• Search
  • Advanced Search

Search

  • HOME
  • Search
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...
A Study on the Disease Course and Prognosis of Cyclic Vomiting Syndrome
Hye Ran Yang, Jae Sung Ko, Jeong Kee Seo
Clin Exp Pediatr. 2001;44(10):1141-1156.   Published online October 15, 2001
Purpose : To describe clinical features of cyclic vomiting syndrome(CVS), managements during episodes, prophylactic drugs as long term therapy and to investigate the disease course and prognostic factors of CVS. Methods : Thirty two children who fulfilled the diagnostic criteria of CVS and who presented to Seoul National University Children's Hospital from March 1989 to December 2000, were included. Data were...
Case Report
Malignant Ectomesenchymoma in a Two-Month-Old Boy
Ji Young Hwang, Ji Hyoun Seo, Jeum-Su Kim, Young-Suk Kim, Sue-Jin Lee, Chan-Hoo Park, Yoo Kyung Kim, Sun Hoo Park, Jung Hee Lee, In Oak Ahn, Ki Hyun Chung, Byung-Kiu Park
Clin Exp Pediatr. 2001;44(8):959-964.   Published online August 15, 2001
Malignant ectomesenchymoma is a rare tumor originating from remnants of migratory neural crest(ectomesenchyme) and composed of neuroectodermal as well as mesenchymal components. Neuroblasts and ganglion cells constitute the neuroectodermal components and rhabdomyosarcoma is the most frequently encountered mesenchymal components. We report a case of malignant ectomesenchymoma in a two-month-old boy who was presented with abdominal pain and urinary difficulty. The...
Original Article
The Effectiveness of Propofol on Pediatric Cardiac Catheterization and Electrophysiologic Study
Woo Saeng Park, Myung Chul Hyun
Clin Exp Pediatr. 2001;44(7):773-777.   Published online July 15, 2001
Purpose : Our purpose was to evaluate the effectiveness of propofol on pediatric cardiac catheterization or radiofrequency catheter ablation. Methods : We measured the serial changes of arterial oxygen saturation, heart rate, systolic and diastolic blood pressure at 4 stages, a baseline(during normal sleep), stage 1(just after loading of propofol 2 mg/kg over 10 minutes), stage 2(10-15 minutes after propofol continuous...
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...
Long-term Clinical Follow-up of Korean Children with Wilson Disease; Twenty Years` Experience
Jin Soo Moon, Jae Sung Ko, Jeong Kee Seo
Clin Exp Pediatr. 2001;44(2):127-138.   Published online February 15, 2001
Purpose : To investigate the clinical features of long-term follow-up, and the treatment responses in Korean children with Wilson disease(WD). Methods : The clinical data from ninety-two children with WD, diagnosed at Seoul National University Hospital from 1976 to 1999, were reviewed. Mean duration of follow-up was 5¡¾4.5 years. We analysed the patient's records based on the clinical manifestations, laboratory findings,...
The Correlation Between the TSH Level in Neonatal Screening Test and the Prognosis of Congenital Hypothyroidism
Hong Sun Park, Kye Shik Shim, Kyuchul Choeh
Clin Exp Pediatr. 2001;44(1):25-31.   Published online January 15, 2001
Purpose : It is important to diagnose and treat newborn patients with congenital hypothyroidism as soon as possible because of neurodevelopmental outcome. If we can detect more severe forms of congenital hypothyroidism with neonatal screening test, the results of treatment will improve. Methods : Sixty-four term infants whose TSH levels in neonatal screening test had been higher than 20μIU were recalled....
Changes of Peripheral IFN-γ-Producing CD4(＋) and CD4(－) Cell Frequencies in Kawasaki Disease
Joung-Min Eom, Heon-Seok Han, Youn-Soo Hahn
Clin Exp Pediatr. 2000;43(10):1350-1355.   Published online October 15, 2000
Purpose : This study was performed to analyze the frequencies of peripheral IFN-γ-producing cells at the single cell level, and to determine concentrations of circulating interferon-γ(IFN-γ) in the acute and subacute phases of Kawasaki disease(KD). Methods : Ten patients with KD were studied and seven healthy children were selected as control subjects. Using immunofluorescent detection of intracellular IFN-γ in CD4(＋) and...
Case Report
A Case of Prader-Willi Syndrome with FUO Diagnosed at Infancy
Jun Ho Kim, Jin Hwa Jeong, Sung Min Cho
Clin Exp Pediatr. 2000;43(8):1142-1148.   Published online August 15, 2000
Prader-Willi syndrome(PWS) was first described by Prader et al in 1956. This syndrome is characterized by diminished fetal activity, low birth weight, infantile hypotonia with feeding problem, temperature instability, early onset of childhood hyperphagia with consequent obesity, short stature, hypogonadism and mental retardation. The deletion of chromosome 15(del 15(q11-13)) was reported by Ledbetter in 1981, which was thought to be...
A Case of Relapsed Acute Promyleocytic Leukemia Induced Remission with Arsenic Trioxide(As2O3)
Jin Hye Park, Heung Sik Kim, Chin Moo Kang
Clin Exp Pediatr. 2000;43(8):1132-1136.   Published online August 15, 2000
Acute promyelocytic leukemia(APL) is a unique entity in the spectrum of acute myelogenous leukemia. It has several characteristic features, including distinctive morphology, chromosomal translocation, t(15:17), disseminated intravascular coagulation and effect on retinoic acid. Retinoic acid which is a derivative of vitamin A induces differentation of APL cells in vitro and in vivo, but its cessation induces relapse of APL. Arsenic...
Original Article
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...
Effect of Growth Hormone Secretion on Serum Concentration of Leptin
Se-Eun Kim, Jeong-Nyeo Lee, Sang-Ook Nam, Woo-Yeong Chung
Clin Exp Pediatr. 2000;43(7):959-966.   Published online July 15, 2000
Purpose : To investigate the effect of growth hormone(GH) secretion on serum concentration of leptin, we evaluated the differences in serum concentration of leptin between patients with growth hormone deficiency(GHD) and GH normal short stature in prepubertal male children, and the relationship between GH and serum leptin level. Also, we analyzed the correlation between serum concentrations of leptin and insulin-like growth factor-I(IGF-I), insulin-like growth...
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...
Mutations in Hepatitis B Virus Precore and Core Promotor in Children with Chronic Hepatitis B Infection - Comparison Between Vertical and Non-vertical Transmission -
Jin Kuk Kim, Yeong Hong Park, Woo Yeong Chung, Chul Ho Kim
Clin Exp Pediatr. 2000;43(6):779-791.   Published online June 15, 2000
Purpose : The aims of this study were to investigate the frequencies and role of hepatitis B virus(HBV) precore and core promotor mutations in children with chronic hepatitis B infection. Methods : Sera from 46 children with chronic hepatitis B infection were analyzed by direct sequencing of polymerase chain reaction product of HBV DNA. In this study, the patients were divided...
Protective Effect of Probucol against Adriamycin-Induced Apoptosis in Cultured Rat Cardiac Myocytes
Sun-Ju Lee, Dong-Seok Lee, Yong-Wook Jung
Clin Exp Pediatr. 2000;43(6):746-754.   Published online June 15, 2000
Purpose : In adriamycin(ADR)-induced cardiomyopathy, several different mechanisms are suggested. However, little information is available regarding the role of apoptosis. In the present study, we examined the induction of apoptosis on ADR treatment and anti-apoptotic effects of probucol, a lipid-lowering drug, and we also studied the changes of bcl-2 expression in order to see the molecular mechanisms underlying the effect...
Apolipoprotein E Polymorphism and Plasma Lipid Levels in Obese Children
Myoung Hee Han, Sun Jun Kim
Clin Exp Pediatr. 2000;43(5):679-685.   Published online May 15, 2000
Purpose : The increased prevalence of dyslipoproteinemia in obese children probably contributes to the high risk of cardiovascular disease associated with being overweight. The genetic makeup is possible one of the factors that influence the impact of obesity on lipid metabolism. A relation between apolipoprotein E(Apo E) polymorphism and lipid metabolism has been convincingly demonstrated in large population. The purpose...
Comparative Reactivity of 2 TU and 5 TU PPD Skin Tests
Dae Sun Jo, Kyeong Mee Lee, Dae-Yeol Lee, Jung Soo Kim
Clin Exp Pediatr. 2000;43(5):638-643.   Published online May 15, 2000
Purpose : These days 5 TU purified protein derivative(PPD) is substituted with 2 TU PPD for the tuberculin skin test, a test essential in the diagnosis of tuberculosis in pediatric population. However, there had been no adequate criteria for the positivity of 2 TU PPD skin test. This study was performed to clarify the criteria of positive reactivity of 2...
Study of Autoantibodies Against Platelet GP IIb/IIIa and GP Ib/IX in Childhood Chronic Idiopathic Thrombocytopenic Purpura
Chanwook Woo, Junghwa Lee, Kwangchul Lee, Soonkyum Kim
Clin Exp Pediatr. 2000;43(3):395-401.   Published online March 15, 2000
Purpose : Chronic idiopathic thrombocytopenic purpura(CITP) is an autoimmune disease caused by autoantibodies reacting to certain antigens, and platelet glycoprotein(GP) IIb/IIIa and GP Ib/IX complexes are thought to be some of those antigens. However, the clinical significance of anti-GP autoantibodies in CITP patients is unknown. In this study, we investigated the clinical correlation between the presence of circulating autoantibodies against...
Physiological Effects of Kangaroo Care(skin-to-skin care) in Small Preterm Infants and Their Parents
Heng-Mi Kim, Soo-Young Kim
Clin Exp Pediatr. 2000;43(3):365-371.   Published online March 15, 2000
Purpose : Kangaroo care is the practice of holding a small premaure infant, naked except for a diaper and hat, against a parent's chest. The purpose of this study is to determine the safety and feasibility of kangaroo care in a NICU as defined by physiological variables. Methods : Ten stable, spontaneously breathing preterm infants, weighing less than 2,000gm were...
Anticancer Effect of Arsenic Trioxide in Acute Promyelocytic Leukemia
Ki Young Song, Jin Hye Park, Yoon Jung Cho, Won Ki Baek, Ki Young Kwon, Heung Sik Kim, Chin Moo Kang
Clin Exp Pediatr. 2000;43(3):327-334.   Published online March 15, 2000
Purpose : Acute promyelocytic leukemia(APL or AML, M3) represents an unique model for cancer research in terms of biological and clinical features. Since 1988, it has been widely confirmed that all-trans retinoic acid(ATRA) can induce complete clinical remission in over 85% of APL patients by a differentiation process, with PML-RARα protein possibly being the direct target of ATRA. However, ATRA...
The Improvement of Cell Viability due to Dilution and Removal of DMSO in Thawing of Stem Cells
Hoon-Kyung Lee, Kyung-Ha Ryu, Il-Tae Whang, Eun Suk Kang, Ki Sook Hong, Kyung-Hyo Kim, Young-Mi Hong, Kyung-Hee Kim, Keun Lee
Clin Exp Pediatr. 2000;43(2):241-246.   Published online February 15, 2000
Purpose : Cryopreserved stem cells from cord blood are usually infused with Dimethyl Sulfoxide (DMSO) immediately after thawing. However, this process may cause cell damage due to osmotic shock, and the administration of DMSO may also cause toxic effects. We studied a new method of increasing cell viability by stabilizing osmolarity by adding dextran 40 and washing out DMSO. Methods :...
Construction and Application of Painting probe for Chromosome band 5q14-q15, 6q23, 16p11.2 and 22q12
Soo-Yeun Park, So Young Park, Joon-Il Kil, Yong-Hyuck Chun, Sun-Hwa Park
Clin Exp Pediatr. 2000;43(2):179-187.   Published online February 15, 2000
Purpose : Chromosome microdissection has become a very powerful approach to generate chromosome band-specific library and painting probes for physical mapping or cytogenetic analysis. We have constructed here band-specific painting probes for human chromosomes by microdissection and polymerase chain reaction(PCR). Methods : We pretreated the microdissected fragments with Topoisomerase I(Topo I) which catalyzes the relaxation of supercoiled DNA, and performed...
Apoptosis, P53, bax and Bcl-2 Protein Expressions in Neonatal rat Hippocampus by Kainic Acid-induced Seizure
Shin Weon Yun, Soo Ahn Chae, Eung sang Choi, Byoung Hoon Yoo
Clin Exp Pediatr. 2000;43(1):85-96.   Published online January 15, 2000
Purpose : Apoptosis is a process of active cell death which has been suggested to be part of hippocampal cell loss caused by kainic acid(KA). Immature rats showed higher susceptibility and mortality to KA but did not develop recurrent seizure, long term behavioral or neuropathologic changes. We investigated whether this was due to age-dependent resistance, and elucidated the molecular mechanics which mediate P53-induced apoptosis,...
Case Report
Follow up of a Case of Primary Intestinal Lymphangiectasia
Su Jeong Ha, Soo Jung Lee, Dae Kyun Koh
Clin Exp Pediatr. 1999;42(10):1452-1458.   Published online October 15, 1999
Primary intestinal lymphangiectasia is a rare congenital disorder characterized by diffuse or localized ectasia of the enteric lymphatics, often in association with lymphatic abnormalities elsewhere in the body. The pathogenesis of these abnormal lymphatic structures is uncertain. Ectatic lymphatics may be located in the mucosa, submucosa, or subserosa, leading to a loss of protein and lymphocytes into the gut or...
  • PubMed Central
  • PubMed
  • Scopus
  • Directory of Open Access Journals (DOAJ)