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Case Report
Autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome in a 10-year-old girl with ulcerative colitis
Jeana Hong, Mi Kyoung Song, Jae Sung Ko, Gyeong Hoon Kang, Woo Sun Kim, Jeong Kee Seo
Clin Exp Pediatr. 2009;52(4):504-507.   Published online April 15, 2009
Autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and primary biliary cirrhosis (PBC) are chronic autoimmune liver diseases. Overlap syndrome is defined as a condition in which the clinical, biochemical, and histological features of these autoimmune diseases are overlapped. Thus, it is difficult to appreciate overlap syndrome as an actual diagnostic entity. Only a few cases of the overlap syndrome of...
Original Article
Polymorphisms of tumor necrosis factor-alpha promotor gene in Kawasaki disease and relation to the risk of coronary artery lesion
Se-hwa Kim, Jang-won Yun, Young-hyuk Lee, Eun-jung Cheon
Clin Exp Pediatr. 2009;52(4):476-480.   Published online April 15, 2009
Purpose : The purpose of this study was to investigate the polymorphisms of the TNF-alpha promotor gene, its susceptibility to Kawasaki disease (KD) and to assess whether the TNF-alpha promotor gene polymorphism was related the risk of coronary artery lesions (CALs). Methods : From January 2003 to January 2007, 51 children (30 boys and 21 girls) with KD and 48...
Case Report
A case of antiepileptic drug hypersensitivity syndrome by lamotrigine mimicking infectious mononucleosis and atypical Kawasaki disease
Su Jung Yoo, Ihl Sung Park, Eun Sook Suh
Clin Exp Pediatr. 2009;52(3):389-391.   Published online March 15, 2009
AHS는 항경련제에 의한 부작용으로 드물게 발생하지만 생명을 위협할 수 있는 지연형 면역반응이다. 보통 항경련제 복용 후 1-8주 사이에 고열, 피부발진, 림프절병증의 임상 증세를 나타내고, 혈액, 간, 신장, 또는 폐 등의 내부 장기를 침범한다. AHS는 항경련제에 의한 부작용으로 증상이 전신적으로 다양하게 나타날 수 있다. 그리고 전염성 단핵구증이나 가와사키병과 같은 전신적 질환들과도 유사한 임상증상들을 보이기 때문에 이런 질환들과 감별하는 것이 중요하고 사망률도...
Original Article
Development of metabolic syndrome and its correlation with insulin resistance in adult patients with Turner syndrome
Joo Hwa Kim, Min Jae Kang, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2009;52(3):370-375.   Published online March 15, 2009
Purpose : The risk of metabolic syndrome (MS) and cardiovascular disease in Turner syndrome (TS) patients is high. We analyzed metabolic factors in adults with TS and evaluated the metabolic risk of insulin resistance. Methods : Forty-three adults with TS were enrolled. The frequency of MS and the values of the metabolic factors were analyzed. Patients were divided into insulin...
Clinical factors causing hyponatremia in patients with mucocutaneous lymph node syndrome
Soo Yeon Kim, Hyun Jung Kim, Jun Seok Choi, Jae Kyung Huh
Clin Exp Pediatr. 2009;52(3):364-369.   Published online March 15, 2009
Purpose : Clinical and laboratory findings predict a severe outcome for mucocutaneous lymph node syndrome. This study aimed to define the clinical characteristics of Kawasaki disease (KD) patients with hyponatremia and to determine the factors associated with its development. Methods : Retrospective studies were performed on 114 KD patients who received an initial high-dose intravenous immunoglobulin (IVIG, single 2 g/kg/dose)...
Clinical feature of neonatal pneumothorax induced by respiratory distress syndrome and pneumonia
Ji-Sun Jung, Sang-Woo Park, Chun-Soo Kim, Sang-Lak Lee, Tae-Chan Kwon
Clin Exp Pediatr. 2009;52(3):310-314.   Published online March 15, 2009
Purpose : Pneumothorax is an important factor responsible for increased mortality and morbidity in neonates. Here, we compared the clinical findings and prognosis for neonatal pneumothorax induced by respiratory distress syndrome (RDS) and pneumonia. Methods : Between January 2001 and December 2005, 80 patients with neonatal pneumothorax induced by RDS and pneumonia and admitted to the NICU of Dongsan Medical...
Case Report
Atypical presentation of Kawasaki disease resembling a retropharyngeal abscess
Eu Jin Kim, Young Su Lim, Ji Eun Yoon, Heon-Seok Han
Clin Exp Pediatr. 2009;52(2):251-255.   Published online February 15, 2009
Kawasaki disease is an acute systemic inflammatory disorder, the etiology of which has not yet been established. The clinical manifestations are non-specific and are common to many pediatric infectious and immunologic diseases. In 2 cases presenting fever, cervical lymphadenopathy, and retropharyngeal abscess-like lesions on the neck shown in a computerized tomography (CT) scan, the diagnosis of Kawasaki disease was delayed....
Atypical presentation of Kawasaki disease resembling a retropharyngeal abscess
Sun-Hee Choi, Yu-Min Lee, Yeong-Ho Rha
Clin Exp Pediatr. 2009;52(2):247-250.   Published online February 15, 2009
Stevens-Johnson syndrome (SJS) presents with widespread blisters, erythematous or purpuric macules, and one or more mucous membrane erosions. Various etiologic factors, including infection, vaccination, drug administration, systemic diseases, physical agents, and food have been implicated as causes of SJS. Mycoplasma pneumoniae is the most common infectious agent to cause SJS in children. In recent literature, M. pneumoniae-induced SJS with mucositis...
Original Article
Low T3 syndrome in Kawasaki disease: Relation to serum levels of tumor necrosis factor-α, interleukin-6 and NT-proBNP
Hye Kyung Cho, Jin A Sohn, Hae Soon Kim, Sejung Sohn
Clin Exp Pediatr. 2009;52(2):234-241.   Published online February 15, 2009
Purpose : We investigated the relationship between thyroid hormone and serum tumor necrosis factor (TNF-α), interleukin (IL-6) and N-terminal fragment of pro-brain natriuretic peptide (NT-proBNP) in patients with Kawasaki disease (KD). Methods : Serum levels of thyroid hormone, TNF-α, IL-6, and NT-proBNP were measured in 52 KD patients in the acute and subacute phase and 10 patients with acute...
Review Article
Consequences of being born small for gestational age : More than being small
Eun-Gyong Yoo
Clin Exp Pediatr. 2009;52(2):152-158.   Published online February 15, 2009
Reduced fetal growth is independently associated with increased risk of health problems in later life, particularly type 2 diabetes and cardiovascular diseases. Insulin resistance appears to be a key component underlying these metabolic complications. It is suggested that detrimental fetal environment may program insulin resistance syndrome. An insulin-resistant genotype may also result in both low birth weight and insulin resistance...
Case Report
Hand-Foot syndrome induced by sorafenib, a multitargeted tyrosine kinase inhibitor, in a patient with advanced renal cell carcinoma
Seung Hyun Lee, Sung Hun Noh, Sun Young Kim, Kyu Yun Jang, Pyoung Han Hwang
Clin Exp Pediatr. 2009;52(1):119-123.   Published online January 15, 2009
Renal cell carcinoma (RCC) arising from epithelial cells of the renal tubules is a highly aggressive and malignant tumor in all ages; however, it rarely occurs in children. the standard treatment for RCC is radical nephrectomy with lymph node dissection when the tumor is localized and can be completely resected. Adjuvant chemotherapy, radiotherapy, and immunotherapy are used for pediatric patients...
Original Article
The relationship between catechol-O-methyltransferase gene polymorphism and coronary artery abnormality in Kawasaki disease
Hyo Jin Lee, Myung Sook Lee, Ji Sook Kim, Eun Ryoung Kim, Sung Wook Kang, Soo Kang Kim, Joo Ho Chun, Kyung Lim Yoon, Mi Young Han, Seong Ho Cha
Clin Exp Pediatr. 2009;52(1):87-92.   Published online January 15, 2009
Purpose : Many gene polymorphisms are associated with coronary artery abnormalities in Kawasaki disease. Catechol-O- methyltransferase (COMT) plays an important role in the metabolism of catecholamines, catechol estrogen, and catechol drugs. Polymorphisms of the COMT gene are reported to be associated with myocardial infarction and coronary artery abnormalities. The aim of this study was to evaluate the relationship between COMT...
Case Report
Wilms` tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome: Successful treatment of the first case with bilateral Wilms` tumors in Korea
Kyung Sun Min, Hee Jo Baek, Dong Kyun Han, Ju Hee You, Tai Ju Hwang, Dong Deuk Kwon, Hoon Kook
Clin Exp Pediatr. 2008;51(12):1355-1358.   Published online December 15, 2008
Wilms` tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome is caused by deletion of chromosome 11p13, including the Wilms` tumor (WT1) and aniridia gene (PAX6) loci. Here, we report the first case of WAGR syndrome in Korea; the patient was a 2-year-old girl with bilateral aniridia from birth who presented with abdominal distention and mental retardation. Cytogenetically, she had...
Original Article
Epidemiologic study of Kawasaki disease in 6 months old and younger infants
Yong Won Park, Ji Whan Han, In Sook Park, Chang Hwi Kim, Sung Ho Cha, Jae Sook Ma, Joon Sung Lee, Tae Chan Kwon, Sang Bum Lee, Chul Ho Kim, Heung Jae Lee, Yong Soo Yun
Clin Exp Pediatr. 2008;51(12):1320-1323.   Published online December 15, 2008
Purpose : The aim of this study was to investigate the epidemiologic status of Kawasaki disease (KD) in infants ≤6 months of age. Methods : For the epidemiologic study of KD in Korea, data from 22,674 KD patients were collected from 1997 to 2005 on a 3-year basis by a retrospective survey. From this survey, data of 1,739 KD patients ≤6...
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....
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