• Journal of Chest Surgery
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• v.38 no.12 s.257
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• pp.815-820
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• 2005

Background: Pulmonary atresia with intact ventricular septum is a rare congenital cardiac disease. There have been various surgical options, but there are still controversies. Material and Method: Fourteen neonates who were operated on between 1999 and 2000 were enrolled in this study. We measured Z-value of tricuspid valve by echocardiography. We performed right ventricular outflow tract transannular patch in 9 patients. Modified Blalock-Taussig shunt was placed according to the condition of patients. Mean follow up period was 46.0 months. Result: There were 2 operative mortality and 1 late mortality. Biventricular repair was possible in 7 patients. One and a half ventricular repair were done in 3 (tricuspid valve Z-value was -2, -2.5 and -3) and single ventricular repair in 2 patients (tricuspid z-value was -4.6, -4.5) The tricuspid valve Z-value for the patients who had biventricular repair and one and a half ventricular repair were -0.8$\pm$1.50 (-3.2$\∼$1.2) and -2.5$\∼$0.5 (-3$\∼$ -2) respectively. All patients who survived had fair to good right ventricular function, good left ventricular function and good clinical states. Conclusion: Neonatal surgical management of pulmonary atresia with intact ventricular septum has a good surgical outcome, z-value of tricuspid may be helpful for the determination of surgical options.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.179-185
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• 2004

Purpose: Perinatal mortality rates have been used as a summary statistic for evaluating child health and medical status. Neonatal mortality rates have decreased over the past 30 years in Korea. To understand the current status of neonatal surgical gastrointestinal diseases in Daegu Busan area, we have studied about neonatal gastrointestinal diseases with their clinical features, postoperative outcome, and mortality rates. Methods: A clinical analysis on 202 neonates who underwent neonatal surgery from January 1996 to July 2003 at Pusan National University, Kyungpook National University, Youngnam University, and Daegu Catholic University was carried out. Results: The main diseases of surgical conditions were anorectal malformation (23.8%), atresia/stenosis of midgut (13.4%) and pyloric stenosis (13.4%). The male to female ratio was 2.8 : 1. Thirty-five cases (17.0%) had one or more associated anomalies including congenital heart disease, cryptoorchidism, hydronephrosis, and chromosomal anomaly. Twenty cases (10.0%) were diagnosed by antenatal ultrasound. Patients with esophageal atresia had the longest hospitalization for 54.6 days. Postoperative complications occurred in 18 cases (8.9%). The main postoperative complications were wound infection (3.5%) and anastomotic leakage (2.5%). Overall mortality was 5.9%. Diaphragmatic hernia showed the highest mortality rate (37.5%), and esophageal atresia (28.6%) and omphalocele (20.0%) were followed. Conclusion: The current status of neonatal surgical gastrointestinal diseases in Daegu Busan area has improved because the disease categories are various, postoperative complications and mortality rates are decreased.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.1
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• pp.31-37
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• 2017

Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is an autosomal recessive disorder of the urea cycle that causes hyperammonemia. Two forms of CPS1D are recognized: a lethal neonatal type and a less severe, delayed-onset type. Neonatal CPS1D cases often present their symptoms within the first days of life. Delayed-onset cases are predominantly adolescents or adults, and infantile delayed-onset cases are rare. Severe hyperammonemia in the neonatal period leads to serious brain damage, coma, and death if not treated promptly. Therefore, early diagnosis and acute treatment are crucial. Despite the improvement of treatments, including continuous hemodialysis, ammonia-lowering agents, and a low-protein diet, the overall outcome of severe forms of hyperammonemia often remains disappointing. As the liver is the only organ in which ammonia is converted into urea, liver transplantation has been considered as an elegant and radical alternative therapy to classical dietary and medical therapies. However, liver transplantation has many disadvantages, such as a considerable risk for technical complications and perioperative metabolic derangement, especially in neonates. Additionally, there is a lack of suitable donor organs in most countries. According to recent studies, liver cell transplantation is a therapeutic option and serves as a bridge to liver transplantation. Here, we report a Korean CPS1D patient with novel mutations in CPS1 who was treated by liver cell transplantation after being diagnosed in the neonatal period and showed a good neurodevelopmental outcome at the last follow-up at six months of age.

• Journal of Dairy Science and Biotechnology
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• v.33 no.3
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• pp.179-196
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• 2015

Because of our aging population, there is increasing concern about the impact of dementia and age-related cognitive decline. Intense research efforts on effective dietary interventions for the prevention or amelioration of dementia and age-related cognitive decline have indicated that dairy products affect physiological health and potentially healthy brain function during aging. Milk is a rich source of proteins and peptides with nutritional and immunotropic activities. The preparation of biologically active proteins and peptides generally requires enzymatic degradation, chemical modification, or the addition of specific co-factors. Milk-derived preparations are widely available in the food industry in the form of hygiene products and infant formulas. However, milk-derived products could also be applied as preventive or therapeutic measures for a wide-range of pathological conditions not only in neonates and infants but also in adults, including the elderly. Because they have no adverse side effects, milk-derived proteins and peptides could be used as a supplementary treatment for dementia and age-related cognitive decline.

• Neonatal Medicine
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• v.18 no.2
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• pp.353-358
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• 2011

Purpose: The goal of this study was to compare the efficacy of oral 25% dextrose treatment or/and pacifier for analgesia in healthy newborns during intramuscular injection of a hepatitis B vaccine. Methods: A prospective, randomized, partially blinded, clinical trial was performed in 132 healthy newborns. They were assigned randomly to 4 treatment groups: control group (2 mL distilled water), dextrose group (2 mL 25% dextrose), pacifier group, dextrose+pacifier group (pacifier coating with 25% dextrose) during intramuscular injection of hepatitis B vaccine. For all groups, Neonatal Infant Pain Scale (NIPS), Neonatal Facial Coding System (NFCS), Premature Infant Pain Profile (PIPP) scores were evaluated before the injection, during the injection, and at 2 minutes after the injection. Pain scores were compared among the 4 groups. Results: Maternal and neonatal characteristics were similar among the 4 groups. 25% dextrose treatment led to lower NIPS pain scores during injection (6.4${\pm}$0.9 vs. 5.5${\pm}$1.7, P=0.01) and after injection (1.6${\pm}$2.0 vs. 0.6${\pm}$0.9, P=0.01) and NFCS pain scores after injection (1.5${\pm}$2.3 vs. 0.7${\pm}$0.8, P=0.04) than control group. The number of neonates who feel the pain (indication of scores: NIPS${\geq}$4, NFCS${\geq}$3) decreased (9 (23.1%) vs. 0 (0%), P=0.04 via NIPS, 7 (17.9%) vs. 0 (0%), P=0.02 via NFCS). However, all treatment groups did not decreased PIPP scores, compared with the control group. Conclusion: Oral 25% dextrose is effective than distilled water or using pacifier with or without 25% dextrose in reducing pain during intramuscular injection of hepatitis B vaccinations. Further study based on this preliminary study need about nonpharmacologic management of pain in newborns.

• Pediatric Infection and Vaccine
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• v.12 no.2
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• pp.186-194
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• 2005

Purpose : Hepatitis A viral infections have been continued after re-emerging since mid 1990s in Korea. The incidence of this disease has been increased in young adults younger than 30 years of age since 2000. This study was performed to evaluate the prevalence of antibody to hepatitis A in Korea(two regions; Incheon and Changwon) in 2005, and was compared with the results of similar studies in mid 1990s. Methods : The study was conducted from January 2005 to June 2005, and consisted of 1,301 enrolled subjects, neonates to 50 years old, living in Incheon and Changwon in Korea. All sera were frozen and stored at $-70^{\circ}C$ until assayed. Anti-HAV IgG antibodies were measured by microparticle enzyme immunoassay(HAVAB, Abbott Lab., IL, USA). Results : The prevalence of anti-HAV IgG was 61.1% in infants younger than 1 year old, 30.5% in 1~5 years, 14.6% in 6~10 years, 1.7% in 11~15 years, 6.5% in 16~20 years, 36.6%in 21~30 years, 77.5% in 31~40 years, and 99.8% in 41~50 years. Statistical differences were not found between male and female, but there was statistical difference in 6~10 years old age group between the two areas. Conclusion : Our study indicate that the prevalence of antihepatitis A virus antibody has shifted from children to old adolescents and young adults. This result suggests that the risk of sudden outbreaks or increasing incidence of hepatitis A viral infections in young adults may be expected in our society. The preventive strategies of hepatitis A including vaccination should be prepared.

• Pediatric Infection and Vaccine
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• v.4 no.1
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• pp.106-115
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• 1997

Objective : To evaluate the immunogenicity and safety afforded by the HG-II$^{(R)}$ recombinant hepatitis B vaccine given to healthy neonates and children and to find the influence of preceding BCG vaccination on immunogenicity. Methods : Three doses of recombinant hepatitis B vaccine with a dose of $10{\mu}g$ were given at birth, 1 and 6 months of age. This study was conducted in three hospitals (Gyeongsang National University Hospital(Group A), Kangnam General Hospital(Group B) and Younsei University Hospital(Group C)) from April, 1995 to June, 1996. Group A and Group B received 2nd dose of hepatitis B vaccine at 1 week after and before BCG vaccine, respectively. Antibidy levels, at 1 month after the 3rd dose of hepatitis B vaccine were determined by a radioimmunoassay. Results : 1) One hundred four infants and ten children were enrolled : 55 infants and 43 infants received 2nd dose of hepatitis B vaccine at 1 week after( After BCG Group) and before BCG vaccine(Before BCG Group), respectively. 2) The seropositive rate was 99.1%, and geometric mean anti-HBs titer was 131.2mIU/ml. 3) The geometric mean titers were 105.5mIU/ml and 162.8mIU/ml in After BCG and Before BCG Group, respectively(p<0.025). 4) Among 359 episodes of vaccination, the occurrence of systemic and local side reaction were reported in 7.8% and 1.4%, respectively. Conclusion : Recombinant hepatitis B vaccine(HG-II$^{(R)}$))was highly immunogenic and safe. The significantly lower geometric mean antibody titer in the BCG preceding group was observed. Well-designed controlled study with the large number of sample size will be required to show the influence of preceding BCG vaccination.

• Pediatric Infection and Vaccine
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• v.12 no.2
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• pp.149-156
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• 2005

Purpose : Acute septic arthritis and acute osteomyelitis are not rare diseases in pediatric population. But when the diagnosis is delayed or inappropriate treatments are given, permanent disabilities of joint or bone can be followed. We analysed clinical manifestations, laboratory findings, X-ray findings, causative microorganisms and antibiotic susceptibility results of the two diseases in children. Methods : During January 1992 and May 2002, we conducted a retrospective study of 103 children who were diagnosed as acute septic arthritis and acute osteomyelitis. We selected out 34 children who had positive culture results in the blood or involved sites. Results : 19 cases were diagnosed as acute septic arthritis and 15 cases were acute osteomyelitis. These diseases were most common in preschool children and next in neonates. Hip joints and tibia were the most common sites in each disease. X-ray findings showed abnormalities in 6 cases(36%) of acute septic arthritis and 7 cases(50%) of acute osteomyelitis on admission. The most common microorganism isolated from the involved sites was Staphylococcus aureus; 12 out of 14 cases in acute septic arthritis and 6 out of 13 cases in acute osteomyelitis. Conclusion : It is difficult to make a clear initial diagnosis of the two diseases. We could not find any differences between these two diseases on clinical manifestations such as fever, swelling, tenderness and limitation of movements in joint and bone. The most common microorganism was Staphylococcus aureus.

• Journal of Chest Surgery
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• v.38 no.4 s.249
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• pp.284-290
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• 2005

We evaluated the early and mid-term results for repair of Ebstein's anomaly. Material and Method: Between January 1989 and June 2004, 29 patients underwent repair of Ebstein's anomaly. The median age was 11.4 years (4 days-50 years). Tricuspid insufficiency equal to or greater than grade 3 was present in 21 $(72.4\%)$ patients. Surgical techniques included tricuspid valve repair with vertical plication of the atrialized ventricle (n=14), Carpentier's technique (n=7), tricuspid valve replacement (n=4), systemic-to-pulmonary arterial shunt (n=2), tricuspid valve repair (n=1), and Fontan operation (n=1). Bi-directional cavopulmonary shunt (BCPS) was required in 5 patients. Among the 2 neonates, one patient underwent successful biventricular repair, and the other patient underwent systemic-to-pulmonary arterial shunt. Follow-up was possible in 21 patients $(75\%)$, and the average follow-up was 37.6 months (3 months-11.3 years). Result: There were $1(3.4\%)$ early and 1 late deaths. Reoperation was required in 4 patients. Two patients underwent tricuspid valve re-replacement, and the other 2 tricuspid valve repair. At recent follow-up, only 2 patients showed tricuspid insufficiency equal to or greater than grade 3, and most patients showed clinical improvement. Excluding the patients who underwent tricuspid valve replacement, the actuarial rate of freedom from reoperation at 1 and 5 years were $94.7\%\;and\;79.0\%$, respectively. Conclusion: Tricuspid valve repair was possible in most patients with good mid-term outcome. Most patients showed clinical and hemodynamic improvement. Indications for the BCPS should be clarified.

• Clinical and Experimental Pediatrics
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• v.50 no.12
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• pp.1194-1199
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• 2007

Purpose : It was generally accepted now a days that the pathogenesis of preeclampsia, small for gestational age (SGA), intrauterine growth retardation and fetal origin of adult diseases were related with a endothelial cell dysfunction. The purpose of this study was to know the relation of such diseases by assessing the level of endothelin-1. Methods : SGA babies, newborns of preeclampsia and normal control mother were included in this study. Isolated endothelial cells were centrifugated and mixed with media in $37^{\circ}C$, 5% $CO_2$ to obtain confluent monolayer of cultured human umbilical venous endothelial cell (HUVEC). Endothelin-1 levels were determined by Endothelin-1 colorimetric (EIA) Kits. We examined the endothelin-1 level in the HUVEC supernatants from SGA baby, and newborns from preeclampsia as well as normal mother. Also, we compared the endothelin-1 level of cultured normal HUVEC incubated with serum from cord blood of SGA, babies of preeclampsia or normal control mother. Results : The endothelin-1 levels in cultured HUVEC supernatants of three groups showed no significant difference but the endothelin-1 levels of cultured normal HUVEC incubated with serum from preeclampsia mother or SGA mother was significantly higher than those from newborns of control mothers (P<0.05). Conclusion : These findings suggest that there may be the factor which affect the endothelin-1 level in serum of cord blood from SGA and preeclampsia.