• Clinical and Experimental Pediatrics
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• v.58 no.6
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• pp.224-229
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• 2015

Purpose: Thyroid dysfunction is common in preterm infants. Congenital hypothyroidism causes neurodevelopmental impairment, which is preventable if properly treated. This study was conducted to describe the characteristics of thyroid dysfunction in very low birth weight infants (VLBWIs), evaluate risk factors of hypothyroidism, and suggest the reassessment of thyroid function with an initially normal thyroid-stimulating hormone (TSH) as part of a newborn screening test. Methods: VLBWIs (January 2010 to December 2012) were divided into two groups according to dysfunction-specific thyroid hormone replacement therapy, and associated factors were evaluated. Results: Of VLBWIs, 246 survivors were enrolled. Only 12.2% (30/246) of enrolled subjects exhibited thyroid dysfunction requiring thyroid hormone replacement. Moreover, only one out of 30 subjects who required thyroid hormone treatment had abnormal thyroid function in the newborn screening test with measured TSH. Most of the subjects in the treatment group (22/30) exhibited delayed TSH elevation. Gestational age, Apgar score, antenatal steroids therapy, respiratory distress syndrome, patent ductus arteriosus, sepsis, intraventricular hemorrhage, postnatal steroids therapy, and duration of mechanical ventilation did not differ between the two groups. Birth weight was smaller and infants with small for gestational age were more frequent in the treatment group. Conclusion: Physicians should not rule out suggested hypothyroidism, even when thyroid function of a newborn screening test is normal. We suggest retesting TSH and free thyroxine in high risk preterm infants with an initially normal TSH level using a newborn screening test.

• Journal of Chest Surgery
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• v.43 no.5
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• pp.518-521
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• 2010

A one-day-old baby was transferred for cyanosis and heart murmur. Echocardiographic evaluation revealed that he had severe neonatal Ebstein's anomaly (Carpentier type C), pulmonary atresia, and pulmonary circulation via patent ductus arteriosus. Because the wall of the atrialized right ventricle was very thin, showed decreased contractility, and the small right ventricle showed pulmonary atresia, we decided that a two-ventricular repair was impossible. When the patient was one-month-old, he underwent right atrium reduction-plasty, a right ventricular exclusion procedure (including atrialized right ventricle resection and functional right ventricle plication), and right modified Blalock-Taussig shunt. He was discharged without specific problems. He received a bidirectional cavopulmonary shunt successfully at 4 months later.

• Journal of Chest Surgery
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• v.41 no.4
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• pp.499-503
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• 2008

Berry syndrome (a distal aortopulmonary window and a right pulmonary artery originating from the ascending aorta, an intact ventricular septum, a patent ductus arteriosus and an interrupted aortic arch) is a rare complex congenital cardiac malformation. We describe a case of one stage repair with using an autologous arterial flap in a 19-day-old neonate who was suffering with this malformation, and we report on the development of postoperative right pulmonary artery stenosis and its successful management with performing multiple balloon angioplasties.

• Clinical and Experimental Pediatrics
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• v.63 no.2
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• pp.56-62
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• 2020

Background: Red blood cell (RBC) transfusion improves cardiorespiratory status of preterm infants by increasing circulating hemoglobin, improving tissue oxygenation, and reducing cardiac output. However, RBC transfusion itself has also been suggested to negatively affect short-term outcomes such as intraventricular hemorrhage (IVH), bronchopulmonary dysplasia (BPD), retinopathy of prematurity (ROP), and necrotizing enterocolitis (NEC) in premature infants. Purpose: This study aimed to analyze the relationship between RBC transfusion and short-term outcomes in very low birth weight (VLBW) infants (birth weight, <1,500 g). Methods: We retrospectively reviewed the medical records of VLBW infants admitted to the Soonchunhyang University Bucheon Hospital between October 2010 and December 2017. Infants who died during hospitalization were excluded. The infants were divided into 2 groups according to RBC transfusion status. We investigated the relationship between RBC transfusion and short-term outcomes including BPD, ROP, NEC, and IVH. Results: Of the 250 enrolled VLBW infants, 109 (43.6%) underwent transfusion. Univariate analysis revealed that all short-term outcomes except early-onset sepsis and patent ductus arteriosus were associated with RBC transfusion. In multivariate analysis adjusted for gestational age, birth weight and Apgar score at 1 minute, RBC transfusion was significantly correlated with BPD (odds ratio [OR], 5.42; P<0.001) and NEC (OR, 3.40; P= 0.009). Conclusion: RBC transfusion is significantly associated with adverse clinical outcomes such as NEC and BPD in VLBW infants. Careful consideration of the patient's clinical condition and appropriate guidelines is required before administration of RBC transfusions.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.4
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• pp.377-387
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• 2020

Purpose: To compare between sodium acetate (SA) and sodium chloride (SC) in parenteral nutrition (PN) with associated metabolic acidosis and neonatal morbidities in preterm infants. Methods: Preterm infants below 33 weeks gestational age, and with a birth weight under 1,301 g were enrolled and further stratified into two groups: i) <1,000 g, or ii) ≥1,000 g in birth weight. The subjects were randomized to receive PN containing SA or SC within the first day of life. The results of routine blood investigations for the first 6 days of PN were collated, and the neonatal outcomes were recorded upon discharge or demise. Results: Fifty-two infants entered the study, with 26 in each group: 29 infants had extremely low birth weight (ELBW). There were no significant differences in birth weight, gestation, sex, exposure to chorioamnionitis and antenatal steroids, surfactant doses and duration of mechanical ventilation between groups. The SA group had significantly higher mean pH and base excess (BE) from days 4 to 6 than the SC (mean pH, 7.36 vs. 7.34; mean BE -1.6 vs. -3.5 [p<0.01]), with a two-fold increase in the mean BE among ELBW infants. Significantly fewer on SA required additional bicarbonate (n=4 vs. 13, p=0.01). The rate of bronchopulmonary dysplasia (BPD) was approximately four-fold lower in SA than SC (n=3 vs. 11, p<0.01). No significant differences were observed in necrotizing enterocolitis, patent ductus arteriosus, retinopathy of prematurity, cholestatic jaundice, and mortality between groups. Conclusion: The use of SA in PN was associated with reduced metabolic acidosis and fewer BPD.

• Journal of Chest Surgery
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• v.12 no.3
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• pp.174-182
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• 1979

This is a report on four cases of successful surgical correction of coarctation of the aorta [COA] in Department of the Thoracic & Cardiovascular Surgery, Hanyang University Hospital. The first case was a postductal type of coarctation of the aorta associated with Patent ductus arteriosus [PDA], Persistent left superior vena cava [LSVC] and richly developed collateral circulation. Blood pressure was measured to be hypertensive at the arm, but hypotensive at the legs. The coarctation of the aorta was corrected with following procedure: Partial resection of the aortic wall with diaphragmatic structure lust above and below the coarctating line of the aorta, and then the defect of the aortic wall was closed by lateral aortographic suture. PDA was closed by ligation procedure. The second case a preductal type of coarctation of the aorta associated with PDA, LSVC, ventricular septal defect [VSD] and poorly developed collateral circulation. Normal blood pressure was measured at the arm, but hypotension was observed at the legs. Correction of coarctation of the aorta was performed under the establishment of tube bypass because of poor collateral circulation. After resection of coarctating short segment, end to end anastomosis was performed without any tension. PDA was closed by division procedure. Simple suture closure of VSD was performed by open heart surgery two weeks after correction of COA. The third case was a long segment COA without any other anomaly. Blood pressure was measured to be hypertensive at the arm, but hypotensive at the legs. Vascular prosthesis was performed using Teflon graft tube after resecting coarctating long segment [6.5 cm] of the aorta. The fourth case was a long segment COA associated with aortic insufficiency and richly developed collateral circulation. Normal blood pressure was measured at the arm, but hypotension was observed at the legs. Vascular prosthesis was performed using Teflon graft tube after resecting coarctating long segment [6.0 cm] of the aorta. Both blood pressure and peripheral pulse on the arm and the legs returned to normal postoperatively in all patients.

• Journal of Chest Surgery
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• v.34 no.8
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• pp.626-629
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• 2001

Congenital polyvalvular disease is a connective tissue disorder affecting more than one heart valve with variable involvement of the entire valvular and subvalvular apparatus. It is frequently associated with the Trisomy 18 and trisomy 13-15 or ventricular septal defect and patent ductus arteriosus. We present an isolated case of congenital polyvalvular disease in a new born baby with a review of the pertinent literatures, which has not been described in Korea. The mass was discovered as a right atrial mass in the prenatal ultrasonography and it was thought to be either a hematoma or a myxoma in the preoperative echocardiography. Microscopic examination of the surgically resected mass showed irregular thickening, nodulation, and additional features of calcification and ossification in the valvular connective tissue on the body of anterior and septal leaflet of tricuspid valve. Congenital polyvalvular disease should be included in the differential diagnosis in cases showing valvular calcification or ossification in the fetal echocardiography.

• Advances in pediatric surgery
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• v.14 no.1
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• pp.21-26
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• 2008

There is significant morbidity and mortality associated with the combination of esophageal atresia (EA) and duodenal atresia (DA). Nevertheless, the management protocol for the combined anomalies is not well defined. The aim of this study is to review our experience with the combined anomalies of EA and DA. From May 1989 to August 2006, seven neonates were diagnosed as EA with DA at Asan Medical Center. In all cases, the type of EA was proximal EA and distal tracheoesophageal fistula (TEF). The diagnosis of DA was made in theprenatal period in 1, at birth in 4, 4 days after birth in 1 (2 days after EA repair) and at postmortem autopsy in 1. Except the one case where DA was missed initially, primary simultaneous repair was attempted. DA repair with gastrostomy followed by EA repair in 2, EA repair followed by DA repair without gastrostomy in 2, and TEF ligation followed by DA repair with gastrostomy in 1. There were two deaths. One baby had a large posterolateral diaphragmatic hernia, and operative repair was not attempted. The other infant who had a TEF ligation and DA repair with gastrostomy expired from cardiac failure due to a large patent ductus arteriosus. Simultaneous repair of EA and DA appears to be an acceptable management approach for the combined anomalies, but more experience would be required for the selection of the primary repair of both anomalies.

• Journal of Yeungnam Medical Science
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• v.2 no.1
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• pp.241-247
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• 1985

Bacterial endocarditis has been well recognized as an important complication of congenital heart disease, such as ventricular septal defect, patent ductus arteriosus or pulmonary stenosis. The incidence of right sided bacterial endocarditis is lesser than left sided bacterial endocarditis. Also, pulmonic valve vegetation has been thought to be relatively uncommon. And pulmonary embolism is common in the patients with right sided bacterial endocarditis. So in a patient with fever and evidence of recurrent pulmonary infarction, changing heart murmurs and scattered pneumonic infiltrates, one should direct attention to the heart as a possible source of the infection. Echocardiography with M-mode, 2-D and Doppler mode represents the only noninvasive technic available for detecting vegetations in bacterial endocarditis. In fact, the technic is more sensitive in identifying these lesions than angiography. We experienced a case of ventricular septal defect with bacterial endocarditis, pulmonic valve vegetation and multiple pulmonary embolism diagnosed with Echocardiogram and lung scan, and confirmed by operation. Patch repair of ventricular septal defect, resection of pulmonic valve and vegetation and artificial valve formation with pericardium were done.

• Journal of Genetic Medicine
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• v.13 no.2
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• pp.99-104
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• 2016

$Cant{\acute{u}}$ syndrome (CS, OMIM 239850) is a very rare autosomal dominantly inherited genetic disease characterized by congenital hypertrichosis, neonatal macrosomia, a distinct facial features such as macrocephaly, and cardiac defects. Since the first description by $Cant{\acute{u}}$ et al. in 1982, about 50 cases have been reported to date. Recently, two causative genes for CS has been found by using exome sequencing analyses: ABCC9 and KCNJ8. Most cases of clinically diagnosed CS have resulted from de novo mutations in ABCC9. In this study, we report three independent Korean children with CS resulting from de novo ABCC9 mutations. Our patients had common clinical findings such as congenital hypertrichosis, distinctive facial features. One of them showed severe pulmonary hypertension and hypertrophic cardiomyopathy, which require medical treatment. And, two patients had a history of patent ductus arteriosus. Although two of our patients had shown early motor developmental delay, it was gradually improved during follow-up periods. Although CS is quite rare, there are the concerns about development of various cardiac problems in the lifetime. Therefore, an accurate diagnosis followed by appropriate management and genetic counseling should be provided to CS patients.