediatrics1, and Surgery2 ,Far Eastern Memorial Hospital,Taipei, TaiwanOBJECTIVE: To investigate the possible role ofNPHS2 combined with CD2AP gene variation insteroid-resistent nephrotic syndrome (SRNS)children in South China.METHOD: Genomic DNA was isolated fromperipheral blood leucocytes.8 exons of NPHS2 and18 exons of CD2AP were amplified by PCR.Mutational analysis was performed in 26 sporadicSRNS children in South China and 20 controls bysequencing directly.RESULTS: The variation analysis revealed 3polymorphisms (288C>T heterozygous in exon 2,954T>C heterozygous and homozygous, 1038A>Gheterozygous in exon 8) in 14 out of 26 patientsstudied, but there was no significant difference in thegenotypic and allelic frequencies of thesepolymorphisms between patients and controls. OneCD2AP heterozygous mutation was detected inintron in 2 SRNS children, but none in any exon. Thesame mutation was not found in controls.CONCLUSION: The hypothesis that SRNS may beassociated with NPHS2 gene variations was notconfirmed in this studied; the correlation betweenCD2AP mutation in intron and SRNS need furthercomfirmed.[Keywords] nephrotic syndrome; steroid resistance; NPHS2 gene;CD2AP geneOBJECTIVES: The aim of this study was to evaluatethe safety and effectiveness of laparoscopic and opensurgery in infants with upper gastrointestinal obstruction.METHODS: A retrospective review of infants less than100 days old who required surgical intervention forupper gastrointestinal obstruction at Far EasternMemorial Hospital was conducted. Age, body weight,operative procedure, operative time, operative bloodloss, complication and postoperative length of stay datawere collected from the medical records. Statisticalanalysis of these data with the χ2 (with or without Yates’correction) and Student t tests were performed. P value

P2-093 Gastroenterology P2-094 GastroenterologyARE CONTAMINATED EGGS THE MAINSOURCE OF FOODBORNE SALMONELLOSISIN INFANTS?ASSOCIATION OF INTERLEUKIN-18 GENEPOLYMORPHISMS WITH SUSCEPTIBILITY TOBILIARY ATRESIAKing-Jun Koh 1 , Lung-Huang Lin 1 , Min-Hsuan Hung 1 ,Yu-Ti Fang 2 , Qing-Dong Ling 2Department of Pediatrics, Cathay General Hospital, Taipei, Taiwan 1Cell Biology Lab, Cathay Medical Research Institute, Cathay GeneralHospital, Taipei, Taiwan 2OBJECTIVES: Salmonella species are recognized as majorzoonotic pathogens for animals and humans. In manycountries, Salmonella is the leading cause of foodborneoutbreaks and infections. The foodborne disease is animportant health and economic issue that burdens the worldover. Epidemiological investigations in Hungary, U.K, U.S,and Germany confirmed that the food most associated withincreased foodborne Salmonellosis was from the eggs. Themain cause of this epidemic is the infections due to S. entericasubsp. enterica serovar Enteritidis (S. Enteritidis), a serotypeprevalent in poultry, particularly in eggs. In Taiwan, peoplepurchase eggs from traditional retail market or supermarket.Here, we want to know the situation of Salmonellacontamination in the washed, commercially processed eggsand the eggs in the traditional retail market.METHODS: From Aug. 2008 to Sep. 2009, we collected eggsfrom chicken layer farms, traditional retail markets,supermarkets and home of the infants who were infected bySalmonella. All of the eggs were sent back to laboratory atroom temperature and exam immediately. The egg shells wereswabbed by sterile swab with normal saline. Then the swabswere streaked onto the plate of Xylose-lysine-desoxycholate(XLD) agar and incubator at 35℃. Suspect Salmonellacolonies were confirmed by serotyping at laboratory usingCenters for Disease Control methods.MAIN RESULTS: There were totally 284 eggs collected. 50eggs were processed eggs which were bought at supermarkets,4 eggs were collect from patients’ home, 160 eggs were fromtraditional retail market and 70 eggs were from the chickenlayer farms. There were some other colonies but noSalmonella species were isolated in this study.CONCLUSION: Contamination of eggs with Salmonella canoccur in several ways: egg shells may be contaminated byfaeces; cleaning procedures maybe improper; the interior ofthe egg may be contaminated by the shell being cracked.Contamination may also be internal, prior to the shellformation through trans-ovarian transmission. An earliersurvey in Taiwan showed no Salmonella detected in eggs andour study revealed the same results. Thus, local Salmonellaenterocolitis infection may not be due to polluted eggs.Contaminated equipment in food processing factory andinfected food handlers may be the main cause of foodproduct-related outbreaks. Although it is not a recentlyrecognized phenomenon and remains controversial, a foodhandler is capable of transmitting Salmonella if acutely ill oras an asymptomatic carrier. More study are needed to clarifywhether the source is from the human carrier or from otherfood contamination.Shun-Feng Lin 1 , Hung-Chang Lee 1, 2 , MD, Tzu-YangChang 3 , PhD, Chun-Yan Yeung 1,4 , MD, Wai-Tao Chan 1 ,MD, Chuen-Bin Jiang 1 , MD, Hui-Wen Chan 3 , MS,Wei-Fang Chen 3 , MS, Hsin-Fu Liu 3 , PhD, Marie Lin 3 , MD,Yann-Jinn Lee 1, 2, 3 , MD, MSDepartments of 1 Pediatrics and 3 Medical Research, Mackay Memorial Hospital,Taipei, Taiwan; 2 Department of Pediatrics, Taipei Medical University, Taipei,Taiwan; 4 Mackay Medicine, Nursing and Management College, Taipei, TaiwanOBJECTIVE: Biliary atresia (BA) is a destructiveinflammatory obliterative cholangiopathy of neonatesthat affects both intrahepatic and extrahepatic bile ducts.Although the etiology is unknown, immunologicallymediated injury of bile ducts triggered byas-yet-unidentified infectious agents is likely to play acritical role. Interleukin-18 (IL-18) is a proinflammatorycytokine that plays an important role in immune,infectious, and inflammatory diseases due to itsinduction of interferon-γ. In this study we investigatedwhether polymorphisms of the IL-18 gene wereassociated with susceptibility to BA.METHODS: Genomic DNA was extracted from wholeblood samples of 50 Taiwanese children with BA and375 ethnically-matched healthy controls. The IL-18 –607C/A, –137 G/C, and +105 A/C polymorphisms weregenotyped using the TaqMan assay.MAIN RESULTS: No statistically significant differencesin genotype, allele, carrier, and haplotype frequencies ofthese IL-18 gene variants were found between childrenwith BA and healthy controls.CONCLUSION: Our data suggest that the IL-18 genedoes not play a major role in BA predisposition in theTaiwanese children.[Keywords]Foodborne Salmonellosis301

P2-095 Gastroenterology P2-096 GastroenterologyA COMPARISON OF DISEASE CAUSED BYSHIGELLA AND COMPYLOBACTERSPECIES 24 MONTHS COMMUNITY BASEDINCREASED MUCOSAL NITRIC OXIDEPRODUCTION IN LACTOSE INTOLERANCE ININFANTSSURVEILLANCE IN 4 SLUMS OF KARACHI,PAKISTANSajid Bashir SoofiPaediatrics and Child Health, Aga Khan University, PakistanPrikhodchenko Nelly, Shumatova T.A., Grigoryan L.A.,Pavlova Y.E.Vladivostok state medical university, Russian FederationDespite the efforts from the international community thediarrheal diseases still poses a major threat to morbidityand mortality in less than five years of age. Bacterialdiarrhoea has also emerged as a public health problem dueto the drug resistant strains of different organisms in manyparts of the world. There is a paucity of population-baseddata about the incidence of shigellosis and Compylobacterinfections in Pakistan. In preparation of vaccine trialsenteric disease surveillance was conducted in 4 urbanslums of Karachi with a population of 59,584 over a 24months period. Cases were detected through passivedetection in study treatment centers. Stool specimens orrectal swabs were collected from all consenting patients.Between January 2002 and December 2003 10,540 entericinfection cases were detected through a passivesurveillance approach. The incidence rate of treateddiarrhea in children under 5 years was 488/1000/year. Inthe population 5 years and older the diarrhea rate was22/1000/year. 576 (7%) Campylobacter isolates weredetected. The most frequently isolated Campylobacterspecies was C.jejuni (29/1000/year in children under 5years). From 8032 stool specimens 394 (5%) Shigellaspecies were isolated. S.flexneri was the dominant species(10/1000/year in children under 5 years) followed byS.sonnei (3.9/1000/year), S.boydii (2.0/1000/year) andS.dysenteriae (1.3/1000/year). Shigellosis andCompylobacterosis infection rates peaked during thesecond year of life. The incidence rate of shigellosisincreased in old age but such a trend was not observed inCampylobacter infections. Of 394 shigellosis patients 123(31%) presented with dysentery in contrast to only 54 (9%)of 576 patients with Campylobacter infections (p

P2-097 Gastroenterology P2-098 GastroenterologyEFFICACY AND SAFETY OF A SYNBIOTICCAPSULE ON THE DURATION ANDFREQUENCY OF ACUTE NON-BLOODYEVALUATE THE TREATMENT EFFECT OFGASTROINTESTINAL MEDICATION INCONSTIPATION OF CHILDRENDIARRHEA IN 2 TO 24 MONTHS OLDFILIPINO INFANTS: A DOUBLE BLIND,PLACEBO CONTROLLED STUDYZendy M. Tolete M.D.; Felizardo N. Gatcheco M.D.,MScDepartment of Pediatrics, Jose R. Reyes Memorial Medical CenterRizal Avenue, Sta Cruz Manila, PhilippinesOBJECTIVE: To determine the efficacy and safetyof synbiotic versus placebo in the treatment of acutenon-bloody diarrhea in patients 2-24 months of age.DESIGN: Randomized, double blind, clinical trialSUBJECTS: Sixty infants aged 2-24mos old withacute non bloody diarrhea, with no to somedehydration, with no other concomitant illness andwithout antimicrobial therapy: 30 in the synbioticgroup and 30 in the control group.METHODS: The subjects were randomized toeither synbiotic or placebo, 1 capsule once a day for5 days. The frequency, consistency and duration ofdiarrhea were then recorded in each group. Intentionto treat, T-test, Mann Whitney U tests, Chi squaretests and Fisher Exact test were used in the analysisof the different variables of both group.RESULTS: The subjects in the synbiotic groupshowed resolution of symptoms as early as the 2 ndday of treatment compared to the placebo groupwhose recovery was noted on the 5 th day oftreatment. As for the degree of dehydration, therewas a significant proportion of the subjects treatedwith synbiotic who were able to regain theirhydration status on the 2 nd day of treatmentcompared to the placebo group. Likewise, meanduration of diarrhea for the synbiotic group was 2.43days and 5.16 days for the placebo group. Noadverse reactions were observed during the course ofthe trial.CONCLUSION: The use of Synbiotic hassignificantly reduced the duration and frequency ofdiarrhea, and has resulted to milder clinical course ofthe disease. It was also found to be safe[Keywords] efficacy, safety, non bloody diarrhea, duration andfrequencyYI-PEI TAI 1 , AN-CHYI CHEN 1 , SHU-FEN WU 1 ,CHUN-CHUN CHUANG 1 , TIEN-KAI TSAI 1,2 , MEE-MEELEONG 1,3 , CHENG-KUO TSAI 1,2 , WALTER CHEN 1 ,CHING-TIEN PENG 11 Children’s Medical Center, China Medical University Hospital, Taichung,Taiwan; 2 Fong Yuan Hospital, Taichung, Taiwan; 3 Pediatric Department, PingTung Christian Hospital, Pintung, TaiwanOBJECTIVE: Constipation, a delay or difficulty in defecation,present for two or more weeks and sufficient to cause significantdistress to the patient is a very common problem during childhood. Wehad reviewed 125 cases treated with gastrointestinal medications andcompared the drug effects of clinical symptoms.METHODS: Patients: Children (younger than 18 year-old) withfulfilled diagnosis of constipation (Rome III criteria). Groups andmedications (1) Mosapride group: Mosapride and Magnesium Oxide(MgO) (2) Metoclopramide group: Metoclopramide and MgO (3) MgOgroup: MgO alone. Exclusion: patients with previous abdominalsurgery, ileus on plain abdominal X-ray, or megacolon were excluded.Regimens: 1. Mosapride 0.12mg/kg/dose (max. 5mg per dose) tid. 2.MgO 12.5mg/Kg/dose (max. 500mg per dose) tid. 3. Metoclopramide0.1mg/kg/dose (max. 5mg per dose) tid Clinical data from 125 children(71 girls and 54 boys) with constipation were reviewed. Mosapridewith MgO had been used in 54(43.2%), Primperan with MgO had beenused in 38(30.4%) and MgO alone in other 33(26.4%). We had usestatistic software (SAS 9.1) to compare the onset age of clinicalsymptom, duration of symptoms, consistency of stool and frequency ofdefecation, abdominal pain, bloody stool of each group.MAIN RESULTS: Fifty-four patients (26 girls and 28 boys) weretreated with Mosapride plus MgO. The mean symptoms onset age was3.25 year-old. The mean duration of symptoms before treatment was15.19 months. The mean frequency of defecation before treatment wasonet ime per 3.52 days and increased to onetime per 1.76 days aftertreatment. Thirty-eight patients (26 girls and 12 boys) were treatedwith Metoclopramide plus Mgo. The mean symptoms onset age was3.88 year-old. The mean duration of symptoms before treatment was7.43 months. The mean frequency of defecation before treatment wasone time per 2.86 days and increased to one time per 2.00days aftertreatment. Thirty-three patients (19 girls and 14 boys) were treatedwith MgO alone. The mean symptoms onset age was 3.19 year-old.The mean duration of symptoms before treatment was 9.34 months.The mean frequency of defecation before treatment was one time per2.62 days and increased to one time per 1.71 days after treatment. Allgroups can change the consistency of stool and improvement ofabdominal pain related to constipation, but no significant difference onthree groups in our study. There was significant improvement offrequency of defecation in the Mosapride group. The improvement ofbloody appearance was best in the MgO group while worst in theMetoclopramide group. No side effect was noted on both groups, suchas extrapyramidal symproms or arrhythmia.CONCLUSION: There is no standard therapy for constipation.Bothprokinetic agents plus MgO and MgO alone group were effective totreat constipation in children, and the safeties were noted also.Combined using prokinetic with MgO to treat constipation may havesynergistic effect. Mosapride is 1st selective 5-HT 4 agonist and it canenhances gastrointestinal motility and emptying. We compared thedrug effect of different medication for constipation, and found bettereffect for improvement of frequency of defecation in Mosapride group.Because of the limited case numbers, long-term studies are needed toconfirm.[Keywords] constipation, medication303

P2-101 Gastroenterology P2-102 GastroenterologyTHE ROLE IN CELL DEATH INCHOLESTASIS MURINEMao-Meng Tiao, 1 Ying-Hsien Huang, 1 Kuo-Shu Tang,1 Chung-Chin Lu 21 Departments of Pediatrics, Chang Gung Memorial Hospital-KaohsiungMedical Center, Chang Gung University College of Medicine, Taiwan. 2Fooyin University Hospital, TaiwanOBJECTIVE: We find that the biogenesis ofmitochondria is response to the early stage ofcholestasis with internal pathway. The regulationpathogenesis of the liver cell apoptosis in this earlystage in cholestatic liver injury is not clear.METHODS: A rat model of cholestasis wasestablished by bile duct ligation (BDL), withsimultaneous creation of the sham group receivinglaparotomy without BDL. Liver LC3B1, LC3B2,Beclin-1, caspase 9 protein expression was analyzedby western blotting. The ATF6, IRE1, BIP RNAlevel was measured by real-time polymerase chainreaction.RESULTS: The liver LC3B-2 and LC3B2/LC3B1ratio at 6-72 hours with caspase 9 activity andBeclin-1 levels at 72 hours significant upregulated.The ATF6, IRE1, BIP significantly decreased at 24-72 hours.CONCLUSIONS: Our results indicate thatregulation of the liver biogenesis is within a fewhours after complete bile duct obstruction. Earlymurine cholestatic liver injury is via autophagypathway.[Keywords] jaundice, mitochondria, apoptosisCONSTIPATION AMONG FILIPINO CHILDREN 1MONTH – 4 YEARS OF AGE: ITS PREVALENCEAND RISK FACTORSAnthony Joseph Reyes TecsonPediatrics, Philippine Pediatric Society, PhilippineBACKGROUND: Constipation accounts for 3% of pediatricoutpatient visits and 10 to 25% of pediatric gastroenterologyvisits. A variety of factors have been proposed as precipitants.However, up until now, there are no local studies yet with regardthe prevalence and risk factors of constipation in pediatricpopulation.OBJECTIVE: To determine the prevalence and risk factors ofconstipation in infants and children 1 month to 4 years oldMETHODS: This was a Cross sectional study. All pediatricpatients aged 1 month to 4 years seen at a tertiary medical centeroutpatient department, regardless of complaint and diagnosiswere included. The information gathered were recorded in avalidated data collection form.RESULTS: A total of 276 one to four year old and 183 onemonth to 12 months old were included. The overall prevalencerate was 12.2%. The prevalence rate in children was 12.7%(35/276) while in infants it was 11.5% (21/183). There was nosignificant association between both sex and age withconstipation in infants and children. In children, family historyof constipation (OR 8.3, CI 3.42-20.17) and allergy (OR 4.74,CI 2.13-10.58) were significantly associated with constipationwhile in infants, it was family history of constipation (OR 6.4,CI 2.11-19.45) and gastrointestinal illness (OR 6.58, CI1.36-31.8). On multivariate analysis, only family history ofconstipation (p=0.001) and family history of gastrointestinalillness (p 0.049) proved to be a significant predictor ofconstipation in infants. Constipation was significantly notedamong children not eating fruits (OR 7.8, CI 3.17-19.2), noteating vegetables (OR 11.26, CI 4.76-26.87), not toilet trained(OR 5.02, CI 2.21-11.59) and those who do not use the toiletdaily to defecate (OR 4.3, CI 1.95-9.54). On multivariateanalysis, family history of constipation, family history of allergyand failure to use the toilet daily were significant factors amongconstipated children. There was no association with the use ofiron supplements (OR 0.65, CI 0.21-1.89), water intake (OR0.44, CI 0.12-5.5), diarrhea (OR 1.2, CI 0.12-5.5) and feverprior to constipation (OR 1.5, CI 0.16-8.02). Likewise there wasno association between age of passage of meconium as well thefrequency and interval of milk intake in infants withconstipation. Moreover, the use of iron supplements (OR 1.31,CI 0.27-6.33) was also not associated with constipation.CONCLUSION: The prevalence rate of constipation amongFilipino children 1 month to 4 years of age is 12.2%. The riskfactors associated with constipation in children were familyhistory of constipation, family history of allergy, no fruit intake,no vegetable intake, irregular toilet use and no toilet training.On the other hand the risk factors identified in infants werefamilial history of constipation and gastrointestinal illness.[Keywords] Constipation304

P2-103 Gastroenterology P2-104 GastroenterologyZINC AND PROBIOTICSUPPLEMENTATION FOR PEDIATRICINPATIENTS WITH DIARRHEA IN ANINDONESIA DISTRICT GENERALOUTCOME OF AN INTERDISCIPLINARYFEEDING REHABILITATION PROGRAM FORCHILDREN WITH NEURODEVELOPMENTALDISABILITIESHOSPITALSiti Chasanah 1 , Ferri Widodo 2 , Diana Lyrawati 1,2 ,Nurcahyo Budi Santoso 31 Laboratory of Pharmacy, Faculty of Medicine, Brawijaya University –Saiful Anwar Hospital, Malang, Indonesia 2 Study Program of Pharmacy, Faculty of Medicine, Brawijaya University,Malang, Indonesia; 3 Department of Pediatrics, Division of Infection,Faculty of Medicine, Brawijaya University – Saiful Anwar Hospital,Malang, IndonesiaBACKGROUND: Zinc and probiotic supplementation(Zn-probiotic) has been shown to accelerate recoveryfrom diarrhea in children in many developing countries,yet their efficacy in children in Indonesia is uncertain.Despite the recommendation of WHO/UNICEF andMinistry of Health Republic of Indonesia for use of zinc,it remains excluded from list of drugs covered by nationalhealth insurance (Askes/SKTM/Jamkesmas).OBJECTIVE: This study aimed at evaluatingsupplementation of zinc-probiotic, to explore contributingfactor to non-adherence, regimen of therapy andassociated clinical outcomes in the management ofdiarrhea in pediatric inpatients.METHOD: A prospective observational study wasconducted in High Nursing Dependency Unit, one ofpediatric wards at Saiful Anwar Hospital, Malang,Indonesia. Data were collected from medical record anddirect observation of all patients with diarrheahospitalized during February to December 2009.RESULTS: From 41 patients, 60% (28 cases) wasprescribed for Zn and probiotic supplementation. Of thesepatients, adherence was observed in 59% patients;whereas the rest did not adhere to therapy due to palatableor difficulties in consuming the Zn formulated as tabletand financial resources of the patients. Supplementationof Zn-probiotics associated with recovery in 60% of thepatients compared to 40% in those who did not receiveZn-probiotic. Other than Zn and probiotic, in somepatients, antibiotics were also used concurrently. Ouranalysis showed that the use of antibiotics did notsignificantly influence time needed for recovery ofdiarrhea; in both groups with or without antibioticsdiarrhea lessen within 1-5 days.CONCLUSION: Our observation indicated thatprescription for supplementation of Zn-probiotic forchildren with diarrhea has reached 60% of the patients,and associated with higher proportion of patientsrecovered from diarrhea. Further studies are warrantedto validated these results to support recommendation ofZn-probiotic being included in the national scheme ofhealth insurance and adjustment of Zn dosage form toimprove adherence.[Keywords]zinc,probiotic,diarrhea,pediatric,high nursedependencyRosanna Ming Sum Wong, Sophelia ChanDepartment of Paediatrics and Adolescent MedicineDuchess of Kent Children’s Hospital and Queen Mary HospitalThe University of Hong Kong, Hong KongWe report the outcomes of an interdisciplinary program inHong Kong which was established in 2008 for themanagement of feeding difficulties in children withneurodevelopmental disabilities. The program was run bya feeding team which included developmentalpaediatrician and neurologist, gastroenterologist,paediatric surgeon, speech therapist, occupationaltherapist, dietitian and nurse specialist. This was a singlecentre longitudinal prospective cohort study designed toinvestigate the outcomes of the feeding rehabilitationprogram. Forty-nine patients were assessed and enrolledfrom March 2008 to March 2009. Thirty-one percent ofthe children had cerebral palsy and 27% hadchromosomal or syndromal disorders. Other diagnosesincluded prematurity, global developmental delay, brainmalformation, neuromuscular disease, traumatic braininjury and brain tumor. Outcome measures includedgrowth as measured by z score for body weight, change inmode of feeding and episodes of aspiration pneumoniarequiring medical attention. The patients were assessed atbaseline, 3-6 months and 6-12 months after intervention.Types of interventions included change in mode offeeding, oromotor therapy, treatment of gastro-esophagealreflux and dietary modification. Six patients hadgastrostomy +/- fundoplication performed and 5 patientswere weaned from nasogastric or orogastric tube feedingto full oral feeding during the period. Statisticalsignificant improvement in Z score for body weight anddecreased episodes of aspiration pneumonia wereobserved.305

P2-105 Gastroenterology P2-106 GastroenterologyFILIPINO PHYSICIANS’ CURRENTPRACTICES IN THE MANAGEMENT OFACUTE DIARRHEA IN INFANT ANDCHILDRENNYLOFAR TINAZA MD, Felizardo N. GatchecoM.D., MScDepartment of Pediatrics, Jose R. Reyes Memorial Medical CenterRizal Avenue, Sta Cruz Manila, PhilippinesBACKGROUND: Acute diarrhea remains to be a majorcause of infant mortality around the world. Over 70,000Filipino children died in the last seven years making it thefourth leading cause of death in children. Morbidity is due tothe consequent dehydration. ORT has become the worldwidemainstay of national diarrheal control programs. Propernutrition is viewed as an important adjunct to therapy alongwith zinc supplementation. The use of probiotic andprebiotics as well as novel drugs like Racecadotril are fastbecoming adjuncts in the management of acute diarrhea inchildren. It is high time for us to make another study on thecurrent practices of Filipino physicians and how these newermodalities have affected their decision-making processes.OBJECTIVE: To describe the current practices ofphysicians in the management of acute diarrhea in infant andchildren (2 months-5 years old).DESIGN: Cross Sectional Study.SETTING: Tertiary Government Hospital.METHODS: Participants (immediate guardians of infantsand children with complaints of diarrhea who had previousconsults with a private physician, local health center or otherhospitals) were given a pre-validated questionnaire dealingwith the management they received during their previousconsults.RESULTS: A total of 400 respondents with children 2months to 5 years old with diarrhea lasting for 3.78 daysbefore consult were included in the study. The proportion ofinfants and children who were managed with ORS werecomparable. However, significantly more infants weremanaged with other fluids such as rice water (p=0.03) andsports drinks like Gatorade (p=0.01) than children. Zincsupplementation (p 0.77) and continued feeding (p 0.13)were comparably prescribed in infants and children. Amongthe most commonly used adjunct in the management of acutediarrhea, vitamin A was noted both for infants and children.A comparable proportion of infants and children were alsogiven probiotics and anti-diarrheal drugs. There was nosignificant difference in the proportion of infants andchildren who were prescribed with antibiotics. Stoolexamination was the most commonly requested laboratoryexam both for infants and children.CONCLUSION: The results showed that the use of ORS,zinc supplementation, and Vitamin A are still the mainstay inthe management of diarrhea in infants and children but asignificant proportion still was prescribed antibiotics. Mostrequested laboratory examination was stool examination.Probiotics and racecadotril are beginning to be recognized asadjuncts to ORT.[Keywords]management, acute diarrhea, infants and childrenA DOUBLE-BLIND RANDOMIZED CLINICALTRIAL ON THE EFFICACY OF SPIRULINA AS ANADJUNCT TO ORT IN THE TREATMENT OFDIARRHEA AMONG INFANTS IN A PRIVATEHOSPITALJocelyn Bongco,MD*, Yambao-Franco,MD*#; FelizardoGatcheco,MD#*United Doctors Medical Center, #Manila Central University, PhilippinesOBJECTIVE: To determine the efficacy of Spirulinapowder as an adjunct to ORS and zinc in the treatment ofacute non-bloody diarrhea among 6-36 months oldchildren.METHODS: Forty-one subjects were randomized afterORS and zinc were administered into Placebo group (n=20) and Spirulina group (n=21). The subjects were giveneither spirulina (60mg/sachet) or placebo, 1 sachet fourtimes a day for five days. Duration of diarrhea, frequency,consistency of stool, amount of stool, condition of thesubjects and adverse reactions were monitored.MAIN RESULTS: The demographic characteristics of thetwo groups were similar, (p>0.05). The spirulina grouphad a shorter mean duration of diarrhea by 1.14 days thanthe placebo group (p

P2-107 Gastroenterology P2-108 GastroenterologyENTEROBIUS VERMICULARIS INFECTIONOF THE APPENDIX AS A CAUSE OF ACUTESAPHO SYNDROME - DEVELOPED IN A CHILDWITH CROHN’S DISEASE ON INFLIXIMABAPPENDICITIS IN A JAPANESEADOLESCENTGORYU FUKUMA, KOUJI NISHIDADepartment of Gastroenterology, Takagi Hospital, JapanThe parasites infection as the cause of appendicitis isnot rare. Enterobius mainly lives in appendix as wellas cecum. The inflammation due to the habitat ofEnterobius in cecum and/or appendix cause appencitisoccasionally. Inflammation in the appendix may causeappendiceal colic even without eliciting an acuteinflammation. This colic due to a parasitic infestationis explained by the hypothesis of appendiceal lumenobstruction. Gastrointestinal infection of Enterobiusvermicularis occurs worldwide and is considered to beone of the most common parasites infection. Thesimple presence of E. vermicularis in the appendixusually produces symptoms which resemble acuteappendicitis although the mechanism for this does notinvolve mucosal invasion by the parasite.A 17 year-old male who took an antiepileptic drugagainst epilepsy presented initially with the pain of theright lower quadrant. He had low-degree fever 37.3degree Cercius but didn’t have diarrhea, hematemesisor itching over anus. At the first admission, he hadabdominal tenderness at the right lower quadrant. HisHgb was 11.9g/dL and serum Fe, 27ug/dL, presentingiron deficiency anemia. The count of white blood cellwas not elevated without eosinophilia and CRP wasslightly elevated 0.96mg/dL.He was diagnosed acute enterocolitis, seen to course.But his fever and abdominal symptoms continued for awhile more. Three months after first admission, hetook abdominal CT scan, which presented mesentericlymph nodes swallen mildly without abnormality ofintestines. was then he took appendectomy. Evenresecting appendix, he complaint intermittent feverand abdominal pain occasionally. A kind ofInflammatory bowel diseases were suspected, weunderwent colonoscopy for him six months afterappendectomy. A lot of worms approximately 1cmlong respectively were detected in colon. The wormswere examined and a diagnosis of Enterobiusvermicularis was made. The patient was treated withpyrantel pamoate twice with 1 week interval and thenhe was asymptomatic. The appendix was surgicallyremoved and Enterobius vermiclaris was notpathologically detected in the specimen. We shouldrule out enterobiasis in patients with symptoms likeappendicitis such as right lower quadrant.[Keywords] parasiteKatsuhiro Arai, Hirotaka Shimizu, Tadahiro Yanagi,Toshihiko Kakiuchi, Sachi KoinumaDivision of Gastroenterology, Department of Medical Specialty, National Centerfor Child Health and Development, Tokyo, JapanOBJECTIVES: The acronym SAPHO (synovitis, acne,pustulosis, hyperostosis, and osteitis) represents asyndrome of peculiar osteoarticular manifestations andvarious chronic dermatologic conditions. Recent reportshave discussed the usefulness of infliximab to control theSAPHO syndrome. We experienced a pediatric case ofSAPHO syndrome which developed in a child whoseCrohn's disease had been managed with infliximab.CASE REPROT: A 12 year-old girl was diagnosed tohave ileocolonic Crohn's disease when she was10year-old. Her disease had been controlled well byinfliximab for about one year. After the 9th dose ofinfliximab, psoriatic lesions of the scalp, trunks, andextremities with palmoplantar pustulosis appeared withleft mandiblar swelling and pain. With some ameliorationof skin lesion with corticosteroids ointment, the 10th doseof infliximab was administered followed by deteriorationof skin and mandiblar lesion. CT and MRI of mandiblerevealed osteomyelitis of left mandible which poorlyresponded to antibiotics. Bone biopsy excluded malignantlesion or infectious etiology, and gallium scintigraphyrevealed inflammation of scalp, mandible, and sternum.Based on these finding, she was diagnosed to haveSAPHO syndrome and intravenous corticosteroidsfollowed by tacrolimus were administered with rapidresponse for both skin and mandiblar lesion.CONCLUSION: SAPHO syndrome is a rarecomplication of pediatric inflammatory bowel disease,and causes significant morbidity. Early diagnosis mayleads to improvement of the condition. However, longterm management of SAPHO syndrome remains stillchallenging.[Keywords] SAPHO Syndrome, Crohn's disease, Infliximab, Children307

P2-109 Gastroenterology P2-110 GastroenterologyA SEVERE OBESITY OF 12-YEAR-OLD BOYWITH ACUTE SEVERE NECROTICHYDROPS GALLBLADDER ASSOCIATED WITHSALMONELLA INFECTIONPANCREATITISCHENG-KUO TSAI, AN-CHYI CHEN, SHU-FENWU, CHUN-CHIN CHUANG, TIEN-KAI TSAI,YI-PEI TAI, MEE-MEE LEONG, WALTER CHEN,CHING-TIEN PENG1 Fong Yuan Hospital, Taichung, Taiwan; 2 Children’s Medical Center,China Medical University Hospital, Taichung, Taiwan; 3 PediatricDepartment, Ping Tung Christian Hospital, Pintung, TaiwanMao-Meng Tiao, Kuo-Shu Tang, Ying-Hsien Huang,Fu-Chen Huang, Chung-Ching LuPediatrics, Chang Gung Memorial Hospital, TaiwanINTRODUCTION: Pediatric acute pancreatitis isuncommon, and severe acute pancreatitis is rare in children.The elevated APACHE score, high C-reactive protein levels,persistent multiple organs dysfunction for more than oneweek, the necrosis seen on CT scan may predict severedisease with a poor prognosis. We reported a 12-years-oldboy who is a sever obese children (135 Kgs) with acutesevere pancreatitis whose Ranson score is seven. In general,patient with Ranson score 7 will get very high rate ofmortality. The 12-year-old boy suffered from severe andpersistent epigastric pain for 2 days before admission. Hewas carried to our emergent department for help. The serumamylase (963U/L) and lipase (2124U/L) were elevated. Theabdominal CT revealed acute pancreatitis and fatty liver. Theclinical course of our patient underwent septic shock, acuterespiratory distress syndrome, acute renal failure, pseudocystof pancreas, intra-abdominal abscess and its Ranson scorewas seven. Percutaneous abscess drainage (PAD) insertionand another eight times of revision were performed forintra-abdomen abscess. The lung condition and changingposition under sedation were another challenge for medicalmembers due to his severe obesity (BW: 135kg, equal to297bounds, BMI: 44.08). After intensive pediatric team care,the patient was discharged under general condition wasstable.DISCUSSION: Why we can success of curing this case? Weconsidered that may relate to some factors. First,prophylactic antibiotics, anti-inflammation agents such asFoy (Gabexate Mesilate) and Sandostatin were givenimmediately and fed via N-J nutritionally when the amylasedown to normal range. Second, when multiple organsdysfunction occurred, we adjusted to adopt team care, thatradiological expert to place PAD, chest man to modulateventilator setting, pediatric nephrologist to performhemodialysis, and endocrinologist to modify hyperglycemia.Third, how to handle a sever obesity children (135Kgs) isgreat challenge to nurse, so excellent nursing care is moreimportant.CONCLUSION: We reported the severe acute necroticpancreatitis complicated with multiple organ failure whichshowed favorable outcome after was given intensive care viateam cooperation. We presented this case to remind clinicalpractician that the severe obesity of the child with acutepancreatitis was a challenge to medical members. Not only tomedical doctor but also to paramedical staff would face manynew challenge. After discharge, the diet and body weightcontrol were another concern for this children.OBJECTIVE: The hydrops gallbladder may be associatedwith diarrhea without right upper quadrant (RUQ)abdominal painMETHODS: A 3-year 2-month old boy had fever anddiarrhea for 5 days. There is periumbilical pain andwatery diarrhea without blood or mucus in the stool butno RUQ pain.MAIN RESULTS: The WBC (11,000/ul) and CRP (12mg/L) showed non-specific findings. The ultrasound wasarranged for the fever persisted and periumbilical painconsisted on the 2 nd admission day. The gallbladderdistended was found with sized 7.0x5.3cm at liverperi-hilar area. There was no RUQ pain or tenderness inthe entire hospital course. At the 4 th admission day, thestool culture grew Salmonella B and ceftriaxone wasprescribed. The fever and diarrhea condition regressed at7th admission days and the gallbladder recovered. Thepatient discharged on the 8 th days of admission withoutany complications.CONCLUSION: The Salmonella enterocolitis may beassociated with hydrops gallbladder that presented withno RUQ abdominal pain and gets good recovery.[Keywords] gallbladder, dilatation, child, salmonella[Keywords] acute pancreatitis308

P2-111 Gastroenterology P2-112 GastroenterologyA 8 YEARS 8 MONTHS OLD BOYDIAGNOSED WITH HYPERTROPHICPYLORIC STENOSIS: A CASE REPORTMee-Mee Leong 1,2 , An-Chyi Chen 2 , Shu-Fen Wu 2 ,Walter Chen 2 , Ching-Tien Peng 2 , Yow-Yue Chin 1 ,Teck-Siang Tok 1 , Solomon Chih-Cheng Chen 11 Ping Tung Christian Hospital, Pediatric Department, Pingtung, Taiwan2 Children’s Medical Center, China Medical University Hospital, Taichung,TaiwanOBJECTIVE: Hypertrophic pyloric stenosis is mostcommon surgical disorder of the stomach and duodenum ininfants. It is rarely happen in childhood and adolescent. Theexact cause of hypertropic pyloric stenosis is unknown.Non-bilious vomiting is the initial sign in clinicalpresentation. The vomiting may or may not be projectile, isusually progressive. Prolonged vomiting may lead todehydration, weight loss and failure to thrive. We reported acase of 8 years 8 months old boy who diagnosedhypertrophic pyloric stenosis.METHODS: A 8 years 8 months old boy presented withnon-bilious vomiting and watery diarrhea for one month.These symptoms had no improvement after treated by thedoctors in many clinics. His parent brought him to ourhospital for help. On physical examination, he looked thinand weak in appearance, as well as he had periumbilicaltenderness. He was admitted to our ward for furtherevaluation and management.MAIN RESULTS: He was admitted to our ward under theimpression of nephrotic syndrome. It was because hisrandom urine analysis and serum electrolytes (sodium,potassium and chloride) showed urine protein greater than300 mg/dL and hypokalemia. On admission, after assessingby our nephrologist, he did not have the problem of nephroticsyndrome, because random urine protein/creatinine ratioshowed less than 0.2, which meant no pathologicalproteinuria. In the meantime, a cystic-like abdominal masswas palpated. Abdominal ultrasound revealed extensivegastric distention. Due to persistent vomiting, abdominal CTscan was done. It revealed pyloric wall thickening andsuspected gastric outlet obstruction. Upper gastrointestinalseries showed positive teat sign and shoulder sign, pyloricstenosis was impressed. Pyloric ring stenosis, gastric andesophageal ulcers, and hemorrhagic gastritis were noted inthe procedure of panendoscopy. There was no metabolicalkalosis. Trace back to his pass history. He had twoadmission histories in our hospital for acute bronchitis andherpangina at the age of 9 month-old and 2 year-old,respectively. His mother claimed that his growth anddevelopment were normal as compared with his peers.Ramstedt pyloromyotomy was arranged by our surgeon aftercorrection of hypokalemia. Hypertrophic pyloric muscle withsevere pyloric canal stricture was identified during operation.The wall thickness was 1.5 cm and the length was 3.5 cm.CONCLUSION: To this patient, the cause of developmentof pyloric hypertrophic stenosis is unknown. Hypertrophicpyloric stenosis is possible to happen in the age beyondinfancy.[Keywords] hypertrophic pyloric stenosisA CASE REPORT –A EIGHT YEARS OLD GIRLGOT WILSON DISEASE WITH INITIALPRESENTATION OF LOBAR PNEUMONIA ,ACUTE YELLOWISH SKIN PRESENTATION ANDHEMOLYTIC ANEMIATien-Kai Tsai 1 , An-Chyi Chen 2 , Shu-Fen Wu 2 , Kang-HsiWu 2 , Chia-Lin Chang 3 Ching-Tien Peng 2 , Walter Chen 2 ,Chun-Chun Chuang 21 Pediatrics, Fong-Yuan hospital Department of Health Executive Yuan, Taiwan2 Pediatrics, China Medical University Hospital, Taiwan; 3 Pediatric Surgery,China Medical University Hospital, TaiwanOBJECTIVE: Wilson disease (WD; hepatolenticular degeneration) is anautosomal recessive disorder characterized by accumulation of intracellularhepatic copper with subsequent hepatic and neurological anormalities. Theincidence is 1/500 to 1/100000 births. If there was no more treatment for thisdisease, high mortality rate related liver cirrhosis will happen in the future.Young aged children who have acute fulminant hepatic failure combined withWD are very rare over the world.MATERIAL & METHODS: A eight years old patient had acute onset ofjaundice and then acute fulnimant hepatic failure was impressed in ourhospital. We arranged series study for the patient and closely observed ofher courses.MAIN RESULTS: The eight years old girl was admitted in our hospital dueto fever for one week and yellowish skin presentation for one day. Commoncold symptoms were noted in recent one week. Due to respiratory distress,we transferred the patient to PICU for further care under severe pneumoniaand was proved by CXR. However, blood sampling revealed anemia andelevated liver function test. We arranged abdominal echo for her. It revealedliver parenchymal disease and hydrops of gallbladder with massive sludgeformation. Due to persisted high level of bilirubin level (T/D bilirubin:8.6/4.9533.25/21.70 mg/dl), we arranged percutaneous transhepaticgallbladder drainage (PTGBD) under impression of obstructed jaundice. Wealso noted that she had hepaticencephalopathy at this time. Due to poorfunction of PTGBD, we arrange laparotomy for T-tube insertion. Noduleformation with coarse surface and blunting liver margin were noted. Livercirrhosis was impressed. Therefore, we arranged series study of Wilsondisease. Laboratory data showed: Ceruloplasmin 16.3mg/dl, serum Cu:1692.8 ug /dl, urine copper: 5080 ug /dl, serum ammonia level:153 ug/dl ,24hr urine copper excretion: 1103.7 ug /day. Plasmapheresis andhemodialysis were performed. We also consulted the surgeon for thepossibility of liver transplantation under hepatic failure. The molecularanalysis showed ATP7B gene mutation. From all the study, symptoms andsigns, she was compatible with criteria of WD. After the plasmapheresis,medication treatment and supportive care, her condition was improvedgradually. Her conscious is relative clear now and waiting for livertransplantation.CONCLUSION: The young children got lobar pneumonia and mildjaundice at initial presentation and wouldn’t showed typical presentation ofliver disease at before. It may let physician making the focus to treat severepneumonia, and easily neglect sign of jaundice. Early diagnosis of WDshould be pursued because of the disease is fatal if not promptly recognizedand treated it. From literature showed that Wilson disease happened withacute hepatic failure in young children was very rare. Our patient give us aclinical experience, when a children got sever hemolytic anemia jaundicewith mild abnormal elevated level of alanine aminotransferase despite thepatient may suffer from other severe disease at initial such as pneumoniashould be considered the possibility of Wilson disease were happened inthese like patient. Liver transplantation is curative and should be consideredfor patients with fulminant hepatic failure. How to early diagnosis of WDwith acute fulminant hepatic failure and effective treatment in young childrenis still a challenge for pediatrician.309

P2-113 Endocrinology P2-114 EndocrinologyTHE MICB GENE AND TYPE 1 DIABETES INCHILDRENChi-Yu Huang 1 , Wei-Hsin Ting 2 , Fu-Sung Lo 3 ,San-Ging Shu 4 , Pao-Ching Chiu 5 , Shao-YinChu 6 ,Chia-Ching Chen 7 , Chao-Hsu Lin 8 , Yi-Lei Wu 9 ,Yann-Jinn Lee 10Pediatrics, Mackay Memorial Hospital 1,2, 9 , Pediatrics, Chang GungMemorial Hospital 3 , Pediatrics, Taichong Veterans General Hospital 4 ,Pediatrics, Kaohsiung Veterans General Hospital 5 , Pediatrics, BuddhistTzu-Chi General Hospital 6 , Pediatrics, Chiayi Christian GeneralHospital 7 , Pediatrics, Mackay Memorial Hospital, Hsin-Tsu 8 , Div ofPediatric Endocrinology, Depart of Pediatrics & Medical Research,Taiwan 10Type 1 diabetes mellitus (T1D) is among the most prevalentendocrinologic diseases in children. The pathogenesis isautoimmune-mediated β-cell destruction involving T- andB-cells. Studies have shown that it is a polygenic disease withthe strongest association with the HLA locus. The MICB gene islocated in the Class I region of this locus. Its encoded MICBprotein is an HLA class I molecule which is stress-induciblesurface molecule and recognized by γδT cells and may play acentral role in the immune surveillance. There is a CAdinucleotide repeat microsatellite in intron 1 of the gene.Association studies using this marker have yielded conflictingresults in different ethnic population. Thus it is worthwhile toinvestigate the association between the gene and diseases in ourpopulation.SUBJECTS: The patients consisted of 279 unrelated childrenwith T1D (138 boys, 141 girls). The age at diagnosis was 7.5 ±3.8 (0.8-17.7) years. The control population comprised 377unrelated adults (133 males, 244 females). All subjects wereethnic Chinese in Taiwan.Determination of the (CA) n-microsatellite polymorphismPCR primers flanking the microsatellite (MICB5F,5'-CTACCTCCTTGCCAAACTTGCTGTTTGTG-3'; MICB5R,5'-AATAGCCATGAGAAGCTATGTGGGGGAG-3') wereaccording to the reported sequence. MICB5F was 5' end-labeledwith fluorescent dye. Alleles were designated according to thenumber of repeats.STATISTICAL ANALYSIS: Evaluation for Hardy-Weinbergequilibrium was performed using PyPop Win32-0.6.0. Allelefrequencies of patients and controls were compared using thetest, with Yates' correction where appropriate (one expectednumber

P2-115 Endocrinology P2-116 EndocrinologyASSOCIATION OF CLEC16A GENE WITHGENETIC SUSCEPTIBILITY AND CLINICALSCREENING OF THE DEPRESSIVE SYMPTOMSIN TEENAGER TYPE 1 DIABETES MELLITUSSTATUS OF TYPE 1 DIABETESYanmei Sang, Wei Zong, Min Liu, Jie YanBeijing Children’s Hospital affiliated to Capital Medical University,Bejing, China, 100045Hui-Pin Hsiao, Gui-Ju Tseng, Mei-Chyn Chao, Bai-HsiunChenDepartment of Pediatrics, Kaohsiung Municipal HsiaoKang Hospital, KaohsiungMedical University, TaiwanOBJECTIVE: To investigate the association ofCLEC16A gene polymorphism with type 1 diabetesin Chinese children.SUBJECTS AND METHODS: 131 Chinesechildren with type 1 diabetes were selected asresearch subjects, all meeting with the 1999 WHOT1DM diagnostic criteria.121 healthy adult blooddonors were selected as normal controls. Saturatedsalting-out method technique were used to extractgenomic DNA from peripheral white blood cells,after which Mass Spectrometry techniques wereused to study the distributions of 17 CLEC16Aalleles in patients and controls. And the relationshipbetween CLEC16A gene polymorphism and geneticsusceptibility and clinical status of type 1 diabetesin Chinese children were studied.RESULT: The distributions of two polymorphisms(rs12921922,rs12931878) of CLEC16A in type 1diabetes and healthy controls have significantdifferences.(1)The alleles of rs12921922 are C andT. The frequencies of T allele are significantincreased in patients (90.8% vs healthy controlsubjects 85%, P = 0.044

P2-117 Endocrinology P2-118 EndocrinologyETIOLOGIC CLASSIFICATION OFTAIWANESE PATIENTS WITH DELAYEDPUBERTY: ONE MEDICAL CENTERREPORTTWO MALE SIBLINGS WITH AMICRODELETION OF THE XP21 REGIONPRESENTING WITH ADRENAL HYPOPLASIAAND MENTAL RETARDATIONYing-Hua Huang, Fu-Sung Lo, Yang-Hau VanDivision of Pediatric Endocrinology, Chang Gung Children’s MedicalCenter, Chang Gung Memorial Hospital, Linkou, TaiwanOBJECTIVE: Delayed puberty is defined as the absence ofsecondary sexual charactristics by an age that is more than 2–2.5SD above the mean for the population (13 years in girls and 14years in boys). It is caused by constitutional delay of growth andmaturation (CD), permanent hypogonadotropic hypogonadism(HypoH), permanent hypergonadotropic hypogonadism(HyperH), and functional hypogonadotropic hypogonadism(FHH). We studied 49 patients with delayed puberty andanalyzed the etiologic classification.METHODS: We performed a retrospective study of clinical andlaboratory data from 49 patients (26 males, 23 females) visitingour endocrine clinics for delayed puberty since Aug 1996 to Nov2009.RESULTS: The most common cause of delayed puberty isHypoH (46% of males and 39% of females). The remainingsubjects could be divided into three categories: those with CD,23% of males and 26% of females; those with FHH, 23% ofmales and 3% of females; and those with HyperH, 8% of malesand 22% of females.CONCLUSION: Our study provides valuable data regarding thevariety and frequency of diagnoses that lead to delayed puberty.[Keywords]delayed puberty, constitutional delay of growth and maturation, permanenthypogonadotropic hypogonadism, permanent hypergonadotropic hypogonadism, andfunctional hypogonadotropic hypogonadismKazuko Yoshimura 1 , Rihito Hanamiya 1 , Makoto Tsutsumi 1 ,Noriyuki Katsumata 2 , Toshio Kojima 3 , Shinichi Hirose 1Department of Pediatrics, Faculty of Medicine, Fukuoka University 1Department of Endocrinology and Metabolism, National Research Institute forChild Health and Development 2 ; Computational Systems Biology Research Group,Advanced Science Institute,RIKEN, Yokohama, Japan 3BACKGROUND: Mutations of the dosage-sensitive sexreversal-adrenal hypoplasia congenital critical regions onthe X-chromosome gene 1, (DAX1) have been identifiedas the cause of X-linked adrenal hypoplasia andgonadotropin deficiency. It is also known that mutationsof DAX1 per se do not cause mental retardation (MR). Wereport two male siblings with adrenal hypoplasia andgonadotropin deficiency and MR resulting from amicrodeletion of the Xp21 region affecting DAX1 and theinterleukin-1 receptor accessory’s protein-like (IL1RAPL)gene, which is known to associated with non-specificMR. CASE1: A five-year-old boy, who in infancyshowed brown skin pigmentation, jaundice, and saltloosing. He was diagnosed with adrenal insufficiency andwas given hydrocortisone, fludrocortisone, and NaCl. Atfour, his adrenal gland could not be found usingabdominal CT. His DQ was 40 and he was hyper-active.CASE2: A three-year-old, Case 1’s younger brother. Atbirth, he had the same brown pigmentation as his brotherand like him was diagnosed with adrenal insufficiency. Atthree, his DQ was 45 and his right adrenal gland could notbe found using CT. In both cases, DAX1 deletion wasfound by Southern Blot, and CGH microarray showed a3Mbp microdeletion in Xp21.1-21.2 involving DAX1 andIL1RAPL.CONCLUSION: The microdeltion identified in thesiblings encompasses not only IL1RAPL but also severalgenes, which are also know to cause non-specific MR.Further research on the phenotypes of cases with variousmicrodeletions in the relevant chromosomal region shouldcontribute to the understanding of the molecularmechanisms of MR.[Keywords] Xp21, mental retardation, microdeletion312

P2-119 Endocrinology P2-120 EndocrinologyMOLECULAR DIAGNOSIS OFPSEUDOHYPOPARATHYROIDISM IA ANDPSEUDOPSEUDOHYPOPARATHYROIDISMIN CHILDRENTHE PREDICTION OF METABOLIC SYNDROMEIN CHILDHOOD OBESITY USING HOMA ANDQUICKI INSULIN SENSITIVITY INDEXES AND IRFEATURESYi-Lei Wu, Daw-yang Hwang, Wei-Hsin Ting, Chi-YuHuang, Hung-Chun Chen, Yann-Jinn LeePediatrcis, Mackay Memorial Hospital, TaiwanPseudohypoparathyroidism (PHP) is due to a lackof sensitivity to the biologic actions of PTH. Serumconcentrations of PTH in patients with PHP arehigh despite hypocalcemia and hyperphosphatemia.Patients with PHP Ia also has Albright hereditaryosteodystrophy (AHO) which typically includesround face, short thick neck, obesity, short terminalphalanges, short 4th and 5th metacarpals,subcutaneous ossifications, and absence of the 4thknuckle (brachydactyly type E). Resistance to othercyclic AMP-dependent hormones, especially TSHand gonadotropins may be present. In a few patientswith deficient Gs protein activity, hypothyroidismrather than hypocalcemia was the initialpresentation of the disorder. We reported moleculardiagnosis on patients with PHP Ia orpseudopseudohypoparathyroidism (PPHP).SUBJECTS AND METHODS: 3 girls had AHO,hypocalcemia, hyperphosphatemia, and elevatedPTH levels. Among them, 2 were siblings and theywere initially diagnosed to have hypothyroidism.Another girl had PPHP with AHO but normal Caand P levels. Genomic DNA from peripheral bloodleukocytes of patients and their relatives wereanalysed. All thirteen exons and intron-exonboundaries of the GNAS gene was amplified byPCR and directly sequenced.RESULTS: The 2 siblings had a mutation Q29X inthe GNAS gene, another girl with PHP had IVS10-2A>G (intron 10 splicing acceptor site mutation).The girl with PPHP had G73D missense mutation.CONCLUSION: We detected 2 novel mutationsand our result expands the spectrum of GNASmutations associated with PHP. or PPHP.Chawkaew Kongkanka 1 , Vichit Supornsilchai 2 , SuttipongWacharasindhu 3Pediatric Endocrinology, Growth and Growth Monitoring Center, ChulalongkornUniversity 1 , Pediatrics, Pediatric Endocrinology Unit 2 , Pediatrics, Faculty ofMedicine, Chulalongkorn University 3 (Thailand)OBJECTIVE: Childhood Obesity is becomingemergence around the world. One of the conditions thatoften found in childhood obesity is insulin resistance (IR).Based on the literature, several researches show thecorrelation of IR and 3 diseases; Type 2 Diabetes Mellitus(T2DM), Cardiovascular disease (CVD), and Metabolicsyndrome (MS). The objective of this study is to predictthe occurrence of metabolic syndrome in childhoodobesity using HOMA and QUICKI insulin sensitivity (IS)indexes and IR features as the predictor variables.METHOD: Pre-collected information of a group ofobese children at the age 10-18 whose Oral GlucoseTolerance Test (OGTT) is performed is extracted fromclinical database. Then, HOMA and QUICKI arecalculated. The information related to IR features such as1) birth weight, 2) history of maternal gestationaldiabetes, 3) positive family histories of diabetes, obesity,hypertension, CVD, and/or stroke, 4) acantosis nigrican(AN), 5) premature pubarche/gynecomatia, 6)dyslipidemia, and 7) fatty liver (NASH) is analyzed andclassified into 2 separated groups; simple obesity andobesity with MS. Obesity is defined as children who havethe percentage of weight for height equal or greater than120%. MS is defined by IDF consensus 2007.RESULTS: Based on the obesity analysis of 45 obesechildren (male 34 and female 11), thirty-two areconsidered simple obesity and 13 considered obesity withMS. For simple obesity HOMA 4.40 ± 4.16, QUICKI0.33 ± 0.44 and severe and morbid 75 % For obesity withMS, HOMA 6.28 ± 4.51, QUICKI 0.30 ± 0.02 and severeand morbid 84.6 %.CONCLUSION: In comparison, obesity with MS groupis less insulin sensitivity than simple obesity by QUICKI(p 0.02). Also, hypertension and dyslipidemia are foundedmore in obesity with MS than simple obesity group.Further study are required for more satisfactory cut pointstatistical in prediction of MS in childhood obesity.[Keywords]metabolic syndrome, childhood obesity, insulin sensitivityindexes313

P2-121 Endocrinology P2-122 EndocrinologyTHE IL4 GENE AND HASHIMOTOMONITORING IN TWINS/TRIPLETSTHYROIDITIS IN CHILDRENYann-Jinn Lee, Chi-Yu Huang, Wei-Hsin Ting,Fu-Sung Lo, Shao-Yin Chu, Chia-Jung Chan,Chen-Mei Hung, Chao-Hsu Lin, Hsin-Jung Li,Wen-Ling Guo, Ching-Cheng TsangDiv of Pediatric Endocrinology, Depart of Pediatrics & Medical Researc,Macaky Memorial Hospital, TaiwanSuttipong Wacharasindhu, ChansudaBongsebandhu-phubhakdi, Suphab Aroonpakmongkol,Taninee Sahakitrungruang , Vichit SupornsilchaiGrowth and Growth Monitoring Center, Endocrine Unit, Department of Pediatrics,Faculty of Medicine, Chulalongkorn University, Bangkok 10250, ThailandHashimoto thyroiditis (HT) is the most common cause ofacquired hypothyroidism in children and adolescents. It is aTh1 mediated thyroiditis with apoptosis of thyroid follicularcells resulting in hypothyroidism. The affected thyroid glandis infiltrated by both B cells and T cells. IL4 is produced byactivated CD4 + T cells, mast cells, and basophils. It is thepivotal cytokine which polarizes the immune responsetoward a Th2 cell response but opposes the Th1 cellinflammatory response. Therefore it is a candidate gene forthe association study on HT.MATERIALS AND METHODS: The patients were 96unrelated children (89 girls) with HT. Their age at diagnosiswas 10.9 ± 3.2 (3.7 – 19.8) years. The controls consisted of686 subjects. All individuals were ethnic Chinese in Taiwan.GENOTYPING OF THE SNPS: dbSNP rs2243250 andrs2243289 of the IL4 gene were determined using thePre-Developed TaqMan Allelic Discrimination Assay.STATISTICAL ANALYSIS: We assessed theHardy-Weinberg equilibrium for rs2243250 and rs2243289,estimated the frequencies of haplotypes and tested pairwiselinkage disequilibrium (LD) between the SNPs usingHaploview 4.1. Statistical differences in genotype, allele,carrier, and haplotype distributions between patients andcontrols were performed using the χ 2 test. Odds ratios and95% confidence intervals were calculated. The Bonferronicorrection was used for multiple comparisons whereappropriate. Two-tailed P c values of less than 0.05 wereconsidered statistically significant.RESULTS: The genotypes of both SNPs were inHardy-Weinberg equilibrium in controls (rs2243250: P=0.82, rs2243289: P=0.86). The frequencies of each allele ofthe SNPs or haplotypes was not significantly differentbetween male and female controls. The 2 SNPs were closelylinked. There was no significant difference in the frequenciesof the genotype, allele or carrier of both SNPs between thepatients and controls. Neither was there in frequencies of thehaplotypes.CONCLUSION: There was no significant associationbetween the IL4 gene and HT in children. Genotyping thetagSNPs of the gene and nearby cytokine genes arenecessary.BACKGROUND: Twins/triplets baby are normally born lighter orshorter than singleton. The time when they catch up their growth hasrarely been studied. Aim of this study is to monitor growth in childrenborn twins/triplets and determine the time when they can catch up theirgrowth.MATERIAL& METHOD: Forty-five children ( 21 Twins, 1 Triplets)were included in this growth monitoring study. They were born at amean gestational age (GA) of 34.6 ±3.5 week with a mean birthweight of 2.2 ± 0.6 kg and a mean birth length of 44.8 ± 3.3 cm. Eight ofthem—5 from twins and 3 from triplets—were born small forgestational age (SGA). Catch-up for length/height and weight is definedwhen their length/height and weight have reached the 3 rd centile fornormal Thai children growth data.RESULTS: As for length, or sometimes, height, 11.9%, 11.9%, 11.1 %and 5% of them still do not catch up at 6 th month, 1 st year, 2 nd year and4 th year of age. As for weight, 14%, 11.1% and 9.7% of them still do notcatch up at 6 th month, 1 st year and 2 nd year of age. All of them catch upfor weight at 4 th year of age. (Table 1)% Noncatch-up forheight% Noncatch-up forweight6 month 1 year 2 year 4 year11.9 11.1 11.1 514 11.1 9.7 0Table 1. Percent of non catch-up Twins/ TripletsOne patient who did not catch up height at 4 year of age has beendiagnosed as having growth hormone (GH) deficiency based onbiochemical criteria of standard GH provocative test. She was born at 32week GA with birth weight of 1.66 kg and length 43 cm which iscategorized as AGA (appropriate for gestational age).CONCLUSION: Ninety percents of children born twins/triples cancatch up their growth by 2 years of age. Weight is caught up in alltwins/triples by 4 years of age. In our study, GH deficiency can occur in1 of 45 (2.2 %) children born twins/triplets. Therefore, children borntwins/triplets who do not catch up their height by 4 years of age shouldhave endocrine evaluation.[Keywords]Twins, Triplets, Growth hormone[Keyword] IL4, Hashimoto thyroiditis, child, case-control study314

P2-123 Endocrinology P2-124 EndocrinologyBENEFIT OF GnRH ANALOG ON HEIGHTPREDICTION IN 21- HYDROXYLASEDEFICIENCY COMPLICATED BY CENTRALPRECOCIOUS PUBERTYEFFECT OF EXTRACELLULAR POTASSIUM ONDELAYED RECTIFIER POTASSIUM CHANNELPROTEINS OF KCNQ3 AND KCNQ5 IN FAMILIALHYPOKALEMIC PERIODIC PARALYSISChansuda Bongsebandhu-phubhakdi 1 , VichitSupornsilchai 2 , Suttipong Wacharasindhu 3Pediatrics, Growth and growth monitoring center 1 , Pediatrics, PediatricEndocrinology Unit 2 , Pediatrics, Faculty of Medicine, ChulalongkornUniversity 3 (Thailand)OBJECTIVE: To examine effect of GnRH analog(Leuprolide acetate) on height prediction incongenital adrenal hyperplasia (CAH) due to21-hydroxylase deficiency (21-OHD) complicatedby central precocious puberty (CPP).METHOD: Data of patients with CAH (21-OHD)complicated by CPP including demographic data,auxanological data, dosage of therapeuticglucocorticoid treatment and monitoring oftreatment by 17OHP level were retrospectivelycollected. Height prediction outcome of pre andpost-treatment with GnRH analog were comparedby Paired-Sample T test.RESULT: Six patients, 3 boys and 3 girls withCAH (21-OHD), documented by clinical,biochemical and hormonal criteria were recruited inthe study. All of them were classical CAH; 3patients with simple virilization form (SV) and 3patients with salt wasting form (SW). The meanpresenting age of CPP was 6 years and 4 months.Duration of GnRH analog treatment was 5-54months (mean=25.2months). Dosage ofglucocorticoid treatment and 17OHP level of preand post-treatment were not significantly different(p=0.449 and 0.149 respectively). Predicted adultheight were improved significantly (p=0.00039)after treatment (11.72±3.4cm), and half of patients’predicted adult height could be reached their targetheight.CONCLUSION: This study emphasizes benefit ofGnRH analog for improving height prognosisoutcome in patients with classical CAH (21-OHD)complicated by CPP.[Keywords]CAH / 21OHD/ central precocious puberty/GnRHagonist/height prognosisJune-Bum Kim ,Yang-Hee ParkDepartment of Pediatrics, Konyang University College of Medicine, KoreaOBJECTIVES: Familial hypokalemic periodic paralysis(HOKPP) is an autosomal-dominant channelopathycharacterized by episodic attacks of muscle weaknesswith concomitant hypokalemia. Mutations in either acalcium channel gene (CACNA1S) or a sodium channelgene (SCN4A) have been responsible for this disease;however, the molecular mechanisms have not yet beenelucidated. Previous studies have suggested that thecombination of sarcolemmal depolarization andhypokalemia has been attributed to abnormalities of thepotassium conductance governing the resting membranepotential. To understand the pathophysiology of thisdisorder, we investigated the expression patterns ofdelayed rectifier potassium channel genes, KCNQ3 andKCNQ5, in skeletal muscle cells from patients withHOKPP (patient cells) and normal individuals (normalcells).METHODS: We obtained skeletal muscle cells frompatients with familial HOKPP and healthy volunteers andinvestigated the mRNA levels of KCNQ3 and KCNQ5and the protein levels therein at normal (4 mM) and high(50 mM, for depolarization) potassium concentrations.MAIN RESULTS: Both patient and normal cellsexposed to 50 mM potassium buffer, which was used toinduce depolarization, did not show significant change inthe mRNA levels of both channel genes; however, theprotein level of KCNQ3 significantly decreased in themembrane fraction of patient cells on the contrary to asignificant increase in normal cells.CONCLUSION: Our results suggest that long termexposure of skeletal muscle cells in HOKPP patients tohigh extracellular potassium alters the KCNQ3localization, which could reduce the normal function ofthis channel protein. These findings may provide animportant clue to understanding the molecular mechanismof familial hypokalemic periodic paralysis.[Keywords]hypokalemic periodic paralysis, delayed rectifier potassiumchannel, depolarization315

P2-125 Medical Genetics/Metabolism P2-126 Medical Genetics/MetabolismPOLYSOMNOGRAPHICCHARACTERISTICS IN PATIENTS WITHMUCOPOLYSACCHARIDOSESHsiang-Yu Lin 1,2,3,4 , Shuan-Pei Lin 1,2,4,5* , Dar-ShongLin 1,2,4 , Ching-Chi Lin 2,4,6 , Chih-Kuang Chuang 2,7 ,Dau-Ming Niu 3,8 , Ming-Ren Chen 1,4 , Hung-ChangLee 1,9Departments of 1 Pediatrics and 2 Medical Research, Mackay MemorialHospital, Taipei, Taiwan; 3 Institute of Clinical Medicine, NationalYang-Ming University, Taipei, Taiwan; 4 Mackay Medicine, Nursing andManagement College, Taipei, Taiwan; 5 Department of Infant and ChildCare, National Taipei College of Nursing, Taipei, Taiwan; 6 Chest Division,Department of Internal Medicine, Mackay Memorial Hospital, Taipei,Taiwan; 7 Medical College, Fu-Jen Catholic University, Taipei, Taiwan;8 Department of Pediatrics, Taipei Veterans General Hospital, Taipei,Taiwan; 9 Department of Pediatrics, Taipei Medical University, Taipei,TaiwanOBJECTIVE: Sleep-disordered breathing is a commonsymptom in mucopolysaccharidoses (MPS). We evaluatedovernight polysomnographic measures in patients with MPS inorder to clarify the nature of their sleep and breathing.METHODS: Overnight continuous EEG-polysomnographicstudies were performed in 24 patients with MPS (22 males and 2females; mean age, 10.8 ± 5.9 years; age range, 2 years to 24years) unselected for sleep disturbance.MAIN RESULTS: The diagnosis of 24 patients with MPS wasconfirmed by two-dimensional electrophoresis of urinaryglycosaminoglycans and enzyme assay in serum, leukocytesand/or skin fibroblasts, which identified 3 with MPS I, 15 withMPS II, 1 with MPS III, 1 with MPS IV, and 4 with MPS VI. Thebody mass index (BMI) was 20.6 ± 2.9 (15.5-26.4). The sleepefficiency was 88.5 ± 4.3%. The baseline arterial oxygensaturation (SpO 2 ) was 96.1 ± 1.5%, with a nadir of 74.5 ± 12.3%.Overall, the average percentage of time subjects spent at 2), and 15/24 patients were classified ashaving severe OSA (RDI>10). Two patients with MPS IIunderwent enzyme replacement therapy both showed reductionsin their RDI (38.9 to 10.8 and 3.5 to 2.0, respectively). We alsosubdivided these 24 patients into 2 groups (prepubertal vs.pubertal and postpubertal), which showed that there weresignificant higher values of RDI, DI, arousal index, and PLMSindex in the pubertal and postpubertal group (p < 0.05).CONCLUSION: Patients with MPS showed a very highprevalence of OSA and pubertal and postpubertal patientsshowed more severe hypoxemia and more sleep disruptionduring sleep compared with prepubertal patients. The completepolysomnography will help to determine the abnormalities ofbreathing during sleep more precisely and urge the clinicians totake necessary action for patients with severe manifestations.[Keywords] mucopolysaccharidoses, obstructive sleep apnea,polysomnography, sleep-disordered breathingIDENTIFICATION AND CARRIER-FREQUENCYDETECTION OF MAPLE SYRUP URINE DISEASEIN THE ABORIGINAL TRIBES OF NORTHTAIWAN: DIFFERENT GENE PREFERENCEFROM THOSE IN SOUTH ONESJia-Woei HouPediatrics, Cathay General Hospital, TaiwanMaple syrup urine disease (MSUD) is a rare disordercaused by a block in the decarboxylation step in thecatabolic pathways of the branched-chain amino acids.Mutations in any one of the four different genesE1-alpha, E1-beta, DBT (E2) and E3 genes areresponsible for this disease. In Taiwan, only few MSUDcases have been diagnose clinically but most of thepatients are from aboriginal tribes. A common 4.7 kbdeletion on E2 gene indicating a founder mutation amongAustronesian aboriginal tribe Paiwan in southern Taiwanhas been reported. Our study identified the molecularchanges of another four affected children and evaluatedthe carrier-frequency in their subpopulations.Homozygous A208T and I281T of E1α were found in 2patients of Hans (non- aboriginal Taiwanese), compoundheterozygous mutations of E2 gene: 4.7 kbdeletion/c.650-651insT (L217F or L217fsX223) andc.650-651insT/c.88-89delAT in 2 patients of Amis,respectively, after direct DNA sequencing and PCR-RFLPstudies. There were no deleted 4.7 kb heterozygote out of423 normal people of Hans (non- aboriginal Taiwanese,n=125), Atayal (n=156), and Saisiyat (n=21), but noted insoutheastern tribes of Amis (1/121). Although theTaiwanese Austronesian aboriginal tribes are consideredto hare a common origin, this E2 4.7 kb deletion may bepreserved in the southern/eastern, but not northernaboriginal tribes of Taiwan.[Keywords]Maple syrup urine disease, Taiwanese aboriginalAustronesian tribe, DBT (E2) gene, carrier frequency316

P2-127 Medical Genetics/Metabolism P2-128 Medical Genetics/MetabolismCASE REPORT: 46,XY,DER(9)T(9;Y) IN APATIENT WITH 9P DELETION SYNDROMEOTOLOGICAL FEATURES OF PATIENTS WITHTURNER SYNDROME IN TAIWANAND SEX REVERSALMeng-Che Tsai 1 , Yen-Yin Chou 2 , Keng-Fu Hsu 3 , MingChen 4 , Pao-Lin Kuo 5 , Shio-Jean Lin 6Department of Pediatrics, National Cheng Kung University Hospital 1 ,Pediatrics, National Cheng Kung University Hospital 2 , Pediatrics,National Cheng Kung University Hospital 3 , Obstetrics and Gynecology,National Cheng Kung University Hospital 4 , Obstetrics and Gynecology,National Cheng Kung University Hospital 5 , Medical Genetics, ChanghuaChristian Hospital, Taiwan 6OBJECTIVE: The chromosome 9p deletion syndrome ischaracterized by trigonocephaly psychomotor retardation,dysmorphic facial features, and abnormal genitalia. Aregion mapped in distal chromosome 9p is thought to playa part in testicular formation. Gonadal dysgenesis inpatients with chromosome 9p deletion and sex reversalraises the quest for candidate factors of sex determination.The aim of this report was to demonstrate laboratoryworkups, clinical features and management toward asex-reversed patient.METHOD: We described 6-year-old patient with facialdysmorphism, severe mental disabilities, autisticbehavior, and ambiguous external genitalia withoutpalpable gonads. Spectral karyotyping and fluorescencein situ hybridization were applied to analyze the aberrantderivative chromosome found in the conventionalG-banding analysis. Array comparative genomichybridization was additionally performed to delineate thesize of deletion region.RESULTS: Chromosome study showed 46, XY, der(9)t(9;Y)(wcpY+), whereas the parental karyotypes werenormal. One copy deletion spanning about 12.8Mbcorresponded to chromosome 9p24.3p23. Exploratorylaparoscopy revealed bilateral hypoplastic testes in theinguinal area with normal regression of internal femalegenitalia. Along with clitoroplasty and vaginoplasty,bilateral gonadectomy was hence performed due toincreased risk of gonadoblastoma in the presence of Ychromosome. Histology showed tissues of epididymis,rete testis, and seminiferous tubules containing mostSertoli cells and few Leydig cells with remarkablydecreased numbers of spermatogonia but no evidence ofmalignancy.CONCLUSION: This present case was a de-novorearrangement of distal chromosome 9p and partial Ywith the breakpoint at band 9p23, and manifestedcharacteristic features and sex reversal. Cytogeneticanalysis was consistent with hypothetical critical locus9p24 harboring genes responsible of male sexdevelopment. Accumulation of clinical evidencecontributed to clarify the genetic mechanism of sexreversal with variable penetrance and expressivity. Theneed for internal gonads investigation in XY sex-reversed9p deletion syndrome was simultaneously advocated.[Keywords]9p deletion syndrome, XY sex reversal, gonadaldysgenesisKai-Chieh Chan 1 , Fu-Sung Lo 2 , Pa-Chun Wang 31 Division of Otology, Department of Otolaryngology Head & Neck Surgery,Chang Gung Memorial Hospital, Linkou, Taiwan; 2 Division of PediatricEndocrinology, Chang Gung Children’s Medical Center, Chang Gung MemorialHospital, Linkou, Taiwan.; 3 Department of Otolaryngology, Cathay GeneralHospital, Taipei, TaiwanOBJECTIVE: According to the Western studies, around 50%of the female Turner syndrome (TS) patients may suffer fromhearing impairment. However, there are few reports of theprevalence(s) of TS hearing loss in the Asia. The aims of thisstudy are to report otological features of TS patients in Taiwanand to analyze the associated factors for hearing loss.METHODS: Patients with TS were recruited for physical andhearing examinations in a consecutive manner. Data of thiscross-sectional study were retrospectively reviewed andanalyzed. Previous otologic history, otoscopic finding,karyotype, craniofacial deformity, bone age, and autoimmunehypothyroidism were assessed to explore their association withhearing loss.RESULTS: Forty-six TS patients (median age 16.8, range 4-33years) were recruited. Twenty one ears (23%) showed abnormalpure tone audiometry threshold. There were 8 (8.7%) conductivehearing loss (CHL) and 13 (14.1%) sensorineural hearing loss(SNHL), respectively. A total of 21 ears (23%) showed highfrequency (8 kHz) sensorineural hearing loss (HFSNHL).History of recurrent acute otitis media (AOM) was found in 21cases (45.7%). Otoscopy revealed that 13 ears (14.1%) withotitis media related appearance, including middle ear effusion (2ears), retracted tympanic membrane (10 ears), andpost-tympanomastoidectomy change in 1 ear. CHL (p=0.03) andHFSNHL (p=0.019) but not SNHL were more commonly seenin those with a history of recurrent AOM. There was nosignificant association between otological findings andkaryotypes (P>0.05) [table 1].CONCLUSION: The incidence of hearing impairment inTaiwanese TS patients is relatively low compared with previousreports. Recurrent AOM may predispose to sequelae to result inCHL and HFSNHL. We suggest that assiduous treatment forotitis media should be emphasized in TS patients to preventfrom hearing impairment.Table 1. Association between clinical features and hearing loss in TS patientsStatistical data: significant association CHL SNHL HFSNHLKaryotype No No NoRecurrent AOM Yes(P:0.03) No Yes(P:0.019)Otitis media related appearance Yes(P:0.001) No Yes(P:0001)High arched palate No No NoLow set ears No No NoMicrognathia No No NoAutoimmune hypothyoidism No No NoBone age> 14 y No No No_________________________________________CHL: conductive hearing loss, SNHL: sensorineural hearing loss, HFSNHL: highfrequency sensorineural hearing loss[Keyword] Turner syndrome, hearing loss317

P2-129 Medical Genetics/Metabolism P2-130 Medical Genetics/MetabolismCLINICAL ANALYSIS OF 6 CHILDRENWITH MUCOPOLYSACCHARIDOSIS OFHUWE1 MIGHT REGULATE THE SPINEFORMATION IN HIPPOCAMPUSCHILDRENYU Tao,Wu Kang-min, Du ZeliWest China Second University Hospital, Sichuan University, Sichuan610041, ChinaYu Tong 1 , Wenming Xu 2 , Meng Mao 3* , Dezhi Mu 4Laboratory of Early Development and Injuries, Center for Research of ChildDevelopment and Disease, West China Second University Hospital, SichuanUniversity 1 , Department of gynaecology and obstetrics, West China SecondUniversity Hospital, Sichuan University 2 , Department of pediatrics, West ChinaSecond University Hospital, Sichuan University, China 3,4 , Corresponding author*OBJECTIVE: To investigate the clinicalcharacteristics of mucopolysaccharide disease.METHODS: To analyze retrospectively the clinicalmanifestations, imaging features and laboratoryexamination of six cases with mucopolysaccharide.RESULTS: There were 6 cases withmucopolysaccharide, of which 4 are boys and 2females. The average age was 49.8 months and bothheight and weight of all cases were

P2-131 Medical Genetics/Metabolism P2-132 Medical Genetics/MetabolismTHE TITLE OF ABSTRACT FUNCTIONALINDEPENDENCE OF TAIWANESECHILDREN WITH VACTERL ASSOCIATIONHsin-Yi Lin 1 , Shuan-Pei Lin 1,2,3,4 , Hsiang-Yu Lin 1,2,3,5 ,Chyong-Hsin Hsu 1 , Jui-Hsing Chang 1 , Hsin-An Kao 1 ,Han-Yang Hung 1 , Chun-Chih Peng 1 , Huang-ChangLee 1 , Ming-Ren Chen 1,3 , Jeng-Daw Tsai 1,61 Department of Pediatrics, Mackay Memorial Hospital; 2 Department ofMedical Research, Mackay Memorial Hospital; 3 Mackay Medicine,Nursing and Management College; 4 Department of Infant and Child Care,National Taipei College of Nursing; 5 Institute of Clinical Medicine,National Yang-Ming University; 6 Department of Pediatrics, Taipei MedicalUniversity, Taipei, TaiwanOBJECTIVES: VACTERL association is a non-randomassociation of birth defects, which may include anomaliesof the vertebral column, limbs, kidneys, and heart; analatresia; tracheoesophageal fistula; and esophageal atresia.The presence of two or more of the defects establishes thediagnosis. An obvious concern for the parent of a childwith a congenital anomaly is how well that child willfunction in life. In some cases, parents might decide toabandon such a child. It is therefore important forpediatricians to provide accurate information onprognosis. The functional independent measure(WeeFIM) for children is a simple scale for assessingperformance in essential functional skills. In this study,we used the WeeFIM questionnaire to assess functionalindependence among Taiwanese children with VACTERLassociation.METHODS: We reviewed the medical records of all 47patients diagnosed with VACTERAL association atMackay Memorial Hospital, Taipei, Taiwan from June1994 to June 2009. We attempted to contact all theparents by phone. Of the 47 children, 25 parents withwhom we spoke on the phone agreed to participate byfilling out a questionnaire and returning it by mail in astamped, self-addressed envelope.and 23 were returned.Statistical analysis was performed using SSPS Statistics17.0 software.MAIN RESULTS: The total WeeFIM scores andsubscores for 3 domains (self-care, mobility, andcognition) correlated significantly with age (P

P2-133 Medical Genetics/Metabolism P2-134 Medical Genetics/MetabolismCLINICAL ASSESSMENT IN PATIENTSWITH LATER-ONSET FABRY MUTATIONCONGENITAL MALFORMATIONS IN JAPANESEPATIENTS WITH MENKES DISEASEIVS4+919G→AHsiang-Yu Lin a,b , Cheng-Hung Huang a,c , Hsiao-ChiYu c , Kah-Wai Chong a , Ju-Hui Hsu c , Pi-Chang Lee c ,Kang-Hsiang Cheng c , Chuan-Chi Chiang d , Huey-JaneHo e , Shih-Jen Chen f , Po-Kang Lin f , Dau-Ming Niu a,c*a Institute of Clinical Medicine, National Yang-Ming University, Taipei;b Department of Pediatrics, Mackay Memorial Hospital and MackayMedicine, Nursing and Management College, Taipei; c Department ofPediatrics, Taipei Veterans General Hospital, Taipei; d Neonatal ScreeningCenter, Chinese Foundation of Health, Taipei; e Section of NewbornScreening, Taipei Institute of Pathology, Taipei; f Department ofOphthalmology, Taipei Veterans General Hospital, Taipei, TaiwanOBJECTIVE: Newborn screening for Fabry disease inTaiwan revealed a high incidence of the later-onset GLAmutation IVS4+919G→A (~1 in 1,500-1,600 males). Weevaluated the clinical manifestations in adult subjectswith this mutation.METHODS: Echocardiography, urine examinations formicroalbumin and creatinine, and ophthalmologicexaminations were performed in 83 subjects with Fabrymutation IVS4+919G→A (19 males and 64 females;mean age, male 57.9 ± 6.4 years, female 39.4 ± 14.3years; age range, 19 years to 82 years).MAIN RESULTS: Plasma α-galactosidase A activitieswere analyzed in all 83 subjects, which showed 1.32 ±1.50 nmol/h/mL plasma for 19 males and 5.97 ± 1.96nmol/h/mL plasma for 64 females (normal range: 7.9 to16.9 nmol/h/mL plasma). Eighty-one subjects receivedechocardiographic examinations and showed that 16subjects (20%) had hypertrophic cardiomyopathy (HCM),including 12 males (63%) and 4 females (6%). Urineexaminations for microalbumin and creatinine wereperformed in 78 subjects, which revealed that 18 subjects(23%) had microalbuminuria, including 4 males (22%)and 14 females (23%). Forty-three subjects receivedophthalmologic examinations, and 40 subjects (93%)were found at least one item of ocular manifestations ofFabry disease, including 7 (16%) with conjunctival vesseltortuosities, 12 (28%) with cornea opacities (corneaverticillata), 19 (44%) with Fabry cataract, and 31 (72%)with retinal vessel tortuosities. We also subdivided thesesubjects into 2 groups according to gender and age (age≧40 years vs. age

P2-135 Medical Genetics/Metabolism P2-137 General PediatricsBIOTIN DEFICIENCY IN A GLYCOGENSTORAGE DISEASE TYPE 1B GIRL FEDONLY WITH GLYCOGEN STORAGEAVAILABILITY OF KEY PAEDIATRICESSENTIAL MEDICINES IN A RESOURCELIMITED COUNTRYDISEASE-RELATED FORMULA1) Kenji Ihara, 2) Kiyomi Abe, 2) Kou Hayakawa, 1) MikaMakimura, 1) Kanako Kojima-Ishii, 1) Toshiro Hara1) Department of Pediatrics, Graduate School of Medical Sciences, KyushuUniversity; 2) Division of Endocrinology and Metabolism, National Centerfor Child Health and Development, JapanBalasubramaniam Ramasamy 1 , Beneragama BVSH 2 , SriRanganathan S 11 Department of Pharmacology, Faculty of Medicine, University of Colombo, SriLanka; 2 Division of Medical Supplies and Technology, Ministry of Healthcare andNutrition, Sri LankaOBJECTIVE: We report a GSD type 1b girl with biotindeficiency caused by an exclusive glucose-containing GSDformula for years, presenting with the appearance of severe skinlesions, and diagnosed by urinary organic acid analysis by gaschromato-spectrometry (GC/MS), and blood acylcarnitineanalysis by tandem mass-spectrometry (MS/MS).CASE: The patient was born to non-consanguineous parents. Atthe age of two months, she was diagnosed as having GSD type Ibby the genetic analysis of G6PT gene. Dietary therapy with theGSD formula was started, however, she was frequentlyhospitalized with hypoglycemic attacks or severe infections. Herblood glucose level declined rapidly when she did not take asufficient amount of GSD formula, so nasogastric administrationof the formula was required. Her appetite gradually declinedrequiring nasogastric infusion both intermittently during daytimehours and continuously during night. Thin hair without apparentcause was noted during infancy. At age 3 years, she sufferedfrom a severe hypoglycemia and status epilepticus, and washospitalized. About one month later, a skin eruption graduallyappeared on her eyelids or angles of her mouth. Topical steroidtreatment along with antibacterial and antifungal cream was noteffective. Five months after the onset of skin symptoms, thediagnosis of biotin deficiency was suspected. Serum biotinconcentration, and biotinidase activity were measured. Herserum free biotin level was low - below the detectable level.Serum biotinidase activity was within normal level. Serumcarnitine levels were below the normal range. Urine GC/MSanalysis showed abnormal elevations of3-methylcrotonylglycine, 3-hydroxyisovaleric acid andmethylcitric acid. Blood MS/MS showed the remarkableelevations of C5-OH and C3/C2 ratio. Consequently, wediagnosed her as having biotin deficiency. Daily oralsupplementation of 0.3 mg of biotin and 300 mg of L-carnitinewas started, and dermatitis disappeared drastically in two weeksand her hair density increased gradually. Three months afterstarting biotin supplementation, her skin lesions had completelydisappeared, her hair growth was normal, and bloodacylcarnitines were fully normalized. We measured the totalbiotin levels in the GSD formula and found that total or freebiotin was not detectable in the formula.CONCLUSION: Patients with GSD are at-risk for biotindeficiency particularly if they receive GSD formula feedingwithout other food. Measurement of serum concentration of freebiotin, urinary organic acid analysis and blood acylcarnitineanalysis are helpful in diagnosing this complication.[Keywords]Glycogen storage disease; GSD formula; biotin deficiency; skin lesion;biotinidase; tandem mass-spectrometry; gas chromatography-mass spectrometryOBJECTIVES: To investigate the availability of key paediatricessential medicines used in the treatment of common childhooddiseases in a resource limited country.METHODS: This national survey assessed the availability of keypaediatric essential medicines using the World Health Organizationand Health Action International methodology. Data were collectedfrom a representative sample of 40 public hospitals (Out PatientsDepartment Pharmacies), 40 private pharmacies and 8 communitypharmacies run by the State. These medicine outlets were selectedusing a multistage clustered approach to represent the wholecountry and to represent different levels of public hospitals. At eachmedicine outlet, data on availability of 25 key paediatric essentialmedicines was collected on the survey day. Mean percentavailability across the country was calculated for the basket of 25medicines as well as for different therapeutic groups.RESULTS: The mean percent availability of the basket of 25medicines was 52% (range: 25-75%) in pubic hospitals compared to80% (range 56-96) in private pharmacies and 88% (range:76-100%) in State community pharmacies. In the public sector,Teaching/General hospitals (range: 48-74%, mean: 62%) hadslightly better availability than the District hospitals (range:25-75%, mean 54%), Peripheral units (range: 30-65%, mean 49%)and primary healthcare dispensaries (range: 25-65%, mean 45%).Eleven of the surveyed medicines were commonly usedanti-infectives and their availability was less in public hospitalscompared to that of private pharmacies and State communitypharmacies. For example amoxicillin suspension was available in45% of public hospitals compared to 100% of private and Statecommunity pharmacies. Similar poor availability in public hospitalswas observed for anti-asthma medicines (beclometasone MDI 50 %,vs.88% in private and state community pharmacies), carbamazepinesuspension (Nil vs. private 42.5%, State community pharmacies62.5%) ferrous suspension (12.5% vs. private 73%, Statecommunity pharmacies 75%), paracetamol syrup (65% vs. private95%, State community pharmacies 100%) domperidone syrup(47.5% vs. private 95%, State community pharmacies 100%), andibuprofen syrup (20% vs. private 90%, State community pharmacies62.5%). Availability in public hospitals as good as to that of privateand state community pharmacies was observed only for paracetamoltablet, oral rehydration salt and chlorphenamine syrupCONCLUSION: The availability of key paediatric essentialmedicines was poor in public hospitals. Better availability in retailpharmacies will drive parents to pay for the medicines or go withoutwhich deprives the children from the benefits of free healthcaresystem and access to effective and safe medicines.[Keywords]Essential medicine, paediatrics, children, access tomedicines321

P2-138 General Pediatrics P2-139 General PediatricsTHE IQS AND SELF-CONCEPTS OF THECHILDREN WITH KIDNEY DISEASESIRON DEFICIENCY ANEMIA IN OVERWEIGHTAND OBESE CHILDREN OF KHOY CITYCHIAN-FANG Grace Cherng 1 , Ching Yuang Lin 2Health Psychology, Chang Jung Christian University, Taiwan 1 , GraduateInstitute of Clinical Medical Science, China Medical University, Taiwan 2AIMS OF THE STUDY: Children with chronic illness maynot be inhibit only on their physical development. It is alsopossible affect negatively on their psychologicaldevelopment. The current study aimed to examine thedifferences between the children with kidney diseases andthe normal children on their psychological development interms of IQs and self-concepts. The results could provideuseful information for understanding the mental needs of thechildren with kidney diseases and critical factors influencingtheir social and psychological development. It is beneficialfor their further psychological and physical health care.METHODS: Two hundred and fifty-three children wereinvited to participate this study, 215 children were withkidney diseases (kidney group), and 38 were without kidneydiseases (non-kidney group). Among of them, 130 wereassessed individually by Wechsler Intelligence scales (IQs),and 235 were asked to answer the researcher-madeself-concepts questionnaire. In sum, there were 110 childrenwhom had been completely assessed by both IQs andself-concepts.MAIN RESULTS: Applying the factor analysis, theself-concepts had been clustered into 12 factors, includinghave company, good friend, shy self, bad-kid, rigid self,fat-and-short self image, bad habit, physical symptoms,optimist, talent, confidence, and not easy going. Applyingt-tests to compare the two groups of children’s scores on the12 factors, the results showed that their scores on 5 factorswere significantly statistically different. That is, children inthe non-kidney group had higher scores on the have company(t=2.57, p

P2-140 General Pediatrics P2-141 General PediatricsASSOCIATION OF D4 DOPAMINERECEPTOR GENE AND SEROTONINANALYSIS OF REASONS FOR CHILDREN WITHRECURRENT LEG PAINTRANSPORTER PROMOTERPOLYMORPHISMS WITH TEMPERAMENT Fan YangPediatric Department, West China 2nd University Hospital, Sichuan University,IN A LONGITUDINAL STUDYTaiwanGuan Hongyan, Dai Yaohua, Liu JirongCapital Institute of Pediatrics, Beijing, P.R. of ChinaOBJECTIVES: Temperament traits are often consideredto be genetic and to show relative stability over time.Effects of D4 dopamine receptor gene (DRD4) andserotonin transporter promoter polymorphisms(5-HTTLPR) length polymorphisms have been reportedon neonatal and infant temperament as well as adultpersonality traits. This study was designed to investigatethe influence of DRD4 and 5-HTTLPR genotype andtheir interaction on children temperament traits in alongitudinal study.METHODS: Child temperament was assessed at 8months in infancy (N=138), 4 years old in preschoolyears (N=98) and 7 years old in early school years(N=87) with the Chinese version of Carey temperamentquestionnaire. Genomic DNA was extracted from 87children in the early school years.MAIN RESULTS: After controlling for gender, infantswith long D4DR (DR4.4) alleles had significantly higherscores on Intensity (F= 4.098, P = 0.048) than infantswith short D4DR alleles (DR4.2). In contrast, children inpreschool with the short homozygous (s/s) 5-HTTLPRgenotype had higher scores on Activity (F= 4.008, P =0.027) than those with the l/s or l/l genotypes. There wasa significant effect on the interaction between 5-HTTLPRand DRD4 on temperament trait in infancy. In the absenceof the short allele of 5-HTTLPR, infants with the DRD4(DR4.4) had higher Intensity score compared to thosewith the DRD4 (DR4.2) (F= 8.622, P = 0.006). Moreover,the genetic effect accounting for “intensity” in infancywas enhanced from 6.7% to 30.1%.CONCLUSION: Regardless of the difference on theallele frequency of DRD4 and 5-HTTLPR betweeneastern and western people, this study corroborates in partprevious results of western studies on the link betweenthe DRD4 gene and human temperament. The significantinteraction of DRD4 and 5-HTTLPR genotype on“intensity” in infancy indicated the importance ofcombined analysis of polymorphisms in the dopaminergicand serotonergic systems in temperament traits, especiallyduring the early life of children. However, due to thelimited size of our sample, these results should beinterpreted with caution.OBJECTIVE: Recurrent leg pain in children is not anuncommon complains by children and parents. Toelvaluate the causes in children with recurrent leg pain.METHODS: 124 children aged 2-16 years old withrecurrent leg pain were recruited. Bone alkinephosphatase (BALP), Anti-streptolysin O (ASO),C-reaction protein (CRP) and cyclic citrullinated peptide(CCP) were measured and X-ray was taken as rotuine.Quantitative ultrasound (US) was used to measure bonespeed of sound (SOS). Children who were younger than 8years old only were measured at mid piece of left tibia,and children who were older than 8 years old weremeasured at mid piece of left tibia and radius.The SOSwas compared with the normal SOS of ordinary childrenaccording to same age and gender automatically thoughsoftware to get the Z . Kruskal-Wallis Test was used tocompare the Z among 124 patients.RESULTS: There were no positive findings in ASO,CRP, CCP and X-ray. 34.7% of the patients had elevatedBALP which is an indicator of biochemical osteomalacia.There was significant decrease in Tibial SOS (Z score-1.61) compared with healthy controls (Z score 0.11).CONCLUSIONS: Bone SOS was reduced in childrenwith recurrent le pain especially in painful tibial regionscompared to healthy controls. Unideal bone densitymaybe one of the reasons of recurrent leg pain.[Keywords] Children, bone density,quantitative ultrasound, recurrentleg pain[Keywords]Child Temperament Promoter Region of theSerotonin Transporter Gene (5-HTTLPR)Dopamine Receptor D4Gene (DRD4) Polymorphism323

P2-142 General Pediatrics P2-143 General PediatricsGROSS ANATOMY AND MICROSCOPY OFFILUM TERMINALESTAFF FEEDBACK ON APPLICATION OFHOSPITAL PLAY SPECIALIST IN JAPANG.Samson Sujit Kumar*, Geeta Chacko, K SrinivasaBabu, Santi*, K.Gajendra* BA Ramakrishna* andVedantam Rajshekhar*Narayana Medical College, Nellore, Andhra Pradesh, India & ChristianMedical College, Vellore, Tamilnadu, IndiaTakahiro Sugiura 1 , Chika Matudaira 2 , Takehiro Morisita 1 ,Shoko Sato 1 , Mirai Muto 1 , Masayo Ueda 1 , Yumiko Okubo 1 ,Keisuke Mizuno 1Department of Pediatrics (Neonatology), Shizuoka Saiseikai General Hospital 1 ,Department of Social Welfare, University of Shizuoka, Junior College, Japan 2OBJECTIVES: 1. Cadaver dissection to look forconnections between filum terminale and nerveroots of cauda equina. 2. Collect a sample of filumterminale from the distal segment in patientsundergoing sectioning of filum. 3. Histological andImmunohistochemical study of filum terminale andconnections.METHODOLOGY: Microsurgical dissection ofthe filum terminale was done on fresh stillborncadavers with no spinal dysraphism. Gross featuresof the filum were recorded. The connectionsbetween the filum and nerve roots, if present weresectioned and processed for histology. Fila (bothinterna and externa) were also sectioned and stainedfor routine histology, collagen, elastic fibres andimmunohistochemistry for neuronal and glialmarkers. A small bit of filum terminale, taken fromthe distal segment in patients undergoing sectioningof filum was also processed.RESULTS: Thirteen stillborn cadavers weredissected (M:7, F:6). Mean gestational age was 33 +2weeks. There were gross connections between thefilum and nerve roots in five cases, which showednerve fibers on histology andimmunohistochemistry. Filum contained islands ofcells positive for GFAP in 10/13 cases,synaptophysin in 3/13, s100 protein in 11/13 casesand nestin in 2/4 cases. Four of the five filumspecimens taken from patients showed nervebundles that were s100 positiveCONCLUSION: For the first time we havedemonstrated neural connections between filum andnerve roots. Filum terminale contains cells positivefor GFAP, synaptophysin, and s100 protein. Thisstudy also shows cells positive for primitive neuralstem cells (nestin positivity). These findings havesignificant implications in spinal cord injurystudies, neural stem cells studies and operation forvarious forms of spinal dysraphism. Also contraryto the literature, conus ends at higher vertebral bodythan L3 at birth.OBJECTIVE: Hospital Play Specialist (HPS) is one ofthe members of the pediatric multi-disciplinary team whoprovides play to support children through their experiencewith medicine and to understand their illness andprocedures in hospital. HPS Japan training project juststarted in 2007. The object of this study is to assess theeffects and problems of introducing this new profession,HPS into our pediatric team and making a workingstructure.METHODS: Staff feedback following application ofHPS after 6 months was obtained by anonymousquestionnaire.RESULTS: Twenty four (Pediatrician 6, Nurse 18)replies were obtained. After the application of HPS,66.7% indicated that they recognized the change inappearance of hospitalized children. All of them indicatethat the change was favorable. 55.1% indicated that`preparation skills’ that help children to understand theirillness and procedures were useful. 56.5% indicated that`destruction skills ‘that reduce children’s stress andanxiety against procedures were useful. 56.5% indicatedthat parent’s participation to their children’s procedureswere useful, while 13% indicated that was not useful atall.CONCLUSIONS: The application of HPS providedfavorable changes in appearance of hospitalized children.Since the recognition of effectiveness of HPS variedconsiderably from person to person,we have to makemore efforts to advocate a development of HPS in Japan.[Keywords] Hospital Play Specialist, Survey[Keywords] Filum terminale, nerve, marker, nesti n324

P2-144 General Pediatrics P2-145 General PediatricsPREVALENCE OF MICRONUTRIENTDEFICIENCIES AMONG WOMEN OFFACTORS ASSOCIATED WITH SHORT SLEEPDURATION AMONG SCHOOL-AGED CHILDRENREPRODUCTIVE AGE GROUP ANDCHILDREN UNDER 5 YEARS OF AGE, INMALDIVES: A CROSS SECTIONAL STUDYSajid Bashir Soofi 1 Imtiaz Hussain 1 Zulfiqar A.Bhutta 1Department of Pediatrics and Child Health Aga khan University KarachiPakistan, PakistanBACKGROUND AND OBJECTIVES: Micronutrientdeficiency is a major global health problem. It is estimatedthat more than 2 billion people worldwide are deficient inkey vitamins and minerals. To address these issues anational micronutrient survey was conducted by Aga khanUniversity in collaboration with Ministry of Health andUNICEF, Maldives. The survey provided a recentbenchmark on the National nutritional status of womenand children. The objectives of this were to review thecurrent nutritional situation, establish its trends, look forits associated factors that influence the nutritional statusand identify issues relating to policy and of programmaticimportance.METHODS: A cross sectional survey of Maldives wasconducted between November–December 2007. 30 by 14cluster technique was used with two-stage clustersampling by PPS. The sample was drawn from all parts ofthe country; all Islands, atolls and regions were included. Itcomprises of 2,520 households, and 180 clusters. Eachregion was divided into 30 clusters and 14 household werechosen systematically from each clusters. Biochemicalassessment included hemoglobin levels, stoolexamination, serum Ferritin, CRP, serum retinol, plasmazinc and urinary iodine.RESULTS: Micronutrient deficiencies were evidentamong women and children. The prevalence of moderateto severe anemia was 15.4%and the proportion contributedby iron deficiency was 13.9%.Serum Ferritin deficiencywas 38.9%, zinc deficiency 73.2% and deficiency of lowto moderate serum retinol was around 44%. Urinary Iodinewas found to be more deficient among women, comparedto children (27% versus 19%).CONCLUSION: Micronutrient deficiencies appear to bea public health issue with surprisingly high levels of irondeficiency anemia, sub clinical vitamin A and zincdeficiency. Iodine deficiency appears to be an issue in aproportion of the population despite widespreadavailability and use of iodized salt. Recommendationshave been suggested to improve the nutritional healthstatus of the community and tackle ensuing challengeswhich may stem from the existing nutritional policies andprograms.[Keywords] Micronutrient, pregnant women and children underfive, MaldivesXiaoming Shen, MD, PhD 3 , Shenghui Li, MD,PhD 1,2From the 1 Shanghai Xin Hua Hospital affiliated with Shanghai Jiaotong UniversitySchool of Medicine, Shanghai, People’s Republic of China; 2 School of PublicHealth affiliated with Shanghai Jiaotong University School of Medicine, Shanghai,People’s Republic of China; 3 Shanghai Key Laboratory of Children’sEnvironmental Health, Shanghai, People’s Republic of ChinaOBJECTIVES: To examine risk factors regarding shortsleep duration among Chinese school-aged children.METHODS: A random sample of 22018 children aged5.08-14.92 years participated in a cross-sectional survey,which was conducted in 8 cities of China in 2005. Aparent-administered questionnaire was used to collectinformation on children's sleep duration and possiblerelated factors from eight domains. Short sleep durationwas defined as total sleep duration < 9 hours per day.RESULTS: 28.9% of the sampled children slept < 9hours per day. The multivariate logistic regressionidentified that, after controlling for age and gender, thosefactors associated with short sleep duration: moretelevision viewing during weekdays, more frequentcomputer/internet using, earlier time of leaving home forschool in the morning, more time on homework duringweekdays and weekends, poor bedtime hygiene (eg,having drinks with caffeine after 6:00PM, doing excitingactivities during bedtime, and irregular bedtime); andshorter sleep duration of parents.CONCLUSIONS: Factors associated with sleep durationcovered multidimensional domains among school-agedchildren. Compared to socioeconomic status, sleepenvironments, and chronic health problems, schoolschedules, lifestyle patterns, and parents’ sleep habits hadgreater impact on children’s sleep duration, indicating theexisting chronic sleep loss in school children could be, atleast partly, intervened by changes in school schedules,routine behavioral modification, and parents’ sleep habitsregulation.[Keywords] children; sleep duration; risk factors, China325

P2-146 General Pediatrics P2-147 General PediatricsEFFECTS OF PHTHALATES EXPOSURE ONTAIWANESE MOTHER AND INFANT:INVESTIGATION ON SERUMCONCENTRATION AND CORRELATIONROLE OF INTEGRATED WELL CHILD CAREPROGRAM IN IDENTIFICATION OF HEALTHRISK FACTORS IN CHILDREN UNDER EIGHTYEARS OLDCheng-Hsien TsaiPediatrics, National Taiwan University Hospital Yunlin Branch, TaiwanPhthalates are synthetic compounds widely used asplasticizers, also called environmental hormones,are reproductive and developmental toxicants inanimal models. Phthalate esters include di-nethylphthalate (DMP), di-n-butyl phthalate (DBP),i-(2-ethylhexyl) phthalate (DEHP) and di-ethylphthalate (DEP) and BBP. Diet is believed to be themain source of DEHP and other phthalates in thegeneral population, and the fetus’s source was fromthe pregnant mother.The aim of this study is to collect serum samplesfrom pregnant women and their infants to measurefive phthalate monoesters using liquidchromatography/tandem mass spectrometry(LC/MS-MS) and the newborns' birth weight,gestational age, and demographic data werecollected. We identified 230 serum phthalate levelsfrom115 pairs of mother and infant and foundsignificant correlation of DEHP between motherand the infant, but not related to maternalnationality, nor gestational age[Keywords]phthalates exposure, serum levels, mother and infant,DEHP, LC/MS-MSMirhadian,Leila. *; Asiri, Shahla.*; Pasha,Afsaneh.*;Lakeh,Mokhtari.Nasrin*Faculty members of Guilan university of medical sciences, Iran*INTRODUCTION: Integrated well child care program isone of the most important changes in children growthmonitoring. This program enables the health workers tovisit children and their families routinely and provides astandard system of well children screening.OBJECTIVE: The aim of this study was to determinehealth risk factors of children under eight years old.METHODS: This is a descriptive cross- sectional studywhich obtained data from 1874 children's health records.Samples were chosen through cluster sampling whichincluded eight health centers in Rasht, Iran. Health riskfactors assessed included :potential bacterial infection ,icter , malnutrition or severe low weight , dysfunctionalweight growth, severe low height, dysfunctional heightgrowth, abnormal and unsatisfactory head circumference,nutritional problems, dental problems, developmentalproblems, incomplete vaccination, incomplete use ofcomplements and abnormal laboratory tests. Alsodemographic characteristics such as sex, birth weight andage of at health care received were assessed.RESULTS: Findings showed that most common healthrisk factors respectively included: weight growthproblems 56.8%, nutritional problems 22.3% andunsatisfactory head circumference 10.4%. There was asignificant relationship between dysfunctional weightgrowth, nutritional problems, unsatisfactory headcircumference and birth weight, age of health carereceived.CONCLUSION: This study indicated that dysfunctionalweight growth makes up for more than half of health riskfactors. The most of weigh growth problems were seen atage of seventh month (94.2%) that may be related tobeginning of beikost. Therefore it can be resulted thatintegrated well child care program well identified healthrisk factors in children under eight years old. Also it canprovide health programmers with data necessary indetermining family educational needs in order to promotechildren health.[Keywords]Well child care – Integrated care – Health risk factors –Children under eight years old326

P2-148 General Pediatrics P2-149 General PediatricsURINARY TRACT INFECTION PRESENTEDAS HYPERBILIRUBINEMIA IN NEWBORNINFANTSHung-Ta Chen, Mei-Jy Jeng, Chia-Feng Yang ,Pei-Chen Tsao, Yu-Sheng Lee, Wen-Jue Soong,Shu-Jen Chen, Ran-Bin TangDepartment of Pediatrics, Children’s Medical Center, Taipei VeteransGeneral Hospital; and Institute of Emergency and Critical Care Medicine,School of Medicine, National Yang-Ming University, Taipei, TaiwanBACKGROUND: Hyperbilirubinemia is a commonreason of admission in newborn infants. Previous studieshave noted that jaundice may be one of the initialsymptoms related to urinary tract infection (UTI).OBJECTIVE: The aim of this study was to evaluate therelated factors of newborn infants with the initialpresentation of hyperbilirubinemia and final diagnosis ofUTI in a tertiary teaching hospital.METHODS: We prospectively investigated all of theneonatal patients admitted with the initial diagnosis ofhyperbilirubinemia between January 2008 and December2008. Data collection is based on laboratory data ofenrolled infants and maternal conditions.RESULTS: A total of 343 neonatal patients admittedwith the diagnosis of hyperbilirubinemia wereinvestigated. Of them, one hundred and twenty-eightcases were excluded because of lack of urinary analysis.Among the enrolled 215 patients with hyperbilirubinemia,forty-three patients had a positive findings of pyouria(white cell counts higher than 5 cell/high power field),twelve patients has a positive urine culture. Pyuria withpositive urinary culture was diagnosed as UTI in 7 cases(3.26%). The isolated organisms included Escherichiacoli (in 3 patients), Klebsiella oxytoxa, Enterobactercloacae, and Coagulase Negative Staphylococcus species.The mean serum bilirubin level of neonates with UTI was16.5 mg/dl which was significantly higher than otherneonates (15.2 mg/dl) without the diagnosis of UTI. Thehighest bilirubin in one case with UTI was 21 mg/dl. Inaddition, Two patients (29%) with UTI had a delayedonset of jaundice (over 7 days of age). The admission agein neonates with UTI was 6.1 days old, but that was notsignificantly different from those without UTI (4.4 daysold). In addition, the mean birth body weight of neonateswith UTI (3272g) was not significantly different from theneonates without UTI (3159g). There was also nosignificant difference in maternal conditions between thetwo groups of infants.CONCLUSIONS: The incidence of UTI in the admittednewborn infants with hyperbilirubinemia was as high as3.26%. Admitted neonates of hyperbilirubinemia mayneed the consideration of performing urinalysis ifpresence of delayed onset of hyperbilirubinemia or ahigher level of serum bilirubin. Therefore, urinalysis forsome jaundiced newborn infants is important to excludethe possibility of UTI.ADOLESCENTS 13-17 YEAR OLD FROMSELECTED SCHOOLS IN METRO MANILA ANDTHEIR GUARDIANS’ CHOICE OFADOLESCENTSEX EDUCATION PROVIDERSta. Ines M.D., Dolores Raquel T.Cardinal Santos Medical Center, San Juan City, PhilippinesOBJECTIVES: This study aims to determine the preferredprovider of sexual education to adolescents according tothe respondents and the association of source ofknowledge with their demographic characteristics.METHODS: This is a cross-sectional school-baseddescriptive study. Randomized high school studentsaged 13 to 17 years enrolled in selected schools in MetroManila on January 2009 and their guardians wereincluded in the study. Informed consent, parental consentand assent/dissent forms were provided and thepreviously validated questionnaires were thenadministered. Descriptive analyses on the quantitativedata and Chi-square test for association were performed.P-value

P2-150 General Pediatrics P2-151 General PediatricsASSESSMENT OF USE OF IODIZED SALT ATHOUSEHOLD LEVEL AND IODINEDEFICIENCY STATUS AMONG CHILDRENUNDER FIVE YEARS AND WOMEN OFTHE EFFECT OF ZINC AS AN ADJUNCTTHERAPY IN THE TREATMENT OF SEVEREPNEUMONIA IN CHILDREN LESS THAN 2YEARS OLD: AN UPDATED META-ANALYSISREPRODUCTIVE AGE GROUP, RESIDINGIN THE PROVINCE OF SINDH, PAKISTAN Mitzi Trinidad AseronPediatrics, De La Salle University Alumni Association, PhilippinesImtiaz Hussain 1 Sajid Bashir Soofi 1 Gulnawaz Khan 1Zulfiqar A. Bhutta 11-Department of Pediatrics and Child Health Aga khan UniversityKarachi Pakistan, PakistanBACKGROUND AND OBJECTIVES: Iodine deficiencydisorders are recognized as the most preventable cause ofmental health disorders worldwide, including Pakistan whereover 70% of the population is estimated to be at risk of Iodinedeficiency disorders. This reported level of iodine deficiencyhas tremendous developmental costs on the country. Theproblem of Iodine deficiency disorders persists even inpresence of salt fortified with iodine. The objectives of thisstudy were to determine the prevalence of iodine deficiency inchildren (0–5 years) and women of reproductive age, and toassess the use of iodized salt at household level.METHODS: A cross sectional survey was conducted inurban and rural settings of Sindh Province, Pakistan fromJanuary 2007 to March 2007. In each household a knowledge,attitudes and practices survey was conducted and a salt samplewas tested for iodine fortification. A Urinary iodine Excretiontest was employed on the study population to estimate theprevalence of iodine deficiency in this survey using thecriteria recommended by WHO/UNICEF/ICCIDD (2001).RESULTS: The results of the present study revealed thatmajority of the respondents reported to be familiar with termof iodine and iodized salt and the availability of salt in nearbymarket. Surprisingly locations wise the orientation level aboutiodine differed from 24% to 74.2% rural to urban areasaccordingly. Iodine test kits were used to check the iodinecontent of household salt, which shows that the coverage ofiodized salt were low in both rural and urban sites, about 39%respondents were reported to be using of iodized salt athousehold level but iodized salt were seen (by labelingobservation) at 21% households at the time of household visitfor interviews. The results for both reported use and observeduse were differed location wise from 57% to 19% and 30.2%to 11.2% respectively in urban and rural. Results of urinaryiodine analysis shows that; about 34% reproductive agewomen were found to be iodine deficient; 14.6% wereseverely iodine deficient and 19.2% moderately deficient.Among children under 5 years of age the prevalence of iodinedeficiency were 22%; 12.2% were severely and 9.7%moderately iodine deficient. This high prevalence (52%) ofiodine deficiency among children belonged to rural areaswhile a high prevalence was observed among women ofreproductive age in urban areas.CONCLUSION: Iodine deficiency still persists amongchildren under 5 years of age and women of the reproductiveage. Strict measures should be employed to counteract thisdeficiency, ensure use of iodine fortified salt, and consequentprevention for mental health defects.[Keywords] Iodine deficiency, Salt use, PakistanBACKGROUND: Reducing severity and mortality frompneumonia in children is the central objective of the World HealthOrganization’s program for the Control of Acute RespiratoryInfection. It was postulated that zinc, an acute phase reactant, iseffective as an adjunct treatment for pneumonia, shortening durationof severe pneumonia and time in the hospital.OBJECTIVES: To assess the effectiveness of zinc given asadjuvant therapy in severe pneumonia in children aged 2 months to24 months.SEARCH STRATEGY: We searched the Cochrane CentralRegister of Controlled Trials (The Cochrane Library, Issue 4, 2009),PubMed and PCHRD Library (Philippine Council for HealthResearch and Development Library) and reference lists of identifiedarticles. Articles published in the Philippine Journal of Pediatricsfrom 1996 to 2008 and proceedings of major conferences werehand-searched. Different training institutions and pharmaceuticalcompanies were also inquired regarding unpublished and ongoingstudies on zinc and its effect on pneumonia.SELECTION CRITERIA: Randomized controlled trials of zincadministration with placebo or control given as adjunct toantimicrobial therapy to children aged 2 to 24 months diagnosedwith severe pneumonia and outcome measures were reduction induration of symptoms (tachypnea, chest Indrawing, fever,hypoxemia and appetite), length of hospital stay and treatmentfailure.DATA COLLECTION: Three reviewers independently assessedtrial quality and extracted data. Study authors were contacted foradditional information.MAIN RESULTS: Four trials involving 599 people were included.Compared to placebo/control, zinc therapy significantly shortenedduration of severe pneumonia symptoms such as: tachypnea (10.41hrs, 95% CI -20.16 to -0.66, four studies, 529 children); fever(21.23 hrs, 95% CI -39.83, -2.62, three studies, 203 children), andloss of appetite (11.49 hrs, 95% CI -21.09 to -1.89, four studies, 340children). Treatment with zinc along with standard antimicrobialtherapy for severe pneumonia is associated with overall reduction inhospital stay (8.34 hrs, 95% CI -16.68 to -0.01, three studies, 523children) and treatment failure (RR 0.21, 95% CI 0.07 to 0.63, threestudies, 300 children).REVIEWERS’ CONCLUSIONS: This new review supports theuse of zinc as adjunct to the standard antimicrobial regimen forsevere pneumonia in children. Zinc therapy significantly improvedthe clinical outcome of children with severe pneumonia by reducingthe duration of symptoms, length of hospital stay and treatmentfailure.[Keywords]Zinc, severe pneumonia, zinc therapy328

P2-152 General Pediatrics P2-153 General PediatricsTOILET TRAINING OF NORMAL THAIINFANTSBenjasuwantep B 1 , Ruangdaranon N. 2Department of Pediatrics, Srinakharinwirot University 1 , Department ofPediatrics, Mahidol University, Thailand 2EVALUATION OF THE EFFECTIVENESS OFZINC FORMULATIONS IN THE TREATMENT OFACUTE DIARRHEA IN AN EPIDEMIC SITUATIONIN PAKISTANMuhammad Atif Habib 1 , Sajid Bashir Soofi 1 , Zulfiqar ABhutta 1Institution1: Department of Paediatrics and Child Health Aga Khan University,PakistanOBJECTIVE: To study the age of which toilettraining was started and succeeded and associatedfactors in normal Thai infants.METHODS: The parents of fifty infants in aresearch titled with “Bowel movements of normalThai infants” were interviewed about (1) age thattoilet training was started (2) the process of toilettraining and (3) age that each infant wassuccessfully toilet trained. The data waslongitudinally collected from July 2003 to May2005. The definition of successful toilet training formost parents or caregivers in Thailand was theinfants’ ability to avoid bowel accidents most of thetimes.MAIN RESULTS: Of which 50 infants, 47 infantshad data of the date of beginning toilet training.10.6% (5 of 47) of them were started toilet trainingat 4 months of age and 80.9% (38 of 47) by 12months. 73.7% of infants in this study were initiallytrained while they had urging. The caretakersobserved infants’ elimination signals and assistedthem with defecation. 45 infants had complete dataof succeeding toilet training. 48.9% (22/45)successfully toilet trained by 12 months and most ofthem (72.72%) succeeded within one month. Theinfants, who were not the first child and were takencare by a well educated mother, were found to starttoilet training lately. There was no statisticalsignificant (p=0.15) between regular bowelmovement and toilet training. However, we foundthat most infants (35/38, 92.11%) had regular bowelmovement before they were started toilet training.CONCLUSIONS: The youngest age of normalThai infants started to be toilet trained was 4months old. About 50% of the infants had fewbowel accidents by 12 months and were defined assuccessful toilet training by Thai parents. Welleducated mother and a later order of birth in afamily of infants were associated with late toilettraining.OBJECTIVE: To evaluate the effectiveness of differentzinc formulations in the treatment of acute diarrhoeaamong young children in the earthquake affected regionof Pakistan in an emergency epidemic situation.METHODS: This was an open randomized trial. Asample size of 200 patients was calculated, the patientswere allocated either of the 2 groups i.e. A (Zinc inTablets form) and B (Zinc in Suspension form), (bothgroups received Standard diarrhoea treatment, in addition3RDA of elemental zinc in the form of tablets andsuspension respectively) by using simple blockrandomization. Recruitment and follow up processes wereexecuted in the camp hospital. Data analysis was done byusing SPSS 14.RESULTS: Most children recovered from the illnesswithin three days after presentation. Significant P-Valueswere established among zinc use and reduction in numberstools on Day 2 and Day 3, with comparatively betteroutcome in group B. Furthermore about 90% of thepatients recovered from diarrhoea within three days ofpresentation 82% from Group A and 96% from group B.On comparison of group A and B the recovery rates forgroup B was found to be significantly higher.CONCLUSION: Our data confirms the notion that zincreduced the duration and frequency of diarrhoea inchildren even in emergency and crisis settings. The studyexplores the use of zinc in different formulations anddelivery mechanism and supports the use of zinc insuspension form and also provides a lead for furtherpopulation based studies.[Keywords]Zinc, Acute diarrhoea, Emergency settings.[Keywords] toilet training, infant329

P2-154 General Pediatrics P2-155 General PediatricsMICRONUTRIENT STATUS OF PREGNANTMOTHERS AND INFANTS IN RURAL SINDHINFLUENCES ON SMOKING AMONG URBANADOLESCENTS : FAMILY OR FRIENDS(PAKISTAN)Imtiaz Hussain 1 Sajid Bashir Soofi 1 Zulfiqar A.Bhutta 1Department of Pediatrics and Child Health Aga khan University KarachiPakistan, PakistanCheah CW 1 , See CS 1 , Joven Mailvaganam 1 , Tay SY 1 , YeoSC 1 and Ling CS 1International Medical University, MalaysiaBACKGROUND AND OBJECTIVES: Deficiencyof micro-nutrients such as vitamin A, iron and iodineaffects more than 2 billion people globally. Accordingto the National Nutrition Survey of Pakistan (2001)these deficiencies are widespread in under 5 childrenin Pakistan. However, the survey did not providerepresentative information on lactating mothers andchildren during the complementary feeding (6-18months). The Aga Khan University (AKU) incollaboration with the United Nations World FoodProgramme and the Micronutrient Initiative undertooka pilot nutrition intervention project for rural mothersand infants to promote use of high protein blendedfood fortified with vitamins and minerals. We presenthere the results from the baseline survey on the statusof micronutrient levels in pregnant women and 6 – 18months old infants.METHODS: The survey was conducted in sixselected public health facilities and catchment villagesof district Matiari of Sindh Province (population 1.2million). Participants [pregnant women (PW) andinfants] were recruited during visits for immunizationor antenatal care and through community stratifiedsampling. Blood samples were obtained throughstandardized methods for estimating hemoglobin (Hb),serum ferritin and serum retinol. Urine samples werealso obtained for estimating iodine status.RESULTS: A representative sample of 484 PW and105 infants were screened. Of these 6.5% PW wereseverely anemic (Hb

P2-156 Neonatology P2-157 NeonatologyINTEGRIN Α V Β8 ENHANCESNEUROPROTECTIVE ROLE OFASTROCYTES IN NEONATALRIBOZYMES TARGETED TO HGTD-P INHIBITEDNEURONAL APOPTOSIS IN NEONATAL RATBRAIN WITH HYPOXIA-ISCHEMIAHYPOXIC-ISCHEMIC BRAIN INJURYDezhi Mu 1,2* , Jinhui Li 1 , Yi Qu 1 , Meng Mao 11 Department of Pediatrics, West China Second University Hospital,Sichuan University, Chengdu 610041, China; 2 Department of Neurologyand Pediatrics , University of California, San Francisco, San Francisco,CA 94143, USAIntegrin α v β 8 plays an important role in cerebralvascular development. It has been proven that α v β 8is a key factor for transforming growth factor-β1(TGF-β1) activation in epithelial cells. However, itis not clear whether α v β 8 can activate TGF-β1 andplay a role in protection during neonatalhypoxic-ischemic (HI) brain injury. In this study,we investigated the relationship between α v β 8 andTGF-β1 activation, and thus the effects of TGF-β1activation in the protection of neurons after HI.Astrocytes and neurons from rat brains werecultured and then subjected to oxygen-glucosedeprivation to generate HI model in vitro. β 8expression was determined usingimmunocytochemistry, Western blot and reversetranscriptase polymerase chain reaction. TGF-β1activation was determined by TGF-β bioassay in atested cell (astrocyte) and reporter cell co-culturesystem. Pro-apoptotic protein, cleaved caspase-3,and anti-apoptotic protein, P53, Bcl-2 and Bcl-xL,were detected using Western blot. Cellular apoptosiswas detected with TUNEL and Flow Cytometry. Wefound that β 8 expression was stronger in astrocytesthan that in neurons in normoxia. HI resulted in arapid and persistent increase of β 8 expression inastrocytes, but a slight and transient increase inneurons. Astrocytes β 8 could induce TGF-β1activation leading to upregulation of P53, Bcl-2 andBcl-xL, and thus attenuated neuronal apoptosis. Ourfindings suggest that β 8 may protect neurons againstapoptosis after HI through TGF-β1 signalingpathway, which may be beneficial for the treatmentof hypoxic-ischemic brain injury.[Keywords]integrin, transforming growth factor-β, astrocyte,hypoxia-ischemia, neuronal apoptosisDezhi Mu, Yi Qu, Meng Mao, Fengyan Zhao, Lin ZhangDepartment of Pediatrics, West China Second University Hospital,Sichuan University, Chengdu 610041, China.OBJECTIVES: Human growth andtransformation-dependent protein (HGTD-P) is a newpro-apoptotic protein and an effector of cell death inducedby hypoxia-ischemia (HI). The function of HGTD-P hasbeen investigated in human prostate cancer cells andmouse neurons cultured in vitro. However, whetherHGTD-P is involved in regulating the apoptosis of ratneurons is not clear, and the relevance of HGTD-P in HIanimal models is still unknown. Therefore, in the presentstudy, we tried to elucidate the role that HGTD-P plays inapoptosis of rat neurons subjected to HI, both in cultureand in the developing rat brain in vivo.METHODS: Samples from primary cultured neurons andpostnatal day 10 rat brains with HI were collected.RT-PCR, Western blotting, and immunocytochemistrywere used to detect the expression and distribution of ratHGTD-P,Caspase 3 and AIF. MTT assay, DAPI, TUNELand flowcytometry were used to detect cell viability andapoptosis. Ribozymes targeted to HGTD-P were designedand their effect were detected.RESULTS: We found that HI up-regulated the mRNAand protein levels of HGTD-P in rat neurons in vitro andin vivo. Ribozymes targeted to HGTD-P inhibited theexpression of HGTD-P, thus rescuing cell viability andattenuating cell apoptosis. In addition, we found thatHGTD-P played its pro-apoptotic role by activatingcaspase 3 and inducing the translocation of apoptosisinducingfactor to nuclear.CONCLUSIONS: Our findings show that HGTD-Pplays a pro-apoptotic role in the developing rat brain afterHI and that it may be a potential target in treatingHI-induced brain damage.[Keywords]HGTD-P, Ribozymes, neonatal, brain,apoptosis331

P2-158 Neonatology P2-159 NeonatologyEFFECTS OF STAT3 EXPRESSION ONNEURONAL APOPTOSIS DURINGHYPOXIA-ISCHEMIA BRAIN DAMAGEMU Dezhi, DENG Rui, ZHAO Feng-yan, TANGbin-zhi, QU YiDepartment of Pediatrics, West China Second University Hospital ,Sichuan University, Chengdu Sichuan , 610041, ChinaOBJECTIVE: To investigate the effects of signaltransducer and activator of transcription 3 (STAT3)expression on neuronal apoptosis and vascularendothelial growth factor (VEGF) expression underhypoxia ischemia condition.METHODS: Hypoxia-ischemia brain injury wasgenerated using 7-d-old SD rats according toRice-Vannucci model. 7-d-old SD rats were dividedinto normal group, sham control group andhypoxia-ischemia (HI) group, brain tissues werecollected at 4、6、8、12 and 24h after treatment. Thepathological changes was observed byhematoxylin-eosin (HE) staining;immunohistochemistry and western blot were usedto detect the expression of STAT3, phosphorylatedSTAT3 (p-STAT3) and VEGF; cell apoptosis wasdetected by TdT-mediated dUTP-biotin nick endlabelling (TUNEL) staining.RESULTS: In the experimental group, Thep-STAT3 protein was significantly increased at 6hours, and began to decrease at 24 hours. However,the expression of p-STAT3 protein in the normalcontrol group was extremely low at each time point.The expression of total STAT3 protein in theexperimental group was not time-dependent, andthere was no significant difference observed whencompared with that of the normal control group. Inthe experimental group, the VEGF expressionreached a peak at 8 hours after operation, and beganto decrease at 24 hours. TUNEL staining showedthat the positive cells were significantly increasedafter HI, with a peak at 24 hour, and the positivecells were significantly more than those of controlgroup.CONCLUSION: The p-STAT3 protein expressionwas significantly increased in neurons after HI, andits peak time was earlier than that of VEGFexpression and neuronal apoptosis, suggesting thatthe activation of STAT3 may induce VEGFexpression, promote vascular repairing, and inhibitneuronal apoptosis after HI.[Keywords]STAT3,Neuronal Apoptosis ,Hypoxia-ischemia BrainDamageEFFICACY OF BRAIN HYPOTHERMIA FORHYPOXIC-ISCHEMIC ENCEPHALOPATHY INNEONATES ACCORDING TO THE UNDERLYINGCAUSESShinkai INOUE, Makoto TSUTSUMI, RyutarouKINOSHIYA, Eiji OHTA, Toshiko MORI, MasatoshiNAKAMURA, Shinichi HIROSEDivision of Neonatology, Center for Maternal Fetal and Neonatal Medicine,Fukuoka University Hospital, JapanOBJECTIVES: To determine the effects of brain hypothermia(BHT) on neonates with hypoxic-ischemic encephalopathy(HIE) according to the underlying causes.METHODS: We reviewed the medical records of 11 patientswho had been given BHT from 2004 to 2009. The cases weredivided into two groups with different underlying causes: theabruption of the placenta group (AP: 6 cases) and the nonreassuring fetal status group (NRFS: n=5). The deliveries for 8cases (5 in AP and 3 in NRFS) were performed by emergencyCaesarean section. The average Apgar scores at 5 min after birthwere 3.3 and 5.0 in AP and NRFS, respectively. There was notmuch difference in blood gases on admission or birth weightsbetween two groups. There were 8 cases (3 in AP and 5 inNRFS) of moderate HIE and 3 of severe HIE (3 in AP). Thus,all of NRFS had moderate HIE whereas all severe HIE wereseen in AP.MAIN RESULTS: BHT began within 3 hours after birth for allthe cases except one AP case who subsequently died of severecomplications of BHT. This was the only death case in thisstudy. Such severe complications occurred in 2 AP cases (1moderate and 1 severe HIE) and were pulmonary hemorrhageand disseminated intravascular coagulation. The case withsevere HIE survived while the case with moderate HIE diedinstead. We hence considered that the death was attributed to theBHT complications. Brain MRI was done before discharging forthe 10 survivals and revealed brain abnormalities in 3 of them;all 3 were AP cases and 2 of them with severe HIE. Theabnormal findings included multiple cystic encephalomalacia in3 patients and intracranial hemorrhage in 1. There were 6 caseswith normal development, 4 with sequelae and 1 death. All thesevere HIE cases in AP had sequelae; there were 2 cases ofsevere cerebral palsy and 1 of mild mental retardation. Thus,only 1 out of 6 AP cases showed a normal brain MRI and nomental retardation. In contrast, only 1 out of 5 NRFS casesshowed mental retardation.CONCLUSION: BHT seemed effective for HIE in severe aswell as moderate cases. Upon application of BHT, however, it isnecessary to take into account the underlying causes of HIE, asthere are significant differences in short-term prognoses for APand NRFS.[Keywords] neonate, brain hypothermia (BHT), hypoxic-ischemicencephalopathy (HIE) Abruption of the placenta, non reassuring fetalstatus (NRFS)332

P2-160 Neonatology P2-161 NeonatologyASSESSMENT OF GROSS MOTORDEVELOPMENT PATTERN IN VERY LOWBIRTH WEIGHT INFANT USING ALBERTAINFANT MOTOR SCALEACCUMULATION OF TRIOSEPHOSPHATEISOMERASE, WITH SEQUENCE HOMOLOGY TOBETA AMYLOID PEPTIDES, IN VESSEL WALLSOF THE NEWBORN PIGLET HIPPOCAMPUSLIN-YU WANG, M.D. 1 , SHAN-TAIR WANG, PhD. 2 ,CHAO-CHING HUANG, M.D. 3Division of Neonatology, Department of Pediatrics, Chei-Mei MedicalCenter; Institute of Clinical Medicine, College of Medicine, NationalCheng Kung University, Tainan, Taiwan 1 ; Department of Public Health,College of Medicine, National Cheng Kung University, Tainan, Taiwan 2 ;Division of Neurology, Department of Pediatrics, National Cheng KungUniversity Hospital: Institute of Clinical Medicine, College of Medicine,National Cheng Kung University, Tainan, Taiwan 3This study examined whether the gross motordevelopmental pattern in the first 18 months of lifeassessed by Alberta Infant Motor Scale (AIMS) couldidentify very low-birth-weight (VLBW) infants withcystic periventricular leukomalacia (PVL).A longitudinal follow-up assessment of the grossmotor developmental pattern by AIMS wereperformed at age 6, 12 and 18 months for 35 VLBWinfants with cystic PVL (Group I), 70 infants withoutcystic PVL (Group II), and 76 term infants (Group III).We found that the Group I infants had the lowestmean supine, prone, sitting and standing scores in thefirst 18 months old compared with the Group II andIII infants. The 2 VLBW groups had significantdifferences in supine, prone and sitting scores but notin standing scores at age 6 months. The differences insupine, prone and sitting scores between the 2 VLBWgroups persisted at age one year. Compared withGroup III infants, the Group II infants showedcomparable gross motor maturation in prone, supineand sitting scores, with the exception of standingscore. The standing score of the Group II infantsimproved and was comparable to that of the GroupIII infants at age 18 months. Group II infants alsoperformed poorly in supine, prone, sitting and standingscores compared with Group III infants at age 6months, but they later caught up with the developmentof Group III infants at age 12 months, with theexception of standing scores till age 18 months.Takashi Kusaka, Masaaki Ueno, Saneyuki Yasuda, KosukeKoyano, Shinji Nakamura, Kenichi Isobe, Susumu ItohFaculty of Medicine, Maternal and Perinatal Center, Kagawa University, JapanWe investigated whether beta -amyloid (A beta)-likeimmunoreactivity was seen in the brains of newbornpiglets. The immunoreactivity for A beta (1-42) and Abeta (1-40) proteins, but not A beta precursor protein, waspresent in CD68-positive perivascular cells of thehippocampus and in parts of the meninges. It wascolocalized with immunoreactivity for receptor foradvanced glycation end product and tumor necrosisfactor-alpha. The protein with a molecular mass of 27kDa, which was recognized by the A beta antibodies, wasidentified as triosephosphate isomerase with sequencehomology to A beta peptides by N-terminal amino acidsequencing, mass fingerprint analysis usingmatrix-associated laser desorption/ionization massspectrometry, and Western blotting. Western blottingassay also revealed that detectable expression of A betaproteins were not seen in the piglet brains. These findingsindicate that triosephosphate isomerase with sequencehomology to A beta peptides accumulates in perivascularcells of the microglia/macrophage lineage located aroundarterial vessels of the newborn piglet hippocampus.This study is supported by Grants-in-Aid for ScientificResearch (C) nos. 19591281, 15591159, and (B) no.17390307 from the Ministry of Education, Culture,Sports, Science, and Technology of Japan.[Keywords]beta-amyloid, glycolysis, hippocampus, piglet,triosephosphate isomeraseIn conclusion, the VLBW infants with cystic PVLperformed poorly in supine, prone, sitting andstanding scores in AIMS at age 6, 12 and 18 months.Assessment of the gross motor developmental patternsusing the AIMS may help to identify VLBW infantswith cystic PVL as early as 6 months of age.[Keywords]Very low birth weight, Cystic periventricularleukomalacia, Cerebral palsy333

P2-162 Neonatology P2-163 NeonatologyPOST-ISCHEMIC-HYPOTHERMIA-INCREASED STRIATAL NEUROGENESIS ISASSOCIATED WITH DECREASEDPTEN SIGNALING PATHWAY INVOLVED INNEURONAL APOPTOSIS IN NEONATAL RATBRAIN WITH HYPOXIA-ISCHEMIAAPOPTOSIS OF NEURONAL PRECURSORSAND NEWBORN NEURONS IN THEDezhi Mu 1, 2* , Deyuan Li 1# , Yi Qu 1# , Meng Mao 1NEONATAL RAT BRAINDepartment of Pediatrics, West China Second University Hospital, SichuanUniversity, Chengdu 610041, China;. 2 Department of Neurology, University ofCalifornia, San Francisco, San Francisco, CA 94143, USAMan Xiong*, Guo-Qiang Chen*, Wen-Hao Zhou#, YiYang, Xiao-Mei ShaoKey Laboratory of Neonatal Diseases, Ministry of Health, Children’sHospital, Fudan University, 399 Wanyuan Road, Shanghai 201102, ChinaDepartment of Neonatology, Children’s Hospital, Fudan University, 399Wanyuan Road, Shanghai 201102, China*These authors contribute equally to this workHypothermia is a potential therapy for cerebralhypoxic ischemic injury in adults and neonates.However, the effects of hypothermia on neurogenesisin the developing brain remain unclear. In thisresearch, 7-day-old rats were subjected to left carotidartery ligation followed by 8% oxygen for 2 h. Theywere divided into hypothermia (rectal temperature,32–33°C for 24 h) and normothermia (36–37°C for 24h) groups immediately after hypoxia–ischemia (HI).To investigate cell proliferation, 50 mg/kg/day5-bromodeoxyuridine (BrdU) was injectedintraperitoneally at 4–6 days after HI, and animalswere sacrificed at 1 or 2 weeks. There was asignificant decrease in infarct volume in thehypothermia group at 7 days after HI compared withthat in the normothermia group. The numbers ofBrdU- and nestin-labeled cells did not changed greatly,but Tuj-1 (β-tubulin III) increased significantly in thestriatum at 1 and 2 weeks after HI in the hypothermiacompared to normothermia group. Neurogenesis wasassessed by doubleimmunohistochemical/immunofluorescent labeling ofBrdU with nestin, Tuj-1 or microtubule-associatedprotein 2 (Map-2). Newborn immature (BrdU + /Tuj-1 + )and mature (BrdU + /Map-2 + ) neurons increasedsignificantly, but neuronal progenitors (BrdU + /nestin + )did not change dramatically in the hypothermiacompared with normothermia group. The apoptosisrate of neuronal precursors, immature and matureneurons, assessed by double labeling of activecaspase-3 and nestin/Tuj-1/Map-2, decreasednoticeably in the hypothermia compared withnormothermia group. We also found that hypothermiasignificantly increased expression of Bcl-2, whichcoexisted with nestin/Tuj-1/Map-2. These resultssuggest that hypothermia-enhanced striatalneurogenesis is associated with increased survival ofnewborn stem cells and neurons by Bcl-2. Theseobservations are noteworthy regarding clinicalhypothermia therapy following cerebral HI injuryduring the perinatal period.Phosphatase and tensin homolog deleted on chromosome10 (PTEN) phosphatase has been linked to its capacity toantagonize the phosphatidylinositol 3-kinase /Aktsignaling pathway. Previous studies have revealed thatForkhead transcriptional factor (FOXO3a) is a criticaleffector of PTEN-mediated tumor suppressor. However,whether PTEN/Akt/ FOXO3a pathway is involved inneuronal apoptosis in developing rat brain afterhypoxia-ischemia (HI) is not clear. In this study, wegenerated HI model using postnatal day 10 rats.Immunohistochemistry and Western blot were used todetect the expression of total and phosphorylation ofPTEN, Akt, and FOXO3a and its target gene Bim. Wefound that dephosphorylation of PTEN was accompaniedby dephosphorylation of Akt and FOXO3a, whichinduced FOXO3a translocation into the nucleus andup-regulated the expression of Bim. Furthermore, wefound that PTEN inhibition by bisperoxovanadiumsignificantly increased the phosphorylation of Akt andFOXO3a, decreased the nuclear translocation ofFOXO3a, and inhibited Bim expression after HI.Moreover, the downregulation of Bim and HGTD-Pcaused by PTEN inhibition attenuated cellular apoptosisin the developing rat brain. Our findings suggest thatPTEN/Akt/FOXO3a pathway is involved in neuronalapoptosis in neonatal rat brain after HI. Agents targetingPTEN may offer a promise to rescue neurons from HIbrain damage.[Keywords]Akt; Bim; FOXO3a; hypoxia–ischemia; neuronal apoptosis;PTEN334

P2-165 Neonatology P2-166 NeonatologyTHE FT4 AND TSH LEVELS IN SERUM OFPREMATURE INFANTS REGARDING THEGROWTH PARAMETRS, METHOD OFSURVIVIN MAY PLAY A PROTECTIVE ROLEDURING HYPOXIA ISCHEMIA BRAIN DAMAGEIN NEONATAL RATSDELIVERY, AND APGAR SCOREBabak AbdiniaPediatric, Shahid Beheshty, IranBACKGROUND & AIMS: Thyroid hormones haveimportant effects on growth and differentiation of bodyorgans specially the central nervous system; It also has arole in metabolism of carbohydrates, lipids and vitamins.Certain studies showed that low level of thyroid hormonesis a risk factor for complication of prematurity, and theysuggested administration of thyroxin for prematureinfants. So this study was done to evaluate FT4 and TSHlevels in preterm infants.MATERIALS & METHODS: This cross-sectionaldescriptive study was done on 99 premature 2-7 day-oldinfants whose gestational age was between 26 to 36 weeksin NICU and a neonatal ward with random sampling. Onemillilier of venous blood was taken, and serum FT4 andTSH level were measured by radio immunoassay.RESULTS: This study showed that with increase ofinfants' weight, FT4 level rises (P=0.4, R=0.05) and TSHlevel decreases (P=0.2, R=0.12). With the increase ofinfants height FT4 level rises (P=0.8, R=0.02) and TSHlevel decreases (P=0.13 R=0.15). While increase of headcircumference did not have a meaningful correlationstatistically with FT4 level in both sexes (P=0.08) but therewas a meaningful correlation in boys (P=0.04), TSH leveldecreases with increasing head circumferences but it wasnot meaningful statistically (P=0.2 R=0.12); The meanvalue of FT4 level between infants born with high and lowapgar score (P

P2-167 Neonatology P2-168 NeonatologyTHE EXPRESSION AND FUNCTIONALASSAY OF TOLL-LIKE RECEPTORS FROMMONOCYTES OF PREMATURE INFANTS INVIVO AND IN VITRONEUROLOGICAL OUTCOME OF ELBW INFANTSAT 2-YEAR CORRECTED AGE: A 10 YEARSCOHORT STUDY IN A SINGLE MEDICALCENTER IN TAIWANChung Min Shen 1 , Yu Ru Kou 2Department of Pediatrics, Cathay General Hospital 1 , Institute of ClinicalMedicine National Yang-Ming University, Taiwan 1,2OBJECTIVES: Premature infants are highlysusceptible to severe bacterial infections. Theexpression of Toll like Receptors (TLRs) and thepossible involvement of innate immunologicdeficiencies of the premature infants have not beenextensively investigated. The aim of this study was toevaluate TLR2 and TLR 4 expressions from preterminfants, health full term newborns and adults.METHODS: Adult healthy volunteers (n=20; meanage, 28 ± 0.8 years old; male, 54 %; female, 46 %)were included for collection of peripheral blood.Health full term newborns (n=36; mean gestationalage, 39.8 ± 0.2 wks; birth body weight, 3114.2 ±218.45 gm; male, 42.7 %; female, 57.3 %) and pretermnewborns (n=21; mean gestational age, 30.4 ± 3.5wks; birth body weight, 1682.9 ± 187.2 gm; male, 57.6%; female, 42.4 %) were included for collection ofcord blood immediately after delivery. Monocytesfrom premature infants were collected every twoweeks after birth subsequently or when got infection.We analyzed the PBMC for protein and mRNAExpression of TLR 2 and TLR4 in vivo. Monocyteswere cultured and had LPS stimulation on Day 0, Day3, Day 5 and Day 7. IL-1β, IL-6, and TNF-α cytokineswere determined by ELISA.MAIN RESULTS: There was no significantdifference of TLR2 and TLR4 basal expression levelbetween full term newborns and adults but was lowerin premature infants. The expression of TLR2 andTLR4 increased in premature infants after birth invivo. However the expression and functional assay ofTLR2 and TLR4 were not increased as expected invitro. The functional assay was lower in prematureinfants than full term infants and adults in vivo and invitro. Protein expression and functional assay of TLR2and TLR4 were decreased when the premature infantsgot infection.CONCLUSION: The increased expression in proteinlevel and functional assay in vivo were not noted fromthe study in vitro. The response of TLR2 and TLR4 inpremature infants getting infection was lower than fullterm infants’.[Keywords]Toll like receptor 2, Toll like receptor 4, prematureinfantMing-Hsia Lin, Hsiang-Yu Lin, Pei-Ying Lin, Hsin-YangHsieh, Hsiao-Yu Chiu, Hung-Chih Lin, Bai-Horng SuDepartment of Pediatrics, China Medical University Children’s Hospital,Taichung, Taiwan.OBJECTIVES: The survival rate of extreme low birth weight(ELBW) infants has improved by advanced antenatal andneonatal intensive care. However, these infants are still at risk ofdeveloping different kinds of morbidity. The aim of thisretrospective cohort study is to identify the relationshipsbetween neonatal interventions and morbidity andneurodevelopment outcome at 2-year corrected age.METHODS: A retrospective cohort study included 481 ELBWinfants (birth weight ≦1000 g) admitted to NICU of ChinaMedical University Hospital from January 1998 to April 2007.The Bayley Scales of Infant Development (BSID) was used tomeasure the mental and motor development in these ELBWinfants at 2-year corrected age. The associations betweenneonatal morbidity and abnormal BSID PsychomotorDevelopmental Index (PDI) and Mental Developmental Index(MDI) scores were estimated in multivariate linear regressionanalyses.MAIN RESULTS: The survival rate of ELBW infants in ourhospital is 60.91% (293/481) and 221 of them received theBayley scales evaluation at 2-year corrected age. The meangestational age was 26.3 weeks and the mean birth weight was821gm (range 460 – 1000gm). Among the 221 ELBW infants,31.5% was born in our hospital, 54.1% were delivered bycesarean sections, 6.8% had severe intraventricular hemorrhage(IVH≧3), 11.4% had periventricular leukomalacia (PVL),23.2% had retinopathy of prematurity (ROP) required treatment,27.4% had bacterial sepsis, 82.2% had received indomethacinfor patent ductus arteriosus, and 9.1% had proven necrotizingenterocolitis. The proportion of abnormal MDI (< 70) was19.5% and was associated with vaginal delivery (p= .0126), lowgestational age (p=.0071) and ROP required treatment(p=.0077). The proportion of abnormal PDI (

P2-169 Neonatology P2-170 NeonatologyEFFECT OF GRADE I-IIINTRAVENTRICULAR HEMORRHAGE ONNEURODEVELOPMENTAL OUTCOME INCHANGES OF BLOOD CHEMISTRY VALUES OFNORMAL FULL-TERM NEWBORN IN THE FIRSTWEEK OF LIFEINFANTS WITH BIRTH WEIGHT < 1250 GM:DOES IT NO MATTER?Wai-Ho Lim, Ming-Chou Chang, Reyin Lien, Kuang-LinLin 1 , Peng-Hong Yang, Ren-Huei Fu, Jen-Fu Hsu,Shih-Ming Chu, Chang-Yo Yang, Tzu-Hui LeiDivision of Neonatology and 1 Pediatric Neurology, Department ofPediatrics, Chang Gung Children’s Hospital, Chang Gung UniversityCollege of Medicine, Taoyuan, TaiwanOBJECTIVE: Intraventricular hemorrhage (IVH) causesdestruction of the germinal matrix and glial precursor cells whichmay lead to neurodevelopment impairment. Outcome of gradeI-II IVH was considered favorable. However, most of the papersavailable were designed to study larger preterm infants and wereperformed before the era of routine antenatal steroids andsurfactant administration. We thus conducted this study todetermine the impact of uncomplicated IVH on psychomotor andmental development among VLBW infants.METHODS: Medical records of VLBW infants withBBW

P2-171 Neonatology P2-172 NeonatologyC-REACTIVE PROTEIN IS USEFUL TODIFFERENTIATE SEPSIS FROMNON-INFECTIOUS NEONATAL SYSTEMICTHE NEONATAL LUPUS COMPLICATED WITHCENTRAL NERVOUS SYSTEM INVOLVEMENT:CASE REPORT AND LITERATURE REVIEWINFLAMMATORY RESPONSE SYNDROMEXiongying ,LinyanDepartment of Neonatology, West China Second Hospital of SichuanUniversity, ChinaMing-Hsia Lin, Hsiang-Yu Lin, Hsin-Yang Hsieh,Chung-Hsing Wang, Hsiao-Yu Chiu, Hung-Chih Lin,Bai-Horng SuDepartment of Neonatology, China Medical University Children’s Hospital,Taichung, TaiwanOBJECTIVE: To evaluate the efficacy of usingC-reactive protein to differentiate sepsis fromnon-infectious SIRS.METHODS: Prospective and observational study.65 neonatal cases with SIRS were enrolled, Patientswere classified into two groups based on clinicalprogress and culture results: sepsis andnon-infectious sirs. blood samples for determiningwhole blood CRP.RESULT: Median whole blood CRP were 7.0、2.0mg / L in sepsis and non-infectious sirs,respectively. P=0.013 was statistical differencebetween the groups. the area under the receiveroperating characteristic (ROC) curve (AUC) forCRP was 0.68(95%CI 0.552~0.808), For thecut-off value of CRP > or = 为 4.5 mg/L,the testwas found to have a sensitivity of 59.0%, specificityof 69.2%, positive likelihood ratio value of 1.91.CONCLUSION: Assessing CRP levels are usefulto differentiate sepsis from non-infectious SIRS.[Keywords] C-reactive protein; Systemic inflammatory responsesyndrome; SepsisOBJECTIVES: Neonatal lupus erythematosus (NLE) isconsidered to that an infant has elevated titers of anti-SSAand SSB antibodies passively through the placenta by themother with an autoimmune disease. The mostwell-known clinical presentations are 1) congenital heartblock 2) cutaneous lesions 3) hepatobiliary dysfunctionand 4) hematological manifestations. The literature onCNS involvement in neonatal lupus is rarely reported inthe past.METHODS: We present a case of a preterm infant withneonatal lupus erythematosus. Macrocephaly andsubdural effusions with mass effect were found when hewas 2 months old. The anti-SSA and SSB antibodies inthe subdural effusions were higher than referral range inserum. The relation articles were searched with thekeywords of “CNS, neonatal lupus, macrocephaly andhydrocephalus” on Pub Med from 1994 to 2009.MAIN RESULTS: Abnormal neuroimages, such ashydrocephalus, subependymal cysts, and vasculopathyreported in neonatal lupus erythematosus are largely inthe form of isolated case reports or a small series of cases.Including our case, from 1994 to 2009, total 35 abnormalneuroimages could be discovered; 14 had hydrocephalus,12 had subependymal cyst/subependymal hemorrhage, 9had decreased cerebral white matter density and etc.CONCLUSION: According to our case, we believe that,in addition to well-known clinical presentations, allpatients with considerable neonatal lupus syndromeshould receive head circumference measurement andneuroimage survey routinely. Besides, test of anti-SSAand SSB antibodies in CSF could be performed forsuspicion of NLE with CNS involvement.[Keywords]Neonatal lupus erythematosus, macrocephaly, congenitalheart block, CNS involvement338

P2-173 Neonatology P2-174 NeonatologyCHANGES OF BIOLOGICAL CLOCKPROTEIN IN HIBD NEONATAL RAT ANDCLOCK GENE EXPRESSION IN PBLC OFHIE NEONATESXing FengDepartment of Newborn Medicine, Soochow University AffiliatedChildren’s Hospital, Suzhou, Jiangsu 215003, ChinaOBJECTIVE: To explore the effects of biologicclock protein on circadian disorder inhypoxic-ischemic brain damage(HIBD) by studyingthe level of CLOCK,BMAL1 in neonatal rat pinealgland and expression of Clock mRNA,Bmal1mRNA in peripheral blood lymphocytes(PBLC) ofhypoxic-ischemic encephalopathy(HIE) neonatesMETHODS: 7-day-old Sprague-Dawley (SD) ratswere randomly divided into 2 groups (36 pupseach). HIBD models were set up according tomodified Levine euthanized 0、2、12、24、36、48hours afterwards. Western Blot analysis was used tomeasure the amount of CLOCK and BMAL1 in ratpineal gland and Semi-quantitative ReverseTranscriptase Polymerase Chain Reaction(RT-PCR)analysis for the expression profiles of Clock andBmal1 in PBLC of neonatal human (18case HIEand 18case normal newborn), respectively.MAIN RESULTS: 1、The level of CLOCK 36hafter HIBD began to increase, and that of 48hdisplayed significantly than the control groups(p

P2-175 Neonatology P2-176 NeonatologyEFFECT OF HYPOXIC ISCHEMIA ANDINFLAMMATION ON THE RISK OFCEREBRAL PALSY IN VERYLOW-BIRTH-WEIGHT INFANTSOPERATIONALIZATION OF CENTRALIZEDBLOOD CULTURE SERVICES FOR RURAL SICKNEWBORN CARE UNIT IN A DEVELOPINGCOUNTRY AND ITS IMPACT1 Lan-Wan Wang; 2 Shan-Tair Wang; 3 Chao-ChingHuangTaiwan Premature Infant Developmental Collaborative Study Group1 Department of Pediatrics, Chi Mei Medical Center; 2 Department ofMedical Research, Chia-Yi Christian Hospital; 3 Department of Pediatrics,National Cheng Kung University Hospital, Taiwan.OBJECTIVE: Very low-birth-weight (VLBW) prematureinfants have a higher incidence of cerebral palsy (CP) thanterm infants. Hypoxic ischemia (HI) andinflammation/infection during the perinatal and postnatalperiods are the two major risk factors of CP in preterminfants. However, whether there is a joint effect of HI andinfection/inflammation on the outcome of CP in VLBWinfants remains unclear. Using a neurodevelopmentalfollow-up cohort of VLBW premature infants in Taiwan, thisstudy was to examine the effect of HI andinflammation/infection, and their joint effect during theperinatal and postnatal periods on the risk of CP.METHODS: From 1995 to 2005, we prospectively enrolledVLBW infants who had been admitted to the neonatalintensive care units of 20 medical centers in Taiwan, and hadfollow-up neurodevelopmental examinations at the correctedage of 6, 12, 18 and 24 months. Medical records,socio-demographic data, and neurological status anddevelopmental scores at each follow-up visit were collected.Infants with severe intraventricular hemorrhage or brainanomalies were excluded. CP was diagnosed by pediatricneurologists at age 24 months. The potential risk factors,including HI and inflammation/infection, of CP wereclassified according to the occurrence during the perinataland postnatal periods. The most significant predictors wereidentified, and the joint effects of HI andinflammation/infection on the risk of CP were examined.MAIN RESULTS: Of the 6812 VLBW infant survivorsduring the study period, 4296 infants (63%) received24-month follow-up examinations, and 3946 infants wereeligible for inclusion into this study. Of the eligible infants,218 had CP, 336 with other neurological impairment, 1099had mild neuromotor dysfunction, and 2293 had normalneurological outcomes. Compared to the infants with normalneurodevelopmental outcome, the infants with CP had asignificantly higher incidence of HI during the perinatal andpostnatal periods, while the two groups did not differ in theoccurrence of inflammatory insults. Frequentapnea/bradycardia was the most influential predictor for CP(p < 0.0001). For infants with mild, moderate and severeapnea/bradycardia, the odds ratios of CP in the presenceversus the absence of sepsis were 1.13 (p = 0.61), 2.14 (p =0.06) and 1.07 (p = 0.90), respectively.CONCLUSIONS: For VLBW premature infants, HI duringthe perinatal and postnatal periods is the predominant riskfactor for CP. Inflammation/infection does not significantlyexert joint effect on HI to increasee the risk of CP.[Keywords]Very low-birth-weight infants, Cerebral palsy,Hypoxic ischemia, Inflammation/InfectionR.Viswanathan 1 ,A.K.Singh 1 ,C.Ghosh 2 ,S.Sardar 11Department of Neonatology, IPGMER, Kolkata, India2 Department of Pediatrics, Suri Sadar Hospital, Birbhum, IndiaBACKGROUND: India is home to the highest number ofnewborn deaths in the world. Mortality remains high in ruralareas. One of the most important causes for this is neonatalsepsis.The gold standard for diagnosis is blood culture, which isusually not available in rural health care setting in India, thereoften being no skilled microbiologist or infrastructure .Thereis little data on incidence or antibiotic resistance profile ofculture proven sepsis in rural areas. WHO and national forumtreatment guidelines are followed empirically with ampicillin,gentamicin as first line and cefotaxime, amikacin as second lineantibiotics, often with poor results. Fungal etiology of sepsis isnot usually considered.OBJECTIVES: To develop a system to provide culture facilityto a remote rural unit, to assess the incidence, etiology andantibiotic resistance profile of neonatal sepsis in rural institutionsetting.METHODS: 20 bedded level II sick newborn care unit in adistrict hospital of Birbhum district of West Bengal, 220 kmfrom Kolkata was selected as the field unit. The hospital hasabout 6000 deliveries per year and admits both inborn andoutborn babies. Centralized laboratory work was done atmicrobiology laboratory of Department of Neonatology,IPGME&R & SSKM Hospital, Kolkata. Training was conductedat field unit for proper collection of blood culture samples fromthe sick neonates.Simple, easily available and cost effectivesample transport mechanisms were evaluated. Blood culture wasdone by BACTEC 9050 system using Peds Plus vial.Identification of isolates was confirmed by standard biochemicalmethods and mini API analyzer. Antibiotic sensitivity testingwas done by Kirby Bauer Disc Diffusion method.RESULTS: 165 samples were collected over a period of sixmonths. 72 (43.6%) cultures were positive. Gram negativeorganisms were predominant (53/72). Klebsiella pneumoniaewas the most common isolate. Candida sp accounted for17/72isolates.Several uncommon organisms likeStenotrophomonas maltophilia, Burkholderia cepacia andCandida utilis were isolated. Almost complete resistance wasnoted among the Gram negatives to 1 st and 2ndline antibiotics -Ampicillin (100%), Gentamicin (100%), Amikacin (71%) and3 rd generation cephalosporins (100%).CONCLUSION: The picture of antibiotic resistance amongneonates with Gram negative sepsis in rural India is grim. Theempirical choice of first line and second line antibiotics needs tobe reviewed. Fungal cause of neonatal sepsis needs to beconsidered. Development of microbiology facility for rural sickneonates is of utmost importance.[Keywords] neonatal sepsis,rural ,blood culture,centralized340