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Volume 54(6); Jun 2011
Review Articles
Excessive crying: behavioral and emotional regulation disorder in infancy
Joon Sik Kim
Korean J Pediatr. 2011;54(6):229-233.   Published online June 30, 2011

In the pediatric literature, excessive crying has been reported solely in association with 3-month colic and is described, if at all, as unexplained crying and fussing during the first 3 months of life. The bouts of crying are generally thought to be triggered by abdominal colic (over-inflation of the still immature gastrointestinal tract), and treatment is prescribed accordingly. According to...

Acute disseminated encephalomyelitis in children: differential diagnosis from multiple sclerosis on the basis of clinical course
Yun Jin Lee
Korean J Pediatr. 2011;54(6):234-240.   Published online June 30, 2011

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system (CNS) that typically presents as a monophasic disorder associated with multifocal neurologic symptoms and encephalopathy. ADEM is considered an autoimmune disorder that is triggered by an environmental stimulus in genetically susceptible individuals. The diagnosis of ADEM is based on clinical and radiological features. Most children with ADEM...

Mammalian target of rapamycin inhibitors for treatment in tuberous sclerosis
Won Seop Kim
Korean J Pediatr. 2011;54(6):241-245.   Published online June 30, 2011

Tuberous sclerosis complex (TSC) is a genetic multisystem disorder that results from mutations in the TSC1 or TSC2 genes, and is associated with hamartomas in several organs, including subependymal giant cell tumors. The neurological manifestations of TSC are particularly challenging and include infantile spasms, intractable epilepsy, cognitive disabilities, and autism. The TSC1- and TSC2-encoded proteins modulate cell function via the...

Original Articles
Outcomes of small for gestational age micropremies depending on how young or how small they are
Hee Joon Yu, Eun Sun Kim, Jin Kyu Kim, Hye Soo Yoo, So Yoon Ahn, Yun Sil Chang, Won Soon Park
Korean J Pediatr. 2011;54(6):246-252.   Published online June 30, 2011
Purpose

The outcomes of small for gestational age (SGA) infants especially in extremely low birth weight infants (ELBWIs) are controversial. This study evaluated the mortality and morbidity of ELBWIs, focusing on whether or not they were also SGA.

Methods

The medical records of 415 ELBWIs (birth weight <1,000 g), who were inborn and admitted to the Samsung Medical Center neonatal intensive care unit...

The metabolic syndrome and body composition in childhood cancer survivors
Young Bae Sohn, Su Jin Kim, Sung Won Park, Se-Hwa Kim, Sung-Yoon Cho, Soo Hyun Lee, Keon Hee Yoo, Ki Woong Sung, Jae Hoon Chung, Hong Hoe Koo, Dong-Kyu Jin
Korean J Pediatr. 2011;54(6):253-259.   Published online June 30, 2011
Purpose

Long-term survivors of childhood cancer appear to have an increased risk for the metabolic syndrome, subsequent type 2 diabetes and cardiovascular disease in adulthood compared to healthy children. The purpose of this study was to investigate the frequency of the metabolic syndrome and associated factors in childhood cancer survivors at a single center in Korea.

Methods

We performed a retrospective review of...

Clinical features of infantile hepatic hemangioendothelioma
Eun Hee Kim, Kyung Nam Koh, Meerim Park, Bo Eun Kim, Ho Joon Im, Jong Jin Seo
Korean J Pediatr. 2011;54(6):260-266.   Published online June 30, 2011
Purpose

Infantile hepatic hemangioendothelioma (IHHE) is the most common type of hepatic vascular tumor in infancy. We conducted this study to review our clinical experience of patients with IHHE and to suggest management strategies.

Methods

We retrospectively analyzed the medical records of 23 IHHE patients (10 males, 13 females) treated at the Asan Medical Center between 1996 and 2009.

Results

Median age at diagnosis was...

Case Reports
A case of de novo duplication of 15q24-q26.3
Eun Young Kim, Yu Kyong Kim, Mi Kyoung Kim, Ji Mi Jung, Ga Won Jeon, Hye Ran Kim, Jong Beom Sin
Korean J Pediatr. 2011;54(6):267-271.   Published online June 30, 2011

Distal duplication, or trisomy 15q, is an extremely rare chromosomal disorder characterized by prenatal and postnatal overgrowth, mental retardation, and craniofacial malformations. Additional abnormalities typically include an unusually short neck, malformations of the fingers and toes, scoliosis and skeletal malformations, genital abnormalities, particularly in affected males, and, in some cases, cardiac defects. The range and severity of symptoms and physical...

A sporadic case of Loeys-Dietz syndrome type I with two novel mutations of the TGFBR2 gene
Jung Sook Ha, Yeo Hyang Kim
Korean J Pediatr. 2011;54(6):272-275.   Published online June 30, 2011

A recently recognized connective tissue disorder, Loeys-Dietz syndrome (LDS) is a genetic aortic aneurysm syndrome caused by mutations in the transforming growth factor-receptor type I or II gene (TGFBR1 or TGFBR2). They have distinctive phenotypic abnormalities including widely spaced eyes (hypertelorism), bifid uvula or cleft palate, and arterial tortuosity with aortic aneurysm or dissection throughout the arterial tree. LDS is...

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