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Original Article
Cardiology
Prediction of unresponsiveness to second intravenous immunoglobulin treatment in patients with Kawasaki disease refractory to initial treatment
Euri Seo, Jeong Jin Yu, Hyun Ok Jun, Eun Jung Shin, Jae Suk Baek, Young-Hwue Kim, Jae-Kon Ko
Clin Exp Pediatr. 2016;59(10):408-413.   Published online October 17, 2016
Purpose

This study investigated predictors of unresponsiveness to second-line intravenous immunoglobulin (IVIG) treatment for Kawasaki disease (KD).

Methods

This was a single-center analysis of the medical records of 588 patients with KD who had been admitted to Asan Medical Center between 2006 and 2014. Related clinical and laboratory data were analyzed by univariate and multivariate logistic regression analyses.

Results

Eighty (13.6%) of the 588 patients...

Age-adjusted plasma N-terminal pro-brain natriuretic peptide level in Kawasaki disease
Heul Jun, Kyung Ok Ko, Jae Woo Lim, Jung Min Yoon, Gyung Min Lee, Eun Jung Cheon
Clin Exp Pediatr. 2016;59(7):298-302.   Published online July 31, 2016
Purpose

Recent reports showed that plasma N-terminal pro-brain natriuretic peptide (NT-proBNP) could be a useful biomarker of intravenous immunoglobulin (IVIG) unresponsiveness and coronary artery lesion (CAL) development in Kawasaki disease (KD). The levels of these peptides are critically influenced by age; hence, the normal range and upper limits for infants and children are different. We performed an age-adjusted analysis of plasma...

Case Report
Neurology
Two cases of familial cerebral cavernous malformation caused by mutations in the CCM1 gene
Im-Yong Yang, Mi-Sun Yum, Eun-Hee Kim, Hae-Won Choi, Han-Wook Yoo, Tae-Sung Ko
Clin Exp Pediatr. 2016;59(6):280-284.   Published online June 30, 2016

Cerebral cavernous malformation (CCM) is a vascular malformation characterized by abnormally enlarged capillary cavities without any intervening neural tissue. We report 2 cases of familial CCMs diagnosed with the CCM1 mutation by using a genetic assay. A 5-year-old boy presented with headache, vomiting, and seizure-like movements. Brain magnetic resonance imaging (MRI) revealed multiple CCM lesions in the cerebral hemispheres. Subsequent...

Original Article
Infection
Pneumocystis jirovecii pneumonia in pediatric patients: an analysis of 15 confirmed consecutive cases during 14 years
Kyung-Ran Kim, Jong Min Kim, Ji-Man Kang, Yae-Jean Kim
Clin Exp Pediatr. 2016;59(6):252-255.   Published online June 30, 2016
Purpose

Pneumocystis jirovecii pneumonia occurs in various immunocompromised patients. Despite the prophylaxis strategies in clinical practice, certain patients develop P. jirovecii pneumonia. This study was performed to investigate pediatric cases with P. jirovecii pneumonia in a single center.

Methods

We identified pediatric patients younger than 19 years with microbiologically confirmed P. jirovecii pneumonia from January 2000 to February 2014. A retrospective chart review...

Endocrinology
Lipopolysaccharide-binding protein plasma levels as a biomarker of obesity-related insulin resistance in adolescents
Ki Eun Kim, Young Sun Cho, Kyung Suk Baek, Lan Li, Kwang-Hyun Baek, Jung Hyun Kim, Ho-Seong Kim, Youn Ho Sheen
Clin Exp Pediatr. 2016;59(5):231-238.   Published online May 31, 2016
Purpose

Lipopolysaccharide-binding protein (LBP) is a 65-kDa acute phase protein, derived from the liver, which is present in high concentrations in plasma. Data regarding the association between circulating plasma LBP levels and obesity-related biomarkers in the pediatric population are scarce. We aimed to determine whether there was a difference in plasma LBP levels between overweight/obese and normal-weight adolescents and to assess...

Neurology
Clinical features and prognostic factors in drowning children: a regional experience
Kyung Lae Son, Su Kyeong Hwang, Hee Joung Choi
Clin Exp Pediatr. 2016;59(5):212-217.   Published online May 31, 2016
Purpose

This study aimed to evaluate the clinical features of children who have survived a water submersion incident, and to identify risk factors for prognosis.

Methods

We retrospectively reviewed the medical records of patients who experienced submersion between January 2005 and December 2014. The patients were classified into 2 groups, according to complications, and prognostic factors were evaluated.

Results

During the study period, 29 children...

Gastroenterology
Diagnostic value of the Vesikari Scoring System for predicting the viral or bacterial pathogens in pediatric gastroenteritis
Dong Ho Shim, Dong Yeon Kim, Ky Young Cho
Clin Exp Pediatr. 2016;59(3):126-131.   Published online March 31, 2016
Purpose

To evaluate the diagnostic value of the Vesikari Scoring System (VSS) as an early predictor of pathogens in children with acute gastroenteritis (AG).

Methods

In this retrospective study, the VSS score, absolute neutrophil count (ANC), and C-reactive protein (CRP) levels were analyzed in 107 hospitalized children with AG, aged 6 months to 17 years. Patients were divided into nonspecific, viral, and bacterial...

A prospective study to assess the efficacy and safety of oral propranolol as first-line treatment for infantile superficial hemangioma
Yeong Ju Yun, Yun Hee Gyon, Sohyoung Yang, Youn Kyung Lee, Joohyun Park, Meerim Park
Clin Exp Pediatr. 2015;58(12):484-490.   Published online December 22, 2015
Purpose

To determine the efficacy and safety of oral propranolol as a first-line treatment for superficially located infantile hemangioma (IH) and propose an assessment tool to measure treatment response.

Methods

Patients with superficial IH under 1 year of age were prospectively recruited between May 2012 and December 2013 at the Department of Pediatrics of Chungbuk National University Hospital. Propranolol was administered to 12...

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...

Prognostic factors in children with extracranial germ cell tumors treated with cisplatin-based chemotherapy
Jinsup Kim, Na Hee Lee, Soo Hyun Lee, Keon Hee Yoo, Ki Woong Sung, Hong Hoe Koo, Jeong-Meen Seo, Suk-Koo Lee
Clin Exp Pediatr. 2015;58(10):386-391.   Published online October 21, 2015
Purpose

To evaluate the outcomes and prognostic factors in children with extracranial germ cell tumors (GCTs) treated at a single institution.

Methods

Sixty-six children diagnosed with extracranial GCTs between 1996 and 2012 were included in the study. Primary treatment was surgical excision, followed by six cycles of cisplatin-based chemotherapy. The survival rates were compared according to the International Germ Cell Cancer Cooperative Group...

Long-term outcome of patients with p22phox-deficient chronic granulomatous disease on Jeju Island, Korea
Hyun Sik Kang, Geol Hwang, Kyung-Sue Shin
Clin Exp Pediatr. 2015;58(4):129-135.   Published online April 22, 2015
Purpose

This study investigated the long-term clinical outcomes of patients with p22phox-deficient chronic granulomatous disease (CGD) on Jeju Island and retrospectively evaluated the effects of interferon-gamma (IFN-γ) prophylaxis.

Methods

The medical records of 15 patients with CGD were retrospectively reviewed. The efficacy of IFN-γ prophylaxis was evaluated by comparing the frequency of severe infections before and after starting continuous prophylaxis with IFN-γ.

Results

At the...

Case Report
DiGeorge syndrome who developed lymphoproliferative mediastinal mass
Kyu Yeun Kim, Ji Ae Hur, Ki Hwan Kim, Yoon Jin Cha, Mi Jung Lee, Dong Soo Kim
Clin Exp Pediatr. 2015;58(3):108-111.   Published online March 20, 2015

DiGeorge syndrome is an immunodeficient disease associated with abnormal development of 3rd and 4th pharyngeal pouches. As a hemizygous deletion of chromosome 22q11.2 occurs, various clinical phenotypes are shown with a broad spectrum. Conotruncal cardiac anomalies, hypoplastic thymus, and hypocalcemia are the classic triad of DiGeorge syndrome. As this syndrome is characterized by hypoplastic or aplastic thymus, there are missing...

Original Article
Molecular typing of uropathogenic Escherichia coli isolated from Korean children with urinary tract infection
Ki Wook Yun, Do Soo Kim, Wonyong Kim, In Seok Lim
Clin Exp Pediatr. 2015;58(1):20-27.   Published online January 31, 2015
Purpose

We investigated the molecular types of uropathogenic Escherichia coli (UPEC) by using conventional phylogrouping, multilocus sequence typing (MLST), and fimH genotyping.

Methods

Samples of patients younger than 18 years of age were collected from the Chung-Ang University Hospital over 2 years. Conventional phylogenetic grouping for UPEC strains was performed by polymerase chain reaction (PCR). Bacterial strain sequence types (STs) were classified on...

Associations of matrix metalloproteinase (MMP)-8, MMP-9, and their inhibitor, tissue inhibitor of metalloproteinase-1, with obesity-related biomarkers in apparently healthy adolescent boys
Youn Ho Shin, Ki Eun Kim, Yong-Jae Lee, Jae-Hwan Nam, Young Mi Hong, Hye-Jung Shin
Clin Exp Pediatr. 2014;57(12):526-532.   Published online December 31, 2014
Purpose

Matrix metalloproteinases (MMPs) have been implicated in atherosclerosis, and therefore, are considered risk factors for metabolic dysfunction in adults. However, there is little data on circulating levels of MMPs and tissue inhibitors of MMPs (TIMPs) with regard to obesity-related biomarkers in the general adolescent population. In the present study, we determined the associations of MMP-8, MMP-9, and TIMP-1 levels and...

Effect of adenotonsillar hypertrophy on right ventricle function in children
Jin Hwan Lee, Jung Min Yoon, Jae Woo Lim, Kyung Og Ko, Seong Jun Choi, Jong-Yeup Kim, Eun Jung Cheon
Clin Exp Pediatr. 2014;57(11):484-488.   Published online November 30, 2014
Purpose

Chronic upper airway obstruction causes hypoxemic pulmonary vasoconstriction, which may lead to right ventricle (RV) dysfunction. Adenotonsillar hypertrophy (ATH) is the most common cause of upper airway obstruction in children. Therefore, we aimed to evaluate RV function in children with ATH.

Methods

Twenty-one children (male/female, 15/6; mean age, 92.3¡¾39.0 months; age range, 4-15 years) with ATH and 21 healthy age- and gender-matched...

Case Report
Inhaled iloprost for the treatment of patient with Fontan circulation
Yong Hyun Kim, Moon Hee Chae, Deok Young Choi
Clin Exp Pediatr. 2014;57(10):461-463.   Published online October 31, 2014

Decreased exercise capacity after Fontan surgery is relatively common and the failure of the Fontan state gradually increases with age. However, there is no further treatment for patients with Fontan circulation. Pulmonary vasodilation therapy is an effective method to solve this problem because pulmonary vascular resistance is a major factor of the Fontan problem. Inhaled iloprost is a chemically stable...

Original Article
Serum procalcitonin as a diagnostic marker of neonatal sepsis
In Ho Park, Seung Hyun Lee, Seung Taek Yu, Yeon Kyun Oh
Clin Exp Pediatr. 2014;57(10):451-456.   Published online October 31, 2014
Purpose

We evaluated serum procalcitonin (PCT) as a diagnostic marker of neonatal sepsis, and compared PCT levels with C-reactive protein (CRP) levels.

Methods

We retrospectively reviewed the medical records of 269 neonates with a suspected infection, admitted to Wonkwang University School of Medicine & Hospital between January 2011 and December 2012, for whom PCT and CRP values had been obtained. Neonates were categorized...

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...

Case Report
Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome
Ji Soo Kim, Min Jae Kim, E Young Bae, Dae Chul Jeong
Clin Exp Pediatr. 2014;57(4):202-205.   Published online April 30, 2014

Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is caused by the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effect of lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids, immunosuppressive agents, and immunoglobulins for managing the disease and controlling hemorrhages. Notably, steroids are the important treatment for treating hypoprothrombinemia and...

Original Article
Predictive value of C-reactive protein in response to macrolides in children with macrolide-resistant Mycoplasma pneumoniae pneumonia
Young Ho Seo, Jang Su Kim, Sung Chul Seo, Won Hee Seo, Young Yoo, Dae Jin Song, Ji Tae Choung
Clin Exp Pediatr. 2014;57(4):186-192.   Published online April 30, 2014
Purpose

The prevalence of macrolide-resistant Mycoplasma pneumoniae (MRMP) has increased worldwide. The aim of this study was to estimate the proportion of MRMP in a tertiary hospital in Korea, and to find potential laboratory markers that could be used to predict the efficacy of macrolides in children with MRMP pneumonia.

Methods

A total of 95 patients with M. pneumoniae pneumonia were enrolled in...

Review Article
Genetic risk factors associated with respiratory distress syndrome
Heui Seung Jo
Clin Exp Pediatr. 2014;57(4):157-163.   Published online April 30, 2014

Respiratory distress syndrome (RDS) among preterm infants is typically due to a quantitative deficiency of pulmonary surfactant. Aside from the degree of prematurity, diverse environmental and genetic factors can affect the development of RDS. The variance of the risk of RDS in various races/ethnicities or monozygotic/dizygotic twins has suggested genetic influences on this disorder. So far, several specific mutations in...

Inflammation and hyponatremia: an underrecognized condition?
Se Jin Park, Jae Il Shin
Clin Exp Pediatr. 2013;56(12):519-522.   Published online December 20, 2013

Timely diagnosis of hyponatremia is important for preventing potential morbidity and mortality as it is often an indicator of underlying disease. The most common cause of eurvolemic hyponatremia is the syndrome of inappropriate antidiuretic hormone (SIADH) secretion. Recent studies have demonstrated that proinflammatory cytokines such as interleukin (IL) 1β and IL-6 are involved in the development of hyponatremia, a condition...

Food protein-induced proctocolitis: Is this allergic disorder a reality or a phantom in neonates?
Jin-Bok Hwang, Jeana Hong
Clin Exp Pediatr. 2013;56(12):514-518.   Published online December 20, 2013

The etiology of small and fresh rectal bleeding in neonates who are not sick is usually unknown; the only known cause is food protein-induced proctocolitis (FPIPC). It has been recently reported that FPIPC is a rare cause of rectal bleeding in newborns, and most cases have been proved to be due to idiopathic neonatal transient colitis. A recommended strategy for...

Original Article
Evaluation of risk for graft-versus-host disease in children who receive less than the full doses of mini-dose methotrexate for graft-versus-host disease prophylaxis in allogeneic hematopoietic stem cell transplantation
Sook Kyung Yum, Hye-Yoon Choi, Jae Wook Lee, Pil-Sang Jang, Nack-Gyun Chung, Dae-Chul Jeong, Bin Cho, Hack-Ki Kim
Clin Exp Pediatr. 2013;56(11):490-495.   Published online November 27, 2013
Purpose

The use of cyclosporine and mini-dose methotrexate (MTX) is a common strategy for graft-versus-host disease (GVHD) prophylaxis in allogeneic transplants. We investigated whether patients who receive fewer than the planned MTX doses are at increased risk for GVHD.

Methods

The study cohort included 103 patients who received allogeneic transplants at the Department of Pediatrics of The Catholic University of Korea College of...

Review Article
Prevalence of rotavirus genotypes in South Korea in 1989-2009: implications for a nationwide rotavirus vaccine program
Van Thai Than, Wonyong Kim
Clin Exp Pediatr. 2013;56(11):465-473.   Published online November 27, 2013

The epidemiology of human group A rotavirus was analyzed by examining genotypic data acquired from 1989 to 2009 in South Korea. This information was derived from all the available published articles on rotavirus studies in South Korea, retrieved from both the PubMed and KoreaMed databases. Four common G types (G1, G2, G3, and G4) and three common P types (P[8],...

Clinical efficacy and mechanism of probiotics in allergic diseases
Ha-Jung Kim, Hyung Young Kim, So-Yeon Lee, Ju-Hee Seo, Eun Lee, Soo-Jong Hong
Clin Exp Pediatr. 2013;56(9):369-376.   Published online September 30, 2013

A complex interplay between genetic and environmental factors partially contributes to the development of allergic diseases by affecting development during prenatal and early life. To explain the dramatic increase in the prevalence of allergic diseases, the hygiene hypothesis proposed that early exposure to infection prevented allergic diseases. The hygiene hypothesis has changed to the microbial hypothesis, in which exposure to...

Original Article
Effects of coagulation factor concentrate prophylaxis in moderate and severe hemophilia A patients at a single hemophilia center in Korea
Byung Suk Moon, Jun Seok Choi, Chur Woo You
Clin Exp Pediatr. 2013;56(7):291-297.   Published online July 19, 2013
Purpose

The aim of this study was to investigate prophylactic treatment effects in Korean patients with severe hemophilia A.

Methods

A prospective study of 32 severe hemophilia A patients was conducted with the approval of the Institutional Review Board at the Eulji University Hospital. Two patients received primary prophylaxis; whereas, the other 30 patients were divided into 2 groups-secondary prophylaxis (n=15) and on-demand...

Case Report
Two cases of chronic pancreatitis associated with anomalous pancreaticobiliary ductal union and SPINK1 mutation
Eun Sam Rho, Earl Kim, Hong Koh, Han-Wook Yoo, Beom Hee Lee, Gu-Hwan Kim
Clin Exp Pediatr. 2013;56(5):227-230.   Published online May 28, 2013

Chronic pancreatitis is a progressive inflammatory disease resulting from repeated episodes of acute pancreatitis that impair exocrine function and eventually produce endocrine insufficiency. Some causes of chronic pancreatitis appear to be associated with alterations in the serine-protease inhibitor, Kazal type 1 (SPINK1), cationic trypsinogen (PRSS1), and cystic fibrosis-transmembrane conductance regulator (CFTR) genes, or with structural disorders in the pancreaticobiliary ductal...

Original Article
The hybrid perventricular closure of apical muscular ventricular septal defect with Amplatzer duct occluder
Soo Jin Kim, June Huh, Jin Young Song, Ji-Hyuk Yang, Tae-Gook Jun, I-Seok Kang
Clin Exp Pediatr. 2013;56(4):176-181.   Published online April 22, 2013
Purpose

Apical muscular ventricular septal defects (MVSDs), especially in small infants, can be difficult to manage using surgical and percutaneous closure. An intraoperative perventricular procedure is a good option for closing apical MVSDs in small children with or without associated cardiac anomalies. We evaluated the results of hybrid perventricular closure of apical MVSDs performed using an Amplatzer duct occluder (ADO).

Methods

We retrospectively...

p-Cresyl sulfate and indoxyl sulfate in pediatric patients on chronic dialysis
Hye Sun Hyun, Kyung Hoon Paik, Hee Yeon Cho
Clin Exp Pediatr. 2013;56(4):159-164.   Published online April 22, 2013
Purpose

Indoxyl sulfate and p-cresyl sulfate are important protein-bound uremic retention solutes whose levels can be partially reduced by renal replacement therapy. These solutes originate from intestinal bacterial protein fermentation and are associated with cardiovascular outcomes and chronic kidney disease progression. The aims of this study were to investigate the levels of indoxyl sulfate and p-cresyl sulfate as well as the...

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