• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.186-190
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• 2014

Citrullinemia type 2 (citrin deficiency) is an autosomal recessive inborn error metabolism, caused by the SLC25A13 gene mutation. Citrin deficiency is associated with two clinical phenotype; neonatal-onset type II citrullinemia (CTLN2), also known as neonatal intraphepatic cholestasis caused by citrin deficiency (NICCD) and adult-onset CTLN2. Clinical manifestations of NICCD include poor growth, intrahepatic cholestasis, liver dysfunction and increased plasma citrulline, methionine, threonine, arginine. The molecular diagnosis could be confirmed by SLC25A13 gene mutation analysis. A 3-month-old male infant with persistent jaundice was referred for evaluation. Newborn screening was normal at birth. Mild elevation of serum ammonia and AST/ALT were observed. Plasma amino acid analysis showed significantly elevated citrulline, methionine, threonine. DNA sequence analysis of the SLC25A13 gene revealed two compound heterozygous mutations, c.[852_855del]($p.Met285Profs^*2$) and [1180+1G>A]. We suggest that NICCD should be considered as one of the cause of in infants with cholestatic jaundice, although the newborn screening was normal.

• Child Health Nursing Research
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• v.19 no.3
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• pp.198-206
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• 2013

Purpose: This study was done to identify the effectiveness of the 'intervention - mother's recorded song' on low-birth weight infants in an neonatal intensive care unit (NICU). Methods: This study was conducted with a nonequivalent control group pre-posttest quasi-experimental design and the participant group was low-birth weight infants who were admitted to the NICU. Forty-eight infants, 24 in each from two groups, the experimental and control group, participated in the study. Results: For physical response according to vital signs, there were no significant statistical differences in heart rate, respiration rate and pulse oximetry saturation between the experimental group and the control group. For behavioral state, there was a significant statistical difference between the experimental and control group. Conclusion: The study results indicate that the intervention using mother's song had some significance as a nursing intervention with positive impacts. Such an intervention can help pediatric nurses improve infants' stabilization of their vital signs and behavioral states. By showing the effectiveness of such an intervention, the results of this study provide further evidence-based information in developing the practice of pediatric nursing.

• Clinical and Experimental Pediatrics
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• v.50 no.7
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• pp.698-701
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• 2007

We report on two premature infants who developed nosocomial infection caused by Chryseobacterium meningosepticum in a neonatal intensive care unit (NICU). One premature infant developed sepsis, meningitis, and hydrocephalus, and was treated successfully with ciprofloxacin plus trimethoprim-sulfamethoxazole combination therapy for 4 weeks and with a ventriculoperitoneal shunt. The other premature infant, who was in a chronically debilitated state, had infection that had colonized only in the respiratory tract but had no clinical signs for 66 days. Extensive environmental surveillance demonstrated that the suction bottle apparatus was the source of infection. We prevented the spread of infection by closing the NICU temporarily, isolating the patients early in their infection, and eradicating the source of infection source.

• Journal of Chest Surgery
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• v.31 no.10
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• pp.931-938
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• 1998

Background: Anatomic correction of transposition of the great arteries by means of the arterial switch operation is now accepted as the therapeutic method of choice. This retrospective study attempts to assess the results of the neonatal arterial switch operation for transposition of the great arteries performed by our newly established institution. Materials and methods: 33 consecutive neonates underwent the arterial switch operation between October 1991 to November 1997. There were 27 neonates with transposition and intact ventricular septum, 3 with ventricular septal defect, and 3 with Taussig-Bing anomaly. The mean age was 10.9$\pm$7.9 days and mean body weight was 3.29$\pm$0.44kg. Results: Overall postoperative hospital mortality was 30.3% (10 patients). The mortality has improved with time; 75% (6 patients) among first 8 consecutive patients before 1994, 20% (2 patients) among 10 patients in 1994 and 1995, and 13.3% (2 patients) among 15 patients since 1996. Univariated analysis of risk factors revealed that earlier date of the operations and one of preoperative events were determinants for operative death. There were two late deaths. A mean follow-up of 17.4$\pm$16.5 months was achieved in all 21 survivors. All were in New York Heart Association functional class I. One patient had mild pulmonary stenosis and two had mild aortic valve regurgitation on their echocardiography. Conclusions: We concluded that we should continue to perform arterial switch operation for neonates with transposition of the great arteries because the mortality of the operation has been improved and the operative survivors have good functional results with low incidence of late complications.

• Neonatal Medicine
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• v.18 no.1
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• pp.137-142
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• 2011

Purpose: In relation to perinatal healthcare, medical institutions and resources are limitative and also are in a state of flux due to the therapeutic specialty. We analyzed requests for interhospital transfers received by Busan 1339 Emergency Medical Information Center (EMIC) to grasp the state of perinatal healthcare delivery system. Methods: This study was conducted on the basis of data inputted into the computing system of Busan 1339 EMIC, between January 1 and December 31, 2009. In connection with 378 pregnant women and 136 newborns who were required to transfer, retrospective analyses were made of the success rate of transfer (SR), the number of contacted hospitals, the time required for transfer and the reason of transfer and refusal. Results: In the case of pregnant women, the SR were 65.5%. They came in contact with 2.7 hospitals, and it took 24.4 minutes. As for the reason of transfer, preterm labor accounted for the highest proportion. In the case of newborns, the SR were 71.3%. They came in contact with 2.4 hospitals, and it took 15.6 minutes. The most common reason of transfer were respiratory symptoms. In the reason of refusal with pregnant women and newborn, the lack of medical staff, medical equipments and wards accounted for great. Conclusion: Many pregnant women and newborns have been transferred to hospitals by EMIC, but the SR has not been higher yet. Accordingly, there is a need to evaluate the propriety of perinatal treatment system, as well as to set up effective perinatal healthcare delivery system.

• The Microorganisms and Industry
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• v.13 no.1
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• pp.21-27
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• 1987

우리나라의 생명공학은 지난 1970년대초 광생물질의 개발, 식품공업의 발전및 농업기술의 개발과 함께 생명공학과 관련되는 산업을 꾸준히 발전시켜 왔으나 전반적으로 기술이 낮은 던계에 머물러 있었고, 1980년대에 들어와서 선진국으로부터 각종기술의 도입과 협력연구 등으로 상당한 수준에 이르게 되었다. 이와 더불어 생명공학의 중요성을 인식하고 국책연구기관및 민간기업을 중심으로 신생명공학기술의 개발에 역점을 두어 기술수준의 제고에 노력한 결과, 몇몇 제품분야에 있어서는 상당한 발전을 보게 되었다. 그러나 미국, 일본을 비롯한 선진국 수준에는 전반적으로 미치지 못하고 아직 초기단계를 벗어나지 못한 상태에 있다. 그러나 정부의 적극적인 지원정책에 힘입어 미간기업의 주도적 연구협력체인 유전공학 연구조합(1982년 3월 설립)과 국책연구기관인 유전공학센터(1985년 2월)가 각각 설립되어 특히 신생명공학의 응용및 개발연구를 활발하게 추진함은 물론 학계에서도 정부정책에 부응하여 관련학과를 신설하는등 관련기초 전문의 교육및 연구를 위하여 최선을 다하고 있으므로 1990년대 이후에는 분야에 따라서는 선진국 수준에 이를 전망이다.

• Journal of Korean Academy of Nursing
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• v.9 no.2
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• pp.7-15
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• 1979

This study was undertaken to determine .the identify the relation between the high risk mother and their baby which then allows the nurse to assess and plen for the delivery of optimal health care to the high risk groups. This study was carried out between January through December 1978. This study sample consisted of 300 pregnant women who visited Ewha womens hospital during this time. The method used to for the collection of data was an“Antepartum High-risk pregnancy scoring form. The questionair included 4 categories: 1) reproductives history 2) Associated conditions 3) pre-sent pregnancy and 4) total risk score . The bind are as follows: 1. The frequency of high risk pregnancy women 149(49.7%) was highest. 2. In the investigation sample high risk factors were related to hypertension and toremia. 3. There was a difference in the high risk scores and newborn babys scores (r = 0.610). 4. Relationship between high risk pregnancy women and least of pregnant women was highest prenatal mortality. Implications of positive assessing of high risk factors by MCH nurse and community health nurses.

• Journal of Chest Surgery
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• v.32 no.1
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• pp.62-65
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• 1999

Infantile lobar emphysema is an uncommon disease affecting newborns and infants with varying degree of respiratory distress, lobar overaeration, mediastinal shift and herniation. Although the etiology of the condition is most commonly idiopathic, there is a clear association with congenital heart disease, particularly in the presence of pulmonary hypertension due to left to right shunt. Sites of predilection are the left main bronchus, the left upper and right middle bronchi. This report describes a two-week-old boy who had right middle lobe emphysema with large ventricular septal defect. At first, patch closure of perimembranous ventricular septal defect was performed. Postoperatively, the patient required continuing assisted ventilation and the lobar emphysema was not improve. One week following the initial operation, right middle lobectomy was successfully performed and the patient was weaned from artificial ventilator on the 5th postoperative day. The patient was discharged with good general condition on the 45th postoperative day.

• The Journal of the Korean dental association
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• v.10 no.5
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• pp.307-316
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• 1972

신생 Ball/c Strain 종 웅성백서 28두를 사용하고, 28두중 14두는 실험군에, 나머지 14두는 대조군에 각각 배당하였다. 실험군과 대조군은 동물희생시간(30분, 1시간, 4시간, 8시간, 12시간, 24시간 48시간)에 따라서 7군으로 구분하고, 각군에 2두식 배정하였다. 실험군은 백서를 Bell Jar내에 넣고, 여기에 창소를 서서히 넣어 Westinghouse Oxygen Analyzer로 30내지 40ppm이 되도록 하고 15분 동안 방치한 후, 이를 밖으로 제거하여 신선한 공기를 5분동안 마시게 하였다. 이런 과정을 반복하여 결국 55분 동안에 45분간 무 산소 상태에 있게 하였다. 악하선의 소편을 2% Paraformaldehyde(0.1M Cacodylate fuffer) 용액에 4시간동안 고정하였고, 탈수후 전현 표본포리법에 따라 Epoxy유지를 혼합하여 이몰하였다. LKB Ultratome으로 1μ의 연속절편을 만들었고 야기시킨후 신생백서의 악하선세포에 세포학적변화를 보고저하였고, 기결과를 요약하면 다음과 같다. 1. 신생 백서에 산소결핍을 야기시켰을때에 나타나는 초기의 세포학적변화는 이를 이르킨 후 1시간과 4시간사이가 절정에 달하였다. 이실험에서 유해적 영향은 공포화하고 점액성 및 장액성세포의 핵이 농축변성하며 장액성분필과기의 방출이 지연되어 세포질첨단에 축적되었다. 이들의 회복은 적어도 4시후에 시작되과, 12시간에는 세포질의 대부분, mrgl 핵의 용태가 정상으로 되돌아간다. 24시간에는 실험군과 대조군간에 있어서 야간의 차이는 인정되나 선소엽은 아직 초기에 볼수있었든 퇴행성변화의 잔여물을 함유하고 있었다. 2. 위에 요약한 본연구의 결과는 노인들이 보고한 산소결핍증이 악하선에 있어서도 단백질합성의 억제현상을 나타냈음을 세포학적으로 확증한 것이라하겠다.

• Clinical and Experimental Pediatrics
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• v.49 no.9
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• pp.966-971
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• 2006

Purpose : 'Programming' describes the process that stimulus at a critical period of development has lifelong effects. The fact that low birth weight links to the risk of elevated blood pressures in adult life is well known. This study aims to examine whether this link is evident in the newborn by investigating the relationship of the intrauterine growth indices and neonatal blood pressure(BP). Methods : We studied 127 neonates who were born at Ewha Womans' Hospital and their mothers enrolled our cohort study during pregnancy. Data on the mothers and details of the birth records were tracked and collected from medical charts. Neonatal BP was measured within 24 hours after birth. Results : Neonatal SBP was positively correlated to intrauterine growth indices; birth weight(BW)(r=0.4), head circumference(HC)(r=0.4), and birth height(r=0.3). However, an inverse relationship existed, between HC/BW ratio and neonatal SBP(r=-0.4). After adjusting for the baby's sex, maternal BP, and gestational age, neonatal SBP still associated with intrauterine growth indices. SBP was 7 mmHg higher in the highest BW group(${\geq}90percentiles$) compared to the lowest group(<10 percentiles). On the other hand, SBP was 17 mmHg lower in the highest HC/BW group(${\geq}90percentiles$) compared in the lowest group(<10 percentiles). Conclusion : This study could not find the evidence that intrauterine growth retardation affect on elevated neonatal BP. It suggests that the initiating events of BP programming may occur during postnatal growth period. To identify the critical starting period that intrauterine growth retardation leads to elevated BP, a study tracking BP changes from birth to childhood is required.