• Clinical and Experimental Pediatrics
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• v.51 no.3
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• pp.315-322
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• 2008

Purpose : Prader-Willi syndrome (PWS) is a complex genetic disorder, caused by the deletion of the paternally derived 15q11-13 region or the maternal uniparental disomy of chromosome 15 (mUPD(15)). In this study, we compared phenotypic differences between those patients whose disease was caused by microdeletion and those caused by mUPD(15). In addition, a comparison of the efficacy of growth hormone (GH) therapy between these two PWS genotypes was analyzed. Methods : Fifty-three patients were diagnosed as having PWS based on molecular and cytogenetic analyses and clinical features. Data that included maternal age, birth weight, a feeding problem in the neonatal period, cryptorchidism, developmental delay or mental retardation, short stature, hypopigmentation, changes in height, weight, and body mass indexes (BMI) before and after GH treatment were obtained by a retrospective review of medical records. The data from the patients with microdeletion were compared with those from the patients with mUPD(15). Results : Of the 53 patients with genetically confirmed PWS, 39 cases had microdeletion and 14 mUPD(15). Maternal ages were significantly higher in the mUPD(15) group, and hypopigmentation and a feeding problem in the neonatal period were more frequent in the microdeletion group. Growth hormone was administered to 20 patients [14 with microdeletion, 6 with mUPD(15)]. There were no differences between the two groups in height velocity, weight and height SDS, and BMI after GH therapy. Conclusion : Phenotype and genotype correlations were observed in Korean PWS patients, such as more advanced maternal ages in the mUPD(15) group and more feeding problems and hypopigmentations in the microdeletion group. Further long-term prospective studies are needed to correlate other aspects of the phenotypes.

• Clinical and Experimental Pediatrics
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• v.51 no.4
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• pp.372-376
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• 2008

Purpose : To identify clinical characteristics of severe respiratory syncytial virus (RSV) in neonatal period and early infancy and provide information in clinical practice. Methods : Twelve neonates and young infants (<6 months) who were infected by respiratory syncytial virus and required mechanical ventilation between March 2005 and July 2007 were enrolled. Diagnosis of RSV infection was made based on the positive results by rapid antigen immunoassay or polymerase chain reaction. Results : There were four premature infants, of whom three were near-term. Birth weight of subject patients was $2.8{\pm}0.6kg$, gestational age was $37{\pm}2weeks$ and the age at the time of admission was $35{\pm}15days$. Nine of them showed apnea and in five patients, apnea itself was an indication for mechanical ventilation. In seven of the apneic patients, apnea was the first manifestation of RSV infection. In three of these seven apneic patients, apnea preceded definite respiratory distress signs or typical stethoscopic findings by 1-3 days. Mean duration of mechanical ventilation was $3{\pm}2days$, and mean duration of stay in intensive care unit was $6{\pm}2days$. Conclusion : RSV is a major cause of severe respiratory tract infection in term or near-term infant younger than 2 months. For apnea could be the first manifestation of the RSV infection, high level of suspicion is required in practice of neonates or young infants who show any upper respiratory infection symptoms during RSV season.

• Clinical and Experimental Pediatrics
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• v.46 no.12
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• pp.1194-1199
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• 2003

Purpose : To determine the change in the limit of viability in fetal infants and the incidence of major complications in neonatal intensive care unit(NICU) at Samsung Medical Center(SMC). Methods : Sevety-three infants weighing less than 800 g at birth and 117 infants born earlier than 27 weeks gestational age, who were admitted to NICU at SMC between November 1994 and December 2002, were enrolled. Their medical records were reviewed with a focus on the survival and major complications, including chronic lung disease, retinopathy of prematurity, intraventricular hemorrhage, and periventricular leukomalacia. The limit of viability in fetal infants was defined as the birth weight or gestational age at which a 50% survival rate is reached and the incidence of major complications were compared by period(period I : Nov. 1994-Dec. 1998, period II : Jan. 1999-Dec. 2002). Results : The limit of viability in fetal infants was markedly lowered from birth weight range of 800-900 g or gestational age range of $26^{+0}-26^{+6}$ weeks in period I to birth weight range of 600-700 g or gestational age range of $24^{+0}-24^{+6}$ weeks in period II. The incidence rates of major complications at the limit of viability were all less than 50% in period II. There was no definite trend toward increased incidence of major complications as birth weight get smaller and gestational age get younger. Conclusion : Our results indicate that at present, fetal infants whose body weight is over 600 g, or whose gestational age is over 24 weeks should be resuscitated vigorously. The risk of major complications at this birth weight or gestational age seems to be not greater than that at a bigger birth weight or an older gestational age.

• The Journal of Korean Obstetrics and Gynecology
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• v.26 no.4
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• pp.82-93
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• 2013

Purpose: The aim of this study was to investigate the relation between peripheral blood leukocyte differential - Granulocyte rate, Lymphocyte rate and its calculated Granulocyte/Lymphocyte ratio - and the obstetric variables. Methods: From 270 cases of women who were in the postpartum care center attached to Won-Kwang Korean Medical Hospital, 36 cases met exclusion criteria. On the obstetric variables of 234 cases, peripheral blood leukocyte differential - Granulocyte rate, Lymphocyte rate and its calculated Granulocyte/Lymphocyte ratio - was analyzed. In the postpartum care center, the obstetric variables were asked of 234 cases of women, and who measured leukocyte differential. Results: Mean granulocyte rate and lymphocyte rate of peripheral blood leukocyte differential showed statistically significant difference from those in healthy people and was high. There was statistically significant difference in granulocyte/lymphocyte ratio according to mood of delivery, parity and period passed from childbirth. But whether postpartum women with anemia or not, maternal age and gender of neonate were not associated with granulocyte/lymphocyte ratio, and which was not significantly correlated with gestational age and neonatal birth weight. Conclusions: These findings suggest that activation of the sympathetic nervous system in postpartum women is higher than in healthy people. And granulocyte/lymphocyte ratio was influenced by mood of delivery, parity and period passed from childbirth. In future, more studies or surveys, with less bias, for the relation between peripheral blood leukocyte differential and the obstetric variables are required.

• Journal of Nutrition and Health
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• v.23 no.2
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• pp.124-134
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• 1990

To investigate nutritional and growth status of premature infants, survey was conducted against 19 premature infants admitted to Nursery of Pediatrics, Young nam University Hospital from Jan. 1984 to July 1988. Variations in feeding patterns as analyzed through the five feeding phases of dextrose, dextrose/hyperal, dextrose/formula, dextrose/hyperal/formula and formula. During 30days from birth, dietary intake, feeding phase, selective anthropometric measurements, biochemical parameters and demograpic information of premature infants were as follows: Distributions of birth weight by gestational age and apgar score showed preamture infants below 1499g were 31 weeks, 4/7(1min/5min) and premature infants from 1500g to 1999g were 33 weeks, 8/9(1min/5min) and premature infants from 2000g to 2500g were 33 weeks, 8/9(1min/5min). Average calorie intakes of premature infants below 1499g, premature infants from 1500g to 1999g and premature infants from 2000g to 2500g were 102.0㎉/kg/day, 119.3㎉/kg/day and 101.7㎉/kg/day. The mean values Na, K, Ca, P for all premature infants remained within the normal level for full term infants throughtout the 25days of this study period. Percentages of inital weight loss showned premature infants below 1499g were 5.4%, premature infants from 1500g to 1999g were 6.4%, premature infants from 2000g to 2500g were 11.4%. Percentages of inital weight loss of the amino acid injection group and the amino acid control group were 9.4% and 9.0%.

• Advances in pediatric surgery
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• v.12 no.2
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• pp.167-174
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• 2006

Central venous catheter (CVC) for long-term venous access is indispensable for various reasons including hyperalimentation, frequent blood sampling, frequent IV drug use in pediatric patients. We report clinical experience of surgical neonates in whom CVC was inserted primarily via great saphenous vein into suprarenal inferior vena cava. From March 2004 to March 2006, we performed CVC insertion via saphenous vein - contralateral side to main wound - into suprarenal inferior vena cava in surgeries of neonates. 2.7Fr or 4.2Fr, single lumen, tunneled Broviac catheters (Bard Access system, Inc, Salt Lake City, Utah) were used. Skin exit site of tunneled catheter was located in ipsilateral flank area just below edge lower rib. At the end of the procedure, location of the catheter tip was confirmed by plain radiography of abdomen. We retrospectively reviewed the admission records of the patients including nursing staff charts. Nine (50.0 %) patients were male and nine (50.0%) were female. Median gestational age was 38 weeks (range, 29-42 weeks) and median birth weight was 3,105 gm (range, 1,040-3,720 gm). Median age at catheter insertion was 38.5 days (range, 1-236 days). The purpose of CVC insertion was short-and long-term hyperalimentation in nine (50.0 %) patients. CVC insertion was performed in operation room under general anesthesia in sixteen (88.9 %) patients (in these cases, CVC insertion was performed just prior to concurrent operation) and neonatal intensive care unit (NICU) under local anesthesia with adequate sedation in two (11.2%). During the admission period (total catheter-indwelling time: 553 days), CVC functioned well without any significant side effects. Transient swelling of the ipsilateral leg (n=1, 5.6 %) and transient migration of catheter tip (n=1, 5.6 %) were noted, which did not affect function of the indwelled CVC. Mean catheter-indwelling time was 30.7days (range, 3-72 days). All catheters were removed electively except two mortality case. Complications, such as thrombosis, infection, kinking or extravasation of drugs, were not observed in our study period. Tunneled trans-great saphenous vein inferior vena cava catheters are not only comparable to cervical CVCs in terms of function and complication rates, but also very beneficial in selected patients, especially those in whom cervical approach is technically impossible or contraindicated.

• Clinical and Experimental Pediatrics
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• v.48 no.12
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• pp.1324-1329
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• 2005

Purpose : The aim of this study was to evaluate recent improvements in the survival rate of extremely low birth weight (ELBW) infants and to identify perinatal and management factors that are associated with improved survival. Methods : Two groups of ELBW infants who were admitted to our neonatal intensive care unit (NICU) during two distinct eras : November 1994-December 1999 (Period 1 : n=100) and January 2000-April 2004 (Period 2 : n=166) were retrospectively reviewed. Results : Despite the younger gestational age and smaller birth weight of the ELBW infants in period 2, not only did their survival rate increased to 75 percent from 60 percent in period 1, but their incidence of morbidities such as bronchopulmonary dysplasia, confimed sepsis and intraventricular hemorrhage (${\geq}$Grade III) also declined. Factors significantly associated with improved survival included the use of antenatal steroids, a longer duration of nasal continuous positive airway pressure and the absence of intraventricular hemorrhage (${\geq}$Grade III). Conclusion : We believe that optimized clinical practice, that emphasized less invasive care, contributed to the recent improvements in the survival rate of ELBW infants.

• Clinical and Experimental Pediatrics
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• v.52 no.3
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• pp.295-302
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• 2009

Purpose : To report our experience of gastrointestinal (GI) operations (OP) performed in very low birth weight infants (VLBWI) and to evaluate their clinical characteristics. Methods : Among the 1,117 VLBWI admitted to the SMC neonatal intensive care unit from November 1994 to February 2007, the medical records of 37 infants who underwent GI OP (except inguinal hernia OP) and 1,080 VLBWI without GI OP were retrospectively reviewed. Results : The mean gestational age ($27^{+6}2^{+3}$ vs. $28^{+5}{\pm}$2^{+6}$) and birth weight (979${\pm}$241 g vs. 1,071${\pm}$271 g) of the 37 VLBWI who underwent the GI OP was lower than the VLBWI without GI OP group (n=1,080). Mortality rates in the GI OP group were significantly higher than in the non GI OP group (28% vs. 15%, P<0.001). The incidence of cholestasis, retinopathy of prematurity and periventricular leukomalacia were higher in the GI OP group than in the non GI OP group, but the incidence of bronchopulmonary dysplasia was not significantly different between the GI OP group and the non GI OP group. For GI OP indications, focal intestinal perforation was most common and showed a more favorable outcome than necrotizing enterocolitis. Compared with an earlier 7-year period, 1994-2000, the incidence and survival rates increased in the subsequent 2001-2007 period. Conclusion : GI OP was associated with high mortality and morbidity in VLBWI. Further efforts to improve outcomes of GI OP in VLBWI should be investigated to improve the quality of care in VLBWI.

• 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.

• Journal of Korean Academy of Nursing
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• v.13 no.1
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• pp.7-21
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• 1983

For the flexible and rational distribution of limited existing health resources based on measurements of individual risk, the socalled Risk Approach is being proposed by the World Health Organization as a managerial tool in maternal and child health care program. This approach, in principle, puts us under the necessity of developing a technique by which we will be able to measure the degree of risk or to discriminate the future outcomes of pregnancy on the basis of prior information obtainable at prenatal care delivery settings. Numerous recent studies have focussed on the identification of relevant risk factors as the Prior infer mation and on defining the adverse outcomes of pregnancy to be dicriminated, and also have tried on how to develope scoring system of risk factors for the quantitative assessment of the factors as the determinant of pregnancy outcomes. Once the scoring system is established the technique of classifying the patients into with normal and with adverse outcomes will be easily de veloped. The scoring system should be developed to meet the following four basic requirements. 1) Easy to construct 2) Easy to use 3) To be theoretically sound 4) To be valid In searching for a feasible methodology which will meet these requirements, the author has attempted to apply the“Likelihood Method”, one of the well known principles in statistical analysis, to develop such scoring system according to the process as follows. Step 1. Classify the patients into four groups: Group $A_1$: With adverse outcomes on fetal (neonatal) side only. Group $A_2$: With adverse outcomes on maternal side only. Group $A_3$: With adverse outcome on both maternal and fetal (neonatal) sides. Group B: With normal outcomes. Step 2. Construct the marginal tabulation on the distribution of risk factors for each group. Step 3. For the calculation of risk score, take logarithmic transformation of relative proport-ions of the distribution and round them off to integers. Step 4. Test the validity of the score chart. h total of 2, 282 maternity records registered during the period of January 1, 1982-December 31, 1982 at Ewha Womans University Hospital were used for this study and the“Questionnaire for Maternity Record for Prenatal and Intrapartum High Risk Screening”developed by the Korean Institute for Population and Health was used to rearrange the information on the records into an easy analytic form. The findings of the study are summarized as follows. 1) The risk score chart constructed on the basis of“Likelihood Method”ispresented in Table 4 in the main text. 2) From the analysis of the risk score chart it was observed that a total of 24 risk factors could be identified as having significant predicting power for the discrimination of pregnancy outcomes into four groups as defined above. They are: (1) age (2) marital status (3) age at first pregnancy (4) medical insurance (5) number of pregnancies (6) history of Cesarean sections (7). number of living child (8) history of premature infants (9) history of over weighted new born (10) history of congenital anomalies (11) history of multiple pregnancies (12) history of abnormal presentation (13) history of obstetric abnormalities (14) past illness (15) hemoglobin level (16) blood pressure (17) heart status (18) general appearance (19) edema status (20) result of abdominal examination (21) cervix status (22) pelvis status (23) chief complaints (24) Reasons for examination 3) The validity of the score chart turned out to be as follows: a) Sensitivity: Group $A_1$: 0.75 Group $A_2$: 0.78 Group $A_3$: 0.92 All combined : 0.85 b) Specificity : 0.68 4) The diagnosabilities of the“score chart”for a set of hypothetical prevalence of adverse outcomes were calculated as follows (the sensitivity“for all combined”was used). Hypothetidal Prevalence : 5％ 10％ 20％ 30％ 40％ 50％ 60％ Diagnosability : 12% 23% 40% 53% 64% 75% 80%.