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AGK protein, defect of respiratory complex deficiency, further impaired OXPHOS function under stress and the production of Cellular ATP. In responding to the cellular energetic deficit, the AGK mutant cells increased the cell proliferation and inhibited the mitochondrial fission through molecular modulation. However, the depletion of ATP production, prolonged elongated mitochondria and increased cellular proliferation will induce abnormal mitosis and genetic destabilization resulting in mitosis catastrophy and cell premature senescence.

48 Mutation Analysis of PRRT2 Gene Mutations in Familial

and Sporadic Paroxysmal Kinesigenic Dyskinesia PRRT2

Syuan-Yu Hong1 I-Ching Chou1,2 Wei-De Lin3,4

Chung-Hsing Wang5,6Sheng-Shing Lin1Yu-Tzu Chang1Zheng-Nan Chin7Chang-Hai Tsai 8,9Fuu-Jen Tsai 4,5

Department of Pediatrics Neurology, Childrens Hospital of China Medical University1, Taichung, Taiwan; Graduate Institute of Integrated Medicine, College of Chinese Medicine, China Medical University2, Taichung, Taiwan; School of Post Baccalaureate Chinese Medicine, China Medical University3, Taichung, Taiwan; Department of Medical Research, China Medical University Hospital4, Taichung, Taiwan; Department of Pediatrics, Childrens Hospital of China Medical University5, Taichung, Taiwan; School of Medicine, China Medical University6, Taichung, Taiwan; Department of Pediatric, Tainan Municipal An-Nan Hospital-China Medical University7, Tainan, Taiwan; China Medical University8, Taichung, Taiwan; Asia University, Taichung, Taiwan9

11,23,45,61178,94,5

123456()789

Background: Paroxysmal kinesigenic dyskinesia (PKD) is

characterized by frequent, recurrent attacks of involuntary movement or posturing in response to sudden movement, stress, or excitement. PKD can be sporadic, or familial with autosomal dominant inheritance. Pathophysiological mechanism of PKD remains not well understood. Mutations in proline-rich transmembrane protein 2 (PRRT2) were recently identified in patients with familial PKD. To extend these genetic reports,we aimed to characterize affected individuals in patients with PKD with PRRT2 mutations

Methods: We reviewed our patients of PKD with the definite diagnosis of PKD, who were seen at China Medical University Hospital. Detailed clinical questionnaires and interviews were conducted with affected and unaffected family members. EEG, and brain magnetic resonance imaging were performed. We screened all 3 coding exons of PRRT2 for mutation in 19 Taiwanese patients with PKD from 9 different families.

Results: Fourteen had familial PKD and five were

apparently sporadic cases. In total, a one-base C inserted at nucleotide 650 (c.650insC) and caused protein translation shifted and stopped after the seventh residue (p.Arg217Pro fs7X) were identified in 13 patients, including 12 familial cases and one apparently sporadic cases.

Conclusions: PRRT2 mutations account for 85.7 % (12 out of 14) of familial PKD and 20 % (one out of 5) apparently sporadic PKD in the Taiwanese cohort. The c.650insC is the most common mutation in our patients. Identification of genes underlying pathogenesis will enhance diagnosis and treatment. Function of PRRT2 and its role in PKD warrant further investigation.

Key words: Paroxysmal kinesigenic dyskinesia, PRRT2, gene, mutation

49 Mortality and Morbidities after Preterm Birth:

Comparison of Three Birth Cohorts in 15 Years in Taiwan

Shih-Hsin Wang1,4, Po-Nien Tsao1,2, Chien-Yi Chen1,

Hung-Chieh Chou1, Wu-Shiun Hsieh1, Kuo-Inn Tsou3, Taiwan Infant Developmental Co

Departments of Pediatrics, National Taiwan University Hospital Childrens Hospital1, Taipei, Taiwan; Research Center for Developmental Biology and Regenerative Medicine, National Taiwan University2, Taipei Taiwan; Departments of Pediatrics, Cardinal Tien Hospital3, New Taipei City, Taiwan; Departments of Pediatrics, Far Eastern Memorial Hospital4, New Taipei City, Taiwan

1,41,21113

1234

Background: Preterm birth is associated with increased

rates of neonatal mortality and morbidities. This is an important public health issue as rates of preterm birth are rising worldwide. There are several interventions to reduce neonatal morbidity, including usage of antenatal corticosteroids, increased use of surfactant replacement and centralized delivery and intensive care for the most immature infants in tertiary hospitals etc.. This study was aimed to evaluate the trends of survival, neonatal morbidity, parental socio-economic status, maternal and perinatal conditions for VLBW (BBW1500gm) babies born in Taiwan during 2007-2011 compared to those born between 1997-2001 and 2002-2006.

Methods: To address this issue, we conducted a retrospective cohort study in very-low-birth-weight (VLBW) infants registered in the Premature Baby Foundation of Taiwan since 1997. A total of 22 neonatal intensive care units in Taiwan participated in the data collection. A series of studies was conducted and dividing into 3 studying periods (birth cohort 1997-2001; n=4647, birth cohort 2002-2006; n=4005, and birth cohort 2007-2011; n=4507) in a population-based sample. Main outcome measures included survival to discharge from hospital, pregnancy and delivery outcomes, and infant morbidity until discharge.

56 7 2015

24

Results: Comparing three cohorts (1997-2001, 2002-2006, 2007-2011), we found higher survival rate of extreme prematurity (GA 24 weeks) (18%, 28.7%, 35.5%) and advanced pregnancy age (>35y/o) (13.4%, 17.4%, 23.2%). For perinatal condition, there were more high-risk pregnancies (74.3%, 86.4%, 89.5%), increased artificial reproductive assistance (11.4%, 10.8%, 21%), more women receiving complete course of antenatal steroid (27.7%, 35.5%, 43.9%), higher C-section rates (54.7%, 60.6%, 69.4%) and higher rates of maternal transfer (22.6%, 29.9%, 40.8%). There was less surfactant usage (46.4%, 51.8%, 40.6%) and ventilator support (78%, 77.3%, 42.4%). The incidence of PDA ligation increased (2.8%, 6.0%, 9.8%, p

25

transfusion group. Besides, we evaluated the effect of transfusion rate on incidence of PDA in ELBW infants.

Results: 84 cases were enrolled in our study, and 33 cases (39.2%) received FFP transfusion during the first three days. 22 of them (67%) had PDA. In no FFP transfusion group, 23 of 51 cases (45.1%) had PDA. As a result, FFP transfusion is related to PDA developing. There was significant difference in the incidence of PDA between FFD transfusion and no FFP transfusion groups. Besides, 12 of the 33 cases receiving FFP transfusion had routine transfusion strategy. 7 case of them were revealed to have PDA (58.3%). In 21 of the 33 cases receiving slowly transfusion strategy, 15 cases of them were found to have PDA (79%). There was no significant difference between routine and slowly infusion groups.

Conclusions: FFP transfusion group had higher incidence of PDA than those who did not receive FFP in ELBW infants. However, different infusion rate did not influence the incidence of PDA.

52 Postligation Cardiac Syndrome (PLCS) in Preterm

Infants Receiving PDA Surgical Ligation Shih-Yun Hsu, Reyin Lien, Kai-Hsiang Hsu, Tai-Wei Wu,

Shih-Ming Chu, Ming-Chou Chiang Division of Neonatology, Department of Pediatrics, Chang

Gung Childrens Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan

Background: Surgical ligation of the ductus arteriosus (DA)

leads to sudden and dramatic changes in cardiovascular physiology of the preterm infant. These changes include a rise in afterload due to the increase in systemic vascular resistance (SVR), a dramatic fall in preload, due to the sudden reduction in pulmonary blood flow, and a compromised myocardial contractility. PLCS depicts a critical condition of acute hemodynamic decompensation which is characterized by significant systolic hypotension and increasing ventilatory requirement typically becomes apparent 6 to 12 hours following PDA ligation. PLCS have been reported to cause increased mortality in preterm infants. The aim of this study was to identify PLCS in our patients and to examine the possible risk factors for development of PLCS.

Methods: We retrospectively retrieved those VLBW preterm infants who received PDA ligation in our NICU during the period between January 2014 till May 2015. By reviewing their medical records, patients of PLCS was identified based on the definition of worsened hypotension and increased ventilator support (Oxygen Index) 48 hours post PDA ligation as compared to prior to surgery. For every PLCS patient, the preterm infant in the same NICU who received the same procedure before and after this index patient, but did not have PLCS, were chosen to serve as control. Demographic data, clinical and echocardiographic features, as well as short term outcome were compared between the study and control groups.

Results: The total incidence of PLCS is 8.3% among preterm neonate who underwent PDA ligation. There were 7 patients of PLCS and hence 14 were collected as control. There GA, BW and gender (M;F) distributions were 25+4 weeks vs 28+2 weeks; 696.7g vs 1130g, and 3;4 vs 9;5. Study Group Control Group OP age (postnatal day) 12.45.8 10.95.5 Prior Ibuprofen use 42.9% 66.7% Echo finding PDA size (mean SD) 2.370.26 3.141.15 LA:Ao 1.39 1.46 Oxygen Index (OI) OP 12.3 4.6 Oxygen Index (OI) OP 32.6 4.8 Severity of hypotension OP 3 1.6 Severity of hypotension OP 3 0.3 Other surgical complications 1/7 0/14 Outcome Survival till hospital discharge 28.6% 100% BPD in survivor 100% 33% Severe ROP in survivor 20% 0% Ventilator days in survivor 75 60.4 Hospital days in survivor 88 84.1

Conclusions: PLCS is a complication of high mortality possibly due to delayed surgical correction of PDA. The optimal timing of surgical ligation of PDA is still under debate. For decision making, both clinical characteristics and echocardiographic criteria should be taken into account. Clinicians should be vigilant about PLCS in preterm infants and post-operative management should be pathophysiologically guided accordingly.

53 Use Electrical Cardiometry to Monitor Cardiac Output

in Preterm Infants with Patent Ductus Arteriosus: a Comparison with Echocardiogram

Kai-Hsiang Hsu, Tai-Wei Wu, Reyin Lien, Shih-Ming Chu,

Jen-Fu Hsu, Ren-Huei Fu, Ming-Chou Chiang, Chang-Yo Yang, Peng-Hong Yang

Division of Neonatology, Department of Pediatrics, Chang Gung Memorial Hospital at Linkou

Background: Electrical cardiometry (EC) is an

impedance-based monitoring that provides noninvasive, continuous and real-time cardiac output (CO) assessment and is of growing interest in the neonatal intensive care setting as it can help guide clinical decision making and evaluate treatment response. Through comparison to transthoracic echocardiogram (TTE), the accuracy of EC has been verified in healthy neonates. Cardiac index (CI) is the product that relates CO to body surface area (CO/BSA) and is proposed to evaluate cardiac performance among infants of different size. EC reference of CI has been reported 2.55 0.37 L/min/m2 in hemodynamically stable neonates. Since patent ductus arteriosus (PDA) is the main cause of hemodynamic instability in preterm infants, we aimed to compare the accuracy of EC to bedside TTE in those with hemodynamically significant PDA (hsPDA).

Methods: We prospectively enrolled preterm infants who had hsPDA and without other structural heart anomaly other than patent foramen ovale. Infants with inotrope support were excluded as well. The hsPDA was defined as ductal diameter 1.5 mm or left atrium to aorta ratio (LA/Ao) 1.5

56 7 2015

26

in echocardiogram. Simultaneous CO measurements by TTE (Sonos 7500, Philips, Boeblingen, Germany) and EC (Aesculon, Osypka Medical, Berlin, Germany) in supine position were recorded. Data were compared by Pearson correlation, Bland-Altman analysis and independent t-test.

Results: A total of 16 paired measurements in 5 preterm infants were compared in this preliminary study. Infants mean age and weight at measurement were 27.8 weeks and 1181 gm, with ductal size 1.94 mm and LA/Ao 1.48. CO by TTE and EC were 0.272 0.056 and 0.268 0.039 L/min (mean SD), which demonstrated a high correlation (r = 0.706, P = 0.002). BlandAltman analysis showed that the bias and percentage error were 0.002 L/min and 30%. In our cases, CI by EC was 2.90 0.33 L/min/m2, which was significant higher than the reference (P = 0.001).

Conclusions: For preterm infants with hsPDA, a good correlation and agreement are seen between TTE and EC in measuring CO. The finding of significantly higher CI in infants with hsPDA is in line with previous reports of supernormal CO in compensation for the steal effect of the hsPDA.

54 Extracorporeal Membrane Oxygenation Support in

Neonates: A Single Medical Center Experience in Taiwan

Chi-Man Kuok1, Po-Nien Tsao1, Shu-Chien Huang2,

Chien-Yi Chen1, Hung-Chieh Chou1, Wu-Shiun Hsieh1, En-Ting Wu1

Departments of Pediatrics1 and Cardiothoracic Surgery2, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan

1121111

12 Background: Extracorporeal membrane oxygenation

(ECMO) was used in patients with cardiopulmonary failure who failed to conventional therapy. We started to use ECMO in neonatal cases who were not congenital heart diseases (CHD) since 2005 in our hospital. This study was retrospectively reported the 10 years experience of neonatal ECMO use, except for CHD, in a tertiary center in Taiwan.

Methods: We reviewed the medical record of neonates who required extracorporeal life support by our ECMO team from July 2005 to June 2015. The patients with CHD were excluded. We analyzed the disease distribution, basic characteristics, age at ECMO initiation, duration of ECMO and short-term outcome etc.

Results: A total of 27 neonates were treated with ECMO during this 10-year period, of which congenital diaphragmatic hernia (CDH) (26%) was the most common diagnosis, and followed by meconium aspiration syndrome (MAS)(22 %) and persistent pulmonary hypertension of newborn(PPHN)/pulmonary hemorrhage (22%). Eight patients (29.6%) received ligation of patent ductus arteriosus under ECMO support. The median duration of ECMO use were 144.4 (4.8~2158.8) hours and hospital day was 36 (1~232) days. The survival rate was 59.3% and 8 of

them died on ECMO. Twenty one (77.8%) patients were transferred from other hospital by ECMO support. The survival rate in ECMO transport group was even better than in-house ECMO group (90.9% v.s 40 %, p=0.024).

Conclusions: Neonates with acute pulmonary or cardiologic problems supported by ECMO can improve the overall survival rate. Our data also supports that ECMO is a useful tool to transfer the critical neonates who were poorly response to ventilator support. To the best of our knowledge, this is the first study to report the experience of neonatal ECMO use in Taiwan.

55 The Analysis of Risk Factors Effecting on Duration of

Oxygen Supply and Ventilator Support in Very Low Birth Weight Preterm Infants

Chung-Hao Su, Mei-Yung Chung, Mei-Chen Ou-Yang,

Feng-Shun Chen, Chih-Cheng Chen, Hsin-Chun Huang Section of Neonatology, Department of Pediatrics,

Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Taiwan

Background: Because of immature brain, immature lung,

surfactant insufficiency and muscle weakness, the very low birth weight (VLBW) preterm infants usually need positive-pressure ventilator support and respiratory care. This study was designed to evaluate the effect of ventilation with neurally adjusted ventilatory assist (NAVA) vs. conventional intermittent mechanical ventilator (CIMV) on reducing duration of oxygen supply and ventilator support in VLBW preterm infants. Otherwise, we also further analyzed the risk factors effecting on duration of oxygen and ventilator support.

Methods: This is a prospective study performed between July 2011 and Jun 2013. Infants of

27

Conclusions: Compared with conventional intermittent mechanical ventilation, NAVA appears to provide rapid weaning of oxygen and ventilator, especially in VLBW preterm infants with severe respiratory distress syndrome treated with surfactant.

56 Bacterial Meningitis Features in Neonates, Neurological

Complications and Impact on Outcomes I-Hsyuan Wu1, Ming-Horng Tsai2, Shih-Ming Chu1, Reyin

Lien1, Jen-Fu Hsu1, Ren-Huei Fu1, Ming-Chou Chiang1 Division of Neonatology, Department of Pediatrics, Chang

Gung Memorial Hospital1, Linkou, Taiwan; Division of Neonatology and Pediatric Hematology/Oncology, Department of Pediatrics, Chang Gung Memorial Hospital2, Yunlin, Taiwan

1211111

12

Background: Neonatal meningitis is uncommon but

associated with high mortality and morbidity. We aimed to describe features of neonatal meningitis, the subsequent complications and determine factors associated with severe disease or death.

Methods: A systemic chart review was performed to identify clinical characteristics and outcomes of all neonates with documented bacterial meningitis from January 2005 to December 2013 in a tertiary-level neonatal intensive care unit. A cohort of neonates with bacteremia was constructed as the control group.

Results: During the study period, a total of 81 episodes of bacterial meningitis, including 59 episodes of primary meningitis in 57 neonates, and 22 episodes of meningitis after ventriculo-peritoneal shunt or extraventricular drainage in 11 neonates were identified. Among the 68 patients, 51.5% were term, 22.1% late preterm, and 26.5% very/extremely preterm infants. The most common pathogens were Group B streptococcus (29.6%), and E. coli (19.8). Neonatal meningitis caused by gram-positive cocci was comparable with those caused by gram-negative bacilli in terms of attributable mortality and complication rates. In total, 53.1% patients were considered to have severe disease, and 16.2% died. When compared with neonates with bacteremia, neonates with bacterial meningitis has a 1.3-fold increased risk of final mortality and 4.8-fold increased risk of infectious complications.

Conclusions: Although neonatal meningitis has an improved outcome in recent years, its high rates of severe disease and neurological sequelae deserve further investigation focusing on optimal treatment strategies and clinical follow up.

57 The Association between Rooming-in Policy and Neonatal Hyperbilirubinemia

Ken-Hsyuan Shan1, Ming-Chih Lin2,3, Teh-Ming Wang2,

Han-Chun Chien2 Division of Pediatrics, Taichung Armed Forces General

Hospital1; Division of Neonatology, Taichung Veterans General Hospital2; School of Medicine, National Yang-Ming University3

12,322 12

1 Background: Baby friendly hospital is the leading concept

for obstetrics care nowadays. Rooming-in is an important part of it, because it can facilitate skin to skin contact and breast feeding. However, whether the baby cared by non-profession mother is association with admission due to neonatal hyperbilirubinemia have never been studied. The aim of this study was to evaluate the association between rooming-in and neonatal hyperbilirubinemia.

Methods: A total of 3341 term neonates were enrolled retrospectively from our nursery between Jan 1, 2011, and Dec 31, 2013.During nursery, rooming-in care was done according to WHO guideline if parents agreed. The endpoint was defined as admission for phototherapy according to the 2004 guideline published by American Academy of Pediatrics. Relative risk between room-in and admission for phototherapy was calculated. Potential confounding factors including exclusive breast feeding, ABO incompatibility, G6PD deficiency and body weight loss (BWL) were adjusted in multiple logistic regression models.

Results: The rooming-in ratio increased(2011:18.62%, 2012:24.13%, 2013: 40.47%) year by year under the promotion of baby friendly hospital. However, the rate of neonatal hyperbilirubinemia also increased (2011: 3.10%, 2012: 7.24%, 2013: 9.03%). The odds ratios (OR) of rooming-in for neonatal hyperbilirubinemia was 7.04(95% CI 4.41, 11.24). The rooming-in group had higher percentage of exclusively breast feeding and BWL >7% in first 3 days of life than non-rooming-in group. After adjustment of potential confounding factors in multiple logistic regression models, the odds ratio for neonatal hyperbilirubinemia was still significant (OR: 8.69 (95% CI: 5.15 , 14.68)).

Conclusions: Rooming-in is an independent risk factor for admission for phototherapy in our study. The visiting frequency by physicians and nursing staffs should be increased in postpartum patient ward. The nursery manpower should be enforced.

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58 Amniotic Fluid Erythropoietin Differentiate Extreme Preterm from Term Birth

Ying-Chun Lu1 Department of Pediatrics, Tri-Service General Hospital;

Department of Pediatrics, National Defense Medical Center1, Taipei, Taiwan

1 1 Background: Animal studies revealed that swallowed

amniotic fluid accounts for about 15% of fetal growth. Our purpose was to investigate whether amniotic fluid (AF) erythropoietin (EPO) concentration could be predominant for Extreme preterm birth (EB) rather than term birth (TB).

Methods: We enrolled 40 healthy pregnant women who planed to deliver babies via cesarean section. Mothers were classified into the EB and TB groups for evaluating differences of AF. Obstetricians collected AF of 30 TB and 10 EB for chemical analysis during the operational delivery. AF EPO concentration was the primary outcome and was determined by Epo-ELISA test. Other AF components including protein, triglycerides, glucose, sodium, and potassium levels were defined as the secondary outcomes and were analyzed by the standard principles of the clinical chemistry laboratory. The analysis was all completed in one medical tertiary center in the northern Taiwan.

Results: Overall, 40 healthy pregnant women with operational birth were analyzed. Characteristics of the extreme preterm neonates were gestational age (GA) ranging from 24 to 32 6/7 weeks and birth body weight (BBW) from 628 to 1500 gm; those of term neonates were from 37 to 41 6/7 weeks of GA and from 2750 to 3955 gm of BBW. Higher levels of EPO were detected in the EB in comparison to the TB (p0.05). Triglyceride, glucose, and sodium levels were higher (p

29

60 Impact of Maternal Hyperthyroidism on Growth and Development of VLBW Preterm Infants

Mei-Yin Lai, Reyin Lien, Kai-Hsiang Hsu, Ming-Chou

Chiang, Shih-Ming Chu, Jen-Fu Hsu, Ren-Huei Fu Division of Neonatology, Department of Pediatrics, Chang

Gung Memorial Hospital

Background: Hyperthyroidism is a condition of high

prevalence among women of childbearing age. Thyroid physiology changes significantly during pregnancy, with an increase in maternal serum thyroxine and a decreased TSH, which would inversely correlate to the increase in hCG during the first trimester. This phenomenon underscores the critical role of thyroid hormone on normal fetal development. However, it also creates the dilemma of setting a normal value for screening thyroid dysfunction during pregnancy. Maternal hyperthyroidism is known to cause adverse obstetrical events such as pre-eclampsia, premature labor, and placental abruption. Affected offspring of these women could have small birth weight, arrhythmia, heart failure, or even long term growth failure and neurodevelopmental abnormalities. The aims of this study were to determine in the cohort of VLBW preterm neonates, if maternal hyperthyroidism would have an impact on growth and neurodevelopmental outcome at their 2-years corrected age.

Methods: This is a retrospective case controlled study by reviewing medical records of infants admitted to NICU of Chang Gang Memorial Hospital during 2011 to 2012. We enrolled all newborns of very low birth weight (VLBW,

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62 The Survey Study of Iron Status in Infants Shu-Chi Mu1,2, Yi-Ling Chen1,3, Cheng-Hui Lin1,

Yung-Ting Guo4, Sin-Chang Li5, Chiao-Ming Chen6 Department of Pediatrics, Shin-Kong Wu Ho-Su Memorial

Hospital1; School of Medicine, Fu-Jen Catholic University2; School of Medicine, Taipei Medical University3; Department of Pediatrics, Taipei Medical University Shuang Ho Hospital4; School of Nutrition & Health Science, Taipei Medical University5; Department of food Science, Nutrition, and Nutraceutical Biotechnology, Shih Chien University6

1,21,31456

12345()6

Background: Iron deficiency (ID) and iron deficiency

anemia (IDA) usually occurs in developing countries, ID and IDA might affect infants emotion, cognition and development. Vitamin D is an essential nutrient in infants. Iron levels were lower in breast milk in literature review; it may cause of nutritional lack in infants. The iron nutritional status of infants was never surveyed in Taiwan.

Methods: In order to investigate the nutritional status of iron in infants, we collected 532 infants aged 1 to 12 months and measured serum iron status from October 2012 to January 2014. We also evaluated the anthropometric, food intake including milk and complementary food, hours of sunlight of infants by questionnaires.

Results: In the IDA infants, their mother significantly gained less weight during pregnancy, and there was a significant positive relationship between pregnancy weight gain and infants hemoglobin. Thus, we demonstrated inadequate weight gain during pregnancy might affect infants iron nutritional status. On the other hand, our results confirmed that mother iron supplementation during pregnancy could improve her iron nutritional status, but it was not correlation with infants iron nutritional status. Breastfed infants appeared iron depletion, but no affected the level of hemoglobin in less than 6 months infants. However, the prevalence of ID and IDA was as high as 67 % in 8-12 months breastfed infants. Breast milk seem to contain less iron may cause poor iron status if long term breastfed. Moreover, our data also showed infants hemoglobin level was positive association with total dietary iron intake. We recommended infants should be fed high quality and quantity complementary food in 8-12 month-old to avoid iron deficiency, specially still breastfed infants.

Conclusions: There was no evidence that ID or IDA affected growth and development in infants. We recommended infants should be fed high quality and quantity complementary food in 8-12 month-old to avoid iron deficiency, specially still breastfed infants.

63 The Survey Study of Vitamin D Status in Infants D Shu-Chi Mu1,2, Li-Yi Tsai1, Ling-Jen Wang1, Yung-Ting

Guo3, Sin-Chang Li4, Chiao-Ming Chen5 Department of Pediatrics, Shin-Kong Wu Ho-Su Memorial

Hospital1; School of Medicine, Fu-Jen Catholic University2; Department of Pediatrics, Taipei Medical University Shuang Ho Hospital3; School of Nutrition & Health Science, Taipei Medical University4; Department of food Science, Nutrition, and Nutraceutical Biotechnology, Shih Chien University5

1,211345 12

345

Background: Studies showed that vitamin D deficiency

results in infection, metabolic diseases, and immunity disorders. Severe lack vitamin D can cause of rickets. Vitamin D levels were lower in breast milk in literature review; it may cause of nutritional lack in infants. The vitamin D nutritional status of infants was never surveyed in Taiwan.

Methods: In order to investigate the nutritional status of vitamin D in infants, we collected 532 infants aged 1 to 12 months and measured serum vitamin D status from October 2012 to January 2014. We also evaluated the anthropometric, food intake including milk and complementary food, hours of sunlight of infants by questionnaires.

Results: In the part of vitamin D nutritional status, the prevalence of borderline and deficient was 56 %; it showed poor vitamin D status in 1-12 month- old infants. Mothers height, weight, weight gain and vitamin D supplementation during pregnancy did not affect serum vitamin D concentration of infants, but infants born in winter or spring had better vitamin D level compared to born in summer and fall. In less than 6 months breastfed infants, the prevalence of vitamin D borderline and deficient was 88 %, and the vitamin D concentrations was significant association with breastfeeding frequency per week and the months of exclusive breastfeeding. In our study, a variety of complementary food was introduced to baby diet in 8 to 12 month-old infants, but the feeding frequency of soy/fish/meat/eggs was not correlation with vitamin D level. We found that infants had better vitamin D status by feeding formula, because most formulas were fortified with vitamin D.

Conclusions: Although vitamin D could be produced by exposure sunlight, but we did not known the conversion rate of infants physical and how long outdoor activity was enough to enhance vitamin D level. It need more study to confirm. We found that infants had better vitamin D status by feeding formula, because most formulas were fortified with vitamin D. Babies with hyperparathyroidism may be associated with vitamin D deficiency. Our data showed vitamin D deficiency may elevate PTH level, but not affect babies growth. Whether vitamin D deficiency will affect immune function and bone mineralization, warrants further in depth studies.

31

64 Reduction of Respiratory Syncytial Virus (RSV) Infection Related Hospitalization in the High Risk Preterm InfantsEffects of Palivizumab Prophylaxis

Palivizumab

Hsiao-Han Lu1, Ching-Hu Chung2, Yuh-Jyh Lin1, Chyi-Her

Lin1 Department of Pediatrics, College of Medicine, National

Cheng Kung University Hospital1, Tainan, Taiwan; Department of Medicine, Mackay Medical College2, New Taipei City, Taiwan

1211 12 Background: RSV is a common cause of lower respiratory

tract infection in infants and resulting in considerable morbidities and mortality. Prematurity and chronic lung diseases (CLD) are risk factors for severe RSV infection. Palivizumab prophylaxis reduces hospitalization due to RSV infection in high risk infants. There are, however, few studies on this topic with population data in a subtropical climate.

Methods: We analyzed the national claims data for the National Health Insurance and linked those with birth-report database from 2008 to 2012 in Taiwan. Data of hospitalization due to RSV infection were collected by ICD-9 code 0796, 46611 or 4801 in hospital admission record. Infants with CLD was selected by ICD-9 code 7707, 51881, 51883 or 51884. CLD was defined as infants less than 37 W gestational ages and required oxygen supplementation 28 days. Palivizumab prophylaxis was given to preterm infants with gestational age (GA)

56 7 2015

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respiratory support of preterm infants was positively correlated with flow rate and body weight. Pressure monitored by a non-invasive method via pressure gauge from distal end of nasal cannulae when these small patients using HHHFNC is practicable in NICU. These data provide us the information about the delivered pressure for HHHFNC in VLBW premature infants weaning from NCPAP.

66 PDGFRa Is Required for Mesenchymal Cell

Differentiation and Expansion of Secondary Crest Myofibroblast Progenitors

A(PDGFR)

Chang-Yo Yang, Reyin Lien, Shih-Ming Chu, Changgong

Li1, Zea Borok2, Parviz Minoo1 Division of Neonatology, Department of Pediatrics, Chang

Gung Childrens Hospital and Chang Gung Memorial Hospital; Chang Gung University College of Medicine, Taoyuan, Taiwan; Departments of Pediatrics1, Keck School of Medicine, University of Southern California; Will Rogers Institute Pulmonary Research Center2, Keck School of Medicine, University of Southern California, Los Angeles, California, USA

1-2

Background: Alveologenesis is the major step in lung

maturation and the least understood phase of lung development. Disruption of alveologenesis is associated with the neonatal chronic lung disease, bronchopulmonary dysplasia (BPD). Platelet-derived growth factor receptor-a(PDGFRa) is expressed throughout lung development, with dynamic changes in expression pattern and cell type specificity. Recent studies indicate that PDGFRa is required for alveologenesis. However little is known about the precise role, timing and cell-type specificity of PDGFRa in alveogenesis.

Methods: To address these questions we used Pdgfra floxed mice in two genetic models to target Pdgfra in lung mesodermal cells at different stages of lung development.

Results: Targeted inactivation of Pdgfra in early embryonic mesoderm via Dermo1-cre resulted in neonatal death coinciding in time with the onset of alveologenesis. Pdgfra-Dermo lungs showed severely dilated distal airspaces, disrupted septation, decreased cell proliferation and increased apoptosis. Realtime PCR analyses revealed that Dermo1cre-mediated Pdgfra inactivation altered expression of multiple cell-type specific markers including those for interstitial myofibroblasts (ATCA2), lipofibroblasts (ADRP, WISP2, and FABP4), endothelial cells (Flk-1) and alveolar type 1 (AQP5) cells. Therefore Pdgfra appears essential for differentiation of, or interaction amongst multiple mesenchymal cell progenitors. To understand a cell type-specific functional role of PDGFRa in alveologenesis, we targeted Pdgfra specifically in myofibroblast progenitors by using a regulable cre model,

Gli1creER, which can be activated by time-specific administration of Tamoxifen. Our previous study demonstrated that Gli1creER targets the differentiating myofibroblast progenitors in neonatal lungs. We found that deletion of Pdgfra in Gli1creER targeted cells during early neonatal stages lead to disruption of alveologenesis. The mutant lungs show dilated alveoli with a reduced number of secondary crests at postnatal day 11. Importantly, lack of PDGFRa on secondary crest myofibroblasts reduced their number (identified by ACTA2 positive) in the mutant lungs. To determine whether the reduction of ACTA2 positive cells was caused by alteration of cell fate or cell proliferation, we used GFP to label and trace the Gli1creER targeted cells in both PDGFRa deficient and control lungs and found that the number of GFP positive cells was reduced in the mutant lungs.

Conclusions: These results indicate that PDGFRa is required specifically for the expansion of secondary crest myofibroblast progenitors during alveologenesis.

67 The Neurodevelopmental Outcome of Preterm Infants

with Prenatal Absent or Reversed End-Diastolic Flow Velocity (AREDV) Using Bayley III Scales at 2 years- A Match Control Study

Yi-Yu Su, Chi-Nien Chen1, Chien-Yi Chen, Hung-Chieh

Chou, Wu-Shiun Hsieh, Po-Nien Tsao Department of Pediatrics, National Taiwan University

Childrens Hospital; Department of Pediatrics, National Taiwan University Hospital Hsin-Chu Branch1

1

1 Background: Prenatal placental insufficiency may

significantly contribute to neonatal mortality and morbidity. There are many parameters to evaluate placental function and umbilical artery flow detection is one of the most convincing methods. Fetal Doppler findings with absent or reversed end-diastolic flow velocity (AREDV) are specific poor prognostic factors for neonatal outcome. However, the long-term neurodevelopmental outcome for those preterm infants with AREDV was still controversial. This study aimed to evaluate the neonatal and two-year long-term neurodevelopmental outcomes of those very low birth weight (VLBW) infants with AREDV by Bayley III.

Methods: We retrospectively collected VLBW preterm infants delivered from January 1, 2011 to December 31, 2012. We specifically identified those infants with AREDV by chart review and clinical characteristics were recorded. Those participants with congenital anomalies, missing data, unclear recorded or loss follow up cases were excluded. Neonatal mortality and morbidity were analyzed and compared between AREDV group and a gestational age (GA)-matched control group. In addition, neurodevelopmental outcome measured by Bayley III was also be evaluated.

Results: Twenty-five AREDV cases survived at least 2 yeas

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old and their two-year Bayley III scales were compared to the GA-matched control group with 50 VLBW infants without prenatal AREDV born in the same period. The AREDV group has significantly lower birth body weight, higher incidence of SGA rate and hypoglycemia as compared to the control group. The incidences of major morbidity were similar to control group. In contrast, the two-year Bayley scales in cognitive and language function were significantly lower in AREDV group as (9712.2 vs 105.115.1, p=0.019) and (96.214.9 vs 103.816.2, p=0.047) separately, but not in the motor function (96.38.9 vs 97.213.2, p=0.355). In addition, the AREDV group has more cases with moderate developmental delay in cognitive (20% vs 6%) and language function (20% vs 8%) test but not in motor function (28% vs 32%) as compared to control group.

Conclusions: The AREDV infants indeed have poor neurodevelopmental outcome in the cognitive, language functions but not in the motor function at 2 years. Early intervention and aggressive physical therapy with rehabilitation program would be recommended.

68 Maternal Nicotine Exposure Induces Epithelial-

Mesenchymal Transition in Rat Offspring Lungs Chung-Ming Chen1,2, Hsiu-Chu Chou3, Liang-Ti Huang4 Department of Pediatrics, Taipei Medical University

Hospital1; Department of Pediatrics2 and Department of Anatomy and Cell Biology3, School of Medicine, College of Medicine, Taipei Medical University; Wan Fang Hospital4, Taipei Medical University, Taipei, Taiwan

1,234 1

234

Background: Maternal nicotine exposure induces lung

injuries and fibrosis in rat offspring. Epithelial mesenchymal transition (EMT) following lung injury is a process in which epithelial cells mediate tissue repair. The aim of this study was to determine the effects of maternal nicotine exposure on the EMT in neonatal rat lungs.

Methods: Nicotine was administered to pregnant SpragueDawley rats by using a subcutaneous osmotic minipump delivering a dose of 6 mg/kg/day at Gestational Days 721 or from Gestational Day 7 to Postnatal Day 14. A control group received an equal volume of saline.

Results: The percentage of 8-hydroxy-2-deoxyguanosine- positive cells in nuclear staining was significantly higher, E-cadherin protein expression was significantly lower, and N-cadherin protein expression was significantly higher in the rats born to prenatal and postnatal nicotine-treated dams than in those born to prenatal saline- and nicotine-treated dams on Postnatal Day 7. These characteristics of the EMT were associated with a significant increase in -smooth muscle actin (SMA) expression on Postnatal Day 21. Rats born to the prenatal and postnatal nicotine-treated dams showed significantly higher -SMA expression and total collagen than in those born to the prenatal saline- and

nicotine-treated dams on Postnatal Day 21. The number of cells expressing fibroblast specific protein-1 and vimentin were higher in the rats born to the prenatal and postnatal nicotine-treated dams than in those born to the prenatal saline- and nicotine-treated dams on Postnatal Days 7 and 21.

Conclusions: Maternal nicotine exposure during gestation and lactation induces the EMT and contributes to lung fibrosis in rat offspring.

69 Survey of School Life of Students with Type 1 Diabete 1 Chiao-Fan Chiu1, Fu-Sung Lo1, Feng-Ju Hsieh1, Chi-Wen

Chang2 Division of Pediatric Endocrinology & Genetics, Chang-Gung

Memorial Hospital and Chang Gung University College of Medicine1; School of Nursing, Chang Gung University2

1112 12 Background: Management of type 1 diabetes care is very

complex, including insulin injections, diet control, exercise, and self-monitoring of blood glucose. Diabetic students had to stay in school five days each week, with at least daily 4-8 hours. Therefore, school life affect blood sugar control very much. We recently conducted a questionnaire survey of school life, we expected to help us understand whether or not school principals, mentors, school nurse, classmates, and environment are friendly for diabetic students.

Methods: We received a total of 324 questionnaires, including 124 boys and 200 girls, aged 14.39 4.77 years (4.24 to 21.4 years), onset of age 8.39 4.42 years, 84 elementary school students, 91 junior high school, 66 senior high school, 57 university. 210 diabetic students filled them by themselves. 24 used insulin pumps.

Results: For school support systems, 289 (79.6%) tutor, 282 (77.7%) school nurse, 115 (31.7%) principals, and 251 (69.1%) physical education teacher, knew their disease. 252 (69.4%) students expressed their school teacher, principal or nurse ever actively took care of them. 231 (63.6%) school personnel can help deal with issues related to diabetes. 135 (37.2%) had normally blood sugar testing at school and home with the same frequency. However, others did not have blood sugar testing by the reasons of too busy, or afraid of being seen. 161 (44.4%) had blood test in the classroom, 68 (18.7%) at the health center, 40 (11%) in the toilet. 125 (34.4%) had insulin injections in the classroom, 60 (16.5%) at the health center, and 59 (16.5%) in the toilet.

Conclusions: Most of the principal or school nurse, and physical education teacher knew their morbidity and had active health care. Less than half of the students were willing to have blood sugar test in the classroom. Only one-third of students were willing to have insulin injection in the classroom. This suggested that not only diabetic students and their families needed diabetic education, but also school personnel and community people needed health education. These educations will provide diabetic students safe and friendly environment.