• Neonatal Medicine
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• v.25 no.4
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• pp.161-169
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• 2018

Purpose: We assessed the influence of antenatal corticosteroid (ACS) on the inhospital outcomes of intrauterine growth restriction (IUGR) infants. Methods: A retrospective study was conducted with singletons born at $23^{+0}$ to $33^{+6}weeks$ of gestation at Seoul National University Hospital from 2007 to 2014. We compared clinical outcomes between infants who received ACS 2 to 7 days before birth (complete ACS), at <2 or >7 days (incomplete ACS), and those who did not receive ACS in IUGR and AGA infants. Multivariate logistic regression using Firth's penalized likelihood was performed. Results: 304 neonates with 91 IUGR neonates were eligible. Among AGA neonates, mortality (adjusted odds ratio [aOR], 0.13; 95% confidence interval [CI], 0.02 to 0.78), hypotension within 7 postnatal days (aOR, 0.20; 95% CI, 0.06 to 0.64), and severe bronchopulmonary dysplasia (BPD) or death (aOR, 0.24; 95% CI, 0.07 to 0.77) were lower in complete ACS group after adjusting for pregnancy induced hypertension and uncontrolled preterm labor. Mortality (aOR, 0.18; 95% CI, 0.04 to 0.78), hypotension (aOR, 0.26; 95% CI, 0.09 to 0.70), and severe BPD or death (aOR, 0.33; 95% CI, 0.12 to 0.92) were also lower in the incomplete ACS group. Among IUGR infants, after adjusting for birth weight and 5-minute Apgar score, inhaled nitric oxide use within 14 postnatal days was lower in both complete ACS (aOR, 0.07; 95% CI, 0.01 to 0.67) and incomplete ACS (aOR, 0.04; 95% CI, 0.01 to 0.37) groups. Conclusion: ACS was not effective in reducing morbidities in IUGR preterm infants.

• Clinical and Experimental Pediatrics
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• v.51 no.11
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• pp.1236-1240
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• 2008

Smith-Lemli-Opitz syndrome (SLOS) is a rare, autosomal recessive disease caused by an inborn error in cholesterol synthesis. Patients with this disease suffer from multiple malformations due to reduced activity of 7-dehydrocholesterol reductase (DHCR7), which increases 7-dehydrocholesterol (7DHC) and 8-dehydrocholesterol (8DHC) concentrations and decreases cholesterol concentration in body fluids and tissue. The SLOS phenotypic spectrum ranges from a mild disorder with behavioral and learning problems to a lethal disease characterized by multiple malformations. Here, we describe a newborn male with ambiguous genitalia who was diagnosed to have type II SLOS during the neonatal period. A clinical examination revealed low levels of unconjugated estriol in the maternal serum, and a variety of fetal ultrasound anomalies, including prenatal growth retardation. After birth, the infant was diagnosed to have congenital heart disease (Tetralogy of Fallot with severe pulmonary artery stenosis), cleft lip and palate, micrognathia, postaxial polydactyly, ambiguous genitalia, and cataracts. Clinical investigation revealed extremely low plasma cholesterol levels and the presence of mutation (homozygote of p.Arg352Gln) in the DHCR7 gene. The patient underwent palliative heart surgery (to widen the pulmonary artery) and received intravenous lipid supplementation. Cholesterol levels increased slightly, but not to normal values. The patient died from cardiopulmonary failure and sepsis 72 days after birth. This report provides the first description of a Korean patient with SLOS confirmed by verification of DHCR7 gene mutation and illustrates the need for early recognition and appropriate diagnosis of this disease.

• Neonatal Medicine
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• v.18 no.1
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• pp.117-123
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• 2011

Purpose: Congenital intrahepatic portosystemic shunts are rare disease and clinically asymptomatic shunts may be detected by chance on ultrasonogram before and after birth. We studied clinical course, treatment and prognosis of congenital intrahepatic portosystemic shunt at prenatal or neonatal period. Methods: Medical records of 8 patients which were diagnosed in intrahepatic portosystemic shunt in Cheil General Hospital from 2006 through 2010 were reviewed retrospectively. Results: Eight patients with congenital intrahepatic portosystemic shunts were identified. Six patients were diagnosed at prenatal radiological screening, including three cases of intrauterine growth restriction and two cases of preterm baby. One case with increased serum ammonia underwent coil embolization. In four cases including one case that presented elevated direct bilirubin, shunts were closed spontaneously within 11th month after birth. Two patients were diagnosed on abdominal sonogram after birth because of elevated direct hyperbilirubinemia, all of whom presented intrauterine growth restriction. Closure of shunts was confirmed during 4th month to 6th month. Conclusion: Congenital intrahepatic portosystemic shunts are clinically asymptomatic mostly and spontaneous closure is expected within 2 years age. But occasionally they have severe complication, so clinical and radiological observation is needed. Specially in cases of intrauterine growth retardation without evident cause, the possible diagnosis of congenital intrahepatic portosystemic shunts should be considered and prenatal and postnatal examination should be performed. When prenatal diagnosis is made, fetal wellbeing should be monitored periodically until spontaneous closure of shunts.

• 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.