Article 56(3); Mar 2013
Review Articles
Right ventricular failure in congenital heart disease
Young Kuk Cho, Jae Sook Ma
Clin Exp Pediatr. 2013;56(3):101-106.   Published online March 18, 2013

Despite developments in surgical techniques and other interventions, right ventricular (RV) failure remains an important clinical problem in several congenital heart diseases (CHD). RV function is one of the most important predictors of mortality and morbidity in patients with CHD. RV failure is a progressive disorder that begins with myocardial injury or stress, neurohormonal activation, cytokine activation, altered gene expression,...

Oxidative stress and the antioxidant enzyme system in the developing brain
So-Yeon Shim, Han-Suk Kim
Clin Exp Pediatr. 2013;56(3):107-111.   Published online March 18, 2013

Preterm infants are vulnerable to the oxidative stress due to the production of large amounts of free radicals, antioxidant system insufficiency, and immature oligodendroglial cells. Reactive oxygen species (ROS) play a pivotal role in the development of periventricular leukomalacia. The three most common ROS are superoxide (O2•-), hydroxyl radical (OH), and hydrogen peroxide (H2O2). Under normal physiological conditions, a balance...

Original Articles
Analysis of the association between necrotizing enterocolitis and transfusion of red blood cell in very low birth weight preterm infants
Seon-Yeong Bak, Sihyoung Lee, Jae-Hong Park, Kyu-Hee Park, Ji-Hyun Jeon
Clin Exp Pediatr. 2013;56(3):112-115.   Published online March 18, 2013
Purpose

To investigate the association between necrotizing enterocolitis (NEC) and red blood cell transfusions in very low birth weight (VLBW) preterm infants.

Methods

We studied were 180 VLBW preterm infants who were admitted to the neonatal intensive care unit of CHA Gangnam Hospital from January of 2006 to December of 2009. The subjects were divided into 2 groups: an NEC group (greater than...

An inhibitory effect of tumor necrosis factor-alpha antagonist to gene expression in monocrotaline-induced pulmonary hypertensive rats model
Jung Hyun Kwon, Kwan Chang Kim, Min-Sun Cho, Hae Soon Kim, Sejung Sohn, Young Mi Hong
Clin Exp Pediatr. 2013;56(3):116-124.   Published online March 18, 2013
Purpose

Tumor necrosis factor (TNF)-α is thought to contribute to pulmonary hypertension. We aimed to investigate the effect of infliximab (TNF-α antagonist) treatment on pathologic findings and gene expression in a monocrotaline-induced pulmonary hypertension rat model.

Methods

Six-week-old male Sprague-Dawley rats were allocated to 3 groups: control (C), single subcutaneous injection of normal saline (0.1 mL/kg); monocrotaline (M), single subcutaneous injection of monocrotaline...

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...

Vascular endothelial dysfunction after anthracyclines treatment in children with acute lymphoblastic leukemia
Woo Jung Jang, Duk Yong Choi, In-Sang Jeon
Clin Exp Pediatr. 2013;56(3):130-134.   Published online March 18, 2013
Purpose

Anthracyclines have been utilized in the treatment of children with acute lymphoblastic leukemia (ALL). Recent studies have shown that anthracyclines may induce toxicity in the vascular endothelium. This study was performed using brachial artery reactivity (BAR) to evaluate vascular endothelial function in ALL patients who were treated with anthracycline chemotherapy.

Methods

We included 21 children with ALL who received anthracycline chemotherapy and...

Case Reports
A girl with sternal malformation/vascular dysplasia association
Na Yong Lee, Hye Kyung Cho, Kyung-Hyo Kim, Eun Ae Park
Clin Exp Pediatr. 2013;56(3):135-138.   Published online March 18, 2013

Sternal malformation/vascular dysplasia association is a rare congenital dysmorphology, which has not yet been reported in Korea. Its typical clinical features include a sternal cleft covered with atrophic skin, a median abdominal raphe extending from the sternal defect to the umbilicus, and cutaneous craniofacial hemangiomata. We report a case of a full-term newborn who presented with no anomalies at birth,...

X-linked recessive myotubular myopathy with MTM1 mutations
Young-Mi Han, Kyoung-Ah Kwon, Yun-Jin Lee, Sang-Ook Nam, Kyung-Hee Park, Shin-Yun Byun, Gu-Hwan Kim, Han-Wook Yoo
Clin Exp Pediatr. 2013;56(3):139-142.   Published online March 18, 2013

X-linked recessive myotubular myopathy (XLMTM) is a severe congenital muscle disorder caused by mutations in the MTM1 gene and characterized by severe hypotonia and generalized muscle weakness in affected males. It is generally a fatal disorder during the neonatal period and early infancy. The diagnosis is based on typical histopathological findings on muscle biopsy, combined with suggestive clinical features. We...

Correspondence
Erratum: Clinical, radiologic, and genetic features of Korean patients with Mucopolysaccharidosis IVA
Na Hee Lee, Sung Yoon Cho, Se Hyun Maeng, Tae Yeon Jeon, Young Bae Sohn, Su Jin Kim, Hyung-Doo Park, Dong Kyu Jin
Clin Exp Pediatr. 2013;56(3):143-143.   Published online March 18, 2013
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