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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...
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