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Case Report
Cardiology
Idiopathic midaortic syndrome with malignant hypertension in 3-year-old boy
Kyung Jin Ahn, Ja Kyoung Yoon, Gi Beom Kim, Bo Sang Kwon, Eun Jung Bae, Chung Il Noh
Clin Exp Pediatr. 2016;59(Suppl 1):S84-S87.   Published online November 30, 2016

Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the branches show stenosis. MAS is associated with diminished pulses in the lower extremities compared with the upper extremities, severe hypertension with higher blood pressure in the upper rather than lower extremities, and an abdominal bruit....

Original Article
Cardiology
Infantile Marfan syndrome in a Korean tertiary referral center
Yeon Jeong Seo, Ko-Eun Lee, Gi Beom Kim, Bo Sang Kwon, Eun Jung Bae, Chung Il Noh
Clin Exp Pediatr. 2016;59(2):59-64.   Published online February 29, 2016
Purpose

Infantile Marfan syndrome (MFS) is a rare congenital inheritable connective tissue disorder with poor prognosis. This study aimed to evaluate the cardiovascular manifestations and overall prognosis of infantile MFS diagnosed in a tertiary referral center in Korea.

Methods

Eight patients diagnosed with infantile MFS between 2004 and 2014 were retrospectively evaluated.

Results

Their median age at the time of diagnosis was 2.5 months (range,...

Alagille syndrome and a JAG1 mutation: 41 cases of experience at a single center
Kyung Jin Ahn, Ja Kyoung Yoon, Gi Beom Kim, Bo Sang Kwon, Jung Min Go, Jin Su Moon, Eun Jung Bae, Chung Il Noh
Clin Exp Pediatr. 2015;58(10):392-397.   Published online October 21, 2015
Purpose

Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis.

Methods

We conducted a retrospective study of 41 patients with Alagille syndrome...

The strong association of left-side heart anomalies with Kabuki syndrome
Ja Kyoung Yoon, Kyung Jin Ahn, Bo Sang Kwon, Gi Beom Kim, Eun Jung Bae, Chung Il Noh, Jung Min Ko
Clin Exp Pediatr. 2015;58(7):256-262.   Published online July 22, 2015
Purpose

Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center.

Methods

A retrospective analysis was conducted for a total of 13 patients with Kabuki...

The relationships of body mass index, waist-to-height ratio, and body fat percentage with blood pressure and its hemodynamic determinants in Korean adolescents: a school-based study
Na Young Kim, Young Mi Hong, Jo Won Jung, Nam Su Kim, Chung Il Noh, Young-Hwan Song
Clin Exp Pediatr. 2013;56(12):526-533.   Published online December 20, 2013
Purpose

Obesity is an important risk factor for hypertension in adolescents. We investigated the relationship of obesity-related indices (body mass index [BMI], waist-to-height ratio [WHR], and body fat percentage [%BF]) with blood pressure and the hemodynamic determinants of blood pressure in Korean adolescents.

Methods

In 2008, 565 adolescents, aged 12-16 years, were examined. The %BF of the participants was measured by bioelectrical impedance...

Implantable cardioverter defibrillator therapy in pediatric and congenital heart disease patients: a single tertiary center experience in Korea
Bo Kyung Jin, Ji Seok Bang, Eun Young Choi, Gi Beom Kim, Bo Sang Kwon, Eun Jung Bae, Chung Il Noh, Jung Yun Choi, Woong Han Kim
Clin Exp Pediatr. 2013;56(3):125-129.   Published online March 18, 2013
Purpose

The use of implantable cardioverter defibrillators (ICDs) to prevent sudden cardiac death is increasing in children and adolescents. This study investigated the use of ICDs in children with congenital heart disease.

Methods

This retrospective study was conducted on the clinical characteristics and effectiveness of ICD implantation at the department of pediatrics of a single tertiary center between 2007 and 2011.

Results

Fifteen patients underwent...

Case Report
Aortic valve replacement surgery for a case of infantile Takayasu arteritis
Hye Won Kwon, Yoon Jung Suh, Ji Seok Bang, Bo Sang Kwon, Gi Beom Kim, Eun Jung Bae, Woong Han Kim, Chung Il Noh
Clin Exp Pediatr. 2012;55(7):254-258.   Published online July 17, 2012

Takayasu arteritis is a chronic inflammatory disease of unknown etiology primarily affecting the aorta and its major branches and usually occurring in the second or third decade of life. Here, we report a case of Takayasu arteritis in a 10-month-old patient. The infant presented with signs of congestive heart failure and severe aortic regurgitation. Echocardiography and computed tomography angiography showed...

Original Article
Clinical characteristics of obese boys and girls in a high school: focused on abdominal fat indices, fatty liver and carotid intima-media thickness
Jung Eun Oh, Jiyoung Jung, Hae Soon Kim, Young Mi Hong, Jung Hyun Yoo, Young Whan Song, Jo Won Jung, Nam Su Kim, Chung Il Noh
Clin Exp Pediatr. 2011;54(7):292-297.   Published online July 31, 2011
Purpose

Our study aimed to evaluated sex differences in clinical features of obese high school students.

Methods

One hundred three obese high school students (body mass index [BMI]≥85th percentile) and 51 control students (BMI<85th percentile) were enrolled in this study. Anthropometric measurements were performed. Fasting serum glucose, insulin, aspartate aminotransferase, alanine aminotransferase, total cholesterol, triglyceride, low-density lipoprotein cholesterol (LDL-C), high-density lipoprotein cholesterol (HDL-C),...

Metabolic syndrome in the overweight and obese adolescents and the impact of obesity on the cardiovascular system
Yong Mi Hong, Young Young Song, Hae Soon Kim, Hae Sook Park, Jung Hae Min, Jo Won Jung, Nam Soo Park, Chung Il Noh
Clin Exp Pediatr. 2009;52(10):1109-1118.   Published online October 15, 2009
Purpose : Metabolic syndrome (MS), characterized by obesity and insulin resistance, elicits risk factors such as hyperlipidemia, hypertension, and glucose intolerance with additive effects on atherosclerosis, leading to cardiovascular diseases. The purposes of this study were to evaluate the prevalence of MS among overweight and obese adolescents and to investigate the impact of obesity on the cardiovascular system. Methods : In...
Case Report
A case of adolescent Kawasaki disease with Epstein-Barr virus-associated infectious mononucleosis complicated by splenic infarction
Byeong Sam Choi, Bo Sang Kwon, Gi Beom Kim, Yoon Kyung Jeon, Jung-Eun Cheon, Eun Jung Bae, Chung Il Noh, Jung Yun Choi, Yong Soo Yun
Clin Exp Pediatr. 2009;52(9):1029-1034.   Published online September 15, 2009
Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology that affects children. There are few reports that describe the Epstein-Barr virus (EBV) as the possible infectious agent of KD. Here, we describe a case of KD in a 15- year-old boy complicated with giant coronary artery aneurysms, pericardial effusion, and splenic infarction. The clinical course of KD was...
Original Article
A study of the development of macrovascular complications and factors related to these complications in young adults with childhood/adolescence-onset type 1 diabetes mellitus
Min Jae Kang, Joo Hwa Kim, Hye Rim Chung, Young Ah Lee, Choong Ho Shin, Sei Won Yang, You Yeh Kim, Seon Mi Jin, Chung Il Noh
Clin Exp Pediatr. 2009;52(2):220-226.   Published online February 15, 2009
Purpose : Macrovascular complications are the main cause of mortality in type 1 diabetes mellitus (T1DM). The purpose of this study was to clarify the presence of early vascular changes and to assess the risk factors of macrovascular complications in young adults with T1DM diagnosed in childhood and adolescence. Methods : Seventy-two patients (23.9¡¾2.4 years) with T1DM diagnosed before 18 years...
Normal blood pressure values and percentile curves measured by oscillometric method in children under 6 years of age
Jin A Sohn, Hee Sook Lee, Kyoung Aha Lim, So Young Yoon, Jo Won Jung, Nam Su Kim, Chung Il Noh, Soon Young Lee, Young Mi Hong
Clin Exp Pediatr. 2008;51(9):998-1006.   Published online September 15, 2008
Purpose : Hypertension is defined as average systolic blood pressure and/or diastolic blood pressure that is ≥95th percentile for gender, age, and height on ≥three occasions. Knowing that blood pressure values increase in children as they grow older, the purposes of this study were to measure blood pressure by an oscillometric device and to determine normal values and percentile curves...
Review Article
Intensive management of acute right heart failure
Gi Beom Kim, Chung Il Noh
Clin Exp Pediatr. 2007;50(11):1041-1048.   Published online November 15, 2007
Not a few patients in children and adolescents are suffering from right ventricular (RV) dysfunction resulting from various conditions such as chronic lung disease, left ventricular dysfunction, pulmonary hypertension, or congenital heart defect. The RV is different from the left ventricle in terms of ventricular morphology, myocardial contractile pattern and special vulnerability to the pressure overload. Right ventricular failure (RVF)...
Original Article
Congenital Heart Disease in Jeju : Postnatal Incidence and Clinical Features
Jung Ha Lee, Gyu Hong Shim, Kyung Sue Shin, Jung Yun Hong, Yeoun Woo Kim, Seong Ho Kim, Chung Il Noh
Clin Exp Pediatr. 2004;47(3):294-303.   Published online March 15, 2004
Purpose : This is a retrospective descriptive study to determine the incidence of congenital heart disease(CHD) in Jeju and to estimate the suitability of the Jeju population as the subject for a regional birth cohort study. Methods : All patients with CHD diagnosed by two dimensional echocardiography in Cheju National University Hospital and Halla General Hospital from January 1999 to...
Follow-up Study of Children with Anthracycline Cardiotoxicity
Hyok Joo Kwon, Young Hwan Song, Soo Jung Kang, Hyoung Jin Kang, Hyoung Soo Choi, Eun Jung Bae, Hee Young Shin, Chung Il Noh, Yong Soo Yun, Hyo Seop Ahn
Clin Exp Pediatr. 2003;46(3):242-249.   Published online March 15, 2003
Purpose : We studied the relationship between anthracycline cumulative dose and anthracycline cardiotoxicity in childhood cancer and followed up 40 children with anthracycline cardiotoxicity. Methods : A retrospective study was performed in 154 children who received anthracycline chemotherapy between January 1995 to December 2000. Cardiotoxicity was defined when the left ventricular fractional shortening(FS) was below 26%; it was divided into two...
Diagnostic Assessment of Pulmonary Atresia with Ventricular Septal Defect; Comparison of Echocardiogram with Cardiac Angiography
Jung Yun Choi, Jeong Jin Yu, Soo Jung Kang, Jae Seong Son, Young Mee Seo, Jin Young Song, Ho Sung Kim, Eun Jung Bae, Chung Il Noh, Yong Soo Yun
Clin Exp Pediatr. 2001;44(2):154-160.   Published online February 15, 2001
Purpose : Patients with pulmonary atresia with ventricular septal defects(PAVSD) have been a formidable surgical challenge. The source of pulmonary blood flow and vascular architecture are important in managing the surgical process. This study aimed to evaluate the usefulness of echocardiography in this process. Methods : This study was prospectively designed to define the role of echocardiography in PAVSD. Non-invasive evaluations...
Infective Endocarditis in Children : Review of 35 Cases over 11 Years(1987-1997)
Jeong Jin Yu, Young Ho Kwak, Jung-Youn Hong, He Sun Jung, Jin Young Song, Hoan Jong Lee, Chung Il Noh, Jung Yun Choi, Yong Soo Yun
Clin Exp Pediatr. 2000;43(4):526-534.   Published online April 15, 2000
Purpose : Infective endocarditis(IE) is a serious complication in children with structural heart disease. We reviewed 35 cases of IE to identify the recent changes in the pattern of preexisting heart diseases, the spectrum of causative organisms and prognosis. Methods : The clinical records of children diagnosed as IE at the Seoul National University Children`s Hospital from January 1987 through December...
Congenital Heart Anomalies in Patients with Clefts of the Lip and/or Palate
Jin Ho Yu, Jeong Jin Yu, June Huh, Chung Il Noh, Jung Yun Choi, Yong Soo Yu
Clin Exp Pediatr. 2000;43(4):520-525.   Published online April 15, 2000
Purpose : The prevalence of congenital heart anomalies is known to be higher in patients with clefts of the lip and/or palate(CL/P). The purpose of this study was to determine the prevalence and type of congenital heart anomalies in patients with CL/P. Methods : We investigated congenital heart anomalies in 756 patients presented with CL/P from January 1986 to December...
Effects of Cisapride on QTc Interval and QT Dispersion in Preterm Infants
Heui Seung Jo, Chang Won Choi, Yun Kyung Lee, Hee Seok Kim, Jun Dong Park, Jun Huh, Beyong Il Kim, Chung Il Noh, Jung-Hwan Choi
Clin Exp Pediatr. 2000;43(2):188-194.   Published online February 15, 2000
Purpose : Cisapride, a prokinetic agent, is widely used in preterm infants with feeding intolerance or gastroesophageal reflux. Although cisapride is regarded as a safe drug, increased QTc interval or ventricular arrhythmia has been reported in adults and neonates. So we prospectively examined the effects of cisapride on the QTc interval and QT dispersion in preterm infants. Methods : QTc interval...
The Relationship between Total Serum IgE, Allergen-Specific IgE, and Skin Prick Test in Children with Atopic Asthma
Myung Hyun Lee, Jin Hwa Cheong, Young Yull Koh, Chung Il Noh, Jung Yun Choi, Yong Soo Yun
Clin Exp Pediatr. 1999;42(3):403-411.   Published online March 15, 1999
Purpose : Skin prick test and determination of allergen-specific IgE antibodies in serum are methods commonly used to diagnose allergies. Several studies indicate that skin test and specific IgE have roughly the same diagnostic precision, although discrepancies exist. The objective of this study was to evaluate the influence of total serum IgE on the relation between skin prick test and...
Atrial Ectopic Tachycardia in Infant
Myung Ja Yoon, Chung Il Noh
Clin Exp Pediatr. 1998;41(5):646-653.   Published online May 15, 1998
Purpose : Atrial ectopic tachycardia(AET) in older children and adult is characterized by being chronic, incessant and intractable. However, the nature of infantile AET is not defined yet. The purpose of this study was to evaluate our experience of infantile AET. Methods : We retrospectively reviewed the medical records of 13 infants(mean onset age, 2.8±2.3 months) diagnosed at Seoul National University...
The Assessment of Pulmonary Parenchymal Damage in Congenital Heart Disease using Lung Perfusion Scan
Hong Ryang Gil, Jae Goo Lee, Kang Uk Lee, Eun Ho Yi, Myung Ja Yun, Chung Il Noh, Jung Yeon Choi, Yong Soo Yun
Clin Exp Pediatr. 1998;41(5):640-645.   Published online May 15, 1998
Purpose : In acyanotic congenital heart disease of children with left to right shunt, 99mTc- Macroaggregate albumin(MAA) was employed to determine the scintigraphic severity of pulmonary parenchymal damage. Methods : These data of lung perfusion scan using 99mTc-MAA were compared with hemodynamic values obtained from cardiac catheterization. Results : The mean Pulmonary arterial pressure(≥30mmHg), total pulmonary resistance(≥2 Wood unit)...
Medical Lecture Course
Syncope in Infants and Children
Chung Il Noh
Clin Exp Pediatr. 1998;41(5):577-584.   Published online May 15, 1998
Original Article
Congenital Heart Disease and Associated Extracardiac Anomalies in Autopsies
Soon Seong Park, Myeong Ja Yoon, Jeong Sun Kim, Jeong Wook Seo, Chung Il Noh, Jung Yun Choi, Yong Soo Yoon, Byung Il Kim, Joong Hwan Choi, Chong Ku Yoon
Clin Exp Pediatr. 1998;41(3):354-362.   Published online March 15, 1998
Purpose : In order to find out how frequently and what kind of extra-cardiac anomalies (CAs) is associated with congenital heart disease(CHD) in general and in each CAs, autopsies with congenital cardiovascular malformations were studied. Methods : Autopsy files of Seoul National University Children' s Hospital from 1991 to 1995 were reviewed to find cases of CHDs. Other extra-CAs were tabulated from the records...
Causes of Syncope in Children
Jae Young Lee, Chung Il Noh, Eun Jung Chun, Sun Sung Park, Myung Ja Yun, Hong Ryang Gil, Ho Sung Kim, Jung Yun Choi, Yong Su Yun
Clin Exp Pediatr. 1998;41(1):81-89.   Published online January 15, 1998
Purpose : Syncope is not rare and is caused by various conditions ranging from common physiologic derangements to life-threatening conditions. However, there are limited reports regarding syncope in children. We retrospectively analyzed our experiences of the syncope in children. Methods : Retrospective analysis of the medical records of patients with syncopal episodes between October 1985 and June 1996. Results : Sixty-eight patients(male; 36, female; 32,...
Interim Results of Single Stage Operation for Coarctation of Aorta with Large Ventricular Septal Defect in Infancy
Ho Sung Kim, Chung Il Noh, Yong Soo Yun, Jung Yun Choi, Jeong Ryul Lee, Yong Jin Kim, Joon Ryang Roh
Clin Exp Pediatr. 1997;40(9):1250-1257.   Published online September 15, 1997
Purpose : There are controversies in the operative methods of aorta with ventricular septal defect in infancy. Recent results of single stage operation for coarctation of aorta with intracardiac defects were regarded as comparable to staged operation. We evaluated our interim results after single stage operation for coarctation of aorta with large ventricular septal defect in infancy. Methods : Twenty six infants who had received...
Case Report
A Report of Familial Dilated Cardiomyopathy
Ee Kyung Kim, Jung Yun Choi, Chung Il Noh, Yong Soo Yun, Ho Sung Kim, Myung Hyun Lee, In Kyu Kim
Clin Exp Pediatr. 1997;40(6):857-861.   Published online June 15, 1997
Two families of dilated cardiomyopathy are reported. In the first family, two sons were affected. The elder child presented with congestive symptoms at the age of 5 months and was diagnosed to have dilated cardiomyopathy. The etiology of cardiomyopathy was thought to be idiopathic until his younger brother presented with near collapse at the age of 5 days and was diagnosed to have the...
Original Article
Intermediate Term Follow-up Results of Hypertrophic Cardiomyopathy in Children
Eun Jung Cheon, Chung Il Noh, Jae Young Lee, Sun Sung Park, Myung Ja Yun, Hong Ryang Kil, Ho Sung Kim, Jung Yun Choi, Yong Soo Yun
Clin Exp Pediatr. 1997;40(5):641-649.   Published online May 15, 1997
Purpose : Hypertrophic cardiomyopathy(HCMP) is a primary cardiac muscular disease with high risk of sudden cardiac death. This study is performed to understand the clinical features of children with HCMP. Methods : Retrospective analysis of the medical records of the patients diagnosed at Seoul National University Children's Hospital between October 1985 and June 1996 was done. Excluded the patients who had...
Case Report
Three Cases of Cardiac Arrythmias Associated with Intravenous Methylprednisolone Pulse Therapy
Jun Ho Lee, Jun Huh, Myung Dong Shin, Dong Kyu Jin, Il Soo Ha, Hae Il Cheong, Chung Il Noh, Yong Choi
Clin Exp Pediatr. 1997;40(3):393-407.   Published online March 15, 1997
Methylprednisolone(MP) is administered by means of intravenous pulse therapy especially in the patients with focal segmental glomerulosclerosis(FSGS). There have been reports of its complications in a few increasing frequency. We experienced three cases of cardiaccomplications during intravenous pulsetherapy.Therewere onecaseof2¡ÆA-V block(Mobitztype I) and two cases of bradycardia and hypotension. In the former,2¡ÆA-V block in the first case deveoloped 45 hours after...
Original Article
Experience of Stent Implantation in Branch Pulmonary Artery Stenosis of the Congenital Heart Diseas
Yong Soo Yun, Eun Jung Cheon, Ho Sung Kim, Chung Il Noh, Jung Yun Choi
Clin Exp Pediatr. 1997;40(3):343-351.   Published online March 15, 1997
Purpose : Branch pulmonary artery stenosis remains a clinically challenging lesion. Results of surgical angioplasty are rarely rewarding, and balloon angioplasty is not uniformly successful. So, endovascular stent has been applied recently to these lesions. But this new modality has not been full yevaluated. The aim of this study is to evaluate the short-term result of stent implantation in postoperative branch pulmonary artery stenosis. Methods :...
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