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백재석,배은정,이상윤,박성섭,김소연,정규남,노정일 대한의학회 2010 Journal of Korean medical science Vol.25 No.10
The Jervell and Lange-Nielsen syndrome (JLNS) is an autosomal recessive syndrome characterized by congenital deafness and cardiac phenotype (QT prolongation, ventricular arrhythmias, and sudden death). JLNS has been shown to occur due to homozygous mutation in KCNQ1 or KCNE1. There have been a few clinical case reports on JLNS in Korea;however, these were not confirmed by a genetic study. We identified compound heterozygous mutations in KCNQ1 in a 5-yr-old child with JLNS, who visited the hospital due to recurrent syncope and seizures and had congenital sensorineural deafness. His electrocardiogram revealed a markedly prolonged corrected QT interval with T wave alternans. The sequence analysis of the proband revealed the presence of novel compound heterozygous deletion/splicing error mutations (c.828–830 delCTC, p.S277del/c.921G>A,p.V307V). Each mutation in KCNQ1 was identified on the maternal and paternal side. With β-blocker therapy the patient has remained symptom-free for three and a half years.
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.
A Case of Pulmonary Thromboembolism Associated With Hypereosinophilia in a Child
안효순,백재석,권보상,김기범,배은정,노정일,최정윤,윤용수 대한심장학회 2009 Korean Circulation Journal Vol.39 No.9
Pulmonary thromboembolism is a very rare event in children, but the mortality rate is reported to be approximately 10%. The majority of children with thromboemboli have multiple risk factors, such as a catheter-related thrombosis, an infection, and a congenital prothrombotic disorder. Hypereosinophilia is very rarely associated with pulmonary emboli in adults; however, this condition has not been reported in children. We present a 12- year-old boy who had a pulmonary thromboembolism and deep vein thrombosis associated with hypereosinophilia and thrombocytopenia. The thromboembolism was managed with anticoagulant therapy and the hypereosinophilia resolved spontaneously. Pulmonary thromboembolism is a very rare event in children, but the mortality rate is reported to be approximately 10%. The majority of children with thromboemboli have multiple risk factors, such as a catheter-related thrombosis, an infection, and a congenital prothrombotic disorder. Hypereosinophilia is very rarely associated with pulmonary emboli in adults; however, this condition has not been reported in children. We present a 12- year-old boy who had a pulmonary thromboembolism and deep vein thrombosis associated with hypereosinophilia and thrombocytopenia. The thromboembolism was managed with anticoagulant therapy and the hypereosinophilia resolved spontaneously.
송미경,배은정,백재석,권보상,김기범,노정일,최정윤,박성섭 대한심장학회 2011 Korean Circulation Journal Vol.41 No.6
QT prolongation is a serious adverse drug effect, which is associated with an increased risk of Torsade de pointes and sudden death. Many drugs, including both cardiac and non-cardiac drugs, have been reported to cause prolongation of QT interval. Although meperidine has not been considered proarrhythmic, we present a unique case of a 16-year-old boy without an underlying cardiac disease, who developed polymorphic ventricular tachycardia, ventricular fibrillation and QT prolongation after an intravenous meperidine injection. He had no mutation in long QT syndrome genes (KCNQ1, KCNH2, and SCN5A), but single nucleotide polymorphisms were reported, including H558R in SCNA5A and K897T in KCNH2.
이한필,방지현,백재석,구현우,박정준,김영휘 대한흉부외과학회 2016 Journal of Chest Surgery (J Chest Surg) Vol.49 No.3
Double outlet right ventricle (DORV) and transposition of the great arteries (TGA) with ventricular septal defect (VSD) and pulmonary stenosis (PS) are complex heart diseases, the treatment of which remains a surgical challenge. The Rastelli procedure is still the most commonly performed treatment. Aortic root translocation including an arterial switch operation is advantageous anatomically since it has a lower possibility of conduit blockage and the left ventricle outflow tract remains straight. This study reports successful aortic root transpositions in two patients, one with DORV with VSD and PS and one with TGA with VSD and PS. Both patients were discharged without postoperative complications.
Valve Sparing Aortic Root Replacement in Children with Loeys-Dietz Syndrome
심형태,박정준,서동주,유정진,백재석,구현우 대한흉부외과학회 2015 Journal of Chest Surgery (J Chest Surg) Vol.48 No.4
Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder that is characterized by aggressive arterial and aortic disease, often involving the formation of aortic aneurysms. We describe the cases of two children with LDS who were diagnosed with aortic root aneurysms and successfully treated by valve-sparing aortic root replacement (VSRR) with a Valsalva graft. VSRR is a safe and suitable operation for children that avoids prosthetic valve replacement.
전현옥,유정진,강소연,서창덕,백재석,김영휘,고재곤 대한소아청소년과학회 2015 Clinical and Experimental Pediatrics (CEP) Vol.58 No.10
Purpose: In 2004, the American Heart Association (AHA) had published an algorithm for the diagnosis of incomplete Kawasaki disease (KD). The aim of the present study was to investigate characteristics of supplemental laboratory criteria in this algorithm. Methods: We retrospectively examined the medical records of 355 patients with KD who were treated with intravenous immunoglobulin (IVIG) during the acute phase of the disease. Laboratory data were obtained before the initial IVIG administration and up to 10 days after fever onset. In 106 patients, laboratory testing was performed more than twice. Results: The AHA supplemental laboratory criteria were fulfilled in 90 patients (25.4%), and the frequency of laboratory examination (odds ratio [OR], 1.981; 95% confidence interval [CI], 1.391–2.821; P<0.001) was a significant predictor of it. The fulfillment of AHA supplemental laboratory criteria was significantly associated with refractoriness to the initial IVIG administration (OR, 2.388; 95% CI, 1.182– 4.826; P=0.013) and dilatation of coronary arteries (OR, 2.776; 95% CI, 1.519–5.074; P=0.001). Conclusion: Repeated laboratory testing increased the rate of fulfillment of the AHA supplemental laboratory criteria in children with KD.