Long-Term Prognosis for Patients with Kawasaki Disease Complicated by Large Coronary Aneurysm (diameter ≥6 mm)

방지석,김기범,권보상,송미경,안효순,송영환,배은정,노정일 대한심장학회 2017 Korean Circulation Journal Vol.47 No.4

Background and Objectives: Some patients with Kawasaki disease (KD) develop large coronary aneurysms and subsequent coronary stenosis or obstruction, leading to ischemic heart disease. This study examined the long-term outcomes of patients with KD complicated by large coronary aneurysms. Subjects and Methods: The medical records of 71 patients (53 men and 18 women) diagnosed with large coronary aneurysms (diameter ≥6 mm) between December 1986 and December 2013 were retrospectively reviewed from our institutional database. Results: The mean age at onset was 4.6±3.3 years, and the mean follow-up duration was 12.5±6.9 years. Maximum coronary artery internal diameter ranged from 6.1 to 25 mm. Giant coronary aneurysms occurred in 48 patients and coronary aneurysms 6-8 mm in diameter developed in 23 patients. Coronary stenosis and/or complete occlusion occurred in 30 patients (42.3%). Catheter and/or surgical interventions (mean: 1.5 interventions, range: 1-5 interventions) were performed in 20 patients (28.2%), 9 months to 18 years after KD onset, resulting in 33.7% cumulative coronary intervention rates at 20 years after onset. There were no differences in cumulative coronary intervention rates between two coronary aneurysm groups (6-8 mm vs. ≥8 mm). Myocardial infarction occurred in 7 patients with a giant aneurysm and there was one death. Conclusions: Long-term survival of patients with KD complicated by large coronary aneurysm was good even though 28.2% of patients underwent multiple catheter or surgical interventions. Careful follow-up is also necessary in KD patients with coronary aneurysms 6-8 mm in diameter, such as those with giant aneurysms.

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)

소아기 피부근염의 임상적 고찰; 진단시 임상증상을 중심으로 한 고찰

이소영,방지석,김희석,김중곤 대한소아청소년과학회 2007 Clinical and Experimental Pediatrics (CEP) Vol.50 No.11

Purpose : Juvenile dermatomyositis (JDM) is the most common of the idiopathic inflammatory myopathies in children. The purpose of this study is to observe demographic, initial presentations, duration of time between disease onset and diagnosis, clinical manifestations and laboratory findings at diagnosis of patients with JDM. Methods : Forty seven patients identified at Seoul National University Children's Hospital from January 1986 to May 2007. Medical records were reviewed retrospectively focusing on initial presentations, clinical manifestations and laboratory findings at the time of diagnosis of patients with JDM. Results : Male and female patients were 25 and 22, respectively and sex ratio was 1.14:1. The average age at the time of diagnosis was 6.51 years. Skin rash (94%) was the most common symptom, followed by the proximal muscle weakness (89%). The disease activity score was 10.8. The duration between the onset of the skin rash and the muscle weakness and diagnosis was 7.18 and 4.70 months, respectively. The serum muscle enzymes, LDH, AST, CK and aldolase, were elevated in the patient with JDM. Autoimmune antibodies, antinuclear antibody, anti SSA antibody and anti SSB antibody, were negative findings. Electromyography findings were consistent with JDM in 88% of the patients, the muscle biopsy was in 91% and all MRI findings were compatible with those of patients with JDM. The most common symptom besides musculocutaneous lesions was the calcinosis (62.5%). The most common site of calcinosis was the pelvic area and buttocks. Conclusion : This study shows that the major symptoms are proximal muscle weakness and cutaneous lesion, and they are important to diagnose JDM. 목 적 : 소아기 피부근염은 피부와 근육을 침범하는 만성 염증성 질환으로 비교적 드문 질환이다. 우리 나라에서는 산발적인 증례 보고는 있어 왔으나 종합적인 분석은 두가지의 보고가 있을 뿐이다. 이에 저자들은 진단 당시에 소아기 피부근염 환자들의 임상적 증상 및 검사 소견을 분석하였다. 방 법 : 1985년 1월부터 2007년 5월까지 서울대학교병원 소아과 면역 질환 분과에서 소아기 피부근염으로 진단받거나 타병원에서 진단 후 전원된 환아 47명을 대상으로 하여 연령 및 성병분포, 초기 임상 증상, 증상 발현으로부터 진단까지의 시간, 혈청근효소, 자가항체, 근전도, 근생검, 자기공명영상, 합병증 등을 분석하였다. 결 과 : 대상 환아들은 모두 47명으로 남자가 25명 여자가 22명으로 남녀 비율은 1.14:1이었다. 진단 당시 연령은 6.51±0.88세(1.5-13.25세)였다. 피부 발진은 모든 환아에서 나타났으나 3례의 환아에서는 진단 후 발생하였고, 얼굴 발진이 가장 많았다. 진단 당시 근쇠약감은 44례의 환아에서 나타났으며, 2례는 진단 후 나타났다. 근위부 근쇠약감은 계단을 잘 오르지 못하는 것이 가장 흔한 증상이었다. 질병 활성도는 평균 10.8점(3-17점)이었으며, 근쇠약감은 평균 4.91점(0-9점), 피부 병변은 평균 5.89점(0-9점)이었다. 피부와 근육의 증상 이외의 증상은 43%에서 관찰되었고, 석회증이 30%, 관절 구축이 21%, 흡인성 폐렴이 13 %로 관찰되었다. 석회증이 발생한 환아 중 47%는 진단 당시부터 관찰되었고 석회증이 발생한 위치는 골반과 둔부가 50%에 해당하였다. 혈청 근효소의 상승은 LDH가 96%, AST가 80%였다. 자가 항체 중 항핵항체가 59%로 가장 많은 양성을 나타내었으며, 이중 반점 모양이 58%에서 관찰되었다. 근전도는 88%에서 근염에 합당한 소견을 보였고, 근생검은 91%에서 피부근염에 합당한 소견을 보였다. 자기공명영상은 9례에서 시행되었고 모두 T2 강조 영상에서 고신호 강도 변화를 보여 근염에 합당하였다. 결 론 : 소아기 피부근염을 진단하기 위해서는 여러가지 진단 방법이 사용되고 있으나, 얼굴 발진, Gottron 발진, heliotrope 발진과 같은 피부 발진과 상하지 근위부 근육의 쇠약감과 같은 임상증상이 중요할 것으로 생각된다.

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.

주의력결핍 과잉운동장애 소아에서 배뇨장애와 변비의 유병률

김준영,이은섭,방지석,오연정,이용주,성태정,이건희,이정원,Kim, Joon Young,Lee, Eun Seob,Bang, Ji Seok,Oh, Yeon Joung,Lee, Yong Ju,Sung, Tae-Jung,Lee, Kon-Hee,Lee, Jung Won 대한소아신장학회 2014 Childhood kidney diseases Vol.18 No.2

목적: 주의력결핍 과잉운동장애(ADHD)는 소아에서 3-5%의 유병률을 보이며 전두엽의 억제기전 저하와 대뇌의 카테콜라민 대사이상이 관련된다고 알려져 있다. ADHD 환아에서 야뇨증을 비롯한 다양한 하부요로계 증상을 동반한다는 보고가 있지만 국내에서는 ADHD 환아들에 대한 보고가 없기에 저자들은 ADHD 환아에서 배뇨증상을 알아보기 위해 본 연구를 시행하였다. 방법: 2009년 10월부터 2011년 3월까지 DSM-IV 진단기준에 근거하여 ADHD로 진단받은 33명의 환아들과 같은 기간 동안 신경계 질환이나 요로계 질환 없이 상기도 감염으로 외래를 내원한 환아 30명을 대상으로 배뇨와 배변에 대한 설문지를 시행하였다. 결과: ADHD 환자군의 평균 연령은 $9.09{\pm}2.8$세(남 28: 여 5) 이었고, 대조군의 평균 연령은 $8.58{\pm}3.1$ (남 20: 여 10) 이었다. ADHD 환아군에서 대조군에 비해 요절박(P=0.017), 절박 요실금(P=0.033), 변비(P=0.045)의 유병률이 대조군에 비해 유의하게 높았고, 복압배뇨, 간헐뇨, 배뇨지연 행동에는 두 군간에 유의한 차이가 없었다. 야뇨증은 ADHD 환아군에서 12.1% (4/33)로 대조군의 0%에 비해 많았지만 통계학적인 유의성은 없었다. 결론: ADHD 환아는 대조군에 비해 요절박, 절박 요실금과 변비의 유병률이 유의하게 높았다. Purpose: Attention deficit hyperactivity disorder (ADHD) has been associated with impairments in frontal inhibitory function and the catecholaminergic system. ADHD is diagnosed in 3-5% of children. Children with ADHD seem develop various forms of urinary problems such as nocturnal enuresis, dysfunctional voiding, and diurnal incontinence. However, no data exist to confirm the presence of these problems in Korean children with ADHD. We investigated the clinical findings of voiding dysfunction in children with ADHD. Methods: Between October 2009 and March 2011, a total of 63 children (33 with ADHD, 30 with an upper respiratory infection, as a control group) were enrolled. ADHD was diagnosed using the diagnostic and statistical manual of mental disorders (DSM)-IV criteria. A comprehensive survey of voiding and defecation was administered. Results: The patient group included 28 boys and 5 girls; the control group comprised 20 boys and 10 girls. The mean age was $9.09{\pm}2.8$ years in the ADHD group and $8.58{\pm}3.1$ years in the control group. Children with ADHD had a statistically significantly higher incidence of urgency (P =0.017), urge incontinence (P =0.033), and constipation (P =0.045). There was no significant difference in the incidence of straining, intermittency, holding maneuvers, or nocturnal enuresis. Conclusion: Children with ADHD in Korea have significantly higher rates of urgency, urge incontinence, and constipation than those without ADHD.

학령전기 만성 기침 환아에서 알레르기 감작의 위험 요인

이은섭 ( Eun Seob Lee ),방지석 ( Ji Seok Bang ),오연정 ( Yeon Joung Oh ),이정원 ( Jung Won Lee ),성태정 ( Tae Jung Sung ),이용주 ( Yong Ju Lee ),이건희 ( Kon Hee Lee ) 대한천식알레르기학회 2015 Allergy Asthma & Respiratory Disease Vol.3 No.2

Purpose: Chronic cough is one of the major symptoms of asthma and allergic sensitization and may appear prior to the onset of asthma. The object of this study was to investigate the risk of allergic sensitization in preschool children with chronic cough. Methods: We reviewed the medical records of 99 preschool children presenting with chronic cough but not with allergic rhinitis, atopic dermatitis, recurrent wheezing, or lower respiratory tract infection between November 2011 and July 2013. Results: Fifty-four children (55%) were sensitized at least one of the following inhalant allergens: Dermatophagoides pteronyssinus, Dermatophagoides farinae, cockroach, Alternaria alternata, dog dander, and cat epithelium. Children with allergic sensitization had a higher blood total IgE levels transformed by common logarithm (1.9±0.6 IU/mL vs. 1.3±0.5 IU/mL, P<0.001) and eosinophils (3.7%±2.5% vs. 2.7%±2.0%, P=0.043), more frequent parental history of allergy (68% vs. 48%, P=0.044) and less frequent history of breast milk feeding (68% vs. 86%, P=0.041) than those without. Conclusion: These results suggest that the ratio of allergic sensitization may be 50% or more in preschool children with chronic cough and that parental history of allergy and formula milk feeding may be associated with allergic sensitization. (Allergy Asthma Respir Dis 2015;3:105-108)

The outcome of percutaneous stent implantation in congenital heart disease: experience of a single institute

김문선,윤자경,김성호,방지석,장소익,이상윤,최은영,박수진,권혜원 대한소아청소년과학회 2018 Clinical and Experimental Pediatrics (CEP) Vol.61 No.6

Purpose: The efficacy of percutaneous stent implantation for congenital heart disease (CHD) in Korea, where stent availability is limited, has not been determined. This study evaluated the acute and midterm results of stent implantation in different CHD subgroups. Methods: Stents were implanted in 75 patients with 81 lesions: (1) pulmonary artery stenosis (PAS) group, 56 lesions in 51 patients; (2) coarctation of the aorta (CoA) group, 5 lesions in 5 patients; (3) Fontan group, 13 lesions in 12 patients; (4) ductal stent group, 3 lesions in 3 patients; and (5) other CHD group, 4 lesions in 4 patients. Mean follow-up duration was 2.1 years (0.1–4 years). Medical records were reviewed retrospectively. Results: The minimum lumen diameter (MLD) in PAS and CoA increased from 5.0 ± 1.9 mm and 8.4 ± 1.6 mm to 10.1 ± 3.6 mm and 12.3 ± 2.5 mm, respectively (P<0.01). In the PAS group, pressure gradient decreased from 25.7 ± 15.6 mmHg to 10.4 ± 10.1 mmHg, and right ventricular to aortic pressure ratio from 0.56 ± 0.21 to 0.46 ± 0.19. In the CoA group, the pressure gradient decreased from 50 ± 33 mmHg to 17 ± 8 mmHg. In the ductal stent group, the MLD of the ductus increased from 2.3 mm to 4.3 mm and arterial oxygen saturation from 40%–70% to 90%. No deaths were associated with stent implantation. Stent migration occurred in 3 patients, but repositioning was successful in all. Stent redilation was performed successfully in 26 cases after 29 ± 12 months. Conclusion: Percutaneous stent implantation was safe and effective, with acceptable short and mid-term outcomes in Korean CHD patients.

임신 중 항고혈압제 및 황산마그네슘을 투여 받은 고혈압 장애를 가진 산모에서 태어난 미숙아의 임상 예후

서봉설 ( Bong Sul Suh ),고근혁 ( Keun Hyeok Ko ),방지석 ( Ji Seok Bang ),오연정 ( Yeon Joung Oh ),이정원 ( Yong Ju Lee ),이용주 ( Jung Won Lee ),이건희 ( Kon Hee Lee ),성태정 ( Tae Jung Sung ) 대한주산의학회 2015 Perinatology Vol.26 No.3

Purpose: Hypertensive disorders of pregnancy increase morbidity and mortality of fetus and neonates. Recently some studies revealed that antihypertensive agents affected the neonatal outcomes. The aim of this study was to investigate the prognosis of preterm infants delivered from the mothers with hypertensive disorders who were treated with antihypertensive agents and magnesium sulfate. Methods: This retrospective study was conducted on preterm infants who were delivered from normotensive mother (control, n=436) and antihypertensive drugs +/- magnesium sulfate treated mother (study, n=150) between January 2009 and December 2013. Study group were divided into two groups based on whether they received antihypertensive drugs only (n=110) and additional magnesium sulfate (n=40). We compared the characteristics of mothers and neonatal outcomes. Results: Study group had shorter gestational age (32.2±3.5 weeks vs. 33.7±3.0 weeks, P=0.000) and lower birth weight (1,810.5 ± 689.2 g, 2,212.1 ± 604.9 g, P=0.000), and higher rate of small for gestational age infants (22% vs 11%, P=0.000). One minute and 5 minutes Apgar score were lower, but duration of hospital days, oxygen supplement and mechanical ventilation were longer in study group. Respiratory distress syndrome, bronchopulmonary dysplasia, patent ductus arteriosus, retinopathy of prematurity, intraventricular hemorrhage occurred more in study group than control. The group treated with magnesium sulfate together with antihypertensive agent had lower 1 minute and 5 minutes Apgar score than the group taken antihypertensive agent only. Conclusions: Mothers with hypertensive disorders have increased the risk of preterm delivery, low birth weight, and high neonatal morbidity rate. Therefore it is important to predict and manage possible complication. Moreover, if magnesium sulfate is taken, careful neonatal monitoring is needed because of possible low Apgar score.

산전검사를 시행하지 않은 산모에서 태어난 신생아의단순 포진 바이러스 2형 뇌염 1례

이은섭 ( Eun Seob Lee ),김준영 ( Joon Young Kim ),이건희 ( Kon Hee Lee ),이정원 ( Jung Won Lee ),이용주 ( Yong Ju Lee ),오연정 ( Yeon Joung Oh ),방지석 ( Ji Seok Bang ),성태정 ( Tae-jung Sung ) 대한주산의학회 2014 Perinatology Vol.25 No.3

Neonatal herpes simplex virus (HSV) encephalitis is a rare disease nowadays because of prenatal screening test and management. It shows progressive central nervous system manifestations affecting predominantly temporal and frontal lobes. Early diagnosis of HSV encephalitis is important since even with the early initiation of high-dose intravenous acyclovir therapy, it results in serious morbidity among survivors. A 14-day-old neonate with fever and poor oral intake was admitted via emergency department. The next day she had seizures and the brain was damaged with permanent sequelae despite of early administration of intravenous acyclovir on day 2 of admission. We report a serious case of HSV encephalitis diagnosed as type 2 HSV by PCR and culture of a newborn who was delivered by a mother without proper prenatal screening test.