- Original Article
- Genetics and Metabolism
- Clinical and molecular characterization of Korean children with infantile and late-onset Pompe disease: 10 years of experience with enzyme replacement therapy at a single center
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Min-Sun Kim, Ari Song, Minji Im, June Huh, I-Seok Kang, Jinyoung Song, Aram Yang, Jinsup Kim, Eun-Kyung Kwon, Eu-Jin Choi, Sun-Ju Han, Hyung-Doo Park, Sung Yoon Cho, Dong-Kyu Jin
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Clin Exp Pediatr. 2019;62(6):224-234. Published online October 4, 2018
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Purpose: Pompe disease (PD) is an autosomal recessive disorder caused by a deficiency of acid alphaglucosidase resulting from pathogenic GAA variants. This study describes the clinical features, genotypes, changes before and after enzyme replacement therapy (ERT), and long-term outcomes in patients with infantile-onset PD (IOPD) and late-onset PD (LOPD) at a tertiary medical center.
Methods: The medical records of 5 Korean... |
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- Cardiology
- Outcomes of transcatheter closure of ductus arteriosus in infants less than 6 months of age: a single-center experience
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Gwang-Jun Choi, Jinyoung Song, Yi-Seul Kim, Heirim Lee, June Huh, I-Seok Kang
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Clin Exp Pediatr. 2018;61(12):397-402. Published online September 19, 2018
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Purpose: Transcatheter device closure of patent ductus arteriosus (PDA) is challenging in early infancy. We evaluated PDA closure in infants less than 6 months old.
Methods: We performed a retrospective review of infants less than 6 months of age who underwent attempted transcatheter device closure in our institution since 2004. To compare clinical outcomes between age groups, infants aged 6–12 months... |
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- Case Report
- A rare type of single coronary artery with right coronary artery originating from the left circumflex artery in a child
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Jong Min Kim, Ok Jeong Lee, I-Seok Kang, June Huh, Jinyoung Song, Geena Kim
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Clin Exp Pediatr. 2015;58(1):37-40. Published online January 31, 2015
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The presence of a single coronary artery is a rare congenital anomaly; such patients often present with severe myocardial ischemia. We experienced the case of a 13-year-old girl with the right coronary artery originating from the left circumflex artery. She visited our Emergency Department owing to severe chest pain; her cardiac enzyme levels were elevated, but her initial electrocardiogram (ECG)... |
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- A pediatric case of Brugada syndrome diagnosed by fever-provoked ventricular tachycardia
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Geena Kim, Ye-Chan Kyung, I-Seok Kang, Jinyoung Song, June Huh, Young Keun On
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Clin Exp Pediatr. 2014;57(8):374-378. Published online August 25, 2014
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Brugada syndrome is a rare channelopathy associated with the SCN5A gene that causes fatal ventricular arrhythmias. This case of Brugada syndrome, in which ventricular tachycardia (VT) was provoked by high fever, is the first report in a Korean child. The boy had retinoblastoma of his left eye diagnosed at 16 months of age. After chemotherapy, he contracted a catheter-related infection... |
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- Original Article
- Transcatheter closure of small ductus arteriosus with amplatzer vascular plug
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Eun Hyun Cho, Jinyoung Song, I-Seok Kang, June Huh, Sang Yoon Lee, Eun Young Choi, Soo Jin Kim
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Clin Exp Pediatr. 2013;56(9):396-400. Published online September 30, 2013
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Purpose The purpose of this study was to share our experience of transcatheter closure of small patent ductus arteriosus (PDA) by using an Amplatzer vascular plug (AVP). MethodsWe reviewed the medical records of 20 patients who underwent transcatheter closure at Samsung Medical Center and Sejong General Hospital from January 2008 to August 2012. The size and shape of the PDAs were evaluated... |
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- The hybrid perventricular closure of apical muscular ventricular septal defect with Amplatzer duct occluder
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Soo Jin Kim, June Huh, Jin Young Song, Ji-Hyuk Yang, Tae-Gook Jun, I-Seok Kang
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Clin Exp Pediatr. 2013;56(4):176-181. Published online April 22, 2013
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Purpose Apical muscular ventricular septal defects (MVSDs), especially in small infants, can be difficult to manage using surgical and percutaneous closure. An intraoperative perventricular procedure is a good option for closing apical MVSDs in small children with or without associated cardiac anomalies. We evaluated the results of hybrid perventricular closure of apical MVSDs performed using an Amplatzer duct occluder (ADO). MethodsWe retrospectively... |
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- Surgical outcome of severe pulmonary arterial hypertension secondary to left-to-right shunt lesions
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Cha Gon Lee, Su In Jeong, June Huh, I-Seok Kang, Heung Jae Lee, Ji-Hyuk Yang, Tae Gook Jun
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Clin Exp Pediatr. 2010;53(2):195-202. Published online February 15, 2010
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Purpose : Despite recent advances in pulmonary hypertension management and surgery, appropriate guidelines remain to be developed for operability in congenital heart disease with pulmonary artery hypertension (PAH). Our aim was to evaluate clinical outcomes of patients with severe PAH who underwent surgical closure of left-to-right shunt lesions (LRSL) on the basis of pulmonary reactivity.
Methods : We retrospectively reviewed 21... |
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- Review Article
- Medical management of adults with congenital heart disease
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I-Seok Kang
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Clin Exp Pediatr. 2006;49(9):937-945. Published online September 15, 2006
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The population of adult patients with congenital heart disease(CHD) has increased dramatically because of improved survival of infants and children with CHD. There are still a substantial number of adults with unrepaired CHD. Many adult patients do not know the possible problems of their disease such as ventricular dysfunction, arrhythmia, and what they should pay attention to. They often consider... |
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- Original Article
- Relationship between depression and resilience in adolescents with congenital heart disease
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Ju Ryoung Moon, Yoen Yi Jung, June Huh, I-Seok Kang, Seung Woo Park, Ji-Hyuk Yang, Tae-Gook Jun, Myung Ja Kim, Heung Jae Lee
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Clin Exp Pediatr. 2006;49(5):523-528. Published online May 15, 2006
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Purpose : The purpose of this study was to investigate the relationship between depression and resilience in adolescents with congenital heart disease(CHD) and to identify the variables associated with depression.
Methods : The Resilience Scale(cronbach’s α=0.92), Children's Depression Inventory(cronbach’s α= 0.72) and Maternal Behavior Research Instrument(cronbach’s α=0.88) were applied and analyzed to assess depression and resilience among 231 adolescents after... |
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- Case Report
- Congenital Pulmonary Vein Stenosis Manifested by Severe Cyanosis in Infancy
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Jae Ju Cho, Woo Sung Park, I-Seok Kang, Tae-Gook Jun, Mi-Jin Jung
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Clin Exp Pediatr. 2004;47(10):1114-1118. Published online October 15, 2004
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Congenital pulmonary vein stenosis(CPVS) with anatomically normal connection, a rare anomaly, usually leads to progressive pulmonary hypertension, cardiac failure in infancy, and death if untreated. Most are combined with other anomalies, particularly left to right shunt lesions. Very often, the detection of CPVS is overlooked on the initial cardiac echocardiogram, because it may be mild in its severity initially, but... |
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- Original Article
- Follow-up in Adult after Total Repair of Tetralogy of Fallot
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Gi Young Jang, Sun Young Kim, Joo Ryung Moon, Joon Huh, I-Seok Kang, Seung Woo Park, Tae Gook Jun, Pyo Won Park, Heung Jae Lee
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Clin Exp Pediatr. 2003;46(7):661-667. Published online July 15, 2003
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Purpose : This study was performed to find the chief clinical problems associated with the ages of adult patients of tetralogy of Fallot(TOF) who had undergone total correction.
Methods : Of the 30 patients who were registered at the Grown-Up Congenital Heart Disease (GUCH) Clinic of Samsung Medical Center for TOF, a retrospective investigation was carried out on 28 patients who... |
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- Original Article
- The Clinical Study on Marfan Syndrome
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Jung Sim Kim, Dong Kyu Jin, See Hwan Ko, Jee Yeon Min, I-Seok Kang, Kye Won Jeon, Yung Lan Choi, Heung Jae Lee, Seung Woo Park, Won Ro Lee, Chong Suh Lee, Sei Yeul Oh, Chang Won Ki, Han Wook Yoo, In Sook Park, Jae Kon Ko
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Clin Exp Pediatr. 1998;41(10):1411-1416. Published online October 15, 1998
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Purpose : The aim of this study was to assess the involvement of several organs patients with Marfan syndrome in Korea. Also the clinical features in childhood patients with Marfan syndrome were assessed.
Methods : Thirty-eight cases of Marfan syndrome were enrolled in this study. Clinical evaluations of the musculoskeletal, cardiovascular and occular system were performed in all cases.
Results : The... |
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- Clinical Manifestations of Recurrent Abdominal Pain May Differ with Helicobacter pylori Infection in Children
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Nam Seon Beck, I-Seok Kang
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Clin Exp Pediatr. 1997;40(7):965-973. Published online July 15, 1997
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Purpose : The role of H. pylori in the pathogenesis of recurrent abdominal pain in
children is uncertain. In order to find a correlation between H. pylori infection and
recurrent abdominal pain in children, the present study was designed to compare the
clinical manifestations between recurrent abdominal pain children with and without H.
pylori infection and to investigate the effects of H. pylori eradication... |
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- Case Report
- Two Cases of Nitric Oxide Inhalation for Treatment of Severe Pulmonary Hypertension after Surgical Repair of Congenital Diaphragmatic Hernia
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Yun Sil Chang, I-Seok Kang, Won Soon Park, Suk Koo Lee, Hun Hahk Kim, Heung Jae Lee
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Clin Exp Pediatr. 1996;39(11):1611-1619. Published online November 15, 1996
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Nitric oxide, an endothelium-derived relaxing factor, is a selective pulmonary vasodilator. We
have built and settled down the delivery system of nitirc oxide gas inhalation for the first time
in Korea. Two newborn babies delvelped near fatal pulmonary hypertension after surgical repair
of a congenital diaphragmatic hernia. All conventional therapeutic measures failed. So we
decided to attempt to use of nitric oxide gas for... |
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- Original Article
- Right Ventricular Area, Dimension, and Volume Measured by Two-dimensional Echocardiography in Normal Children
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I-Seok Kang, Jung-Yun Choi
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Clin Exp Pediatr. 1994;37(10):1340-1349. Published online October 15, 1994
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The usefulness of two-dimensional echocardiography to measure the right ventricular volume was assessed in 58 normal children(mean age 5 years and 3 months). From the apical 4-chamber view(A4CV), the subcostal RV sagittal view(SCRVSV), the subcostal right anterior oblique view(SCRAOV), and the subcostal RV coronal view(SCRVCV), the dimensions and areas of the right ventricle were measured. The right ventricular volumes and... |
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