선천성 심질환과 관련한 감염성 심내막염의 임상 양상 변화

백재숙,방지석,배은정,노정일,이환종,최정연,윤용수,손대원,오병희 대한심장학회 2007 Korean Circulation Journal Vol.37 No.12

Background and Objectives : The relative proportion of infective endocarditis (IE) with congenital heart disease (CHD) has increased because of improved survival with CHD. This may afect the current profile of IE with CHD. The aim of this study is to assess the changing profiles of IE with CHD. Subjects and Methods : Al cases diagnosed period (period I-prior to 1995, period II-after 1996). Duke criteria were used for the definition of diagnosis. Results: As a whole, 121 episodes ocurred (63 episodes in period I and 58 episodes in period II). Although the mean age was similar for patients diagnosed in the two periods, the proportion of younger age patients was larger during period II. During period II, the number of non-surgical ventricular septal defect (VSD)similar in patients for both periods. S. viridans was the most comon causative organism in patients during both periods. Post-surgical cases and IE after a dental procedure increased during period II. The in-hospital mortality rate, total complication rate, and proportion of IE requiring early surgery were not diferent for patients in either period. However, the interval to early surgery was shorter for patients during period II (period I 35± 21.1 days, period II 22± 16.5 days, p= 0.041). Conclusion : with similar clinical features exhibited during the two diferent periods. The presence of non-surgical VSD as a major underlying defect indicates that one needs to pay more attention to the education of the population at risk. (Korean Circ J 2007 ;37:635-640) 배경 및 목적 선천성 심질환에 대한 수술 방법과 치료 성적의 향상으 로 선천성 심질환과 관련된 감염성 심내막염의 발생은 점 차 증가하고 있으며 이들 환자에서 감염성 심내막염은 중요 한 합병증이다. 이 연구의 목적은 선천성 심질환을 가진 소 아 및 성인에서 발생한 감염성 심내막염의 변화 추이에 대한 평가이다. 방 법 1985년 1월부터 2006년 5월까지 감염성 심내막염으로 서울대병원에서 입원 치료를 받았던 환자들 중 선천성 심 질환을 가진 환자를 대상으로 하여 환자들의 의무 기록을 후 향적으로 분석하였고, 두 기간으로 (1995년 기준) 분류하여 결과를 분석하였다. 결 과 1985년부터 2006년 5월까지 선천성 심질환과 동반한 감 염성 심내막염은 121예에서 발생하였으며, 기간 1 (1985년부 터 1995년까지)에 63명, 기간 2 (1996년부터 2006년 5월까 지)에 58명이었다. 발병시의 평균 연령은 15.4±14.3세 였고, 기간 1은 16.8±12.6세, 기간 2는 12.9±14.1세로 두 기 간 사이에 차이는 없었다. 동반된 심질환으로 교정을 시행하 지 않은 심실 중격 결손은 감소하였으나, Rastelli 수술을 시행한 경우는 증가하였다. 원인균으로 viridans streptococci가 양 기간 모두에서 가장 많았으며, 포도구균은 수술 직후 증례가 증가했음에도 오히려 감소하는 소견을 보였다. 사망률, 합병증, 수술적 치료를 시행한 증례는 두 기간 사이 에 차이는 없었지만, 수술적 치료는 기간 2에서 좀 더 일찍 이루어졌다. 결 론 감염성 심내막염은 선천성 심질환에서 중요한 합병증이 며, 이에 대한 관리와 예방 교육이 지속적으로 유지되어야 한다. 특히, 인공 도관을 이용한 교정술을 시행한 경우는 더 욱 주의가 필요할 것이다. 또한 작은 심실 중격 결손도 간 과되어서는 안 된다.

Childhood Brugada Syndrome in Two Korean Families

이윤식,백재숙,김소연,서상원,권보상,김기범,배은정,박성섭,노정일 대한심장학회 2010 Korean Circulation Journal Vol.40 No.3

Since the first descriptions of Brugada as a new clinical entity defined by sudden cardiac death in patients with typical electrocardiogram (ECG) patterns, Brugada syndrome (BS) has been increasingly diagnosed. This syndrome is known as a disease that is inherited via an autosomal dominant trait, and the SCN5A mutation has been found in 20-25% of BS patients. Because BS primarily manifests in adulthood, little information is available on BS during childhood. Although there have been several reports on adult BS in Korea, pediatric BS has not been reported. Herein, we report on childhood BS in two families. One infantile BS patient and his family had a novel SCN5A mutation (c.4035G>T, p.W1345C, heterozygote) in domain III of the sodium channel.

Outcomes of Chronic Peritoneal Dialysis by Various Modalities in Korean Children - A Single Center Study

이성하,백재숙,이현경,한경희,최현진,이범희,조희연,정해일,최용,하일수,Lee, Sung-Ha,Baek, Jae-Suk,Lee, Hyun-Kyung,Han, Kyoung-Hee,Choi, Hyun-Jin,Lee, Bum-Hee,Cho, Hee-Yeon,Cheong, Hae-Il,Choi, Yong,Ha, Il-Soo Korean Society of Pediatric Nephrology 2007 Childhood kidney diseases Vol.11 No.2

목 적 : 단일 기관에서 만성복막투석을 시행 받고 있는 소아 환자를 대상으로 후향적 단면연구를 시행하여 복막투석 방법에 따른 투석 결과의 차이가 있는지에 대하여 연구해 보고자 한다. 방 법 : 단일 기관에서 2004년과 2007년 사이에 복막투석을 시행 받았던 환자들 중 투석 후 3개월에서 15개월 사이에 복막투석 적절도에 대한 평가가 이루어졌던 35명의 환자를 대상으로 하였다. 연구 당시 시행하고 있던 복막투석 방법에 따라 CAPD 군, CCPD 군과 NIPD 군으로 환자들을 분류하였고 각각 17명, 13명, 5명의 환자들이 포함되었다. 각 군 사이에 항고혈압제를 복용하고 있는 환자 수, 체중, 신장 및 일반화학 검사와 혈색소 검사를 포함한 혈액 검사 결과의 차이가 있는지 비교 분석하였다. 투석 및 잔여 신기능을 통한 주간 $Kt/V_{urea}$, Ccr과 초여과양 및 소변량에 대한 비교 분석도 시행하였다. 표준 방법을 통한 복막평형검사를 시행하여 개인의 복막 투과성에 대해서도 알아보았다. 결 과 : 각 군 간에 항고혈압제를 복용하고 있는 환자의 비율, 체중과 신장 Z-score의 월간 변화 및 혈액검사 평균 수치의 차이는 유의하지 않았다. CAPD 군과 CCPD 군에서, 복막투석 및 잔여 신기능을 통한 주간 Kt/Vurea, Ccr 및 수분 배설 의 차이는 없었다. NIPD 군의 복막투석을 통한 주간 Ccr이 다른 두 군에서보다 유의하게 낮았지만, 잔여 신기능을 통한 보상으로 총 주간 Ccr에는 차이가 없었다. 복막 평형 검사 결과 저 투과성(low transporter) 및 저 평균 투과성(low average transporter)으로 분류되었던 환자들을 대상으로 비교하였을 때, CAPD를 통한 주간 Ccr이 CCPD를 통한 주간 Ccr 보다 유의하게 높았다. 결 론 : 본 연구의 결과를 토대로, 소아 만성복막투석 환자들에 있어서 투석 방법은 환자의 선호도에 의하여 자유롭게 선택될 수 있어야 할 것을 제안하는 바이다. 또한 초여과의 실패 및 부적절한 투석으로 투석 방법의 변경이 불가피한 상황에서, 복막 평형 검사는 투석 방법 변경이 적절이 이루어질 수 있도록 도와주는 귀중한 정보를 제공해 줄 것이다. 투석 방법 간의 명확한 비교를 위하여 향후 복막염의 빈도, 영양상태 및 장기간의 성장등 다양한 투석 성적을 비교하는 추가 연구가 필요할 것으로 생각된다. Purpose : A single center cross sectional retrospective study was performed to compare the outcomes of different peritoneal dialysis(PD) modalities in Korean children. Methods : Among children dialyzed with PD between the year 2004 and 2007, 35 children had reliable data on PD adequacy after 3 to 15 months of dialysis. Subjects were grouped by their modalities; 17, 13 and 5 children were on continuous ambulatory PD(CAPD), continuous cyclic PD(CCPD) and nightly intermittent PD(NIPD), respectively. Body weight and height, number of patients taking anti-hypertensives and laboratory data including biochemical and hemoglobin levels were compared. Dialysis adequacy including weekly Kt/Vurea, creatinine clearance (Ccr) and daily water removal were also compared. Patients were sub-grouped by their peritoneal permeability characteristics. Results : The percentage of patients taking anti-hypertensives, monthly change in Z-scores of body weight and height and laboratory data did not differ among the groups. Patients on CAPD and CCPD showed similar dialysis adequacies. Weekly dialytic Ccr was significantly lower in the NIPD group compared to the others. But total Ccr was not different when residual renal function was added. Weekly dialytic Ccr by CAPD was significantly higher than that of CCPD in low and low-average transporters. Conclusion : We propose that modality can be selected flexibly according to the patients' preferences. And peritoneal permeability characteristics provide valuable information for adjusting PD prescriptions in ultrafiltration failure or in inadequate dialysis. Further study of other clinical performance measures should be performed to clarify the comparable outcomes in different PD modalities.

Pulmonary Atresia with Ventricular Septal Defect and Major Aorto-Pulmonary Collateral Arteries: Management Strategy at Our Hospital and the Results

방지석,백재숙,Ling Zhu,배은정,노정일,최정연,윤용수 대한심장학회 2007 Korean Circulation Journal Vol.37 No.8

Background and Objectives:Based on our previous studies on pulmonary atresia with ventricular septal defect (VSD) and major aorto-pulmonary collateral arteries (MAPCAs), a management strategy for this disease was formulated at our hospital in 1999. To evaluate this strategy, it was applied to the new patients and their outcomes were measured. Subjects and Methods:The subjects were a cohort of newly diagnosed patients who were without any prior interventions and these patients were treated at our hospital from January 2000 to December 2003. The management strategy focused on promoting the growth of the pulmonary arterial confluence (PAC), if present, by performing a right ventricle-pulmonary artery conduit operation (RV-PA conduit). If the PAC was large or absent, then the management plan was discussed among the surgeons after performing an exhaustive work-up. Results:Seventeen patients were enrolled and their age at the time of diagnosis ranged from 8 days to 34 months with a median age of 3 months. The initial surgical method varied from one-stage total correction (4 cases) to a conduit operation with or without additional MAPCAs procedures (13 cases). All but three of the patients survived their initial surgery. There were two late deaths. In addition to the 3 survivors from one-stage total correction, 4 patients underwent a staged total corrective operation. The 5 remaining patients are still a subtotal correction state or a palliated state. Conclusion:When a large PAC is present, one-stage total correction is the best surgical option. However, when the PAC is small, then RV-PA conduit without any vascular procedures may be a good alternative. This staged approach makes a larger PAC, which enables surgeons to create a much easier vascular anastomosis later on. (Korean Circulation J 2007;37:348-352)

소아 급성림프모구백혈병 환자에서 6-mercaptopurine과 6-thioguanine 사용 후 발생한 급성 췌장염 1예

윤종형,백재숙,김은경,강형진,신희영,안효섭 대한혈액학회 2008 Blood Research Vol.43 No.1

Although pancreatitis is known as a common complication during the treatment of acute lymphoblastic leukemia, acute pancreatitis that’s induced by 6-mercaptopurine or 6-thioguanine is very uncommon. We experienced the case of an 11-year-old boy with consecutive acute pancreatitis, and this was induced by 6-mercaptopurine and 6-thioguanine during maintenance chemotherapy of childhood acute lymphoblastic leukemia. We report here on this along with a review of the pertinent literature.

Outcome of Transcatheter Closure of Oval Shaped Atrial Septal Defect with Amplatzer Septal Occluder

송진영,이상윤,백재숙,심우섭,최은영 연세대학교의과대학 2013 Yonsei medical journal Vol.54 No.5

Purpose: For the successful completion of transcatheter closure of atrial septal defects with the Amplatzer septal occluder, shape of the defects should be considered prior to selecting the device. The purpose of this study is to evaluate the results of a transcatheter closure of oval shaped atrial septal defect. Materials and Methods:From November 2009 until November 2011, cardiac computed tomography was performed on 69 patients who needed a transcatheter closure of atrial septal defect. We defined an oval shaped atrial septal defect as the ratio of the shortest diameter to the longest diameter ≤0.75 measured using computed tomography. A trans-thoracic echocardiogram was performed one day after and six months after. Results: The transcatheter closure of atrial septal defect was performed successfully in 24 patients in the ovoid group and 45 patients in the circular group. There were no serious complications in both groups and the complete closure rate at 6 months later was 92.3% in the ovoid group and 93.1% in the circular group (p>0.05). The differences between the device size to the longest diameter of the defect and the ratios of the device size to the longest diameter were significantly smaller in the ovoid group (1.8±2.8 vs. 3.7±2.6 and 1.1±0.1 vs. 1.2±0.2). Conclusion: Transcatheter closure of an oval shaped atrial septal defect was found to be safe with the smaller Amplatzer septal occluder device when compared with circular atrial septal defects.

Pulmonary Arterial Hypertension in Children:A Single Center Experience

김형우,김기범,백재숙,배은정,노정일,최정연,윤용수,제현곤 대한심장학회 2008 Korean Circulation Journal Vol.38 No.12

Background and Objectives: Pulmonary arterial hypertension (PAH) is a rare disease with a poor prognosis. The aim of this study was to characterize PAH in pediatric patients by evaluating the patients demographics, clinical and hemodynamic variables, treatments, and outcomes. Subjects and Methods: Sixty-five patients who were diagnosed with PAH at Seoul National University Children’s Hospital between January 1985 and August 2007 were retrospectively reviewed. Results: There was no difference in gender distribution (males, 33; females, 32). The mean age at the time of diagnosis was 5.7±5.2 years and the mean follow-up period was 6.3±5.5 years. The major causes of PAH were congenital heart disease (CHD) in 32 patients (49.2%) and idiopathic PAH in 11 patients (16.9%). The most common presenting symptom was dyspnea in 44 patients (67.7%). The mean cardiothoracic ratio was 58.9±8.3%, which decreased to 55.9±8.3% after vasodilator therapy (p=0.011). The mean pulmonary arterial pressure at the time of cardiac catheterization was 59.7±18.7 mmHg and the mean pulmonary vascular resistance was 14.9±9.7 wood units·m2. Forty-three of 65 patients (66.2%) had vasodilator therapy (prostacycline, sildenafil, and bosentan). A statistically significant decrease in tricuspid valve regurgitation velocity (4.8±0.8 m/sec vs. 3.6±1.0 m/sec, p=0.001), and an increase in diastolic dimension of the left ventricle (28.3 ±12.1 mm vs. 33.2±10.2 mm, p=0.021) on echocardiography before and after vasodilator therapy was demonstrated. The 5-, 10-, and 15-year survival rates were 96%, 92% and 65%, respectively. Six of 65 patients (9.2%) with PAH died. There was no significant correlation between outcome and the immediate response to the vasodilators. Conclusion: PAH is a devastating disease which is rare in children. PAH in children has a variable pattern cause and progression, the confirmation of which requires analysis of detailed registries from nationwide hospitals. Background and Objectives: Pulmonary arterial hypertension (PAH) is a rare disease with a poor prognosis. The aim of this study was to characterize PAH in pediatric patients by evaluating the patients demographics, clinical and hemodynamic variables, treatments, and outcomes. Subjects and Methods: Sixty-five patients who were diagnosed with PAH at Seoul National University Children’s Hospital between January 1985 and August 2007 were retrospectively reviewed. Results: There was no difference in gender distribution (males, 33; females, 32). The mean age at the time of diagnosis was 5.7±5.2 years and the mean follow-up period was 6.3±5.5 years. The major causes of PAH were congenital heart disease (CHD) in 32 patients (49.2%) and idiopathic PAH in 11 patients (16.9%). The most common presenting symptom was dyspnea in 44 patients (67.7%). The mean cardiothoracic ratio was 58.9±8.3%, which decreased to 55.9±8.3% after vasodilator therapy (p=0.011). The mean pulmonary arterial pressure at the time of cardiac catheterization was 59.7±18.7 mmHg and the mean pulmonary vascular resistance was 14.9±9.7 wood units·m2. Forty-three of 65 patients (66.2%) had vasodilator therapy (prostacycline, sildenafil, and bosentan). A statistically significant decrease in tricuspid valve regurgitation velocity (4.8±0.8 m/sec vs. 3.6±1.0 m/sec, p=0.001), and an increase in diastolic dimension of the left ventricle (28.3 ±12.1 mm vs. 33.2±10.2 mm, p=0.021) on echocardiography before and after vasodilator therapy was demonstrated. The 5-, 10-, and 15-year survival rates were 96%, 92% and 65%, respectively. Six of 65 patients (9.2%) with PAH died. There was no significant correlation between outcome and the immediate response to the vasodilators. Conclusion: PAH is a devastating disease which is rare in children. PAH in children has a variable pattern cause and progression, the confirmation of which requires analysis of detailed registries from nationwide hospitals.

The results of cardiopulmonary exercise test in healthy Korean children and adolescents: single center study

이준석,장소익,김성호,이상윤,백재숙,심우섭 대한소아청소년과학회 2013 Clinical and Experimental Pediatrics (CEP) Vol.56 No.6

Purpose: The cardiopulmonary exercise test (CPET) is an important clinical tool for evaluating exercise capacity and is frequently used to evaluate chronic conditions including congenital heart disease. However,data on the normal CPET values for Korean children and adolescents are lacking. The aim of this study was to provide reference data for CPET variables in children and adolescents. Methods: From August 2006 to April 2009, 76 healthy children and adolescents underwent the CPET performed using the modified Bruce protocol. Here, we performed a medical record review to obtain data regarding patient’ demographics, medical history, and clinical status. Results: The peak oxygen uptake (VO2Peak) and metabolic equivalent (METMax) were higher in boys than girls. The respiratory minute volume (VE)/CO2 production (VCO2) slope did not significantly differ between boys and girls. The cardiopulmonary exercise test data did not significantly differ between the boys and girls in younger age group (age, 10 to 14 years). However, in older age group (age, 15 to 19 years), the boys had higher VO2Peak and METMax values and lower VE/VCO2 values than the girls. Conclusion: This study provides reference data for CPET variables in case of children and adolescents and will make it easier to use the CPET for clinical decision-making.

Pulmonary Hypertension in Preterm Infants With Bronchopulmonary Dysplasia

안효순,배은정,김기범,권보상,백재숙,김이경,김한석,최정환,노정일,윤용수 대한심장학회 2010 Korean Circulation Journal Vol.40 No.3

Background and Objectives: With the increasing survival of preterm infants, pulmonary hypertension (PH) related to bronchopulmonary dysplasia (BPD) has become an important complication. The aim of this study was to investigate the characteristics and outcome of PH in preterm infants with BPD and to identify the risk factors for PH. Subjects and Methods: We reviewed the records of 116 preterm infants with BPD cared for at a single tertiary center between 2004 and 2008. Results: Twenty-nine (25%) infants had PH >2 months after birth. PH occurred initially at a median age of 65 days (range, 7-232 days). Severe BPD, a birth weight <800 g, long-term ventilator care and oxygen supplementation, a high ventilator setting, infection, and a patent ductus arteriosus (PDA)were related to PH based on univariate analysis (p<0.05). The infants who had longer oxygen supplementation were significantly more likely to have PH (odds ratio, 18.5; 95% confidence interval, 4.1-84.6; p<0.001). PH was improved in 76% of infants after a median of 85 days (range, 20-765 days). Four infants (14%) died. The death of 3 infants was attributed to PH. Conclusion: BPD was frequently complicated by PH. Although PH resolved in the majority of infants, PH in preterm infants with BPD can be fatal. Regular screening for PH and adequate management are required. :

Cardiac Resynchronization Therapy in Infant with Dilated Cardiomyopathy during Extracorporeal Membrane Oxygenator

방지현,박천수,오유나,고재곤,강소연,백재숙 대한흉부외과학회 2015 Journal of Chest Surgery (J Chest Surg) Vol.48 No.1

Although heart transplantation is a final therapeutic option in pediatric patients with dilated cardiomyopathy (DCMP),the shortage of pediatric heart donors is a major obstacle. In adults with DCMP characterized by cardiac dyssynchrony,cardiac resynchronization therapy (CRT) is known to be an effective treatment option. However, there is alack of evidence on the effectiveness of CRT in infants with DCMP. Several studies have reported improvement inhemodynamics and cardiac performance following CRT in infants with DCMP. Here, we report CRT in an infantwith DCMP during extracorporeal membrane oxygenation with 5 months of follow-up.