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Case Report
Genetics and Metabolism
Compound heterozygous mutations of ACADS gene in newborn with short chain acyl-CoA dehydrogenase deficiency: case report and literatures review
Se Jin An, Sook Za Kim, Gu Hwan Kim, Han Wook Yoo, Han Hyuk Lim
Clin Exp Pediatr. 2016;59(Suppl 1):S45-S48.   Published online November 30, 2016

Short-chain acyl-CoA dehydrogenase deficiency (SCADD) is a rare autosomal recessive mitochondrial disorder of fatty acid β-oxidation, and is associated with mutations in the acyl-CoA dehydrogenase (ACADS) gene. Recent advances in spectrometric screening for inborn errors of metabolism have helped detect several metabolic disorders, including SCADD, without symptoms in the neonate period. This allows immediate initiation of treatment and monitoring, so...

Neurology
Glucose transport 1 deficiency presenting as infantile spasms with a mutation identified in exon 9 of SLC2A1
Hyun Hee Lee, Yun Jung Hur
Clin Exp Pediatr. 2016;59(Suppl 1):S29-S31.   Published online November 30, 2016

Glucose transport 1 (GLUT-1) deficiency is a rare syndrome caused by mutations in the glucose transporter 1 gene (SLC2A1) and is characterized by early-onset intractable epilepsy, delayed development, and movement disorder. De novo mutations and several hot spots in N34, G91, R126, R153, and R333 of exons 2, 3, 4, and 8 of SLC2A1 are associated with this condition. Seizures,...

Review Article
General Pediatrics
Apparent life-threatening event in infancy
Hee Joung Choi, Yeo Hyang Kim
Clin Exp Pediatr. 2016;59(9):347-354.   Published online September 21, 2016

An apparent life-threatening event (ALTE) is defined as the combination of clinical presentations such as apnea, marked change in skin and muscle tone, gagging, or choking. It is a frightening event, and it predominantly occurs during infancy at a mean age of 1–3 months. The causes of ALTE are categorized into problems that are: gastrointestinal (50%), neurological (30%), respiratory (20%),...

Neurology
White matter injury following rotavirus infection in neonates: new aspects to a forgotten entity, 'fifth day fits'?
Jung Sook Yeom, Chan-Hoo Park
Clin Exp Pediatr. 2016;59(7):285-291.   Published online July 31, 2016

That rotavirus infection can cause neurological symptoms in young children has been well established. However, it is surprising why rotavirus infection has been overlooked as a cause of neonatal seizures for many years, despite significant research interest in neonatal rotavirus infection. Neonates are the age group most vulnerable to seizures, which are typically attributed to a wide range of causes....

Case Report
Gastroenterology
A giant choledochal cyst in infancy: a case report
Nursel Yurttutan, Suleyman Cuneyt Karakus, Naim Koku, Mustafa Demirci, Ramazan Ucak
Clin Exp Pediatr. 2016;59(5):239-241.   Published online May 31, 2016

Choledochal cyst is a dilation that encloses the intrahepatic or both extra- and intrahepatic portions of the biliary ducts. Postnatally, ultrasonography is the initial diagnostic modality of choice, allowing for precise measurements of intra- or extrahepatic duct dilatation and identification of stones and sludge. Symptoms depend on the age at presentation. Common bile duct malformations should be considered as a...

Original Article
Gastroenterology
Efficacy of proton pump inhibitors and H2 blocker in the treatment of symptomatic gastroesophageal reflux disease in infants
Hamid Reza Azizollahi, Mandana Rafeey
Clin Exp Pediatr. 2016;59(5):226-230.   Published online May 31, 2016
Purpose

Gastroesophageal reflux disease (GERD) occurs in pediatric patients when reflux of gastric contents presents with troublesome symptoms. The present study compared the effects of omeprazole and ranitidine for the treatment of symptomatic GERD in infants of 2-12 months.

Methods

This study was a clinical randomized double-blind trial and parallel-group comparison of omeprazole and ranitidine performed at Children Training Hospital in Tabriz, Iran....

Cardiology
Correlation of B-type natriuretic peptide levels and echocardiographic parameters in preterm infants with patent ductus arteriosus
Hyun Ah Jeong, Jeonghee Shin, Eunji Kim, Eun Hee Lee, Byung Min Choi, Chang Sung Son, Joo Won Lee
Clin Exp Pediatr. 2016;59(4):183-189.   Published online April 30, 2016
Purpose

This study aimed to evaluate the correlation, according to postnatal age, between plasma B-type natriuretic peptide (BNP) levels and echocardiographic parameters for the assessment of patent ductus arteriosus (PDA) in preterm infants with respiratory distress.

Methods

We enrolled 42 preterm infants with respiratory distress who underwent serial echocardiographic evaluation with simultaneous plasma BNP measurements until ductal closure. The correlations between BNP levels...

Neonatology (Perinatology)
Modification of nutrition strategy for improvement of postnatal growth in very low birth weight infants
Ah Young Choi, Yong Wook Lee, Mea-young Chang
Clin Exp Pediatr. 2016;59(4):165-173.   Published online April 30, 2016
Purpose

To identify the effects of modified parenteral nutrition (PN) and enteral nutrition (EN) regimens on the growth of very low birth weight (VLBW) infants.

Methods

The study included VLBW infants weighing <1,500 g, admitted to Chungnam National University Hospital between October 2010 and April 2014, who were alive at the time of discharge. Subjects were divided according to 3 periods: period 1...

Case Report
Neonatology (Perinatology)
Successfully treated infective endocarditis caused by methicillin-resistant Staphylococcus Aureus in extremely low birth weight infant
Sehwa Jung, Kyung Uk Jeong, Jang Hoon Lee, Jo Won Jung, Moon Sung Park
Clin Exp Pediatr. 2016;59(2):96-99.   Published online February 29, 2016

Survival rates of preterm infants have improved in the past few decades, and central venous catheters play an important role in the intensive medical treatment of these neonates. Unfortunately, these indwelling catheters increase the risk of intracardiac thrombosis, and they provide a nidus for microorganisms during the course of septicemia. Herein, we report a case of persistent bacteremia due to...

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,...

Neonatology (Perinatology)
Clinical features of Bednar's aphthae in infants
Seung-Woo Nam, Seol Hee Ahn, Son-Moon Shin, Goun Jeong
Clin Exp Pediatr. 2016;59(1):30-34.   Published online January 22, 2016
Purpose

Although Bednar's aphthae are common and regress spontaneously, these lesions may lead to feeding intolerance and are often misdiagnosed, rendering examinations useless. This study sheds new light on the clinical features of Bednar's aphthae.

Methods

Sixteen neonates and infants were newly diagnosed with Bednar's aphthae via routine health check-ups in an outpatient clinic. Medical records were retrospectively reviewed, and the following parameters...

Review Article
Endocrinology
Catch-up growth and catch-up fat in children born small for gestational age
Won Kyoung Cho, Byung-Kyu Suh
Clin Exp Pediatr. 2016;59(1):1-7.   Published online January 22, 2016

Infants born small for gestational age (SGA) are at increased risk of perinatal morbidity, persistent short stature, and metabolic alterations in later life. Recent studies have focused on the association between birth weight (BW) and later body composition. Some reports suggest that fetal nutrition, as reflected by BW, may have an inverse programing effect on abdominal adiposity later in life....

Original Article
Severe vitamin D deficiency in preterm infants: maternal and neonatal clinical features
Sook-Hyun Park, Gi-Min Lee, Jung-Eun Moon, Heng-Mi Kim
Clin Exp Pediatr. 2015;58(11):427-433.   Published online November 22, 2015
Purpose

We investigated the vitamin D status of preterm infants to determine the incidence of vitamin D deficiency.

Methods

A total of 278 preterm infants delivered at Kyungpook National University Hospital between January 2013 and May 2015 were enrolled. The serum concentrations of calcium, phosphorous, alkaline phosphatase, and 25-hydroxyvitamin D (25-OHD) were measured at birth. We collected maternal and neonatal data such as...

Low levels of tissue inhibitor of metalloproteinase-2 at birth may be associated with subsequent development of bronchopulmonary dysplasia in preterm infants
Choae Lee, Jaewoo An, Ji Hee Kim, Eun Sun Kim, Soo Hyun Kim, Yeon Kyung Cho, Dong Hyun Cha, Man Yong Han, Kyu Hyung Lee, Youn Ho Sheen
Clin Exp Pediatr. 2015;58(11):415-420.   Published online November 22, 2015
Purpose

Bronchopulmonary dysplasia (BPD) is characterized by inflammation with proteolytic damage to the lung extracellular matrix. The results from previous studies are inconsistent regarding the role of proteinases and antiproteinases in the development of BPD. The aim of the present study was to investigate whether matrix metalloproteinase (MMP)-8, MMP-9, tissue inhibitor of metalloproteinase (TIMP)-2, and TIMP-1 levels in the serum of...

Review Article
Early-onset epileptic encephalopathies and the diagnostic approach to underlying causes
Su-Kyeong Hwang, Soonhak Kwon
Clin Exp Pediatr. 2015;58(11):407-414.   Published online November 22, 2015

Early-onset epileptic encephalopathies are one of the most severe early onset epilepsies that can lead to progressive psychomotor impairment. These syndromes result from identifiable primary causes, such as structural, neurodegenerative, metabolic, or genetic defects, and an increasing number of novel genetic causes continue to be uncovered. A typical diagnostic approach includes documentation of anamnesis, determination of seizure semiology, electroencephalography, and...

Original Article
The efficacy and safety of Montelukast sodium in the prevention of bronchopulmonary dysplasia
Sang Bum Kim, Jang Hoon Lee, Juyoung Lee, Seung Han Shin, Ho Sun Eun, Soon Min Lee, Jin A Sohn, Han Suk Kim, Byung Min Choi, Min Soo Park, Kook In Park, Ran Namgung, Moon Sung Park
Clin Exp Pediatr. 2015;58(9):347-353.   Published online September 21, 2015
Purpose

The purpose of this study was to evaluate the efficacy and safety of Montelukast sodium in the prevention of bronchopulmonarydysplasia (BPD).

Methods

The Interventional study was designed as a multicenter, prospective, and randomized trial, with open labeled and parallel-experimental groups, 66 infants were enrolled and allocated to either the case group (n=30) or the control group (n=36) based on gestational age (GA)....

Evaluation of three glucometers for whole blood glucose measurements at the point of care in preterm or low-birth-weight infants
Joon Ho Hwang, Yong-Hak Sohn, Seong-Sil Chang, Seung Yeon Kim
Clin Exp Pediatr. 2015;58(8):301-308.   Published online August 21, 2015
Purpose

We evaluated three blood glucose self-monitoring for measuring whole blood glucose levels in preterm and low-birth-weight infants.

Methods

Between December 1, 2012 and March 31, 2013, 230 blood samples were collected from 50 newborns, who weighed, ≤2,300 g or were ≤36 weeks old, in the the neonatal intensive care unit of Eulji University Hospital. Three blood glucose self-monitoring (A: Precision Pcx, Abbott;...

Comparison of gastric and other bowel perforations in preterm infants: a review of 20 years' experience in a single institution
Do Kyung Lee, So Yeon Shim, Su Jin Cho, Eun Ae Park, Sun Wha Lee
Clin Exp Pediatr. 2015;58(8):288-293.   Published online August 21, 2015
Purpose

In this study, we aimed to review the clinical presentation of preterm infants with gastrointestinal perforations and compare the clinical features of gastric perforation with other intestinal perforations.

Methods

The medical records of preterm neonates with pneumoperitoneum, admitted to the neonatal intensive care unit (NICU) between January 1994 and December 2013, were retrospectively reviewed.

Results

Twenty-one preterm infants underwent exploratory laparotomy to investigate the...

Iron and vitamin D status in breastfed infants and their mothers
Yu Sun Kang, Joon Hwan Kim, Eun Hee Ahn, Eun-Gyong Yoo, Moon Kyu Kim
Clin Exp Pediatr. 2015;58(8):283-287.   Published online August 21, 2015
Purpose

We assessed the relationships between iron and vitamin D statuses in breastfed infants and their mothers and evaluated the determinants of iron and vitamin D deficiencies in breastfed infants.

Methods

Seventy breastfed infants aged 4-24 months and their mothers participated in this study from February 2012 to May 2013. Complete blood counts, total iron binding capacity, and levels of C-reactive protein, iron,...

Thyroid dysfunction in very low birth weight preterm infants
Ji Hoon Lee, Sung Woo Kim, Ga Won Jeon, Jong Beom Sin
Clin Exp Pediatr. 2015;58(6):224-229.   Published online June 22, 2015
Purpose

Thyroid dysfunction is common in preterm infants. Congenital hypothyroidism causes neurodevelopmental impairment, which is preventable if properly treated. This study was conducted to describe the characteristics of thyroid dysfunction in very low birth weight infants (VLBWIs), evaluate risk factors of hypothyroidism, and suggest the reassessment of thyroid function with an initially normal thyroid-stimulating hormone (TSH) as part of a newborn...

Validity of bag urine culture for predicting urinary tract infections in febrile infants: a paired comparison of urine collection methods
Geun-A Kim, Ja-Wook Koo
Clin Exp Pediatr. 2015;58(5):183-189.   Published online May 22, 2015
Purpose

Catheter urine (CATH-U) and suprapubic aspiration (SPA) are reliable urine collection methods for confirming urinary tract infections (UTI) in infants. However, noninvasive and easily accessible collecting bag urine (CBU) is widely used, despite its high contamination rate. This study investigated the validity of CBU cultures for diagnosing UTIs, using CATH-U culture results as the gold standard.

Methods

We retrospectively analyzed 210 infants,...

Review Article
Nutritional strategy of early amino acid administration in very low birth weight infants
Byong Sop Lee
Clin Exp Pediatr. 2015;58(3):77-83.   Published online March 20, 2015

Relative to a fetus of the same gestational age, very low birth weight (VLBW) infants are more likely to be underfed and to undergo growth restriction during their early hospital stay. The current trend towards "early and aggressive" nutritional strategies in VLBW infants aims to overcome the early nutritional deficiency and thereby boost postnatal catch-up growth, simultaneously improving long-term neurodevelopmental...

Original Article
Postnatal weight gain in the first two weeks as a predicting factor of severe retinopathy of prematurity requiring treatment
Jongmoon Kim, Jang Yong Jin, Sung Shin Kim
Clin Exp Pediatr. 2015;58(2):52-59.   Published online February 28, 2015
Purpose

This study aimed to investigate the relative weight gain at 2-week intervals up to 6 weeks after birth to predict retinopathy of prematurity (ROP) requiring treatment among very low birth weight infants.

Methods

A total of 211 preterm infants with birth weights <1,500 g and gestational age <32 weeks were retrospectively reviewed. The main outcome was the development of ROP requiring treatment....

Case Report
Neonatal invasive Streptococcus gallolyticus subsp. pasteurianus infection with delayed central nervous system complications
Jung-Weon Park, So-Hee Eun, Eui-Chong Kim, Moon-Woo Seong, Yun-Kyung Kim
Clin Exp Pediatr. 2015;58(1):33-36.   Published online January 31, 2015

Group D streptococci are known to cause newborn septicemia and meningitis, but the Streptococcus bovis group strains rarely cause serious neonatal infections in Korea. Central nervous system (CNS) complications of neonatal S. bovis group infection have rarely been reported. In adults, S. bovis group strains cause bacteremia and endocarditis, and are associated with gastrointestinal malignancy. However, only a few studies...

Review Article
Necrotizing enterocolitis in newborns: update in pathophysiology and newly emerging therapeutic strategies
Young Youn Choi
Clin Exp Pediatr. 2014;57(12):505-513.   Published online December 31, 2014

While the survival of extremely premature infants with respiratory distress syndrome has increased due to advanced respiratory care in recent years, necrotizing enterocolitis (NEC) remains the leading cause of neonatal mortality and morbidity. NEC is more prevalent in lower gestational age and lower birth weight groups. It is characterized by various degrees of mucosal or transmural necrosis of the intestine....

Adrenal and thyroid function in the fetus and preterm infant
Hye Rim Chung
Clin Exp Pediatr. 2014;57(10):425-433.   Published online October 31, 2014

Adrenal and thyroid hormones are essential for the regulation of intrauterine homeostasis, and for the timely differentiation and maturation of fetal organs. These hormones play complex roles during fetal life, and are believed to underlie the cellular communication that coordinates maternal-fetal interactions. They serve to modulate the functional adaptation for extrauterine life during the perinatal period. The pathophysiology of systemic...

Efficacy and safety of growth hormone treatment for children born small for gestational age
Il Tae Hwang
Clin Exp Pediatr. 2014;57(9):379-383.   Published online September 30, 2014

Recombinant growth hormone (GH) is an effective treatment for short children who are born small for gestational age (SGA). Short children born SGA who fail to demonstrate catch-up growth by 2-4 years of age are candidates for GH treatment initiated to achieve catch-up growth to a normal height in early childhood, maintain a normal height gain throughout childhood, and achieve...

Original Article
Clinical characteristics and serum N-terminal pro-brain natriuretic peptide as a diagnostic marker of Kawasaki disease in infants younger than 3 months of age
Hyun Kyung Bae, Do Kyung Lee, Jung Hyun Kwon, Hae Soon Kim, Sejung Sohn, Young Mi Hong
Clin Exp Pediatr. 2014;57(8):357-362.   Published online August 25, 2014
Purpose

The incidence of Kawasaki disease (KD) is rare in young infants (less than 3 months of age), who present with only a few symptoms that fulfill the clinical diagnostic criteria. The diagnosis for KD can therefore be delayed, leading to a high risk of cardiac complications. We examined the clinical characteristics and measured the serum levels of N-terminal pro-brain natriuretic...

Fecal calprotectin concentration in neonatal necrotizing enterocolitis
Jung Min Yoon, Ju Yi Park, Kyung Ok Ko, Jae Woo Lim, Eun Jeong Cheon, Hyo Jeong Kim
Clin Exp Pediatr. 2014;57(8):351-356.   Published online August 25, 2014
Purpose

Among the many factors associated with acute intestinal mucosal infection, numerous studies have proposed the usefulness of fecal calprotectin. The aim of this study was to evaluate the usefulness of fecal calprotectin in the diagnosis of necrotizing enterocolitis (NEC).

Methods

We collected 154 stool samples from 16 very low birth weight and premature newborns at the Konyang University Hospital neonatal intensive care...

Review Article
Mesenchymal stem cells transplantation for neuroprotection in preterm infants with severe intraventricular hemorrhage
So Yoon Ahn, Yun Sil Chang, Won Soon Park
Clin Exp Pediatr. 2014;57(6):251-256.   Published online June 30, 2014

Severe intraventricular hemorrhaging (IVH) in premature infants and subsequent posthemorrhagic hydrocephalus (PHH) causes significant mortality and life-long neurological complications, including seizures, cerebral palsy, and developmental retardation. However, there are currently no effective therapies for neonatal IVH. The pathogenesis of PHH has been mainly explained by inflammation within the subarachnoid spaces due to the hemolysis of extravasated blood after IVH. Obliterative...



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