• 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.

• Journal of Chest Surgery
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• 제38권2호
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• pp.172-174
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• 2005

유미흉은 드물지만 심각한, 잘 알려져 있는 흘부 및 심장 수술의 합병증이다. 동맥관 개존증 결찰 및 대동맥 축착증 교정술을 시행 받은 3개월 된 남자 환아에서 수술 후 유미흉이 발생하였다. 환자는 흉강 배액과 함께 옥트레오타이드 주사요법과 중쇄지방산 분유를 이용한 보존적 방법으로 성공적으로 치료되었다. 이에 저자들은 수술 후 발생한 유미흉 환자에서 옥트레오타이드를 사용한 보존적 치료로 좋은 결과를 치험하였기에 보고하는 바이다.

• Journal of Chest Surgery
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• 제18권4호
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• pp.574-581
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• 1985

Surgical treatment for PDA has been pivotal in historical development of surgery for congenital heart disease. A clinical study on 36 cases of operated PDA were performed during period from Aug. 1981 to Jul. 1985 at the Department of Thoracic & Cardiovascular Surgery in Chonbuk University. The following results are obtained. 1. The 8 males and 28 females ranged in age from 2 yrs, to 24 yrs, [mean 11 yrs.] 2. Chief complaints of the patients were dyspnea on exertion in 61%, palpitation in 39%, frequent URI in 12%, and no subjective symptoms in 11%. 3. On auscultation, continuous machinery murmur heard in 94% and systolic in 14%. 4. Radiologic findings of chest P-A showed increased density of pulmonary vascularity in 94%, cardiomegaly in 69%, and within normal limits in 5% of the patients. 5. EKG findings of the patients revealed LVH in 69%, RVH in 6%, BVH in 6%, and within normal limits in 17%. 6. Of the 36 patients, cardiac catheterization was performed in 34 patients. The results showed mean Qp/Qs = 2.25, mean Pp/Ps=0.42, and mean systolic pulmonary arterial pressure=53mmHg. 7. Surgical methods were as followed: The 32 case of ductal ligation and one case of division & suture technique for PDA through the left posterolateral thoracotomy were done. And 2 cases of ductal ligation one suture closure through the pulmonary artery were performed under the cardiopulmonary bypass. 8. Intraoperative complication was ductal rupture with division 8< suture for PDA and transient hoarseness in 1, recanalization in 1, and urethral stricture in 1 case postoperatively. 9. One patient died due to ductal rupture intraoperatively and operative mortality was 2.8%.

• Journal of Genetic Medicine
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• 제11권2호
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• pp.86-90
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• 2014

Smith-Lemli-Opitz syndrome (SLOS) is a rare autosomal recessive disorder caused by 7-dehydrocholesterol reductase deficiency. The characteristic clinical features are syndactyly of the second and third toes, facial dysmorphism, multiple malformations, and intellectual disability. Few cases of SLOS have been reported in Korea. We observed a male patient with SLOS who presented with typical facial features, undescended testes, microcephaly, bilateral syndactyly of the second and third toes, and cardiac defects, including patent ductus arteriosus and atrial septal defect. Mutation analysis of the DHCR7 gene identified compound heterozygous mutations of c.907G>A (p.Gly303Arg) and c.1055G>A (p.Arg352Gln). In a review of the literature, c.1054C>T (p.Arg352Trp) was the most common mutation reported in Far East Asian countries. This report describes the clinical features, biochemical data, molecular characteristics, and clinical outcome of a Korean patient with SLOS.