칼슘이온 감작이 포함된 Transgelin의 혈관 평활근 수축성 조절

제현곤,제현동,Je, Hyun-Gon,Je, Hyun-Dong 대한약학회 2009 약학회지 Vol.53 No.3

The present study was undertaken to determine whether transgelin participates in the regulation of vascular smooth muscle contraction and, if so, to investigate the mechanism. By PCR homology cloning, the cDNA sequence of ferret transgelin was determined and phosphorothioate antisense and random oligonucleotides were synthesized and introduced into strips of ferret aorta by a chemical loading procedure. Treatment of ferret aorta with transgelin antisense oligonucleotides resulted in a significant decrease in protein levels of transgelin to sham- or random sequence-loaded muscles, but no change in the protein levels of actin. Contraction in response to a phorbol ester was significantly decreased in antisense-treated muscles compared to sham- or random sequence-loaded controls. Neither basal intrinsic tone nor the contraction in response to phenylephrine was significantly affected by the antisense treatment. The data indicate that transgelin plays a significant role in the regulation of contraction and suggest that in a tonically active smooth muscle transgelin may function as a signalling protein to facilitate PKC or ERK-dependent signalling rather than thick filament regulation including $Ca^{2+}$ or calmodulin dependent regulation of myosin light chain kinase.

신생아 패혈증의 다양한 선별검사들의 진단적 가치

제현곤,정영미,정수진 대한소아청소년과학회 2006 Clinical and Experimental Pediatrics (CEP) Vol.49 No.11

Purpose : To evaluate various sepsis screening tests, individually and in combination, to formulate a guideline for the diagnosis of neonatal sepsis. Methods : The study was a retrospective cohort study. It took place at the neonatal intensive care unit of the Paediatric Department, Il Sin Christian Hospital, Busan, Korea, over a period of 68 months from 1st, April, 2001 to 31st, December, 2005. This study evaluated 100 neonates having clinical features of sepsis and 100 normal asymptomatic neonates and used screening tests including C-reactive protein (CRP), total leukocyte count (TLC), absolute neutrophil count (ANC), immature neutrophils to total neutrophil count ratio (I/T ratio), thrombocytopenia, degenerative changes in the neutrophils and gastric aspirate cytology (GAC) for the diagnosis of neontal sepsis. Results : The sensitivity of CRP and ANC was high. CRP had 86 percent sensitivity for group-A (proven sepsis) and 74 percent sensitivity for group-B (probable sepsis) and 94 percent specificity for group-A, B. ANC had sensitivity of 72 percent for group-A and 62 percent for group-B and 86 percent specificity for group-A, B. For group-A, sensitivity, specificity of GAC for polymorphs was 74 percent and 94 percent respectively. As for sensitivity, specificity of platelet count for group-A was 64 percent and 89 percent respecively. The sensitivity, specificity and predictive values (PV) of the individual tests and different test combinations were also calculated for group-A and B. Conclusion : For the detection of culture negative cases in neonatal sepsis, screening tests including CRP, TLC, ANC, thrombocytopenia, cytoplasmic vacuolization in the neutrophils and GAC for polymorphs have high sensitivity. A combination of three tests has higher sensitivity. 목 적 : 신생아 패혈증을 진단하기 위해 단독 그리고 복합적인 다양한 선별검사를 분석하기 위해 일신기독병원의 신생아집중치료실에서 2001년 4월 1일부터 2005년 12월 31일까지 68개월 동안 시행했다.방 법 : 패혈증의 임상증상이 있는 100명의 신생아와 증상이 없는 정상 신생아를 대상으로 연구를 시행했다. 신생아 패혈증의 진단을 위해 CRP, 총 백혈구 수, 총 호중구 수, 미숙호중구/총호중구 비(I/T비), 혈소판 수, 호중구의 변성, 다형 핵 백혈구에 대한 GAC를 사용했다.결 과 : CRP는 A군에서는 50례 중 40례에서(86%) 양성이었고 B군에서는 50례 중 37례에서(74%) 양성이었으며 특이도는 94%였다. 총 호중구 수는 민감도와 특이도가 각각 A군에서는 72%와 86%였고 B군에서는 62%와 86%로 단일 검사상 민감도가 두 번째로 높았다. A군에서 GAC와 혈소판 수에 대한 각각의 민감도는 74%와 64%였다. A군과 B군에 대해 검사를 개별적으로 시행했을 경우와 함께 시행했을 경우에 대한 민감도, 특이도, 그리고 예측도를 계산했다. 결 론 : CRP, 총 백혈구 수, 총 호중구 수, 혈소판 수, 호중구의 변성과 다형 핵 백혈구에 대한 GAC는 신생아 패혈증이 없는 배양 검사 상 음성인 경우를 알아내는데 높은 민감도를 나타냈다. 더욱이 3가지 검사를 함께 시행할 경우에 민감도가 증가했다.

신생아에서 첫 태변 배출과 초뇨 배출 시간 결정인자

이혜진,제현곤,손상희 대한소아청소년과학회 2006 Clinical and Experimental Pediatrics (CEP) Vol.49 No.5

Purpose : To evaluate the factors which contribute to the time of the first stool and the first urine passage. Methods : We retrospectively reviewed a chart of 1,221 infants ≥34 weeks of gestational age admitted to the normal newborn nursery of Il Sin Christian Hospital, Busan, from November 2004 to April 2005. We compared the time to first stool and urine according to maternal factors(maternal age, parity, mode of delivery, meconium-stained amniotic fluid, and maternal diabetes) and infant factors (gender, Apgar score, gestational age, type of feeding during first 24 hours, age at the first feeding, number of feeds during the first 12 hours and age at discharge). Results : In total, 95.3 percent of our infants had passed their first stool by 24 hours and 99.8 percent of them had a stool by 36 hours. A total of 95.8 percent of our infants had passed urine by 24 hours of age and 98.3 percent of them by 36 hours. Comparing preterm and term infants, the time to first urine is 6.5±5.8 hours and 12.1±6.6 hours, respectively(P=0.000). The time to first stool is 20.7±13.5 hours and 10.0±6.3 hours, respectively(P=0.000) Early-fed infants were significantly earlier in time to first urine(P=0.023) and first stool(P=0.012). There was no statistically significant relationship between the number of feeds in 0-12 hours, mode of delivery, Apgar score, parity, gender, type of feeding, maternal diabetes and the time of the first urine and first stool. Conclusion : Gestational age, birth weight and age at first feeding were significantly related to the time of the first urine and first stool passage. When there is delayed passage of the first urine and first stool, we should consider close observation of other associated symptoms and other factors previously mentioned, to avoid extensive evaluation and intervention. 목 적 : 신생아의 첫 태변 및 초뇨 배출 시간은 건강 상태의 양호한 정도를 나타내는 표지이다. 첫 태변 배출 시간과 초뇨 배출 시간에 영향을 미칠 수 있는 여러 인자들에 대해 조사하고 이를 비교 평가 분석함으로써 그 임상적 의의를 비교 관찰하였다.방 법 : 2004년 11월부터 2005년 4월까지 본원에서 분만된 재태 연령 34주 이상의 신생아 1,221명을 대상으로 하였다. 모체의 인구학적 변수(모체 연령, 분만력, 분만 방법, 양수의 태변 착색 여부, 그리고 모체 당뇨 여부)와 영아의 변수(성별, Apgar 점수, 재태 연령, 출생 체중, 첫 24시간 동안의 수유 유형, 첫 수유 시 연령, 출생 후 12시간까지 수유 횟수, 퇴원 시 연령, 첫 번째 배뇨 시 연령, 첫 번째 태변 배출 시 연령)에 따른 태변 배출 및 초뇨 배출 시간을 비교 분석하였다.결 과 : 본 연구에서, 58.8%가 12시간 이전에, 95.3%가 24시간 이전에, 99.8%가 36시간 이전에 초뇨가 배출되었다. 그리고 68.3%가 12시간 이전에, 95.8%가 24시간 이전에, 98.3%가 36시간 이전에 태변이 배출되었다. 미숙아와 만삭아를 비교하였을 때 초뇨 배출시 연령은 각각 6.5±5.8시간, 12.1±6.6시간이었고(P=0.000), 첫 태변 배출시 연령은 각각 20.7±13.5시간, 10.0±6.3시간(P=0.000)으로 미숙아의 경우에 초뇨가 더 빨리 배출되고 태변이 더 늦게 배출되는 경향을 나타냈다. 수유를 빨리 시작한 경우에 초뇨나 첫 태변 배출 시간이 통계학적으로 유의하게 더 빨랐다. 출생 후 첫 12시간 동안의 수유 횟수는 초뇨 배출 시간과는 큰 상관이 없었다(P=0.778). 수유 횟수가 많을수록 24시간 이내에 첫 태변을 배출한 영아가 더 많았으며 이는 통계학적으로 유의하였다(P=0.000). 분만 방법(질식 분만, 수술 분만), Apgar 점수, 출산력, 성별, 수유 유형, 모체 당뇨 여부 등은 초뇨 배출 및 첫 태변 배출시 연령과는 통계학적으로 유의성은 없었다.

만삭아에서의 괴사성 장염의 위험인자와 임상증상

정영미,제현곤,손상희 대한소아청소년과학회 2006 Clinical and Experimental Pediatrics (CEP) Vol.49 No.5

Department of Pediatrics, Il Sin Christian Hospital, Busan, Korea Purpose : The purpose of this study was to determine those factors which could contribute to the development of necrotizing enterocolitis(NEC) in fullterm. Methods : We retrospectively reviewed the medical record of 20 full-terms with NEC(≥modified Bell's staging criteria IIa) who were admitted to the Neonatal Intensive Care Unit of Il Sin Christian hospital from January 1998 through July 2005, and for each case, the next 2 healthy newborns were matched as controls. Results : Mean gestational age and birth weight in the fullterm with NEC group was 38.42 weeks and 2,915 g; in the healthy fullterm without NEC group, it was 38.61 weeks and 3,148 g. When compared with the control group, NEC infants had a significantly higher frequency of chorioamnionitis, protracted diarrhea. As for Apgar score at 1 min <7, respiratory problem, congenital heart disease. there were no differences in frequency of preeclampsia, maternal diabetes, maternal drug abuse, meconium-stained amniotic fluid, polycythemia or exchange transfusion. Conclusion : Most of these full term infants have a predisposing factor before developing NEC. Our study suggested that NEC in fullterm infants was significantly associated with protracted diarrhea, and congenital heart disease. 목 적 : 본 연구의 목적은 만삭아에서 신생아 괴사성 장염의 위험인자를 알고자 함이다.방 법 : 1998년 1월 1일부터 2005년 8월 1일까지 본원 소아과 신생아실에 입원한 환아 중 신생아 괴사성 장염으로 진단된 20례와 각각의 경우에 대해 짝짓기 방식으로 선정된 건강한 만삭아 40례를 대상으로 병력지 검토를 통하여 후향적으로 조사되어졌다.결 과 : 괴사성 장염군의 평균 재태연령과 출생 체중은 38.42주와 2,915 g이었고 대조군은 38.61주와 3,148 g이었다. 대조군과 비교 하였을 때 신생아 괴사성 장염군 환아들에서 모체의 융모 양막염, 지연된 설사, 1분 아프가 점수 <7, 호흡기 문제, 선천성 심질환의 빈도가 유의하게 높았다(유의수준 <0.05). 반면 전자간증, 모체의 당뇨, 모체의 약물 남용, 태변 착색된 양수, 다혈구증, 교환 수혈의 빈도에는 유의한 차이가 없었다.

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.