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소아 감염성 심내막염의 임상양상 : 11년간(1987-1997)의 고찰
유정진,곽영호,홍정연,정혜선,송진영,이환종,노정일,최정연,윤용수 대한소아청소년과학회 2000 Clinical and Experimental Pediatrics (CEP) Vol.43 No.4
Purpose : Infective endocarditis(IE) is a serious complication in children with structural heart disease. We reviewed 35 cases of IE to identify the recent changes in the pattern of preexisting heart diseases, the spectrum of causative organisms and prognosis. Methods : The clinical records of children diagnosed as IE at the Seoul National University Children's Hospital from January 1987 through December 1997, were reviewed retrospectively. Duke criteria was used for diagnosis. Cases were categorized into primary group(PG) IE in an unoperated heart and post operative group(POG), and the latter further into early POG(within 2 months after operation) and late POG. Results : There were 35 cases of IE developed in 34 patients; 18 cases in the PG, 6 cases in the early POG, and 11 cases in the late POG. Male to female ratio was 16 : 19. Mean age of POG, especially early POG was less than that of PG(early POG : late POG : PG=1.65 years : 6.5 years : 8.34 years, P=0.0267). Preexisting heart diseases were identified in 30 cases; rheumatic heart disease 1 case and congenital heart disease(CHD) 29 cases. Causative organisms were identified in 80%; viridans streptococci, 10 cases(33.3%); pneumococci, 2 cases; Group-D streptococci, 3 cases; staphylococci, 8 cases; Gram(-) organisms, 5 cases and Candida albicans, 2 cases. Vegetation was detected in 88.9% of PG and 64.7% of POG. The most common indication for surgery was uncontrolled infection, which were required in 9 cases. The overall mortality rate was 12.1%. Mortality in POG was higher than that of PG(23.5% versus 0%, P=0.033). Conclusion : The discrepancy of mean age among patient groups and the high proportion of patients belonging to POG, were consistent with the increase in the number of newly risky population that survived after cardiac surgery. A more aggressive consideration for operative management may improve the treatment results. 목 적 : 감염성 심내막염(IE)은 구조적 심장질환이 있는 환아들에서 심각한 결과를 초래할수 있는 중요한 합병증으로서, 근래 들어 기저 심장질환의 양상과 원인균의 빈도 변화 등이 있어 왔다. 그 임상양상의 변화된 특징을 관찰하고자 본 연구를 시행케 되었다.방 법 : 1987년 1월에서 1997년 12월 사이에 IE 진단으로 서울대학교 어린이 병원서 입원치료를 받았던 환아들을 대상으로 그 임상양상을 후향적으로 고찰하였다. IE 진단기준은 Duke criteria를 이용하였고, 환아들은 기존 심질환의 수술과 무관하게 발생한 일차군, 수술후 2개월 내외에 발병한 조기 및 만기 수술후 발병군으로 구분 하였다.결 과 : 총 35례중 일차군 18례, 조기 및 만기 수술후 발병군 각각 6례, 11례로 구분되었다. 남녀 비는 16 : 19, 전체 평균 연령은 6.61±5.56세 이었으며, 술후 발병군에서 연령이 낮았다. 기존 심질환으론 류마티성 심질환 1례, 선천성 심질환(CHD) 29례, 심질환 없던 예가 5례였다. 선천성 심질환중 심실 중격결손(VSD)가 13례로 가장 많았으며, 청색증형 CHD는 10례이고 술후 발병군에서 유의하게 많았다. 그외 사망율이 수술후 발병군에서 높았으나 증상, 증후, 검사 소견, 원인균 종류, 합병증 발생 및 수술 적응증에선 발병군간에 유의한 차이를 보이지 않았다. 원인균으론 viridans streptococci가 10례(33.3%)로 가장 많았으며, 폐렴구균 2례, D 그룹 연쇄상구균이 3례, 포도상구균 8례(S. aureus 6례, S. epidermidis 2례), 그람음성균이 5례, Candida albicans 2례 등이었다. 우종(vegetation)은 일차군에서 88.9%, 술후 발병군에서 64.7%에서 발견되었다. 합병증으론 심부전이 전체 환자들중 48.6%로 가장 많았으며, 중추신경계 합병증 14.3%, 폐허혈 11.4 %, 기타부위 색전증 8.5%, 심근농양 및 진균성 동맥류 각각 2.9%이었다. 수술 적응증으론 조절되지 않는 감염이 9례로 가장 많았으며 그람음성균의 경우 1례를 제외하고 모두 포함되었고, 연쇄상 구균의 경우 이에 포함된 예가 없었다. 치료 결과 의학적 권고에 반한 퇴원 2례, 투약 치료후 생존 14례, 수술후 생존 15례, 투약 치료중 사망 3례, 수술후 사망 1례로 사망율 12.1 %이었다.
생후 3개월에서 7세 미만 정상 소아에서의 관상동맥 직경
유정진,조숙경,박용민,이란,정소정,배선환 대한소아청소년과학회 2008 Clinical and Experimental Pediatrics (CEP) Vol.51 No.6
Purpose:This study was designed to investigate normal domestic values for the diameter of the left main coronary artery (LCA), the left anterior descending coronary artery (LAD) and the right coronary artery (RCA). These data are necessary to define dilatation of coronary arteries in Kawasaki disease cases. Methods:Study subjects were 43 normal healthy children whose ages ranged from 3 months to 6 years. They children visited Konkuk University hospital for echocardiograph examination between March 2005 and November 2007. Measurements of coronary arterial diameters at each branch were done by off-line analyses of recorded images. Simple regression analysis of each the measurements were performed using the body size (body surface area, etc.) as the independent variable. Results:Body surface area was significantly related to the diameters of LCA (r2=0.20, P=0.0038), of LAD (r2=0.41, P< 0.0001), and of RCA (r2=0.30, P=0.0002). In the regression model, the estimates of the y-intercept were 1.703, 1.058, and 1.007; the estimates of the regression coefficient were 0.971, 1.175, and 1.177; and the estimates of the standard deviation were 0.315, 0.221, and 0.282 with respect to the three coronary arteries. Conclusion:A the linear regression model of the diameters of three coronary arteries adjusted for body surface area was produced. With these results, the Z-score calculation of the diameter of three coronary arteries, based on normal domestic data, will be possible. (Korean J Pediatr 2008;51:629-633) Purpose:This study was designed to investigate normal domestic values for the diameter of the left main coronary artery (LCA), the left anterior descending coronary artery (LAD) and the right coronary artery (RCA). These data are necessary to define dilatation of coronary arteries in Kawasaki disease cases. Methods:Study subjects were 43 normal healthy children whose ages ranged from 3 months to 6 years. They children visited Konkuk University hospital for echocardiograph examination between March 2005 and November 2007. Measurements of coronary arterial diameters at each branch were done by off-line analyses of recorded images. Simple regression analysis of each the measurements were performed using the body size (body surface area, etc.) as the independent variable. Results:Body surface area was significantly related to the diameters of LCA (r2=0.20, P=0.0038), of LAD (r2=0.41, P< 0.0001), and of RCA (r2=0.30, P=0.0002). In the regression model, the estimates of the y-intercept were 1.703, 1.058, and 1.007; the estimates of the regression coefficient were 0.971, 1.175, and 1.177; and the estimates of the standard deviation were 0.315, 0.221, and 0.282 with respect to the three coronary arteries. Conclusion:A the linear regression model of the diameters of three coronary arteries adjusted for body surface area was produced. With these results, the Z-score calculation of the diameter of three coronary arteries, based on normal domestic data, will be possible. (Korean J Pediatr 2008;51:629-633)
Pulmonary hypertension due to obstructive sleep apnea in a child with Rubinstein-Taybi syndrome
최형순,유정진,김영휘,고재곤,박인숙 대한소아청소년과학회 2012 Clinical and Experimental Pediatrics (CEP) Vol.55 No.6
Rubinstein-Taybi syndrome (RTS) is characterized by peculiar facies,mental retardation, broad thumbs, and great toes. Approximately onethird of the affected individuals have a variety of congenital heart diseases. They can also have upper airway obstruction during sleep,due to hypotonia and the anatomy of the oropharynx and airway,which make these patients susceptible to obstructive sleep apnea (OSA). In our case, pulmonary hypertension was caused, successively,by congenital heart defects (a large patent ductus arteriosus and arch hypoplasia) and obstructive sleep apnea during early infancy. The congenital heart defects were surgically corrected, but persistent pulmonary hypertension was identified 2 months after the operation. This pulmonary hypertension was due to OSA, and it was relieved by nasal continuous positive airway pressure. This case is the first report of pulmonary hypertension from OSA in a young infant with RTS.