• Title/Summary/Keyword: 선천성 심질환

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Non-invasive Fdlow-up of Pulmonary artey by EBT Other Palliatrve Shunt Operatin (청색증형 선천성 심질환아의 고식적 수술 이후 EBT에 의한 폐혈관계이 비침습적 추적 검사)

  • 김민정;박영환;홍유선;이종균;최규옥;조범구
    • Journal of Chest Surgery
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    • v.33 no.1
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    • pp.7-19
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    • 2000
  • Background :To assess the accuracy of Electron-Beam Tomography(EBT) in following evaluation of the pulmonary vascular system after a shunt operation in the cyanotic con-genital heart disease with pulmonary stenosis or pulmonary atresia. Material and Method : Sixteen patients(M:F=11:5) who received Blalock-Taussig(n=8) bidirectional cavo-pulmonary shunt(n=10) and unifocalization (n=2) were ncluded in the study. We evaluated the patency of the shunt the morphology of intrapericardial and hilar pulmonary arteries(PA) peripheral pulmonary vascularity by background lung attenuation and the abundance of arterial & venous collateral. Angiography(n=12) and echocardiography(n=20) were used as the gold standard for the comparison of EBT results. Result: EBT was consistent with angiogram/ echo in 100% of the evaluation for the patency of the shunt and in 12(by angiogram 100%) and 19(by echo 95%) for the detection the hypoplasia stenosis or interruption of central PA In measuring of PA EBT and angiogram corrlated(r=0.91) better than EBT-echo(r=0.88) or echo-angiogram(r=0.72) Abundant systemic arterial collateral were noted in 4 and venous collateral in 3 cases. In evaluating the peripheral pulmonary vascularity the homogenous and normal-ranged lung attenuation(m=6) decreased but homo-genous attenuation(n=1) segment-by-sgment heterogeneous attenuation(n=3) homogenous but asymmetrical attenuation(n=3) segment-by-segment heterogeneous attenuation(n=3) homogenous but asymmetrical attenuation(n=3) and venous congestion(n=2) were observed nd 12 of them were compatible with the blood flow pattern revealed by cardiac catheterization. Conclusion: EBT was accurate in the integrated evaluation of the pulmonary vascular system after the shunt including the patency of the shunt operaion the morphology and dimension of the central and hilar PAs and the loco-regional pulmonary flow in the lung parenchyma. It suggests the useful information about the need of secondary shunt operation the proper timing time for total repair and the need of interventional procedure prior to total repair.

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Standard Index Development for Comparing the Glomerular Number (사구체 수의 증감을 비교할 수 잇는 기준 개발)

  • Kim, Yong-Jin;Lee, Chi-Eun;Lee, Kook-Joo;Choi, Young-Ho;Hong, Jung-Suk
    • Journal of Yeungnam Medical Science
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    • v.18 no.1
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    • pp.85-93
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    • 2001
  • Background: The number of glomerulus has been considered one of the etiologic factors especially for focal segmental glomerulosclerosis. However, glomeruli are too many to calculate them correctly. Although the fractionator method has became convinced, in which they used selected sections, not whole kidney sections, with same intervals, it is also very hard to get good results. Because it is still very time-consuming and laborous work which leads to make big observers' biases. Methods and Materials: We designed the index for glomerular number to estimate the tendency of increase or decrease of the number of it in different kidneys and which was evaluated by other conventional methods including fractionator method. Index was based upon the theory by Nyengaard; "the number of glomerulus correlates with the weight of kidney, which is positively correlated with body weight". Calculating formula is the number of glomeruli/surface areas of cortices, which contain calculated glomeruli multiplies by kidney weight/body weight. Results: We applied this index to kidneys of FGS/Kist mouse and those of RFM/Nga mouse. The former is spontaneous glomerulosclerosis model with heavy protein uria and renal failure and the latter is the mother side of FGS/Kist mouse but has no glomerular disease or protein uria. The number of glomerulus of FGS/Kist mouse was decreased by 30% to those of RFM/Nga mouse. Conclusion: This index was useful and reliable for estimating the relative glomerular number between two groups.

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Clinical Features of Brain Abscesses in Neonates and Children: A Single Center Experience from 1997 to 2006 (단일기관에서 조사한 소아 뇌농양의 임상양상(1997-2006))

  • Lee, Teak Jin;Chu, Jin-Kyong;Kim, Ki Hwan;Kim, Khi Joo;Kim, Dong Soo
    • Pediatric Infection and Vaccine
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    • v.15 no.1
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    • pp.30-35
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    • 2008
  • Purpose : We evaluated clinical presentations of brain abscesses, including predisposing factors, causative organisms, and mortality rate in neonates and children. Methods : We retrospectively reviewed the medical charts of neonates and children with brain abscesses treated at Severance Hospital from January 1997 through December 2006. Results : Among 27 neonates and children with brain abscesses, overall mortality was 22 % and 38% of survivors developed neurologic sequelae. The mortality rate was 38% in 8 infants with brain abscesses. The most common location of brain abscesses were frontal and parietal lobes, followed by temporal lobe. There were 7 cases with multiple brain abscesses. Streptococci (33%), Staphylococci (27%), and Gram-negative enterics (20%) were commonly isolated. The common predisposing conditions were neurosurgical procedure (30 %), cyanotic congenital heart disease (15%), and sinusitis/otitis (7%). Fever (74%), headache (37%), nausea/vomiting (33%), and altered mental status (33%) occurred commonly. Compared with children older than 1 year of age, infants were associated with multiple brain abscesses (63%, P=0.011) and high rates of death or neurologic sequelae (88%, P= 0.033). Conclusion : We should have a high index of suspicion in order to recognize the condition as early as possible, especially in infancy with brain abscesses who presents vague or nonspecific symptoms and signs.

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Neurofibroma of the Larynx (후두의 신경섬유종)

  • 김종선;윤태현;노관택
    • Proceedings of the KOR-BRONCHOESO Conference
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    • 1979.05a
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    • pp.9.5-9
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    • 1979
  • Somewhat less than 100 cases of neurogenic tumors of the larynx are reported in the literature. This is slowly growing tumor and is mostly schwannomas and neurofibromas, and could affect any age group. Since the first description of Von Recklinghausen's disease in 1882, about 20 cases of laryngeal involvement have been reported in the world literature. In this paper two cases of laryngeal neurofibroma will be presented: one congenital laryngeal neurofibroma in a 2-month-old boy, and one large laryngeal neurofibroma in Von Recklinghausen's disease in a 28 year old woman. The importance of conservative surgical procedure is discussed with. case presentation and literature review.

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Impact of fetal diagnosis of congenital heart disease on parents (선천성 심질환의 산전 진단이 보호자의 임신 유지 결정에 미치는 영향)

  • Choi, Eun Young;Lee, Chang Hoon;Yoon, Myung Ja;Han, Eun Sook;Hong, Joon Suk;Jung, Yun Sook;Choi, Jung Yun
    • Clinical and Experimental Pediatrics
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    • v.49 no.10
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    • pp.1073-1078
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    • 2006
  • Purpose : This study was performed to assess how a fetal diagnosis of congenital heart disease affects parents, as regards pregnancy management and care of infants after birth. Methods : Database search to find out abnormal fetal echocardiography performed at Seoul National University Children's Hospital from July 1988 to June 2003 revealed 370 examinations. After excluding both arrhythmias without structural cardiac disease and multiple pregnancies, 299 pregnancies remained and this data formed the basis of this analysis. We retrospectively reviewed the medical records with special attention to pregnancy outcomes and also tried to find out factors influencing parental decisions on whether to continue or terminate pregnancy. Results : In this study, the mean gestation age at diagnosis was $28{\pm}6.0weeks$. The mean age of mothers was $30{\pm}3.9$ years old. Younger gestational ages at diagnosis(P=0.000), more severe grades of fetal heart disease(P=0.002) and younger mothers(P=0.014) correlated with terminations of pregnanies. But the grades of fetal status, the grades of associated anomaly, whether in-vitro-fertilization was carried out or not and numbers of previous children were not significant. Conclusion : This study found that the earlier gestational ages at diagnosis, younger maternal age and higher grades of fetal heart disease tended to lead parent to select abortions. Fetal echocardiographies were performed too late. Moreover Koreans have a biased view that malformation is a something incurable and a tragedy not only to oneself, but also to a family. So parents select terminations of pregnancy, even in curable cases. This is very unethical.

Sevoflurane Insufflation Sedation for the Dental Treatment of a Patient with Pulmonary Arterial Hypertension : A Case Report (폐동맥 고혈압 환자의 치과치료에서 세보플루란 흡입 진정의 사용 : 증례보고)

  • Chi, Seongin;Kim, Seungoh
    • Journal of the korean academy of Pediatric Dentistry
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    • v.42 no.1
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    • pp.75-79
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    • 2015
  • Pulmonary arterial hypertension (PAH) is a common complication of Congenital heart defects (CHD) with left-to-right shunts, and PAH with increased pulmonary vascular resistance (PVR) is associated with considerable morbidity and mortality. General anesthesia (GA) can be life-threatening in patients with PAH, because the positive pressure ventilation during GA increases pulmonary arterial pressure and decreases pulmonary blood flow. This may also lead to hypoxia. Therefore, spontaneous ventilation may be safer than positive pressure ventilation in patients with PAH. A five-year-old male child, weighing 11 kg, with medical history showing a total correction of Tetralogy of Fallot (TOF) in 2009 and ongoing treatment with hypertension (HTN) medicine since 2007, visited the Dankook University Dental Hospital. He had multiple dental caries, and the treatment was completed under sevoflurane insufflation sedation via nasal cannula. The patient remained sedated throughout the operation while maintaining normal vital signs and spontaneous respiration. In conclusion, sevoflurane insufflation sedation may be a safer alternative to GA for the dental treatment of patients with PAH.

Surgical Treatment of Ebstein Anomaly (Ebstein 기형의 외과적 치험)

  • 이종호;김병렬
    • Journal of Chest Surgery
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    • v.32 no.1
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    • pp.5-9
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    • 1999
  • Background: Ebstein anomaly is a rare congenital disease distinguished by its unique deformity in tricuspid valve and right ventricle & atrium. In its surgical treatment , tricuspid valve reconstruction and valve replacement are well known method, but various surgical methods were suggested. Material and Method : From January 1984 to December 1995, 8 patients with Ebstein anomaly underwent surgical correction. Age and sex distribution, clinical symtoms, radiologic findings, preoperative studies, operative findings, operative methods and its results were analyzed. Result: The sex ratio was 5 to 3(male : female). Patients' ages were averaged 17.6(2-28) years. In all cases, it showed typical deformities of the tricuspid valve. Associated anomalies were permenant foramen ovale, atrial septum defect, pulmonary stenosis. Surgical procedures included tricuspid valve replacement(n=4) and tricuspid valve reconstuction(n=4). Two cases of sinus tachycardia and complete AV block occured postoperatively. There were two hospital death and no late death. All survivors are in NYHA class I or II with median follow up of 64.8 months. 2-D echocardiogram disclosed improvement tricuspid regurgitation during the follow up period. Conclusion: Even though operative method of Ebstein anomaly should be decided according to each anatomical characteristics, we recommended that tricuspid valvuloplasty and plication can be one of the good methods method in the selective cases.

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Determination of Suitable Antecedent Rainfall Days for Curve Number Estimation (CN값 산정을 위한 적정 선행강우일수의 결정)

  • Lee Myoung Woo;Choi Seoung An;Kim Hung Soo;Shim Myung Pil
    • Proceedings of the Korea Water Resources Association Conference
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    • 2005.05b
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    • pp.553-557
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    • 2005
  • 유역의 강우-유출 분석에서 유효우량의 산정은 매우 중요하다고 할 수 있다. 이러한 유효우량의 산정에서 대상호우에 대한 유출량 자료가 있는 경우는 침투지수법(infiltration index method)중 하나인 $\Phi$-지표법이나 W-지표법을 사용하여 그 양을 산정할 수 있다. 그러나 대상호우에 대한 유출량 자료가 없는 경우는 침투지수법을 이용하여 유효우량을 산정 할 수 없으며, 이러한 경우 유역의 토양 특성과 식생피복 상태에 대한 자료만으로 총우량으로부터 유효우량을 산정할 수 있는 NRCS(Natural Resources Conservation Service)의 유효우량 산정방법이 널리 사용되고 있다. NRCS유효우량 산정 방법은 선행토양함수조건(antecedent moisture condition, AMC)을 이 용하여 유출곡선지수(runoff curve number, CN)를 결정하는데, 이때 AMC의 산정을 위해 선행5일강우량(total 5-day antecedent rainfall)을 그 기준으로 하고 있으나, 이는 미국의 유역을 대상으로 하여 얻어진 결과이므로 이를 국내 유역에 검증 없이 적용하는 데에는 문제가 있을 것으로 예상되었다. 따라서 본 연구에서는 HEC-HMS모형을 이용하여 선행강우일수을 변화시켜 가면서 실제 유역의 강우-유출에 적용하여 본 뒤 강우-유출을 가장 잘 모의하는 선행강우일수을 결정하였다. 이를 위해, IHP 대상유역인 보청천의 탄부소유역에 대하여 AMC의 산정을 위한 선행강우량을 1일부터 7일까지 변화시키며 적용한 결과 탄부소유역에서는 선행2일강우량이 가장 적합한 결과를 주는 것으로 나타났다. 국내 유역에서 NRCS유효유량 산정방법을 통한 강우-유출모의시 보다 정확한 값을 산정하기 위해서는 대상유역에 적합한 선행 강우일수의 결정이 중요하리라 판단된다.인 분석을 수행하고, 배수갑문 개방에 의한 수질개선효과를 최대화하기 위한 환경관리 방안 제시에 중점을 두어 수행하였다.ncy), 환경성(environmental feasibility) 등을 정성적으로(qualitatively) 파악하여 실현가능한 대안을 선정하였다. 이렇게 선정된 대안들은 중유역별로 검토하여 효과가 있을 것으로 판단되는 대안들을 제시하는 예비타당성(Prefeasibility) 계획을 수립하였다. 이렇게 제시된 계획은 향후 과학적인 분석(세부평가방법)을 통해 대안을 평가하고 구체적인 타당성(feasibility) 계획을 수립하는데 토대가 될 것이다.{0.11R(mm)}(r^2=0.69)$로 나타났다. 이는 토양의 투수특성에 따라 강우량 증가에 비례하여 점증하는 침투수와 구분되는 현상이었다. 경사와 토양이 같은 조건에서 나지의 경우 역시 $Ro_{B10}(mm)=20.3e^{0.08R(mm)(r^2=0.84)$로 지수적으로 증가하는 경향을 나타내었다. 유거수량은 토성별로 양토를 1.0으로 기준할 때 사양토가 0.86으로 가장 작았고, 식양토 1.09, 식토 1.15로 평가되어 침투수에 비해 토성별 차이가 크게 나타났다. 이는 토성이 세립질일 수록 유거수의 저항이 작기 때문으로 생각된다. 경사에 따라서는 경사도가 증가할수록 증가하였으며 $10\% 경사일 때를 기준으로 $Ro(mm)=Ro_{10}{\times}0.797{\times}e^{-0.021s(\%)}$로 나타났다.천성 승모판 폐쇄 부전등을 초래하는 심각한 선천성 심질환이다. 그러나 진단 즉시 직접 좌관상동맥-대동맥 이식술로 수술적 교정을 해줌으로써 좋은 성적을 기대할 수 있음을 보여주었다.특히 교사들이 중요하게 인식하는 해방적 행동에 대한 목표를 강조하여 적용할 필요가 있음을 시사하고

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Clinical Considerations of the Surgical Closure of the PDA in the Premature Infants (미숙아 동맥관 개존증의 외과적 교정에 관한 임상적 고찰)

  • 김상익;박철현;현성열;김정철;권진형;박국양
    • Journal of Chest Surgery
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    • v.32 no.8
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    • pp.702-708
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    • 1999
  • Background: Surgical closure of the PDA in premature infants with complications or contraindications to indomethacin use, or recurrence of symptomatic PDA is a safe and effective procedure with low operative risk and minimal complications. Material and Method: From April 1996 to August 1998, 11 premature infants with body weight under 1.5 kg at operation underwent operation for a symptomatic PDA (male:5, female: 6). Associated dise ases were congenital heart disease(7), hyaline membrane disease(6), intraventricular hemor rhage(4), pneumonia(4), pneumothorax(3), hyperbilirubinemia(2), necrotizing enterocolitis(2), renal failure(1), epilepsy(1), and hydrocephalus(1). Surgical techniques are hemoclipping(8) and ligation(3). The size of PDA was 3~6 mm (5.0$\pm$1.2). Result: Systolic and diastolic blood pressure rised and heart rates decreased after PDA closure. ABGA improved postoperatively. There were no surgical complications. Six infants with improved ABGA data were weaned from mechanical ventilatory support. The follow-up durations after discharge were 3 month to 12 month. Five deaths were not related to operation. The causes of death were hyaline membrane disease(2), bronchopulmonary dysplasia with pneumonia(1), sepsis(1), and con gestive heart failure with respiratory distress syndrome(1). Conclusion: Early operative closure is the treatment of choice in most premature infants with a hemodynamically significant shunt(PDA), recurrence of symptomatic PDA, complications of Indomethacin, or contraindi cations to Indomethacin.

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Availability of Cardiac Troponin T as a Marker for Detecting Perioperative Myocardial Damage in Patients with Open Heart Surgery (개심술후 발생한 심근손상에서 진단 표지자로서의 Troponin T의 임상적 유용성)

  • 김태이;정태은;이동협;이정철;한승세
    • Journal of Chest Surgery
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    • v.31 no.1
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    • pp.20-27
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    • 1998
  • It is well known that troponin T(below TnT) is present in the myocardial cells and released during myocardial damage, so it`s very specific enzyme to myocardium. Availability of cardiac specific TnT in assessing perioperatively myocardial damage was evaluated from 34 open heart surgery patients. They consisted of 11 ischemic heart, 13 acquired valvular heart and 10 congenital heart cases. Patients were divided into two groups, group A(patients with myocardial damage) and group B(patients without myocardial damage), according to the symptom of chest pain suspecting angina and the ECG findings of ST segment and T wave changes which show myocardial ischemia and injury. Serum TnT levels were measured by enzyme immunoassay method preoperatively, immediately postoperatively, postoperative day 1, day 2, day 3, and day 7. We observed and analyzed the changes of serum TnT levels in two groups and compared the serum TnT levels with CK-MB levels measured at the same time. In group A, serum TnT levels showed 1.37$\pm$0.26$\mu$g/L, 3.16$\pm$0.66$\mu$g/L, 2.39$\pm$0.74$\mu$g/L, 2.49$\pm$0.76$\mu$g/L, and 1.23$\pm$0.60$\mu$g/L, immediate postoperatively, postoperatively day1, day2, day3, and day7, respectively. It was observed there were significant differences compared with those of group B(0.38$\pm$0.04$\mu$g/L, 0.34$\pm$0.05$\mu$g/L, 0.25$\pm$0.03$\mu$g/L, 0.24$\pm$0.04$\mu$g/L, and 0.11$\pm$0.03$\mu$g/L) during identical periods(P<0.01). Serum CK-MB level in group A significantly elevated to 145.04$\pm$35.08 IU/L on the postoperative day 1 compared to group B(31.28$\pm$5.87 IU/L, P<0.05), However, it stiffly decreased from day 2 and returned to preoperative level at day 3. When serum TnT level more than 1.0$\mu$g/L is thought to reflect myocardial damage, serum TnT had 100% of sensitivity and 87% of specificity in diagnosing the postoperative myocardial damage(p<0.01). I conclusion, serum TnT levels increased significantly at very early stage of myocardial damage and persisted much longer period than CK-MB. This suggests that serum TnT has more advantage and availability in assessing the perioperatively myocardial damage than any other tests.

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