- Case Report
- Cardiology
- Idiopathic midaortic syndrome with malignant hypertension in 3-year-old boy
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Kyung Jin Ahn, Ja Kyoung Yoon, Gi Beom Kim, Bo Sang Kwon, Eun Jung Bae, Chung Il Noh
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Clin Exp Pediatr. 2016;59(Suppl 1):S84-S87. Published online November 30, 2016
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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.... |
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- Original Article
- Cardiology
- Infantile Marfan syndrome in a Korean tertiary referral center
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Yeon Jeong Seo, Ko-Eun Lee, Gi Beom Kim, Bo Sang Kwon, Eun Jung Bae, Chung Il Noh
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Clin Exp Pediatr. 2016;59(2):59-64. Published online February 29, 2016
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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. MethodsEight patients diagnosed with infantile MFS between 2004 and 2014 were retrospectively evaluated. ResultsTheir median age at the time of diagnosis was 2.5 months (range,... |
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- The strong association of left-side heart anomalies with Kabuki syndrome
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Ja Kyoung Yoon, Kyung Jin Ahn, Bo Sang Kwon, Gi Beom Kim, Eun Jung Bae, Chung Il Noh, Jung Min Ko
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Clin Exp Pediatr. 2015;58(7):256-262. Published online July 22, 2015
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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. MethodsA retrospective analysis was conducted for a total of 13 patients with Kabuki... |
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- Alagille syndrome and a JAG1 mutation: 41 cases of experience at a single center
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Kyung Jin Ahn, Ja Kyoung Yoon, Gi Beom Kim, Bo Sang Kwon, Jung Min Go, Jin Su Moon, Eun Jung Bae, Chung Il Noh
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Clin Exp Pediatr. 2015;58(10):392-397. Published online October 21, 2015
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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. MethodsWe conducted a retrospective study of 41 patients with Alagille syndrome... |
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- Implantable cardioverter defibrillator therapy in pediatric and congenital heart disease patients: a single tertiary center experience in Korea
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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
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Clin Exp Pediatr. 2013;56(3):125-129. Published online March 18, 2013
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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. MethodsThis 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. ResultsFifteen patients underwent... |
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- Case Report
- Aortic valve replacement surgery for a case of infantile Takayasu arteritis
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Hye Won Kwon, Yoon Jung Suh, Ji Seok Bang, Bo Sang Kwon, Gi Beom Kim, Eun Jung Bae, Woong Han Kim, Chung Il Noh
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Clin Exp Pediatr. 2012;55(7):254-258. Published online July 17, 2012
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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... |
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- A case of adolescent Kawasaki disease with Epstein-Barr virus-associated infectious mononucleosis complicated by
splenic infarction
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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
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Clin Exp Pediatr. 2009;52(9):1029-1034. Published online September 15, 2009
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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... |
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- Gastric neurofibroma in von Recklinghausen disease : a cause of upper gastrointestinal bleeding
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Bo Sang Kwon, Jeong Ok Shim, Jeong Kee Seo, Hye Ran Yang, Jae Sung Ko, Seong Eun Jung, Woo Sun Kim, Gyeong Hoon Kang
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Clin Exp Pediatr. 2006;49(2):203-207. Published online February 15, 2006
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Neurofibromatosis type 1 (von Recklinghausen disease, NF1) involves the central and peripheral nervous systems as well as the skin, bone, endocrine, gastrointestinal and vascular systems. The gastrointestinal neurofibroma associated with NF1 has been infrequently reported. We report our experience with a 15-year-old boy who had a gastric plexiform neurofibroma with upper gastrointestinal bleeding and underwent a tumorectomy because of massive... |
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