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Original Article
Preliminary analysis of metabolic syndrome components in Korean adolescents by using Korean national health and nutrition examination Survey pooling data (1998, 2001, and 2005)
Kyoung Huh, Mi Jung Park
Clin Exp Pediatr. 2008;51(12):1300-1309.   Published online December 15, 2008
Purpose : This study aimed to estimate age- and gender-specific cut points for metabolic syndrome (MS) components, including body mass index (BMI), blood pressure (BP), triglycerides, high-density lipoprotein (HDL) cholesterol, and glucose. Methods : Data from the 1998, 2001, and 2005 Korean NHANES (National Health and Nutrition Examination Survey) were analyzed (n=4164; 2,139 boys and 2,025 girls, aged 10-19 years)....
Case Report
A case of Smith-Lemli-Opitz syndrome diagnosed by identification of mutations in the 7-dehydrocholesterol reductase (DHCR7) gene
Mee Rim Park, Jung Min Ko, Chong-Keun Cheon, Gu-Hwan Kim, Han-Wook Yoo
Clin Exp Pediatr. 2008;51(11):1236-1240.   Published online November 15, 2008
Smith-Lemli-Opitz syndrome (SLOS) is a rare, autosomal recessive disease caused by an inborn error in cholesterol synthesis. Patients with this disease suffer from multiple malformations due to reduced activity of 7-dehydrocholesterol reductase (DHCR7), which increases 7-dehydrocholesterol (7DHC) and 8-dehydrocholesterol (8DHC) concentrations and decreases cholesterol concentration in body fluids and tissue. The SLOS phenotypic spectrum ranges from a mild disorder with...
A case of vancomycin-induced drug hypersensitivity syndrome
Kyung Sun Min, Woo Yeon Choi, Eun Song Song, Dong Kyun Han, Young Kuk Cho, Jae Sook Ma
Clin Exp Pediatr. 2008;51(11):1228-1231.   Published online November 15, 2008
Drug hypersensitivity syndrome (DHS) has rarely been reported in association with vancomycin treatment. Here, we describe an 11-year-old girl who developed fever and a maculopapular rash on day 18 of intravenous vancomycin for treatment of infective endocarditis. The patient presented with fever, a maculopapular skin rash, hepatitis, and acute renal failure caused by vancomycin-induced DHS. The symptoms resolved in less...
A case of restless legs syndrome in a child presenting with growing pains
Dong Soon Kim, Hong Beom Shin, Young Min Ahn
Clin Exp Pediatr. 2008;51(11):1222-1227.   Published online November 15, 2008
Restless legs syndrome (RLS) is a common neurological sleep disorder in adults characterized by the following diagnostic criteria: an urge to move that is usually associated with unpleasant sensations and symptoms that are worse at rest, relieved by movement, and most severe at night. The definite diagnosis of RLS in children is stricter and consists of self-description of leg discomfort...
Original Article
Study of the risk factors for pulmonary interstitial emphysema related to mechanical ventilator care
Sang Yeob Kim, Pil Sang Lee, Sang Geel Lee
Clin Exp Pediatr. 2008;51(11):1179-1184.   Published online November 15, 2008
Purpose : Pulmonary interstitial emphysema (PIE) primarily occurs in preterm infants suffering from respiratory distress syndrome (RDS) and kept under mechanical ventilator care. Therefore, this study aimed to examine various risk factors for PIE, to identify conditions that can decrease the possibility of PIE development. Methods : PIE classification was conducted for 183 patients diagnosed to have RDS and receiving mechanical...
Clinical significance of the mechanical properties of the abdominal aorta in Kawasaki disease
Mi Jin Kim, Mi Jin Kim, Sang Yun Lee, Sang Yun Lee, Yong Bum Kim, Yong Bum Kim, Hong Ryang Kil, Hong Ryang Kil
Clin Exp Pediatr. 2008;51(9):1012-1017.   Published online September 15, 2008
Purpose : This study aimed to assess the mechanical properties of the abdominal aorta in school-aged patients treated for Kawasaki disease and in normal, healthy children. Methods : This study examined 28 children with Kawasaki disease who had been followed up on and 30 healthy subjects of the same age and gender. We recorded systolic (Ps) and diastolic (Pd) blood pressure...
The pharmacological treatment of patent ductus arteriosus in premature infants with respiratory distress syndrome: oral ibuprofen vs. indomethacin
Soo Jin Lee, Ji Young Kim, Eun Ae Park, Sejung Sohn
Clin Exp Pediatr. 2008;51(9):956-963.   Published online September 15, 2008
Purpose : Indomethacin is widely used for the prophylaxis and treatment of patent ductus arteriosus (PDA); however, it is associated with side effects such as renal failure, intraventricular hemorrhage, and gastrointestinal bleeding. Intravenous ibuprofen has been shown to be as effective as indomethacin in prompting PDA closure. If treatment with oral ibuprofen is as effective as indomethacin, it would have...
Case Report
A case of acute respiratory distress syndrome associated with congenital H-type tracheoesophageal fistula and gastroesophageal reflux
Heewon Chueh, Myo Jing Kim, Jin-A Jung
Clin Exp Pediatr. 2008;51(8):892-895.   Published online August 15, 2008
H-type tracheoesophageal fistula (TEF) is extremely rare in infants and children, and clinical manifestations of this condition are diverse based on its severity. Some cases of congenital TEF diagnosed in adulthood have been reported, which indicate the difficulty of early diagnosis of this disease. Gastroesophageal reflux (GER) may induce chronic aspiration, pulmonary aspiration, apparent life-threatening events, and failure to thrive....
Original Article
Effects and adverse-effects of growth hormone therapy in children with Prader-Willi syndrome: A two year study
Su Jin Kim, Joong Bum Cho, Min Jung Kwak, Eun Kyung Kwon, Kyung Hoon Paik, Dong-Kyu Jin
Clin Exp Pediatr. 2008;51(7):742-746.   Published online July 15, 2008
Purpose : The objective of this study was to evaluate the effects and adverse side-effects of growth hormone (GH) therapy in children with Prader-Willi syndrome (PWS). Methods : Forty-one patients who had been treated with GH for more than two years (24 boys and 17 girls, mean age 7.3?.3 years during treatment initiation) were enrolled for this study. Results : After...
Is routine screening examination necessary for detecting thromboembolism in childhood nephrotic syndrome?
Mun Sub Kim, Ja Wook Koo, Soung Hee Kim
Clin Exp Pediatr. 2008;51(7):736-741.   Published online July 15, 2008
Purpose : The incidence of thromboembolic episodes in children with nephrotic syndrome (NS) is low; however, these episodes are often severe. Moreover, both pulmonary thromboembolism (PTE) and renal vein thrombosis (RVT) rarely show clinical symptoms. This study was performed to determine the benefits of routine screening in the detection of thrombosis in childhood NS. Methods : Among 62 children with...
Clinical characteristics of severe meconium aspiration syndrome
Chang Won Choi, Beyong Il Kim, Hyun Ju Lee, Kyoung Eun Joung, Gyu Hong Shim, In Suk Lim, Jin-A Lee, Ee-Kyung Kim, Han-Suk Kim, Jung-Hwan Choi
Clin Exp Pediatr. 2008;51(7):713-721.   Published online July 15, 2008
Purpose : This study aims to describe the clinical characteristics of severe meconium aspiration syndrome (MAS) which required mechanical ventilation over 48 h and to delineate the progress of respiratory failure and radiographic findings in severe MAS. Methods : Twelve infants admitted to the Neonatal Intensive Care Unit (NICU) of the Seoul National University Bundang Hospital diagnosed with severe MAS from...
Case Report
A case of simultaneously identified glycogen storage disease and mucopolysaccharidosis
Ju Young Lee, Jeong Ok Shim, Hye Ran Yang, Ju Young Chang, Choong Ho Shin, Jae Sung Ko, Jeong Kee Seo, Woo Sun Kim, Gyeong Hoon Kang, Jeong Han Song, Jong Won Kim
Clin Exp Pediatr. 2008;51(6):650-654.   Published online June 15, 2008
Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which...
A case of Hallermann-Streiff syndrome with aphakia
Myung Chul Lee, Im Jeong Choi, Jin Wha Jung
Clin Exp Pediatr. 2008;51(6):646-649.   Published online June 15, 2008
Hallermann-Streiff syndrome is a rare disease. Approximately 150 cases have been reported, including 6 cases in Korea. The authors experienced a case of Hallermann-Streiff syndrome in a 6-year-old female with aphakia. The syndrome is characterized by a bird-like face, dental abnormalities, hypotrichosis, atrophy of the skin, bilateral microphthalmia, and proportionate dwarfism. A brief review of the literature was conducted.
Review Article
Epidemiologic characteristics of type 1 diabetes in children aged 14 years or under in Korea, 1985-2000
Choong Ho Shin
Clin Exp Pediatr. 2008;51(6):569-575.   Published online June 15, 2008
Type 1 diabetes mellitus (T1DM) develops in genetically susceptible individuals as a result of progressive autoimmune destruction of beta cells. There is a large global variation in incidence among children aged 0-14 years. The incidence of T1DM in Korea is very low. The latest survey in Korea was conducted in 2001 by the Korean Society of Pediatrics to analyze ...
Epidemiology of the metabolic syndrome among Korean children and adolescents
Mi Jung Park
Clin Exp Pediatr. 2008;51(6):564-568.   Published online June 15, 2008
The prevalence of obesity in Korean children is estimated to be around 10%, and has increased significantly over the past 20 years. Metabolic syndrome, which includes central obesity, glucose intolerance, insulin resistance, dyslipidemia, and hypertension, is a well documented risk factor for cardiovascular diseases, but there is no general consensus concerning its definition for children. In this study, ablut 30-40%...
Case Report
A case of two sisters births from mother with phenylketonuria lacking mental retardation
Chang-Seok Ki, Jin Kyung Kim
Clin Exp Pediatr. 2008;51(5):546-550.   Published online May 15, 2008
In this untreated classic phenylketonuria (PKU) case, mental retardation is severe; however, there have been individuals­like the mother of this case­who have escaped mental retardation and all the other potential sequelae of phenylketonuria, despite having high blood phenylalanine levels, and very poor dietary control. It appears that they have nearly normal brain phenylalanine levels despite high blood phenylalanine (Phe) levels....
Cerebro-oculo-facio-skeletal syndrome : A case report
So Hee Lee, Seong Jin Hong, Jung Hwa Lee, Soo Yun Oh, Sun Heum Kim, Duk Hwan Kho, Kyo Sun Kim
Clin Exp Pediatr. 2008;51(4):435-438.   Published online April 15, 2008
The Cerebro-oculo-facio-skeletal (COFS) syndrome is a rare autosomal recessive disorder characterized by multiple abnormalities that involve the brain, face, eyes, and extremities. COFS syndrome is regarded as a degenerative disorder of the brain and spinal cord caused by a mutation of the DNA repair genes. We report on an 8-month-old girl with COFS syndrome who exhibited growth and developmental delay,...
Original Article
Phenotype-genotype correlations and the efficacy of growth hormone treatment in Korean children with Prader-Willi syndrome
Keun Wook Bae, Jung Min Ko, Han-Wook Yoo
Clin Exp Pediatr. 2008;51(3):315-322.   Published online March 15, 2008
Purpose : Prader-Willi syndrome (PWS) is a complex genetic disorder, caused by the deletion of the paternally derived 15q11-13 region or the maternal uniparental disomy of chromosome 15 (mUPD(15)). In this study, we compared phenotypic differences between those patients whose disease was caused by microdeletion and those caused by mUPD(15). In addition, a comparison of the efficacy of growth hormone...
Therapeutic response of cyclosporine and outcome in steroid resistant nephrotic syndrome
Hyung Soon Choi, Joo Hoon Lee, Young Seo Park
Clin Exp Pediatr. 2008;51(3):293-298.   Published online March 15, 2008
Purpose : The aim of our study was to evaluate the therapeutic response to cyclosporine, time to remission and side effects in steroid resistant nephrotic syndrome (SRNS). Methods : This study included 22 children with idiopathic SRNS who were treated with cyclosporine between June 1989 and August 2006. Medical records were reviewed retrospectively. Results : The mean age of patients at diagnosis...
Discrimination between obstructive sleep apnea syndrome and primary snoring in children : comparison of clinical parameters and behavioral disturbance
Hyun-Joo Seo, Jae Suk Lee, Hong-Beom Shin, Eui-Joong Kim, Hyun-Joon Shim, Young-Min Ahn
Clin Exp Pediatr. 2008;51(3):267-275.   Published online March 15, 2008
Purpose : To determine whether primary snoring could be distinguished from obstructive sleep apnea syndrome (OSAS) by clinical evaluation and symptom scores. Methods : 56 snoring and 20 asymptomatic subjects were recruited and polysomnography was used to confirm that there were 39 OSAS, 17 primary snoring, and 20 control subjects. We evaluated the size of the childrens adenoids and tonsils....
Oxygenation index as a respiratory parameter of respiratory distress syndrome in preterm infants
Ji Hyun Jeon, Ran Namgung, Min Soo Park, Kook In Park, Chul Lee
Clin Exp Pediatr. 2008;51(2):145-149.   Published online February 15, 2008
Purpose : To examine whether changes of oxygenation index (OI) by postnatal age were different by the number of surfactant administration, and different between subgroups of survival and death. Methods : From January 2005 to June 2006, preterm infants (n=84) diagnosed as respiratory distress syndrome (RDS) and treated with surfactant and ventilator were included. They were divided into two groups: Group...
Effects of puromycin aminonucleoside on the cytoskeletal changes of glomerular epithelial cells
Jun Ho Lee, Tae Sun Ha
Clin Exp Pediatr. 2008;51(1):54-61.   Published online January 15, 2008
of microvilli, but also separated the intercellular gaps and linear ZO-1. PAN induced oxidative stresses in time and dose dependent manners and increases of intercellular permeability in anti-oxidants inhibitable manners. High concentration of PAN induced not only actin polymerization and disorganization, but also the conglomerulation and internal dislocation of α-actinin protein. The intensities of fluorescences of ZO-1 protein were diminished...
Multiple extrarenal manifestations in hemolytic uremic syndrome: A case report
Eugene Kim, So-Young Kim
Clin Exp Pediatr. 2007;50(12):1261-1265.   Published online December 15, 2007
Extrarenal manifestations of hemolytic uremic syndrome (HUS) have increasingly been recognized and may be major determinants of mortality and morbidity. Although microthrombi are often found in the pulmonary and coronary circulation, apparent lung and cardiac involvement are clinically infrequent. We describe here a 10-month-old boy with HUS who developed pulmonary hemorrhage, acute respiratory distress syndrome and dilated cardiomyopathy. Complete renal...
The clinical manifestations, the short- and long-term outcomes of Bartter syndrome
Hye Won Park, Joo Hoon Lee, Young Seo Park
Clin Exp Pediatr. 2007;50(12):1231-1240.   Published online December 15, 2007
Purpose : Bartter syndrome is a renal tubular defect in electrolyte transport characterized by hypokalemia, metabolic alkalosis and other clinical signs and symptoms. The aims of this study were to analyze the clinical manifestations and the short- and long-term outcomes of Bartter syndrome. Methods : We retrospectively reviewed clinical history, laboratory finding of blood and urine, renal ultrasonography, and hearing...
Case Report
Costello syndrome: three sporadic cases
Ji Youn Kim, Mi Jeong Kim, Eun Song Song, Young Kuk Cho, Young Youn Choi, Jae Sook Ma
Clin Exp Pediatr. 2007;50(10):1024-1029.   Published online October 15, 2007
Costello syndrome (CS) is a rare multiple congenital abnormality syndrome characterized by a typical coarse face, developmental delay, psychomotor and growth retardation, neurologic abnormalities, cardiac and cutaneous anomalies, severe feeding difficulties with postnatal growth failure, and increased risk of tumors. Since Costello first described it in 1971 and again in 1977, over 100 cases have been reported worldwide. It was...
Original Article
Clinical observation of meconium aspiration syndrome and effect of suctioning through endotracheal intubation on prognosis of meconium aspiration syndrome
Yong Ho Yoon, Kyung Ah Kim, Sun Young Ko, Yeon Kyung Lee, Son Moon Shin
Clin Exp Pediatr. 2007;50(10):959-964.   Published online October 15, 2007
Purpose : The purpose was to investigate the clinical considerations of patients affected by meconium aspiration syndrome (MAS) and the effect of suctioning through endotracheal intubation immediately after delivery on the prognosis of MAS. Methods : A total of 44 inpatients diagnosed as MAS in the Neonatal Intensive Care Unit (NICU) at Cheil Hospital were selected from January 2004 to...
Review Article
Hemolytic uremic syndrome
Hye Won Park
Clin Exp Pediatr. 2007;50(10):931-937.   Published online October 15, 2007
The hemolytic uremic syndrome (HUS) is a rare disease of microangiopathic hemolytic anemia, low platelet count and renal impairment. HUS usually occurs in young children after hemorrhagic colitis by shigatoxin-producing enterohemorrhagic E. coli (D+HUS). HUS is the most common cause of acute renal failure in infants and young children, and is a substantial cause of acute mortality and morbidity; however,...
Case Report
A case of hepatopulmonary syndrome in a child with fatty liver disease secondary to hypopituitarism after craniopharyngioma resection
Sun Ju Im, Hyun Seok Park, Hyoung Doo Lee, Jae Hong Park, Hee Ju Park
Clin Exp Pediatr. 2007;50(8):794-798.   Published online August 15, 2007
Hepatopulmonary syndrome is a triad that includes: hepatic dysfunction, intrapulmonary vascular dilatation and abnormal arterial oxygenation. The incidence of intrapulmonary vascular dilatations, in adults with end-stage liver disease, has been reported to be 13% to 47%, however the incidence in children is unclear and the cases in Korean children have never been reported. The hepatopulmonary syndrome may occur as a...
Basal cell nevus syndrome (gorlin syndrome) confirmed by PTCH mutations and deletions
Hee Moon Kim, Chul Hee Lee, Sung Koo Kim, Tae Jung Sung
Clin Exp Pediatr. 2007;50(8):789-793.   Published online August 15, 2007
Basal cell nevus syndrome (BCNS), also known as Gorlin syndrome, is a rare autosomal dominant disorder characterized by variable manifestations, including multiple basal cell carcinomas, odontogenic keratocysts of the jaw, skeletal anomalies including scoliosis and bifid ribs, palmar and plantar pits, calcification of the falx cerebri, and biparietal frontal bossing. We report a case of a 9-year-old boy with the...
A case of dapsone syndrome
Yoo Jong Won, Ok Lan Kim, Seung Taek Yu, Young Wook Yoon, Du Young Choi
Clin Exp Pediatr. 2007;50(5):493-496.   Published online May 15, 2007
Diamino-diphenyl-sulfone (Dapsone) is widely used in the treatment of leprosy and a variety of blistering skin diseases. It sometimes has adverse side effects with common usual doses, such as skin, nervous system, gastrointestinal tract, liver, kidney and hematologic toxicity. One of these side effects is a rare but serious hypersensitivity reaction called dapsone syndrome, which occurs several weeks after the...
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