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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...

Expression of peroxisome proliferator-activated receptor (PPAR)-α and PPAR-γ in the lung tissue of obese mice and the effect of rosiglitazone on proinflammatory cytokine expressions in the lung tissue
Seung Lok Ryu, Jae Won Shim, Duk Soo Kim, Hye Lim Jung, Moon Soo Park, Soo-Hee Park, Jinmi Lee, Won-Young Lee, Jung Yeon Shim
Clin Exp Pediatr. 2013;56(4):151-158.   Published online April 22, 2013
Purpose

We investigated the mRNA levels of peroxisome proliferator-activated receptor (PPAR)-α, PPAR-γ, adipokines, and cytokines in the lung tissue of lean and obese mice with and without ovalbumin (OVA) challenge, and the effect of rosiglitazone, a PPAR-γ agonist.

Methods

We developed 6 mice models: OVA-challenged lean mice with and without rosiglitazone; obese mice with and without rosiglitazone; and OVA-challenged obese mice with and...

Case Report
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...

A case of mucolipidosis II presenting with prenatal skeletal dysplasia and severe secondary hyperparathyroidism at birth
Ju Sun Heo, Ka Young Choi, Se Hyoung Sohn, Curie Kim, Yoon Joo Kim, Seung Han Shin, Jae Myung Lee, Juyoung Lee, Jin A Sohn, Byung Chan Lim, Jin A Lee, Chang Won Choi, Ee-Kyung Kim, Han-Suk Kim, Beyong Il Kim, Jung-Hwan Choi
Clin Exp Pediatr. 2012;55(11):438-444.   Published online November 23, 2012

Mucolipidosis II (ML II) or inclusion cell disease (I-cell disease) is a rarely occurring autosomal recessive lysosomal enzyme-targeting disease. This disease is usually found to occur in individuals aged between 6 and 12 months, with a clinical phenotype resembling that of Hurler syndrome and radiological findings resembling those of dysostosis multiplex. However, we encountered a rare case of an infant...

Magnetic resonance imaging and spectroscopic analysis in 5 cases of Pelizaeus-Merzbacher disease: metabolic abnormalities as diagnostic tools
Eun Lee, Mi-Sun Yum, Hae-Won Choi, Han-Wook Yoo, Su Jeong You, Eun-Hye Lee, Tae-Sung Ko
Clin Exp Pediatr. 2012;55(10):397-402.   Published online October 29, 2012

Pelizaeus-Merzbacher disease (PMD) is a rare, X-linked recessive disorder characterized by dysmyelination in the central nervous system. PMD results from deletion, mutation, or duplication of the proteolipid protein gene (PLP1) located at Xq22, leading to the failure of axon myelination by oligodendrocytes in the central nervous system. PMD may be suspected when there are clinical manifestations such as nystagmus, developmental...

A case of cytomegalovirus-negative Ménétrier's disease with eosinophilia in a child
Keun Hyung Son, Jeong Ja Kwak, Jae Ock Park
Clin Exp Pediatr. 2012;55(8):293-296.   Published online August 23, 2012

Ménétrier's disease is a rare form of acquired gastropathy characterized by giant rugal folds in the stomach and protein-losing gastropathy. Children with Ménétrier's disease tend to follow a benign self-limited course with symptoms typically completely resolving within 2 to 10 weeks in contrast to the chronic course in adults. A 9-year-old girl presented with a history of gradually worsening abdominal...

Original Article
Neuroprotective effects of L-carnitine against oxygen-glucose deprivation in rat primary cortical neurons
Yu Jin Kim, Soo Yoon Kim, Dong Kyung Sung, Yun Sil Chang, Won Soon Park
Clin Exp Pediatr. 2012;55(7):238-248.   Published online July 17, 2012
Purpose

Hypoxic-ischemic encephalopathy is an important cause of neonatal mortality, as this brain injury disrupts normal mitochondrial respiratory activity. Carnitine plays an essential role in mitochondrial fatty acid transport and modulates excess acyl coenzyme A levels. In this study, we investigated whether treatment of primary cultures of rat cortical neurons with L-carnitine was able to prevent neurotoxicity resulting from oxygen-glucose deprivation...

Review Article
Effects of probiotics on the prevention of atopic dermatitis
Nam Yeun Kim, Geun Eog Ji
Clin Exp Pediatr. 2012;55(6):193-201.   Published online June 21, 2012

Atopic dermatitis (AD) is an immune disorder that is becoming increasingly prevalent throughout the world. The exact etiology of AD remains unknown, and a cure for AD is not currently available. The hypothesis that appropriate early microbial stimulation contributes to the establishment of a balanced immune system in terms of T helper type Th1, Th2, and regulatory T cell (Treg)...

Original Article
Successful and safe treatment of hemangioma with oral propranolol in a single institution
Sun Hee Chung, Dong Hyuk Park, Hye Lim Jung, Jae Won Shim, Deok Soo Kim, Jung Yeon Shim, Moon Soo Park, Hong Hoe Koo
Clin Exp Pediatr. 2012;55(5):164-170.   Published online May 21, 2012
Purpose

Dramatic improvement of hemangioma to propranolol has been recently reported; however, details on dose and duration of treatment, potential risks, and monitoring have not been determined. The objective of this study is to describe and analyze the use of propranolol as a first-line treatment or as a single therapy in management of complicated hemangioma.

Methods

A retrospective chart review of eight patients...

Correlation between glomerular filtration rate and urinary N acetyl-beta-D glucosaminidase in children with persistent proteinuria in chronic glomerular disease
Jeong Deok Hong, In Seok Lim
Clin Exp Pediatr. 2012;55(4):136-142.   Published online April 30, 2012
Purpose

Urinary excretion of N acetyl-beta-D glucosaminidase (NAG) and β2-microglobulin (β2-M) was increased in the presence of proximal tubular damage. Based on these urinary materials, we investigated the ability of expecting renal function in chronic glomerular diseases. In this study, we evaluated the relationship between glomerular filtration rate (GFR) urinary NAG, and urinary β2-M.

Methods

We evaluated 52 children with chronic kidney disease...

Comparison of the accuracy of neutrophil CD64 and C-reactive protein as a single test for the early detection of neonatal sepsis
Young Kwang Choo, Hyun-Seok Cho, In Bum Seo, Hyeon-Soo Lee
Clin Exp Pediatr. 2012;55(1):11-17.   Published online January 31, 2012
Purpose

Early identification of neonatal sepsis is a global issue because of limitations in diagnostic procedures. The objective of this study was to compare the diagnostic accuracy of neutrophil CD64 and C-reactive protein (CRP) as a single test for the early detection of neonatal sepsis.

Methods

A prospective study enrolled newborns with documented sepsis (n=11), clinical sepsis (n=12) and control newborns (n=14). CRP,...

Validation study of the Dinamap ProCare 200 upper arm blood pressure monitor in children and adolescents
Chong Guk Lee, Hyang Mi Park, Hye Jung Shin, Jin Soo Moon, Yeong Mi Hong, Nam Soo Kim, Il Soo Ha, Myeong Jin Chang, Kyeong Won Oh
Clin Exp Pediatr. 2011;54(11):463-469.   Published online November 30, 2011
Purpose

To validate the Dinamap ProCare 200 blood pressure (BP) monitor against a mercury sphygmomanometer in children 7 to 18 years old in accordance with the 2010 International Protocol of European Society of Hypertension (ESH-IP2) and the British Hypertension Society (BHS) protocol.

Methods

Forty-five children were recruited for the study. A validation procedure was performed following the protocol based on the ESH-IP2 and...



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