• Clinical and Experimental Pediatrics
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• v.51 no.11
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• pp.1172-1178
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• 2008

Purpose : Congenital chylothorax is an accumulation of lymphatic fluid within the pleural space. It is a common cause of unidentified hydrops fetalis. We examined the perinatal history, clinical manifestation, diagnosis, treatment, and outcome in 6 newborns diagnosed to have congenital chylothorax with hydrops fetalis. We also studied the effect of octreotide therapy for congenital chylothorax in relation to conservative treatment. Methods : We retrospectively reviewed the medical records of 6 patients diagnosed to have congenital chylothorax with hydrops fetalis among 27,907 newborns who were born at the Cheil General Hospital and Womens Healthcare Center between January 2004 and July 2007. The diagnosis of chylothorax is based on the analysis of pleural fluid before and after milk feeding. Results : Incidence of congenital chylothorax in this study was 0.021%. All 6 cases were noted in over the 92% lymphocyte in pleural analysis. Transudate was changed into chyle with increasing triglyceride levels above 200 mg/dL after milk feeding. Three of 6 infants improved with conservative treatment, including thoracostomy and assisted ventilation. The others had persistent symptoms despite conservative treatment and responded to octreotide therapy. A complication, specifically vomiting was noted in 1 case during octreotide therapy. Conclusion : In this study, octreotide therapy resulted in a safe and excellent outcome. Therefore, octreotide therapy is considered in severe refractory congenital chylothorax in conservative treatment. Further studies are required to determine appropriate guidelines for octreotide therapy.

• Neonatal Medicine
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• v.17 no.1
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• pp.64-74
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• 2010

Purpose : The aim of this study was to determine the risk factors, clinical characteristics and prognosis for the development of periventricular leukomalacia (PVL) in preterm infants according to the extent and site of the PVL. Methods : The medical records of infants (under 36 weeks of gestational age) delivered from January 1999 to December 2008 were reviewed. Twenty-five preterm infants with were PVL were diagnosed by brain magnetic resonance imaging (MRI) and an addition 50preterm infants with no brain lesions were enrolled in this study. The perinatal and neonatal risk factors for the development of PVL was determine in these infants. Mental and Psychomotor Developmental Indices (MDI, PDI) were assessed by a clinical psychologist using the Bayley Scales of Infant Development II. We compared the differences of the clinical characteristics and prognosis according to brain MRI findings. Results : Maternal fever, young maternal age, extended oxygen use, hypotension within the first week of birth, use of inotropics within the first week of birth, and respiratory distress syndrome were the risk factors associated with PVL (P <0.05). In the multivariate analysis, maternal fever and extended oxygen use were statistically significant independent risk factors (P <0.05). The mean MDI and PDI scores of the PVL group (74.4$\pm$ 27.8 and 58.0$\pm$17.7) were significantly lower than those of the control group (103.5$\pm$8.9 and 101.7$\pm$16.1, P <0.05). Conclusion : Maternal fever and extended oxygen use were independent risk factors for PVL. We should pay attention to infants who had the risk factors and follow them up closely by brain imaging study and Bayley Scales of Infant Development II.

• Clinical and Experimental Pediatrics
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• v.52 no.3
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• pp.289-294
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• 2009

Purpose : To assess the usefulness of magnetic resonance imaging (MRI), karyotyping, brainstem auditory evoked potential (BAEP), electroencephalogram (EEG), tandem mass screening test, and newborn metabolic screening test in children with language delay for diagnosing underlying diseases. Methods : From January 2000 to June 2007, a retrospective chart review was performed for 122 children with language delay who visited the Child Neurology Clinic at Yeungnam University Hospital and who underwent neuropsychologic tests and other diagnostic evaluations for underlying diseases. They were grouped into phenomenological diagnostic categories, and test results were analyzed according to the underlying diseases. Results : Of 122 patients, 47 (38.5%) had mental retardation, 40 (32.8%) had developmental language disorders, 23 (18.9 %) had borderline IQ, and 12 (9.8%) had autism spectrum disorder. In 26 (21.3%) cases, the causes or relevant clinical findings to explain language delay were found. Eight (10.4%) of 77 MRIs, 6 (8.0%) of 75 EEGs, and 4 (5%) of 80 BAEPs showed abnormal results. Results directly attributed to diagnosing underlying diseases were 2 hearing defects in BAEPs and 1 bilateral perisylvian cortical dysplasia in MRIs. No abnormal results were found in karyotyping, tandem mass screening tests, and new-born screening tests. Conclusion : Commonly used tests to diagnose the cause of language delay are not very effective and should only be used selectively, according to patient characteristics. However, despite the low diagnostic yields from these tests, because many patients show abnormal results, these tests are useful when conducted in complete evaluation.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.143-147
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• 2005

Purpose : The aim of the this study was to evaluate the effect of various perinatal conditions on TSH and thyroid hormone levels in cord blood. Methods : Cord blood samples were collected from 130 neonates immediately after birth. TSH, $T_3$, and free $T_4$ levels were measured by the radioimmunoassay(RIA) method. The effects of gestational age, sex, birth weight, delivery method, perinatal asphyxia, maternal diabetes mellitus(DM), and preeclampsia on TSH and thyroid hormone levels were assessed by ANOVA test, Student t-test, and multiple regression analysis. Results : Birth weight and sex did not affect TSH and thyroid hormone levels. TSH level increased according to gestational age(P<0.05). TSH level was $4.42{\pm}0.66{\mu}IU/mL$ in infants born vaginally, which was higher than that of cesarian section delivery($3.31{\pm}0.33{\mu}IU/mL$)(P<0.05). TSH level was $5.18{\pm}0.93{\mu}IU/mL$ in asphyxiated newborns and $2.97{\pm}0.84{\mu}IU/mL$ in non-asphyxiated newborns(P<0.05). TSH level in infants with maternal DM($8.911{\pm}1.25{\mu}IU/mL$) was higher than that of infants without maternal DM($4.32{\pm}0.42{\mu}IU/mL$)(P<0.05). TSH level was $5.28{\pm}0.42{\mu}IU/mL$ in infants with maternal preeclampsia and $3.65{\pm}0.46{\mu}IU/mL$ in infants without maternal preeclampsia(P<0.05). Thyroid hormones were lower in infants with perinatal asphyxia(P<0.05). In asphyxiated infants, $T_3$ level was $75.33{\pm}55.65ng/mL$ and free $T_4$ was $0.54{\pm}0.21ng/mL$. $T_3$ and free $T_4$ level was $109.85{\pm}41.77ng/mL$ and $0.76{\pm}0.22ng/mL$ each in infants without perinatal asphyxia. Among the perinatal factors, gestational age, 1 min Apgar score and maternal DM influenced TSH level independently. Conclusion : In our study, cord blood TSH and thyroid hormone levels were affected by perinatal stress events.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.191-196
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• 2005

Purpose : Short stature is one of the characteristic features of Turner syndrome. We investigated the factors affecting final adult height(FAH) in patients with Turner syndrome. Methods : The study group was comprised of 60 patients who were diagnosed with Turner syndrome by chromosomal study and clinical phenotypes and attained FAH. Data were obtained from retrospective review of the medical records. We analyzed the factors influencing FAH in growth hormone(GH) treated and GH untreated groups. Results : Sixty patients were enrolled; 48 patients received GH treatment, and 12 patients did not. Mean duration of GH treatment was 35.8 months(range 4 to 120 months), and mean dosage of GH was $0.8{\pm}0.2IU/kg/wk$ in GH treated group. Mean growth velocity was $5.6{\pm}2.0cm/yr$, which was significantly higher than that during pretreatment period. In the GH treated group, mean chronological age, bone age, mean height, and height standard deviation(SD) score at GH treatment were $12.2{\pm}2.7yr$ $10.3{\pm}2.5yr$ $127.5{\pm}10.1cm$ and $-3.1{\pm}1.1$, respectively. In the GH treated group, the mean FAH and SD score of FAH were $146.9{\pm}5.8cm$ and $-2.7{\pm}1.2$, respectively, which showed significant differences compared with those of the GH untreated group. Analyzing the factors affecting FAH in GH-treated patients, only the SD score of height at the time of treatment was significantly related to FAH. Conclusion : GH treatment leads to an increment in FAH in patients with Turner syndrome. Average FAH gain was as much as 5.8 cm. SD score of height at the time of GH treatment was the only factor influencing FAH.

• Journal of Preventive Medicine and Public Health
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• v.40 no.5
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• pp.363-370
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• 2007

Objectives: The principal objective of this study was to determine the relationship between maternal exposure to air pollution and low birth weight and to propose a possible environmental health surveillance system for low birth weight. Methods: We acquired air monitoring data for Seoul from the Ministry of Environment, the meteorological data from the Korean Meteorological Administration, the exposure assessments from the National Institute of Environmental Research, and the birth data from the Korean National Statistical Office between January 1, 2002 and December 31, 2003. The final birth data were limited to singletons within $37{\sim}44$ weeks of gestational age. We defined the Low Birth Weight (LBW) group as infants with birth weights of less than 2500g and calculated the annual LBW rate by district. The air monitoring data were measured for $CO,\;SO_2,\;NO_2,\;and\;PM_{10}$ concentrations at 27 monitoring stations in Seoul. We utilized two models to evaluate the effects of air pollution on low birth weight: the first was the relationship between the annual concentration of air pollution and low birth weight (LBW) by individual and district, and the second involved a GIS exposure model constructed by Arc View 3.1. Results: LBW risk (by Gu, or district) was significantly increased to $1.113(95%\;CI=1.111{\sim}1.116)\;for\;CO,\;1.004(95%\;CI=1.003{\sim}1.005)\;for\;NO_2,\;1.202(95%\;CI=1.199{\sim}1.206\;for\;SO_2,\;and\;1.077(95%\;CI=1.075{\sim}1.078)\;\;for\;PM_{10}$ with each interquartile range change. Personal LBW risk was significantly increased to $1.081(95%\;CI=1.002{\sim}1.166)\;for\;CO,\;1.145(95%\;CI=1.036{\sim}1.267)\;for\;SO_2,\;and\;1.053(95%\;CI=1.002{\sim}1.108)\;for\;PM_{10}$ with each interquartile range change. Personal LBW risk was increased to $1.003(95%\;CI=0.954{\sim}1.055)\;for\;NO_2$, but this was not statistically significant. The air pollution concentrations predicted by GIS positively correlated with the numbers of low birth weights, particularly in highly polluted regions. Conclusions: Environmental health surveillance is a systemic, ongoing collection effort including the analysis of data correlated with environmentally-associated diseases and exposures. In addition. environmental health surveillance allows for a timely dissemination of information to those who require that information in order to take effective action. GIS modeling is crucially important for this purpose, and thus we attempted to develop a GIS-based environmental surveillance system for low birth weight.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.222-230
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• 2005

Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

• Journal of Chest Surgery
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• v.41 no.1
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• pp.1-11
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• 2008

Background: The Damus-Kaye-Stansel (DKS) procedure is a proximal MPA-ascending aorta anastomosis used to relieve systemic ventricular outflow tract obstructions (SVOTO) and pulmonary hypertension. The purpose of this study was to review the indications and outcomes of the DKS procedure, including the DKS pathway and semilunar valve function. Material and Method: A retrospective review of 28 patients who underwent a DKS procedure between May 1994 and April 2006 was performed. The median age at operation was 5.3 months ($13\;days{\sim}38.1\;months$) and body weight was 5.0 kg ($2.9{\sim}13.5\;kg$). Preoperative pressure gradients were $25.3{\pm}15.7\;mmHg$ ($10{\sim}60\;mmHg$). Eighteen patients underwent a preliminary pulmonary artery banding as an initial palliation. Preoperative main diagnoses were double outlet right ventricle in 9 patients, double inlet left ventricle with ventriculoarterial discordance in 6,. another functional univentricular heart in 5, Criss-cross heart in 4, complete atrioventricular septal defect in 3, and hypoplastic left heart variant in 1. DKS techniques included end-to-side anastomosis with patch augmentation in 14 patients, classical end-to-side anastomosis in 6, Lamberti method (double-barrel) in 3, and others in 5. The bidirectional cavopulmonary shunt and Fontan procedure were concomitantly performed in 6 and 2 patients, respectively. Result: There were 4 hospital deaths (14.3%), and 3 late deaths (12.5%) with a follow-up duration of $62.7{\pm}38.9$ months ($3.3{\sim}128.1$ months). Kaplan-Meier estimated actuarial survival was $71.9%{\pm}9.3%$ at 10 years. Multivariate analysis showed right ventricle type single ventricle (hazard ratio=13.960, p=0.004) and the DKS procedure as initial operation (hazard ratio=6.767, p=0.042) as significant mortality risk factors. Four patients underwent staged biventricular repair and 13 received Fontan completion. No SVOTO was detected after the procedure by either cardiac catheterization or echocardiography except in one patient. There was no semiulnar valve regurgitation (>Gr II) or semilunar valve-related reoperation, but one patient (3.6%) who underwent classical end-to-side anastomosis needed reoperation for pulmonary artery stenosis caused by compression of the enlarged DKS pathway. The freedom from reoperation for the DKS pathway and semilunar valve was 87.5% at 10 years after operation. Conclusion: The DKS procedure can improve the management of SVOTO, and facilitate the selected patients who are high risk for biventricular repair just after birth to undergo successful staged biventricular repair. Preliminary pulmonary artery banding is a safe and effective procedure that improves the likelihood of successful DKS by decreasing pulmonary vascular resistance. The long-term outcome of the DKS procedure for semilunar valve function, DKS pathway, and relief of SVOTO is satisfactory.