A Case of Multiple Giant Coronary Aneurysms with Large Mural Thrombus due to Kawasaki Disease in a Young Infant

최은나,김정태,김유리아,유병원,최덕영,최재영,설준희,이승규,김동수,박영환,Choi, Eun Na,Kim, Jeoung Tae,Kim, Yuria,Yoo, Byung Won,Choi, Deok Young,Choi, Jae Young,Sul, Jun Hee,Lee, Sung Kye,Kim, Dong Soo,Park, Young Hwan The Korean Pediatric Society 2005 Clinical and Experimental Pediatrics (CEP) Vol.48 No.3

가와사끼병은 주로 소아연령에서 발생하는 급성 열성 혈관염으로 관상동맥의 확장이 치명적 합병증으로 나타날 수 있다. 저자들은 3개월된 영아에서 반복적인 정맥용 면역글로불린, 스테로이드, 경구용 methotrexate의 치료에도 불구하고 진행되는 관상동맥 병변으로 다발성 거대 관상류, 협착 및 관상동맥 내 혈전을 보이며 심전도상 심근 허혈 소견과 함께 심장 자기공명영상에서 좌심실의 확장과 전벽 및 중격의 심한 운동저하를 나타낸 증례를 보고한다. 환아는 보존적 요법에 반응하지 않는 관상동맥병변으로 내원 88일째 수술적으로 우측 거대관상동맥류를 절개하고 Gore-tex tube를 삽입하여 각각의 측부순환과 연결하였으며, 좌측 관상동맥 협착부위는 세로로 절개한 후 심장막 반(pericardial patch)을 삽입, 확장하였으나, 수술 후 심폐부전으로 사망하였다. Kawasaki disease is an acute systemic vasculitis of unknown origin. Giant coronary aneurysm is one of the most serious complications, although peripheral artery vasculitis can produce life-threatening events. Myocardial ischemia and infarction can be caused by coronary artery stenosis, aneurysm, and stagnation of blood flow in coronary arteries which triggers thromboembolism. Atypical presentation in young infants often interferes with prompt diagnosis and timely treatment, resulting in poor outcomes. We describe a 3-month-old infant with multiple giant coronary aneurysms with flow stagnation, stenosis and large mural thrombus due to Kawasaki disease. He presented with a prolonged course of severe coronary involvement in spite of all measures to reduce coronary complications. Finally, surgical intervention was tried because of the worsening coronary artery abnormalities. The patient died of acute cardiorespiratory failure shortly after weaning from cardiopulmonary bypass.

소아 IgA 신병증의 예후와 관련한 임상병리학적 고찰

권재훈,최은나,박지민,정현주,이재승,Kwon Jae-Hun,Choi Eun-Na,Park Jee-Min,Jeung Hyeun-Joo,Lee Jae-Seung 대한소아신장학회 2003 Childhood kidney diseases Vol.7 No.1

목적 : IgA 신병증으로 진단된 소아환자를 대상으로 질병의 예후와 관련된 연구는 부족한 상태이다. 이에 저자들은 조직생검을 통해 확진된 IgA 신병증 환아의 임상경과를 관찰함으로써 소아들에서 장기 예후에 영향을 줄 수 있는 인자들의 특성을 알아보고자 하였다. 대상 및 방법 : 1981년부터 2000년까지 연세의료원에서 신생검을 시행하여 병리 소견상 IgA 신병증으로 확진된 15세 이하의 57명 환아에서 후향적 방법으로 자료 분석을 시행하였다. 모든 환아들은 조직 생검 당시 혈뇨나 무증후성 단백뇨($<40\;mg/m^2/day$)를 보였으며 정상 신기능 및 혈압을 유지하였다. 진단 당시의 임상검사소견, 병리소견 등을 바탕으로 IgA 신병증의 합병증인 심한 단백뇨(${\ge}40\;mg/m^2/day$), 약물복용을 요하는 고혈압. 만성 신부전 등의 발생에 대해 관찰하였다. 결과 : 대상 환아들의 진단 시 평균 연령은 4세에서 15세까지 $9.5{\pm}2.8$세였으며 42명(74%)이 남자였다. 단독 육안적 혈뇨는 20명(35%), 단독 현미경적 혈뇨는 3명(5%)이며 단독 단백뇨는 15(26%)이었다. 육안적 혈뇨와 단백뇨를 같이 동반한 경우는 15명(26%)이며 현미경적 혈뇨와 단백뇨는 15명(26%)에서 보였다. $7.0{\pm}3.5$년의 평균추적 관찰 기간동안 전체 57명의 환아 중 단백뇨 및 혈뇨가 완전히 소실된 경우는 38명(67%)이었고 진단 당시처럼 혈뇨나 단백뇨가 지속되나 IgA 신병증의 부작용은 발생하지 않은 경우는 12명(21%)이었다. 반면, 단지 7명(12%)의 환아에게서 IgA 신병증의 합병증이 발생하였는데 이 중에서 신증후군 정도의 단백뇨는 4명(7%), 약물복용을 요하는 고혈압은 1명(2%)이었고 2명(4%)의 환아는 말기 신부전으로 이행하였다. 발병 연령을10세 전후로 나누었을 때(P<0.01)와 Lee와 Haas의 병리조직학적인 분류(P<0.05)는 IgA 신병증의 합병증 발생과 밀접한 연관을 보인 반면, 진단당시 증상이나 성별은 통계적 유의성을 보이지 않았다. 57명 중 7명의 환아에서 평균 $3.4{\pm}3.5$년후에 신조직검사를 재실시하였다. 이 중 3명의 환아는 처음 class와 같았으며, 1명의 환아는 class III에서 class IV로 진행된 반면, 3명의 환아는 class IV에서 각각 class I, class II 및 class III로 변하였다. 결론 : 본 연구를 통해 소아의 IgA 신병증은 더 이른 시기에 진단 받았을 때 합병증의 발생 및 궁극적인 신부전으로의 이행이 적게 나타났다. 또한 추적 신조직 검사상 조직의 변화소견을 볼 때, 소아 IgA 신병증이 성인에서의 것보다 더 좋은 예후를 보이는 근거가 될 수 있다. Purpose : This study was performed to determine the natural history of histologically confirmed IgA nephropathy in pediatric patients who presented with hematuria and proteinuria. Patients and Methods : We reviewed the clinical course of 57 patients diagnosed with IgA nephropathy at the age of 15 years or younger from 1981 to 2000. All patients presented with hematuria or minimal proteinuria($<40\;mg/m^2/day$) and had normal renal function and blood pressure at the time of renal biopsy. Based on the clinical and pathological findings at the time of diagnosis, we sought for complications of IgA nephropathy such as heavy proteinuria(${\ge}40\;mg/m^2/day$), hypertension, and chronic renal failure. Results : The mean age at presentation was $9.5{\pm}2.8$ years(4 to 15 years) and 42(74%) were male. Isolated gross hematuria was observed in 20 patients(35%), microscopic hematuria in 3(5%), minimal proteinuria in 4(7%), both gross hematuria and minimal proteinuria in 15(26%), and both microscopic hematuria and minimal proteinuria in 15(26%). During a median follow-up of $7.0{\pm}3.5$ years, 38(67%) had complete resolution of hematuria and proteinuria, 12(21%) had persistently abnormal urinalysis without development of adverse events. Only 7(12%) developed adverse events : 4(7%) developed severe proteinuria, 1(2%) became hypertensive, and 2(3%) developed Impaired renal function. By univariate analysis using the chisquare test, the age at presentation(>10 years)(P<0.01) and poor histological classes of the Lee or Haas classification at onset(P<0.05) were significantly correlated with adverse events, whereas sex and clinical signs at onset were less concordant. Conclusion : We can conclude that the prognosis of IgA nephropathy diagnosed in early childhood is better and a good correlation exists between the clinical manifestations of this disease and the histological classes.

I급 부정교합자에서 발치와 비발치를 통한 교정치료 전·후의 입술위치 변화

이화현(Hwa-Hyun Lee),최은나(Eun-Na Choi),조미숙(Mi-Suk Cho),김정술(Jung-Sool Kim) 한국구강보건과학회 2013 한국구강보건과학회지 Vol.1 No.1

This study targeted 36 people in case reports with non-extraction that was completed the orthodontic treatment with non-extraction in Class I malocclusion patients and 48 people in case reports with extraction that was completed the orthodontic treatment with premolar extraction treatment. It classified it again into adults and adolescents, measured Lateral Cephalogram before and after treatment, and then comparatively observed a change in Lip and perioral soft tissue according to the movement of anterior teeth. Hence, the following results were obtained. 1. As for IMPA, U1 to FH, E line, the upper and lower lips were reduced figure regardless of adolescents and adults. Thus, the aesthetic facial profile was indicated to have been improved. 2. A change in the upper and lower lips on E line in the non-extraction group was increased in adults and decreased in adolescents. Thus, the improvement in aesthetic facial profile was shown more in the adolescent group. 3. As for IMPA, U1 to FH, E line, the figure in the upper and lower lips was changed. Thus, the aesthetic facial profile was improved. However, significance in correlation was indicated only in the upper and lower lips. 4. In both the extraction group and the non-extraction group, the upper lips were indicated to have influence upon the movement in the lower lips.

방광요관역류와 신반흔

남희영,신준헌,이준호,최은나,박혜원,Nam, Hee-Young,Shin, Joon-Heon,Lee, Jun-Ho,Choi, Eun-Na,Park, Hye-Won 대한소아신장학회 2006 Childhood kidney diseases Vol.10 No.2

Purpose : Vesicoureteral reflux(VUR) is the major risk factor of urinary tract infection(UTI) in children and may result in serious complications such as renal scarring and chronic renal failure. The purpose of this study was to evaluate the relationship between VUR and renal scar formation, the usefulness and correlation of various imaging studies in reflux nephropathy, and the spontaneous resolution of VUR. Methods : We retrospectively reviewed 106 patients with VUR with no accompanying urogenital anomalies in the Department of Pediatrics, Bundang CHA Hospital during the period from Jan. 1996 to Mar. 2005. Ultrasonography and $^{99m}Tc$-dimercaptosuccinic acid(DMSA) scan were performed in the acute period of UTI. Voiding cystourethrography(VCUG) was performed 1 to 3 weeks after treatment with UTI. Follow-up DMSA scan was performed 4 to 6 months after treatment and a follow-up VCUG was performed every 12 months. Results : The mean age at detection of VUR was $13.8{\pm}22.2$ months and the male to female ratio was 2:1. The incidence of renal scarring showed a tendency of direct correlation between severity of VUR(P<0.001) and abnormal findings of renal ultrasonography(P<0.01). 63.2%(24 of 38 renal units) of renal parenchymal defects present in the first DMSA scan disappeared on follow-up DMSA scans. Follow-up DMSA scans detected renal scars in 7(14%) of 50 renal units with ultrasonographically normal kidneys. Meanwhile, ultrasonography did not show parenchymal defects in 7(36.8%) of 19 renal units where renal scarring was demonstrated on a follow-up DMSA scan. The spontaneous resolution rate of VUR was higher(75%) in cases with low grade(I to III) VUR(P<0.01). Conclusions : The presence and severity of VUR and abnormal findings of renal ultrasonography significantly correlated with renal scar formation. DMSA scan was useful in the diagnosis of renal defects. Meanwhile renal ultrasonography was an inadequate method for evaluating renal parenchymal damage. Therefore, follow-up DMSA scans should be performed to detect renal scars even in children with low-grade VUR and normal renal ultrasonography.

원저 : 체외 수정으로 임신된 조기 출산 쌍태아와 정상 임신된 조기 출산 쌍태아의 임상적 고찰

신준헌 ( Jun Hun Shin ),남희영 ( Hee Young Nam ),이초애 ( Cho Ae Lee ),최은나 ( Eun Na Choi ),이규형 ( Kyu Hyung Lee ) 대한주산의학회 2006 Perinatology Vol.17 No.4

목적 : 본 연구의 목적은 체외 수정시술로 임신된 조기 출산 쌍태아와 자연 임신된 조기 출산 쌍태아의 신생아 예후를 알아보고자 기획되었다. 방법 : 60쌍의 체외 수정시술로 임신된 산모(체외 수정군)와 34쌍의 자연 임신된 산모(자연 임신군)의 조기 출산 쌍태아의 신생아 예후를 후향적으로 분석하였다. 산모와 산과적 특성도 비교하였다. 모든 출생아는 재태 주령 35주 미만이었고 2000년 1월부터 2004년 12월 사이에 분당차병원에서 태어났으며 모두 신생아 집중치료실에 입원했던 환아였다. 통계학적 방법으로는 Student t-검정과 χ2 검정을 시행하였다. 결과 : 산과적 특성상, 산모의 연령과 초산부의 비율이 체외 수정군에서 유의하게 높게 나타났으나 다른 특성들은(조기 진통, 조기 양막 파수, 임신성 고혈압, 임신성 당뇨, 제왕 절개 분만) 두 군간에 비슷한 양상을 보였다. 신생아 예후로는, 재태 주령, 출생 체중, 재원 기간, Apgar 점수, 신생아 사망률과 질병 이환율을 분석하였는데 자연 임신군과 비교하여 체외 수정군에서 유의한 차이를 발견하지 못했다. 결론:본 연구에서 체외 수정군이 산모의 나이와 초산부 비율을 제외하고는 산과적 특성과 신생아 예후에서 정상 임신군과 유의한 차이를 보이지 않았다. Objective : The aim of this study was to evaluate and compare neonatal outcome of premature twins conceived by in vitro fertilization (IVF) to those of naturally conceived. Methods : We retrospectively analyzed neonatal outcome of sixty pairs of premature twins conceived by IVF (IVF twin group) and 34 pairs that naturally conceived (natural twin group). Maternal and obstetric characteristics were also compared. All were born before 35 weeks of gestation between January 2000 and December 2004 at Pochon-Cha Hospital and admitted to neonatal intensive care unit. Student t-test and χ2 test were used for statistical analysis. Results : For obstetrical characteristics, maternal age and rates of nulliparous were sibnificanty twin group. But, other parameters (preterm labor, premature rupture of membranes, pregnancy-induced hypertension, gestational diabetes mellitus) were similar between two groups. For neonatal outcome, gestational age, duration of hospital stay, Apgar score, neonatal mortality and morbidity were reviewed. All parameters were no significant differences in IVF twin group compare to natural twin group Conclusions : In our study, IVF twin group had no significant differences in obstetric characteristics (except for maternal age and parity) and neonatal outcome compare to natural twin group.