• Journal of Chest Surgery
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• v.32 no.6
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• pp.567-572
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• 1999

Background: Surgical correction of the full spectrum of esophageal atresia with tracheoesophageal fistula has improved over the years, but the mortality and morbidity assoiated with repair of these anomalies still remains high. Material and Method: We retrospectively analyzes 27 surgically treated patients with esophageal atresia and tracheoesophageal fistula at Dong-A University Hospital between January 1992 and March 1997. Result: There were 21 male and 6 female patients. Mean birth weight was 2.62$\pm$.385 kg(2.0~3.4 kg). Twenty- four(88.9%) had esophageal atresia with distal tracheoesophageal fistula, and 3(11.1%) had pure esophageal atresia. Four(14.8%) infants were allocated to Waterston risk group A, 18(66.7%) to group B, and 5(18.5%) to group C. In eighteen(66.7%) infants with associated anomalies, cardiovascular anomalies were the most common. Three had a gap length of 3.5 cm or greater(ultra-long gap) between esophageal segments, 7 had 2.0 to 3.5 cm(long gap), 8 had 1.0 to 2.0 cm(medium gap), and 9 had 1 cm or less(short gap) gap length. Among 27 neonates, 3 cases underwent staged operation, late colon interposition was done in 2, and all other 24 cases underwent primary esophageal anastomosis. Oerative mortality was 2/27(7.4%). Causes of death included acute renal failure(n=1), empyema from anastomotic leak(n=1), necrotizing enterocolitis(n=1), sepsis(n=1), insulin-dependent diabetus mellitus(n=1 . There were 4 anastomosis- related complications including stricture in 3, leakage in 1. Mortality was related to the gap length(p<.05). Conclusion: Although the complication rate associated with surgical repair of these anomalies is high, this does not always implicate the operative mortality. The overall survival can be improved by effective treatment for combined anomalies and intensive postoperatve care.

• Clinical and Experimental Pediatrics
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• v.49 no.8
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• pp.851-856
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• 2006

Purpose : The purpose of this study is to find out the diagnostic significance of serum bile acid on total parenteral nutrition induced cholestasis in premature infants. Methods : Infants without cholestasis were classified into postnatal days and each change of serum bile acid was measured and analyzed. Also, the serum direct bilirubin, serum bile acid, ${\gamma}$-glutamic acid transferase, and alkaline phosphatase of premature infants with total parenteral nutrition induced cholestasis were measured for comparison and analysis of their correlation. Results : Changes of serum bile acid analysis after birth showed no significant difference between boys and girls, between premature infants and term infants without cholestasis. Serum bile acid levels are constant after two weeks after birth in neonates without cholestasis. In premature infants with total parenteral nutrition induced cholestasis, the increase of serum direct bilirubin over 2 mg/dL was $34.9{\pm}18.3$ days after birth, and the increase of serum bile acid was $28.1{\pm}18.3$ days. Its increase was about 1 week faster than serum direct bilirubin, however, there was no statistical significance(P=0.114). Comparing analysis of serum bile acid, ${\gamma}$-glutamic acid transferase, and alkaline phosphatase, serum bile acid showed the highest correlation to serum direct bilirubin(r=0.487, P=0.000). Conclusion : Serum bile acid is an important parameter of total parenteral nutrition induced cholestasis in premature infants and will be useful for early diagnosis and treatment.

• Clinical and Experimental Pediatrics
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• v.45 no.9
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• pp.1126-1133
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• 2002

Purpose : Prader-Willi syndrome(PWS) is a complex disorder affecting multisystems with characteristic clinical features. Its genetic basis is an expression defect in the paternally derived chromosome 15q11-q13. We analyzed the clinical features and genetic basis of PWS patients for early detection and treatment. Methods : We retrospectively studied 24 patients with PWS in Department of Pediatrics, Samsung Medical Center, from September 1997 to September 2001. We performed cytogenetic and molecular genetic techniques using high resolution GTG banding techniques, fluorescent in situ hybridization and methylation-specific PCR for CpG island of SNRPN gene region. Results : The average birth weight of PWS patients was $2.67{\pm}0.47kg$ and median age at diagnosis was 1.3 years. The average height and weight of PWS patients under one year at diagnostic time were located in a 3-10 percentile relatively, and a rapid weight gain was seen between two and six years. Feeding problems in infancy and neonatal hypotonia were the two most consistently positive major criteria in over 95% of the patients. In 18 of the 24 cases(75%), deletion of chromosome 15q11-q13 was demonstrated and one case among 18 had an unbalanced 14;15 translocation. In four cases without any cytogenetic abnormality, it may be considered as maternal uniparental disomy and the rest showed another findings. Conclusion : We suggest diagnostic testing for PWS in all infants/neonates with unexplained feeding problems and hypotonia. It is necessary for clinically suspicious patients to undergo an early genetic test. As the genetic basis of PWS was heterogenous and complex, further study is required.

• Clinical and Experimental Pediatrics
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• v.51 no.5
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• pp.481-486
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• 2008

Purpose : The aims of this study were to test the efficacy of vapocoolant spray to decrease the symptoms associated with pain in newborns undergoing heel stick and intramuscular injection and compare the pain relief effect of oral glucose. Methods : Randomized, controlled study including sixty newborns undergoing heel stick and intramuscular injection. Group 1 was heelsticked, Group 2 was intramuscular injected, Group A did not recieve any treatment, Group B recieved 30% glucose solution orally, Group C was applied vapocoolant spray symptoms and signs associated with pain at heel stick and intramuscular injection were measured with the premature Infant Pain Profile (PIPP) scale. Results : There was no significant difference in the PIPP score between intramuscular injected group control and heel stick group control (P=0.07). The mean PIPP score of Group 1A (control) $10.6{\pm}2.4$, Group 1B $5.5{\pm}2.0$, Group 1C $5.2{\pm}1.8$. The mean PIPP score 1B and 1C were significantly lower than control (1B P<0.001, 1C P<0.001). The mean PIPP score of Group 2A (control) $12.5{\pm}1.4$, Group 2B $7.0{\pm}1.7$, Group 2C $6.4{\pm}1.6$. The mean PIPP score 2B and 2C were significantly lower than control (2B P<0.001, 2C P<0.001). Conclusion : The antinociceptive effect of vapocoolant sparay is as effective as 30% oral glucose solution for pain control. So this study support the use of vapocoolant spray for reducing pain during painful procedure in the neonatal intensive care units.

• Pediatric Infection and Vaccine
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• v.7 no.1
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• pp.136-142
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• 2000

Purpose : Although there are a lot of the reports about the persistence of anti HBs titer of plasma derives HBV vaccine, it is difficult to find the follow up studies of the recombinant HBV vaccine. We performed this study to compare the persistence of anti HBs titer by vaccination schedule and the seronegative rate of 5 years later according to Anti HBs titer after basic immunization in neonatal period by recombinant HBV vaccination. Methods : This study was performed on 420 neonates at Pusan Moon Hwa Hospital from April to December 1993, followed up for 5 years after basic immunization by recombinant HBV vaccine. The anti HBs titer test was done by radioimmunoassay(RIAAUSAB, Abbott laboratories). The positive anti HBs level that would protect against HBV infection was defined as a level equal to or greater than 10mIU/mL. Results : In this study the seronegative rate after 5 years was 5% in 2 month schedule group, 25.5% in 6 month schedule group(P>0.05). In 2 month schedule group the seronegative rate was 20% when anti HBs titer is lower than 200mIU/mL, 0% when more than 200mIU/mL(P>0.05). In 6 month schedule group the seronegative rate was 66.7% when anti HBs titer was lower than 200mIU/mL, 40% when 200~499.9mIU/mL, 23.9% when 500~999.9mIU/mL, 22.5% when more than 1000mIU/mL. Conclusion : In this study the seronegative rate after 5 years of recombinant HBV vaccination was 5~25.5%. The persistence of anti HBs titer was statistically irrelevant to schedule. The seronegative rate after 5 years was statistically irrelevant to anti HBs titer after basic immunization.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.1
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• pp.18-23
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• 2016

Objective: Newborn screening leads to improved treatment and disease outcomes, but false-positive newborn screening results may impact include parental stress and anxiety, perception of child as unhealthy, parent-child relationship dysfunction, and increased infant hospitalizations. The purpose of this study was to investigate of the false positive rates and the causative factors of false positive results in Tandem Mass Spectrometry (TMS) in single center. Methods: Records were reviewed for all 18,872 subjects who were born in Cheill General Hospital, during January 1st, 2012 to December 31st, 2014. 17,292 neonates (91.62%) were tested for tandem mass screening almost in 2-5th day of life. Newborn babies whose first results were abnormal had been tested repeatedly by same methods in 7-14 day. If the results were abnormal again, further evaluation was performed. TMS analysis included data for the 43 disorders screened for using TMS broken down into three categories: fatty acid oxidation disorders, organic acidurias, and aminoacidopathies. The impact of several factors on increased false positive rates was analyzed using a multivariate analysis: time from birth to sample collection, birth weight, birth height, BMI, gender, gestational age, delivery type. Results: Males of the subjects were 8942 (51.7%), female 8350 (48.3%), the mean gestational age was $38.6{\pm}1.7$ weeks, the average birth weight $3,155.6{\pm}502.4g$, the average birth height $49.1{\pm}2.9cm$, and the average BMI $13.0{\pm}3.8(kg/m^2)$. Vaginal delivery cases were 9713 (56.2%), caesarean section 7,579 (43.8%). The average date of the inspection was $2.8{\pm}1.1$ days. 224 cases were identified as TMS positive. All the subjects were false positive (222/17,292, 1.30%) except 2 cases (1 male; benign phenylketonuria and 1 female; Short chain acyl-CoA dehydrogenase deficiency). The false positive rates were 0.61% in fatty acid oxidation disorders, 0.25% in organic acidurias, and 0.45% in aminoacidopathies. In our study, the date of inspection got late, the false positive rates got higher. Because almost the cases of late test date were in treatment in neonatal intensive care unit so their test date was affected by their medical conditions. False positive rate was higher in extreme immaturity${\leq}27$ weeks than newborns of gestational age >27 weeks [OR=6.957 (CI=1.273-38.008), p<0.025] and extremely low birth weight<1,000 g than newborns of birthweight ${\geq}1,000g$ [OR=5.616 (CI=1.134-27.820), p<0.035]. Conclusion: False positive rate of TMS was 1.30% in Cheil General Hospital. Lower gestational age and birth weight impacted on increased false positive rates. Better understanding of factors that influence the reporting of screening tests, and the ability to modify these important factors, may improve the screening process and reduce the need for retesting. of screening tests, and the ability to modify these important factors, may improve the screening process and reduce the need for retesting.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.178-187
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• 2002

Purpose : The detection of hydronephrosis(HN) with antenatal ultrasonography was first reported in the 1970s. Prenatal HN is diagnosed with an incidence of 1:100 to 1:500 on antenatal screening. Recently, the purpose of antenatal screening has changed from simple detection to selection for specific diagnosis-based management. this study is to evaluate the usefulness of antenatal sonography for HN and to investigate the differential causes of HN and their clinical outcomes. Patients and methods : 11,783 live neonates with prenatal ultrasonographic examination at Ajou University School of Medicine, from Sep. 1994 to Aug. 2001 were analyzed. Results and conclusion : Hydronephrosis (>10 mm) was detected in 119 (1.0%) cases antenatally and among these, 91 were proved to have HN postnatally Males were three times more affected than females. Additional imaging studies revealed that ureteropelvic junction obstruction was the most common postnatal diagnosis (47%), followed by multicystic dysplastic kidney, vesicoureteral junction obstruction and vesicoureteral reflux. During 20 months' follow-up(3 to 72 months), 58(48%) renal units showed spontaneous resolution and surgical interventions were necessary in 10 (7.4%) of postnatally confirmed hydronephrotic renal units.

• Pediatric Infection and Vaccine
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• v.23 no.3
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• pp.165-171
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• 2016

Purpose: Human parechovirus (HPeV) is an increasingly recognized pathogenic cause of central nervous system (CNS) infection in neonates. However, HPeV infections have not been studied in older children. This study determined the prevalence and clinical features of HPeV CNS infection in children in Korea. Methods: Reverse transcription polymerase chain reaction assays were performed using HPeV-specific, 5' untranslated, region-targeted primers to detect HPeV in cerebrospinal fluid (CSF) samples from children presenting with fever or neurologic symptoms from January 1, 2013, to July 31, 2014. HPeV genotyping was performed by sequencing the viral protein 3/1 region. Clinical and laboratory data were retrospectively abstracted from medical records and compared with those of enterovirus (EV)-positive patients from the same period. Results: Of 102 CSF samples, six (5.9%) were positive for HPeV; two of 21 EV-positive samples were co-infected with HPeV. All samples were genotype HPeV3. Two HPeV-positive patients were <3 months of age and four others were over 1 year old. While HPeV-positive infants under 1 year of age presented with sepsis-like illness without definite neurologic abnormalities, HPeV-positive children over 1 year of age presented with fever and neurologic symptoms such as seizures, loss of consciousness, and gait disturbance. The CSF findings of HPeV-positive patients were mostly within the normal range, whereas most (73.7%) EV-positive patients had pleocytosis. Conclusions: Although HPeV is typically associated with disease in young infants, the results of this study suggest that HPeV is an emerging pathogen of CNS infection with neurologic symptoms in older childhood.

• Korean Journal of Veterinary Research
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• v.28 no.2
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• pp.213-219
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• 1988

The measurement was investigated with 18 heads of fetus(60, 90, 120 days of gestation) and neonate in Korean native goats. The results were summerized as follows: 1. The crown-rump length of fetuses at 60, 90, 120 days of gestation and neonate was 8.71, 20.83, 31.10 and 34.93 cm, respectively. 2. The length of small intestine at 60, 90, 120 days of gestation and neonate was 32.28, 157.10, 303.52 and 457.06 cm, respectively. 3. The length of large intestine at 60, 90, 120 days of gestation and neonate was 9.20, 37.70, 82.06 and 94.46 cm, respectively. 4. The ratio of intestinal length to crown-rump length at 60, 90, 120 days of gestation and neonate was 4.76, 9.45, 12.40 and 15.79 times, respectively. 5. At 60 days of gestation, the total length of the vertebral column was $7.40{\pm}0.72cm$, The mean length of each segment of the vertebral column was $1.55{\pm}0.20cm$ in cervical, $2.29{\pm}0.21cm$ in thoracic, $1.46{\pm}0.10cm$ in lumbar, $0.51{\pm}0.04cm$ in sacral and $1.59{\pm}0.17cm$ in coccygeal vertebrae. 6. At 90 days of gestation, the total length of the vertebral column was $16.52{\pm}0.80cm$. The mean length of each segment of the vertebral column was $3.72{\pm}0.12cm$ in cervical, $5.09{\pm}0.26cm$ in thoracic, $3.22{\pm}0.04cm$ in lumbar, $1.97{\pm}0.03cm$ in sacral and $2.64{\pm}0.35cm$ in coccygeal vertebrae. 7. At 120 days of gestation, the total length of the vertebral column was $26.35{\pm}0.34cm$. The mean length of each segment of the vertebral column was $6.09{\pm}0.16cm$ in cervical, $7.81{\pm}0.07cm$ in thoracic, $5.08{\pm}0.07cm$ in lumbar, $3.07{\pm}0.02cm$ in and $4.31{\pm}0.02cm$ in coccygeal vertebrae. 8. In the neonate, the total length of the vertebral column was $32.41{\pm}1.57cm$. The mean length of each segment of vertebral was $7.70{\pm}0.25cm$ in cervical, $9.97{\pm}0.68cm$ in thoracic, $5.58{\pm}0.44cm$ in lumbar, $3.85{\pm}0.15cm$ in sacral and $5.05{\pm}0.06cm$ coccygeal vertebrae. 9. The chest girth at 60, 90, 120 days of gestation and neonate was $6.13{\pm}0.51$, $13.45{\pm}0.84$, $20.28{\pm}1.53$ and $22.94{\pm}1.75cm$, respectively. 10. The head length at 60, 90, 120 days of gestation and neonate was $2.93{\pm}0.07$, $6.67{\pm}0.13$, $8.84{\pm}0.51$ and $9.76{\pm}0.44cm$, respectively. 11. The width of the head at 60, 90, 120 days of gestation and neonate was $2.20{\pm}0.13$, $4.45{\pm}0.11$, $5.33{\pm}0.20$ and $5.51{\pm}0.32cm$, respectively.

• Journal of Chest Surgery
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• v.39 no.6 s.263
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• pp.434-439
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• 2006

Background: Deep hypothermic circulatory arrest during repair of aortic arch anomalies may induce neurological complications or myocardial injury. So we surveyed if the regional cerebral and myocardial perfusion might eliminate those potential side effects. Material and Method: From March 2000 to December 2004, 62 neonates or infants with aortic arch anomaly underwent one stage biventricular repair using the regional perfusion technique by single surgeon. Preoperative diagnosis of the arch anomaly consisted of coarctation (n=46), interruption of the aorta (n=12), hypoplastic left heart syndrome (n=2) and truncus areteriosus (n=2). Combined anomalies were ventricular septal defect (n=51), TAPVR (n=1), PAPVR (n=1) and atrioventricular septal defect (n=2). Arterial cannula was inserted at the innominate artery. Result: The mean regional perfusion time of brain was $28{\pm}10min$. Operative mortality rates was 0 (0/62). Late death was 1 (1/62) during $11{\pm}7$ months of follow-up. Neurologic complications consisted of transient chorea in 1 case. There was no reoperation associated with arch anolamy. Pulmonary complication associated with arch repair occurred in f case which was managed by aortopexy. Conclusion: One-tage rch repair using the regional profusion is safe and effective in minimizing the neurologic and myocardial complications.