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Case Report
A Case of Protein Losing Enteropathy Associated with Henoch-Sch nlein Purpura
Kee Dae Kim, Chang Whan Oh, Eun Young Lee, Jae Young Kim
Clin Exp Pediatr. 2005;48(2):224-227.   Published online February 15, 2005
Henoch-Sch nlein purpura(HSP) is an IgA mediated immune complex vasculitic disease characterized by non-thrombocytic purpura, arthritis, gastrointestinal manifestations, and glomerulonephritis. HSP related glomerulonephritis induces hypoproteinemia and edema in some cases. Protein-losing enteropathy is another rare but known manifestation of HSP leading to hypoproteinemia and edema. We report a 6-year-old girl with HSP who showed edema caused by intestinal protein loss,...
Original Article
Gene Expression of Metalloproteinases, Tissue Inhibitors of Metalloproteinases and Cytokines in Adriamycin-induced Cardiomyopathy
Young Mi Hong
Clin Exp Pediatr. 2005;48(2):197-203.   Published online February 15, 2005
ity has been reported to occur in both clinical and experimental forms of dilated cardiomyopathy. There was no report about MMP in adriamycin(ADR)-induced cardiomyopathy. The purpose of this study was to investigate gene expression of MMP and tissue inhibitor of metalloproteinases(TIMP) in ADR-induced cardiomyopathy and clarify the relationship between MMP and cytokines. Methods : Male Sprague-Dawley rats were divided into two...
Serum and Cerebrospinal Fluid(CSF) Nitric Oxide, Macrophage Inflammatory Protein-1α and Lactoferrin Levels in Aseptic Meningitis
Young Seo, Jung Yeon Shim, Jae Won Shim, Deok Su Kim, Hye Lim Jung, Moon Soo Park
Clin Exp Pediatr. 2005;48(1):48-54.   Published online January 15, 2005
Purpose : The pathologic mechanisms of central nervous system(CNS) injuries in human meningitis are not yet completely understood. Recent studies indicate that the host inflammatory responses are as important in brain damage as the infecting organisms and toxins. There have been some reports on the relationship of nitric oxide(NO), macrophage inflammatory protein-1α(MIP-1α), and lactoferrin in bacterial meningitis, but few reports...
The Tumor Suppressor Function of PTEN/MMAC1 through the Regulation of IGFs and IGFBPs
Ho Keun Yi, Dong Jin Hwang, Sun Young Kim, Dae Yeol Lee, Pyoung Han Hwang
Clin Exp Pediatr. 2004;47(8):884-891.   Published online August 15, 2004
Purpose : PTEN/MMAC1, a novel tumor suppressor gene, is mutated in a variety of advanced and metastatic cancers. It acts as a phosphatase, and thereby, regulates the PI-3 kinase/Akt pathway. In this study, we examined to evaluate the new function of anti-tumor effects of PTEN/MMAC1 through the regulation of the IGFs-IGFBPs in gastric cancer cells. Methods : PTEN/MMAC1 was expressed in...
Protein C as a Differential Marker for Bacterial Infection among Pediatric Patients with Fever
Eun Ha Mo, In Hae Nam, Kyung Duk Park
Clin Exp Pediatr. 2004;47(8):839-843.   Published online August 15, 2004
Purpose : This study was performed to find the clinical significance in protein C as a differential marker in the beginning stage of infection and prognosis factor in severe infection among pediatric patients who were admitted due to fever. Methods : A total of 40 pediatric patients who had temperatures higher than 37.5℃ on admission at the Department of Pediatrics,...
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...
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...
Case Report
Angiotensin Converting Enzyme Inhibition Modulates Mitogen Activated Protein Kinase Family Expressions in the Neonatal Rat Kidney
Byung Min Choi, Mee-Hye Oh, Kee Hwan Yoo
Clin Exp Pediatr. 2004;47(3):325-331.   Published online March 15, 2004
Purpose : In a developing kidney, the renin-angiotensin system(RAS) is markedly activated and is thought to play an important role in postnatal renal growth and maturation. We previously demonstrated that angiotensin converting enzyme(ACE) inhibition in a developing rat kidney increases apoptosis and decreases its related gene expressions, which may account for renal growth impairment. Among the mitogen-activated protein kinases(MAPK) family...
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...
Serum ECP, RANTES and Eotaxin Levels in Infants with Bronchiolitis
Jong Seo Yoon, Mi Hee Lee, Joon Sung Lee
Clin Exp Pediatr. 2004;47(2):170-176.   Published online February 15, 2004
Purpose : Viral respiratory infection, especially respiratory syncytial(RS) virus, can predispose sufferers to the development of asthma by mechanisms that are presently undetermined. The role of inflammatory mediators in pathogenesis of asthma and RS virus disease is not well-understood. We investigated the performance of serum eosinophil cationic protein(ECP), regulated on activation normal T-cell expressed and secreted(RANTES) and eotaxin levels on...
Prediction of Intravenous Immunoglobulin Non-responders in Patients with Kawasaki Disease
Gi Bum Lee, Ji-Won Lee, Kyung-Yil Lee
Clin Exp Pediatr. 2004;47(1):90-94.   Published online January 15, 2004
Purpose : We evaluated the effects of intravenous immunoglobulin(IVIG) on the levels of laboratory indices examined serially according to the responsiveness to IVIG therapy in children with Kawasaki disease(KD). Methods : Children with KD(n=63) who had been treated with IVIG at a dosage of 2.0 g/kg were classified into two groups : the IVIG-resistant(consistent fever over 48 hours after initiation...
Peripheral Blood Eosinophil Counts and Serum ECP in Adolescents with Long-term Asthma Remission and Persistent Bronchial Hyperresponsiveness : Comparison with Adolescents with Symptomatic Asthma
Hee Kang, Young Yoo, Jinho Yu, Yang Park, Young Yull Koh
Clin Exp Pediatr. 2003;46(10):1013-1018.   Published online October 15, 2003
Purpose : Bronchial hyperresponsiveness(BHR) in asthma is thought to be a consequence of underlying airway inflammation. But the mechanism responsible for persistent BHR in adolescents with long-term asthma remission is poorly understood. The aim of this study was to examine whether BHR in adolescents with asthma remission is associated with peripheral blood eosinophilia and/or increased serum levels of eosinophil cationic...
Alteration of Biochemical Profiles after High-Dose Intravenous Immunoglobulin Administration in Kawasaki Disease
Ji-Won Lee, Kyung-Yil Lee
Clin Exp Pediatr. 2003;46(8):817-820.   Published online August 15, 2003
Purpose : Intravenous immunoglobulin(IVIG) has been used as an immunomodulatory treatment for several immune-mediated diseases. The early effect of high-dose IVIG on biochemical profiles including lipids and proteins was evaluated in patients with Kawasaki disease(KD). Methods : Twelve children with KD(nine boys) were treated with IVIG of 2 g/kg over 12 hours. Serial sera were collected from the patients four times...
Differential Excretion of Urinary Eosinophil Protein X after Methacholine Challenge Test in Children with Asthma
Su-A Shin, Jae-Won Oh, Ha-Baik Lee
Clin Exp Pediatr. 2003;46(5):495-499.   Published online May 15, 2003
Purpose : Eosinophil is one of the important inflammatory cell involved in the airway inflammation in childhood asthma. It has been demonstrated that markers of eosinophil activation, including eosinophil cationic protein or eosinophil protein X(EPX), are increased in childhood asthma. Furthermore, they are related to disease activity and are assumed to be helpful in monitoring the treatment effect as urinary...
Clinical Significance of C-reactive Protein in Measles
Sang-Lim Choi, Kyung-Yil Lee, Hyung-Shin Lee, Ja-Hyun Hong, Kyung-Tai Whang
Clin Exp Pediatr. 2003;46(5):480-483.   Published online May 15, 2003
Purpose : We evaluated clinical manifestations and laboratory findings in patients with measles according to C-reactive protein(CRP) concentration. Methods : A retrospective analysis was performed using the medical records of patients with measles at The Catholic University of Korea, Daejeon St. Mary's Hospital from October 1999 to May 2000. We divided the patients with measles into four groups according to CRP...
Differential Response of Surfactant Protein-A Genetic Variants to Dexamethasone Treatment
Eul Soon Kim, In Kyu Lee, Myung Ho Oh, Chong Woo Bae
Clin Exp Pediatr. 2003;46(4):335-339.   Published online April 15, 2003
Purpose : Surfactant protein A(SP-A) is involved in surfactant physiology and structure, and plays a major role in innate host defense and inflammatory processes in the lung. Steroid therapy is widely used for mothers who threaten to deliver prematurely and also used commonly in the management of preterm infants with chronic lung disease. Two SP-A genes(SP-A1, SP-A2) and several alleles...
The Usefulness of Spot Urine Protein/Creatinine Ratio in Evaluating Proteinuria in Children and the Correlation between 24-hour Urinary Protein Amount and Spot Urine Protein/Creatinine Ratio
Seon Young Hong, Ji Young Kim, Woo Yeong Chung
Clin Exp Pediatr. 2003;46(2):173-177.   Published online February 15, 2003
Purpose : Recently, different results about factors affecting accurate quantitation of 24-hr urinary protein(24UP) amount using spot urine protein/creatinine ratio(PCR) have been reported. The current study was designed to evaluate correlation between 24UP amounts and PCR in children, and the effect of 24UP amounts, age, sex, and glomerular filtration rate(GFR) on this correlation. Methods : Among 94 patients who visited the...
Blood Eosinophil and Serum Eosinophil Cationic Protein as a Marker of Bronchial Hyperresponsiveness in Children with Suspected Asthma
Yang Park, Hee Kang, Eun Kyeong Kang, Young Yull Koh
Clin Exp Pediatr. 2002;45(12):1577-1584.   Published online December 15, 2002
Purpose : Airway inflammation is considered to be a characteristic feature of asthma, and eosinophils are recognized as the most important inflammatory cells. This study aims to assess the importance of blood eosinophil count and serum eosinophil cationic protein(ECP) levels as a non- invasive marker of bronchial hyperresponsiveness(BHR) in children with suspected asthma. Methods : This study used data from 87...
Serological Analysis and Epidemiologic Characteristics of Group A Streptococci in Seoul(1998-2000)
Sun-Hee Choi, Yun-Ho Kim, Sung-Ho Cha, Ki-Sang Kim, Young-Hee Lee
Clin Exp Pediatr. 2002;45(11):1368-1372.   Published online November 15, 2002
Purpose : Group A streptococci have a cell wall which consists of M protein and T protein. T protein is known to be helpful in the understanding of the epidemiology of group A streptococci. To study the epidemiologic characteristics, we serotyped T protein of group A streptococci obtained from patients admitted to hospitals, or who visited OPD in five districts...
Effect of Xanthine Oxidase Inhibitor on Cerebral Hypoxia-Ischemia in Neonatal Rats
Dae-Ho Choi, Yeon-Kyun Oh, Seung-Tak Park
Clin Exp Pediatr. 2002;45(6):732-742.   Published online June 15, 2002
Purpose : In order to evaluate the hypoxia-ischemia(H-I) induced neurotoxicity and the protective effect of xanthine oxidase(XO) inhibitor(allopurinol), cell number, cell viability, lactate dehydrogenase(LDH), protein synthesis(PS) and protein kinase C(PKC) activity were measured in cerebral neurons and astrocytes. Methods : Cytotoxic effect was measured by in vitro assay at 12-72 hours after H-I on cerebral neurons and astrocytes derived from 7-day...
Case Report
A Case of Rett Syndrome with MECP2 Gene Mutation
Jin Kyung Kim, Chang Seok Ki, Jong Won Kim
Clin Exp Pediatr. 2002;45(4):540-544.   Published online April 15, 2002
Rett syndrome is an X-linked dominant, progressive neurodevelopmental disorder, with a prevalence estimated to be one in 10,000-15,000 girls, which is thought to be the second most common genetic causes of mental retardation in females after Down syndrome. Patients with classic Rett syndrome show an apparently normal neonatal period, followed by developmental regression and deceleration of head growth, accompanied by...
Original Article
Clinical Study of the Correlation of Tumor Necrosis Factor Alpha and the Proteinuria of Henoch-Schönlein Nephritis and Idiopathic Nephrotic Syndrome
Dong-Ho Jeong, Jeong-Hyun Park, Hye-Cheon Jeong, Hyun-Hoe Koo, Jun-Ho Lee, Tae-Sun Ha
Clin Exp Pediatr. 2002;45(2):240-246.   Published online February 15, 2002
Purpose : It is not clear that the development of glomerular injury and aggravation by tumor necrosis factor alpha(TNF-α) is related to intrarenal or serum concentration of TNF-α. So, we studied the relationship between the concentration of TNF-α and aggravation of glomerular damage in the Henoch-Schönlein nephritis(HSN) and idiopathic nephrotic syndrome(INS). Methods : We collected the sera and urines of...
Conversion of Total Atrio-pulmonary Connection to Total Cavo-pulmonary Connection - Review of Indications and Hemodynamic Characteristics -
Jung Ho Seo, Jong Kyun Lee, Jae Young Choi, Jun Hee Sul, Sung Kyu Lee, Young Whan Park, Bum Koo Cho
Clin Exp Pediatr. 2002;45(2):199-207.   Published online February 15, 2002
Purpose : Since the successful application of total atrio-pulmonary connection(TAPC) to patients with various types of physiologic single ventricles in 1971, post-operative survival rates have reached more than 90%. However some patients have been shown to present with late complications such as right atrial thrombosis, atrial fibrillation and protein losing enteropathy eventually leading to re-operation to control the long-term complications....
Changes of Body Weight and Lipid Profiles According to Apolipoprotein E Polymorphism in Children with Antiepileptics
Seung Hee Jang, Eun Kyoung Choi, So Hee Eun, Sun Jun Kim
Clin Exp Pediatr. 2002;45(1):88-94.   Published online January 15, 2002
Purpose : Weight changes, especially weight gain, is a side effect of antiepileptics(especially valproate and carbamazepine). This may be sufficiently severe to cause noncompliance or to require the withdrawal of effective treatment. Unfortunately, the exact mechanism of weight change is not illustrated. Several reports and our experiment suggested that weight gain highly correlated with a familial tendency of obesity. The genetic makeup is a...
Clinical Features of Food Protein-Induced Enterocolitis Syndrome
Jeong Sik Seo, Hai Lee Chung
Clin Exp Pediatr. 2001;44(7):758-763.   Published online July 15, 2001
Purpose : Food protein-induced enterocolitis syndrome(FPIES) is a symptom complex of severe vomiting and diarrhea which is known as a food-related gastrointestinal hypersensitivity disorder without evidence of IgE-mediated sensitivity. We described the clinical characteristics of FPIES in young infants. Methods : Twenty-eight patients, aged 7 to 120 days, were included who were diagnosed as FPIES by clinical criteria and food challenges....
Case Report
A Case of Protein-losing Enteropathy in Congestive Heart Failure : Resolution with Prednisolone
Jong-Woon Choi
Clin Exp Pediatr. 2001;44(5):587-591.   Published online May 15, 2001
Protein-losing enteropathy can result from primary or secondary intestinal lymphangiectasia. Secondary intestinal lymphangiectasia develops as a result of lymphatic obstruction or elevated lymphatic pressure. Cardiac lesions such as constrictive pericarditis, post-Fontan procedure state, and chronic congestive heart failure increase lymphatic pressure and may cause intestinal lymphangiectasia and protein-losing enteropathy. An 11-year-old girl who underwent corrective surgery for Tetralogy of Fallot...
Original Article
Identification of Novel Mutations and Three Most Common Mutations in the Human ATP7B Gene of Korean Patients with Wilson Disease
Han-Wook Yoo, Gu-Hwan Kim, Ji-Won Chung, Chang-Yeon Lee, Kyung-Mo Kim
Clin Exp Pediatr. 2001;44(5):569-576.   Published online May 15, 2001
Purpose : Wilson disease is an autosomal recessive disorder of copper transport, which is probably the most common inherited metabolic disorder in Korea. It is characterized by defective biliary excretion of copper and impairment in the corporation of copper into ceruloplasmin. In Wilson disease, synthesis of a defective copper transporting enzyme leads to the accumulation of copper in the liver, brain and kidney. The...
A Study of Surface Physical Properties of New Surfactant Using Synthetic Peptides of Surfactant Protein-B
Chong-Woo Bae, Yong-Mook Choi, Joo Hyun Kang, Sang-Chul Lee, Kil Lyong Kim, Kyung-Soo Hahm
Clin Exp Pediatr. 2000;43(8):1059-1067.   Published online August 15, 2000
Purpose : To produce a new generation of artificial pulmonary surfactant(PS), surfactant protein (SP)-B from human PSwas isolated, and the amino acid sequences of these proteins were studied. Artificial peptides of human SP-B were synthesized. New artificial PS preparations which were composed of phospholopids and two artificial synthetic SP-B peptides were made, and the surface physical properties of these new PS preparations were tested. Methods...
Insulin-like Growth Factor(IGF)-I and IGF-Binding Protein-3 in Relation to Hemoglobin Concentration in Healthy Infants
Yoon Ki Kang, Dae-Yeol Lee
Clin Exp Pediatr. 2000;43(7):967-973.   Published online July 15, 2000
Purpose : Insulin-like growth factor(IGF-I) and IGF binding protein(IGFBP)-3 is thought to play an important role in fetal erythropoiesis. The objective of this study was to establish a relation between IGF-I, free IGF-I, IGFBP-1, and -3 with hemoglobin level in healthy term, 3-month, and 12-month old infants. Methods : Healthy term infants(n=20) were enrolled at birth, as well as 3 months(n=30) and 12 months(n=30) of...
Induction of Apoptosis of Ewing`s Sarcoma Cells by Regulating Fusion Protein Expression
Ho Keun Yi, Ran Ju Kim, Dae Yeol Lee, Jung Soo Kim, Pyoung Han Hwang
Clin Exp Pediatr. 2000;43(7):942-951.   Published online July 15, 2000
Purpose : Fusion genes(EWS-Fli-1 and EWS-erg ) function as transcription activators and are essential for maintaining tumorigenic properties in Ewing`s sarcoma cells. Several reports have noted that Ets family transcription factors bind with CBP(CREB binding protein) in vitro. To understand the interaction of fusion proteins and CBP, we studied the CBP protein in TC135 cells expressing the EWS-Fli-1 gene. We...
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