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Original Article
Statistical Analysis of 1,000 Cases of Kawasaki Disease Patients Diagnosed at a Single Institute
Dae Hwan Hwang, Kyoung Mi Sin, Kyong Min Choi, Jae Young Choi, Jun Hee Sul, Dong Soo Kim
Clin Exp Pediatr. 2005;48(4):416-424.   Published online April 15, 2005
Purpose : To find the risk factors associated with coronory artery lesions, non-responsiveness to intravenous immunoglobulin(IVIG) treatment, and recurrences in Kawasaki disease patients. Methods : We retrospectively analyzed 1,000 Kawasaki disease patients who were admitted to Yonsei University Medical Center from September 1990 to December 2003. We compared between responder and non-responder groups to IVIG treatment as well as between relapsed...
Case Report
A Case of Multiple Giant Coronary Aneurysms with Large Mural Thrombus due to Kawasaki Disease in a Young Infant
Eun Na Choi, Jeoung Tae Kim, Yuria Kim, Byung Won Yoo, Deok Young Choi, Jae Young Choi, Jun Hee Sul, Sung Kye Lee, Dong Soo Kim, Young Hwan Park
Clin Exp Pediatr. 2005;48(3):321-326.   Published online March 15, 2005
Kawasaki disease is an acute systemic vasculitis of unknown origin. Giant coronary aneurysm is one of the most serious complications, although peripheral artery vasculitis can produce life-threatening events. Myocardial ischemia and infarction can be caused by coronary artery stenosis, aneurysm, and stagnation of blood flow in coronary arteries which triggers thromboembolism. Atypical presentation in young infants often interferes with prompt...
Original Article
Factors Affecting Final Adult Height in Patients with Turner Syndrome
Jae Hyun Kim, Sung Soo Lee, Su Young Hong, Hye Rim Chung, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2005;48(2):191-196.   Published online February 15, 2005
Purpose : Short stature is one of the characteristic features of Turner syndrome. We investigated the factors affecting final adult height(FAH) in patients with Turner syndrome. Methods : The study group was comprised of 60 patients who were diagnosed with Turner syndrome by chromosomal study and clinical phenotypes and attained FAH. Data were obtained from retrospective review of the medical records....
Serum Albumin Concentrations and Clinical Disorders by Gestational Ages in Preterm Babies
Mia Lee, Soo Youn, Baek Keun Lim, Jong Soo Kim
Clin Exp Pediatr. 2005;48(2):148-153.   Published online February 15, 2005
Purpose : The aim of this study was to determine the reference ranges of serum albumin levels depending on the gestational ages of preterm infants. We also intended to compare the mean serum albumin levels between groups of preterm infants that did not develop clinical disorders later, and groups that did develop clinical disorders such as respiratory distress syndrome, intraventricular...
Case Report
A Case of Pyomyositis and Toxic Shock Syndrome Caused by Methicillin Resistant Staphylococcus aureus
Yang Kyong Kim, Dal Hyon Kim, Soon Ki Kim, Byong Kwan Son, Young Jin Hong
Clin Exp Pediatr. 2005;48(1):88-92.   Published online January 15, 2005
Pyomysitis is a primary acute bacterial infection of large skeletal muscule, usually occuring in the absence of specific cause of infection. Pyomyositis has been reported mainly in tropical countries and was rare in temperate climates. but it has been recognized with increasing frequency. Toxic shock syndrome(TSS) is an acute mutisystemic disease characterized by high fever, hypotension, multisystem dysfunction and erythematous...
A Case of Rothmund-Thomson Syndrome with Pure Red Cell Aplasia, Autoimmune Hemolytic Anemia and Chronic Respiratory Infection
Jung Hyun Lee, Eun Seok Roh, Yoo Rah Hong, Jae Sun Park, Ghi Seok Seo, Bang Hur, Mi Hyang Kim
Clin Exp Pediatr. 2004;47(12):1351-1355.   Published online December 15, 2004
Rothmund-Thomson syndrome(RTS), or poikiloderma congenita, is a rare, multisystem disorder. It is inherited genetically as an autosomal recessive trait, occurring predominantly in females(1.4 : 1). The RTS is comprised of poikiloderma, short stature, sparse hair, juvenile cataracts, skeletal defects, dystrophic teeth and nails, photosensitivity, and hypogonadism. We report a case of RTS who died of bleeding from esophageal varices, pulmonary...
A Case of Mycoplasma pneumoniae Pneumonia Complicated with Guillain-Barr Syndrome and Encephalitis
Soon Bum Lee, Hee Jung, Yong Seok Lee, Bum Sun Kwon, Jeesuk Yu
Clin Exp Pediatr. 2004;47(12):1338-1348.   Published online December 15, 2004
The most common pathogen of respiratory tract infection among school-age children and adolescents is Mycoplasma pneumoniae, which causes clinical manifestations of pneumonia, acute asthmatic attack, pharygitis, and tonsilitis. It can also cause extrapulmonary infections that involves skin, the nervous system, the digestive system, the cardiovascular system, and the hematopoietic system. It is reported that the central nervous system symptoms may...
A Case of Jarcho-Levin Syndrome with Intrathoracic Kidney
Shin Yun Byun, Myoung Hwa Sung, Jung Mi Choi, Tae Hong Kim, Kyu Geun Hwang, Jin A Jung
Clin Exp Pediatr. 2004;47(11):1225-1227.   Published online November 15, 2004
In 1938, Jarcho and Levin initially described shortening of the trunk, prominent occiput, broad forehead, multiple vertebral defects and ribs anomaly, short neck, increased anteroposterior chest diameter, lordosis, kyphoscoliosis. After that, Jarcho-Levin syndrome is an eponym that has been used to describe a variety of clinical phenotypes. We examined a girl who was suspected as suffering from Jarcho-Levin syndrome because...
A Case of Fetal Alcohol Syndrome with Persistent Pulmonary Hypertension of the Newborn
Jin-Ha Chang, Ran Namgung, Min-Soo Park, Kook In Park, Jin-Sung Lee, Chul Lee
Clin Exp Pediatr. 2004;47(11):1220-1224.   Published online November 15, 2004
Fetal alcohol syndrome can be suspected in infants born to mothers with a prenatal history of alcohol abuse if the child exhibits characteristic facial features, together with intrauterine growth retardation, multiple neurological abnormalities, and multiorgan defects. If only a few of the above criteria are satisfied, the term fetal alcohol effects is used. We experienced a neonate who presented with...
A Case of Weaver Syndrome
Jun Chul Byun, Chun Soo Kim, Sang Lak Lee, Tae Chan Kwon, Hee Jung Lee
Clin Exp Pediatr. 2004;47(11):1216-1219.   Published online November 15, 2004
Weaver syndrome is a very rare overgrowth syndrome with accelerated skeletal maturation, unusual facies and clinodactyly. We experienced an extremely rare case of Weaver syndrome in Korea. The clinical manifestations were macrocephaly, unusal facies, simian crease, clinodactyly, decreased muscle tone, accelerated bone age and congenital heart diseases. We report this with a brief review of related literature.
Original Article
Prevalence of Metabolic Syndrome in Obese Children
Jin Ha Chang, Duk Hee Kim, Ho-Seong Kim, In Kyoung Choi, My Young Cheong, Dong Kee Kim
Clin Exp Pediatr. 2004;47(11):1149-1156.   Published online November 15, 2004
Purpose : Obesity has been known to be a cause of insulin resistance and dyslipidemia, and along with coronary artery disease and diabetes, is associated with metabolic syndrome(MS). This study aimed to ascertain the cause and prevalence of MS in obese children and adolescents. Methods : Two hundred and seventy-seven school children, who showed more than a 95th percentile of body...
Methylenetetrahydrofolate Reductase C677T and A1298C Polymorphisms and Risk of Down Syndrome
Kyu Young Chae, Jin Hee Han, Ji Yeong Seo, Min Jung Cho, Sehyun Kim, Nam Keun Kim
Clin Exp Pediatr. 2004;47(10):1053-1057.   Published online October 15, 2004
Purpose : The C677T polymorphism of the methylenetetrahydrofolate reductase(MTHFR) has been suggested as a risk factor of maternal meiotic nondisjunction for Down syndrome. Recently, a second genetic polymorphism in MTHFR at position 1298 was reported. However, a positive association between the A1298C MTHFR polymorphism and Down syndrome has not been reported. Therefore, this study was undertaken to determine which polymorphism...
Case Report
Isolated Intracranial Granulocytic Sarcoma as a Relapse Following Unrelated Bone Marrow Transplantation for Myelodysplastic Syndrome in a 1 Year-Old Infant
Kyung Ran Son, Hoon Kook, So Youn Kim, Hee Jo Back, Seok Joo Kim, Ha Young Noh, Mi Jeong Kim, Ic Sun Choi, Shin Jeong, Jong Hee Nam, Tai Ju Hwang
Clin Exp Pediatr. 2004;47(9):1008-1012.   Published online September 15, 2004
Isolated relapse of myeloid leukemia as a granulocytic sarcoma(GS) following allogeneic bone marrow transplantation(BMT) is very rare manifestation, and usually associated with a poor prognosis. We report a case of isolated intracranial GS in an infant with myelodysplastic syndrome(MDS) following unrelated BMT. A 7 month-old girl was diagnosed with refractory anemia with excess blasts (RAEB). During observation for a couple...
Original Article
Surfactant Therapy for Neonatal Respiratory Distress Syndrome : Experience in Korea Over 15 Years
Chong-Woo Bae, Young-Min Kim
Clin Exp Pediatr. 2004;47(9):940-948.   Published online September 15, 2004
Purpose : Surfactant replacement therapy in neonates with respiratory distress syndrome(RDS) has been used in Korea since 1990. We undertook a collective study of trials from multi-hospitals to evaluate results of neonates with RDS who were treated with surfactant during the past 15 years in Korea(Period I, P-I, 1990-91; Period II, P-II, 1996; Period-III, P-III; 2002). Methods : There were 60...
Case Report
A Case of Shwachman-Diamond Syndrome
Jeong Won Kwak, Suk Kim, Young Tak Lim
Clin Exp Pediatr. 2004;47(8):900-903.   Published online August 15, 2004
Shwachman-Diamond syndrome(SD syndrome) is a rare genetic disorder chracterized by pancreatic insufficiency, short stature, skeletal abnormalities and bone marrow dysfunction. Exocrine pancreatic insufficiency and neutropenia are the main components of the syndrome. A hallmark of SD syndrome is varying severity of pancreatic dysfunction due to acinar maldevelopment. The hematologic abnormalities associated with SD syndrome include varying cytopenias, marrow aplasia, myelodysplasia...
Two Cases of Rotor Syndrome in Siblings
Hwa-Jin Park, Eun-Sung Kim, Ji-Young Chung, Sung-Ho Cha, Deog-Yoon Kim
Clin Exp Pediatr. 2004;47(8):892-895.   Published online August 15, 2004
Rotor syndrome is a rare benign familial disorder characterized by chronic, fluctuating, nonhemolytic and predominantly direct bilirubinemia with normal liver tissue. We have recently experienced two cases of Rotor syndrome in a brother and sister. They revealed icteric sclerae with mild hepatomegaly in physical examination. Laboratory findings showed increased serum bilirubin with direct bilirubin predominance. The urinary excretion of total...
Bronchiolitis Obliterans Associated with Stevens-Johnson Syndrome
Ko Un Chun, Soo Yun, Byung Ho Cha, Hae Yong Lee
Clin Exp Pediatr. 2004;47(7):803-806.   Published online July 15, 2004
Bronchiolitis Obliterans(BO) is one of the rare inflammatory lung disease that primarily affects bronchi and bronchioles, followed by partial or complete obstruction. In children, the etiology of BO has been under consideration, but infections and toxic inhalation, connective tissue diseases, lung transplantation, congestive pulmonary edema, etc., may be causes of BO. Even though steroid therapy was reported as an effective...
A Case of CATCH22 Syndrome with First Attack of Hypocalcemic Seizure at 13 Years of Age
Young Won An, Mi Jin Jung, Jee Suk Yu, Young Seok Lee, Han Wook Yoo
Clin Exp Pediatr. 2004;47(7):794-798.   Published online July 15, 2004
The acronym 'CATCH22' is characterized by many clinical manifestations such as cardiac defects, abnormal face, thymic and parathyroid hypoplasia, cleft palate and hypocalcaemia. It is now known to arise from chromosome 22q11.2 microdeletion, and it is also called 22q11.2 deletion syndrome. Hypocalcemia occurs in more than 50% cases of this syndrome, most frequently in neonatal periods, with some exceptions. Our...
Original Article
Methylenetetrahydrofolate Reductase(MTHFR) Gene Expression in Kawasaki Disease
Hye Ryung Choi, Ae Ra Joo, Hae Soon Kim, Sejung Sohn, Young Mi Hong
Clin Exp Pediatr. 2004;47(7):774-778.   Published online July 15, 2004
Purpose : Hyperhomocysteinemia is known as an independent risk factor for cardiovascular diseases such as atherosclesosis and myocardiac infarct. A common mutation in 5, 10-methylenetetrahydrofolate reductase(MTHFR) gene results in a valine for alanine substitution, which makes enzyme thermolabile and reduces enzymal activity. We examined the relation of MTHFR genetic mutation and Kawasaki disease. Methods : We extracted DNA from the peripheral...
Association between the Human Surfactant Protein-A(SP-A) Gene Locus and Respiratory Distress Syndrome in Korean Neonates
Jae Hoon Choe, Myung Ho Oh, Jung Ho Ko, Sun Young Kim, In Kyu Lee, Chong Woo Bae
Clin Exp Pediatr. 2004;47(7):735-739.   Published online July 15, 2004
Purpose : Respiratory distress syndrome(RDS) is caused by a deficiency of pulmonary surfactant, which is a lipoprotein complex. Both low levels of surfactant protein A(SP-A) and SP-A alleles have been associated with RDS. However, the genes underlying susceptibility to RDS are insufficiently known. The candidate-gene approach was used to study the association between the SP-A gene locus and RDS in...
Diagnostic Approach of Wiskott-Aldrich Syndrome
Dong Jin Hwang, Jun Won Yang, Sun Young Kim, Ho Keun Yi, Dae Yeol Lee, Pyoung Han Hwang
Clin Exp Pediatr. 2004;47(7):726-734.   Published online July 15, 2004
Objective : A probable diagnosis of Wiskott-Aldrich syndrome(WAS) should be considered in any boy presenting with unusual bleeding, congenital thrombocytopenia and small platelets. The definitive diagnosis of WAS is usually made by the detection of WASP gene mutation or a decrease or absence of the WAS protein(WASP) in blood cells using molecular genetic analysis. However, these methods are too time-consuming...
Matrix Metalloproteinases, Tissue Inhibitors and Cytokines in Patients with Kawasaki Disease
Ae Ra Cho, Young Mi Hong
Clin Exp Pediatr. 2004;47(6):656-664.   Published online June 15, 2004
Purpose : Kawasaki disease(KD) is a multisystemic inflammatory vasculitis of unknown etiology, but immunological abnormalities have been documented and implicated in the pathogenesis of KD. Matrix metalloproteinases(MMPs) have proteolytic activity against connective tissue proteins, and increased activity of MMPs and a quantitative imbalance between MMP and tissue inhibitor of MMP (TIMP) can result in several pathologic conditions. MMP and TIMP...
Comparison of Cytokine Expressions among Kawasaki Disease and Its Symptom-related Diseases
Ran Lee, So Hyun Park, Yu Jeong Kim, So Young Kim, Hyun Hee Kim, Wonbae Lee
Clin Exp Pediatr. 2004;47(5):567-573.   Published online May 15, 2004
Purpose : Kawasaki disease(KD) is an important acute febrile systemic vasculitis disease. Various cytokines have been studied to evaluate the pathogenesis of vascular injury. Some cytokines have been implicated for vascular injury, but there has been no direct evidence. We compared cytokine profiles of KD with Henoch-Sch nlein purpura(HSP), and febrile infections. Methods : We investigated the serum concentrations of...
Cause and Prognosis of Pediatric Acute Respiratory Failure by Intrapulmonary Lesion
Hye-Young Lee, Ji-Suk Park, Hee-Ra Choi, Ji-Hyun Seo, Jeum-Su Kim, Jae-Young Lim, Myoung-Bum Choi, Chan-Hoo Park, Hyang-Ok Woo, Hee-Shang Youn
Clin Exp Pediatr. 2004;47(5):555-560.   Published online May 15, 2004
Purpose : Respiratory failure arises from derangements in pulmonary gas exchange. The causes may be classified as due to either lung disease or respiratory pump dysfunction. Problems with lung mechanics is an important cause of acute respiratory failure in children clinically. The aims of this study were to survey the cause and prognosis of children diagnosed with acute respiratory failure...
Prevention of Chronic Lung Disease with Early Dexamethasone Treatment in less than 32 Weeks Premature : Randomized Controlled Study
So Yun Shim, Su Jin Cho, Eun Ae Park
Clin Exp Pediatr. 2004;47(5):521-526.   Published online May 15, 2004
Purpose : The optimal timing and the consequences of dexamethasone therapy in chronic lung disease remain unknown. The purpose of this study was to determine whether early dexamethasone therapy would reduce the incidence of chronic lung disease and to determine the adverse effects and complications of prematurity associated with such therapy. Methods : Twenty neonates with hyaline membrane disease(dexamethasone n=10, placebo...
The Effect of Post-natal Weight Changes on the Development of Bronchopulmonary Dysplasia in Infants with Respiratory Distress Syndrome
Hye One Jhon, Young Min Kim, Hee Joo Hong, Chong Woo Bae
Clin Exp Pediatr. 2004;47(3):269-274.   Published online March 15, 2004
Purpose : The subjects were compared and measured for differences in the aspects of weight changes between the groups that developed bronchopulmonary dysplasia(BPD) after treatment and those that did not, to analyze the relations between the patterns of early weight change after birth and the development of BPD. Methods : Retrospective analysis of medical records were performed for birth weight and...
The Effect of Histologic Chorioamnionitis on the Development of Respiratory Distress Syndrome and Chronic Lung Disease in Preterm Infants
Heui seung Jo, Beyong Il Kim, Chang Won Choi, Jun Dong Park, Chong Jai Kim, Jung-Hwan Choi
Clin Exp Pediatr. 2004;47(2):150-156.   Published online February 15, 2004

Purpose: Histologic chorioamnionitis may play a role in the development of respiratory distress syndrome(RDS) and chronic lung disease(CLD) independently or through its association with preterm birth. We investigated the relationship between histologic chorioamnionitis and clinical complications including, RDS and CLD, of preterm infants. Methods: Clinical data were collected retrospectively from 478 preterm infants(gestational period≤34 weeks) who were admitted to the neonatal...
Case Report
Atypical Hemolytic Uremic Syndrome Associated with Streptococcus pneumoniae Infection
Hyun Sug Lee, Ja Wook Koo, Sang Woo Kim, Hyun Soon Lee
Clin Exp Pediatr. 2004;47(2):217-222.   Published online February 15, 2004
Hemolytic uremic syndrome is the most common cause of acute renal failure in childhood. Atypical hemolytic uremic syndrome which is not usually associated with prodromal symptoms has a higher mortality rate and more tendency to progress to chronic renal failure. Children younger than two years of age are a more susceptible to pneumococcal infection and can develop more serious disease....
Original Article
Pharmacokinetics of Cyclosporine A and Its Therapeutic Effect in Children with Renal Diseases
Woo Sung Chun, Min-Soo Park, Jae Seung Lee, Juyun Yu, Moon Sung Park, Ki-Soo Pa
Clin Exp Pediatr. 2004;47(2):193-203.   Published online February 15, 2004
Purpose : To know the body handling properties and anti-proteinuric effect of cyclosporine A(CsA) in children with renal diseases, 34 patients with nephrotic syndrome or glomerular diseases were included to treatment trials and evaluated. Methods : Microemulsion formula CsA, 5 mg/kg/day was administered orally in two divided doses for 9.3?.6 months. Pharmacokinetic studies of CsA were done twice at beginning and...
Case Report
Four Cases of Staphylococcal Scalded Skin Syndrome
Chul Han Park, Yoo Jin Jeong, Won Jung Choi, Sat Byul Park, Heung Sik Kim, Chin Moo Kang
Clin Exp Pediatr. 2004;47(1):106-110.   Published online January 15, 2004
Staphylococcal scalded skin syndrome is a systemic disease with a clinical spectrum ranging from subcorneal pustules, patterned exfoliation to extensive erosion and peeling of skin by the exfoliative toxin of group II Staphylococcus aureus. This disease occurs mainly in infancy and children below five years and it isn't easy to differentiate from other vesicular diseases clinically, but skin biopsy shows...
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