• Clinical and Experimental Pediatrics
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• 제58권6호
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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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• 제43권5호
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• pp.518-521
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• 2010

생후 1일된 환아가 청색증과 심잡음을 주소로 전원되었다. 심장 초음파 검사를 시행한 결과, 폐동맥 폐쇄가 동반되어 동맥관 의존성 폐혈류를 보이는 증상이 심한 엡스타인 기형(Carpentier type C)이었다. 심방화된 우심실의 벽은 매우 얇고 수축력이 저하되어 있었으며 기능적 우심실의 크기가 매우 작고 폐동맥 폐쇄가 동반되어 양심실 교정이 불가능하다고 판단하였다. 생후 1개월 째 우심방 절제 성형술, 심방화된 우심실의 광범위한 절제 후 봉합 폐색, 변형 Blalock-Taussig 단락술을 시행하였다. 환아는 별다른 문제 없이 퇴원하였으며 생후 5개월에 양방향성 상대정맥-폐동맥 단락술을 시행받았다. 저자 등은 심한 증상을 나타내는 신생아 엡스타인 기형에서 우심실 제외 수술을 시행하여 좋은 결과를 얻었기에 보고하는 바이다.

• Journal of Chest Surgery
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• 제41권4호
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• pp.499-503
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• 2008

원위부 대동맥-폐동맥창, 우폐동맥 대동맥기시, 온전한 심실중격결손, 개방성동맥관, 대동맥궁차단으로 구성된, 소위 Berry syndrome이라고 불리는 이 기형은 매우 드문 복잡 심기형이다. 생후 19일된 신생아에서 자가동맥 피판을 이용한 일차 완전교정술과 추적관찰에서 발생한 우폐동맥 협착 및 그의 풍선성형술에 의한 성공적 치료를 문헌고찰과 함께 보고한다.

• Clinical and Experimental Pediatrics
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• 제63권2호
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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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• 제23권4호
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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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• 제12권3호
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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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• 제34권8호
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• pp.626-629
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• 2001

선천선 다발성 판막질환(Congenital Polyvalvular Disease)은 결체조직의 이상으로 인해 한 개 이상의 심장판막에 비정상적 기형을 초래하는 질환으로 그 원인은 아직 확실히 알려져 있지 않다. 이 질환은 제 18번 또는 13∼15번 삼염색체 증후군에서 자주 관찰되며, 심실중격결손증, 동맥관 개존증 등의 심장기형을 동반하기도한다. 환아는 산전 초음파 검사에서 우심방내의 종괴가 발견되었고, 출생 후 시행한 심초음파 검사에서 삼첨판위의 혈종 또는 점액종이 의심되어 수술을 시행하였다. 종괴는 삼천판막의 전판막첨과 중격판막첨으로부터 완전히 제거되었고 병리학적 검사에 불규칙하게 두꺼워지고 결절화되어 있었으며, 석회화와 골화의 소견을 보였다. 태아의 산전 심초음파 검사에서 판막에 석회화 소견이 관찰될 때 선천성 다발성 판막질환도 염두에 두어야 할 의미 있는 소견이라고 생각된다. 저자들은 선천성 다발성 판막질환 1예를 경험하여 이의 임상 및 조직소견을 문헌 고찰과 함께 보고하고자 한다.

• Advances in pediatric surgery
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• 제14권1호
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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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• 제2권1호
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• pp.241-247
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• 1985

저자 등은 1985년 8월 7일 본 영남대학교 의과대학 부속병원 내과에 입원한 선천성 심실중격결손증 환자에서 우측심실의 세균성 심내막염, 폐동맥 판막, 세균성 증식물 및 다발성 폐동맥 색전증을 초음파심음향도로 진단하고 이를 수술로 확인하였기에 이에 보고하는 바이다.

• Journal of Genetic Medicine
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• 제13권2호
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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.