Article 57(3); Mar 2014
Review Articles
Evidence for adverse effect of perinatal glucocorticoid use on the developing brain
Young Pyo Chang
Clin Exp Pediatr. 2014;57(3):101-109.   Published online March 31, 2014

The use of glucocorticoids (GCs) in the perinatal period is suspected of being associated with adverse effects on long-term neurodevelopmental outcomes for preterm infants. Repeated administration of antenatal GCs to mothers at risk of preterm birth may adversely affect fetal growth and head circumference. Fetal exposure to excess GCs during critical periods of brain development may profoundly modify the limbic...

Clinical utilization of cord blood over human health: experience of stem cell transplantation and cell therapy using cord blood in Korea
Young-Ho Lee
Clin Exp Pediatr. 2014;57(3):110-116.   Published online March 31, 2014

Cord blood (CB) has been used as an important and ethical source for hematopoietic stem cell transplantation (SCT) as well as cell therapy by manufacturing mesenchymal stem cell, induced pleuripotential stem cell or just isolating mononuclear cell from CB. Recently, the application of cell-based therapy using CB has expanded its clinical utility, particularly, by using autologous CB in children with...

Original Articles
Ten years of experience in the prevention of mother-to-child human immunodeficiency virus transmission in a university teaching hospital
Jung-Weon Park, Tae-Whan Yang, Yun-Kyung Kim, Byung-Min Choi, Hai-Joong Kim, Dae-Won Park
Clin Exp Pediatr. 2014;57(3):117-124.   Published online March 31, 2014
Purpose

Administration of antiretroviral drugs to mothers and infants significantly decreases mother-to-child human immunodeficiency virus (HIV) transmission; cesarean sections and discouraging breastfeeding further decreases this risk. The present study confirmed the HIV status of babies born to mothers infected with HIV and describes the characteristics of babies and mothers who received preventive treatment.

Methods

This study retrospectively analyzed medical records of nine infants...

Long-term follow-up of Fanconi anemia: clinical manifestation and treatment outcome
Byung Gyu Yoon, Hee Na Kim, Ui Joung Han, Hae In Jang, Dong Kyun Han, Hee Jo Baek, Tai Ju Hwang, Hoon Kook
Clin Exp Pediatr. 2014;57(3):125-134.   Published online March 31, 2014
Purpose

The aim of this study was to characterize Korean patients with Fanconi anemia (FA), which is a rare but very challenging genetic disease.

Methods

The medical records of 12 FA patients diagnosed at Chonnam National University Hospital from 1991 to 2012 were retrospectively reviewed.

Results

The median age at diagnosis was 6.2 years. All patients showed evidence of marrow failure and one or more...

Outcomes of chronic dialysis in Korean children with respect to survival rates and causes of death
Hye Jin Chang, Kyoung Hee Han, Min Hyun Cho, Young Seo Park, Hee Gyung Kang, Hae Il Cheong, Il Soo Ha
Clin Exp Pediatr. 2014;57(3):135-139.   Published online March 31, 2014
Purpose

Adult Korean patients on chronic dialysis have a 9-year survival rate of 50%, with cardiovascular problems being the most significant cause of death. The 2011 annual report of the North American Pediatric Renal Trials and Collaborative Studies group reported 3-year survival rates of 93.4% and relatively poorer survival in younger patients.

Methods

In this study, we have reviewed data from Korean Pediatric...

Lowe syndrome: a single center's experience in Korea
Hyun-Kyung Kim, Ja Hye Kim, Yoo-Mi Kim, Gu-Hwan Kim, Beom Hee Lee, Jin-Ho Choi, Han-Wook Yoo
Clin Exp Pediatr. 2014;57(3):140-148.   Published online March 31, 2014
Purpose

Lowe syndrome is a rare, X-linked recessive disorder caused by mutations in the OCRL gene. It involves multiple anatomic systems, particularly the eyes, central nervous system, and kidneys, and leads to profound growth failure and global developmental delay. This study evaluated the clinical and genetic characteristics of Korean patients with Lowe syndrome.

Methods

The clinical findings and results of genetic studies were...

Case Reports
Congenital muscular dystrophy type 1A with residual merosin expression
Hyo Jeong Kim, Young-Chul Choi, Hyung Jun Park, Young-Mock Lee, Heung Dong Kim, Joon Soo Lee, Hoon-Chul Kang
Clin Exp Pediatr. 2014;57(3):149-152.   Published online March 31, 2014

Congenital muscular dystrophy type 1A (MDC1A) is an autosomal recessive disorder characterized by hypotonia, elevated serum creatine kinase level, delayed motor milestones, white matter changes observed by brain magnetic resonance imaging, and normal intelligence. A mutation in the laminin α2 (LAMA2) gene, located at 6q22-23, is a genetic cause of MDC1A. Patients have merosin (laminin α2)-deficient skeletal muscles. However, the...

Toxic epidermal necrolysis induced by lamotrigine treatment in a child
Youngsuk Yi, Jeong Ho Lee, Eun Sook Suh
Clin Exp Pediatr. 2014;57(3):153-156.   Published online March 31, 2014

Toxic epidermal necrolysis is an unpredictable and severe adverse drug reaction. In toxic epidermal necrolysis, epidermal damage appears to result from keratinocyte apoptosis. This condition is triggered by many factors, principally drugs such as antiepileptic medications, antibiotics (particularly sulfonamide), nonsteroidal anti-inflammatory drugs, allopurinol, and nevirapine. Lamotrigine has been reported potentially cause serious cutaneous reactions, and concomitant use of valproic acid...

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