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Pediatric Emergency Room Presentation of Congenital Heart Disease
이윤식,백재석,권보상,김기범,배은정,노정일,최정윤,윤용수 대한심장학회 2010 Korean Circulation Journal Vol.40 No.1
Background and Objectives: Only a few studies have specifically investigated the reasons for emergency room (ER) visits in patients with congenital heart disease (CHD). The aim of this study was to identify the major reasons for ER presentation among patients with CHD that were acutely and seriously ill at a tertiary medical center in Korea. Subjects and Methods: All 368 admissions of patients with CHD via the ER from 2003 to 2008 were enrolled. We conducted a retrospective study with review of the medical records. Results: Eighty two patients were newly diagnosed as having CHD. Their major presentations were: symptoms of heart failure (41.5%), murmur (31.7%), and cyanosis (18.3%). There were 286 visits that were cases with known CHD. Their major presentations were respiratory tract infection (24.1%, 2.7±4.1 years of age), dysrhythmia (16.4%, 16.7±9.5 years), symptoms of heart failure (14.3%, 7.6±9.4 years), aggravated cyanosis (5.6%, 0.8±1.4 years), protein-losing enteropathy (4.9%), hemoptysis (4.5%), drug side effects (4.1%), and infective endocarditis (3.0%). There were significant correlations between the age distributions and major modes of presentation. Surgical treatments were required within 1 month in 38%, and 2.7% of all patients died during hospitalization. The patient group with respiratory infections and CHD showed the highest mortality (5.8%). Atrial flutter was the most frequent arrhythmia (70.2%)and 70% of these patients were post-Fontan surgery condition. The causes of heart failure in the patients with previous surgical repair were: pulmonary hypertension, myocardial dysfunction, valve regurgitation, and uncorrected lesions. Conclusion: Improved understanding of the common problems in the ER can help prepare clinicians to manage patients that present with CHD.
Childhood Brugada Syndrome in Two Korean Families
이윤식,백재숙,김소연,서상원,권보상,김기범,배은정,박성섭,노정일 대한심장학회 2010 Korean Circulation Journal Vol.40 No.3
Since the first descriptions of Brugada as a new clinical entity defined by sudden cardiac death in patients with typical electrocardiogram (ECG) patterns, Brugada syndrome (BS) has been increasingly diagnosed. This syndrome is known as a disease that is inherited via an autosomal dominant trait, and the SCN5A mutation has been found in 20-25% of BS patients. Because BS primarily manifests in adulthood, little information is available on BS during childhood. Although there have been several reports on adult BS in Korea, pediatric BS has not been reported. Herein, we report on childhood BS in two families. One infantile BS patient and his family had a novel SCN5A mutation (c.4035G>T, p.W1345C, heterozygote) in domain III of the sodium channel.
Long QT Syndrome: a Korean Single Center Study
이윤식,권보상,김기범,오세일,배은정,박성섭,노정일 대한의학회 2013 Journal of Korean medical science Vol.28 No.10
The long QT syndrome (LQTS) is a rare hereditary disorder in which affected individuals have a possibility of ventricular tachyarrhythmia and sudden cardiac death. We investigated 62 LQTS (QTc≥0.47 sec) and 19 family members whose genetic study revealed mutation of LQT gene. In the proband group, the modes of presentation were ECG abnormality (38.7%), aborted cardiac arrest (24.2%), and syncope or seizure (19.4%). Median age of initial symptom development was 10.5 yr. Genetic studies were performed in 61; and mutations were found in 40 cases (KCNQ1 in 19, KCNH2 in 10, SCN5A in 7, KCNJ2 in 3, and CACNA1C in 1). In the family group, the penetrance of LQT gene mutation was 57.9 %. QTc was longer as patients had the history of syncope (P=0.001), ventricular tachycardia (P=0.017) and aborted arrest (P=0.010). QTc longer than 0.508 sec could be a cut-off value for major cardiac events (sensitivity 0.806, specificity 0.600). Beta-blocker was frequently applied for treatment and had significant effects on reducing QTc (P=0.007). Implantable cardioverter defibrillators were applied in 6 patients. Congenital LQTS is a potentially lethal disease. It shows various genetic mutations with low penetrance in Korean patients.
이윤식,Lee, Yun-Sik 한국데이터베이스진흥원 2006 디지털콘텐츠 Vol.4 No.-
대용량 콘텐츠의 수요가 증가하면서 이를 전송해주는 CDN서비스에 대한 관심이 증폭되고 있다. 콘텐츠 공급자의 공급 망 서비스 대행에서 출발한 CND은 인터넷 서비스 영역이 확장됨에 따라 CDN영역도 자연스럽게 확대되고 있다.